Mural nodules of ovarian mucinous borderline tumors are rare. In this study, we report a case of mural nodules of clear cell carcinoma in an intestinal type mucinous borderline tumor of the ovary. The patient was a 54-years-old woman presented with back and pelvic pain for 3 months. A right-sided multiloculated ovarian mass approximately 20 cm was identified on the CT scan. CA-125 was moderately elevated. She underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and bilateral pelvic and para-aortic lymphadenectomy. Grossly, the right ovarian mass showed a multiloculated cystic mass with mucinous fluid. There were papillations in the internal surface and two mural nodules were seen. Microscopic examination revealed that the cystic mass was an intestinal type borderline mucinous tumor. The mural nodules showed a classic histology of clear cell carcinoma with tubulocystic and papillary growth patterns. This is an extremely rare case of mural nodules of clear cell carcinoma arising in a mucinous borderline tumor. Mucinous ovarian tumors were first described in 1809 by Ephraim McDowell . BorderlWe hereby report a case of mural nodules of clear cell carcinoma in an intestinal-type borderline mucinous ovarian tumor. Although clear cell carcinomas have been found in association with mucinous cystadenomas and endometriosis in the past –10, to oThe patient was a 54-years-old G2P2 caucasian woman with a past medical history of hypertension, hyperlipidemia, hernia, obesity and laparoscopic cholecystectomy. She complaint of 3 months of pelvic and back pain. The pain was described as aching, occurring on daily basis and 4/10 in an scale of severity. She also had early satiety, occasional nausea, and abdominal distention for the past 6 months. She had vaginal spotting for the last 2 weeks at the moment of presentation.Physical examination revealed a large pelvic-abdominal mass, approximately 20 cm in greatest dimension. Abdominal-peilvic CT scan revealed a 20 × 10 × 7 cm right multiloculated ovarian mass. CA-125 was 43 U/ml . The patient was scheduled for surgery. A exploratory laparotomy was performed with frozen section consultation. The intraoperative diagnosis was at least borderline mucinous ovarian neoplasm. A total abdominal hysterectomy, bilateral salpingo-oophorectomy, and bilateral pelvic and aortic lymphadenectomy were performed. Ovarian staging, including multiple biopsies and pelvic washings, was done. After surgery, she started 6 cycles of chemotherapy with carboplatin/taxol. At present, patient was on her second cycle without any complication related to the treatment.The right ovary was enlarged and completely distorted by a mass which measured 25 × 22 × 15 cm and weighted 5.2 kg. The ovary had attached 7 cm in length fallopian tube which was unremarkable. The external surface of the ovary was intact, smooth, and glistening. Upon serial sectioning, the ovarian parenchyma was completely replaced by a multiloculated cystic mass with mucinous fluid inside the cystic spaces. The internal surface of the cyst was mostly smooth with areas of prominent friable papillae. Two solid nodules, representing mural nodules, were identified and measured 5 and 3 cm in greatest dimension . These mThe ovarian tumor was composed by multiple cystic spaces and glands with variable size and shapes; some were lined by a single layer of intestinal-type epithelium, resembling mucinous cystadenoma. Multiple delicate papillary infoldings were identified. The cells in those areas were more crowded, stratified in two or more layers with loss of the polarity. Occasional mitotic figures and mild to moderate cellular atypia were seen . Severe The mural nodules showed abrupt transition from the surrounding mucinous borderline tumor and microscopically displayed a tubulocystic, papillary, and focally solid proliferation of neoplastic polyhedral cells with abundant clear cytoplasm . The nucThe tumor was diagnosed as intestinal borderline mucinous tumor with mural nodules of clear cell carcinoma.Mucinous ovarian tumors are among the most difficult ovarian tumors for surgical pathologist to interprete. In the current WHO classification of ovarian neoplasms, mucinous tumors are classified within the surface epithelial-stromal tumor category . Unlike Even though rare, mural nodules have been described in benign, borderline, and malignant mucinous ovarian tumors. The mural nodule could be benign  or malignant in nature , 4. BaguClear cell carcinoma was first described in 1939 by Schiller . The terDutt and Berney reported for the first time a clear cell carcinoma arising in a mucinous cystadenoma, also with associated endometriosis . This reIn our case, the mural nodules showed characteristic features of clear cell carcinoma with tubulocystic and papillary architectures. The neoplastic cells had abundant clear cytoplasm and moderate to severe atypia. The stroma was extensively hyalinized as it is frequently described in this entity. The intestinal type borderline mucinous tumor areas were classical, showing cystic spaces with papillae lined by focally stratified intestinal-type epithelium and without evidence of stromal invasion. The overall gross appearance and microscopic findings in both the borderline tumor and clear cell carcinoma are classic; we feel that immunohistochemical or special studies are not necessary for the diagnosis.In summary, we have reported an exceedingly rare case of clear cell carcinoma arising in an ovarian mucinous borderline tumor of the intestinal type. Careful gross examination and extensive sampling are crucial for the correct diagnosis. Since malignant neoplasm within mural nodules has a significant impact in the prognosis of the patients, pathologists have to be able to identify these mural nodules accurately.


Primary hepatic carcinoid tumor (PHCT) is very rare and difficult to diagnose before biopsy or operation. We report a patient with a small PHCT and review cases in the literature.A 48-year-old Chinese female with underlying hepatitis B virus (HBV) infection was found to have a low echoic hepatic nodule by abdominal ultrasound. Tumor markers were negative. Dynamic liver computed tomography scans showed enhancement of the nodule in the arterial phase and early washout in the portal phase. Hepatocellular carcinoma (HCC) was considered based on the image findings and underlying HBV infection. However, the tumor biopsy revealed a malignant neoplasm that originating from neuroendocrine cells. Pre-operative and intra-operative investigations for the possible other origin of carcinoid tumor were negative, so PHCT was confirmed.A small and asymptomatic PHCT is extremely rare. PHCT should be one of the differential diagnoses in patients with small hepatic tumor, even in regions with high prevalence of HBV infection and HCC. Pre-operative biopsy is necessary to avoid misdiagnosis even when HCC is highly suspected clinically. Carcinoid tumors are neuroendocrine origin neoplasm and may produce serotonin or other functional peptide hormones. They are well-differentiated and low-grade malignant neoplasms. Approximately 74% of all carcinoid tumors arise from the gastrointestinal (GI) tract and the liver is a common site for metastases . HoweverA 48-year-old Chinese female with HBV infection for 15 years was regularly followed up at gastroenterology outpatient department by abdominal ultrasound (US) and serum alpha-fetoprotein (AFP) screening every six months. In May 2005, abdominal US demonstrated a low echoic nodule, 1.6 × 1.6 cm in size in the sixth segment of the liver and mild parenchymal liver disease Fig. . She wasThe origin of PHCT remains unknown. The cells may originate from ectopic pancreatic or adrenal tissue found within the liver or from scattered neuroendocrine cells in the intrahepatic biliary epithelium . Some weIncluding our case, we found 94 cases of PHCT (including primary hepatic neuroendocrine tumor) in the literature -5,7-15 aThe case we presented was a small and asymptomatic PHCT. In the literature, only 9.5% (7/74) and 13.1% (11/84) of cases were small-sized tumors (≦ 3 cm) and asymptomatic PHCTs, respectively. Their prognosis was good and the survival rate was 100 % for the 7 small-sized tumor cases and 90.1% (10/11) for the asymptomatic cases, only one of which died as a result of renal cell carcinoma.The rarity of PHCT makes it difficult to diagnose accurately before biopsy or resection. In previous reports, 4 4.3%) and 5 (5.3%) cases were diagnosed as HCC -10 and c% and 5 (A small and asymptomatic PHCT is extremely rare. PHCT should be one of the differential diagnoses in patients with small hepatic tumor, even in regions with high prevalence of HBV infection and HCC. Pre-operative biopsy is necessary to avoid misdiagnosis even when HCC is highly suspected clinically.PHCT: Primary hepatic carcinoid tumor; HBV: Hepatitis B virus; HCC: Hepatocellular carcinoma; GI: Gastrointestinal; US: Ultrasound; CT: Computed tomography; AFP: Alpha-fetoprotein; IHC: Immunohistochemical.Written informed consent was obtained from the patient for publication of this case report, photographic and radiographic images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.CL analyzed and interpreted the patient data and was a major contributor in writing the manuscript; CH analyzed and interpreted the patient data and was a major contributor in writing the manuscript; CH performed the histological examination of the liver biopsies and provided the histological images; HP performed the images investigation of the patient and was a major contributor in writing the manuscript; KH performed the images investigation of the patient and was a major contributor in writing the manuscript; CL contributed to the writing and revising of the manuscript; YC contributed to the writing and revising of the manuscript.All authors read and approved the final manuscript.


Internal hernias are a rare cause of small bowel obstruction and are estimated to account for 1% to 5% of cases. Herniation through a defect in the sigmoid mesocolon constitutes 6% of all internal hernias.In this case report we describe a rare case of a fit and healthy 60-year-old man, with no previous history of abdominal surgery, who presented with signs and symptoms of small bowel obstruction as a result of an incarcerated transmesosigmoid hernia. The hernia was reduced and the incarcerated loop of small bowel was found to be viable. The patient made a good recovery and was discharged home on the fourth post-operative day.Internal hernias can cause considerable morbidity and mortality, so prompt diagnosis is paramount. Transmesosigmoid hernias are most common in the paediatric population; however, our patient was 60 years old. This report highlights the importance of considering an internal hernia as a cause of small bowel obstruction in individuals of all age groups and especially in those without a previous history of abdominal surgery. Small bowel obstruction is a frequent surgical emergency. The most common causes of small bowel obstruction are post-operative adhesions and abdominal wall hernias .In patients without a previous history of abdominal surgery other, less-common causes should be considered. Internal hernias are an infrequent cause of small bowel obstruction and are estimated to account for 1% to 6% of all cases.9g/dl. Plain abdominal radiography demonstrated two prominent loops of small bowel in the right lower quadrant. He was initially managed conservatively. A Ryle's nasogastric tube was inserted and he was resuscitated with intravenous fluids. A computed tomography (CT) scan of his abdomen and pelvis (Figure A 60-year-old man presented acutely with a 1-day history of colicky central abdominal pain, abdominal distension, vomiting and absolute constipation. He had no previous history of abdominal surgery. Physical examination revealed dry mucous membranes and tenderness in the right lower quadrant. Bowel sounds were increased and digital rectal examination was normal. Laboratory investigations revealed a leucocytosis of 11.4×10At laparotomy, a mechanical small bowel obstruction due to an incarcerated internal hernia was found. A loop of ileum had herniated through a congenital defect in the sigmoid mesocolon Figure . This waAn internal hernia is the protrusion of a viscus through a normal or abnormal peritoneal or mesenteric aperture within the abdominal cavity. It is estimated to account for 0.6% to 5.8% of small bowel obstructions with transmesosigmoid hernias constituting 6% of all internal hernias .Previously, paraduodenal hernias were regarded as the most common type of internal hernia; however, it has recently been reported that transmesenteric hernias are increasing in incidence . TransmeCongenital internal hernias of the sigmoid mesentery are divided into three categories: intersigmoid, intramesosigmoid and transmesosigmoid . TransmeIn cases of transmesosigmoid hernias, patients tend to present acutely with abdominal pain and signs of small bowel obstruction ,9. HowevThe management of internal hernias requires reduction of the hernia and repair of the defect by either a laparoscopic or open approach. In these cases there is a high incidence of small bowel ischaemia and infarction and resection of the strangulated small bowel segment may be necessary. Iatrogenic internal hernias can be successfully managed by laparoscopy  and lapaThis case highlights a rare cause of intestinal obstruction in a patient with no previous abdominal surgery. Interestingly, congenital internal hernias are more common in the paediatric population . The curThe authors declare that they have no competing interests.DC drafted the article, performed the literature search and acquired the radiology images. DK assisted in performing the surgery, acquired the intraoperative images and reviewed the manuscript. EM supervised and edited the manuscript. SR and EMcD performed the surgery. All authors have read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Dentin dysplasia is a rare hereditary disturbance of dentin formation characterized by defective dentin development with clinically normal appearing crowns, severe hypermobility of teeth and spontaneous dental abscesses or cysts. Radiographic analysis shows obliteration of all pulp chambers, short, blunted and malformed or absent roots and peri-apical radiolucencies of non carious teeth.We present a case of dentin dysplasia type I in a 12-year-old Iranian boy, and the clinical, radiographic and histopathologic findings of this condition and treatment are described.There are still many inconclusive issues in the diagnosis and management of patients with dentin dysplasia. The diagnostic features of this rare disturbance will remain incompletely defined until additional cases have been described. Early diagnosis of the condition and initiation of effective regular dental treatments may help these patients to prevent or delay loss of dentition. Dentin dysplasia (DD) is an autosomal dominant hereditary disturbance in dentin formation, which may present with either mobile teeth or pain associated with spontaneous dental abscesses or cysts. It is a rare anomaly of unknown etiology that affects approximately one patient in every 100,000  but it wThis article describes an uncommon case of DD type I, subtype 1a, in a 12-year-old Iranian boy, highlighting the clinical and radiographic variations of the defect as confirmed by histopathologic examination.A 12-year-old Iranian boy was referred to the Pedodontics Department of Shaheed Beheshti University Medical Sciences, Dental School due to excessive painful swelling of his mandibular left cheek region. Clinical examination showed that the following teeth were present in his mouth:7654321 12 C45677654321 12 C4567The crowns of his teeth had normal morphologic characteristics, but the color of his teeth was slightly more yellow than expected for a patient of his age Figure . Oral hyOn the basis of the clinical and radiographic appearance, a diagnosis of DD type I, subtype 1a, was suspected. Clinical and radiographical examination of the patient's parents and siblings revealed no cases of DD type I, and there were no previous cases of this disturbance in the familial history. The following treatment plan was formulated: dietary and oral hygiene instructions, fluoride supplements, surgical enucleating of the cystic lesion at the left first permanent molar region, restoration of the carious teeth, and extraction of the left primary canine and primary first molar, which were carious and mobile.The cystic lesion was enucleated and sent for histopathological examination. Based on the pedodontist's and surgeon's view, it was decided to extract the remaining root of the lower left first permanent molar during surgery because of extensive caries. The histopathological features were consistent with the clinical diagnosis of a radicular cyst. The cystic cavity was lined with a variable thickness of non keratinized stratified squamous epithelium with arch-shaped appearance and exocytosis in the underlying connective tissue was severely infiltrated by chronic inflammatory cells. Extravasated red blood cells and hemosiderin pigments were also seen Figure . The extet al. [et al. [The pathogenesis of DD is still unknown in the dental literature. Logan et al.  proposed [et al.  proposed [et al. , but in  [et al. . Some as [et al.  but our There were no variations in the morphology of the affected teeth in our patient but there are reports that have suggested possible variations in the morphology of teeth affected by this type of dysplasia ,12. HistManagement of patients with dentinal dysplasia has presented dentists with many problems. Extraction has been suggested as a treatment alternative for teeth with pulp necrosis and peri-apical abscess. Follow-up and routine conservative treatment is another choice of treatment plan in DD . AnotherDentin dysplasia type I is a rare inherited abnormality of the dentin that leads to premature exfoliation of the primary and permanent teeth. Early diagnosis of the condition is important for initiation of effective preventive treatment. In this regard, the pediatric dentist has an important role in the early diagnosis of this disorder and in guiding patients in the selection of measures to prolong the retention of affected teeth.DSPP: dentin sialophosphoprotein.Written informed consent was obtained from the patient's parents for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.LT wrote and supervised the manuscript. FM carried out the pathologic issues and took the ground section. MM and LM carried out all dental treatments and completed the literature review.


Peritoneal mesothelioma is a rare tumor with diagnostic and therapeutic problems. The peritoneum is the second most common site for development of mesothelioma, which in 30–45% of cases is associated with a synchronous pleural mesothelioma. Clinical symptoms and findings may be confusing and diagnosis can be easily overlooked especially in cases where there is no previous asbestos exposure.We report a case of malignant peritoneal mesothelioma in a 75-year-old woman who presented with ascites which, in the absence of inhalational exposure to asbestos, caused diagnostic confusion, and evaded radiological detection.We concluded from this case that Peritoneal Mesothelioma although rare but should be considered among the differential diagnosis of Ascites. Malignant mesothelioma of the peritoneum is rare but rapidly fatal malignancy. The median survival rang from 5 to 12 months, mainly because of lack of effective treatment. The incidence is approximately one per 1,000,000; approximately one fifth to one third of all mesotheliomas are peritoneal. Half of reported cases have a history of asbestos exposure. The diagnosis of peritoneal mesothelioma is often delayed, in part because of the usually long latent period  and because the common presenting symptoms of weight loss, usually with a full abdomen, malaise, and abdominal discomfort, are mild and nonspecific. Because of its unusual nature, the disease has not been clearly defined in terms of its natural history, diagnosis, or management. Treatment options with intravenous chemotherapy are far from satisfactory. However, because malignant peritoneal mesothelioma usually remains confined to the peritoneal cavity for most of its natural history, regional chemotherapy is an attractive option.A 75-year-old woman was admitted with a five-week history of progressive dyspnoea, ascites, leg edema and lethargy. She had a previous history of pulmonary tuberculosis 50 years earlier and hypertension which was well controlled with atenolol. She had never smoked and had no known previous exposure to asbestos. She had a family history of lung and large bowel cancer but no history of mesothelioma.Clinical examination revealed bilateral pedal pitting edema but no signs of chronic liver disease. She had moderate ascites but normal cardiovascular and respiratory system examinations. Blood investigations including full blood count (FBC), urea and electrolytes, liver function tests (LFTs), inflammatory markers, hepatitis, viral and autoimmune screens were all normal. Initial ultrasound scan and computed tomography (CT) scan of the abdomen and pelvis were unable to demonstrate any significant abdominal pathology other than ascites. A diagnostic ascitic tap yielded transudate with a serum ascites-albumin gradient (SA-AG) of 32. Ascitic fluid culture and culture for acid-fast bacilli (AFB) were also negative. The ascites recurred despite multiple drainages. She had a repeat CT scan which showed extensive peritoneal nodularity and omental cake along with massive ascites. A diagnostic laparoscopy with omental biopsy was performed. It showed infiltration of the omentum with a poorly differentiated malignant tumor consisting of sheaths of cells with fairly uniform nuclei containing prominent nucleoli Figure .A barium enema and flexible sigmoidoscopy showed no bowel pathology apart from mild diverticular disease with pedunculated polyps which were found to be benign on histology. Immunochemical staining of the biopsy showed features consistent with diffuse mesothelioma. She was referred to the oncology department after a multidisciplinary meeting discussion and chemotherapy was planned with cisplatin and pemetrexed. She responded well initially and the ascites resolved with improved general wellbeing, but the treatment was later stopped when she developed side effects in the form of recurrent vomiting and general malaise. Currently she is having palliative treatment and is under regular oncology follow up.Mesothelioma is a neoplasm originating from the mesothelial surface lining cells of serous cavities. Most often, mesotheliomas involve the serosal membranes of the pleura and peritoneum. Sometimes mesothelial proliferations are identified in other locations. On rare occasions, it has been reported in the tunica vaginalis of the testis ,2. AmongSymptomatology is insidious and poses difficult problems in diagnosis and treatment. People with peritoneal mesothelioma generally present with one of two types of symptoms and signs: those with abdominal pain, usually localized and related to a dominant tumor mass with little or no ascites, and those without abdominal pain, but with ascites and abdominal distention .Ultrasonography and CT scan of the abdomen can provide important information during the diagnostic process. Nevertheless, a definite diagnosis can only be established by laparoscopy or open surgery with biopsy to obtain histological examination along with immunocytochemical procedures. Laparoscopy is an important tool in the diagnosis of some unusual causes of ascites, such as primary mesothelioma, which are usually overlooked by other diagnostic modalities, such as ultrasound, CT and cytology of the ascitic fluid. However, laparoscopy can greatly complicate management by facilitating tumor dissemination to port sites .Pathologically, a positive immunostain for calretinin has markedly increased the accuracy of diagnosis . PrognosOver the past decade, the management of these patients has evolved similarly to ovarian cancer treatment and now involves cytoreductive surgery, heated intraoperative intraperitoneal chemotherapy (HIIC) with cisplatin and doxorubicin, and early postoperative intraperitoneal paclitaxel. These perioperative treatments are followed by adjuvant intraperitoneal paclitaxel and second-look cytoreduction. This multimodality treatment approach with cytoreductive surgery and intraperitoneal chemotherapy has resulted in a median survival of 50 to 60 months ,4.This case denotes the importance of considering the differential diagnosis of common clinical problems. Peritoneal mesothelioma, although uncommon, should be considered in people presenting with ascites, in particular in those where the initial diagnosis is not clear. A history of asbestos exposure may not be present and radiological investigations may miss the diagnosis so thorough clinical assessment and a broad-thinking approach is important.AFB: acid-fast bacilli; CT: computed tomography; FBC: full blood count; HIIC: heated intraoperative intraperitoneal chemotherapy; LFT: liver function test; SA-AG: serum ascites-albumen gradient.The authors declare that they have no competing interests.IA was involved in writing the case, the literature review and obtaining patient consent. WCT was involved in proof reading and final refinement. AK was involved in proof reading and final refinement. JI was involved in the final revision and in making modifications following suggestions by the reviewers.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


A case of squamous cell carcinoma with sarcomatous stroma of the mesopharynx ispresented. The patient was a 62-year-old man who complained of a foreign body sensation. Endoscopic examination revealed a large pedunculated mass arising from the posterior wall of the mesopharynx. The lesion was surgically resected, using a cutting snare by the endo-oral approach, and was completely removed. A diagnosis of squamous cell carcinoma with sarcomatous stroma was made histopathologically. The clinicopathological features of this case are described and compared with those of previously reported cases.


Uterus didelphys with blind hemivagina and ipsilateral renal agenesis (Herlyn Werner-Wunderlich Syndrome) is a rare congenital anomaly. It mostly presents with severe dysmenorrhea and a palpable mass due to unilateral hematocolpos. A patient with dysmenorrhea from a double uterus and an obstructed hemivagina is a diagnostic dilemma because the menses are regular. We report a case of a 14-year-old girl with this condition who was diagnosed as uterus didelphys with unilateral hematocolpos and hydrosalpinx with ipsilateral renal agenesis on the basis of sonography and confirmed by laparoscopic examination. The association of uterus didelphys with unilateral obstructed hemivagina with hematometrocolpos and hematosalpinx with ipsilateral renal agenesis has been recognized. Sonography shows characteristic imaging findings, which we found in a 14- year- old girl presenting with dymenorrhea along with a progressively enlarging pelvic mass associated with normal menstrual cycles.A 14-year-old girl was admitted to our institute with complaints of recurrent pelvic pain, mainly at the time of menses, and a gradually increasing lower abdominal swelling since menarche, which was attained at the age of 12 years. Her menstrual cycles were regular and flow was adequate.General physical examination was unremarkable except for mild pallor. Per abdominal examination revealed a well-defined cystic mass arising from the pelvis.The patient was referred to our department for ultrasonography of abdomen and pelvis (USG), which showed two widely divergent uterine horns with no communication between them . The rigUltrasonography of abdomen and pelvic region revealed an empty right renal fossa. On the basis of imaging findings, a diagnosis of uterus didelphys with unilateral obstructed hemivagina with resultant hematometrocolpos and hydrosalpinx with ipsilateral renal agenesis was made.Vaginal examination under anesthesia showed a large bulge high up on the right lateral wall. A vaginal septotomy was given and 200 mL of chocolate-colored fluid was drained. Subsequent USG revealed normal-sized bilateral uterine cavities and cervices. The cystic pelvic mass however persisted. The patient underwent laparoscopy, which confirmed the diagnosis, and laparoscopic drainage of the hydrosalpinx was performed.rds of the vagina develop from the paired mullerian ducts while the lower 1/3rd of the vagina develops separately from the urogenital sinus. The association of uterus didelphys with obstructed hemivagina with renal agenesis ipsilateral to the side of obstruction can be explained by embryologic arrest at the 8th gestation week, which simultaneously affects the mullerian and metanephric ducts. Certain other renal anomalies may also be associated, such as renal dysplasia, double collecting system and ectopic ureter.[The uterus, fallopian tubes, cervix and upper 2/3Mullerian duct anomalies (MDA) are congenital anomalies of the female genital tract that result from nondevelopment or nonfusion of the mullerian ducts or failed resorption of the uterine septum.5Incidence of these anomalies is believed to be between 0.5 and 5.0%.7Majority of these cases are diagnosed at menarche. Early and accurate diagnosis is vital because untreated cases may develop retrograde tubal reflux and endometriosis. It may also cause impaired fertility and obstetric complications later in life.MDAs are divided into six groups on the basis of the Buttram and Gibbons system.The specific association of uterus didelphys, obstructed hemivagina with unilateral hematocolpos and ipsilateral renal agenesis is well recognized. The obstResection of the vaginal septum is the treatment of choice for obstructed hemivagina. In cases where the obstructed hemivagina reaches the hymeneal ring, a limited resection– marsupialization and insertion of a Foley's catheter may be performed during an initial surgical procedure, allowing the remaining vaginal septum to be removed later. AlternatImaging modalities used to diagnose this condition include ultrasonography, conventional and sonohysterosalpingography and magnetic resonance imaging (MRI). Computed tomography (CT) has a limited role in evaluation of the female pelvis, although the latest three-dimensional multiplanar CTs can diagnose this condition and associated urinary anomalies, it is mostly not preferred because of radiation exposure. Ultrasound is a cheap, noninvasive, widely available imaging modality that accurately helps in the diagnosis of this condition. The vaginal septum however is difficult to visualize on ultrasound and is best shown on MRI.


The incidence of soft tissue tumours, both malignant and benign, is very common. However, the coexistence of 4 types of histopathology is rare and the aim of this article is to present one treated in our Department. An 87-year-old Greek man was treated in our Department for a huge tumour on his back, under local anaesthesia. The pathology report of the specimen referred 4 types of neoplasia. This case represents this incidence in a giant liposarcoma of the back. Soft tissue tumours are very common in the general population. Most of them are benign and they require excision for functional and appearance reasons. In literature the incidence of 4 types of histopathology in a specimen is very rare and our An 87-year-old Greek man, who had been generally active and his past medical history was free of disease, was referred to our hospital for a giant swelling on his back. He has had this tumor since at least 10 years, but he had not undergone an operation as it has been asymptomatic Fig. The patient was referred for a surgical removal of the tumor. Due to his age, he has not taken general anesthesia and the whole operation has been under regional anesthesia. Intraoperatively, a giant liposarcoma, 18 × 14 × 6 cm, was found and been removed and also another tumor, 12 × 10 × 8 cm, white and homogenous Fig. , 4, 5.The pathology report referred 4 types of neoplasia. There was growth of mesenchymal neoplasma with figure of non-differentiated liposarcoma. There was also growth of well-differentiated liposarcoma Fig. , whereasThe perioperative period was uneventful and the patient was discharged home 3 days later.The existence of 4 histological types of neoplasia is very rare and was treated surgically under local anesthesia. Preoperative evaluation of such tumours is essential in order to determine the borders and characterize the tumour . This caThe authors declare that they have no competing interests.PP analyzed and interpreted patient's files and wrote the paper. PK and ES contributed in the writing and also assisted at the operation. AL and HG performed the histological examination. PM assisted at the operation and looked after the patient postoperatively. TP was the responsible professor for the patient, decided and performed the operation.All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


We describe an exceptional case of non-fatal septic rupture of the ascending aorta in a patient with sternal dehiscence, deep sternal wound infection (DSWI) and pleural empyema after aortocoronary bypass surgery. Routine follow-up computed tomography (CT) detected a mediastinal pseudoaneurysm originating from the ascending aorta. Thereby, massive and irregular sternal bone defects and contrast-enhancing mediastinal soft tissue suggest osteomyelitis and highly-active and aggressive DSWI as initial triggers. Urgent thoracotomy 1 day later included ascending aorta reconstruction, total sternum resection and broad wound debridement. Follow-up CT 1 year later showed a regular postoperative result in a fully recovered patient. Sternal dehiscence is a frequent complication after aortocoronary bypass surgery with an incidence estimated at 1% to 6% in a normal population . Broken A 65-year-old male white was referred to our department for routine follow-up CT for sternal dehiscence, DSWI and pleural empyema developing after aortocoronary bypass operation 9 months before. His history included congenital portal vein stricture with recurrent ascites, acute myocardial infarction 4 months ago, systemic hypertension, hypercholesteremia, renal insufficiency, penicillin allergy and methicillin-resistant staphylococcus aureus in the blood culture. Medication consisted of spironolactone, metoprolol succinate, furosemide, ramipril, acetylsalicylate, zinc aspartate and zolpidem tartrate. Physical examination was carried out in the Department of Cardiac Surgery right before the CT and revealed pressure-like pectoral pain and non-physiological sternal movement. Laboratory showed elevated c-reactive protein (58 mg/l), ferritin 1450 μg/l) and gamma-glutamyl transferase (77 U/l), whereas choline esterase (3110 U/l) and hemoglobin 12.5 g/dl) were reduced. Electrocardiogram and tropoine T were unremarkable. CT detected massive sternal bone defects, inhomogenous bone matrix and irregular sternotomy edges as evidence for long-lasting chronic osteomyelitis (Figure 450 μg/l .5 g/dl wMidline sternotomy is by far the most common surgical approach in cardiac surgery. One disadvantage of this technique is the relatively frequent occurence of sternal dehiscence with instability . It is dWritten informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.CMS conceived the study. TH prepared the figures. CMS and TH performed the CT-examination. CMS and US did the background literature search and drafted the manuscript. UT was the cardiac surgeon. BAR participated in study design and coordination. HUK and GMR were involved in the conception of the study and the critical review of the intellectual content of the manuscript. All authors have read and approved the final manuscript.


Intraductal and invasive adenocarcinoma of duct of Luschka is rare. To the best of our knowledge, this is the second case report of intraductal and invasive carcinoma arising from ducts of Luschka.Patient presented to hospital with signs and symptoms of chronic cholecystitis and cholelithiasis. Ultrasound examination revealed thickening of gallbladder wall with abnormal septation around liver bed. Patient underwent laparoscopic cholecystectomy and resection of the adjacent liver bed. Histologic examination confirmed an intraductal and invasive adenocarcinoma arising from Luschka ducts.Adenocarcinoma of ducts of Luschka should be considered among differential diagnoses for the patients with typical clinical presentations of chronic cholecystitis and cholelithiasis. Initially described by Herbert von Luschka, the ducts of Luschka are aberrant small bile duct or ductules in liver bed and/or in sub-peritoneal region around wall of gallbladder adjacent to liver bed. The incidence of duct of Luschka varies from 1 percent to 50 percent . Florid A 31-year year old Hispanic women presented in a family practice clinic with severe right upper quadrant pain for past several week. The abdominal pain was accompanied by nausea, vomiting and loss of appetite. Physical examination revealed tenderness in the right upper quadrant. The patient gave history of two admissions in a local ER for similar complaints where she received medical treatment. An ultrasound examination showed gallstones, thickening of gallbladder wall with abnormal septation around liver bed and congenital absence of right kidney. A pre-operative diagnosis of cholecystitis and cholelithiasis was rendered and an MRI was recommended for further evaluation of the thickened wall of gallbladder fundus in liver bed. However, the patient underwent laparoscopic cholecystectomy and resection of the adjacent liver bed. Intraoperative findings included densely adherent gallbladder in the liver bed and a thickened gallbladder fundic wall in and around cholecystic fossa. Frozen section of the thickened area was interpreted as invasive carcinoma. Patient underwent a second look exploratory laparotomy and additional resection of the liver bed. No residual intraductal or invasive adenocarcinoma was seen in the surgical specimen.The gallbladder measures 9 × 4 × 3 cm and shows an indurated localized thickened fundic wall area measuring 2 × 1.5 × 1 cm located around the fundus and liver bed. Cut surface of the thickened area was off-white and firm. The gallbladder mucosa was normal. Three tan-yellow stones were present within gallbladder lumen. Gallbladder neck and cystic duct were grossly unremarkable.The entire liver bed and thickened portion of gallbladder wall was submitted for histological examination. Microscopic examination showed fibrous thickening of the gallbladder bed containing a meshwork of benign ductules and small 1–2 mm thick wall ducts. Some of these ductules were cystically dilated Figure . The intDucts of Luschka may connect with intrahepatic bile ducts but do not communicate with gallbladder lumen. These ducts are known source of bile leak or biliary peritonitis after cholecystectomy. Anomalous bile ducts are found distributed within the connective tissue of gallbladder bed. Gallbladder in this region is not covered by serosa and connective tissue layer is continuous with interlobular connective tissue of the liver. Gallbladder bed may contain two types of anomalous bile ducts: bile duct of Luschka and cystohepatic duct . Ducts oGross thickening of fundic portion of gallbladder wall is seen in such benign lesions as xanthomatous cholecystitis, adenomyomatous hyperplasia, Rokitansky-Aschoff sinuses and florid proliferation of bile ducts of Luschka. Likewise gallbladder adenocarcinoma may produce similar gross thickening of fundic portion of the gallbladder. Therefore above described entities constitute important differential diagnoses of lesions causing thickening of gallbladder wall.The adenocarcinoma described in this report seems to be arising from Luschka ducts. The following observations support its origin from the duct of Luschka: 1) The bulk of the tumor is confined to the gallbladder bed between liver, fundic portion of gallbladder wall, and lateral subperitoneal connective tissue with minimal invasion into the adjacent liver and adventitial layer of gallbladder. 2) The coexistence of foci of intraductal carcinoma within some of associated Luschka ducts/ductules. 3). The co-existence of benign small isolated thick wall ducts of 1–2 mm diameter as well as meshwork of Luschka ductules in gallbladder bed adjacent to the tumor.4) Absence of a primary tumor elsewhere. 5) The presence of the tumor in an area where duct of Luschka are normally prevalent.It may be difficult to delineate exact origin of a locally advanced malignancy from biliary structures such as gallbladder wall, Luschka ducts, cystohepatic ducts and liver within gallbladder fossa because of their close proximity. One can speculate that some of the locally advanced bulky tumors in cholecystic fossa  could poWritten consent was obtained from the patient for publication of this case report, a copy of this consent is available with editorial office.The authors declare that they have no competing interests.MJ wrote the case history and collected all clinical information. PX was responsible for literature review, medline search and wrote the first draft. AG reviewed the paper and made suggestions. MC reviewed the paper and made suggestions. AH interpreted data and critically revised the manuscript. All authors read and approved the final manuscript.


Agressive angiomyxoma is a rare mesenchymal neoplasm. It mainly presents in females. We here present a case of angiomyxoma presenting as huge abdominal lump along with gluteal swelling. Case note is described along with brief review of literature. Aggressive angiomyxoma is a rare mesenchymal tumor occurring predominantly in the pelvi-perineal region of females. We report such a case presented as abdominal as well as gluteal lump, a very unusual presentation. Patient underwent laparotomy and tumor was successfully excised.An eighteen year old unmarried female presented with progressive distension of abdomen and swelling in left gluteal region for last ten months. It was associated with mild dull aching pain in lower abdomen and bilateral flanks. She was having normal menstrual history. Examination revealed distended abdomen and a non-tender, diffuse, firm and dull mass was felt all over abdomen. Free fluid in peritoneum could not be demonstrated. There was another 10 × 8 cm boggy swelling on upper postero-medial aspect of left thigh having cross fluctuation with abdominal swelling. On ultrasonography 30 × 18 × 16 cm complex cystic mass was found occupying whole abdomen and pelvis with internal septations and echoes displacing bowel loops posteriorly with mild right sided hydro-ureteronephrosis. CECT scan (Figure Patient underwent laparotomy and the tumor was excised. The tumor was found to be bilobed, weighing about 15 Kg. Figure . Rectum The histopathology of the specimen showed capillary and cavernous vascular spaces stuffed with blood and separated by edematous fibrous and myxomatous tissue. The fibrous tissue is in form of interlacing or parallel bands of collagen with edema. The myxomatous tissue comprise of stellate cells with fibrillary in mucinous setting Figure . Immuno-Aggressive angiomyxoma is an uncommon mesenchymal neoplasm occurring predominantly in the pelvi-perineal region of adults, first described in 1983 by Steeper and Rosai . A few cAlthough a rare diagnosis, aggressive angiomyxoma can present with unusual features. Detailed radiological examination is helpful in suspecting the problem, but histology is gold standard for diagnosis. Wide excision is curative and prognosis of such tumor is good.The authors declare that they have no competing interests.AKK and RK: operating surgeons, SK: collected clinical details including photographs, summarized the case history and prepared final draft, NA: conducted a literature search and prepared first draft. All authors read and approved the final manuscript.A fully informed written consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent can be sent to Editor-in-Chief of this journal if article is accepted for publication.


Campylobacter coli is a rare cause of bacteremia. We report here the first case of C.coli spondylodiscitis complicated by an aortic aneurysm. Outcome was favourable with surgery and antibiotic therapy. Campylobacter fetus is a rare but well-recognized cause of bacteremia [Campylobacter coli bacteremia have been reported in the literature. To the best of our knowledge, no aortic aneurysm due to C.coli has been previously described. We report here the first case of C.coli spondylodiscitis complicated by an aortic aneurysm.While cteremia -3, only C.coli with the API Campy identification system, and was susceptible to all tested antibiotics. Treatment with a combination of cefotaxime plus gentamicin was immediately started. MRI showed abnormalities consistent with a L4-L5 spondylodiscitis associated with prevertebral and left psoas abscesses. Abdominal CT scan showed a ruptured abdominal aortic aneurysm and a contiguous left psoas collection . Postoperative evolution was favourable except for slight resolvable renal failure. CT scan carried out two weeks after surgery showed a marked reduction in the size of the collection and a decrease in the CRP level to 16 mg/L at the same time. Transoesophagal echocardiography was normal. A coloscopy was performed which revealed only a 5 mm non-resecable caecal polyp. Cefotaxime was stopped after two weeks and ciprofloxacin was switched to oral form and is still ongoing. 4 months after surgery, the CT scan showed the disappearance of the initial collection. Two years later, the patient continues to be in good health and remains totally asymptomatic.C.coli bacteremia remains unknown [C.coli bacteremia episodes are often reported in immunocompromised patients or in children [C.coli bacteremia may be underestimated due to infrequent blood sampling at the early stage of infection, and inappropriate culture conditions [Except for one study , the inc unknown  but seem unknown . This cochildren ,7-12. Onchildren ,13. Thernditions .C.coli. We found only one case of meningitis due to this pathogen, but observed no arthritis, endocarditis, pneumonia or prostatitis. We have no evidence for an associated endocarditis and colonoscopy revealed only a small non-inflammatory polyp. We also did not find any cause of immunosuppression in our patient except for age >65 years. Skirrow et al [Campylobacter bacteremia to be 1.5/1,000 intestinal Campylobacter infections in their total population, and 5.9/1000 in patients aged 65 or over.A combination of cephalosporin and gentamicin was chosen in an attempt to obtain a synergistic bactericidal effect during the first ten days of treatment. Ciprofloxacin was prescribed as prolonged antibiotic therapy in an attempt to obtain permanent high concentrations at the infected site. Although a 3 months duration of treatment for abdominal aortic aneurysms infected with Campylobacter has been evocated in two cases [Although bacteremia may be misdiagnosed, aortic aneurysm and spondylodiscitis are more highly visible infections. Nevertheless, no cases of these localizations have been reported for ow et al  found thwo cases ,16, conswo cases ,18. A prwo cases -22.Concerning the spondylodiscitis, despite some reported studies -25, as dC.coli bacteremia, but it is unclear as to whether the aneurysm rupture was secondary to bacteremia or to erosion of the arterial wall by the abscess.We believe that the spondylodiscitis was primarily due to C.coli, is poor [C.coli was always confirmed by PCR in these studies. In accordance with these data, the possibility of misdiagnosing Campylobacter species in the present case seems to be very low.A limitation of our case report is that species identification was made with the API Campy identification system and not by PCR. As previously reported the sensibility of the API Campy test, particularly for identification of  is poor -28. DespC.coli may be responsible for spondylodiscitis complicating bacteremia. Like C.fetus, C.coli may, under conditions not yet elucidated, cross the intestinal barrier and cause severe metastatic infections.Although it had not yet been described, Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.XL have made substantial contributions to acquisition of data, have been involved in drafting the manuscript. CD have been involved in drafting the manuscript. CC have made substantial contributions to acquisition of data. LDG have made substantial contributions to acquisition of data. BSB have made substantial contributions to acquisition of data. YY have given final approval of the version to be published. ES have made substantial contributions to conception of the case redaction, have given final approval of the version to be published. All authors read and approved the final manuscript.


A case report of omental torsion, which is a rare differential diagnosis of the acute abdomen. Intraoperative diagnosis and treatment by resection are the current management of choice, however with increasing use of pre-operative imaging this may need to be reconsidered. The four layered fatty sheet of peritoneum is known as omentum and suspends from the greater gastric curvature to surrounding organs with attachments to the diaphragm . OmentalA 44 year old female patient presented to the Emergency Department complaining of generalised abdominal pain for three days, localising to the right iliac fossa. Accompanying symptoms were nausea and constipation, but bowels had opened on day of presentation. No urinary symptoms, past medical history of note or regular medication were present.9/L), the blood count and serum biochemistry were normal on first presentation.On examination the patient was haemodynamically stable and apyrexial. The abdomen was soft, not distended, with localised tenderness in the right iliac fossa without peritonitis. Apart from a mild leukocytosis  and fibrinoid exudates Figures  &3.rd to 5th decade of life with a slight male predominance (3:2) [Omental torsion is a rare cause of abdominal pain presenting mainly in the 3ce (3:2) . The omeOmental torsion may be primary or secondary. One third of cases are a result of primary torsion, which is unipolar with no underlying pathology or distal fixation -7. In prPatient with omental torsion present with constant, non-radiating pain of increasing severity, nausea and vomiting. Clinically 50% of patients have a low grade fever and leukocytosis ,5. TheseOn laboratory findings a moderate leukocytosis is present in 50% of cases . ImagingThe current investigation tool and therapeutic management of choice is laparoscopy proceeding to laparotomy, identifying and removing the infarcted section of omentum. Normal appendix, gallbladder and pelvic cavity make the diagnosis of omental torsion likely. Free serosanguineous fluid as a result of haemorrhagic extravasion is a characteristic finding in the peritoneal cavity. In the literature the treatment of choice included additional appendicectomy to prevent future diagnostic problems. Successful conservative management has also been reported ,13.Histology findings of haemorrhagic infarction and fat necrosis confirm the diagnosis with the presence of fibrosis indicative of a longer disease process .The prognosis for primary omental torsion is good with fast post operative recovery and minimal morbidity. The natural disease progress if left untreated will result in fibrosis, necrosis and occasional autoamputation and clinical improvement ,14. ProgLeft sided omental torsion may be commonly misdiagnosed as diverticulutis and managed conservatively, resulting in less common diagnosis .Omental torsion presents with non specific symptoms of an acute abdomen and is mainly diagnosed intraoperatively during diagnostic laparoscopy. Awareness of omental torsion as a differential diagnosis in the acute abdomen and careful inspection of omentum in a "negative laparoscopy" are recommended for appropriate management of the surgical patient . HoweverThe authors declare that they have no competing interests.NB performed the literature review and drafted the paper. PS reviewed the manuscript and provided the figure. The manuscript was read and approved by all authors.Written informed consent was obtained from the patient for publication of this case report.


Hamartomas are non-neoplastic lesions constituted by a mixture of tissues indigenous to the region. Respiratory epithelial adenomatoid hamartomas are characterised by glandular proliferation lined by ciliated airway epithelium. Their localisation in the nasal cavity is rare and most frequent cases described so far were associated with the posterior nasal septum.A 9-year-old Caucasian boy presented with long-standing nasal obstruction. A large right nasal mass was evident on physical and CT examinations. It was surgically removed from the anterior nasal septum under general anaesthesia. Histologically, the diagnosis of REAH was established. The tumour lined by stratified squamous and ciliated respiratory epithelium was characterised by prominent glandular proliferation. By immunohistochemistry, the tumour was positive for cytokeratins, smooth muscle actin, vimentin, laminin, collagen type IV, CD8, and CD68. No S-100 immunoreactivity was observed. The patient has been asymptomatic for 12 months with completely healed lining of the nose.Respiratory epithelial adenomatoid hamartoma, although rare, must be taken into consideration in differential diagnosis of nasal exophytic lesions. Glandular lesions of the sinonasal tract are uncommon and may cause a diagnostic dilemma. If common salivary gland tumours are excluded, most of the other glandular lesions include respiratory epithelial adenomatoid hamartomas (REAHs), inverted schneiderian papillomas and sinonasal adenocarcinomas (SNACs) of the intestinal (ITAC) and nonintestinal (non-ITAC) types . HamartoA 9-year-old Caucasian boy presented with long-standing nasal obstruction. Physical examination revealed a large mass covered with normal mucosa, originating from the right side of nasal septum and located 3 mm far from the vestibule. The lesion was polypoid in shape, soft to palpation but more indurate than inflammatory nasal polyp . CT scanHistopathologically (HE staining), prominent glandular proliferation containing serous as well as mucous components  was founImmunohistochemistry revealed pan-cytokeratin  immunostaining in both types of surface lining epithelium  as well The reported localisation of the tumour is unusual and REAHs have rarely been described in children ,3. The aKi-67 staining has been found restricted to the basal/myoepithelial layer, demonstrating a low proliferation index . In our Respiratory epithelial adenomatoid hamartoma, although rare, must be taken into consideration in differential diagnosis of nasal exophytic lesions. Hamartoma of the anterior nasal septum can be effectively removed with indications and extent of the resection like in benign tumours.


Abduction deficit in the elderly is commonly caused by sixth cranial nerve palsy due to microvasculopathy. However, not all such cases are of neurogenic origin, as our case report shows.We present the case of a 75-year-old woman who was generally unwell, developed acute diplopia and was found to have a right abduction deficit in a quiet eye with no gross orbital signs and symptoms. A computed tomography scan of the head and orbits revealed a metastatic mass in the right lateral rectus muscle. Systemic evaluation confirmed widespread thoracic and abdominal metastases from an occult systemic malignancy. Lateral rectus metastasis from an occult systemic malignancy was masquerading as abducens palsy.Orbital metastasis involving extraocular muscles can present as isolated diplopia with minimal local signs and the absence of a history of systemic malignancy. A detailed history and systemic examination can identify suspicious cases, which should be investigated further. The clinician should avoid presuming that such an abduction deficit in the elderly is a benign neurogenic palsy. Binocular diplopia from an abduction deficit is commonly caused by sixth cranial nerve palsy as a result of microvasculopathy. This is a relatively benign condition that usually improves spontaneously. However, there are conditions with high morbidity and mortality that can masquerade as abducens palsy, as our case shows.A 75-year-old woman with a 1-week history of diplopia was referred to the ophthalmology clinic from the medical ward, where she was being treated for atypical pneumonia. She had been unwell for a few months with loss of appetite and weight. Sputum acid-fast bacillus smear and culture tests for tuberculosis were negative. There were no specific symptoms or signs suggestive of giant cell arteritis (GCA) but the erythrocyte sedimentation rate (ESR) was 98 mm/hour and the C-reactive protein was more than 139 mg/dl, which prompted the physicians to arrange a temporal artery biopsy. This was later reported as normal.Ophthalmic examination showed corrected visual acuity of 6/6 bilaterally. There was subtle localized episcleral injection near the right lateral rectus muscle insertion. The posterior segment revealed a normal optic disc and macula bilaterally. Ocular motility revealed an abduction deficit in the right eye Figure .Computed tomography (CT) scanning of the head and orbits revealed a mass in the belly of the right lateral rectus suggestive of a metastatic lesion Figures  and 3. SThe orbit is a rare site of distant metastases with incidence varying from 1% to 13% in the reported series of all orbital tumours [The diagnosis of orbital metastasis is often unexpected  when an The main symptoms of orbital metastasis at presentation include diplopia 48%), proptosis (26%) and decreased vision (16%) . Diplopi%, proptoThe definitive diagnosis of orbital metastasis can be established with either fine needle aspiration biopsy or an open biopsy. We were unfortunately unable to perform biopsy and hence obtain histological diagnosis due to our patient's deteriorating health.The prognosis in patients with orbital metastases is often poor as the primary tumour can only be found in one-third of patients . The medWhen dealing with a patient with an abduction deficit, a logical and systemic approach based on its causes is required. Sixth cranial nerve palsy is the most common cause of abduction deficit but myogenic and/or orbital conditions such as dysthyroid eye disease, idiopathic orbital inflammation, muscle entrapment (after blow-out fracture) and orbital metastasis must also be kept in mind . MyastheIt cannot be assumed that all cases of abduction deficit are of neurogenic aetiology. A minimal work-up in elderly patients should include blood pressure check, blood glucose and an ESR test. A raised ESR points towards GCA, a treatable condition that can cause extraocular muscle paresis by affecting either the ocular cranial nerves or the extraocular muscles. A detailed history and thorough general physical examination can prove invaluable, especially in atypical cases. Scans of the brain and orbits are not recommended as routine, but should be requested in suspicious cases. It is good practice to liaise with a radiologist and neurologist in such cases. In our patient, the history of prolonged illness accompanied by weight loss, increased ESR and negative temporal artery biopsy raised our suspicions and led to subsequent investigations.Myogenic and neuromuscular causes for extraocular muscle palsies are not very common and are easily missed. Metastatic muscle involvement, which is rarely described in the ophthalmic literature as a cause of such palsies, needs to be included in the differential diagnoses in suspected cases.We can draw the following conclusions:1. A paretic abduction deficit should not invariably be assumed to be of neurogenic origin. The danger of assuming abduction deficit as a benign ischaemic sixth nerve palsy is especially high in elderly patients.2. Metastatic orbital tumours are relatively rare but must be included in the differential diagnoses in suspicious cases.3. Orbital metastasis involving extraocular muscles can present as isolated diplopia in the absence of a history of systemic malignancy.4. Local ophthalmic signs may be subtle or minimal in orbital and/or myogenic causes of such palsies.5. It is important to communicate with radiologists when requesting imaging so as to ensure the scans include all areas of interest, namely bothorbits and the brain, in cases of suspected orbital metastasis.CT: computed tomography; ESR: erythrocyte sedimentation rate; GCA: giant cell arteritis; MRI: magnetic resonance imaging.The authors declare that they have no competing interests.MTM, AR and MYS were all involved in the care of this patient. MTM and AR co-wrote the report. As the senior clinician, MYS supervised the work. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


This is the first case report of Mirizzi syndrome associated with hepatic artery pseudoaneurysm.A 54-year-old man presented with painful obstructive jaundice and weight loss. Computed tomography showed a hilar mass in the liver. Following an episode of haemobilia, angiography demonstrated a pseudoaneurysm of a branch of the right hepatic artery that was embolised. At surgery, a gallstone causing Mirizzi type II syndrome was found to be responsible for the biliary obstruction and a necrotic inflammatory mass and haematoma were found to be extending into the liver. The mass was debrided and drained, the obstructing stones removed and the bile duct drained with a t-tube. The patient made a full recovery.This case highlights another situation where there may be difficulty in differentiating Mirizzi syndrome from biliary tract cancer. Mirizzi syndrome  is oftenA 54-year-old Caucasian man presented with a 4-month history of pain in the right hypochondrium after eating, weight loss and obstructive jaundice. On examination, the patient had a tender mass in the right upper quadrant of his abdomen. Biochemical tests showed changes consistent with obstructive jaundice  1792 U/litre). An abdominal ultrasound scan (USS) showed gallstones in a thick-walled gallbladder and intrahepatic duct dilatation, but no common bile duct (CBD) stones or impacted stone in Hartmann's pouch. Magnetic resonance cholangiopancreatography (MRCP) showed dilatation of the intrahepatic ducts above a common hepatic duct stricture; no gallstone was seen in relation to this. At endoscopic retrograde cholangiopancreatography (ERCP), the hilar stricture was confirmed and stented. Brush cytology was negative for neoplasia. The patient's jaundice resolved. Computed tomography (CT) showed a mass surrounding the gallbladder fossa and a ring-calcified lesion . Aneurysms account for about 10% of cases. Haemobilia can occur secondary to gallstones or neoplasia, and angiography is the most useful diagnostic modality for haemobilia .Aneurysms of branches of the hepatic arteries are rare . At risk groups include patients following orthotopic liver transplant, abdominal trauma, pancreatic pseudocysts, polyarteritis nodosa and percutaneous liver biopsy .In this patient, the haemobilia occurred before the percutaneous biopsies were taken. The presence of haemobilia and hepatic artery aneurysm suggested a benign rather than malignant process. Mirizzi syndrome is likely to have produced cholecystitis, gallbladder perforation and aneurysm formation secondary to sepsis and inflammation.No case of Mirizzi syndrome has been reported with a hepatic artery pseudoaneurysm. Embolisation of the aneurysm and open cholecystectomy resulted in a good outcome for this patient. Even when symptoms, signs and radiological appearances are indicative of cancer, benign conditions should be considered when histology is unable to confirm the diagnosis.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.OA prepared the manuscript and performed the literature review. RF prepared the case materials and contributed to the manuscript. BD provided expert opinion and reviewed and corrected the manuscript. AG provided expert opinion and reviewed the radiology.


Carcinoid tumors are characteristically low grade malignant neoplasms with neuroendocrine differentiation that arise in various body sites, most commonly the lung and gastrointestinal tract, but less frequently the kidneys, breasts, ovaries, testes, prostate and other locations. We report a case of a carcinoid of renal origin with synchronous single liver metastases on radiological studies.A 45 year-old patient who presented with abdominal pain was found on CT scan to have lesions in the right ovary, right kidney, and left hepatic lobe. CA-125, CEA, and CA 19-9 were within normal limits, as were preoperative liver function tests and renal function. Biopsy of the liver mass demonstrated metastatic neuroendocrine tumor. At laparotomy, the patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy, radical right nephrectomy with lymphadenectomy, and left hepatectomy. Pathology evaluation reported a right ovarian borderline serous tumor, well-differentiated neuroendocrine carcinoma of the kidney (carcinoid) with 2 positive retroperitoneal lymph nodes, and a single liver metastasis. Immunohistochemistry revealed that this lesion was positive for synaptophysin and CD56, but negative for chromogranin as well as CD10, CD7, and CD20, consistent with a well-differentiated neuroendocrine tumor. She is doing well one year after her initial surgery, with no evidence of tumor recurrence.Early surgical intervention, together with careful surveillance and follow-up, can achieve successful long-term outcomes in patients with this rare malignancy. Neuroendocrine carcinomas may originate in a wide variety of tissues and organs, including those that do not normally contain neuroendocrine cells . These tWe evaluated a 45 year-old patient who presented initially with abdominal pain. Abdominal and pelvic CT scan showed lesions in the right ovary, right kidney, and left hepatic lobe. The right kidney mass was 8.0 cm in diameter, with areas of calcification in the periphery of the tumor inferiorly Figure . In addiCarcinoid tumors are characteristically low grade malignant neoplasms with neuroendocrine differentiation that arise in various body sites, most commonly the lung and gastrointestinal tract, but less frequently the kidneys, breasts, ovaries, testes, prostate and other locations. The prognosis of carcinoid tumors of renal origin is unclear due to the rarity of these lesions. The pathogenesis of renal carcinoid tumors (RCT) is controversial. Several hypotheses support that RCT are derived from interspersed neuroendocrine cells associated with acquired and congenital abnormalities such as metaplasia of pyelocaliceal urothelium induced by chronic inflammation, misplaced or entrapped neural crest or pancreatic tissue in the kidney during embryogenesis, activation of gene sequences common to neuroendocrine programmed cells in multipotent stem cells, or concurrent congenital abnormalities -12.et al. [In 2006, an extensive review of the literature on primary RCT was published by Romero et al. . In thiset al. . Only 7%et al. . MitoticThe octreotide scan is considered the most important investigation for surveillance after resection. Following chromogranin and 5-HIAA is also recommended, even in the absence of symptoms. Additional neuroendocrine markers can be tested if they were found to be positive prior to surgery. CT and/or MRI can be used as imaging studies for surveillance. New metastases have been reported as long as 7 years after resection, indicating that long term follow up is needed.et al. [The treatment of liver metastases from RCT is not well defined due to scarcity of cases. Most of the experience regarding the treatment of metastatic neuroendocrine disease in the liver comes from those tumors originating in the gastrointestinal tract, and in these cases, the mainstay of treatment is resection -15. In oet al.  have proet al. ,18,19, wTumor recurrence has been a major problem after surgical treatment. Resection, ablation, or both in combination can be used to treat tumor recurrence . ExtensiEarly surgical intervention, together with careful surveillance and follow-up, can achieve successful long-term outcomes in patients with this rare malignancy.The authors declare that they have no competing interests.RG and RGR conceived of the study; RG conducted literature review and prepared the draft manuscript; RG, HJ, TDJ, PPM, and RGR performed critical editing of content and helped in preparation of the manuscript; RG and DR edited the final version. All authors read and approved of the final version of the manuscript


It revealed a complex, predominantly solid hepatic lesion with 3 × 3 cm and a marked, essentially peripheral, Doppler blood flow. Fetal echocardiography showed a normal heart besides a vena cava displacement by the hepatic mass. Fetal Hepatic hemangioma was suspected. Follow-up ultrasounds were unchanged. Pregnancy evolved well. At 36 weeks of gestation was spontaneously delivered a 3300 g boy whose examination revealed a visible thoracoabdominal circulation and a palpable liver. No skin lesions, namely hemangiomas or petechiae were identified. Postnatal magnetic resonance imaging confirmed the diagnosis of Hepatic hemangioma. Treatment was initiated with prednisolone followed by interferon. After 2 years, there is no active lesion.Hepatic tumors in children account for only 1 to 5% of all pediatric tumors. Hepatic hemangioma is, however, the third most common tumor of the liver in childhood. We report a case of an antenatal diagnosis of a hepatic tumor detected on a first obstetric ultrasound, at 26 Hepatic tumors in children account for only 1 to 5% of all pediatric tumors. Hepatic Hemangioma (HH) is, however, the third most common tumor of the liver in childhood . It is aWhile small HH can be asymptomatic lesions incidentally discovered during imaging of the abdomen, larger tumors may act as arteriovenous shunts resulting in high-output congestive heart failure and hepatic dysfunction that may lead to fetal non-immune hydrops (unrelated to Rh disease). Other complications include coagulopathy, hemolytic anemia and tumor rupture. Diagnosis is most often based on characteristic radiological findings  -7. BiopsA 27 year-old, healthy, gesta 3 para 2 Caucasian Portuguese woman presented for an obstetric ultrasound at 26 weeks of gestational age (GA) to an outpatient sonography department. Until then she had no pregnancy surveillance. A complex, predominantly solid hepatic lesion was noted, measuring approximately 3 cm in diameter. No other fetal anatomic abnormalities were detected.th gestational week revealed a heterogeneous, predominantly solid hepatic lesion with mostly hypoechoic texture, a diameter of 3 cm and a marked, essentially peripheral, Doppler blood flow  showed a nodular mass with 7.3×5.1 cm affecting both lobes of the liver with involvement of celiac trunk and hepatic hilum, marked by low signal intensity on T1-weighted images and high signal intensity on T2-weighted images ,4. After2/day with daily dose increased up to 1 MU/m2/day for 3 months, with progressive regression of the hepatic tumor and LFTs.Treatment was initiated with prednisolone (3 mg/Kg/day) but after 4 months the lesion showed the same characteristics, with a sustained deterioration of LFTs. An alternative medical treatment with subcutaneous α-2a-interferon was then started; beginning with 500.000 U/mBesides clinical surveillance, LFTs and MRI were used in follow-up. After 2 years the boy is asymptomatic, with normal LFTs  and within utero and post-natally. Some case reports of antenatal ultrasound diagnosis have been described showing the enormous value of a previous evaluation by allowing the pediatric team to be prepared.Hepatic Hemangioma is a rare benign neoplasia representing, however, the most common vascular tumor of the liver in the neonate ,2. As prSince biopsy may result in massive haemorrhage, diagnosis is most often based on characteristic radiological findings -7. A posIn our case, a complex fetal hepatic lesion was diagnosed at an obstetric ultrasound performed at 26 weeks GA. Differential diagnosis included benign and malignant conditions such as congenital hemangioma, mesenchymal hamartoma, hepatoblastoma and embryonal sarcoma. The strongest alternative diagnosis for this large, solitary, stable lesion was hemangioma. In fact, the stabilized growth of the lesion with no associated anomalies allowed a more confident surveillance of the pregnancy. Neonatologist team was aware of the case so that postnatal abdominal ultrasound and MRI were promptly provided. As MRI was consistent with HH, biopsy was avoided.Natural history of small, asymptomatic HH is spontaneous involution and no treatment is required. Larger or symptomatic lesions need aggressive management which may include medical therapy with CS or agents with strong antiangiogenic effect, irradiation, selective embolization or surgical intervention -10.As it was a large, symptomatic tumor with a deterioration of the hepatic function, expectant management was excluded and medical treatment with CS was initiated. This is usually the first choice as it is associated with fewer side effects. The unresponsiveness to CS determined the use of a second-line medical therapy with interferon, with very good results as seen in literature . Given t


Maffucci syndrome, a congenital mesodermal dysplasia characterized by multiple enchondromas and hemangiomas, was first described in 1881, and 200 cases have been reported in the literature since then. Its etiology is unknown, there is no predilection for race or sex, and the development of lesions usually occurs in puberty. The risk of sarcomatous transformation is about 25%.The initial investigation of the case reported here focused on the diagnosis and treatment of malignancy, and the first diagnostic hypothesis was thrombosed hemangioma. After histopathologic confirmation of soft tissue sarcoma, examinations were performed to stage the tumor and enchondromas were found in ribs. The final diagnosis was Maffucci syndrome with malignant transformation.Treatment should aim at symptom relief and early detection of malignancies; no therapy is indicated for asymptomatic patients. As in the case reported here, bone or soft tissue lesions that grow or become painful should be biopsied. Maffucci syndrome was first described in 1881, but its etiology remains unknown. About 200 cases have been described in the literature to date . EnchondThis congenital mesodermal hyperplasia is nonhereditary, and patients are usually asymptomatic at birth. Symptoms usually develop in puberty .The risk of malignant change is about 25%. Sarcomatous transformation may be seen in bones and soft tissue lesions, but is more frequently found in enchondromas, and its incidence ranges from 15 to 57% [This report describes the case of a 29-year-old patient with a diagnosis of Maffucci syndrome with malignant transformation and a tumor in the right thigh diagnosed as retiform hemangioendothelioma.A 29-year-old white woman presented with a tumor that had appeared on the right thigh two years before. Physical examination revealed a hard nodule about five centimeters in diameter with no signs of inflammation. Ultrasound showed a heterogeneous tumor measuring 5.0 × 6.5 × 4.5 cm and coarse calcifications within the tumor. Because of hemangiomas in upper extremities, the initial hypothesis was thrombosed hemangioma.Histopathological analysis of excisional biopsy specimen showed fusiform and vasoformative tissue. Immunohistochemical analysis suggested a well-differentiated myxoid liposarcoma.After the diagnosis of soft tissue sarcoma, complete tumor staging was performed using chest radiographs and CT, which showed lytic bone lesions in fourth and seventh anterior ribs, and nuclear MRI of right thigh, which showed a large, predominantly cystic and heterogeneous lesion. Therefore, radical tumor resection was performed. Material was sent to the Mayo Clinic, and the final diagnosis was a low-grade vascular neoplasm consistent with retiform hemangioendothelioma and not liposarcoma, as previously thought.After one-year follow-up, the patient noticed a new tumor on the right thigh and inguinal region, and biopsy confirmed lymph node metastasis. Chest CT revealed several lung lesions, but etiology was not confirmed by biopsy. Chemotherapy with doxorubicin was initiated, and full clinical response was achieved.Maffucci syndrome is a rare clinical entity characterized by multiple enchondromas, hemangiomas and, less often, lymphangiomas -3. The dBone changes are caused by congenital defects in endochondral ossification and lead to deficits in final bone growth and bone irregularities. Enchondromas are more often found in hand phalanges, metacarpal bones, foot bones, tibia, fibula, radius and ulna ,3. DistrHemangiomas are usually found along soft tissues as bluish nodules, but may also be found in internal organs and mucous membranes, particularly in the brain, eyes, and gastrointestinal tract .Pathological fractures are found in 26% of the cases; other possible complications are hemorrhage, short stature, pleural effusion and cranial nerve paralysis due to endochondral compression .The differential diagnosis from Ollier disease should be established because, although chondrodysplasia is found in both, Maffucci syndrome is associated with risk of malignancy . SarcomaRetiform hemangiomas (RHE) are extremely rare low-grade variations of well-differentiated angiosarcoma, characterized by high recurrence rates and low metastatic potential . In the Treatment should aim at symptom relief and early detection of malignancies. Surgical procedures consist of osteotomy and curettage of bone lesion. Other therapeutic options are sclerotherapy, irradiation and vascular lesion surgery. Excision is the procedure of choice for soft tissue tumors .Bone and soft tissue lesions that grow or become painful without a history of trauma should be examined for malignancy and biopsied. No treatment is indicated for asymptomatic patients without malignant transformation, and prognosis is usually good .The authors declare that they have no competing interests.FTL carried out overall design of the manuscript and was responsible for the writing of the report. JSA participated in manuscript design and helped to draft the manuscript. GNA was responsible for all the literature review and contributed to the discussion. FOB contributed to case description and gave final approval of the version to be published.JF conceived the theory behind the report and helped to draft the manuscript. All authors read and approved the final manuscript.Written consent was obtained from the patient or their relative for publication of study.


Hepatoid adenocarcinoma is an extrahepatic tumor characterized by morphological similarities to hepatocellular carcinoma. Hepatoid adenocarcinoma of the stomach is a cancer with an extremely poor prognosis with few cases reported. Here, we describe a 75-year-old Spanish man referred to our hospital with a history of abdominal pain, general fatigue, anorexia and sickness. Initial study revealed anemia, and computed tomography scan and abdominal ultrasonography showed multiple metastases to the liver with hepatocellular carcinoma characteristics in a liver with no cirrhotic change. Further study included a serum level of alpha-fetoprotein (AFP), which resulted markedly elevated, and a conclusive esophagogastroduodenoscopy describing an elevated tumour growing through the cardia and gastroesophageal junction with foci of necrosis and haemorrhage. Gastric biopsies of the tumor revealed poorly differenciated adenocarcinoma, with hepatoid differentiation. After a diagnosis of AFP-producing hepatoid adenocarcinoma of the stomach with multiple liver metastases was made, pallitive total gastrectomy, without liver resection, was performed. Patient recovered well after surgery, and entered into a palliative systemich chemotherapy protocol. Although this illness is recognized as having poor prognosis, the patient remains alive 8 months after the operation. Accurate diagnosis of hepatoid adenocarcinoma of the stomach is important, and should be suspected under certain circumstances. We describe this rare case of hepatoid adenocarcinoma of the stomach, and review the literature concerning the clinicopathological aspects. AFP levet al. , AFP-proet al. ,12. In 1ngements . Later I [et al. ,15 propo [et al. . This tyA 75-year-old man presented at the emergency department of Vega Baja Hospital, Orihuela, Alicante, on February 2008, with of a 15-day history of abdominal pain, general fatigue, anorexia and sickness. His medical history included hypertension, adenomatous colonic polyposis, upper gastrointestinal bleeding secondary to agiodysplasia treated with enterectomy in 2005, obstructive pulmonary chronic disease related to hemp industry, and right adrenal adenoma since 2005.On physical examination only paleness of skin and conjunctiva was detected. He was referred to Internal Medicine Department fur further study. Haematological investigations revealed anemia (Hemoglobine (Hb), 7.1 g/dl; hematocrit (Ht), 25% and high levels of AFP  and CEA (460 ng/mL). Chest and abdominal radiograph were negative. After blood transfussion, other image test were performed. Abdominal ultrasonoraphy showed liver with muliple nodules, biggest in left liver lobe (56 × 51 mm.), highly suspicious for hepatocellular carcinoma  and/or ACT  as well as AFP. AAT and ACT have immunosuppressive and protease-inhibitory properties that enhance invasiveness ,17. AlsoThe efficacy of palliative gastrectomy in patients with incurable advanced gastric cancer remains debatable. Previous reports suggested that palliative gastrectomy might be beneficial for younger patients (aged under 70 years) with one mestastatic site , while oPalliative chemotherapy should be advised to all patients with advanced gastric cancer and good performance status. Analysis of results of trials carried out until 2003 on this topic showed chemotherapy to be superior to best supportive care alone. Combination chemotherapy compared with monochemotherapy was also associated with significantly higher overall  response rates but nevertheless resulted in similar survival. Cisplatin and 5-fluorouracil combination with or without epirrubicin used by then to represent the most effective regimens for advanced gastric cancer . New comIn conclusion, we report a case hepatoid adenocarcinoma of the stomach, a relatively rare entity, with well defined clinicopathological features, and usually associated to recognized poor prognosis, in which the realisation of palliative surgery and the administration of novel palliative systemic chemotherapy may have had major impact on survival.Wrtitten informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written informed consent is available for review by the Editor-in-Chief of this Journal.The authors declare that they have no competing interests.EGM designed and coordinated the case report. JGP conceived of the case report.VGO helped to draft the manuscript. FMP was responsable for surgery aspects. JARM was responsable for pathology assesment. MAM revised the manuscrpt critically for important intellectual content. All authors read and approved the final manuscript.


Hodgkin's disease (HD) most commonly presents with progressive painless enlargement of peripheral lymph nodes, especially around the cervical region. A few children have systemic symptoms and weight loss. At the time of diagnosis, osseous involvement is uncommonA case is described of Primary Multifocal Osseous Hodgkin's Lymphoma in a seven-year-old boy. He presented with a painful swelling in the sternum, and further investigations revealed deposits in his L1 vertebra, the left sacro-iliac joint and the right acetabulum.The clinical, radiological and histological features of this disease can mimic other medical conditions, including Tuberculosis, making the diagnosis difficult and often leading to delays in treatment. This is a very rare condition and we believe this to be the youngest reported case in the literature. Hodgkin's disease (HD) most commonly presents with progressive painless enlargement of peripheral lymph nodes, especially around the cervical region. A few children have systemic symptoms and weight loss. At the time of diagnosis, osseous involvement is uncommon and even in the late stages only 9–35% of cases have any bony involvement . It is tWe present a case of a seven-year-old boy diagnosed with primary multifocal osseous Hodgkin's lymphoma. We believe this to be the youngest such reported case and only the third ever reported paediatric case of primary multifocal osseous Hodgkin's lymphoma in the English literature.99 bone scan , ESR (90), white cell count (22.3) with a neutrophilia 17.0) and a hypochromic microcytic anaemia (Hb: 9.3). Technecium7.0 and aA CT scan of the chest Figure  and sterFive weeks after discharge he represented because of an enlarging sternal mass and the development of back pain with no associated neurology Figure . He remaTreatment with chemotherapy was started following the current UKCCSG (United Kingdom Childrens Cancer Survey Guidelines) for Hodgkin's disease, and antituberculous treatment was stopped. Staging showed no lymphadenopathy in the chest or abdomen. Two weeks after commencement of chemotherapy a dramatic decrease in soft tissue involvement around the spinal cord was seen on a repeat MRI. Figure . The timet al., [The incidence of skeletal Hodgkin's disease varies from 9–14% during the course of the disease with up to 30–50% at post mortem . GrangerThere are thirty three clearly reported cases of primary osseous lymphoma at either single or multiple sites, in all ages, in the scientific literature since 1927. Table et al., [The two cases presented by Ostrowski et al.,  were amoet al., [Gross et al.,  presenteet al., .et al., [There have been two paediatric cases identified by our literature review of patients with POHL at a single site. A case report by Citow JS et al.,  of a 54 Radiologically, bony lesions of Hodgkin's disease may be lytic, sclerotic or mixed. One study showed that 75% were lytic, 13.6% mixed and 11.4% mixed .In all reported cases, the correct diagnosis was only reached after extensive and repeated investigations and review of the histology. The average time to diagnosis from initial presentation was 6–8 months. The most frequent misdiagnosis was osteomyelitis. Our case highlights the difficulties in diagnosing this rare form of Hodgkin's disease.TB in England has increased by 25 per cent over the last 10 years. Most TB in England occurs among people in inner cities – two in every five cases are in London  declare that they have no competing interests.CL, SG, JU and JK all contributed to the literature review. CL,SG and NC have written and revised the manuscripts. All authors read and approved final manuscript for publication.


Hepatoid adenocarcinoma is a special type of extrahepatic alpha-fetoprotein-producing adenocarcinoma, which has a morphologic similarity to hepatocellular carcinoma. We report a patient with underlying hepatitis B virus infection and hepatoid adenocarcinoma with liver metastasis mimicking hepatocellular carcinoma.We present the case of a 56-year-old Chinese female with underlying hepatitis B virus infection, who was found to have multiple hepatic tumors by abdominal ultrasound and an elevated level of serum alpha-fetoprotein. Hepatocellular carcinoma was considered based on the image findings, the elevated level of serum alpha-fetoprotein. and underlying hepatitis B virus infection. Moreover, the subsequent endoscopy revealed gastric tumor. However, the tumor histology of the stomach and liver revealed glandular adenocarcinoma with hepatoid foci. The final diagnosis is hepatoid adenocarcinoma of the stomach with liver metastasis.Hepatoid adenocarcinoma is an aggressive tumor with liver metastasis being the first clinical manifestation of the neoplasm. Hepatoid adenocarcinoma of the stomach with liver metastasis should be considered in older patients with elevated serum alpha-fetoprotein and multiple hepatic tumors with underlying chronic liver disease. An upper gastrointestinal endoscopy should be performed to exclude the possibility of hepatoid adenocarcinoma originating from the stomach to avoid potential misdiagnosis and inappropriate therapy. Hepatoid adenocarcinoma (HAC) is a special type of extrahepatic adenocarcinoma, which has a morphologic similarity to hepatocellular carcinoma (HCC) . The firA 56-year-old Chinese female presented on admission to our gastroenterology ward on May 2007, with a history of abdominal dull pain of 2 weeks duration and a weight loss of 6 kg duration a 2-month period. The abdominal pain became progressively worse and most tender in the right upper quarter (RUQ). She had had HBV infection for 15 years but follow up was irregular. She denied alcohol or smoking history. Result of physical examination showed RUQ tenderness and hepatomegaly. Results of laboratory workup revealed aspartate aminotransferase level of 130 IU/L, alanine aminotransferase level of 50 IU/L, total bilirubin level of 0.6 mg/dl and alkaline phosphatase level of 609 IU/L. The level of serum AFP was elevated (9457 ng/ml) but the levels of CEA and carbohydrate antigen 19-9 (CA19-9) were within the normal range. Serum was positive for hepatitis B surface antigen (HBsAg), but negative for HBeAg. The serum anti-HCV was also negative.Abdominal ultrasound (US) demonstrated multiple hepatic tumors at the bilateral lobe and mild chronic liver disease. Abdominal computed tomography (CT) showed multiple hepatic tumors sized 3-7 cm in the bilateral lobes of the liver and wall thickening in the stomach . No cirrGastric biopsy revealed a moderately differentiated tubular adenocarcinoma and poorly differentiated adenocarcinoma morphologically . HoweverAFP is detected in the serum of adults with HCC, hepatoblastoma, those with germ cell tumor , and those with non-cancerous liver disease with liver regeneration, such as hepatitis and cirrhosis. AFP-producing malignant tumors have been reported in several different organs such as the lung, pancreas, colon, ovary and urinary bladder -8. The sThe diagnosis of HAC depends on recognition of the characteristic histological features . The hisThe resemblance of HAC to HCC may be problematic in the diagnosis of hepatic tumors. In our study, multiple hepatic tumors were the first tumor manifestation and the patient had underlying HBV infection and markedly elevated AFP. Thus, the diagnosis of HCA presenting as hepatic tumors may be a great challenge for the pathologist and clinician, and the differential diagnosis of HAC from HCC is difficult. HCCs arising in non-fibrotic liver and without underlying known risk factors are rare, but according to a European study they may represent up to 13% of HCCs . HoweverIn our study, the tumor of both stomach and liver was positive for Hep Par1. Hep Par 1 is a developed monoclonal antibody that reacts with a hepatocyte-specific epitope . An 82% HAC is an aggressive tumor with liver metastasis being the first clinical manifestation of the neoplasm. HAC of the stomach with liver metastasis should be considered in older patients with elevated serum AFP and multiple hepatic tumors with underlying chronic liver disease. An upper GI endoscopy should be performed to exclude the possibility of HAC originating from the stomach. Although rare, this entity deserves wide recognition among pathologists and clinicians to avoid potential misdiagnosis and inappropriate therapy.


Achondrogenesis is a lethal osteochondrodysplasia characterized by hypoplasia of the bones and is associated with various anomalies varying in severity. Based on clinical, radiologic, and histopathologic features, two types are distinguished.The prenatal ultrasound examination of a 32-year-old Turkish woman who was referred to our clinic at 33 weeks and 6 days of gestation revealed fetal micromelia together with several other anomalies. The female baby died shortly after birth and was diagnosed with achondrogenesis type I based on the clinical and radiographic findings.Ultrasonography is important in prenatal diagnosis and for distinguishing lethal skeletal dysplasias in order to counsel the parents about future recurrent risks. As it is a uniformly lethal disease, a definitive prenatal diagnosis of achondrogenesis may be an indication for pregnancy termination. Achondrogenesis is a skeletal dysplasia, characterized by extremely shortened limbs, a normal to poorly ossified skull, a poorly ossified spine and pelvis, and severe pulmonary hypoplasia . The incA 32-year-old Turkish woman, gravida 3, para 2, was referred to our prenatal diagnosis and treatment clinic because of the diagnoses of gestational diabetes mellitus and polyhydramnios, and abnormal results of an ultrasound examination performed by her obstetrician. First-degree consanguinity was noted between the couple. Her first pregnancy had ended in a cesarean section at 33 weeks' gestation with a diagnosis of severe preeclampsia and breech presentation. Although it had not been possible to make an exact diagnosis, the appearance of the male baby had suggested dwarfism. The baby had died within 3 hours of birth. Her second pregnancy had been complicated by polyhydramnios, preeclampsia, and gestational diabetes mellitus and had ended in the birth of a healthy male baby.Prenatal sonograms of the fetus of the present gestation revealed extreme micromelia, narrow thorax, high abdomen/thorax ratio, pulmonary hypoplasia, and poor mineralization of the skull and vertebrae. Polyhydramnios and a pseudo-hydropic appearance, pleural and pericardial effusion, and low lying ears were also observed Figure . During Chondrodysplasias affect the growing cartilage, with an incidence of 1 to 3 in 10,000 births, and form a heterogeneous group of genetic origin skeletal dysplasias. Most chondrodysplasias are caused by mutations in various types of collagen genes. Collagen type II is coded by a large gene COL2A1 on 12q13.11-q13.12 and defects in collagen type II are caused by point mutations of this gene. The pathological changes in patients are observed in the growth plate, nucleus pulposus, and vitreous body, where the abnormal collagen type II is distributed .Achondrogenesis, a lethal form of congenital chondrodystrophy, is characterized by extreme micromelia. The prenatal diagnosis of achondrogenesis is based on additional findings of narrow thorax, and poor mineralization of the skull and vertebrae. Polyhydramnios and a pseudo-hydropic appearance are also common . These fAchondrogenesis may be differentiated from other skeletal dysplasias by having the most severe degree of limb shortening. The demineralization is only a differential diagnosis in osteogenesis imperfecta and hypophosphatasia, which do not present with the same degree of limb shortening .With a considerable phenotypic heterogeneity seen in achondrogenesis , types ICharacterization of demineralization is important for differentiating between type I and II cases. When the demineralization affects the skull and iliac wings the presumptive diagnosis is type I; when the skull appears normally mineralized the presumptive diagnosis is type II. When demineralization is present on sonography, an X-ray may confirm it. However, in the absence of demineralization on ultrasound, radiological demineralization cannot be presumed. Since the recognition of demineralization by ultrasound is fraught with false negatives, there will be a tendency to over-report the type II form . In our Many cases of fetal skeletal dysplasias detected antenatally with ultrasonography have been reported. A final diagnosis was obtained in most cases by fetopathological examination and radiographic studies and molecular testing as deemed necessary. Also well recognized antenatally in this series was achondrogenesis with type II predominance. Witters et al. have described 38 dysplasias of which two cases were achondrogenesis type II, diagnosed at 12 and 18 weeks . In the Similar to the other lethal short limb dysplasia, achondrogenesis is lethal due to pulmonary hypoplasia . InfantsSpecific prenatal diagnosis by ultrasound is helpful in distinguishing lethal skeletal dysplasias for counseling parents about future recurrent risks.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.MZT diagnosed and managed the case with full responsibility, and analyzed and interpreted the patient data. MK assisted MZT in the management of the case and was the major contributor in writing the manuscript. CT contributed to the writing and editing of the manuscript. GG assisted MZT in the management of the case and provided data and accompanying images. OH was the general consultant and advised in the management of the case and writing of the manuscript. All the authors read and approved the final manuscript.