Germ-cell tumors (GCT) are a histologically and biologically diverse group of neoplasms which primarily occur in the gonads but also develop at different extragonadal sites in the midline of the body. The head and neck region including the upper respiratory tract is a very rare location for such tumors in both children and adults, which can cause diagnostic and therapeutic difficulties.We describe here two new cases of multilineage tumors including sinonasal teratocarcinosarcoma [SNTCS], and congenital oronasopharyngeal teratoma (epignathus) and compare their features with those of a new case of a rare salivary gland anlage tumor [SGAT], an entity for which the pathogenesis is unclear (i.e. hamartoma versus neoplasm). We correlate their presenting clinico-pathological features and compare histologic and cytogenetic features in an attempt to elucidate their pathogenesis and biologic potentials.Cytogenetic analysis revealed chromosomal abnormalities only in the case of SNTCS that showed trisomy 12 and 1p deletion. Both cytogenetic abnormalities are characteristically present in malignant germ cell tumors providing for the first time evidence that this rare tumor type indeed might represent a variant of a germ cell neoplasm. The SGAT and epignathus carried no such cytogenetic abnormalities, in keeping with their limited and benign biologic potential.The comparison of these three cases should serve to emphasize the diversity of multilineage tumors (hamartomas and GCT) of the upper respiratory tract in regards to their biology, age of presentation and clinical outcomes. Malignant tumors of germ cell origins are more likely to affect adults with insidious symptom development, while benign tumors can nevertheless cause dramatic clinical symptoms which, under certain circumstances, can be fatal. Upper respiratory tract tumors are relatively common neoplasms whose frequency, distribution, histological type, and clinical behavior are primarily determined by the patient's age, sex and genetic aberrations .Germ-cell tumors are a heterogeneous group of neoplasms that primarily occur in the gonads (both ovaries and testes) but can also occur at extragonadal sites along the midline of the body. The head and neck region including the upper respiratory tract is a rare location for such tumors in both children and adults and only a few cases have been reported in the available literature . Not surMalignant sinonasal tumors are very rare and represent less than 1% of all cancers and approximately 3% of malignancies of the head and neck region . DespiteIn contrast to malignant germ cell tumors, benign teratomas of the oronasopharyngeal region  are composed of mature, highly organized structures. They are rare congenital tumors constituting less than 2% of all congenital teratomas, and with an incidence estimated at from 1:35.000 to 1:200.000 live births . The mosThe outcome and survival of newborns with epignathus are generally unfavorable. The most important factors that determine the outcome include: size of the tumor, degree of facial distortion, airway obstruction, difficulties in management and uni/bidirectional growth pattern of the tumor . Newly dHar-El et al  is a very rare, probably hamartomatous tumor of the nasopharynx of neonates . Due to El et al  in 1985, in 1994 . Since t in 1994 . It typi in 1994 .Cytogenetic studies are particularly useful in determining the germ cell origin of a neoplasm, particularly malignant ones, because they frequently carry a characteristic chromosomal gain of 12 p. In contrast, benign tumors (mature teratomas) show no chromosomal abnormalities . BecauseHerein, we describe for the first time a case of SNTCS with trisomy 12 with a subclone characterized by an additional deletion of 1 p. We further describe and contrast the case with an epignathus and SGAT that showed no cytogenetic aberrations.Medical records and histopathological reports were retrospectively analyzed. All samples were processed and analyzed using routine pathology techniques, i.e. gross examination, frozen sections (when requested) and formalin-fixation, paraffin-embedding for histologic examination. Hematoxylin and eosin (H&E) stain as well as automated immunohistochemistry (IHC) were used to characterize and diagnose these tumors.The following primary antibodies were used in IHC: cytokeratin AE1/3, HMB-45, epithelial membrane antigen (EMA), S100, chromogranin, CD99 ; cytokeratin 5/6, smooth muscle actin (SMA), alpha-feto protein (AFP), glial fibrillary acidic protein (GFAP), synaptophysin, neuron specific enolase (NSE), myoglobin, myogenin, and p53 ; vimentin, desmin, bcl-2 . Standard indirect biotin-streptavidin detection method with DAB chromagen was used. and labeled by nick translation according to manufacturer's protocol (Vysis/Abbott). Briefly, 200 ng of each rhodamine-5-dUTP labeled BAC clone was precipitated together with 5 times Human Cot-1 DNA  and a spectrum green alpha-satellite probe for the centromeric region of chromosome 12. Prior to hybridization the slides were pretreated using the VP 2000 automated slide processor  following a modified version of the manufacturer's recommended protocol. Co-denaturation of the DNA probes and patient slide was performed on a HYBrite™ instrument  at 80° C for 5 minutes, followed by an overnight hybridization at 37°C, post-wash in 2 × SSC/0.1% NP-40 at 73° C and 2 × SSC at room temperature for 2 minutes each, and counter-stain with DAPI II. Hybridization signals were assessed in 100 interphase nuclei and images were acquired using the Cytovision Image Analysis System .Fluorescence in situ hybridization (FISH) was performed on 4–5 μm unstained paraffin-embedded tissue sections utilizing the LSI 1p36/1q25 dual color DNA probe  and a dual-color DNA probe mixture combining BAC probes specific for 12p13.2 (RP11-418C2 and RP11-434C1) with CEP 12 (Vysis/Abbott) for ploidy determination. BAC clones were selected according to their genomic location using the UCSC Genome Browser The representative tumor samples were disaggregated with scalpels and collagenase and cultured in RPMI-1640 media supplemented with 20% fetal bovine serum and antibiotics for 4–6 days as previously described . MetaphaThe case reports were shared with Creighton's University Institutional Review Board; it is however the policy of this Board not to review case reports.An 85 year old African American female presented to her primary care physician with complaint of spitting up blood. Imaging studies of the chest did not identify a cause. Five months later she presented with complaints of sinus symptoms and CT scan identified a "large right nasal polyp". Her primary care physician treated her for sinusitis, but three weeks later she presented again complaining of shortness of breath and difficulty moving air through her right nostril. She also reported a bloody nose of three weeks duration and the self discovery of a new nasal polyp one week prior to presentation. On physical exam a large polypoid lesion filled the right naris and MRI identified an extensive right nasal passage mass eroding through the cribriform plate of the right ethmoid sinus into the cranial cavity Figure . A tissuReview of the biopsy materials showed an extensively necrotic tissue with scattered viable epithelial and mesenchymal elements. The different elements blended morphologically into one another. Atypical immature epithelial, mesenchymal and primitive neuroectodermal components were recognized at high magnification Figure . ImmunohBased on the clinical presentation, histologic findings and immunohistochemical characteristics, a diagnosis of teratocarcinosarcoma was made.Conventional cytogenetic analysis of 20 metaphase cells identified a hyperdiploid clone characterized by trisomy 12, with an additional subclone characterized by a del(1 p). Karyotype of the tumor was described as: 47, XX, +12/47, idemA 30-year-old G2P1 woman presented at 29 weeks gestation with signs of polyhydramnios. Her first pregnancy had been uneventful. She was referred to the hospital because ultrasound revealed a large mass in the mouth of the live female fetus filling the oral cavity but not protruding out of the mouth. Labor was induced with successful delivery, but postnatal intubation was unsuccessful and the infant died shortly after delivery.Post mortem examination revealed a female fetus with growth parameters in keeping with 28 weeks of gestation. There was no growth retardation. Petechiae were present on the anterior chest and neck region.A tumor was visible through the slightly opened mouth appearing as a tongue-like structure with a blunt border and skin-like surface. On dissection, the tumor was found to arise from the hard palate and completely filled the oral cavity and upper aero/digestive tract with dilatation of the pharynx Figure . No tranThere was no facial dysmorphy, cleft palate or other congenital anomalies. There was no pulmonary hypoplasia.Histologic examination of the tumor showed variable mature tissue types including bone with bone marrow, hyaline cartilage, epidermis and thick dermis with hairy follicles and sebaceous glands Figure . SubcutaThere was extensive intraalveolar, interstitial and subpleural hemorrhage in the immature pulmonary parenchyma of both lungs, along with petechiae, confirming asphyxia as a cause of death.Fluorescent in-situ hybridization (FISH) with LSI DNA probes for 1p36 and 1q25 was normal and did not identify chromosomal gains or losses in either. LSI ETV6 (TEL) probe at 12p13 and a homebrew probe set for ETV6 and CEP12 were unsuccessful.A 12-month old male presented with intermittent airway obstruction and otitis media with bilateral middle ear effusions. The clinical impression was of hypertrophied adenoids, but on closer examination, a mass at the midline posterior nasopharynx was discovered. It was excised and submitted for pathologic examination.A 1.7 × 1.0 × 0.6 cm lobulated, light gray to tan, fragment of soft tissue was received for examination. Histopathologic examination revealed a tumor covered by stratified squamous epithelium that extended into the stroma forming a submucosal network of branching tubular structures. These ducts were lined by cuboidal or low columnar epithelium that frequently was transformed into squamous-type lining. The stroma was predominately densely collagenous with myoepithelial cells; focal areas of loose, myxoid stroma (that contained plasma cells) were also present Figure . No cartFluorescent in-situ hybridization (FISH) with LSI DNA probes for 1p36 or 1q25 was normal and did not identify chromosomal gain or loss in either region. LSI ETV6 (TEL) probe at 12p13.2 and a homebrew probe set for ETV6 and CEP12 were also normal showing 2 copies per cell nucleus Figure .This study reports three new cases of upper respiratory tract tumors, all showing complex, and multilineage histology. The tumors are reported in patients of diverse ages, with significantly varied clinical signs. The presentation of each lesion reflected in part the age of the patient, severity of disease and the biological potential of the tumor. The histologically mature (benign) congenital oronasopharyngeal teratoma (epignathus) caused death from upper airway obstruction in a neonate. The highly malignant SNTCS in an 85-year-female had delayed diagnosis and treatment due to insidious development in the upper respiratory tract and also resulted in the fatal outcome. A hamartomatous SGAT was diagnosed in a 12 month old male and prompt appropriate treatment avoided potentially life-threatening severe respiratory distress and feeding difficulties .The biologic potential of germ cell tumors generally correlates with their gain of chromosomal aberrations. These aberrations are detectable by conventional cytogenetics when fresh tissue is available for cultures. Additionally fluorescent in-situ hybridization (FISH) can be used to detect these abnormalities in formalin fixed paraffin embedded tissues. In our cases we were able to perform cytogenetic analysis on SNCTS and identify trisomy of 12 with a subpopulation of cells showing additional deletion of 1p chromosome. These are the first reported cytogenetic abnormalities found in SNTC.Salem et al [et al [Trisomy 12, as a primary or secondary event, is a well known cytogenetic abnormality occurring in majority of malignant germ cell tumors . The finem et al  recentlyem et al ,28,29. Nl [et al  to propol [et al .Bussey et al [In our cases, neither the SGAT nor epignathus carried such cytogenetic aberrations. This result combined with its histopathological and clinical features of SGAT support the thesis that SGAT might be a hamartomatous, developmental disorder rather than true neoplastic lesion. A study of ey et al  concludeey et al ,31.Histologically, SNTCS is composed of various tissues including epithelial, mesenchymal, and neural elements including teratoid elements. SNTCS lacks features of embryonal carcinoma, choriocarcinoma, and seminoma . This chDehner et al [SGAT is quite rare tumor with only 24 cases (including the case presented in this paper) described since er et al  introducer et al .The majority of patients with SGAT present with symptoms  in the neonatal period or first weeks of the life. Our case is the first described with delayed presentation (12 months) and the oldest previously described patient presented with the tumor at the age of three months . For unkHistopathological features of SGAT include the presence of the stratified squamous epithelium that extends into a loose, myxoid stroma forming a submucosal network of branching tubular structures . Areas with necrosis and cystic degeneration might also be present . Only onSGAT exhibits benign behavior and no recurrences after complete surgical resection were reported in the literature . It may Teratomas have origins in totipotential germ cells and along with neuroblastoma they are the most common congenital tumors. Nevertheless, head and neck  teratomas are exceptionally rare comprising less than 2% of all congenital teratomas . They arOur case of epignathus provides lessons for both diagnosis and care of patients with this tumor. Uncharacteristically, the tumor was not associated protrusion from the oral cavity, intracranial extension or other midline anomalies including cleft palate as the most common anomaly associated with epignathus ,17. NeveThe comparison of these three cases should serve to emphasize the diversity of multilineage tumors (hamartomas and GCT) of the upper respiratory tract in regards to their biology, age of presentation and clinical outcomes. Malignant tumors of germ cell origins are more likely to affect adults with insidious symptom development, while benign tumor can nevertheless cause dramatic clinical symptoms which, under certain circumstances, can be fatal.The authors declare that they have no competing interests.SV carried out autopsy, participated in diagnostics, conceived the study design, wrote and drafted the manuscript. SKC, SZ, IS, WML, and JM participated in diagnostics, wrote and approved the final manuscript. SD and NB carried out autopsy, wrote and approved the final manuscript. ZG participated in diagnostics, conceived the study design, wrote and approved the final manuscript. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:


This case illustrates the unusual complication of granulomatous peritonitis following rupture of a dermoid cyst in pregnancy resembling disseminated ovarian carcinoma. To the best of the authors' knowledge, this is the first report of this complication during advanced pregnancy in the literature.A dermoid cyst ruptured during surgical removal in the second trimester of pregnancy in a 27-year-old primigravida. Postoperatively the patient suffered pulmonary embolism and leakage of sebaceous material through the abdominal wound. She gradually developed significant abdominal distension, gastrointestinal symptoms and lost more than 8 kg of weight in the 12 weeks postoperatively. The baby was delivered at 31 weeks by a technically challenging caesarean section owing to severe dense adhesions obscuring the uterus. Bowel resection was performed for suspected malignant infiltration and adhesion causing obstruction. She had a protracted convalescence with an ileostomy and mucus fistula. Histology confirmed granulation without malignancy. One year following the surgical treatment, she had recovered well and was planning her next pregnancy.Although granulomatous peritonitis following rupture of a dermoid cyst is very rare, awareness is the key to diagnosis and appropriate management. Per-operative frozen section may be helpful. Mature cystic teratoma (dermoid cyst) is one of the most common benign ovarian neoplasms discovered during pregnancy 24–40%) ,2. They 4–40% ,2.9/l and the C-reactive protein range was 76–163.4 ng/dL. Repeated bacteriological cultures were negative.A booking ultrasound scan at 13 weeks gestation in a 27-year-old primigravida showed an 11 × 9 × 10 cm smooth-walled, mixed echogenic mass in the pouch of Douglas (POD) suggesting a dermoid cyst and a viable singleton intra-uterine pregnancy. A repeat scan four weeks later showed an increase in the size of the cyst of 1 cm in each dimension. CA-125 assay was within the normal limits in pregnancy. Alpha-foetoprotein (AFP) was not tested owing to the difficulty in interpretation during pregnancy. Following extensive discussion of different treatment options and timings, the patient opted for surgical removal. The left ovary together with the cyst was removed at 18 weeks of pregnancy. The cyst was impacted in the pelvis owing to the gravid uterus and unfortunately it ruptured during removal. The peritoneal cavity was washed meticulously with 0.9% saline solution. In view of the almost definite clinical diagnosis of an ovarian dermoid cyst, the peritoneal fluid was not sent for cytology. The right ovary was examined per-operatively and looked clinically normal. Histology of the cyst confirmed the diagnosis of a mature cystic teratoma (figure A magnetic resonance imaging (MRI) scan showed a collection of fluid occupying the POD and subsequently a subcutaneous collection was found during an ultrasound scan. She was treated with antibiotics empirically. Seven weeks following her initial surgery, she developed painful induration of the wound. She underwent wound exploration that revealed copious, thick, sebaceous-type material tracking extra-peritoneally up to the costal margin and umbilicus. Bacterial culture was again reported to be negative.She developed dependent oedema, abdominal distension, intermittent vomiting and significant weight loss despite advancing pregnancy. In view of the patient's continuing deterioration and significant psychological distress, it was decided to deliver the baby.She underwent an elective caesarean section at 31 weeks gestation following a course of antenatal steroids for foetal pulmonary maturation. A caesarean section was performed under epidural anaesthesia in the presence of a general surgeon. The anterior abdominal wall was morbidly adherent to the uterus making identification of the uterus virtually impossible. Eventually the 'lower segment' was exposed with the fundus remaining obscured by the dense adhesions. The caesarean section was performed with a J-shaped incision. The baby was delivered in good condition. The patient underwent general anaesthesia following delivery. There were diffuse adhesions with the bowel adhered to the uterine fundus and lower part of the pelvis. There were large lumps of possible granulation tissue on the bowel clinically mimicking malignancy. The bowel was compromised with adhesions producing kinking and bowel stasis. About 85 cm of the ileum and 45 cm of the caecum and colon involved with the mass suggesting infiltrating malignancy were resected by colorectal surgeons. The abdominal wall, rectus muscles, sheath and bowel were infiltrated by sebum-like material. Following extensive exploration and drainage, the patient was left with a mucus fistula on the left side of the abdomen and an ileostomy on the right side. Histology of the specimen confirmed granulation and inflammation consistent with granulomatous peritonitis without any evidence of malignancy figure . She sufOver the following six weeks , her condition improved both physically and mentally. The inflammatory markers returned to normal. She is presently thinking about going back to work and the possibility of conceiving again.teraton, meaning monster, and was used initially by Virchow in the first edition of his book on tumours, which was published in 1863 (see [The word teratoma is derived from the Greek word 863 (see ). Since 863 (see . Benign 863 (see . This fa863 (see . In preg863 (see . Treatme863 (see ,8. When 863 (see . Over 20863 (see  reportedClement et al  and AchtRuptured BCT of the ovary mimicking gynaecological malignancy is uncommon and could be misdiagnosed . Intra-aAlthough granulomatous peritonitis following rupture of a dermoid cyst is very rare, awareness is the key to diagnosis and appropriate management. Peroperative frozen section may be helpful. Optimal management avoids unnecessary repeated major surgeries and provides a good long-term outcome with minimal complications.The authors declare that they have no competing interests.Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journal.SM and ZF collected the data, and designed the case report with significant contributions from ZKA and REH, SM formatted and finalised the draft. All authors read and approved the final manuscript.


Primary splenic angiosarcoma is a rare neoplasm of vascular origin carrying a very poor prognosis, partly due to its high metastatic potential. This disease presents frequently with splenic rupture and hemorrhage. We report the case of a 17-year-old woman who presented with rupture of a primary splenic angiosarcoma.9/liter). After initial fluid resuscitation and stabilization, abdominal ultrasound and computed tomography were performed, revealing a large quantity of intraperitoneal free fluid, an enlarged spleen, and a heterogeneous low-density signal within the splenic parenchyma, which showed varying degrees of contrast enhancement. At laparotomy a huge  actively bleeding spleen was identified and splenectomy was performed. Histopathology showed a primary splenic angiosarcoma. After an uneventful recovery, the patient was discharged on the sixth postoperative day.The patient presented with diffuse abdominal pain and distention. Clinical examination revealed severe tenderness in the left upper abdominal quadrant, a palpable abdominal mass, and hemodynamic instability with a systolic arterial blood pressure of 75 mmHg and heart rate of 135 beats per minute. Blood tests revealed anemia (hemoglobin 7.0 g/dl) and thrombocytopenia (platelets 70 × 10Primary splenic angiosarcoma is rare. Although this malignancy is usually encountered in advanced age, there have been a few reported cases among younger patients. The case reported here presented with splenic rupture, was treated by laparotomy and splenectomy, and the patient is disease free 16 months after surgery. Primary angiosarcoma or hemangiosarcoma of the spleen is an extremely rare malignancy, the pathogenesis of which is not completely understood, with high metastatic potential and an exceedingly poor prognosis, regardless of the treatment regimen. This aggressive disease entity usually presents in adults in their sixth to seventh decade with only eight cases reported in the literature in people aged below 18 years of age. The reported median survival rates range from 4.4 months to 14 months, depending respectively on whether the diagnosis is made after splenic rupture or is based on clinical findings .These mesenchymal tumors can easily be overlooked and splenic rupture is the most frequently encountered presenting manifestation. We report a case of splenic rupture due to angiosarcoma in a 17-year-old woman. This is the ninth reported case of this condition in a patient aged under 18 years of age and, to the best of the authors' knowledge, is the second case presenting with splenic rupture in this age group.9/liter). An abdominal ultrasound showed a large quantity of intraperitoneal free fluid and an enlarged spleen. These findings were confirmed by an abdominal computed tomography (CT) scan  and thrombocytopenia (platelets 70 × 10Laparotomy revealed a huge, actively bleeding spleen; more than one liter of blood had filled the intraperitoneal cavity. No abnormal adhesions were detected between the spleen, the diaphragm, or other organs, and macroscopic examination of all four abdominal quadrants did not reveal any other pathology. Splenectomy was performed along with peritoneal lavage with warm saline to evacuate the clots. Intraoperatively, the patient received two units of packed red blood cells, one unit of platelets and two units of fresh frozen plasma.The splenic weight was 1530 g, its greatest diameter was 19 cm, and macroscopically it appeared nodular and spongy with hemorrhagic characteristics Figure  and 2B, After an uneventful recovery, the patient was discharged on the sixth postoperative day. The patient remains symptom and disease free after a follow-up period of 16 months, in contrast to the median survival of 4.4 months of patients with primary angiosarcoma of the spleen and associated splenic rupture .Angiosarcoma originating from the spleen is a very rare mesenchymal malignant tumor of vascular origin, consisting of atypical and anaplastic endothelial cell concentrations. Other primary foci include the skin, soft tissues, breast and bone marrow. Its main histological feature is vascular channel formation with a sarcomatous stroma and papillary appearance due to endothelial cell proliferation, although an undifferentiated neoplasm is also a typical finding. Angiosarcoma is the most common of the primary malignant splenic tumors, which affect 0.14 to 0.25 per million people. This neoplasm shows a slight male predominance, with no predilection for race, geographic location or inheritability . OverallAlthough the natural history of this tumor is not clear, ionizing radiation, arsenic, vinyl chloride and chemotherapy for lymphoma have been implicated as causative factors, and it has been reported that the pathogenesis of splenic angiosarcoma requires the existence of a benign pathology such as a hemangioma or hemangioendothelioma. Recently, a case of angiosarcoma of the splenic capsule was reported and correlated with inert foreign body tumorigenesis . There wThe clinical manifestations of primary splenic angiosarcoma range from asymptomatic disease to splenic rupture and lethal hemorrhage. Non-specific symptoms such as left upper abdominal discomfort, dizziness and weight loss, as well as signs such as thrombocytopenia and an enlarged spleen, which is present in more than of 50% of cases, are not diagnostic and may be easily associated with other pathologic conditions. Anemia, present in 70% of cases, may be of normocytic, schistocytic or megaloblastic type. Splenic rupture occurs in 13% to 32% of patients, and is the presenting and most serious manifestation in the majority of cases. It is the worst prognostic factor for survival, because it puts patients at increased risk of peritoneal dissemination with direct implantation of neoplastic tissue or vascular access and hematogenous spread. This finding is verified by the fact that splenectomy before rupture of the organ is accompanied by better survival rates .Splenic rupture represents a serious complication, frequently encountered in patients with angiosarcoma of the spleen, and results in a fatal outcome in a significant proportion of cases. The presence of consumption coagulopathy with profound fibrinogenopenia and thrombocytopenia may lead to the development of disseminated intravascular coagulation, characterized by a lack of systemic fibrinolysis activation and profuse intraperitoneal hemorrhage, further decreasing survival rates .Imaging modalities are invaluable for the differential diagnosis between other benign and malignant splenic tumors, although diagnostic accuracy is lacking. CT scans may reveal an enlarged spleen with hypo- or hyperattenuating areas on nonenhanced scans. Areas of hyperattenuation are likely to reflect acute hemorrhage or hemosiderin deposits. Contrast enhancement may be similar to that of a hepatic cavernous hemangioma, although the pattern of enhancement is variable. On contrast-enhanced scans, there may be peripheral or heterogeneous contrast enhancement. On MRI, ill-defined nodular lesions with low- or high-signal intensity may be seen on both T1- and T2-weighted images depending on the age of blood products and the presence of necrosis. High-signal intensity is related to subacute hemorrhage or tumor necrosis and low-signal intensity is related to chronic hemorrhage or fibrosis in the tumor. Massive splenic calcifications on CT scan or MRI have been reported as a prominent suggestive finding of angiosarcoma ; howeverSplenic angiosarcoma, although rare, must be considered in the differential diagnosis of patients with hematologic abnormalities of unexplained origin and parenchymal lesions on spleen imaging. Definitive diagnosis requires laparotomy and splenectomy, since the risk of splenic rupture is enhanced with percutaneous splenic biopsies. Since metastatic disease is encountered in the majority of cases in patients with splenic angiosarcoma, the surgical approach with splenectomy is a more diagnostic than therapeutic modality. Although no effective chemotherapeutic protocol for angiosarcoma has yet been established, combined treatment with cyclophosphamide, adriamycin, vincristine and prednisone or other agents after splenectomy has been employed in a few cases, with fewer relatively good results . As therThe authors declare that they have no competing interests.AM carried out the operation and contributed to acquisition of consent and critical review of the manuscript. PG contributed to manuscript conception, research, acquisition of data, drafting and writing of the manuscript. LT contributed to manuscript conception, research, acquisition of data, drafting and writing of the manuscript. HM contributed to manuscript conception, research, acquisition of data, drafting and writing of the manuscript. EEL contributed to manuscript conception, research, acquisition of data, drafting and writing of the manuscript. AP contributed to organizing, drafting and critical review of the manuscript. DP assisted in the operation and contributed to writing of the manuscript. KF contributed to organizing and drafting of the manuscript, and critically revised the manuscript. PK assisted in the operation and contributed to critical review of the manuscript. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Primary pleural angiosarcoma is an extremely rare tumor. We report the case of a patient who presented with recurrent massive bilateral hemothoraxes. Although thoracoscopy was performed, biopsy samples of the pleura were inconclusive. The delayed onset of skin metastases led to the diagnosis of angiosarcoma, however the patient died from pleuropulmonary progression before treatment could be started. We review the literature of primary pleuropulmonary angiosarcoma and discuss its treatment modalities.


Neurofibromatosis type-1 (NF1), also known as von Recklinghausen disease, is a common autosomal dominant condition with an approximate incidence of one per 3000 births. NF1 involves multiple systems of the body. Abdominal involvement occurs in the form of neurofibroma and tumour growth in the liver, mesentery, retroperitoneum, gastric and bowel. Gastrointestinal neoplasms have a reported occurrence of 2–25%. Two cases are reported herein as well as a review of the literature of the intra-abdominal manifestations of NF1, including a discussion on the radiological appearance and diagnosis. The article provides an insight into the intriguing variety of clinical problems that such patients may present. Neurofibromatosis (NF) is an autosomal dominant disorder that can involve any organ system. NF is not a single entity but a group of heterogeneous multisystemic neurocutaneous disorders. There are two distinct types: neurofibromatosis type-1 (NF1) or von Recklinghausen disease, which affects 85% of patients, and neurofibromatosis type-2 (NF2) or bilateral acoustic neuromas/schwannomas, which affects 10% of patients. Abdominal involvement in patients with NF1 has been described in the form of neurofibromas within the liver, mesentery, retroperitoneum and gastrointestinal (GI) tract. Large-bowel intussusception has also been reported. Small-bowel leiomyomas, adenocarcinomas with neuroendocrine function, GI tract vasculopathy, GI tract bleeding, pseudo-obstruction and protein-losing enteropathy also may occur.Two patients of NF1 with different sets of complaints were referred to our department for computed tomography (CT) scan of the abdomen.A 35-year-old male patient with a 5–6-month history of vague, intermittent abdominal pain presented to us for CT examination of the abdomen. An ultrasound of the abdomen revealed lymphadenopathy at the porta hepatis, peripancreatic and retroperitoneal regions.Contrast-enhanced CT examination was performed through the abdomen, from the domes of the diaphragm to the pubic symphysis. The examination revealed multiple, well-defined, oval to round, soft tissue attenuating masses in the liver, porta hepatis, peripancreatic region and retroperitoneum. In addition, a similar lesion was noted in the right lateral abdominal wall . The lesThis patient was managed conservatively on medical therapy and followed up for 3–4 months, during which the patient did well. Patient was suggested to follow up at six-monthly intervals.A 43-year-old male patient came for CT examination of the abdomen with complaints of recurrent vomiting and occasional hemetemesis, and abdominal distension especially after meals. An ultrasound of the abdomen was unremarkable.Contrast-enhanced CT examination was performed through the abdomen, from the domes of the diaphragm to the pubic symphysis. The examination revealed a large, well-defined, polypoidal soft tissue mass with mild to moderate post-contrast enhancement along the greater curvature of the stomach. In addition, diffuse thickening of gastric wall in the region of the pyloric antrum was observed with over-distension of the stomach suggesting gastric outlet obstruction . Post-coThe patient underwent partial gastrectomy along with Billroth type-II gastrojejunostomy. The post-operative as well as the 6-month follow-up period was uneventful.NF1 is a multisystemic disorder that may affect any organ in the body. Clinical presentation depends on the organ system involved. In NF1, gastrointestinal tumors occur most frequently followed by thoracic tumors, arterial involvement and endocrine tumors in the decreasing order of their frequency.1 AbdominoIn one study, majority of the neoplasms were located in the small intestine (72%). Neurofibromas, found in 52% of patients, were the most frequently diagnosed benign neoplasms followed by leiomyomas (13%), ganglioneurofibromas (9.8%) and gastrointestinal stromal tumor (6.5%). Adenocarcinoma was present in 23% of patients. Neurofibromas may be diffuse and submucosal, making radiographic visualization sometimes very difficult. Auerbach plexus is the usual site of origin. Benign neurofibromas undergo malignant transformation in 5–15%, especially in patients above the age of 40 years.[Imaging plays an important role in the diagnosis, evaluation and follow-up of patients with abdominal manifestations of NF1. Barium studies may demonstrate intraluminal mass lesions. Sometimes, intussusception of the bowel may be detected along with intestinal obstruction. Features of malabsorption may also be seen.Solitary neurofibroma is mostly hyperechoic with coarse internal echoes and is lobulated but with smooth and well-defined margins. Biliary obstruction may be demonstrated, and tumour metastasis into the liver along the portal vein may be seen as infiltrative hypoechoic masses around the porta hepatis and intrahepatic portal branches. Duplex color and spectral Doppler ultrasonography reveal the vascular complications of NF1 such as aneurysms and stenoses.Biliary strictures and bile duct intraluminal neurofibromas are rarely associated with NF, but if present, may be visualized by antegrade or retrograde cholangiographic imaging.CT examination may demonstrate solid fusiform masses along the distribution of nerves, with central areas of low attenuation and occasional calcification. The masses usually are well defined and have homogeneous low attenuation, equal to or slightly more than water, but lower than muscles. AsymmetrMagnetic resonance imaging (MRI) is considered to be the modality of choice. In one series, MR imaging added information in the initial and follow-up clinical evaluation of these patients.Surgical excision is the treatment of choice for all symptomatic tumours occurring in patients with NF1. As the benign tumours occurring in these patients always carry some risk of malignant degeneration, surgical excision of such tumours is advocated. But as tIn conclusion, patients with NF1 with GI symptoms are at risk for GI neoplasms from which they are likely to experience significant morbidity. Due to its rarity, a high degree of suspicion is needed for timely diagnosis. Delay in the diagnosis of GI NF is common. The average interval from onset of GI symptoms to the diagnosis and detection of GI neoplasms was 2.8 years in one series. Diagnosi


Primary extracranial meningiomas occur very rarely. We present a rare case of extracranial meningioma of the transitional variant which was excised satisfactorily. There was no suggestion of any connection to the intracranial compartment or cranial nerves. The underlying galea was uninvolved, suggesting the true extracranial nature of this tumour. This rare diagnosis should nonetheless be kept in the differential diagnosis of scalp tumors. Common sites of reported occurrence are skin, orbit, paranasal sinuses, temporal fossa and oral cavity. Other sites include nasal cavity, parotid gland, bifurcation of carotid artery, little finger of right hand, brachial plexus, foot, scalp, face and paravertebral region.1Various classification schemes have been proposed to classify these tumors379An 18-year-old patient was referred to us with history of a slowly growing small nodule on the right posterior frontal area of scalp since infancy (present size 5 cm × 7 cm × 4 cm). Massive bleeding had aborted a previous attempt at excision one month back. This was followed by ulceration and foul discharge.On examination, a large fleshy ulcerated mass was present over the right posterior frontal region, which was firm, nontender and freely mobile over the periosteum. The overlying skin could not be pinched up separately. The surrounding skin was normal, with no associated lymphadenopathy.General physical examination was normal. Contrast-enhanced CT scan of the head showed an extracranial soft tissue mass with calcification over the right posterior frontal region without any evidence of involvement of the calvarium or intracranial structures.The mass was excised with 2.5-cm margins, and the defect was closed with split thickness skin graft. The mass had well-defined margins and the tumour did not involve the periosteum.transitional meningioma.On histopathological examination, the deeper dermis and subcutaneous fat were infiltrated by a tumor composed of syncytial as well as whorls of spindle cells with poorly defined cell borders around blood vessels. The nuclei were elongated to oval with finely distributed chromatin and inconspicuous nucleoli. Some nuclei appeared vesicular due to cytoplasmic inclusions. Abundant psammoma body formations were also seen with areas of collagenization. The findings were suggestive of Most of the reported primary extradural meningiomas have been of meningothelial or psammomatous origin,189The lesion may be mistaken clinically for cutaneous lesions including cysts, skin tag, nevi, vascular lesions, and fibroma. It might be associated with circumscribed alopecia, congenital melanocytic nevus, adenomatous hyperplasia of the eccrine glands and with congenital localized hypertrichosis (hair tufts). Association with von Recklinghausen's disease and malformations of fingers and toes and ovarian fibroma have also been reported.13Most of the ectopic meningiomas had occurred within the orbit, probably originating from the arachnoid cells in the sheath of the optic nerve.


Peritonitis due to peptic ulcer perforation is a surgical emergency with a high risk of mortality and morbidity.We present a rare case of a 54-year-old Caucasian man who underwent an emergency laparotomy for peritonitis caused by perforation of two peptic ulcers. The first was located on the anterior wall of the duodenum and the second was posterior, pre-pyloric, close to the lesser curvature.To the best of our knowledge, this is only the second report in the medical literature of a simultaneous perforation of two peptic ulcers; though rare, every surgeon performing open or laparoscopic repair of a perforated peptic ulcer should be aware of the possibility of simultaneous perforation. Helicobacter pylori infection and the consumption of non-steroidal anti-inflammatory drugs (NSAIDs). Ulcer incidence increases with age for both duodenal ulcers (DUs) and gastric ulcers (GUs) and DUs [H. pylori infection, advanced age, comorbidities and adjunct therapy with drugs such as corticosteroids, anticoagulants and bisphoshonates. Complications  can occur in patients with peptic ulcers of any etiology. Perforation occurs in about 5% to 10% of patients with active ulcer disease. Duodenal, antral and gastric body ulcers account for 60%, 20% and 20% of perforations, respectively, of peptic ulcers [Peptic ulcer disease  remains one of the most prevalent and costly gastrointestinal diseases . The ann and DUs  emerge tc ulcers ,5. Surgic ulcers . Since tA 54-year-old Caucasian Greek man presented to the Accident and Emergency department of our hospital with a 20-day history of abdominal pain, vomiting and loss of appetite. He mentioned an eight kg weight loss over the last 20 days, as he had been drinking almost exclusively water due to his symptoms. He had not presented to any hospital facility earlier because he lived in a remote area in the mountains. On admission, he had the septic image of paleness, tachypnea, tachycardia (110 beats/minute) and a fever of 38.5°C, as well as a rigid abdomen. Abdominal and plain chest X-rays demonstrated free gas under both the hemidiaphragms. After initial resuscitation (placement of intravenous lines and nasogastric tube followed by adequate administration of fluids), our patient underwent an emergency exploratory laparotomy. Our patient's worsening clinical image and his deteriorating clinical signs (tachypnea and tachycardia), along with the presence of his acute abdomen led us to conclude that an emergency laparotomy constituted the treatment of choice. In the face of the emergency situation a computed tomography (CT) scan was not performed. Laparotomy revealed peritonitis due to a perforated ulcer on the anterior wall of the duodenum, which was sutured, while the suture line was reinforced with an omental patch (Figure Perforation of a peptic ulcer is a surgical emergency that still carries a risk of mortality. We successfully managed a rare and difficult case of simultaneous perforation of duodenal and gastric ulcers that could have been easily misdiagnosed and undertreated. Retrospectively studying our case, we can state that there is a growing experience with laparoscopic techniques for management of peptic ulcers. A Graham patch, with or without a laparoscopic vagotomy for perforated peptic ulcers is probably the most appropriate minimally invasive approach when in experienced hands ,9. NeverFinally, every surgeon should strictly follow one of the basic principles of abdominal surgery and perform a thorough examination of the peritoneal cavity in every case of diffuse peritonitis, even if the underlying pathology appears to be obvious.In summary, emergency physicians and surgeons should maintain a high level of clinical suspicion as a second perforative peptic lesion, though a rare possibility, could exist and could potentially be lethal.The authors declare that they have no competing interests.KD was the primary surgeon for the case, conducted a thorough literature research, and was the chief author in terms of writing the paper. TG performed a consultation regarding the anti-reflux treatment, and co-authored the paper. KC assisted with the linguistics and performed literature research. BK assisted with the literature research. BA performed literature research and checked the final version of the manuscript. GA was the attending surgeon for the case and checked the paper. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Ectopic ovarian tissue is an unusual entity, especially if it is an isolated finding thought to be of embryological origin.An 11-year-old, morbidly obese female presented with left flank pain, nausea, and irregular menses. Various diagnostic procedures suggested a large ovarian cyst, and surgical resection was performed.Histologically, the resected mass was not of tubal origin as suspected, but a serous cystadenoma arising from ovarian tissue. The patient's two normal, eutopic ovaries were completely uninvolved and unaffected. A tumor arising from ectopic ovarian tissue of embryological origin seems the most likely explanation. We suggest refining the descriptive nomenclature so as to more precisely characterize the various presentations of ovarian ectopia. Ectopic ovarian tissue is a rare phenomenon, with an incidence estimated between 1 in 29,000 and 1 in 700,000 gynecologic admissions. A more accurate estimate is difficult due to a confusing and still disputed classification system, as well as the frequently asymptomatic nature of the condition. We report a case of what is best described as a giant serous cystadenoma arising from an accessory ovary in a morbidly obese 11-year-old girl.An 11-year-old girl presented with two bouts of abdominal and left flank pain during a 5-month period, described as non-radiating and 8 out of 10 in intensity. The pain was accompanied by nausea and one episode of vomiting. The patient also noticed a decrease in urinary frequency during the same interval. She denied fever, dysuria, hematuria, or bloody stools. Past medical and family history was unremarkable. The patient had no history of hospitalizations, surgeries, or chronic illness. Menarche was at the age of 10 followed by irregular cycles, occurring every 40 to 50 days with very heavy flow.right adnexa, suggesting an ovarian tumor [Figures Physical examination revealed a morbidly obese  adolescent girl. Her abdomen was soft and depressible and no masses were identified on palpation. Various imaging studies were performed including a pelvic ultrasound, which identified an 18.7 cm × 10.0 cm × 15.4 cm cystic lesion that extended into the abdomen to about the level of the umbilicus. Two MRI studies were ordered which identified a large cystic structure that appeared to originate from the left fallopian tube, with which it shared its blood supply. On gross inspection, it was a smooth, multilobulated, fluid-filled mass with no attachments to the left ovary itself. On close examination, the patient's two ovaries showed no signs of torsion or necrosis: both were smooth and atraumatic. Due to the size and location of the cyst, a left salpingectomy was performed in order to remove it completely. The patient was left with two intact ovaries and her right fallopian tube. The presence of the two normally situated ovaries was documented on follow-up sonogram.At laparotomy the cyst was found attached to the fimbriated end of the Due to the identification of two eutopic ovaries and the attachment to the mass to the left fallopian tube, a post-operative presumptive diagnosis of a left paratubal cyst was made. On histological examination the specimen was shown to be lined by columnar epithelial cells with abundant cilia, and contain primary follicles, corpora albicans, Graafian follicles, and areas of fibrin deposition Figure . The finaccessory ovary as having close proximity and some form of association to a eutopic ovary and its associated blood supply. The term supernumerary ovary was reserved for ovarian tissue far removed from the eutopic ovaries and with a separate blood supply. The former is often found attached to the fallopian tube or one of the various ligamentous structures of the ovarian-uterine complex; the latter can be found anywhere along the embryological migratory path of the ovarian primordium, including the mesentery, retroperitoneal space, and omentum [We are aware of at least 50 published reports of additional ovarian tissue since Wharton published his seminal description in 1959 [ omentum .supernumerary and accessory are somewhat misleading because their definitions by Wharton presuppose two normal ovaries and an embryologic origin for the additional ovarian tissue. It has been suggested that up to 50% of cases of additional ovaries are actually post-inflammatory or post-surgical implants [ectopic, subcategorized as either post-surgical, post-inflammatory, or truly embryological [in addition to two eutopic ovaries and 2) that which exists in place of a eutopic ovary because it is the result of defective migration or development of an ovarian primordium [The terminology employed has caused substantial confusion on the subject. The terms implants ,4. Lachmological . Unfortusupernumerary by the Wharton criteria. The detection of both supernumerary and accessory ovaries is often associated with tumors or cysts, perhaps precisely because these are symptomatic and require subsequent workup. Some authors support the idea that this association is due to increased pathological potential of the ectopic tissue [About 36% of reported cases of ectopic ovary are associated with urogenital anomalies . The majThe most common masses identified are mature teratomas and mucinous cystadenomas, present in up to one fifth of patients . In addiDespite the strong association with pathological processes, supernumerary and accessory ovarian tissue has been notoriously difficult to diagnose preoperatively. It is usually an incidental finding or a surprise histopathological diagnosis after resection of a clinically relevant mass, as occurred in this case. It can be suspected on the basis of hormonal abnormalities, such as continued cyclic endometriosis pain  or intacThe patient's young age and impressive weight are unusual features of this case. To our knowledge, there have only been five previously reported cases of additional ovaries diagnosed in children under the age of eighteen. This includes the two neonatal diagnoses reported by Kuga et al . If the ectopic continue to refer to any inappropriately placed ovarian tissue, regardless of etiology or the presence of two eutopic ovaries. The description can be fine-tuned according to the salient features of the specific presentation and its suspected etiology, e.g. "extra/additional" if accompanied by normal ovaries, or "malformed" if the product of faulty migration or malformation of a would-be eutopic gonad. One can invoke the term "implant" when that etiology is suspected, and Lachman's proposed adjectives "post-surgical" and "post-inflammatory" applied. All permutations of etiology and location can thus be accurately and completely described , not previously possible. The terms supernumerary and accessory should retain their traditional Whartonian definitions in that they refer to distinct presentations of additional (extra) ovarian tissue.To improve the precision of the terminology, we would propose that the term Our case represents an accessory ovary according to the Wharton criteria, given its adnexal location and a blood supply continuous with that of the fallopian tube. We believe that the tissue is truly embryologically ectopic, to reference Lachman's nomenclature, because of the absence of previous pelvic or abdominal surgery or disease; also significant is the smooth, atraumatic appearance of the eutopic ovaries at laparotomy. To our knowledge, this is the second report of a serous cystadenoma arising from an accessory or supernumerary ovary, and it is among the largest masses reported arising from either.The author(s) declare that they have no competing interests.All authors have read and approved the final manuscript for publication.1) SS performed manuscript writing, literature review, and collection and analysis of pertinent clinical information.2) TT participated in the clinical management of the patient, the surgery in which the sample was removed, collection and analysis of pertinent clinical information, and literature review.3) LR was the attending physician on the case, and therefore performed the surgery and managed the clinical care of the patient; he also gave the authorization for final publication.4) JW participated in literature review, and collection and analysis of pertinent clinical information.Written consent was obtained from the patient's legal guardian (mother) for the publication of this case report and any accompanying images. A copy of this written consent is available for review by the Editor-in-Chief of this journal.


Tumor thrombus in the portal vein is a common complication of hepatocellular carcinoma, but an extremely rare complication of common bile duct cancer.A 78-year-old woman was referred to our department because of jaundice. Laboratory data showed severe liver dysfunction with high serum levels of total bilirubin and CA19-9. Computed tomography showed lesions in the middle bile duct and main portal vein. FDG PET scan and 3D imaging showed hot spots in the same location as those revealed by CT. Under a diagnosis of middle bile duct cancer with portal vein tumor thrombus, the patient underwent surgery. At laparotomy, the main tumor was found to be located in the middle bile duct with a tumor thrombus, 2 cm in diameter, in the main portal vein. The patient underwent pancreatoduodenectomy with thrombectomy. Histological examination showed that this thrombus had the same histological features as those of the main bile duct cancer (poorly differentiated adenocarcinoma). The postoperative course was uneventful and the patient is doing well without any signs of recurrence 18 months after surgery.To our knowledge, this is the first report of successful resection of middle bile duct cancer with portal vein tumor thrombus. Bile duct cancer (BDC), classified as cancer of the middle or distal bile duct, was the 6th leading cause of cancer-related death in Japan in 2004 . SurgicaPortal vein tumor thrombus (PVTT) is sometime detected in patients with advanced hepatocellular carcinoma (HCC) or hilar cholangiocarcinoma (CCA) by preoperative imaging ,3. AlthoA 78-year-old woman was admitted to our department with jaundice in July 2005. Laboratory data showed severe liver dysfunction with high serum levels of total bilirubin  and CA19-9 . Computed tomography (CT) showed a mass in the middle bile duct and main PV Figure  and 1b. th week) was instituted 3 months after surgery. Currently, she is doing well without any signs of recurrence 18 months after surgery.The patient's postoperative course was uneventful. Adjuvant chemotherapy using Gemcitabine (1200 mg/day for 3 consecutive weeks with a rest in the 4PVTT, which is occasionally observed in HCC, hilar CCA, pancreatic endocrine tumor and liver metastases from cancers of the gastrointestinal tract cancer including colorectal cancer, is caused by direct cancerous invasion into the PV and is associated with poor prognosis ,3,5-8.HCC shows a high incidence of PV invasion, and is reportedly found in 64.7% of patients at autopsy . The incBetween 1988 and 1997, 651 cases of BDC were resected in Japan with a 5-year survival rate of 23% for middle BDC and 32% for lower BDC ,11. In tRecently, FDG-PET scan has been widely adopted for diagnosis of pancreato-biliary malignancy and metastatic liver tumors, and is reported to be useful for detection of extrahepatic metastasis -17. FDG-As mentioned above, the prognosis of malignancies associated with PVTT is usually poor. Our patient received standard adjuvant chemotherapy using gemcitabine,  and is cWe have described the first reported case of BDC with PVTT, with special reference to the diagnostic utility of FDG-PET scan. Even if the condition is advanced, surgical treatment combined with adjuvant chemotherapy may be feasible in selected patients.BDC: Bile duct cancer; PVTT: portal vein tumor thrombous; FDG-PET: Positron emission tomography with fluoro-2-deoxy-D-glucose; PV: Portal vein; HCC: hepatocellular carcinoma; CCA: cholangiocarcinoma, PD: pancreatoduodenectomy.The authors declare that they have no competing interests.MS preparation of the manuscript, concept and design and critically revising the manuscript. YI preparation of manuscript and concept and design, TS preparation of manuscript and concept and design, ST preparation of manuscript and concept and design, TF preparation of manuscript and concept and design, KM preparation of manuscript and concept and design, KK preparation of manuscript and concept and design. All authors read and approved final manuscript for publication.


An accurate, early diagnosis and treatment of adenomatous polyp can curtail progression to colorectal cancer. F-18 fluorodeoxyglucose positron emission tomography (F-18 FDG PET) reveals the biochemical changes associated with the development of many cancers which precede the appearance of gross anatomical changes that may be visualized during surgical resection or via imaging with MR or CT.We detail the history of a 64 year old female who had a whole-body FDG PET scan as a part of an employee wellness program. A dose of 12.2 mCi of F-18 labeled FDG was administered.A focal cecal uptake with a standardized uptake value (SUV) of 8.9 was found on the PET scan. Conversely, only normal mucosa was observed during a colonoscopy done 2 months after the PET scan. Motivated by the PET scan finding, the colonoscopist performed a biopsy which revealed a villous adenoma without high grade dysplasia. Pathology from tissue extracted during an exploratory laparatomy completed one month later found the lesion to be a villous adenoma with high grade dysplasia.Whole-body FDG PET scan revealed the biochemical metabolic changes in malignancy that preceded the appearance of any gross anatomical abnormality. A positive FDG PET scan indicative of colorectal cancer should be followed up with a colonoscopy and biopsy even in a visibly normal mucosa. We hereby report a case of advanced adenoma which was detected on a whole-body FDG PET scan despite the fact that the mucosa had a normal appearance on follow up colonoscopy. F-18 fluorodeoxyglucose positron emission tomography (F-18 FDG PET) reveals the biochemical changes associated with the development of many cancers which precede the appearance of gross anatomical changes that may be visualized during surgical resection or via imaging with MR or CT.A dose of 12.2 mCi was given to the patient following a 4 hour fasting period. The tracer incorporated for 1 hour prior to image acquisition. A 6 bed position, whole-body PET scan was performed on a Siemens HR+ PET scanner. Each bed position included a 2 minute transmission followed by a 5 minute emission scan. Reconstruction parameters were 2 iterations/8 subsets (OSEM) with a 4 mm smoothing Guassian filterThe patient is a 64 year old female who had a whole body FDG-PET scan in June 2004 as part of an employee wellness program. The scan was negative except for a focal abnormality in the cecal area which had an SUV of 8.9 Figure . The patTwo months after the FDG-PET scan, a colonoscopy was performed. There was no visible abnormality in the cecum Figure  but promPolyp is a small growth of tissue shaped like the head or stalk of a mushroom. They are classified histologically as neoplastic (adenomatous) or non neoplastic . AdenomaIn this educational case report, the patient had a high grade villous adenoma which was discovered due to its increased FDG uptake on a whole-body PET scan but the mucosa looked normal on the colonoscopy. FDG PET scanning detects biochemical changes which precede gross morphological changes in cancerous tissue. Increased cell surface glucose transporter protein 1 to 5 and increased level of hexokinase pathway explain the high FDG uptake in malignancy.Narrow Band imaging and Chromoendoscopy with indigo carmine can increase the yield of conventional colonoscopy. ,4 The reAccording to the current guidelines , since aFDG PET has a high sensitivity in diagnosing premalignant and advanced colonic neoplasia -14. Van Whole-body FDG PET scan revealed the biochemical metabolic changes in malignancy that preceded the appearance of any gross anatomical abnormality. A positive FDG PET scan with a focal uptake in colon should be considered a significant finding indicative of colorectal cancer and follow up colonoscopy and biopsy should be recommended even in a visibly normal mucosa.The author(s) declare that they have no competing interests.BR drafted the manuscript, BR and PS reviewed the literature,RC did the colonoscopy; AB admitted the patient and followed up with the patient,JM and MS were involved with imaging the patient; YD interpreted the pathology report.All authors read and approved the final manuscript.


Because intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) is believed to show a better clinical course than non-papillary biliary neoplasms, it is important to make a precise diagnosis and to perform complete surgical resection.th postoperative date. He has been regularly followed at outpatient department with no evidence of recurrence.We herein report a case of malignant IPMN-B treated by right trisectionectomy with caudate lobectomy and extrahepatic bile duct resection. Radiologic images showed marked dilatation of the left medial sectional bile duct (B4) resulting in a bulky cystic mass with multiple internal papillary projections. Duodenal endoscopic examination demonstrated very patulous ampullary orifice with mucin expulsion and endoscopic retrograde cholangiogram confirmed marked cystic dilatation of B4 with luminal filling defects. These findings suggested IPMN-B with malignancy potential. The functional volume of the left lateral section was estimated to be 45%. A planned extensive surgery was successfully performed. The remnant bile ducts were also dilated but had no macroscopic intraluminal tumorous lesion. The histopathological examination yielded the diagnosis of mucin-producing oncocytic intraductal papillary carcinoma of the bile duct with poorly differentiated carcinomas showing neuroendocrine differentiation. The tumor was 14.0 × 13.0 cm-sized and revealed no stromal invasiveness. Resection margins of the proximal bile duct and hepatic parenchyma were free of tumor cell. The patient showed no postoperative complication and was discharged on 10Considering a favorable prognosis of IPMN-B compared to non-papillary biliary neoplasms, this tumor can be a good indication for aggressive surgical resection regardless of its tumor size. Biliary intraductal neoplasms occur in both intrahepatic and extrahepatic bile ducts and are proposed to have two types; a flat and a papillary type ,2. NeoplA 43-year-old male patient was referred for a huge cystic tumor of the liver, which was detected in abdominal ultrasonography (US) performed for right upper quadrant discomfort in local clinic. He was admitted to our hospital for further evaluation. He had no other symptom or past medical history of liver disease. He did not smoke and was a social drinker. His physical examination on admission revealed vaguely enlarged liver palpable three finger breadths below the right costal margin without definite tenderness; otherwise, there was no significant finding including vital sign.Initial laboratory values included WBC of 9,490/μL, albumin of 4.2 g/dL, total bilirubin of 0.8 mg/dL, AST/ALT of 58/65 IU/L, alkaline phosphatase of 387 IU/L, γ-GTP of 764 IU/L, α-FP of 0.7 ng/mL, CEA of 3.1 ng/mL, CA 19-9 of 21.1 U/mL. Liver computed tomography (CT) and magnetic resonance imaging showed marked dilatation of the left medial sectional bile duct (B4) resulting in a bulky cystic mass with multiple internal papillary projections. This cystic mass was so huge as to displace the parenchyma of most right hemiliver, left medial section, and caudate lobe, compressing both portal pedicles, main hepatic veins, and inferior vena cava Figure . No intrNeither ascites nor peritoneal metastatic nodule was detected during initial intraperitoneal exploration. A single enlarged regional lymph node was encountered and excised for frozen section, and the result was free of tumor cell. Therefore, further dissection of lymph node was not performed. The tumor was adherent to but detachable from the left portal pedicle, the inferior vena cava, and the left hepatic vein. The remnant left lateral sectional bile ducts were also dilated but had no macroscopic intraluminal tumorous lesion, which was ascertained by intraoperative cholangioscopy. Consequently the planned right trisectionectomy with caudate lobectomy and extrahepatic bile duct resection could be successfully performed. Macroscopic examination of the resected specimen revealed a cystic dilatation of the intrahepatic bile ducts with intraluminal mucin and multiple papillary tumors Figure .Postoperative PT/INR level was normal and the values of total bilirubin and AST/ALT were peak on postoperative date (POD) 1, 6.7 mg/dL and 149/71 IU/L, respectively; after that they were gradually normalized. The histopathological examination for the resected specimen yielded the final diagnosis of mucin-producing oncocytic intraductal papillary carcinoma of the bile duct and revealed no stromal invasiveness Figure . The tumTwo types of biliary intraductal neoplasms preceding invasive cholangiocarcinoma have been identified so far; a flat type neoplastic lesion called BilIN, which develops into non-papillary cholangiocarcinoma, and a papillary type called IPMN-B with malignancy potential ,2. IPMN-Accumulated radiologic and endoscopic information on IPMN-B makes a diagnosis not so difficult. Specific findings of CT or US and cholangiograms, such as marked biliary dilatation and amorphous filling defects within the dilated bile ducts, could raise suspicion and simultaneous endoscopic demonstration of mucobilia is specific for the diagnosis of IPMN-B ,8,18. BeBecause IPMN-B is considered to have a favorable prognosis after complete surgical resection, an aggressive surgery deserves to be recommended regardless of tumor size and extent -10. The The unique pathological feature of our case was the two poorly differentiated carcinomas showing neuroendocrine differentiation. These tumors were not considered as metastatic lesion from an extrahepatic origin but instead were recognized as direct protrusion from the main tumor or connected daughter nodules; because they were abutting on the main cystic tumor and both the main tumor and the two adjacent carcinomas showed positivity in specific staining for neuroendocrine differentiation. The clinical significance of the two poorly differentiated carcinomas has not been determined but may be related to recurrence; they showed microvascular invasion. There has been no report on IPMN-B with accompanying carcinoma of neuroendocrine differentiation or intrahepatic daughter nodules.IPMN-B is subdivided on the basis of histology and mucin gene protein  expression into two to four subtypes; columnar and cuboidal types ; pancreaOver the last two decades, not a few cases on IPMN-B have been accumulated and its concept has continued to evolve: two types of intraductal biliary neoplasms, histopathological features and subtypes, resemblance to IPMN-P, diagnosis, radiologic findings and classifications, treatment strategy, surgical outcome, and prognosis. Therefore, IPMN-B deserves accepting as a discrete disease entity with a caution, being one definite type of intraductal biliary neoplasms and a biliary counterpart of IPMN-P. However, since there are still controversies on IPMN-B, more continual reports and studies are warranted to draw a full consensus on IPMN-B.We report herein a case diagnosed with typical findings of IPMN-B and successfully treated by right trisectionectomy with caudate lobectomy and extrahepatic bile duct resection. Considering a favorable prognosis of IPMN-B compared to that of non-papillary biliary neoplasms, this tumor can be a good indication for aggressive surgical resection regardless of tumor size. Furthermore, for establishing the concept of IPMN-B, more continual reports and studies are warranted.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.WJS participated in management of the patient, collecting the patient's data, and drafting the manuscript. SJ carried out management of the patient, conceived of the study, participated in its design and coordination, and helped to draft the manuscript. All authors read and approved the final manuscript.


Ectopic liver tissue is a rare entity, reported to occur in several intra-, retro- and extra- peritoneal sites, including the gallbladder. It is usually detected incidentally, during laparoscopy, laparotomy, or autopsy. Several possible mechanisms may explain the development of liver ectopia. Although ectopic liver tissue is usually asymptomatic, it behaves like orthotopic liver, developing the same pathologic conditions.We describe the case of a 54-year-old woman who was found to have a nodule attached to the gallbladder wall without any connection with the main liver, during an elective laparoscopic cholecystectomy for gallstone disease. The nodule was removed with the gallbladder and identified histologically as normal ectopic liver tissue.It would seem sensible to resect the ectopic tissue if encountered during cholecystectomy for gallstones. Laparoscopic management of ectopic liver can be feasible. Ectopic liver is a rare developmental anomaly in which liver tissue is situated outside the liver and has no hepatic connection . EctopicA 56- year old woman with a 10 month history of multiple attacks of biliary colic was found to have gallstones on ultrasound scan and underwent a routine laparoscopic cholecystectomy.A serosal encapsulated brownish mass, attached to the serosa of the gallbladder by a fibrous pedicle figures  and 2, wThe mass, measuring 15 mm × 5 mm × 7 mm, looked macroscopically like normal liver tissue figure .Histological examination revealed liver tissue containing normal tissue elements, i.e. portal tracts with bile ductules and vessels and normal hepatocytes with no disturbance in architecture or cholestasis figures  and 10, The patient made an uneventful postoperative recovery and was discharged home 24 hours later.Anatomic anomalies of the liver have been classified as accessory lobe of the liver with attachment to native liver, and ectopic liver tissue without connection to the liver proper . The terThe incidence of ectopic liver tissue attached to the gallbladder has been reported as low, but is likely to increase with the new diagnostic methods. In a 1940 review by Eiserth  of 5500 Several theories have been proposed to explain the development of ectopic liver at different sites: development of an accessory lobe of the liver with atrophy or regression of the original connection to the main liver; migration or displacement of a portion of the cranial part (pars hepatica) of the liver bud to other sites; dorsal budding of hepatic tissue before the closing of the pleuroperitoneal canals; trapping of hepatocyte-destined mesenchyma in different areas and entrapment of nests of cells in the region of the foregut following closure of the diaphragm or umbilical ring .The close relationship of the developing hepatic parenchymal cell cords to the pars cystica and early fetal duodenum explains why ectopic liver tissue could be found in the wall of the gallbladder, the gastrohepatic ligament, the umbilical cord, the adrenal glands, the diaphragm, the pancreas, the pylorus and the splenic capsule if a portion of the pars hepatica is displaced. Dorsal budding of hepatic tissue before closing of the pleuroperitoneal canals may explain how ectopic liver develops in the thoracic cavity, in locations such as esophagus, pericardium, intrapleural or extrapleural .Although the ectopic tissue is usually attached to the serosa of the gallbladder, as in the present case, or lies within its wall, it can also occur in the gallbladder lumen .Ectopic liver is sometimes associated with other congenital anomalies such as biliary atresia, agenesis of the caudate lobe, omphalocele, bile duct cyst or cardiac and conotruncal anomalies, but not when the heterotopic tissue is in the gallbladder ,8.Ectopic livers are supplied by an autonomous artery, not derived from the hepatic artery and some lesions do not have a portal vein system and a ductal connection with the billiary tract . EctopicThe natural course of ectopic liver tissue is unpredictable. The anomaly is relatively common in the perinatal period but disappears during postnatal remodelling .Symptoms occur rarely, and ectopic livers have been reported to cause recurrent abdominal pain due to torsion, compression of adjacent organs, intraperitoneal bleeding, as well as obstruction of the esophagus, portal vein, neonatal gastric outlet and pylorus ,13. Few Ectopic livers are subject to the same risk factors of disease and pathological processes that can affect the liver proper. Reported examples ,6 includEctopic liver is usually an incidental finding during a laparoscopy, laparotomy or autopsy performed for unrelated reasons, most commonly for diseases of the gallbladder ,15. DeteAn awareness of this entity may prevent mistaken diagnoses on those rare occasions when it is encountered. It would seem sensible to resect the ectopic tissue if encountered during cholecystectomy for gallstones, but to leave it alone if seen incidentally during other procedures. The removal of a known asymptomatic ectopic liver should be considered because of the potential for torsion and because of the increased propensity for malignancy. For obvious reasons, timely surgical treatment is necessary in the case of complicated ectopic liver. Laparoscopic management of ectopic liver can be feasible as in the case that we have presented.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.IT was advising doctor, the main surgeon and involved in drafting the manuscript and revising it critically for content. LP was the pathologist involved in analyzing the specimen. NN and AE were auxiliary surgeons. EA, MC and KG were involved in revising the draft critically for content. TC, carried out strategic planning for treatment of the patient and was involved in revising the draft critically for content. All authors have given final approval of the version to be published.


Synchronous composite tumors though described are uncommon. Moreover, simultaneous occurrence of synchronous tumors involving the same tissue or organ at multiple sites is even less common. We report a case of acute lymphoblastic leukemia (ALL) and basal cell carcinoma (BCC) occurring simultaneously in multiple skin sites. Several cases showing an association between cutaneous malignancies and lymphoproliferative disorders have been reported. Some of these cases included ALL and BCC and occurred often in the pediatric population with the BCC arising as a post-ALL therapy sequela. Other rare genetic causes may be considered. To our knowledge this is the first time that the synchronous occurrence of these two malignant processes in the same tissue involving multiple sites in an elderly patient is described. Composite malignancies are an interesting phenomenon that gives rise to questions related to etiologies, diagnostic implications, and treatment. We present an interesting case of a composite multifocal acute lymphoblastic leukemia (ALL) and basal cell carcinoma (BCC). Independently the two malignancies occur in two opposite extremes of age and their occurrence as a composite tumor is extremely unusual. In fact, to the best of our knowledge, composite ALL and BCC has never been reported before in the English literature.A previously healthy 76 year-old caucasian man presented with multiple slowly growing translucent tan and pink skin patches and nodules, involving the left upper limb, right supraclavicular fossa and the posterior surface of the right ear. These lesions showed ill-defined borders and were of variable sizes, which ranged from 1 cm to 3.5 cm in maximum dimension. Skin ulceration, lymphadenopathy and organomegaly were absent. Clinically, the skin lesions were diagnosed as BCC and were treated by wide excision.Pathology confirmed the presence of multiple BCCs and in addition the three biopsy sites showed diffuse infiltration of the dermis by large, atypical cells with blastic morphology and displaying perineural and perivascular accentuation Fig. , 2. OccaComposite double malignancies involving similar and multiple locations are extremely rare. Such tumors usually occur in a setting of immune deficiency or a genetic predisposition. BCC and ALL are two tumors that affect different age groups with the former almost always occurring in elderly and the latter in children. ALL associated with metachronous but not synchronous BCC has been reported -3. Such In our case, the presence of a dermal vasocentric undifferentiated large cell tumor in a setting of BCC occurring in an adult raised the differential of several hematological malignancies such as granulocytic sarcoma, acute myeloid leukemia, blastoid mantle cell lymphoma, ALL, and Burkitt's lymphoma, though the latter two tumors show a predilection for young age. In addition solid tumors such as neuroblastoma, malignant melanoma and Merkel cell carcinoma were also included. As the tumor was not in bone marrow and involved an elderly patient without a previous history of chemo- or radiotherapy, there was no consideration of haematogones in the differential diagnosis. With the aid of immunohistochemistry, the tumor was diagnosed as precursor B-ALL. Although skin involvement with B-ALL is frequent, it is interesting that this patient presented initially with a skin manifestation with multifocal BCC rather than the usual manifestations of ALL such as bone marrow failure, hepatosplenomegaly, or lymphadenopathy. The findings of pancytopenia and marrow failure were discovered only later.In conclusion we report an unusual case of composite multifocal precursor B-ALL and BCC. To the best of our knowledge this is the first case report in the English literature of the synchronous multifocal occurrence of these tumors in an adult. The case presented here emphasizes the value of awareness of composite tumors. The awareness of occurrence of composite tumors is crucial so that the patient can receive adequate therapy. Consideration of tumors in the differential diagnosis that fit the morphology but not the usual demographic profile, even in the absence of clinical suspicion, is a safe practice if accompanied by further ancillary studies to document or rule them out.


CYP21 gene coding for 21-hydroxylase; one was heterozygous for the I172N mutation and the other compound heterozygous for the I172N and I2splice mutations. The masses were not removed since myelolipomas are considered benign tumors, and the tumor size did not increase during four- and nine-year observation periods. An adrenal myelolipoma is an important exception to the rule that large tumours should be removed. Untreated CAH with prolonged excessive ACTH stimulation might contribute to the growth of adrenal masses. CAH should be considered as a differential diagnosis of patients with adrenal masses or adrenal myelolipomas.We present incidentally discovered adrenal myelolipomas in two adult males with untreated congenital adrenal hyperplasia (CAH). The patients had simple virilizing form of CAH due to mutations in the  Adrenal incidentalomas are common; the challenge is to rule out malignancy or excess hormone secretion . MyeloliIn 1999 a 50-year-old man presented with a right adrenal mass 5.7 × 5.1 × 1.9 cm. Medical history revealed precocius puberty at age 4, and he was diagnosed with “Morbus Glandulae Suprarenalis” when he was 9. Urinary 17-ketosteroids were found elevated, but suppressible with dexamethasone. He was treated with cortisone acetate 12.5 mg thrice daily from age 9, but at 18 he stopped the medication, after which he felt that his general wellbeing is improving. After this time he was lost to follow-up. At the age of 37 he underwent radioiodine treatment for thyrotoxicosis. He was otherwise healthy, working full time, and was the father of one child.In spite of radioiodine treatment he felt he had persistent goitre, and his general practitioner referred him to a cervical CT, which due to his short stature included the upper abdomen. Pronounced bilateral adrenal hyperplasia and a 5.7 cm in diameter tumor described as a typical adrenal myelolipoma  was found . CYP21 (steroid 21-hydroxylase) gene revealed the c. 515T > A point mutation, which results in the predicted amino acid change I172N. The mutation was present in hemi- or homozygous form . The adrenal mass caused no symptoms and was considered highly likely benign and therefore left untreated. The tumor and hyperplasia did not change radiologically over a nine-year subsequent observation period. The patient continues to do well without steroid medication, but is instructed to take steroids during stress.Further investigation revealed height 164 cm, weight 76 kg, and blood pressure 120/80 mmHg. On palpation the patient had no enlargement of the thyroid gland. Genital examination showed normal penile size and subnormal testicular volume of 12 mL bilaterally. Biochemical investigation revealed low basal cortisol, which did not increase after ACTH stimulation . He had A previously healthy 68-year-old man, father of one child, presented in 1994 with fatigue and nausea. For many years he had suffered arthralgic pain and had been treated with antiflogistics. The last months he had experienced problems with nocturia and impotence. He was referred to ultrasonography of the abdomen, on which a tumor in the right adrenal was detected. An adrenal CT scan showed a 4 × 4 × 2.5 cm tumor in the right adrenal with high fat content, which was radiologically considered to be a myelolipoma. The left adrenal was evaluated as normal. CYP21 revealed compound heterozygozity for the I172N mutation (c. 515T > A) and the I2 splice mutation . Further clinical investigation showed height 165 cm, blood pressure 140/80 mmHg, and normal male genitalia with testicular tenderness bilaterally. Biochemical investigation showed high basal level of 17-OH-progesterone, which was suppressible with dexamethasone, and an impaired cortisol response to ACTH stimulation . AdrenalThe myelolipoma was not removed and the tumor size did not increase during a four-year observation period. The patient was started on treatment with 5 mg prednisolone daily for one year, later cortisone acetate 12.5 mg twice a day whereupon his condition improved. The patient died suddenly, 73 years old, in his home; the cause of death remains unknown.We here report two male patients who presented late in life with large adrenal myelolipomas. One was previously treated for CAH, whereas one was diagnosed with 21-hydroxylase deficiency after being diagnosed with the adrenal tumor at 68 years of age. Both tumors were left untreated and did not grow over five- and nine-year observation periods. An adrenal mass normally requires surgical removal if the largest diameter exceeds 3 cm . UndiagnHowever, large myelolipomas could be dangerous if they start bleeding and give symptoms with pain, nausea, vomiting, and hypotension . BecauseIncreased prevalence of adrenal tumors has been reported in CAH patients and in heterozygote mutation carriers, possibly due to increased levels of ACTH which acts as a growth factor –15. It hIt was postulated early in the 1950s that ACTH elevation might induce transformation of adrenal tissue into myeloid tissue , and myeCYP21 mutations, showing inconsistent results which could be due to small samples [To our knowledge less than 20 cases of adrenal myelolipomas have been reported in patients with CAH to date , 18–20.  samples , 13, 15.The diagnosis of CAH in a patient with an incidental adrenal mass is important as the patient is at risk of adrenal crises . Such paIn conclusion, congenital adrenal hyperplasia, per se, should be included in the differential diagnosis of adrenal masses. If suspected clinically, assay of cortisol, ACTH, and 17OH-progesterone should be performed. Adrenal tumors are common in CAH and might require surgical removal, but myelolipomas are benign tumors that usually can be left untreated. Whether, treatment of CAH halts myelolipoma growth is uncertain.


Pneumatosis intestinalis, in association with portal venous gas, is a rare finding in children and young adults. In radiological studies, it is characterized by gas-filled cysts within the bowel-wall. It is often a sign of the serious significant underlying illness and is associated with a poor prognosis. A case of pneumatosis intestinalis and portal venous gas associated with abdominal tuberculosis in a child is presented here. Despite responding well to anti-tubercular treatment, he died suddenly at home, two months after discharge. It is recommended that cases with pneumatosis intestinalis should be carefully observed, although symptoms appear to be improving. Pneumatosis intestinalis (PI) or pneumatosis cystoides intestinalis, in association with portal venous gas, is a rare finding in children and young adults. It is characterized by gas-filled cysts within the bowel wall , 2.Du Vemoi first described PI in 1783 , 5.The relative importance of these conditions to PI in non-neonates is not clear because of the most previous reports which share an underlying diagnosis . In neonWe present here the first case of PI and portal venous gas associated with abdominal tuberculosis in a Turkish child and a brief review of the literature.An eight-year-old child was admitted to our hospital  in December 2007, with a two-year history of increasing abdominal distention, abdominal pain, weight loss, anorexia, and malaise. He had been seen and investigated by several paediatricians in other hospitals, including a diagnostic laparotomy. Histopathological diagnosis from the laparotomy was reported as ‘reactive lymph nodes’. He had no significant past medical history but there was a strong family history of tuberculosis: his grandmother, aunt, and maternal uncle had pulmonary tuberculosis, and his maternal uncle had died from pulmonary tuberculosis one year ago.On examination, he looked ill. He was apyrexial with no lymphadenopathy and had no Bacillus Calmette-Guerin scar. His height was on 50th percentile but his weight was only on 3rd percentile. Abdominal examination revealed diffuse distention and tenderness. The rest of the examination was unremarkable.On investigation, skin test for tuberculosis with 5 TU was negative. Haemoglobin and WBC were normal. Erythrocyte sedimentation rate was 20 mm/h. Serum adenosine deaminase level was high (34.80 U/L) . Antigliadin IgA, IgG, and anti-endomysial antibodies were negative. Serum IgG, IgA, and IgM levels were normal. P-ANCA, ASCA, ANA, and anti-ds-DNA were negative. He was HIV-negative.Plain abdominal x-rays showed dilated loops of bowel with extensive intramural gas, and free intra-abdominal gas was present . An abdoAn abdominal coronal and axial compound tomography (CT) images confirmed extensive small-bowel pneumatosis, pneumoperitoneum, hepatic portal venous gas, and chilaiditi syndrome  and 3.We performed upper and lower gastrointestinal endoscopy. In the upper gastrointestinal tract, antral gastritis and duedonitis were detected. In the lower gastrointestinal endoscopy, we could not pass sigmoid. So, no samples were sent for biopsy.Mycobacterium tuberculosis was positive in the histopathologic specimens from the mesenteric lymphnodes. Study of chest x-ray did not show any evidence of active or healed lesions of tuberculosis.Stool samples and gastric aspirates were negative for acid-fast bacilli. Polymerase chain reaction (PCR) for The patient appeared relatively stable with no clinical evidence of abdominal sepsis or bowel infarction. A surgical opinion recommended conservative treatment. His feeding was insufficient at admission but his appetite increased with treatment.A diagnosis of abdominal tuberculosis was made based on the positive PCR in the histopathologic specimen and positive family history of tuberculosis, and a four-drug anti-tuberculosis regimen (streptomycin+isoniazid+rifampicin+pyrazinamide) was started. Over the first three weeks, clinical symptoms resolved, abdominal distention decreased, and he gained 2 kg in weight. After three weeks, abdominal CT showed a little improvement in the small-bowel pneumatosis, pneuperitoneum, and hepatic portal venous gas  and 5. BPI is a radiological sign characterized by cystic or linear collections of gas in the subseroza or submucosa of the gastrointestinal tract , 7. ThreM. tuberculosis is a rare cause of the infectious conditions underlying PI  patients were aged less than 20 years , 13. M. lying PI . Accordilying PI .We report the first case of PI and HPVG with abdominal tuberculosis in a child. Tuberculosis is still a considerable problem in developing countries , 16. TheWe present this case to raise awareness of the diagnosis of abdominal tuberculosis in patients with PI and portal venous gas in endemic regions for tuberculosis or developing countries. PI and portal venous gas are an uncommon condition and often have a poor prognosis. Abdominal tuberculosis may be an unusual presentation in children with pneumatosis and portal venous gas. We recommend that cases with PI should be carefully observed, although symptoms appear to be improving.


Purpose. Primary extra-osseous osteosarcomas are uncommon            lesions, and those originating within the lung are especially rare, with few case reports            existing in the literature.            Patient. We report the case of a 48-year-old male smoker with a primary            osteosarcoma of the right lower lung lobe.            Results. Diagnosis was based on histopathological findings of a poorly            differentiated sarcoma with malignant cellular components of osteoid and chondroid matrix,            along with immunohistochemical and electron microscopy confirmation.            Extensive clinical and radiographic evaluation failed to reveal any tumor at other anatomical            sites.


An ingested foreign body often passes the gastrointestinal tract without any complications. Foreign bodies, such as dentures, fish bones, chicken bones, and toothpicks, have been known to cause perforation of the GI tract.We are presenting a case of a fifty-year-old male with acute abdomen; diffuse fibro purulent peritonitis, i.e. ileum perforation, caused by accidentally ingesting a chicken wishbone. He was treated surgically with ileum resection, and temporary ileostomy. After four months, intestinal continuity was established in the second operation.Intestinal perforation by a chicken bone is rare and affects the left colon or distal ileum. The lack of information of ingestion and detection of chicken bones preoperatively are of interest to be considered in the differential diagnosis of acute abdomen, which in this case was treated surgically. Ingesting a foreign body is not an uncommon occurrence and most foreign objects pass uninterrupted through the gastro intestinal tract without any complications . In a feWe are presenting a case with acute abdomen, i.e. ileum perforation, caused by accidentally ingesting a chicken wishbone.A fifty-year-old male was presented in the surgical emergency clinic with abdominal pain, nausea, and vomiting. The symptoms had started two days earlier. He used tobacco, but denied having consumed alcohol. The patient's height was 170 cm and his weight was 120 kg. His vital signs were: blood pressure 120/80 mmHg; pulse rate 90 beats/min.; respiration rate 19/min.; body temperature 38.5°C. His past medical history consisted of surgery for a thrombophlebitis in his right arm one year earlier.Upon physical examination, a distended and diffusely tender abdomen with right lower abdomen rebound was revealed. The patient's skin and mucosa were pale.9/liter; serum creatinine: 126 mol/L; blood urea nitrogen: 8.6 mmol/L; blood sugar: 11.6 mmol/L; Na: 141 mmol/L; K: 4.0 mmol/L; Ca++: 1.08 mmol/L; urinalysis: a lot of mucus, 4–6 Leucocytes; some epithelial cells. Plain abdominal radiography showed mechanical obstruction. Urgent abdominal ultrasound revealed mechanical obstruction, dilated small bowels and free liquid in the peritoneal cavity.The emergent laboratory tests revealed as follow: white blood cells (WBC): 12.6 × 10Clebsiela spp. was isolated in the stained abdominal liquid and treated with the proper antibiotics. Four months after the first operation and losing 25 kg, the patient underwent reconstruction of bowel continuity, and discharged on the tenth postoperative day with normal bowel movements.Laparotomy was performed in general anesthesia on the day the patient was admitted. Intra operative findings revealed diffuse fibro purulent peritonitis with adhesions between small bowels; and about 40 cm from Bauchini valve the presence of a sharp chicken wishbone perforated the ileum at the ante mesenteric site Figure . The walRetrospectively, after the first operation the patient admitted that four days earlier he had rapidly eaten and swallowed several mouthfuls of chicken meat without chewing and accidentally ingested a chicken bone.Foreign bodies (FB) accidentally ingested mostly pass through the gastrointestinal tract (GT) without any consequences . The mosSmall bowel perforations by FB are rarely diagnosed preoperatively because clinical symptoms are usually non-specific and mimic other surgical conditions, such as appendicitis and caecal diverticulitis . GreaterThe risk of perforation is related to the length and the sharpness of the object .Overeating, rapid eating, or a voracious appetite may be contributing factors for ingesting chicken bones. The mean time from ingestion to perforation was 10.4 days . Most peThe clinical presentation includes peritonitis, abdominal abscess formation , perineuPatients with FB perforations in the stomach, duodenum, and large intestine were significantly more likely to be febrile, to have chronic symptoms, to have a normal total white blood cell count, and to be asymptomatic or present with an abdominal mass or abscess, compared to those with FB perforations in the jejunum and ileum .The diagnosis was reached during laparotomy in more than 90% of the cases ,5,11,12.Although the imaging of findings can be nonspecific, the identification of a chicken bone with an associated mass or extra luminal collection of gas in patients with clinical sign of peritonitis, mechanical bowel obstruction, or pneumoperitonem strongly suggests the diagnosis ,13,19,20The treatment usually involves resection of the bowel, although occasionally repair has been described . The mosDuring laparotomy we found diffuse fibro purulent peritonitis and adhesions. A tiny sharp-pointed object was found penetrating the inflamed portion of the distal ileum Figure . Soon thThe HP diagnosis revealed that a macroscopic and histological feature of examined samples responds to Ileitis non specific, or the perforation of the small intestine Figure .Intestinal perforation by a chicken bone is rare and affects the left colon or distal ileum. The lack of information of ingestion and detection of chicken bones preoperatively are of interest to be considered in the differential diagnosis of acute abdomen, which in this case was treated surgically.We have written consent form from the patient for publication of this case report and accompanying images.The authors declare that they have no competing interests.FTH, SHIH, DSK and SHMH performed the surgery and general anesthesia. HLGL and FK has made Histopathology. FTH and ASK made substantial contributions to the concept, design and definition of intellectual content along with the literature search of the manuscript. All authors have participated sufficiently in the work to take public responsibility for appropriate portions of the content.All authors read and approved the final manuscript.