Acute abdomen with hemoperitoneum is a very rare entity in postmenopausal women due to gynecologic conditions. A 54-year-old, postmenopausal woman was brought to emergency department with severe abdominal pain. Physical examination revealed acute abdomen findings with 15 cm pelvic mass on the right adnexal region. Immediate exploratory laparotomy was performed. During laparotomy 1000 cc of bloodstained fluid, ruptured and actively bleeding large mass arising from right ovary was observed. Right salpingo-oopherectomy was performed in emergency conditions, and pathology report revealed an adult type of granulosa cell tumor. After this result, staging surgery was performed and patient was diagnosed as granulosa cell tumor stage 1 c. Cisplatin, etoposide, and bleomycin chemotherapy was given. Clinicians should be aware of granulosa cell tumors which may occur at any age and prone to rupture. Frozen section will be helpful in order to avoid incomplete surgeries especially in postmenopausal women presented with intra-abdominal bleeding. Granulosa cell tumors of the ovary are rare neoplasms that originate from sex-cord stromal cells and they comprise 2–5% of all ovarian cancers . Women mA 54-year-old, gravida 3, para 2 woman was brought to emergency department with severe right lower abdominal pain. The patient who was at menopause for 2 years had a postmenopausal bleeding history for two months, and she had abdominal distension for three months. Physical examination revealed abdominal tenderness and acute abdomen findings with stable vital signs, and pelvic examination revealed nearly 15 cm pelvic mass on the right adnexal region. Her hemoglobin count was 11.7 gr/dl. Abdominal ultrasound and pelviabdominal tomography examination confirmed the mass in the right adnexa measuring 13 × 12 × 8 cm with multiple septations and also revealed free fluid at Morisson's space and the cul-de-sac. Because of the clinical findings and 1 gr/dl hemoglobin fall in three hours, an immediate exploratory laparotomy was performed. During laparotomy 1000 cc of bloodstained fluid, ruptured and actively bleeding large mass arising from right ovary was observed. Omentum, the left ovary, and the uterus were normal, and right salpingo-oopherectomy was performed in emergency conditions. Postoperative recovery was uneventful, and the patient was discharged from hospital after endometrial sampling was performed because of patient's history of postmenopausal bleeding. Pathology report revealed an adult type of granulosa cell tumor, the mitotic rate was 9 mitoses per 10 HPFs (high power fields), and endometrial biopsy result was complex hyperplasia. According to this result, the patient was evaluated as granulosa cell tumor at least stage 1 c, and staging surgery with type II hysterectomy + left unilateral salpingo-oopherectomy + infracolic omentectomy + bilateral pelvic-para-aortic lymphadenectomy + fluid sampling for cytologic examination was performed. Patient was finally diagnosed as granulosa cell tumor stage 1 c according to the pathology result of staging surgery and 6 cycles of cisplating + etoposid + bleomycin chemotherapy was given.Abdominal pain is the most common surgical emergency, the most common cause for a surgical consultation in the emergency department, and the most common cause for nontrauma-related hospital admissions . The difGynecologic conditions still cause acute abdomen in postmenopausal women. As the number of postmenopausal women increases, physicians will have more opportunities to treat elderly women with gynecological complications. The gynecologic causes of acute abdomen in postmenopausal women can be adnexal torsion, pelvic abscess, pyosalpinx, and hemoperitoneum –10. OvarClinicians should be aware of granulosa cell tumors which may occur at any age and prone to rupture. Frozen section will be helpful in order to avoid incomplete surgeries in malign conditions especially in postmenopausal women presented with intra-abdominal bleeding.


We report a case of an adrenal collision tumor composed of a small cell lung carcinoma metastasis and a benign adrenal adenoma identified preoperatively on FDG-PET, CT and MRI and confirmed pathologically.The patient’s history, preoperative imaging characteristics, postoperative course, and histopathology are described. A review of the literature addressing adrenal collision tumors is provided.A 47-year-old female was found to have a left upper lobe lung mass and an adrenal lesion on imaging. FDG-PET, CT and MRI of the adrenal suggested a metastatic lesion adjacent to an adrenal adenoma. CT-guided biopsy of the adrenal gland was consistent with a small cell lung cancer metastasis. The patient underwent systemic chemotherapy and had complete resolution of the left upper lobe mass. Post-treatment FDG-PET demonstrated a persistently enlarged adrenal gland with decreased but persistent FDG uptake. The patient underwent adrenalectomy and pathologic examination demonstrated a small cell lung cancer/adenoma collision tumor.This case and a review of the literature demonstrate that FDG, CT and MR imaging can all characterize the separate components of collision tumors within the adrenal gland. Composite tumors are those that have two different histologic types intermixed, whereas collision tumors are those in which two tumors abut each other or are in close proximity . CollisiHere we report an unusual case of a patient noted to have two left adrenal lesions on imaging, which were correctly characterized radiologically as a metastasis adjacent to a benign adrenal adenoma. This was confirmed by fine needle aspirate cytology and pathology after adrenalectomy. To our knowledge this is the first reported case of a small-cell lung carcinoma and adrenal adenoma collision tumor.A 47-year-old woman developed cough and hoarseness that persisted after a course of antibiotics. A computed tomography (CT) scan of the chest revealed a 6.0-cm mass in the left upper lobe of the lung and bulky hilar adenopathy. In the left adrenal, two lesions were identified on non-contrast-enhanced images: a low-attenuation 2.9 × 3.1-cm lesion suggestive of a lipid-rich adenoma, and a higher attenuation 1.2 × 1.4-cm nodule suspicious for metastatic disease (Figure The patient remained free of disease for 1 year, but was then found to have pancreatic metastases. She died 4 months later, approximately two years after her original diagnosis.in vitro and clinical evidence suggests that peri-tumoral tissue can facilitate the growth of tumor cells in and around the primary tumor via paracrine signaling [The reason why collision tumors develop is unclear. Malignancies have been found to promote local and distant sites of disease, and it is plausible that these same factors could facilitate the growth of second primary tumors. The initial tumor may create a milieu that makes it easier for a second tumor to grow ,3; in viignaling ,5. Primaignaling . Also plignaling .et al. [Collision tumors are rare in the adrenal gland at imaging, occurring in 2 out of 104 patients MRI in a study by Schwartz et al. -13. In 9et al. . This caVarious radiologic techniques are available for characterization of adrenal masses discovered incidentally during imaging performed for other purposes . ApproxiThe decision to pursue adrenalectomy for this patient was based on the patient’s age, the excellent treatment response, and the persistent adrenal lesion noted on CT that had a discordant FDG-PET response compared to the primary site of disease. Additionally, the literature supports the possible benefit of resection of a solitary site of hematogenous metastasis in non-small-cell lung cancer . The effIn summary, we report the first case in the literature of an adenoma-small-cell lung cancer collision tumor in the adrenal gland. This case and the literature review demonstrate that FDG-PET, CT and MRI can all distinguish two separate components of collision tumors within the adrenal gland and, in the case of adrenal adenoma, provide a definitive diagnosis for that component. When a satisfactory post-treatment evaluation of such lesions is not possible with these imaging modalities, surgical intervention in select patients should be considered.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.CT: Computed tomography; MRI: Magnetic resonance imaging; FDG-PET: Fluorodeoxyglucose positron emission tomography; SUV: Standardized uptake value; TTF-1: Thyroid transcription factor-1.The authors declare that they have no competing interests.BRU performed the literature review, and designed and drafted the manuscript. JS, RJD, JAC, and DMP assisted in drafting the manuscript and designing the figures. VES conceived the study and assisted in drafting the manuscript. All authors read and approved the final manuscript.


Synovial sarcoma is a rare malignant mesenchymal tumor mainly arising in the peri-articular tissue in young adults. There are few cases reported in other areas.We report the case of a 29-year-old Saudi woman of Arabian ethnicity with synovial sarcoma arising primarily from the ileum who presented with abdominal pain, a palpable mass and incomplete intestinal obstruction. A literature review was performed to gather information on this rare gastrointestinal tract sarcoma.Although it is a rare tumor of the pre-articular tissues, synovial sarcoma can present, in exceedingly rare cases, in unusual anatomical sites such as the gastrointestinal tract. We believe the reporting of all rare or unexpected presentations of sarcoma will eventually improve our understanding of this relatively unusual malignancy. Synovial sarcoma is a malignant mesenchymal tumor of uncertain histogenesis. It may be biphasic, monophasic, or poorly differentiated . SynoviaA 29-year-old Saudi woman presented to our Emergency Room with a five-day history of epigastric and lower abdominal pain associated with lower abdominal and pelvic fullness that she attributed to fibroids. The pain was intermittent, colicky in nature and associated with nausea and vomiting. She denied any history of fever and there had been no change in her bowel habits or urinary symptoms. For several months she had been followed up regularly at the gynecology clinic due to the lower abdominal heaviness and distension. Her gynecological history revealed a nulliparous woman with no vaginal bleeding. Her medical history was significant for epilepsy since childhood, which was well controlled by medication, and a laparoscopic appendectomy one year ago. On examination, she was alert and conscious. Her vital signs were stable. Her abdomen was distended with a palpable pelvic-abdominal mass that was hard, non-tender and slightly mobile. Her liver and spleen were within the normal range and she had normal bowel sounds. No lymph nodes were enlarged and digital rectal examination revealed no abnormalities.A computed tomography (CT) scan of her abdomen and pelvis showed a pelvic/abdominal mass adjacent to the distal small bowel loops separate from the uterus and ovaries, heterogeneously enhancing, measuring roughly 65 × 99 mm with multiple areas of necrosis Figure . A CT scThe case was discussed by the multidisciplinary tumor board and the advice was to confirm the diagnosis to eliminate the possibility of an ovarian origin of the tumor because of its very close proximity.Surgical exploration was indicated due to the finding of a large mass that was composed of a cystic and also a large solid component that needed to be investigated to eliminate the possibility of lymphoma or tuberculosis, which is quite common in our population. However, there was some radiological confusion about the origin of this mass and to some colleagues it was necessary to confirm it with a histological biopsy. For this reason, our patient underwent diagnostic laparoscopy, which showed a moderate amount of ascetic fluid with a large mass in the lower abdomen. When a biopsy was attempted, a large vein on the mass ruptured and bleeding made visualization extremely difficult. Therefore, the procedure was converted to an open one through a small lower midline incision. A 10 × 10 cm mass arising from the terminal ileum, occupying the pelvis and reaching to the umbilical area was found.The uterus, ovaries and the urinary bladder were not involved. After a minimal dissection the tumor was excised with 10 cm free margins of small bowel and primary side-to-side anastomosis was performed. Our patient tolerated the procedure very well and her recovery period was unremarkable. She was discharged home on the seventh post-operative day. She was reviewed on an out-patient basis by our colleagues in radio-oncology and due to the radical nature of the surgery she was not offered any adjuvant treatment. She is still being followed up by our surgical oncology team and has shown no evidence of local or distant metastasis based on clinical and CT scan results after 24 months of follow-up.Gross pathology revealed a segment of small bowel measuring 17 × 2 cm. It was adherent to a portion of ascending colon. An 8 × 7 × 3 cm white solid mass with a homogeneous cut surface was bulging from the serosal side of the small bowel; it was 4 cm away from the proximal resection margin. The tumor was adherent to the large bowel but not infiltrating it grossly. Few lymph nodes were identified within the mesenteric fat. Microscopy showed that the tumor was composed of cellular spindle cells with a mild to moderate degree of pleomorphism arising from the small bowel wall and mainly involving the submucosa, muscularis propria and serosa. Focal mucosal erosion was identified. The tumor cells showed a moderate amount of eosinophilic cytoplasm, evenly distributed nuclear chromatin and a few conspicuous nuclei Figure . The mitImmunohistochemical testing results showed that the tumor was positive for S100, epithelial membrane antigen (EMA), B cell lymphoma 2 (Bcl 2), and vimentin Figure . CD99 teA cytogenetic study of the specimen was conducted on a formalin fixed paraffin embedded tumor sample. The result of a real-time polymerase chain reaction (RT-PCR) procedure for synovial sarcoma-associated fusion transcript (SYT-SSX1 and SYT-SSX2) was negative.Our patient was seen periodically in our surgical oncology clinic and she has remained disease free for 24 months with no clinical or radiological evidence of local recurrence.Gastrointestinal sarcoma accounts for 0.1% to 3% of all gastrointestinal (GI) malignancies and approximately 10% of all sarcomas ,14. In tThe median age at diagnosis was 41.5 years . There is no significant sex predilection with a male to female ratio of 1:1.15. There are 11 and 12 cases of GI tract synovial sarcoma arising from the esophagus  and stomach, respectively, representing the most common sites. The presenting symptoms were pain, obstruction or bleeding. The histological subtypes were monophasic in 15 patients and biphasic in 11 patients including two patients with a poorly differentiated component. The remaining patients' histological subtypes have not been reported. All of the reported cases were treated surgically. The number of patients who received adjuvant chemotherapy or radiotherapy or both are five, four and three, respectively. Only one patient had metastasis at first presentation. Billings  reportedIn soft tissue synovial sarcoma, numerous studies over the years have reported that it is a high grade malignancy with a high rate of metastasis leading to death; 10-year survival rates between 24% to 68% and 11% to 56% have been reported ,33.et al. [In the article by Bergh et al. , the invIn general, sarcomas are relatively rare tumor entities, particularly in the GI tract. For many reasons, it is easy to confuse the subtypes of sarcomas, including the histological similarities, the experience of the centers dealing with such tumors, proper handling, and staining and immunohistochemical findings. It is also likely to mistake such extremely rare presentations of an unusual malignancy such as synovial sarcoma for relatively more common tumors such as GISTs, which can present with more or less the same clinical scenario. The clinical importance comes from the fact that sarcomas carry a slightly worse prognosis and need a closer follow-up not to miss any sign of local recurrence or distant metastasis. We also believe that any rare presentation of any subtype of sarcoma should be carefully reported and documented to add to the relatively small pool of cases in comparison with other common malignant tumors, in order to increase our knowledge and understanding of sarcoma in general.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.AA collected the data and informed consent, wrote the manuscript, organized the text and photos and conducted the literature review. MF reviewed the histopathological diagnosis, provided the magnified histological slide photos, followed up with other universities for confirmation of histology and histochemical diagnosis. YA provided the surgical details, followed up our patient's progress and details of her history, and reviewed the article and revised the manuscript. All authors read and approved the final manuscript.


We report on a very rare case of a supratentorial primitive neuroectodermal tumor in an adult, which presented with intracerebral hemorrhage, and review the relevant medical literature.A 42-year-old Caucasian man complained of a sudden headache and nausea-vomiting. The patient rapidly deteriorated to coma. An emergency computed tomography scan showed an extensive intraparenchymal hemorrhage that caused significant mass effect and tonsilar herniation. During surgery, an increased intracranial pressure was recorded and extensive bilateral decompressive craniectomies were performed. A cherry-like intraparenchymal lesion was found in his right frontal lobe and resected. The patient died in the intensive care unit after approximately 48 hours. The resected lesion was identified as a central nervous system primitive neuroectodermal tumor.Supratentorial primitive neuroectodermal tumors must be considered in the differential diagnosis of space-occupying lesions in adults. Spontaneous supratentorial hemorrhage due to primitive neuroectodermal tumors is an extremely rare but potentially lethal event. Primitive neuroectodermal tumors (PNET) constitute a heterogeneous group of embryonal malignancies that are composed of undifferentiated or poorly differentiated cells and are characterized by an aggressive clinical behavior ,2. They PNETs in adults usually present with signs of increased intracranial pressure (ICP), seizures and/or focal neurological deficits -15. HoweA 42-year-old Caucasian man was admitted to the emergency department of a district hospital in a comatose condition . His relatives reported that he initially complained of a sudden onset of headache and had several episodes of emesis. He was sedated, intubated and urgently transported to our teaching hospital. On arrival at our facility, he was unresponsive to verbal and painful stimuli, and his pupils were bilaterally dilated and non-reactive. An urgent computed tomography (CT) scan of his brain showed an extensive intraparenchymal hemorrhagic lesion, causing significant mass effect and tonsilar herniation , and extensive bilateral decompressive craniectomies were urgently performed. During the surgery, and after the evacuation of the hematoma, a cherry-like intraparenchymal lesion was found in his edematous right frontal lobe, which was resected Figure . DecomprA head CT scan at the end of surgery showed diffuse areas of low-density in both brain hemispheres, suggesting severe ischemia Figure b. His coThe surgical specimen was sent for microscopic and immunohistochemical pathological analysis. Permanent specimen sections were stained with hematoxylin and eosin and were assessed via immunohistochemistry for vimentin, synaptophysin, glial-fibrillary acidic protein and Ki-67 to measure the proliferation index. Uniform neoplastic medium-sized cells, with round to oval hyperchromatic nuclei, were present and positive for vimentin, synaptophysin and cluster of differentiation 56, but were negative for glial-fibrillary acidic protein Figure . The Ki-The term PNET was introduced in 1973 for describing initially supratentorial, pediatric brain tumors that were largely undifferentiated or primitive but contained small foci of glial or neuroblastic differentiated cells of primitive neuroectodermal origin . The preWe performed a literature review in MEDLINE and PubMed, limited to the English language and ranging from January 1973 to December 2012. We used as key terms the words ‘PNET’ or ‘primitive neuroectodermal tumour’, intracerebral hemorrhage’ or ‘intracerebral haemorrhage’ or ‘intracerebral bleeding’ and ‘adults’, ‘man’, ‘woman’. No patients younger than 20 years old were included in the literature review. Altogether, 278 articles were retrieved. Two of the authors (EKP and AGB) read the abstracts of the gathered articles and all irrelevant articles were eliminated. The full-text articles of the remaining hits were gathered and thoroughly studied and their references scrutinized for further relevant cases. The results are presented in Table Twenty six publications have reported on 70 supratentorial not otherwise specified PNET cases in adults. These tumors occurred in adults up to 88 years old  with a sMost patients present with symptoms and signs of raised ICP such as headaches, nausea and/or vomiting, confusion and papilledema because of the rapid tumor growth -8,13,15,Adult PNETs demonstrate no specific imaging characteristics in comparison to their counterparts in children. They may appear as isodense or hyperdense lesions on a CT scan, with intratumoral calcifications or microhemorrhages . On magnOn macroscopic examination, adult PNETs usually appear as lobulated, purple-grayish or pinkish masses. On histology, the tumor cell population consists of undifferentiated small cells with ill-defined, scanty cytoplasm, and round or oval cells with hyperchromatic nuclei ,3. MitotMYCN or MYCC gene amplifications, and polysomies of chromosomes 2 and 8 [MYCN or MYCC gene amplifications has been shown to be associated with decreased survival. Similarly, polysomies of chromosomes 2 and 8 have been individually associated with decreased survival in children, and the combined presence appears to be an even more unfavorable prognostic factor [Newer genetic analysis studies have demonstrated that PNETs are characterized by  2 and 8 . Variousc factor . The vasExtensive surgical resection of adult PNET is the usually recommended mode of treatment, followed by radiotherapy and chemotherapy Table . Postsuret al. [et al. [The prognosis for adults with supratentorial PNETs seems to be worse than that of their pediatric counterparts -5,7,29. et al.  reportedet al. ,16,26,27et al. . By cont [et al.  reportedSupratentorial PNETs must be considered in the differential diagnosis of space-occupying lesions in adults. Spontaneous supratentorial hemorrhage is an extremely rare but potentially lethal presentation of PNET in adults.Written informed consent was obtained from the patient’s relatives 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.All authors contributed in writing the manuscript. EKP and AGB performed the literature review. KNF was responsible for the text editing. AGB was responsible for writing the manuscript. KNP was in charge of manuscript supervision and is the corresponding author. All authors read and approved the final manuscript.


Adenofibroma is an extremely uncommon benign tumor composed of glandular and fibrous tissues. It occurs more often in the endometrium but it can also occur in the cervix and extrauterine sites. We report a case of a 32-year-old asymptomatic woman with cervical adenofibroma, first detected in a routine endovaginal ultrasound, as a cervical mass containing multiple cystic components. Histopathologic findings diagnosed its nature. As adenofibromas are very rare, we present this case with a brief review of the literature. Endometrial adenofibroma is a rare benign mullerian mixed tumor composed of benign epithelial and mesenchymal components. The major proportion of adenofibromas arise from the endometrium (90%); less often occur in the cervix  and in oWe report a case of an adenofibroma of the uterine cervix in a young patient.A 32-year-old, nulliparous, woman went to her gynaecologist for routine procedures. She had no complaints and referred regular menstrual cycles, with oral combined contraception. Pelvic examination was normal. Endovaginal ultrasound revealed an intracervical multicystic mass, with 45 mm in its maximum diameter . DiagnosIt was in 1971, by Abell, that adenofibroma of the uterine cervix was first described . There hIn fact, as a result of its low incidence, the origin of this tumor is still a source of debate; some authors believe it represents a form of endometriosis with extreme smooth muscle metaplasia—endomyometriosis . More rePhysical examination can be either normal, as the patient presented herein, or it can reveal an enlarged uterus with a polypoid tumor projecting from the cervix.The sonographic pattern produced by this tumor is an echogenic intracavitary mass with heterogeneous multicystic components, with well-defined margins and low-resistance blood flow. Adenofibroma must be differentiated from endometrial polyp, hyperplasia, and carcinoma. Macroscopically, adenofibroma presents as a broad-based polypoid lesion; commonly has a fibrous consistency and contains dilated cystic spaces. Its size ranges from 2 to 20 cm, with an average diameter of 7 cm [In adenofibroma, the stromal component is by definition morphologically benign. However, it is described the possibility of this tumor to invade the myometrium and the pelvic veins, to recur or even metastasize , 8, 9. WIn conclusion, although rare, adenofibromas should be considered in the differential diagnosis of a patient with a cervical mass and abnormal vaginal bleeding without clinical evidence of malignancy. Detailed histopathological study is required to differentiate adenofibromas from adenosarcoma.


Purpose: To report adult cases of superior orbital apocrine hidrocystoma. Methods: Retrospective case series of three patients with superior orbital apocrine hidrocystoma and blepharoptosis with review of the clinical aspects of each of the cases. Results: All three cases presented with blepharoptosis. Two of the cases had occult hidrocystoma, and one was visibly subcutaneous at presentation. Conclusions: Although rare and more common along the eyelid margin, apocrine hidrocystomas may occur in the orbit leading to secondary blepharoptosis and should be included within the differential diagnosis of orbital cysts. Physicians should therefore be aware of this possibility. Hidrocystoma cysts derive from sweat glands, and are classified according to the type of secretion as either eccrine or apocrine ,2,3. EccTheir occurrence in the orbit is extremely rare ,7,8,9,10Case 1: A 46-year-old white man presented a 25-year history of a painless slowly progressive mass in the medial right upper orbital rim. There was no history of previous trauma or ocular disorders. Clinical examination revealed a discrete superior right eyelid ptosis and a nodule located medially below the right supraorbital margin. It was bluish, round, soft, non-tender, vascularized, had no fixation to the overlying skin or the deeper planes, and measured 1.5 × 1.5 cm . Eccrine hidrocystoma, in contrast, are usually small (1–3 mm), located exclusively in the periorbital and lateral canthal area and they are absent at either the upper or lower eyelid margins or in the perimarginal skin, but may be rarely present in the dermis typically at the mid-level of the upper eyelid pretarsal skin, or in the lower eyelid skin near the inferior pole of the lower eyelid tarsus .Sudoriferous cysts arising from the sweat glands of Moll are usually found on the eyelids and have apocrine origin ; they arHidrocystomas are microscopically distinguished based on the production of secretion in two varieties: (1) apocrine in which the lining cells release the apical part of their cytoplasm and the remainder of the cell being viable; and 2) eccrine, which sustain no loss of cell structure . Apocrin eccrine,There are more sophisticated tests to distinguish apocrine from eccrine hidrocystoma in the unaided light microscopy cases: human milk fat globulin-1 (1.10.F3) monoclonal antibody, cytoplasmic granules containing epidermal growth factor, gross cystic disease fluid protein-15 (GCDFP-15), cytokeratin 7 (CK7), alpha-smooth muscle actin (αSMA) ,15,16 anHistological findings also enable hidrocystomas to be distinguished from other lesions such as: cystadenoma, which will show a cystic lesion with an adenomatous component; dermoid cysts characterized by keratinizing squamous epithelium with a keratohyalin layer and fibrous wall that may contain adnexal structures  ,16,17; cA failure to evaluate the cytologic subtleties may lead to misdiagnosing hidrocystoma. The most common error is to mislabel a cyst as a hidrocystoma instead of conjunctival cyst . A true Though surgical excision is not generally difficult, incomplete excision of orbital cysts carry high risk for recurrence . Eyelid Fortunately in our three cases the diagnosis was benign, but this may not always be the case and some orbital lesions, if not fully excised, carry malignant transformation potential. As highlighted in this case series, unusual blepharoptosis patients should be referred to an oculoplastic-experienced surgeon with orbital experience to ensure optimal patient safety and outcome.In conclusion, although rare and most common along the eyelid margin, hidrocystoma should be included in the differential of orbital cysts. It is important to be aware of the variable presentation of orbital hidrocystomas. Unusual blepharoptosis, dysmotility, globe displacement, and/or proptosis are warning signs of a potential underlying secondary cause, such as an orbital mass. Patients presenting with unusual blepharoptosis  should undergo upper eyelid palpation, eyelid eversion, a thorough ocular exam, extraocular movement testing, be considered for orbital imaging and a surgical approach that will help exclude an anterior orbital mass as the cause.


Mucinous cystic neoplasms (MCN) are an uncommon form of exocrine neoplasms of the pancreas. Symptoms are most often vague and this makes the diagnosis more difficult. The current case is one of three cases yet reported where the MCN caused left-sided portal hypertension leading to the formation of isolated gastric varices and subsequent bleeding from the varices. In the previously reported cases the main symptom was hematemesis. However in the current case the patient experienced no hematemesis, only isolated incidents of dark coloured diarrhea, but the main symptoms were those of iron-deficiency anemia. We present the case report of a 34-year-old woman who presented with dizziness and lethargy and was found to have 12 cm MCN in the pancreas. Mucinous cystic neoplasms (MCN) are a rare form of exocrine pancreatic tumors . Even thA 34-year-old woman presented to the emergency room with a few days history of dizziness and lethargy. Severe anemia was detected . The patient had not experienced hematemesis or fresh blood in stool but she had experienced an isolated incident of diarrhea, dark in color, two days before presenting to the hospital and at least one time before in the same month. Three weeks earlier she had been admitted to the hospital with the same symptoms and was found to have iron-deficiency anemia. Upper gastrointestinal endoscopy and colonoscopy did not reveal any explanation for the anemia. She was discharged, after receiving a blood transfusion, with a planned capsular endoscopy (CE) but presented to the hospital again with worsening symptoms before the CE was performed.Her past medical history was unremarkable except for hypothyroidism and her only regular medication was the thyroxin replacement. A year earlier she had suffered a motor vehicle accident with a rather serious frontal collision, but examination at the emergency reception did not indicate need for computerized tomography (CT) scan to be undertaken at the time. She was a nonsmoker and used alcohol in moderate amounts.At the current presentation physical examination revealed tachycardia (126 beats per min) but normal blood pressure (117/66 mmHg) and otherwise normal vital signs. Examination of the abdomen revealed no tenderness, abnormal masses, or organomegalies. Apart from the marked microcytic anemia, other blood tests were within normal range, including liver tests. A slight elevation of the tumor marker CA 19-9 (41.2 U/mL) was found. An upper gastrointestinal endoscopy revealed isolated gastric varices in the fundus of the stomach and red spots in the mucosa of the varices indicating a previous recent bleeding . HoweverLaparotomy demonstrated that the tumor had massive adhesions to surrounding organs. In order to resect the tumor a distal pancreatectomy was performed along with a splenectomy, a sleeve resection of the stomach, and a partial hemicolectomy. Macroscopic pathological examination displayed a multilocular cystic tumor, 12 cm in greatest dimension with one large dominating cyst and multiple smaller ones, containing yellowish fluid and no papillary structures on the internal surface. The cyst walls were fibrous and were attached to the surrounding organs. Microscopic examination showed a mucinous, columnar epithelium lining the cystic surfaces, without any cellular or nuclear atypia. The subepithelial stroma was of ovarian-type. These findings were compatible with the final diagnosis of mucinous cystadenoma . Given tMucinous cystic neoplasms (MCN) of the pancreas are relatively uncommon, comprising about 2–5% of all exocrine neoplasms of the pancreas . They ocLesions of the body or tail of the pancreas can, in rare situations, cause hindrance to blood flow in the splenic vein and lead to an increased collateral blood flow through the short gastric veins in the fundus of the stomach to the coronary vein and portal vein . This veIn spite of having the potential to grow considerably and most often being situated in the body or tail of the pancreas, mucinous cystadenomas are an unusual cause of left-sided portal hypertension and isolated gastric varices. Two cases have been reported before the current case, one from Taiwan  and the


The presentation of myeloid sarcoma (MS) in the bone is common; however, rarely does the tumor occur in the sacral spine, and in a normal patient with no history of acute myeloid leukemia. The present study describes the rare case of a previously healthy 24-year-old male patient, who presented with a history of six months of repeated pain in the right leg and hip and limping for less than a month, who was diagnosed with sacral MS. Despite receiving surgical management and chemotherapy promptly subsequent to the diagnosis and undergoing close observation following the treatment, the patient still developed metastases to multiple sites of the brain. Taking into account the similar presentation of this rare disease to other entities, the early and accurate diagnosis of MS is vital, and the condition should be considered as a threatening manifestation with the possibility of metastasis to other sites of the body. Myeloid sarcoma (MS), also known as granulocytic sarcoma or chloroma, is a rare neoplastic condition that is characterized by the occurrence of one or more tumor masses, consisting of immature myeloid cells presenting at an extra-medullary site . MS is sA 24-year-old male patient was admitted to the West China Hospital of Sichuan University  on 29 March, 2012 with a history of six months of repeated pain in the right leg and hip and limping for less than a month. The patient had not initially paid attention to the symptoms but the pain later was so severe that it became difficult for him to sleep and walk normally. The symptoms were also accompanied by frequent urination and constipation and by slight urinary incontinence while sneezing. There was no past history of medical illness, including AML, or surgery.Physical examination on admission revealed slight hypoesthesia in the right hip and bilateral lower extremities. Other neurological examination results were unremarkable. MRI of the spine revealed a mass lesion within the spinal canal extending from L5 to S2. The mass had intermediate signal intensity on the T1- and T2-weighted images and showed relatively homogeneous enhancement in contrast-enhanced scans. The lesion extended from the sacral hole into the right paravertebral soft tissue. Destruction of the adjacent bone was also observed . The priFollowing the preoperative examinations, the patient underwent L5-S2 decompression semi-laminectomy under general anesthesia on 31 March, 2012. The tumor was partially resected, as it was closely adherent to the dural sac and surrounding structures. Pathological material obtained at surgery revealed malignant MS. As shown in The patient received chemotherapy twice following the surgery with daunorubicin (70 mg/day) for three days and cytosine arabinoside (400 mg/day) for seven days, along with platelet transfusion during the second course of chemotherapy and other symptomatic treatment. Improvements were observed in the MRI following the treatment with a reduction in the size of the tumor ; howeverMS is a rare tumor of immature granulocytic cells that appears concomitantly in 15–35% of cases with AML. It can affect any organ system, such as in the head, neck, bone and skin, and is less commonly found in the central nervous system (CNS) , particuThe diagnosis of MS relies on a number of examinations, including bone marrow aspiration, biopsy or peripheral blood smears and immunohistochemical detection. More importantly, myelo- and/or monoblasts in the MS lesions exhibit antigenic profiles and express myeloid- and monocytoid-associated antigens, including CD13, CD14, CD33, CD64, CD68 and c-Kit (CD117), as well as lysozyme . StaininIn the present case, MRI was used to detect the abnormality within the spine and later estimate the efficacy of the treatment. MRI is said to be the technique of choice in diagnosing intraspinal or paraspinal pathology and is useful in distinguishing MS from other entities, i.e. hemorrhage or abscess , althougApproximately two years after the diagnosis of the disease in the present patient, metastases, which may have travelled through the CSF, were found in multiple sites of the CNS; to the best of our knowledge, such an incidence has not yet been reported in any case of MS of the sacrum. Careful investigation and diagnosis are of the utmost importance in cases of MS and may have a significant impact on the treatment, survival rate and prognosis of the patient. Numerous cases of MS have been misdiagnosed as lymphoproliferative disorders, particularly lymphoma . AlthougThe optimal treatment of MS is not yet well defined; however, the options include surgical resection, intravenous and/or intrathecal chemotherapy, radiation therapy or any combination of these treatments. A previous review showed that patients receiving systemic chemotherapy may have an advantage with 46% one-year survival as compared with 16% one-year survival in patients treated with other methods . To dateIn conclusion, the present study describes a rare case of sacral MS in a previously healthy, non-leukemic patient associated with metastasis of the tumor to the brain. Although the patient has been relieved of the symptoms and the tumor mass has reduced in size, the treatment of the metastases remains to be determined. Several points can be made from the current case. Firstly, sacral MS, which presents with similar symptoms to other spinal lesions, cannot be neglected and should be kept under the differential diagnosis of spinal mass lesions. Secondly, important examinations, such as MRI, bone marrow aspiration and pathological and histological staining, can provide an accurate diagnosis if carried out promptly. Finally, although several methods have been proposed, the patient in this case received prompt surgical resection and induction chemotherapy, which may have been the factor that has led him to survive for approximately two years, to date. Routine MRI should be considered as an important investigative step for metastasis.


Acute generalized exanthematous pustulosis (AGEP) is a rare and severe subtype of drug eruption, characterized by acute, extensive, non-folicular, sterile pustules overlying an erythematous skin, associated with fever and leukocytosis.Case report of a 6-year-old female child with encephalopathy and chronic lung disease, who was treated with intravenous antibiotics, antipyretic and antiepiletics during hospitalization for sepsis. She evolved with diffuse erythemata and non follicular pustules on her trunk, flexural areas, palms and soles.The drug history, clinical presentation, cutaneous histopathological findings  and the prompt regression of the lesions after withdrawal of the suspected drugs were consistent to ratify the diagnosis of AGEP.AGEP is an uncommon reaction , usually seen after drug exposure and clinicians should keep in mind the possibility of this skin eruption.Written informed consent was obtained from the patient for publication of this abstract and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


Desmoid tumors are rare soft tissue tumors derived from fascia and connective tissue of the muscular layers. The abdominal region is the most frequent site of involvement, whereas involvement of sternal manubrium is rare. We report the case of a rare desmoid tumor in the sternal manubrium mimicking radiological and metabolic features of malignant tumor, which was successfully treated by sternal resection and reconstruction with autogenous rib grafts. Desmoid tumors are rare, benign, soft-tissue neoplasms, and are characterized by infiltrative growth and a tendency towards local recurrence. Common sites of desmoid tumors include abdominal wall, extremities, shoulder, neck, and chest wall. The sternum is the least common site. We herein report a rare desmoid tumor of sternal manubrium mimicking malignant tumor such as chondrosarcoma, which was successfully treated by complete resection of the tumor and immediate sternal reconstruction with autogenous rib grafts.18F-fluorodeoxyglucose–positron emission tomography (FDG-PET) revealed metabolically active areas around the manubrium, both sternoclavicular joints, and bilateral first sternocostal joints, which was suggestive of malignant tumor of the sternal manubrium. The patient refused to undergo preoperative fine-needle aspiration of the lesion. Thus the patient underwent a radical en bloc resection of the tumor and sternal reconstruction with autogenous rib grafts. At surgery, an expansile lesion involving the entire manubrium, both sternoclavicular joints, and bilateral first sternocostal joints was found. En bloc radical manubriectomy was performed including portions of the bilateral clavicles and first ribs. The specimen was removed 4 cm beyond the margin of the lesion. Then through a left anterior lateral incision, about 10 cm of the left fifth rib was harvested from the subperiosteum and carved into two parts with preservation of intact periosteum and pleura. Slots were cut into the ends of the two rib clips, bilateral clavicles, and the edge of the sternum using an electric drill. The two rib grafts were placed to form a T shape between the edges of the residual sternum and bilateral clavicles using rib nails, and then fixed with stainless steel wires. Histopathological examination revealed long fascicles of spindle-shaped cells with slight atypia arranged in a collagenous stroma, indicating a diagnosis of desmoid tumor  scan of the chest showed a 4× 4 cm mass based on the sternal manubrium, with no apparent involvement of the mediastinal structures Figure A and B. r Figure A and B. Desmoid tumors are relatively rare, benign, soft-tissue neoplasms most often derived from fascial or musculoaponeurotic structures, accounting for 0.03% of all neoplasms . This tuCT and FDG-PET can reveal the size, location and metabolic feature of tumors, but histopathological assessment is required to make the definitive diagnoses when the main differential diagnoses are malignant tumors such as chondrosarcoma, as in our patient. Preoperative biopsies or surgical removals are often performed to obtain the final diagnoses. Our patient chose surgical resection of the lesion directly without preoperative biopsy. Histopathologically, the desmoid tumors are mainly characterized by focal increased cellularity, rare mitotic activity, bland cytology, and absence of herringbone pattern, which are different from chondrosarcoma .Desmoid tumors are locally aggressive tumors with a high local recurrence rate . If mediThe autogenous rib has been considered as one of the most efficacious materials to be used in sternal reconstruction. In our initial report, we proposed the use of autogenous rib grafts as a safe and effective choice for the reconstruction of sternal defects . In thisIn conclusion, we describe an extremely rare case of desmoid tumor in the sternal manubrium mimicking malignant tumor, which was successfully treated by complete resection of the tumor and immediate sternal reconstruction with autogenous rib grafts. The patient has been disease free after 46 months of follow up without any other treatment. We consider that the sternal reconstruction with autogenous rib grafts is a simple and effective solution to a complex problem.Although desmoid tumors of the sternum have been described in a few case reports, they are still rare entities. Radical en bloc resection of desmoid tumor of the sternum with a negative margin and sternal reconstruction with autogenous rib grafts is safe, effective and feasible.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.


Encapsulating peritoneal sclerosis (EPS) is a rare surgical complication that can occur after intraperitoneal treatment. It is also a serious and potentially fatal complication of continuous ambulatory peritoneal dialysis. The present report describes a case of surgically treated EPS that probably occurred as a complication of hyperthermic intraperitonal chemotherapy (HIPEC).A 39-year-old man required sigmoidectomy for serosal invasive advanced sigmoid colon cancer. HIPEC with oxaliplatin, 5-fluorouracil and mitomycin C were given as adjuvant therapy. Subsequently, intestinal obstruction developed at 15 months postoperatively, and the patient was hospitalized. Abdominal computed tomography showed a dilated small intestine enveloped by a thickened membrane. We found no evidence of peritoneal recurrence, but exploratory surgery revealed EPS, probably caused by HIPEC. We peeled the capsule off of the intestine. The patient’s postoperative course was uneventful, and sufficient nutritional intake after surgery was noted. Seven months after surgery, he is well with no recurrence.The surgical treatment via peritonectomy and enterolysis for postoperative EPS appears safe and effective. A diagnosis of EPS should be considered when intestinal obstruction does not show improvement with conservative treatment in patients who have undergone HIPEC, provided the possibility of peritoneal cancer recurrence is excluded. The use of cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is a novel approach that offers improved survival after peritoneal surface malignancy, including colorectal peritoneal metastases (PM) -3. HIPECEncapsulating peritoneal sclerosis (EPS) is a rare surgical complication that can occur after intraperitoneal treatment. It is also a serious and potentially fatal complication of continuous ambulatory peritoneal dialysis (CAPD) . Some reA 39-year-old man with serosal invasive advanced sigmoid colon cancer underwent surgery at Shiga University of Medical Science Hospital. Sigmoidectomy with regional lymph node dissection followed by reconstruction with stapled functional end-to-end anastomosis was performed. HIPEC was subsequently delivered as described previously . BrieflyThis report described a rare case of EPS after HIPEC that was successfully treated via the surgical approach. EPS causes intestinal obstruction, because the small intestine is enveloped by a secondary thick fibrosis. Several report showed the morbidity rates after HIPEC were 20.8-53.3% . Major cPreviously, 9 cases of EPS after IPC and 1 case of EPS after HIPEC were reported (Table An intraperitoneal temperature of 42-43°C is necessary to obtain the maximal therapeutic effects of HIPEC . PreviouSteroid therapy is considered to be effective for preventing EPS, because the “second hit” in development of EPS may be related to various cytokines. The second hit theory whereby dialysis fluids contribute to peritoneum damage. Cases of peritoneal dialysis-related EPS have responded favorably to corticosteroid therapy alone . In our In our case, incision and resection of the sclerosing peritoneum resulted in improvement of stenosis without postoperative complications. It is important that the enterolysis is performed without damaging the encapsulated intestine. The overall morbidity rate of surgery for EPS is reported as approximately 60% . HoweverPreoperative distinction among adhesion, EPS, or PM can be difficult when small bowel obstruction occurs in patients who have received HIPEC. A diagnosis of EPS should be considered if intestinal obstruction does not show improvement with conservative treatment, provided that the possibility of PM is excluded. Early laparotomy and resection of thickening fibrosis are important to improve the patient’s quality of life. In addition, ongoing efforts to increase the feasibility and safety of intraperitoneal chemotherapy are warranted.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 of this journal.EPS: Encapsulating peritoneal sclerosis; HIPEC: Hyperthermic intraperitoneal chemotherapy; PM: Peritoneal metastasis; CAPD: Continuous ambulatory peritoneal dialysis; IPC: Intraperitoneal chemotherapy; CT: Computed tomography.None of the authors have a conflict of interest or have received financial support.KT drafted the manuscript, and conducted a literature search. HS, TS and HO conducted a literature search and contributed to drafting the manuscript. MI evaluated histopathological examination. HS, TS, HO, EM, YE and TT performed the operation and reviewed the manuscript and gave final approval for publication. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2482/14/57/prepub


A 10-year-old boy presented for evaluation of a slowly growing congenital scalp lesion  and 2. HWhat is nevus sebaceus (NS) of Jadassohn?What are the stages of clinical progression?What is the rationale for excision versus observation?When should excision be performed?-Nevus sebaceus of Jadassohn is a congenital abnormality first described by the dermatologist Josef Jadassohn in 1895.2During infancy, NS typically appears as a smooth or velvety yellow-orange well-circumscribed plaque. Classically, it is located on the vertex of the scalp but has been described on other areas such as the face or neck.4,6,7Individuals with NS may develop benign or malignant tumors, with increasing evidence supporting the former to be the majority. A meta-analysis of 4900 cases found secondary tumor development in 24% of patients, most commonly benign basaloid proliferations such as trichoblastomas.42 lasers have been shown to enhance cosmesis but can only reach the papillary dermis and are therefore not recommended because of the possibility of malignant degeneration of remaining cells in the lower dermis.6The timing of surgical intervention is controversial. While smaller lesions may be technically easier to excise, younger patients may not be amenable to local anesthesia or able to safely tolerate a general anesthetic. Secondary tumor transformation seems to be seen almost exclusively in adults, as one retrospective analysis found that 96% of all NS-derived malignant tumors occurred in patients older than 18 years and the remaining 4% in patients aged 11 to 17 years.Nevus sebaceus is a rare congenital abnormality that can lead to cosmetic deformity, alopecia, or malignant transformation in rare circumstances. The lesion is usually present at birth, slowly enlarges with age, and typically grows most rapidly during the pubertal hormonal surge. The most common malignant transformation is into basal cell carcinoma, but the most common growth is a benign trichoblastoma. No definitive consensus on surgical intervention versus close observation exists. In the absence of worrisome signs for malignant transformation, management of NS is a decision best made by the child, his or her family, and the surgeon, based on the safety/tolerability of anesthesia, their expectations of oncologic safety, and the understanding of more extensive excision required should increased growth occur.


Intramuscular hemangiomas are unique vascular tumors which are benign in nature, most commonly occurring in the trunk and extremities. When present in head and neck, they most frequently involve the masseter and trapezius muscles, accounting for less than 1% of all hemangiomas. Most of these lesions present with pain and discomfort and some patients may demonstrate progressive enlargement. Due to their infrequency, deep location, and unfamiliar presentation, these lesions are seldom correctly diagnosed clinically. Our report is a clinically misdiagnosed case of a painful soft tissue mass in the right side masseteric region of a 23-year-old female patient, confirmed as intramuscular hemangioma based on imaging studies and histopathologic examination, treated by surgical excision which had no recurrence after a 3-year followup. Hemangiomas are vascular neoplasm, constituting 7% of all benign tumors . These aA 23-year-old lady reported to the Department of Oral Medicine & Radiology, Sri Ramachandra University and Research Institute, with a complaint of swelling in the right side of the face for the past 6 months. She had incidentally noticed the swelling which remained of the same size for 3 months, after which it became more bulging. Over the last 3 months she experienced intermittent pricking type of pain. Medical history was unremarkable. Clinical examination revealed a diffuse swelling in the right mandibular body region measuring about 2 × 2 cm in dimension, which was warm, tender, and soft in consistency. The swelling was mobile in the horizontal direction and showed restricted mobility in the vertical plane. Overlying skin was pinchable . On clenRoutine hematological investigations were within normal limits. Posteroanterior view of mandible showed no pathology; ultrasonography showed a 2.2 × 0.6 cm mixed echoic lesion within the right side masseter muscle with a speck of calcification . Left maUnder general anesthesia, preauricular skin incision was made, dissecting lateral to the parotid gland, and skin flaps were raised. Within the masseter a small bulging mass, measuring about 2 × 2.5 cm in dimension, was evident. The branches of the facial nerve were preserved. The external carotid artery was looped and proximal vascular control was achieved; small feeding vessels were individually ligated and blood loss during the procedure was minimal. The mass was completely removed with a margin of normal surrounding muscle to prevent recurrence. Primary closure was done. Postsurgically she was prescribed with antibiotics and analgesics for 5 days. There was mild postoperative facial edema, which subsided within twenty days with no evidence of pain and significant cosmetic problem. Histological examination revealed fibrofatty tissue and fragments of muscle with several thick walled and thin walled vessels with occasional nerves. There were also areas of hemorrhage and congestion, suggestive of a venous hemangioma . After aHemangiomas are benign vascular neoplasms or hamartomas, which are indigenous to the site of origin. Intramuscular hemangiomas are very rare with masseter muscle accounting for 5% of all intramuscular hemangiomas; other frequently involved muscles are trapezius, extraocular muscles, sternocleidomastoid, and temporalis. Their growth may be accelerated with a growth spurt or trauma and tend to enlarge slowly. They can spontaneously regress. Malignant transformation is rare. A sudden increase in size on taking oral contraceptive pills has also been reported . They arMost hemangiomas can be diagnosed on clinical examination and do not require any investigation or any treatment as they tend to subside spontaneously. However, imaging is needed in cases of deep hemangioma with normal overlying skin or in cases of clinically atypical soft-tissue masses. When imaging is used, it is important to choose the modality based on the specific lesion and clinical situation. Conventional radiographs help in identifying phlebolith and calcifications, but they may not be specific. Ultrasonography and magnetic resonance imaging (MRI) are the commonly used modalities of choice. In intramuscular hemangiomas, Colour Doppler sonography is exclusively useful to demonstrate the vascular structures in and around the muscle and to evaluate the pathological changes like fibrosis and to detect calcifications. In our case presence of calcification was not evident on plain radiography and MRI, but sonography demonstrated its presence. Hemangioma can be distinguished from other soft tissue lesions by the features of abundant vascularity and high blood flow velocity. Colour Doppler signal in a well-defined hypoechoic mass with heterogeneous echotexture should raise the possibility of hemangioma . HemangiOn pathologic analysis, vascular lesions can be classified as capillary, cavernous, venous, and arteriovenous malformations depending on the predominant anomalous vascular channels.In our case, imaging studies revealed that the lesion is present within the masseter muscle and the differential diagnosis includes masseteric hypertrophy which can be unilateral or bilateral and mostly asymptomatic. Etiology includes defective occlusion, temporomandibular joint disorder, congenital and functional hypertrophies, and emotional disorders. Increased masseteric bulk in sonography is diagnostic. Soft tissue abscesses are mostly due to bacterial infections and appear as focal hypoechoic area with echogenic debris, pus, and occasionally gas. Malformations can be grouped as either high-flow (arteriovenous) or low-flow  vascular lesions. Flow characteristics are best demonstrated by Doppler sonography. Lymphatic malformations are present from birth and are usually detected before the age of 2 years. They generally appear cystic with thick or thin septae with interspersed solid areas on sonographic examination.The treatment of choice is total excision. Surgical excision is associated with 9–28% recurrence rate [Intramuscular hemangioma should be considered in the differential diagnosis, whenever a soft tissue lesion with pain in a skeletal muscle of a young adult is encountered. Sonography and MRI are excellent diagnostic aids in such lesions.


Chondrosarcoma is a common malignant bone tumor, which accounts for 20% of all malignant bone tumors. It often occurs in the long bones, but the incidence of scapular chondrosarcoma is rare. Here, we describe a case of a large chondrosarcoma occurring in the scapula which was treated with Malawer limb salvage surgery. The patient retained considerable limb function after complete removal of the tumor tissue as assessed at the follow-up visit two years and ten months following surgery. Chondrosarcoma is a malignant bone tumor and it is the second highest cause of morbidity among adult bone tumors. It accounts for 20% of all malignant bone tumors. Chondrosarcomas constitute a heterogeneous group of neoplasms that share the common feature of producing the cartilage matrix . GeneralChondrosarcoma does not usually develop in the osteoid tissue. It usually occurs in cartilage of the pelvis, femur, tibia and humerus. Scapular chondrosarcoma is rare, accounting for about 5 to 7% of all reported cases of chondrosarcoma . Until tIn this study, we present a case of a large chondrosarcoma in the scapular area. Based on the preoperative evaluation of the surrounding soft tissue and the reconstruction design, the patient was treated with Malawer limb salvage surgery. No complications were reported postoperatively and the patient retained limb function.The patient was in compliance with the Declaration of Helsinki. A 59-year-old farmer with a personal history of a lump on his right scapula for the past five years was admitted to hospital. In two years prior to the first hospital visit, the lump had grown significantly and had restricted the activity of the right shoulder. The patient did not have a family history of tumors.Physical examination indicated no apparent spread to the superficial lymph nodes. There was a huge, irregular lump in the region of the right shoulder, with the superior border reaching the acromion, the left border reaching to 1 cm from the spinal column, the inferior border reaching the seventh rib, and the lateral border reaching the midaxillary line. The lump was nodular, hard and caused a pressing pain. There was an obscure boundary between the tumor and the surrounding tissues, the skin around the tumor was red but there was no venous engorgement or vascular pulsation Figure  and compSurgery is the leading treatment for chondrosarcoma. In the past, amputation was always adopted as the treatment for malignant tumors in the scapular area, but this destroyed the balance of the body, negatively impacted the appearance and functions of the body and caused emotional harm to the patients. In recent years, there have been an increasing number of studies in oncology and biological therapy for the treatment of tumors as well as the development of reconstruction science when sufficient tissue is not available. Therefore, 90 to 95% of the malignant tumors can now be safely treated with limb salvage surgery . StatistShoulder girdle resections were classified according to the classification system proposed by Malawer and colleagues in 1991 . It is bFor the case presented in this study, the tumor in the scapular area was adjacent to important nerves and blood vessels, and also involved various surrounding soft tissues. Therefore, the requirement to expose the tumor, excessive bleeding and avoiding nervous dysfunction made the surgery difficult to perform. Moreover, there are many complications that may follow function reconstruction procedures. However, with reasonable reservation and reconstruction of the soft tissue and bone structure, the upper limb function can be preserved. In this case, based on preoperative radiography, the patient’s diagnosis was IIB phase of a G1T2MO tumor according to the AJCC (American Joint Committee on Cancer) staging system, without apparent involvement of nerves and blood vessels, nearby chest wall or proximal humerus. Due to the low invasive ability of chondrosarcoma, we believed that it was possible to perform the surgical resection with wide margins and preserve the soft tissue in order to preserve the proximal humerus that provided a pivot for the elbow, wrist and hand. The ultimate goal was to improve the quality of life if complete resection of the tumor could be performed.et al. reported 134 patients who underwent a limb-sparing resection, and after a minimum of two years follow- up, local tumor control, associated with good functional outcomes, was achieved in the majority of patients [et al. reported that the functional outcome of total scapula prosthesis is superior to forequarter amputation [et al. found that the scapular endoprosthesis was functionally superior to humeral suspension after total scapulectomy [Limb-sparing surgical procedures have replaced the forequarter amputation as the surgical treatment of choice for tumors in and around the shoulder girdle. Bickels patients . There apatients . Inactivpatients . Artificputation . The aimulectomy .The reconstruction of the bone and the joint depends on the reconstruction of the soft tissue regardless of the method that may be adopted. The crucial factor in limb salvage surgery is the careful evaluation of the condition of the soft tissue. If it is assessed that no available soft tissue can be preserved and there is no appropriate way of reconstruction, then limb salvage surgery will lead to a huge economic burden and emotional harm. Our principle is to save as much soft tissue as possible on the condition that the tumor is completely resected. Rotator cuff, deltoid and musculus biceps brachii are the key muscles in sustaining shoulder function; therefore, these are preserved better in order to form a triangle and to efficiently transfer the articular forces. In the present case, the chondrosarcoma was large but the soft tissue and the skin were in good condition. We estimated that there would be enough soft tissue and skin to cover the trauma after the enlarged resection. We took the activity level of the shoulder into consideration, so that the function of the shoulder may be preserved postoperatively. During the operation, our judgment was proved correct and the soft tissue was good enough to perform the limb salvage surgery. After the surgery, the patient retained shoulder function and his basic living requirements could be met even though the appearance was not good.In the present case, the tumor was large and many nearby tissues had been invaded, especially anteriorly. The tumor was very near to the axillary nerves and blood vessels. Therefore, it was very difficult to perform the limb salvage surgery and considerable risks were associated with the surgical procedure. Moreover, wide resection of tumor can lead to loss of viability of the skin and the soft tissue. All these issues need to be considered and planned carefully preoperatively. This case is a unique one and cannot provide a therapeutic guide for chondrosarcoma. However, the operation was a great success and the patient showed good recovery. This case provides an example for the treatment of giant chondrosarcomas.Written informed consent was obtained from the patient for the publication of this report and any accompanying images.AJCC: American Joint Committee on Cancer; CT: computer tomography; MSTS: Musculoskeletal Tumor Society Score.The authors declare that they have no conflicts of interest concerning this article.FC, GYL, QZ, DSD, JCWcarried out the operation; FC drafted the manuscript; HH carried out the histology analysis; GYL carried out the patient follow-up; JCW performed the design of the operation and coordination and helped to draft the manuscript. All authors read and approved the final manuscript.


Facial necrotizing fasciitis is extremely rare. Most of the cases reported in literature are related to dental, sinus, tonsillar and salivary glands causes, but rarely as consequence of skin infection. We report a unique case of lower lip cellulitis, which was initially misdiagnosed as angioedema and subsequently progressed into lower lip necrotizing fasciitis.This is a case report of necrotizing fasciitis involving the lower lip as a consequence of infected skin acne in a 19 year old black female. The patient had been diagnosed earlier as a case of angioedema by a physician and treated accordingly. She was mildly anemic, hyponatremic and hypokalemic. Treatment was started immediately by incision, drainage and full debridement of the whole necrotic tissue. Triple antibiotic therapy was administered and daily irrigation and dressing were performed until full recovery. Complete healing occurred within a month by secondary intention.This case demonstrates the misdiagnosis of a lip swelling leading to the development of labiofacial necrotizing fasciitis, a serious and life threatening condition. Lip angioedema is a common condition; however, lip fasciitis is rare. A broad differential diagnosis in case of lower lip swelling is essential to avoid inappropriate treatment delay. Necrotizing fasciitis (NF) is a rare but potentially fatal infection . Its rapFacial NF is extremely rare. In 1997 Shindo et al. found only 35 reports of facial NF in their review . Lip sweA 19-year-old black female presented with painful lower lip swelling of 2 weeks duration. She was initially seen by a physician whom she claimed had given her anti-allergic medication and painkillers in the form of tablets for 1 week, but the situation worsened and the swelling and pain increased. She was then prescribed intravenous penicillin for another week with no improvement, after which she presented to our hospital for assessment and further management. There was lower lip swelling with discharge from the exfoliated crusted lower lip surface. The condition had started as a small pimple on her chin just below the lower lip on the left side which was painful and increasing in size. She attempted to open it and did not use any antibiotic. After 48 h she became febrile and her lower lip became swollen. She went first to a physician, then presented to our dental hospital. The patient’s medical and family history was non-contributory, and there was no history of trauma, hospitalization or current medication except those prescribed by the physician. She was lactating and had a 6 month old boy.The patient’s oral cavity was examined carefully and good oral hygiene was noted. There were no obvious odontogenic causes or lip paraesthesia. Panoramic radiograph was done to exclude odontogenic causes and was insignificant. A thorough facial examination followed and a small crusted pimple was noticed on the left side of the chin. There was extensive swelling involving the whole lower lip and extending to involve the chin and submental area Fig.  with pusBased on the history and clinical presentation, the diagnosis of necrotizing fasciitis was made and a swab of the site was taken for culture and sensitivity. Treatment started with lower lip debridement and exploration where necrosis of the middle part of the lower lip was noted. A segment of necrotic fascia on the left side of the chin was found and removed with preservation of the overlying skin except over the acne-affected area where skin was removed Fig. . MultiplNecrotizing fasciitis is an infection of the deeper tissues that results in progressive destruction of muscle fasciae and overlying subcutaneous fat. The muscle tissue itself is frequently spared because of its generous blood supply .A definitive diagnosis of NF is established surgically with visualization of fascial planes and subcutaneous tissue. However, clinical clues and diagnostic tools should be used in combination to help make an early diagnosis . The cliTreatment of NF consists of early and aggressive debridement of necrotic tissue, together with broad-spectrum antibiotics and fluids support. It is important to mention that NF can progress in an insidious manner and by the time it has been diagnosed the condition would have progressed to a late stage. In this patient’s case, immediate surgical debridement of the whole necrotic tissue deep to the orbiculariousor is muscle was done until healthy tissue was visible. Extensive fasciotomy with exposure and exploration of all involved compartments was done. Absence of growth in culture and sensitivity results could be explained by the effect of the antibiotics prescribed before she presented to the hospital. Daily dressing was performed and debridement was kept to the minimum essential to minimize the lip defect that might result from generous tissue removal.Patients with NF usually have an associated systemic condition such as diabetes mellitus, arteriosclerosis, obesity, metastatic neoplasm, old age, hypothyroidism, alcoholism, neoplasms, cirrhosis, drug abuse, poor nutritional state, or use of corticosteroids . In our Facial NF is generally due to a dental or pharyngeal abscess , radiotherapy, or an unknown cause . Lower lThis case illustrates a consequence of misdiagnosis of NF. When confronted with lip swelling the clinician must consider the broad spectrum of differential diagnoses and consider a developing NF on the list. Triple antibiotic therapy, investigations, correction of deficiencies, dressing and debridement are essential components of the proper management of NF.


Carcinosarcoma (CS) is a rare mixed mesodermal malignancy most commonly affecting the female reproductive organs, respiratory tract, head, and neck. Though infrequent, it may affect the gastrointestinal tract, most often the oesophagus and only very rarely the rectum. Histologically, it is composed of two distinct elements of epithelial and mesenchymal origin. Clinically, it is a very aggressive tumour with many patients presenting with metastatic lymph nodes or distant metastases at the time of diagnosis. Prognosis is poor despite intervention with the majority of patients dying within six months. Due to the rarity of this condition, there are no specific treatment guidelines presently available. We describe the case of an 80-year-old patient with carcinosarcoma of the rectum with discussion of the immunohistochemistry and review the available literature pertaining to this rare presentation. An 80-year-old female presented with a four-week history of fresh rectal bleeding, associated with poor appetite and weight loss. There were no other symptoms relating to change in bowel habit. Past medical history included ischemic heart disease, atrial fibrillation, hypertension, Parkinson's disease, and hysterectomy several years previously for benign disease. Abdominal examination was unremarkable; however, a low rectal tumour was palpated anteriorly on digital rectal examination. Subsequent colonoscopy showed a large solitary polypoidal growth in the lower rectum. Biopsy of the lesion provided a histological diagnosis of CS based on the presence of two clearly identifiable components on HPE. The tumour consisted of malignant glands set within an atypical stroma consisting of fascicles of spindle cells with thin, oval tapered nuclei and eosinophilic cytoplasm with perinuclear vacuolation. The stromal spindle cells showed florid mitotic activity. Further immunostaining confirmed the nature of the two components with the glandular tissue staining positively for CK20 and CEA. The stromal component did not stain positively for epithelial markers and was negative for cytokeratins. Staging computed tomography (CT) scan of the chest, abdomen, and pelvis and pelvic magnetic resonance imaging (MRI) were performed . A low rFollowing a five-week course of preoperative radiotherapy, the patient underwent radical laparoscopic-assisted abdominoperineal excision of the rectum (APER) with curative intent. Postoperative recovery was uneventful. Histological examination of the resected tumour revealed small amount of residual disease consisting largely of a poorly differentiated adenocarcinoma with a cellular stroma. The malignant tumour showed distinct carcinomatous  and sarcGiven the heterogeneous nature of the tumour, the two components showed distinct immunohistochemistry. The epithelial component stained positively for CK20  and CEA Eight-month follow-up did not show any evidence of recurrence and she was clinically well; however, she subsequently developed locoregional recurrence with pulmonary metastases at 16 months. In view of her frailty and significant cardiac risk factors, she was not considered for chemotherapy and succumbed to the disease 25 months after surgery.The term carcinosarcoma is used to describe a rare mixed mesodermal malignancy consisting of an epithelial component most often a mid to high grade adenocarcinoma and a sarcomatous element of mesenchymal origin which may be differentiated or undifferentiated . CS dispThe presentation and malignant behavior of CS is very similar to that of adenocarcinoma with the carcinomatous component showing a strong tendency to metastasise to lymph nodes and distant sites whereas the sarcoma plays only a very minor role in metastatic spread . PatientThe histogenesis of carcinosarcoma is poorly understood and multiple hypotheses have been proposed. Further characterization of the nature of the tumour itself has been achieved through immunohistochemistry, confirming the presence of distinct components. The most commonly observed pattern of staining is reactivity in the adenocarcinomatous component to the epithelial markers, CK20 and CEA , 9. The Within the large bowel, there are reports of carcinosarcoma from the caecum to the rectum; however, there does not appear to be any significant difference in terms of the potential to metastasize or the overall prognosis . A reviePrognosis of CS within the colon is poor with the longest reported survival being 49 months , 10–12 . Given tEvidence supporting the addition of adjuvant chemoradiotherapy in terms of improving long term outcome is lacking; however, the addition of RT for the patient in the current case did result in downstaging of the disease, allowing for resection with curative intent. Chemotherapy was not a viable option due to her significant cardiac comorbidities. As was done in this patient, strict follow-up is essential given the aggressive nature of this malignancy to detect any local recurrence or metastasis. Although this patient succumbed to the disease after 25 months, she remained disease-free until 16 months post-op which is significantly longer than all other reported cases of rectal carcinosarcoma.Rectal carcinosarcoma is a rare, aggressive mixed type of malignancy with mesenchymal and epithelial components. Immunohistochemistry is the gold standard for diagnosis, identifying two key features: differential staining of the distinct components and the lack of staining for epithelial markers in the sarcomatous component. Aggressive treatment similar to adenocarcinoma protocols with strict follow-up can improve survival rates.


Mucocele, a common benign cystic lesion of minor salivary gland and associated ducts develops following extravasation or retention of mucous material in the subepithelial tissue. Occurrence of mucocele of tongue is considered less frequent when compared to a higher incidence of mucocele in the lower lip of young patients. Different modalities of treatment, such as conventional surgical excision followed by newer techniques like cryosurgery, electrocautery have been proposed to completely remove the lesion and reduce the chances of recurrence. Herewith, we report a novel treatment technique using alginate impression material to aid in complete excision of mucocele of glands of Blandin-Nuhn.How to cite this article: Kumaresan R, Karthikeyan P, Mohammed F, Fairozekhan TA. A Novel Technique for the Management of Blandin-Nuhn Mucocele: A Case Report. Int J Clin Pediatr Dent 2013;6(3):201-204. They commonly appear as translucent bluish nodules that arise mostly on the lower labial mucosa followed by tongue, but other regions of oral cavity may also be affected.24The variation in color depends on size of lesion, its proximity to mucosal surface and elasticity.5Mucoceles of the glands of Blandin-Nuhn are exophytic and may resemble pyogenic granulomata, polyps or squamous papillomata. Clinically, a mucocele shows a positive history of trauma in most cases, rapid onset, alterations in size, bluish color and fluid-filled consistency.8Treatment usually consists of surgical excision of the lesion including the involved minor salivary gland. While other techniques, such as cryosurgery and laser ablation were indicated for complete removal of the lesion.The present report discusses a case of mucocele from glands of Blandin-Nuhn treated by surgical excision, where an unexpected surgical difficulty was overcome.A 9-year-old boy was brought to our Oral Surgery Clinic by his mother with the chief complaint of swelling on the ventral aspect of the tongue of 1 month duration. The swelling had gradually increased in size and interfered with speech and mastication. There was no history of trauma to the area, and the medical history was not significant.On clinical examination, a bluish, nonulcerated, oval shaped mass measuring about 25 × 15 mm in size was noticed on the left ventral aspect of the tongue. The swelling was painless, soft and mucosa over the lesion was tensed . On aspiSurgical removal of the lesion was planned under local anesthesia with 2% lignocaine. A vertical incision was made on the ventrum of the tongue and excision of the lesion was attempted. In course of dissecting the lesion from the underlying tissue, the cystic lining ruptured leading to an immediate extravasation of mucos . This main toto together with the associated minor salivary glands  by definition, is a cavity filled with mucos. They commonly present as either the mucous extravasation type or mucos retention type. The former is the primary cause of mucocele formation involving exudation of mucos into the connective tissue and surrounded by a granulation tissue envelope. The mucos retention type on the other hand appears due to a decrease or absence of glandular secretion produced by blockage of the salivary gland duct.1413Diagnosis is principally clinical. The appearance of mucocele is pathognomonic and the following data are considered helpful in the clinical diagnosis: a history of trauma, rapid onset, variation in size, a fluid-filled consistency, bluish color and recovery of mucos with aspiration.6 laser ablation7, micromarsupialization and steroid injection.8 Baurmash5 has advised on completely unroofing the lesion along its entire periphery to visualize and remove all of the glands present to prevent recurrence of the lesion.Small mucoceles are treated by surgical excision which includes the servicing mucous glands with evacuation of its contents. On the other hand large lesions may be treated by cryosurgery,2 In addition, incomplete excision of the cystic lining may also result in recurrence of the lesion. Hence, clearly demarcating the limit of the lesion is of prime importance in order to make the surgical excision complete and easier. Filling the cystic cavity with ultraflow rubber based impression material or alginate impression material presurgically improves the visual access for surgical excision.14 However, use of alginate is not recommended in areas where extension into surrounding tissue planes is possible leading to a foreign-body reaction in the tongue as a result of residual alginate within the tissue.15During surgical excision on the ventral surface of the tongue, all the minor salivary gland tissues in the surgical field may be electively excised to avoid iatrogenic recurrence of a mucocele by residual salivary gland tissue left behind.In our case, during surgical exploration of the mucocele an unexpected rupturing of the lesion had occurred making the surgical exploration difficult. A thin mix of alginate impression material injected into the lesion clearly delineated the entire lesion and complete excision was made possible. No foreign-body reaction was noted post-operatively.The use of alginate impression material to define the boundaries of the lesion is an effective, safe and economical way of treating an anterior lingual salivary gland mucocele, whilst ensuring complete removal and minimal chance of a recurrence. If performed correctly, this technique will undoubtedly prove to improve the surgical compliance thereby curtailing the recurrence of mucocele in the oral cavity.


Lymphangiomas are rare, benign tumors. An intra-abdominal location of these lesions is rarer still and there are only a few reports describing laparoscopic resection for retroperitoneal lymphangiomas, especially in tumors that mimic pancreatic tumors.We present the case of an asymptomatic 30-year-old Japanese woman in whom a cystic tumor was found incidentally in close approximation to the pancreas. Because the tumor was located in the retroperitoneal space and the body of the pancreas was compressed, we were unable to distinguish a cystic lymphangioma from cystic pancreatic tumors. We started the procedure laparoscopically with five ports. The tumor was in fact separated from the pancreas and was dissected free from the body of the pancreas using scissors and laparoscopic coagulating shears. The left gastric vessels, which were compressed by the tumor, were preserved. As we realized that the tumor was connected to the retroperitoneal lymphatic tissue, we completed the procedure by performing a cystectomy without rupture. The specimen was extracted using a plastic bag. Our patient was discharged on postoperative day 7 without any complications. There is no evidence of recurrence during a >2-year observation period.In addition to the therapeutic significance in differentiating between a cystic lymphangioma in close approximation to the body of the pancreas and a pancreatic cystic neoplasm, the laparoscopic approach is feasible and effective.The online version of this article (doi:10.1186/s13256-015-0760-z) contains supplementary material, which is available to authorized users. Lymphangiomas are rare and benign tumors, which are caused by a malformation or a blockage of lymphatic vessels . While iA 30-year-old Japanese woman presented to our hospital with chronic cough and had a workup in the internal medicine department. She had a past history of hyperlipidemia. A chest computed tomography scan showed a cystic lesion attached to the body of her pancreas, and a diagnosis of cystic pancreatic tumor was made. Blood tests showed neither inflammation nor anemia. Her serum amylase, lipase, aminotransferase, and bilirubin levels were within normal limits. Tumor markers including CEA, CA19-9, and AFP were not elevated. A contrast-enhanced computed tomography scan of her abdomen showed a 5 cm tumor  and scissors. Branch vessels from the left gastric artery and vein were divided and the compressed left gastric vessels were preserved. The dissection was done easily and bluntly. We dissected the tumor from the pancreas gently using a lymph node dissection technique similar to that in gastric cancer, and were able to separate the mass from the pancreas. Eventually, the tumor was connected only to retroperitoneal lymphatic tissue around the celiac axis. At this stage it became clear that this was a case of cystic lymphangioma; as such, we finished the operation with cystectomy only. The specimen was extracted using a plastic bag . An additional movie file shows this image in more detail . The tumor was within the lesser sac, which was incised and a window created. Because the left lateral segment of the liver was large and obscured the surgical view, we added a 5-mm port to elevate the lateral segment. The tumor was located in the retroperitoneal space and was attached to the body of the pancreas. We began the dissection of the tumor from the opposite side of the pancreas using laparoscopic coagulation shears (LCS, Harmonic ACEA histopathological examination showed that the cyst was lined by flattened endothelium. Immunostaining was positive for CD31, D2-40, AE1/AE3, and SMA. Other markers including CD34 and calretinin were negative. The diagnosis was made of a retroperitoneal cystic lymphangioma (Figs. A cystic tumor in the retroperitoneal space has many potential diagnoses, particularly when located adjacent to the pancreas, in which case the decision to operate is more difficult . LymphanLymphangiomas are benign lesions characterized by proliferating lymphatic vessels, and are classified as either hamartomas or lymphangiectasis. Since lymphangiomas are tumors that develop before birth, in most cases they are discovered when patients are young . Adult cet al. espouse the concept that an extended resection should not be performed for benign lymphangiomas [Makni angiomas . In the et al. in 1994 [The first report of laparoscopic resection for retroperitoneal lymphangioma was published by Targaroma  in 1994 . Subsequ in 1994 , 7–15. TA retroperitoneal lymphangioma in close approximation to the pancreas is easily and safely resected laparoscopically. In addition to the therapeutic significance in differentiating between a cystic lymphangioma in close approximation to the body of the pancreas and a pancreatic cystic neoplasm, the laparoscopic approach is feasible and effective.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.


Superior mesenteric artery syndrome (SMAS) after a surgical operation is very rare. We experienced an extremely rare case of ileal pouch-anal anastomosis with subsequent development of SMAS requiring duodenojejunostomy. A 74-year-old Asian woman underwent total colectomy, ileal pouch-anal anastomosis (J-pouch), covering ileostomy, splenectomy, and distal pancreatectomy for treatment of descending colon cancer associated with ulcerative colitis. She complained of abdominal discomfort and vomiting 17 days postoperatively. Computed tomography (CT) revealed fluid collection at the pancreatic stump. We diagnosed a pancreatic fistula and performed CT-guided drainage. SMAS was thereafter diagnosed by contrast-enhanced CT, which revealed a narrow aortomesenteric angle of 36° and short aortomesenteric distance of 2 mm. The SMAS did not respond to conservative therapy. Finally, we performed duodenojejunostomy. This case illustrates that ileal pouch-anal anastomosis might induce relative stretching of the superior mesenteric artery and flatten it against the aorta, resulting in SMAS. Superior mesenteric artery syndrome (SMAS) is a rare disease defined as obstruction of the third portion of the duodenum caused by compression of this region between the superior mesenteric artery (SMA) and aorta. This syndrome was first observed more than 150 years ago. Diagnosis of SMAS is very difficult because of its nonspecific symptoms, including nausea, vomiting, abdominal pain, anorexia, and early satiety. The causes of SMAS are loss of intraabdominal fat, changes in spine extension, and surgical operations [Among patients with SMAS who have a history of surgery , the most common surgical operations are those performed to treat colorectal cancer. In the most recent few decades, ileal pouch-anal anastomosis (IPAA) has been the gold standard reconstruction technique following the performance of total proctocolectomy. IPAA is performed to preserve the sphincter in patients with ulcerative colitis (UC) after total proctocolectomy. The SMA is the main vascular pedicle for the constructed ileal pouch during the performance of IPAA. Stretching of the SMA associated with IPAA might cause SMAS.To the best of our knowledge, SMAS after IPAA has been reported only five times -6. We he2). A computed tomography (CT) scan revealed invasion of the colon cancer to the tail of the pancreas. Clinical biochemistry and hematology tests showed evidence of slight inflammation. Her carcinoembryonic antigen and carbohydrate antigen levels were within normal limits.A 74-year-old Asian woman with chronic UC was diagnosed with descending colon cancer. She was 144.3 cm tall and weighed 50 kg  with distal pancreatectomy to treat the pancreatic cancer invasion. On postoperative day 17, she complained of abdominal discomfort and vomiting. A CT scan revealed fluid collection at the pancreatic stump (Figure Conservative therapy for the PF, including total parenteral nutrition and administration of a proton pump inhibitor, protease inhibitor, and other medications, was not effective. An upper gastrointestinal series revealed an obstruction in the third portion of the duodenum Figure A. GastroConservative treatment for SMAS and specific treatment for the PF were performed, including administration of an elemental diet, gastrointestinal prokinetic agents, and other medications. However, the patient repeatedly vomited, and the PF did not improve. Finally, side-to-side duodenojejunostomy was performed 110 days postoperatively. After duodenojejunostomy, an upper gastrointestinal series showed no duodenal distension. Gastroduodenal endoscopy showed no signs of GERD or food residue in the stomach. The PF improved soon after the duodenojejunostomy Figure . We remoSMAS was first described in 1861 by Rokitansky . In patiOne cause of SMAS is believed to be loss of the intraabdominal fat that separates the SMA from the aorta. Other reported causes of SMAS include traction of the SMA and changes in spine extension . There aSurgical treatments that may be considered for SMAS include laparoscopic, robotic, or open duodenojejunostomy -13. In tIn summary, we experienced a very rare case of SMAS after construction of an IPAA. The SMAS was induced by stretching of and traction on the SMA associated with IPAA. The SMAS caused high pressure to develop in the duodenum and main pancreatic duct, resulting in PF formation. This PF might have reduced the fat pad between the SMA and aorta, resulting in prolonged SMAS. Surgical treatment was finally required Figure . ManagemWritten 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.


Undifferentiated embryonal sarcoma of the liver (UESL) in adults, especially over 30 years old, is quite rare. We report two adult UESL patients that one of them survived 62 months and one is now surviving more than 65 months treated with repeated hepatic resections and radio-frequency ablations. Although UESL is an entirely unusual and aggressive tumor, multidisciplinary treatments including repeated hepatic resections and radio-frequency ablations may provide a longer survival. Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant tumor commonly observed in children . UESL inA 52-year-old female was admitted to the hospital to treat a huge liver tumor with complaints of dyspnea, severe abdominal distension, and lower-extremity edema. Hepatitis B virus surface antigen (HBs-Ag) and hepatitis C virus antibody (HCV-Ab) were negative. The serum level of DUPAN-II was 810 U/ml and that of CA125 was 157 IU/ml, and alpha-fetoprotein (AFP), protein induced by vitamin K absence (PIVKA-II), carcinoembryonic antigen (CEA), and carbohydrate antigen 19–9 (CA19-9) were in the normal range. Computed tomography (CT) showed a huge low-density tumor with a clear margin, 23 cm in diameter, and showed little enhancement after administration of contrast medium Fig. . MagnetiA 53-year-old female was admitted to our hospital with a huge liver tumor with symptoms of epigastralgia and back pain. Her HBs-Ag and HCV-Ab were negative. Serum level of PIVKA-II was elevated to 10,369 mAU/ml, and AFP, CEA, and CA19-9 were in the normal range. CT showed a huge low-density tumor sized 18 × 14 cm that showed mild enhancement after administration of contrast medium Fig. . MR imagUESL in adult is extremely rare and the prognosis of UESL patients is very poor , 4, 7. ATwo cases of adult UESL patients were occasionally admitted to our hospital in the same period. Instead of the huge size of the tumors, both patients were successfully treated with the initial hepatic resections. The first patient survived 62 months with receiving a total six times of hepatic resection, twice RFAs, and two times of TACEs. The second patient received three times of hepatic resection and a TACE and RFA and is still alive more than 65 months from the initial treatment. Both patients received no adjuvant chemotherapy or radiotherapy.UESL has been reported to be chemotherapy-sensitive or is highly sensitive to TACE , 7. HoweNoguchi et al. reported a long-term survival case of adult UESL treated with hepatic resection, adjuvant chemotherapy, and additional radiotherapy . They alIn conclusion, although adult UESL is a rare and aggressive malignancy, repeated hepatic resections and RFAs for the recurrent diseases may provide a longer survival.Written informed consents were obtained from patients and their family for publication of this case report and any accompanying images. Copies of the written consents are available for review by the Editor-in-Chief of this journal.