Neuroendocrine carcinoma is an aggressive neoplasm that mainly affects elderly Caucasians and typically arises in sun-exposed areas of the skin. The disease is rather rare and only a relatively few cases present with no apparent primary lesion.We report a case of an 81-year-old Caucasian male with neuroendocrine carcinoma, which initially presented as a large retroperitoneal mass. Pathological and immunohistochemical analysis of the transabdominal CT-guided biopsy specimen revealed tissue consistent with neuroendocrine carcinoma. The patient underwent exploratory laparotomy and the mass was successfully excised along with an associated mesenteric lymph node.There are currently two possible explanations for what occurred in our patient. First, the retroperitoneal mass could be a massively enlarged lymph node where precursor cells became neoplastic. This would be consistent with a presumptive diagnosis of primary nodal disease. Alternatively, an initial skin lesion could have spontaneously regressed and the retroperitoneal mass represents a single site of metastasis. Since Merkel cell precursors have never been identified within lymph nodes, the latter theory seems more befitting. Moreover, metastasis to the retroperitoneal lymph nodes has been reported as relatively common when compared to other sites such as liver, bone, brain and skin.Wide local excision of the primary tumor is the surgical treatment of choice for localized disease. We propose that further studies are needed to elucidate the true efficacy of chemotherapy in conventional as well as unconventional patients with neuroendocrine carcinoma. Neuroendocrine carcinoma, also known as Merkel cell carcinoma, is an aggressive neoplasm that mainly affects elderly Caucasians and typically arises in sun-exposed areas of the skin. In this report, we examine a rare case of neuroendocrine carcinoma, which initially presented as a retroperitoneal mass. After extensive workup, no primary site was identified in the skin or elsewhere. Relatively few cases present with no apparent primary lesion.An eighty-one year old Caucasian male presented in June 2006 to the Emergency Department of the Brooklyn Hospital Center with a one-month history of blood- tinged stools. He was admitted to the medical service for further management. The surgical team was consulted for evaluation of pain associated with a palpable mass in the right iliac fossa. The pain was described as dull, non-radiating and constant in nature. It was rated a 3 on a 10-point intensity scale and there were no reported exacerbating or ameliorating factors. The patient's history was significant for intermittent constipation and repair of an incarcerated umbilical hernia three weeks prior.On examination, vital signs were normal. The abdomen was non-distended with a 3-cm, midline, infraumbilical scar. Tenderness was noted on deep palpation of the right lower quadrant and a large mass was felt measuring approximately 5-cm × 5-cm. The lower margin could not be felt. The mass was also palpable on digital rectal exam. Femoral pulses were normal. Laboratory values were unremarkable. Fecal occult blood test was negative. Colonoscopy showed the cecum high in the right upper quadrant with no evidence of a cecal mass. Contrast computed tomography (CT) of both abdomen and pelvis Figure  revealedChest X-ray was unremarkable. Magnetic resonance imaging (MRI) of the pelvis Figure  confirmeThe patient then underwent exploratory laparotomy and the mass was successfully excised along with an associated mesenteric lymph node. Final pathologic analysis of the mass was consistent with biopsy findings and the mesenteric lymph node was negative for tumor. Extubation was delayed due to difficult intubation and the patient was transferred to the surgical intensive care unit for observation. He was extubated the following day and transferred to the surgical floor where he had an uneventful recovery and was discharged on postoperative day eight. The patient's care was then transferred to an oncologist at another institution where he is currently being monitored and evaluated for treatment with various chemotherapeutic regimens.Neuroendocrine carcinoma is an aggressive, usually cutaneous neoplasm with a propensity for metastasis. The tumor mainly affects elderly Caucasians. Although it is widely referred to as Merkel cell carcinoma, the term neuroendocrine carcinoma of the skin is more appropriate due to the tumor's histologic and histochemical characteristics . The disMerkel cells reside in the basal layer and hair follicles of the skin's epidermis and are associated with mechanoreceptors in the dermal papillae . It has Sun exposure is thought to play a major role in pathogenesis, but some cases present with lesions in non-sun-exposed areas. In our case, sun-exposure seems to have played no role, as the tumor apparently did not even involve the skin. Immunosuppression can also predispose to the disease, but it was not a factor in this case since the patient was not immunosuppressed. There are currently two possible explanations ,11-13 foIt is difficult to accurately diagnose neuroendocrine carcinoma due to its similarity to other poorly differentiated "small blue cell tumors" like small cell carcinoma of the lung . HistopaStaging is done according to criteria developed by the Memorial Sloan-Kettering Cancer Center . Stage IIn 2005, Allen et al reported five-year disease-specific survival for patients with neuroendocrine carcinoma to be 64% . In 2006The aggressive nature of this disease necessitates frequent follow-up. The presence of risk factors including tumors larger than 2-cm, truncal location, male sex, age over 65, nodal or distant disease at presentation and duration of disease before presentation [Wide local excision of the primary tumor is the surgical treatment of choice for localized disease. This approach along with nodal dissection and locoregional irradiation in clinically node-positive patients or elective lymph node resection in clinically node-negative patients has been associated with both decreased rate of and longer interval to recurrence. The value of chemotherapy is still undetermined . Thus, oThe author(s) declare that they have no competing interests.VB interpreted data and critically revised the manuscript. IDO reviewed the literature, prepared the manuscript and obtained patient consent. BN provided the case and critically revised the manuscript. PQX provided the pathological images and assisted in interpreting them. All authors read and approved the final manuscript.


Primitive neuroectodermal tumor (PNET) of the chest wall or Askin's tumor is a rare neoplasm of chest wall. It most often affects children and adolescents and is a very rare tumor in adults. In this case report, we present an Askin's tumor occurred in a 73-year-old male. The patient was admitted with a history of 3-month lower back pain and cough. In computed tomography, there was a lesion with dimensions of 70 × 40 × 65 mm in the superior segment of the lower lobe of the left lung. Positron emission tomography/computed tomography with 18F-flourodeoxyglucose revealed a pleural-based tumor in the left lung with a maximum standardized uptake value of 4.36. No distant or lymph node metastases were present. The patient had gone through surgery, and wedge resection of the superior segment of left lobe and partial resection of the ipsilateral ribs were performed. Pathology report with immunocytochemistry was consistent with PNET and the patient received chemotherapy after that. Primitive neuroectodermal tumors (PNETs) and Ewing Sarcoma are closely related malignant, small, round-cell tumors of soft tissues and bones . Both ofPNETs are malignant tumors of the central nervous system and usually found in infants, children, and young adults. PNETs arise from the primitive nerve cells of the nervous system but they can also occur in outside the central nervous system  including bone in the extremities, pelvis, and the chest wall. PNETs of the chest wall were originally reported by Askin et al.  in 1979 Herein, we report a 73-year-old male patient with Askin's tumor and describe findings on dual-modality positron emission tomography/computed tomography (PET/CT) carried out with 18F-flourodeoxyglucose (18F-FDG). Askin's tumor is a disease of pediatric age group and few young adults have been reported . In oldeA 73-year-old male patient without any history of smoking was admitted to hospital for lower back pain and cough on January 2009. From his history it was learnt that he was suffering from these symptoms for the last 3 months. Computed tomography confirmed the presence of a lesion with dimensions of 70 × 40 × 65 mm in the superior segment of the lower lobe of left lung. Invasion to the pleura and ipsilateral 6th, 7th, and 8th were also observed Figures . Fine-neThe patient referred to nuclear medicine department for PET/CT. Dual-modality PET/CT imaging was performed after intravenous injection of 370 Mega Bequerel (10 mCi) 18F-FDG with a PET/CT scanner  from the top of the head to the middle of the thigh. Positron emission tomography attenuation correction was based on the CT data and coregistered attenuation corrected PET image and CT image were reviewed. PET/CT revealed a lesion in the superior segment of the lower lobe of left lung. Maximum calculated standardized uptake value (SUVmax) of the lesion was 4.36 . No addiSince no distant and lymph node metastases were present and the patient was operable, he had gone through surgery. During surgery, a lesion arising from the chest wall, infiltrating the adjacent ribs, was identified. Left thoracotomy, wedge resection of superior segment of the lower lobe of left lung, and partial resection of 6th, 7th, and 8th ribs were performed. Light microscopy showed small size round cells with scanty cytoplasm assembling in alveolar structures . After iPNET of chest wall is a distinct clinical and pathological entity arising from the soft tissue of the chest wall, occasionally from the rib cage and rarely from the periphery of the lung . Radiologic features of central and peripheral PNETs have been documented , 7. SoftIn spite of the well-known radiologic features, limited data exists in the respect of the use of PET/CT fusion imaging in PNETs. Belonging to the same family, PET/CT has been shown to be useful for the detection of recurrence in Ewing sarcoma . PET/CT Ewing family of tumors are high-grade malignancies which causes to expect high standardized uptake values (SUVs). However, reported SUVs are not high. Györke et al.  studied In our patient, SUVmax of the lesion was calculated as 4.36 which was low like the other Ewing family of tumors and concordant with the findings of Györke et al. . HoweverExcept bronchoalveolar carcinomas, carcinoid tumors, and some subcentimeter-sized adenocarcinomas, FDG uptake is usually high in lung tumors. In a study by Tournoy et al., the role of 18F-FDG PET/CT in the staging of mediastinal lymph nodes was investigated and mean SUVmax of 8.34 was reported among all histologic subtypes of nonsmall cell carcinomas . So besiThe present case is unusual for the age of presentation and demonstrating the 18F-FDG PET/CT findings. Reported adult patients with Askin's tumor are usually young adults. Our patient is older than the reported patients previously. We concluded that Askin's tumor must be kept in mind as one etiologic possibility in small round cell tumors of the thoracic wall not only in children and young adults but also in elderly. PET/CT may be helpful for characterization of the metabolic activity of the tumor and for investigation of distant metastases.


Inflammatory pseudotumor is rare benign mass composed of chronic inflammatory cell infiltration and proliferating fibrous tissue. Some cases of inflammatory pseudotumor show abundant infiltrating IgG4-positive plasma cells and obliterative phlebitis, which are the pathologic hallmarks of autoimmune pancreatitis.A 77-year-old Japanese man was admitted to our hospital because of epigastric pain. A solitary mass with delayed enhancement by dynamic computed tomography was present in the left hepatic lobe. Endoscopic retrograde cholangiography showed only segmental stenosis of the left intrahepatic bile duct. No abnormal findings were detected in the pancreas. The patient was clinically diagnosed as having intrahepatic cholangiocarcinoma and underwent surgery. Histological examination of the hepatic mass and bile duct wall showed abundant IgG4-positive plasma cell infiltration with obliterative phlebitis. The final diagnosis was IgG4-related hepatic inflammatory pseudotumor with sclerosing cholangitis. Delayed enhancement by computed tomography is a characteristic feature of IgG4-related inflammatory pseudotumor similar to that of autoimmune pancreatitis.IgG4-related hepatic inflammatory pseudotumor unassociated with autoimmune pancreatitis should be one of the entities considered for differential diagnosis of liver tumors. Delayed enhancement on computed tomography might be useful finding for diagnosing IgG4-related hepatic inflammatory pseudotumor. Inflammatory pseudotumor (IPT) of the liver is a rare, benign lesion composed of chronic inflammatory cell infiltration and proliferating fibrous tissue. This disease is becoming an important entity to consider for differential diagnosis in patients with hepatic space-occupying lesions.Recently, hepatic IPT cases associated with autoimmune pancreatitis (AIP) have been reported -3 since We have previously reported that AIP is frequently associated with sclerosing cholangitis (SC) . SC assoA 77-year-old Japanese man with a 30-year history of alcohol-drinking was admitted to our hospital because of epigastric pain. His laboratory data on admission showed an elevated level of γ-glutamyl transpeptidase (114 IU/l).Computed tomography (CT) revealed a low-density mass in the left lateral segment. The mass was slightly enhanced in the early phase and further enhanced in the delayed phase . The masMagnetic resonance imaging (MRI) showed that the mass was slightly hypointense on T1-weighted images, and slightly hyperintense on T2-weighted images. Magnetic resonance cholangiopancreatography (MRCP) revealed stricture of the intrahepatic bile ducts with prestenotic dilatation in the left lateral segment . EndoscoMacroscopic examination of the resected left hepatic lobe revealed a whitish nodular mass measuring 4 x 3 cm spreading along the hilar bile duct to B3 . The proHistological examination showed diffuse and severe lymphoplasmacytic infiltration  and obliHistopathologically, this case of hepatic IPT showed the characteristic features of plasma cell granuloma. Abundant plasma cells were positively stained with anti-IgG4 antibody, and obstructive phlebitis was evident. These findings were similar to those of AIP. This IPT protruded from the thick wall of the bile duct and was associated with SC. There was no evidence of AIP in the pancreas.IPT can occur in various organs such as the lungs, liver, spleen and pancreas, and is a heterogeneous disease entity. Histologically, IPT has been subcategorized as plasma cell granuloma or inflammatory myofibroblastic tumor. IPTs of the plasma cell granuloma type show infiltration of numerous IgG4-positive plasma cells and obliterative phlebitis, which are the pathologic hallmarks of AIP ,8.The etiology of hepatic IPT remains elusive. Previous reports have indicated that cholangitis can be responsible for its development -14. The Autoimmune hepatitis (AIH) is one of the autoimmune disorders of the liver and histoligically characterized by a dense mononuclear and plasma cell infiltration of the portal areas. However, AIH usually do not form mass and abundant IgG4-positive plasma cells are not observed. These points were different from IgG4-related hepatic IPT.The present case was treated surgically under a suspected diagnosis of intrahepatic cholangiocarcinoma because of segmental bile duct stricture and portal obstruction. After surgery, we reassessed the imaging findings, and noted homogeneous delayed enhancement on dynamic CT, whereas MRI showed low intensity in the T1- and slightly high intensity in the T2-weighted images, similar to the CT and MRI features of AIP. We then evaluated the CT findings for five cases of IgG4-related IPT we had encountered . All of these cases showed delayed enhancement. Three cases in which the lesions were small showed homogeneous enhancement, while two cases in which the lesions were large showed heterogeneous enhancement.IgG4-related hepatic inflammatory pseudotumor unassociated with autoimmune pancreatitis should be one of the entities considered for differential diagnosis of liver tumors. IgG4-related hepatic IPT with sclerosing cholangitis had the characteristic CT findings of the other IgG4-related IPT. Delayed enhancement on CT might be useful finding for diagnosing IgG4-related hepatic IPT. If the serum IgG4 level is elevated, liver tumor biopsy and IgG4 staining are recommended.


Although unusual, hemangioma is the most common primary splenic neoplasm. Splenectomy is indicated when the tumor is large, with increased risk of hemorrhage. The laparoscopic approach is preferred for most elective splenectomies. Although technically feasible, laparoscopic splenectomy can be a challenge in the patient with splenomegaly. We present herein a case of an 18-year-old male asymptomatic patient who underwent laparoscopic splenectomy for the incidental finding of splenomegaly caused by a large splenic hemangioma. Laparoscopic splenectomy appears to be a safe and effective procedure, in appropriately experienced hands, for patients with splenomegaly, given the spleen's fragile anatomy and its relationship to other abdominal viscera. Splenic hemangioma is a rare disorder, but remains the most common benign neoplasm of the spleen . SplenecAn 18-year-old Greek male student presented for screening test in order to get a health certificate. Physical examination revealed splenomegaly, although the patient was asymptomatic. No other clinical signs were detected. Ultrasonography and Magnetic resonance imaging (MRI) showed a hypoechogenic mass involving the spleen and the splenomegaly . 45 MM Vascular/Thin). Each jaw was positioned anterior and posterior to the splenic vessels . 45 MM Vascular/Thin). The use of hemoclips is minimized throughout the procedure and especially around the hilum because the clips may interfere with future applications of a stapling device. The stapler will not function if a clip is caught within its jaws, and this can result in significant bleeding from hilar vessels. When possible, the splenic artery and vein are divided separately. However, mass hilar stapling is increasingly practiced with good long-term results. This is especially true for the magistral configuration of the splenic artery. The placement of a drain is not routinely recommended because it may be associated with postoperative left subphrenic abscess [The operation is begun with a thorough search of the abdominal cavity for the presence of accessory splenic tissue. Meticulous surgical technique to prevent splenosis secondary to splenic trauma during dissection is as important as a thorough search for accessory spleens . A 1-cm  abscess ,5. In ouLS is a safe procedure for patients with splenomegaly. Surgeons require advanced laparoscopic skills to deal with the challenge of splenectomy given the spleen's fragile anatomy and its relationship to other abdominal organs, especially the pancreas. Cautiousness is indicated in patients with portal hypertension and severe cardiopulmonary disease because they have a higher incidence of intraoperative bleeding and postoperative complications.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 his journal.The authors declare that they have no competing interests.CK, CE, MG, and GA analyzed and interpreted the data concerning the splenectomy operation. KV gathered the data concerning the patient case. PS, NT and EF contributed in the reference data. All authors read and approved the final manuscript.


There are several non-neoplastic lesions which mimick intramedullary spinal cord neoplasm in their radiographic and clinical presentation. These can be classified as either infectious  and non-infectious  inflammatory lesions, idiopathic necrotizing myelopathy, unusual vascular lesions and radiation myelopathy. Although biopsy may be indicated in many cases, an erroneous diagnosis of intramedullary neoplasm can often be eliminated pre-operatively.the authors report a very rare case of intramedullary non-specific inflammatory lesion of unknown origin, without signs of infection or demyelinization, in a woman who showed no other evidence of systemic disease.Intramedullary lesions that mimick a tumor can be various and difficult to interpret. Preoperative MRI does not allow a certain diagnosis because these lesions have a very similar signal intensity pattern. Specific tests for infective pathologies are useful for diagnosis, but histological examination is essential for establishing a certain diagnosis. In our case the final histological examination and the specific tests that we performed have not cleared our doubts regarding the nature of the lesion that remains controversial. There are several non-neoplastic lesions which mimick intramedullary spinal cord neoplasm. These can be classified as either infectious  and non-infectious  inflammatory lesions, idiopathic necrotizing myelopathy, unusual vascular lesions and radiation myelopathy. Although biopsy may be indicated in many cases, an erroneous diagnosis of intramedullary neoplasm can often be eliminated pre-operatively.A 71-year-old Italian woman presented a 2-month history of numbness and pain involving the left leg. She underwent orthopedic evaluation and articular ankle echography for the diagnostic suspicion of Baker cyst, that were negative. She also underwent lumbosacral MRI that did not show any signs of degenerative or traumatic injuries. One month later she developed radicular pain in both legs with hypoesthesia. She underwent cervico-dorsal MRI with contrast that showed a gadolinium-enhancing lesion within the spinal cord at T5-6 with maximum diameter of 11 mm. In the axial sequences the lesion seemed to be completely intramedullary without any signs of bulging. The neuroradiological aspects of the lesion were interpreted as an intramedullary astrocytoma or ependymoma. Neurologically, she had symmetric tendon reflexes, exaggerated in the legs, Babinsky sign on both legs, moderate paraparesis, hypoesthesia and dysesthesia of the entire left leg and left thorax below the T5 metamer. One month after the first MRI, during recovery, she underwent another dorsal MRI with contrast, which confirmed the presence of this intramedullary gadolinium-enhancing lesion at level T5-T6: this was interpreted as hemangioblastoma or ependymoma but, according to the neuroradiologist, it was impossibile to exclude other diagnostic hypothese , but no fungal or bacteria were found. Also the non-neoplastic nature of the lesion was demonstrated by immunohistochemical studies, which confirmed the mixed nature of lymphocytes, and by the polyclonality of plasma cells with positivity for kappa and lambda light chains of immunoglobulins fig , 3. PostWe will discuss in detail the differential diagnosis we considered:Intramedullary tumours of the spinal cord are rare. The most common are astrocytomas and ependymomas which together account for 90%. These lesions can cause significant difficulties in the differential diagnosis between inflammatory diseases such as multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM), and vascular abnormalities and neoplasms. Because the clinical characteristics of neoplastic and non-neoplastic spinal cord lesions may be very similar, we rely on MRI for making a correct diagnosis. The MRI makes it possible to locate tumours in the extradural, intradural or extramedullary spaces, or within the cord itself; the tumour's location and its MRI characteristics may actually identify its specific type. In some instances, however, it is quite difficult to identify the exact nature of the pathological changes without a complete and detailed history and clinical examination . In addiGranulomatous lesions affecting the spinal cord are principally tubercolosis, sarcoidosis, brucellosis and histocytosis X . In our In addition, non-tumoral intamedullary lesions generally originate from bacterial, fungal or parasytic localizations, but is unusual for an intramedullary abscess to be present so soon in the absence of systemic disease, as in our case. In these cases the most frequent etiopathology of infection depends on intravenous drug use and immune deficiency disorders, aspects that were not present in our patient . The lesAnother intamedullary lesion that mimicks a tumor can be an MS localization. Isolated spinal cord involvement has been rare and can be the initial manifestation of MS . MS is cAnother lesion which may mimick an intramedullary tumor is radiation melyopathy . The mosIn our experience it is considered appropriate strategy decompression surgery to be performed as soon as the symptoms given by compression of cord manifested by worsening paraparesis or paraplegia. All of that is supported later by histological analysis that can guide intraoperative tank towards the complete removal or to a simple biopsy then integrated with medical therapy. In our case, the therapeutic strategy "wait and see" was based exclusively on the aggravation of the clinical, especially neurological symptoms, radiological outcome and appearance of the extemporaneous histological lesions that favored an injury repetitive small cell lung cancer. All these aspects justified up to us the complete removal of the lesion, then the result is justified by the complete regression of symptoms.Intramedullary lesions that mimick a tumor can be various and difficult to interpret. Preoperative MRI does not allow a certain diagnosis because these lesions have a very similar signal intensity pattern. Specific tests for infective pathologies such as toxoplasmosis and TBC, besides specific tests for sarcoidosis, are useful for diagnosis. Histological examination is often essential for establishing a certain diagnosis. In our case the worsening of symptoms oriented us to a decompressive surgical strategy and total removal of the lesion, also in relation to the extemporary histological examination: this proved correct because of the drastic improvement observed in symptomatology and the total regression, without recrudescence, of symptoms and disease at 12 months follow-up. The final histological examination and the specific tests that we performed have not cleared our doubts regarding the nature of the lesion that remains controversial.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 have not been influenced by any financial or personal relationship with people or organizations in preparation of this study.All authors have made substantial contributions to in the design of the article:AL was responsible for editing, English editing, correction, search of the literature, conception and design, and has contributed in surgical technique. VDN was responsible for editorship of the manuscript. DED was responsible for the search of the literature. MC was responsible for the search of the literature. RT was responsible for the English editing. MA was responsible for the histology consulting and pathology examination. AS is the principal surgeon and was responsible for editing RD is the principal surgeon and was responsible for editing.


Ewing’s sarcoma is second most common primary malignant bone neoplasm in children and adolescents. We report a case of Ewing’s sarcoma of ilium in an 18-year-old female mimicking clinically and radiologically inflammatory arthritis of hip joint, a rare entity not mentioned in literature.An 18-year-old girl presented with pain hip, limp and fever off and on. Patient had restricted range of motion, raised erythrocyte sedimentation rate, leucocytosis and radiograph showed reduced joint space. Magnetic resonance imaging pelvis revealed an altered marrow signal of acetabulum with a large soft tissue component. Histopathology revealed a malignant round cell tumor consistent with Ewing’s sarcoma.Classical clinical and radiological presentation of Ewing’s sarcoma of ilium may not be the rule. One should be highly suspicious of the disease even if there is no direct pointer to the disease as was encountered in our case. Ewing’s sarcoma is a highly malignant tumor of bone and soft tissue in children and adolescents. It was named after James Ewing, who first described it as a distinct entity . ES is tWe report a case of 18-year-old female patient, Asian Indian in origin presenting with one-month history of pain right hip, limp and fever off and on. On examination patient was febrile, there was localized tenderness over ilium, no swelling, had restricted internal and external rotation of right hip and an apparent lengthening of 1.5 cm. Patient had no rectal or urinary complaints. Investigations revealed a sedimentation rate of 45 mm, hemoglobin 10.6 gm%, TLC of 12500, PCR for tuberculosis was negative. Plain radiograph pelvis in AP view showed reduced right hip joint space with subtle, hard to pick changes in the right ilium . DigitalEwing’s sarcoma accounts for approximately 10% of malignant bone tumors. The male: female ratio is 3:2. Clinically presents as pain with swelling and a palpable mass. Back pain and parathesias can be associated with paraspinal or pelvic tumors. Common nonspecific systemic manifestations include fever, fatigue, weight loss, and anemia mimicking acute or chronic infection. Additional symptoms and signs depend on the specific location of the tumor. Ewing’s sarcoma can affect all bones, but is slightly more common in tubular bones. The flat bones most often involved are the ilium, ischium, rib, and scapula. More than 50% of patients have a soft tissue component. Ewing’s sarcoma is one of the few bone sarcomas that affect the bones of the hands and feet, although in less than 5% of patients. Hematogenous dissemination is frequent to the lung, bones, and bone marrow. Extraosseous extension is common. On plain radiographs classic appearance is an intramedullary lesion with aggressive permeative or moth-eaten bone destruction. Periosteal new bone can be laminated or interrupted with a “sunburst” appearance. A Codman’s triangle is less commonly seen. A characteristic, but not specific, appearance is subperiosteal extension with subperiosteal bone destruction producing a concave “saucerization” defect.After going through literature on ES of ilium and surfing through pubmed data base, no case of ES of the ilium mimicking inflammatory arthritis hip has been reported to the best of our knowledge. Although ES ilium mimicking sacroiliitis have been reported ,8. The rBefore starting treatment patients should be evaluated by specialists from all disciplines  as early as possible. The surgeon or orthopedic oncologist who will perform the definitive surgery should be involved prior to or during the biopsy so that the incision can be placed in an acceptable location. The successful treatment of patients with Ewing family of tumors (EFT) requires systemic chemotherapy in conjunction with either surgery or radiation therapy or both modalities for local tumor control. In general, patients receive preoperative chemotherapy prior to instituting local control measures. Multidrug chemotherapy for EFT always includes vincristine, doxorubicin, ifosfamide, and etoposide. Most protocols use cyclophosphamide as well. We did not use cyclophosphamide in our patient. Duration of primary chemotherapy ranges from 6 months to approximately 1 year. While surgery is effective and appropriate for patients who can undergo complete resection with acceptable morbidity, children who have unresectable tumors or who would suffer loss of function are treated with radiation therapy alone. Those who undergo gross resections with microscopic residual disease may benefit from adjuvant radiation therapy. Prognosis depends on extent of the disease, size and location of the tumor, presence or absence of the tumor metastasis, tumor response to therapy, age,and disease relapse. Most centers today report long term survival of 60% to 70%. The worst prognostic factor is the presence of distant metastasis. Even with aggressive treatment, patients with metastasis have only a chance of 20% long term survival. Histological grades are of no prognostic significance. As already mentioned fever, anemia and elevation of WBC, ESR and lactate dehydrogenase have been reported to indicate more extensive disease and a worse prognosis.The classical clinical and radiological presentation of ES of iliac bone may not be the rule; one should be highly suspicious of the disease even if there is no direct pointer to the disease as was encountered in our case. Orthopaedicians, rheumatologists and radiologists should be alert to this rare atypical occurrence.


Retrorectal hamartomas or tail gut cysts are rare congenital anomalies most commonly seen in a retrorectal location; most common in middle aged women. This article describes the radiological appearance in two cases of tail gut cysts in males, one a child with a visible perianal swelling since birth and the other, a 72-year-old man with symptoms for one week. In both, the tailgut cysts were in a right perirectal location. Presentation in such a location in males, at extremes of age, is unusual for tailgut cysts. Retrorectal hamartomas or tailgut cysts are rare congenital anomalies arising from the vestiges of the embryonic hindgut. They are more common in middle-aged women and usuaA 19-month-old male child presented to the pediatric outpatient department for evaluation of a large swelling in the perianal region, which was present since birth and had gradually increased in size. An earlier attempt at aspiration at one year of age revealed that it contained brownish fluid. An initial reduction in size of the mass following the aspiration was followed by gradual increase. On examination, the mass was compressible and nontender. CT scan showed a homogenous, well-defined, cystic lesion in the right perirectal/perianal region, displacing the rectum to the left . No soliin toto. Histopathology revealed a cystic lesion lined by cuboidal and columnar epithelium supported by fibroconnective tissue and skeletal muscle fibers, consistent with a cystic hamartoma.The mass was excised A 72-year-old male presented with urinary incontinence for a week. On rectal examination, there was mild tenderness and evidence of extrinsic compression of the rectum and anal canal from the 7 o'clock to 12 o'clock positions, without any mucosal involvement. Pelvic MRI showed a large ovoid, well-defined cystic lesion in close apposition to the right rectal wall, displacing the rectum to the left. Intralesional hyperintensity on both T1- and T2-weighted images, with layering suggestive of high protein or hemorrhagic contents was noted . The patTailgut cysts are rare developmental cysts which, along with duplication cysts, are categorized under enteric cysts. The tail2Presentation is usually as an asymptomatic retrorectal mass or with abdominal pain or constipation. Some patients may present with recurrent retrorectal abscesses or following multiple procedures for anal fistulae. On gross appearance they are unilocular or multilocular cystic lesions with mucoid contents. Microscopy shows a variety of epithelia without the presence of villi and crypts. An incomplete muscle layer is often associated with the cyst wall. Infected cysts show cyst wall fibrosis and breakdown of the epithelial lining. Rarely, malignant transformation may occur in the cyst.Assessment with USG is of limited value, showing only a multilocular cyst with debris and gelatinous material seen as internal echoes. On CT scan, tailgut cysts appear as a discrete, well-marginated mass of water or soft-tissue density as seen in our first patient. Calcification of the thin wall may rarely be seen. In case et al, as being specific for tailgut cysts.[et al.[A contrast study may have helped clarify the possibility of infection within the cyst. Though typically hypo intense on T1W and hyper intense on T2W images, a heterogeneous appearance on MRI may be seen owing to mucin, proteinaceous material, or hemorrhage within the cyst.7 T1 and ut cysts. However,s.[et al. A similaMost previous reports on tailgut cysts have described them as being more common in middle-aged women;–68 their623We thus highlight the importance of considering tailgut cyst as a possible differential in any case of perirectal cyst, irrespective of age and sex, and the importance of imaging in assessing the characteristics and extent prior to surgery.


Background. Teratomas are the most common tumors. They are usually localized in the sacrococcygeal area, while the pharyngeal localization is very rare. The number of cases of stomatopharyngeal teratomas detected prenatally via sonography is very small. Case Report. We present the case of a 24-year-old primipara at 18 weeks' gestation, that at the routine ultrasound scan, the fetus was found with an echogenic mass, filling the stomatopharyngeal cavity and protruding from the mouth. Other abnormalities were not found. Termination of pregnancy was achieved using misoprostol. A female stillborn fetus with a weight of 250 g and length of 25.5 cm was delivered. The postmortem and pathologic examination confirmed the diagnosis. Conclusion. Pharyngeal teratomas can be diagnosed with the use of ultrasounds in utero facilitating parents' counseling in early time. Teratomas are tumors developedfrom totipotent stem cells and contain tissues of ectodermal, endodermal, andmesodermal origin . A fetal+6 weeks of pregnancy or theultrasound fetal measurement and quadrant blood test  during the second trimester of pregnancy were notperformed. She was healthy, used no medication, had not been exposed to knownradiation and there were no malformed neonates within her family. Sonographicexamination revealed a single live fetus, 17+5 weeks of gestation withbiparietal diameter of 44 mm, head circumference of 155 mm, femur length of 26 mm, and abdominal circumference of 144.5 mm. There was a complex mass fillingthe oropharynx and emerging from the fetal mouth (Figures A healthy 24-year-oldprimigravida was presented to the Department of Obstetrics and Gynaecology,Tzaneio General State Hospital, Piraeus, Greece, for the first visit of herpregnancy. The combined nuchal translucency and biochemical markers screeningtest for aneuploidies between the 11 and 13Figures  and 4.The autopsy revealed anexophytic palatine mass that measured 6 × 4 × 2 cm and was attached to theoropharynx and nasopharynx. Histological analysis confirmed the diagnosis ofpharyngeal teratoma. There were no additional malformations or fetalchromosomal aberrations. Also, parental chromosomes were normal. The parentswere reassured that this was a sporadic occurrence and that recurrence risk wasextremely low.Teratomas are the most commoncongenital neoplasms, which account for 25–35% of all neonatal tumors andpresent with an incidence of 1 in 20 000 to 1 in 40 000 live births , 9. Howα-fetoprotein assays areusually used to screen for neural tube defects and autosomal trisomies. However, elevated levels of maternal serum α-fetoprotein have also beenreported in pregnancies with fetal defects such as tracheoesophageal fistula,esophageal obstruction, sacrococcygeal teratoma, and anorectal atresia. Thephysiological mechanism of maternal serum α-fetoprotein elevation interatomas is probably the loss of fetal serum into the amniotic cavity combinedwith esophageal and tracheal obstruction by the mass [α-fetoproteincan lead to a sonographic investigation and the diagnosis of the tumor [The clinical presentation ofcongenital pharyngeal teratoma varies. Maternal serum the mass . The highe tumor . Itmay he tumor . The solhe tumor . These the tumor . In our The prognosis of pharyngealteratoma is poor , 11. It Pharyngeal teratomas can besuspected with the use of ultrasounds in utero facilitating parents'counseling in early time. However, the final diagnosis of pharyngeal teratomasis ultimately a pathological diagnosis. If the parents decide to continue thepregnancy, consideration should be given utilizing the exit procedure for airmanagement at delivery. Finally, it is important to reassure parents that theyare not at increased risk of bearing another child with these lesions.


Xanthogranulomatous cystitis (XC) is a rare benign disease of unknown etiology. A case of XC in a 30-year-old male is presented due to sparcity of such case report in medical literature. Patient evaluation included clinical, biochemical and radiological studies before treatment. Histological study revealed the rare diagnosis. Patient was asymptomatic at eight weeks follow-up after treatment. Xanthogranulamatous changes has been reported to occur in many sites2 includiE. coli and urine cytology revealed no malignant cells. Ultrasonography showed thickened wall urinary bladder with normal capacity and large hypoechoic lesion of 60 mm×55 mm on left lateral wall of urinary bladder with good vascularity on color doppler. Contrast enhanced computed tomography (CT) demonstrated 65 mm×55 mm growth present on left lateral wall of urinary bladder. No invasion of surrounding organ noted [A 30-year-old male presented with sixth month history of urgency, frequency, dysuria, hematuria and lower abdominal pain. He had no significant past medical history. Physical examination was normal. Laboratory studies revealed normal hematological and biochemical profile. Urinalysis shows 10–20 red blood cells and plenty of white blood cells per high power field. Urine culture grew an noted .After treatment with culture specific antibiotic cystoscopy was done, it revealed approximately 60×50 mm mass protruding from left lateral wall of bladder . CompletPostoperative course was uneventful. The patient received antibiotics for four weeks. At follow-up after three months, he was asymptomatic and urine culture was sterile.XC is a rare benign chronic inflammatory disease and majority of the reported cases are associated with urachal diverticula. It was f6Since medical treatment is ineffective, conservative management is rarely employed. The cura6


A subpubic cartilaginous cyst is a rare and innocent defect originating from the symphysis pubis and may present as a vulvar mass and/or chronic abdominal pain. The symphysis pubis is a non-synovial amphiarthrodial joint that forms a fibrocartilaginous union between the two pubic bones and in general lies without mainstream interest, despite its clinical relevance. This case report focuses attention on this joint.A 55-year-old Caucasian woman presented with a painful and rapidly increasing vulvar mass. Imaging techniques revealed a cyst-like structure originating from a degenerated symphysis pubis. The cyst was excised, however, recovery was complicated by a symphysiolysis.A subpubic cartilaginous cyst is a rare cystic lesion originating from the symphysis of the pubic bone which presents as a vulvar mass which might be complicated by symphysiolysis. Several diseases affect the symphysis pubis and result in significant discomfort and disability in patients. The symphysis pubis is therefore clinically important and should not be omitted in the differential diagnosis of a vulvar mass and/or chronic abdominal pain. The subpubic cartilaginous cyst (SCC) is a rare cystic lesion originating from the symphysis of the pubic bone which presents as a vulvar mass and/or chronic abdominal pain . These lA 55-year-old woman, G4P3, presented to her physician with a painful vulvar mass that had developed over approximately six months. The mass had suddenly became painful after rising, resulting in an antalgic walking pattern for three days. After this, the pain had subsided spontaneously, however, the mass increased more rapidly. The patient was not on any medication. Her medical history revealed carpal tunnel syndrome, ganglion cyst in the foot, and vaginal uterus extirpation. Physical examination revealed a solitary vulvar mass at the cranial site of the vulva, directly under the clitoris. The mass was round, smooth and was connected to the symphysis pubis. There was no connection to the mucous membrane of the vulva. There were no palpable masses in the groins.Subsequently, ultrasonography was performed which revealed a 3.7 × 2.9 × 4.0 cm large mass which was in a close relationship with the symphysis pubis. Conventional X-ray examination of the pelvis showed a relatively wide symphysis pubis with a sclerotic aspect Figure . A cortiAspiration and deposition of local steroids was attempted once with a 16 G needle: this resulted in a temporary reduction of the mass, however, the mass did not disappear. Subsequent aspiration was no longer possible, as no fluids could be withdrawn. Next, resection of the cyst was performed under general anesthesia, revealing a cyst containing yellow, granular debris and some serous fluids Figure  and 2B. Histopathologic analysis of the resection specimen revealed degenerated cartilage and synovial and connective tissue, surrounded by a connective tissue structure identified as the wall of the cyst Figure  and 2B.A subpubic cartilaginous cyst is a rare and poorly understood form of a ganglion cyst, arising from the symphysis pubis which was first described in 1996  or with Diagnosis is based upon clinical examination and radiological evaluation. The radiological appearance of the SCC depends on the mucinous material and chondrocytic elements in the contents. This results in a dense presentation on ultrasonography, a high density in CT images and a heterogeneous intensity on T1 and T2 MRI sequences .The treatment of choice is resection, as aspiration is not possible due to the bulky content of the cysts and recurrences have not been reported in the literature although follow-up was limited (three years) . In one The resection of the SCC was complicated by a symphysiolysis . Multiple terms have been used for this unambiguously defined condition that is rather defined as pain in the symphysis pubis ranging from mild discomfort to severely debilitating pain . SPD is Pseudomonas aeruginosa[The most common inflammatory disease of the symphysis pubis is osteitis pubis, a self-limiting, non-infectious inflammation of the symphysis following trauma , pelvic eruginosa. Treatmeeruginosa. FurtherWe describe a patient with degenerative changes in the symphysis pubis resulting in a rarely described subpubic cartilaginous cyst and symphysiolysis. The diseases of the symphysis pubis are summarized in this article and their clinical relevance is underscored.CT: computed tomography; MRI: magnetic resonance imaging; SCC: subpubic cartilaginous cyst; SPD: symphysis pubis dysfunction.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.RH and NG performed the surgery and were major contributors to the manuscript. HK performed the radiological studies and reviewed the manuscript. JW performed the histological studies and reviewed the manuscript. All authors read and approved the final manuscript.


Brainstem gliomas are rare in adults. They most commonly occur in the pons and are most likely to be high-grade lesions. The diagnosis of a high-grade brainstem glioma is usually reached due to the presentation of rapidly progressing brainstem, cranial nerve and cerebellar symptoms. These symptoms do, however, overlap with a variety of other central nervous system disorders. Magnetic resonance imaging is the radiographic modality of choice, but can still be misleading.A 48-year-old Caucasian woman presented with headache and vomiting followed by cerebellar signs and confusion. Magnetic resonance imaging findings were suggestive of a demyelinating process, but the patient failed to respond to therapy. Her condition rapidly progressed and she died. At autopsy, a high-grade invasive pontine tumor was identified. Histological evaluation revealed glioblastoma multiforme.While pontine gliomas are rare in adults, those that do occur tend to be high-grade and rapidly progressive. Progression of symptoms from non-specific findings of headache and vomiting to rapid neurological deterioration, as occurred in our patient, is common in glioblastoma multiforme. While radiographic findings are often suggestive of the underlying pathology, this case represents the possibility of glioblastoma multiforme presenting as a deceptively benign appearing lesion. Brainstem gliomas are rare in adults, with approximately 100 cases reported per year [A 48-year-old otherwise apparently healthy Caucasian woman presented to our clinic complaining of headache, nausea and vomiting for 5 days. Physical examination and laboratory tests were within normal limits. She was diagnosed with tension headache and sinusitis and discharged on trimethoprim-sulfamethoxazole. The patient returned to the clinic 3 days later with resolution of her headache but continued vomiting, approximately twice per day. She was treated with an over-the-counter proton pump inhibitor for suspected gastroesophageal reflux.Over the following week, she developed ataxia, diplopia and recurrence of her headache. She presented to the emergency department, at which time a T2-weighted magnetic resonance image (MRI) scan of the head showed increased signaling of the left temporal lobe, bilateral pontine areas, left peridentate nucleus and cervical spinal cord. These findings were thought to represent an acute demyelinating process. At this point, clinical suspicion for myasthenia gravis was high, and she was administered a trial of pyridostigmine without improvement.During her hospitalization, she developed a blood pressure of 190/90 mmHg, a serum sodium level of 123 mEq/l, a glucose level of 176 mg/dl, and a white blood cell count of 12,800 cells/ml. Clinical examination during her stay showed progression of neurological symptoms with decreased sensorium, hallucinations, decreased attention span, and gait disturbance. A lumbar puncture showed increased opening pressure. Based on the radiographic, laboratory and clinical findings, a diagnosis of acute demyelinating encephalopathy was made; suspicions of infectious encephalitis, collagen vascular disease, and toxic encephalitis were high. She received plasmapheresis without improvement.A follow-up T2-weighted MRI was performed, which showed increasing enlargement of the bilateral pontine regions with hydrocephalus and mass effect on the fourth ventricle. Because of the absence of pathological enhancement of the pons, this was felt to be an aggressive demyelinating process or a low-grade glioma. The patient continued to deteriorate neurologically, became comatose and died approximately 4 weeks after admission.At autopsy, the brain was edematous with a weight of 1375 g. The pons and medulla showed an infiltrative mass with extension into the cerebellum. Histological examination revealed a poorly differentiated infiltrative astrocytoma with nuclear pleomorphism, increased mitotic activity and focal necrosis Figures . StaininThe clinical presentation of brainstem gliomas is often non-specific and misleading. Radiological imaging is imperative to demonstrate a brainstem lesion but, as seen in this patient, is not always capable of detecting the true nature of the lesion. Based on the signaling pattern, our patient's MRI abnormalities were thought to represent a diffuse demyelinating pattern. On subsequent MRI, an enlarging brainstem mass was identified but was felt to represent an area of demyelination or, at most, a low-grade glioma. Autopsy confirmed the diagnosis of GBM with high-grade and infiltrative features.Although they account for 30% of pediatric posterior fossa tumors , gliomasWhile histological examination is required for definitive diagnosis, due to the difficult nature of obtaining tissue from the brainstem, radiographic imaging is often the preferred method of diagnosis and classification . ComputeMisdiagnoses associated with brainstem gliomas are reported in the literature. Cases have been mistaken for cervical myelopathy , meningiet al. performed a retrospective analysis of 45 cases of brainstem gliomas [et al. reported an autopsy series of 25 brainstem gliomas [Badhe  gliomas ; 24% occ gliomas . GBM wasThe ability to recognize brainstem tumors early in the disease process is imperative to prevent mass effect on surrounding structures, which can be rapidly fatal. Radiographic imaging by MRI is the most accurate tool for initial evaluation but can be misleading, as low-grade tumors, inflammatory diseases and demyelinating processes can have a similar appearance to high-grade lesions. Likewise, the clinical signs of brainstem tumors overlap with a variety of other central nervous system diseases. The rarity of brainstem gliomas in adults makes recognition all the more difficult. In order to provide rapid and early treatment, a high index of suspicion must be maintained for new onset of brainstem and cerebellar symptoms that fail to respond to treatment for more common disorders.Written informed consent was obtained from the next-of-kin of 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.SL and LH secured the case, conducted the literature review, and participated in the preparation of the manuscript. Both authors read and approved the final manuscript.


Objective: Nodular fasciitis is a benign proliferative spindle-cell lesion found in the subcutaneous fascia that presents as a rapidly growing mass with rich cellularity and mitotic activity, leading to it frequently being mistaken for a sarcoma. Histomorphologic features and immunohistochemical profile are useful in proper diagnosis. The treatment is complete excision, and recurrence is uncommon. A rare presentation and treatment of nodular fasciitis in the cheek of an infant is described here. Methods: The lesion was resected by direct transcutaneous approach, and the skin was closed with 2 layers of purse-string polydioxanone sutures followed by fast-absorbing gut sutures to reapproximate the epidermis. Results: Postoperatively, the patient had a wound dehiscence on the cheek, which healed secondarily with wound care. A small area of palpable tumor unable to be resected remained stable after 4 months of follow-up, and facial nerve function was preserved. Conclusions: Although infrequent in both children and the oral mucosa, nodular fasciitis should be considered in the differential diagnosis of facial tumors in infants and young children. Nodular fasciitis is defined by the World Health Organization as a benign and reactive fibroblastic growth extending from the superficial fascia into the subcutaneous tissue or muscle.A 3-month-old girl with no significant medical history or history of trauma presented to an outside emergency department with a mass in the left cheek that had been rapidly growing over the course of 1 month Fig . On examOver the course of several days, the patient was evaluated by pediatric oncology, pediatric surgery, pediatric plastic surgery, and oral and maxillofacial surgery. Computed axial tomographic scans were performed with contrast showing a solid mass in the subcutaneous tissues of the left cheek unable to be separated from the buccinator muscle Fig . The undThe child was scheduled for resection of the lesion because of its continued proliferation and evidence of ulceration through the overlying skin Fig . The lesSurgical pathology confirmed the previous diagnosis of a benign myofibroblastic lesion. The histology showed a spindle cell lesion with a nodular growth pattern possessing eosinophilic cytoplasm and elongated tapered nuclei Fig . There wPostoperatively the patient had a wound dehiscence on the cheek, which healed secondarily with wound care. The small area of palpable tumor remained stable after 4 months of follow-up, and facial nerve function was preserved Figs  and B.Konwaler et al.Nodular fasciitis is extremely uncommon in children; it most commonly presents in individuals in their third to fifth decades of life. When seen in children, it may be confused with other more common soft tissue tumors such as fibroma, lipoma, desmoid tumor, sarcoma, chondroma, myxoma, malignant fibrous histiocytoma, schwannoma, atypical fibroxanthoma, or parotid tumor.11Nodular fasciitis accounts for 0.025% of all pathologic diagnoses, with less than 4% of those cases occurring in children aged 0–9 years of age.9This case demonstrates that, although infrequent in both children and the oral mucosa, nodular fasciitis should be considered in the differential diagnosis of facial tumors. Nodular fasciitis can be differentiated from other facial tumors, including sarcomatoid carcinoma, fibrosarcoma, leiomyosarcoma, and neurofibroma, by histopathological examination of the tissue. Accurate diagnosis and timely and comprehensive treatment and follow-up are necessary to avoid inappropriate measures and limit potential morbidities.


Many causes have been described for acute gastric dilatation and necrosis . The onset of symptoms is generally catastrophic and surgical therapy must be emergent and aggressive because mortality is elevated for delayed diagnosis. We describe a case of sudden shock aroused after an orthopedic operation due to gastric ischemic rupture, apparently not ascribable to causes previously reported in the lay literature.A 79-year-old woman underwent right hip replacement for femoral bone fracture. Past medical history was significant for hypertension, without any upper gastrointestinal pathology reported. In the first two post-operative (p.o.) days, there was no evidence of clinical or blood abnormalities apart from leukocytosis  and thus refeeding was started since the day after operation. Proton pump inhibitors were given in order to prevent stress ulcers or gastritis. On p.o. day 3, after a complaint of epigastric pain, a sudden hemodynamic shock manifested, with a blood pressure of 60/40, pallor, anuria, tachycardia and obnubilation, requiring fluid resuscitation and intubation for respiratory insufficiency. A nasogastric tube was positioned immediately after intubation. Physical examination evidenced hypertympanism with epigastric tenderness. No significant cardiac abnormalities were found on cardiogram and cardiac ultrasound (US); abdominal X-rays and US did not clarify the diagnosis while the computed tomography (CT) scan  evidenced a great gastric and intestinal dilatation, with diaphragm elevation and intraperitoneal free liquid and air . Acute rTill date, more than 60 cases of gastric acute ischemia and perforation have been reported since its first description by Duplay in 1833, ascribing various causes, but this is the first report in the aftermath of orthopedic surgery. Gastric dilatation might be very important, so as to provoke abdominal compartment syndrome. In the c5We acknowledge that shock in the post-operative period might have many causes, one of which could be idiopathic gastric necrosis, especially in the older patient complaining of abdominal distension and pain, and prompt diagnosis and aggressive treatment are mandatory, but sometimes not sufficient to achieve success.


Cavernous hemangiomas of the orbit are benign vascular malformations, commonly encountered in adults. Although they are infrequent in pediatric population their diagnosis and course are of a great significance, mainly because they can cause visual disturbances such as amblyopia that can ensue, and secondarily due to their cosmetic and psychological effect. Special attention is required in follow up and treatment. Additionally, a systemic evaluation is necessary in order to discover asymptomatic lesions elsewhere in the body carrying a risk of complications.The authors describe the clinical course, diagnosis, therapeutic approach and prognosis of an infant with an orbital cavernous hemangioma accompanying intracranial lesions. A female infant 18 months of age, presented with a mass in the left upper eyelid, causing blepharoptosis. Preoperative magnetic resonance imaging and angiography of the brain and the orbits showed a hemangioma of the left upper eyelid and intracranial lesions to the left temporal fossa and the pons. At the age of 2 years and 8 months she was admitted again due to severe eyelid swelling, intense strong pain, exophthalmos and collateral ophthalmoplegia. Two operations were performed to remove the orbit mass. Histological examination, showed characteristics of cavernous hemangioma.The atypical presentation of cavernous orbital hemangioma with early infantile onset, merits attention. Cavernous hemangiomas are uncommon vascular malformations located in the brain . They arWe describe a case of a female infant with an orbital cavernous hemangioma and accompanying intracranial lesions, which was implicated with serious thrombosis, optic nerve atrophy, severe visual loss and strabismus.A Greek female infant, 18 months of age, was referred to the pediatric clinic for evaluation of a mass in the left upper eyelid.According to the patient's perinatal and medical history, she was born after a 36-weeks twin pregnancy, following in-vitro fertilization (IVF), with a birth weight of 2.200 g, small-for-gestational age. There was no positive family history of hemangiomas. There were no indications of deficiency in her developmental status. Also there was no history of trauma, seizures or focal neurological deficits.The physical examination revealed no pathologic findings with the exception of a soft and painless mass in the left upper eyelid, causing incomplete closure of the eyelid and only mild irregular discoloration of the lid skin. An increase in tumor volume and depth of bluish hue when the child cried were noticeable. Also there was a small subcutaneous mass in the upper lip and a café-au-lait spot with smooth margins on the right leg. No other skin spots were observed.The ophthalmological evaluation showed that both eyes were equal in size and eye motility was normal. No strabismus was recognized. Direct ophthalmoscopy showed no abnormality in either anterior segment. The fundus examination in both eyes was normal. Skiascopic examination revealed no particular refractive errors, except for a small degree of hyperopia.Subsequent examination of the skin and magnetic resonance imaging (MRI) of the brain and the orbits (with T1 and T2 weighted sequences) and magnetic resonance angiography (MRA) were performed. A lesion resembling cavernous hemangioma was found in the superior part of the left orbit  and 10/10 (right eye) in the Snellen optotype, total atrophy of the left optic nerve and small angle divergent strabismus.Cavernous hemangiomas are non-infiltrative, low-flow hamartomas, usually observed in adults and thought to be benign . In the In our patient there was a combination of early age of onset (infancy), cavernous hemangiomas in orbit and lip, cerebral cavernoma with lesions in left temporal fossa, left cavernous sinus and pons, and asymptomatic clinical profile in relation to cerebral angiomas. The examinations for hemangiomas elsewhere in the body and the familial history of clinical noticeable hemangiomas were negative.The pathogenesis of orbital cavernous angiomas is uncertain . GardnerThe natural history of orbital cavernous hemangiomas may be quite variable. It may be that of benign tumours causing progressive clinical manifestations from mass effect due to their slow growth or sometimes they may not grow after a certain age or remain asymptomatic ,5,11. ThChildren with an infantile hemangioma in the eyelid and orbit are at risk of a variety of ocular problems including functional amblyopia, strabismus, proptosis and optic nerve compression ,14. The The diagnosis and follow up imaging of cavernous angiomas  are best provided by MRI . In our Various methods have been used to treat infantile hemangiomas of the eyelid and orbit. According to Herter et al. , operatiThe choice of management in cases of cerebral cavernous angiomas must take into account both the possible natural evolution of the lesions and the risk of surgery. Therapeutic strategies for cerebral cavernomas take into account age, sex, location of the lesions, the efficacy of medical treatment for patient's secondary epilepsy and risk factors for severe potentially life-threatening hemorrhage . In chilCavernous hemangiomas of the orbit are benign vascular malformations, commonly encountered in adults. Although they are infrequent in pediatric population their diagnosis and course are of a great significance, mainly because they can cause visual disturbances such as amblyopia that can ensue, and secondarily due to their cosmetic and psychological effect. Special attention is required in follow up and treatment. Additionally, a systemic evaluation is necessary in order to discover asymptomatic lesions elsewhere in the body carrying a risk of complications.IVF: in-vitro fertilization; MRA: magnetic resonance angiography; MRI: magnetic resonance imaging.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."EE was a major contributor in writing the paper; ET performed the histologic examination; IA analysed and interpreted the visual data of the patient; SG contributed to final revision of the paper; MA provided the final approval for the version to be published, DP provided the histological images and AS concepted and designed the study".


Gallstone ileus of the colon is an exceedingly rare cause of large-bowel obstruction. It is usually the result of fistula formation between the gallbladder and large bowel facilitating entry of the stone into gastrointestinal tract. Contrast enhanced abdominal computed tomography is an important diagnostic aid. Surgical management is the treatment of choice to prevent the disastrous complications of large-bowel obstruction. We describe the case of a 92-year-old man who presented with symptoms and signs of large-bowel obstruction. Radiological investigation showed a large gallstone impacted in the sigmoid colon. Open enterolithotomy was undertaken relieving the obstruction and the patient made a full recovery. Colonic gallstone ileus is an exceedingly rare cause of mechanical large-bowel obstruction. It occurs most commonly due to the passage of a solitary large stone or several smaller stones through a cholecystocolonic fistula into the colon. It is a disease of high morbidity and mortality due to the diagnostic challenge, late presentation, advanced patient age, and comorbid states. We report a case of gallstone ileus of the sigmoid colon and review the available literature.A 92-year-old male was admitted to hospital with a 3-day history of intermittent colicky lower abdominal pain associated with absolute constipation and abdominal distension. Clinical examination revealed a distended abdomen with Left iliac fossa tenderness but no overt peritonism. He had an episode of acute cholecystitis in the previous year. A CT scan at that time demonstrated a 3.8 cm stone in the gallbladder. No subsequent intervention was planned due to his cardiorespiratory comorbidities.9/L. Liver function tests and serum amylase were entirely normal. Initial abdominal X-rays showed gaseous distension of the large bowel with no evidence of pneumobilia. A subsequent contrast-enhanced CT scan of the abdomen demonstrated a 3.8 cm opacity impacted in the midsigmoid colon  of the colon is an extremely rare cause of large-bowel obstruction. It has been shown to account for 2% to 8% of all cases of GSI , 2. It iThe mechanism of obstruction usually results from a large gallstone greater than 2 cm in diameter entering the colon through a cholecystocolonic fistula. The fistulous connection usually forms after a preceding episode acute cholecystitis leads to inflammation and adhesions forming between the gallbladder and colon, usually at the hepatic flexure. The gallstone impacts distally and causes a mechanical obstruction. The point of impaction is usually a pathological narrowing of the colon which may be a result of diverticular disease , 4 or prThe typical presentation is that of large-bowel obstruction with abdominal pain and distension being the main features. However, rarer presentations such as diarrhoea or ascending cholangitis can occur . As in GAbout 7% of GSI have been shown to settle with conservative management. However, in the majority of cases operative management is required. In the main part operative intervention for colonic GSI is the same as small bowel GSI. The definitive management involves enterolithotomy, cholecystectomy, and fistula closure at one operation . This alIn conclusion GSI of the colon is a rare but important cause of large-bowel obstruction. The diagnosis may be very difficult given the lack of distinguishing clinical features but should be considered if there has been a history of gallstone disease. If plain abdominal films do not reveal the diagnosis, computed tomography will allow visualisation of the fistula and level of obstruction. Treatment depends on the fitness of the patient but should ideally involve relief of the obstruction, cholecystectomy, and repair of the fistula.


A rare case of synchronous angiomyolipoma and oncocytoma in the same kidney of a 70 year old man is presented. A left renal mass was found incidentally by ultrasound. Computerized tomography and magnetic resonance imaging revealed a 1,3 cm mass in the mid-portion of the left kidney, whereas on the lower pole of the same kidney, a 3,3 cm mass was also revealed, consistent with angiomyolipoma. A working diagnosis of renal cell carcinoma was made. A radical nephrectomy was performed. Microscopically, the tumor of the lower pole was found to be an angiomyolipoma, whereas the mid-portion tumor was an oncocytoma. Until now, only 16 cases of unilateral simultaneous presence of renal angiomyolipoma and oncocytoma have been reported. Of these cases, all except one were female and three were associated with the tuberous sclerosis complex. It is well worth remarking, that renal oncocytoma overlap with other renal neoplasms, therefore nephrectomy remains the treatment of choice.Renal angiomyolipoma and oncocytoma are uncommon neoplasms and their simultaneous presence in the same kidney is rare. Only 16 cases have been reported until now in the literature. The purpose of this paper is to present an additional case without evidence of tuberous sclerosis. Renal angiomyolipoma and oncocytoma represent uncommon neoplasms and their simultaneous presence in the same kidney is rather rare. To the best of our knowledge, only 16 cases have been reported in the literature . OncocytWe present a case report of renal angiomyolipoma and oncocytoma without evidence of tuberous sclerosis.The patient was a 70 year old asymptomatic male with a history of cholelithiasis in whom a solid 1,3 cm medial left renal mass was diagnosed incidentally by ultrasound. There was no significant past medical history, specifically seizures or mental retardation. On physical examination, head, neck, heart and lungs were normal. Neurological testing gave normal results. Blood renal tests were normal. Computed tomography (CT) showed a 1,3 cm well- defined, exophytic solid mass without cystic characteristics in the mid- portion of the left kidney, whereas on the lower pole of the same kidney, a 3,3 cm mass that contained a significant amount of fat was revealed. This mass was radiographically compatible to an angiomyolipoma. Invasion or infiltration into the perinephric fat, collecting system of vessels or regional lymphadenopathy and metastases were not encountered. On subsequent magnetic resonance imaging (MRI), the two renal masses were confirmed. In the mid- portion of the left kidney a 1,3 cm well- defined, homogenous mass was described, which appeared hypointense relatively to the renal cortex on T1- weighted images and isointense on T2- weighted images (Figure On gross examination, at the lower pole of the kidney an ovoid, yellowish 2,8 × 1,8 × 1,3 cm lesion which seemed to invade in the perinephric fat was found. In addition, there was a firm, well- encapsulated, brownish 1,7 × 1,2 × 1,0 cm tumor in the mid- portion of the kidney. Careful sectioning of the kidney did not reveal any additional lesion.In the mid- portion of the kidney, the neoplasm exhibited a uniform population of plump cells arranged in alveolar- type nests and trabeculae with a granular, acidophilic cytoplasm. The morphological features were those of an oncocytoma Figure . The lowImmunohistochemistry carried out on the sections of the oncocytoma showed negativity for antibody for vimentin and a weak positivity for antibody for CK 7.The final confirmed diagnoses included an angiomyolipoma of the lower pole and oncocytoma of the mid- part of the left kidney.Renal oncocytomas, first described by Zippel in 1942 . HoweverRenal angiomyolipomas are uncommon, benign tumor- like formations, consisting of mature adipose tissue, smooth muscle cells and thick- walled blood vessels and account for less than 1% of surgically removed tumors. The perivascular epithelioid cell is thought to be the cell of origin of angiomyolipomas .A common immunophenotype of these tumors is the consistent expression of melanoma- associated antigens, particularly HMB- 45. Angiomyolipomas are commonly associated with tuberous sclerosis, which is an autosomal dominant disorder characterized by seizures, mental retardation, skin lesions and hamartomatous lesions in many organs . Rarely,The diagnosis or renal angiomyolipoma is usually straight forward due to its characteristic sonographic appearance and its typical CT morphology. Ultrasonographically, most angiomyolipomas are hyperechogenic lesions resembling the echogenicity of the renal sinus fat; the fat component accounts for the negative attenuation values on CT scans and the hyperintensity on T1- weighted MR images . HoweverAs the risk of haemorrhage in angiomyolipomas less than 4 cm in diameter is minor, the surgical treatment in asymptomatic cases is generally reserved for those tumors that are greater than 4 cm. Surgical excision, including radical nephrectomy, may remain the treatment of choice; nonetheless, in the appropriate setting, partial nephrectomy, enucleation, or wedge resection can be alternative options .Although concurrence of renal cell carcinoma and oncocytoma within the same kidney is well recognized , the simThe size of both tumors varied greatly and in some patients the presence of two tumors was not detected preoperatively . In patiIn conclusion, we present an additional case of an oncocytoma associated with an angiomyolipoma, which is remarkable due to the fact that it was detected in a male patient. With respect to preoperative diagnosis, these neoplasms remain a challenge for the surgeon because they mimic renal cell carcinoma in the majority of cases. Our case did not occur in the setting of tuberous sclerosis. Of the 16 cases reported in the literature, only three cases associated with tuberous sclerosis complex have been documented. However, if angiomyolipomas are found incidentally in nephrectomy specimens along with other tumors, tuberous sclerosis should be excluded retrospectively.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 journal's Editor-in Chief.The authors declare that they have no competing interests.TT - responsible for critical revision of scientific content; AY - drafted the manuscript; MK - contributed substantially to manuscript conception and design; GA - assisted in the preparation of the manuscript; EKV - performed histopathological and immunohistochemical analyses and contributed to the pathology content; ND - have made substantial contributions to manuscript conception and acquisition of data; IV - the surgeon, approved the final version of the manuscript for publication; All authors read and approved the final version of the manuscript.


We report a rare case of gastrointestinal perforation following dacarbazine infusion for metastatic melanoma. The condition is attributed to a responding malignant melanoma in the gastrointestinal tract.A 52-year-old Caucasian man presented with abdominal pain and distension, malaise, night sweats, dysphagia and early satiety. A computed tomography scan showed massive ascites, lymphadenopathy and liver lesions suspect for metastases. An upper gastrointestinal endoscopy was performed and revealed multiple dark lesions of 5 mm to 10 mm in his stomach and duodenum.2 every three weeks and he was discharged one day after the first dose. On the sixth day he was readmitted with severe abdominal pain. A chest X-ray showed the presence of free intraperitoneal air that was consistent with gastrointestinal perforation. His lactate dehydrogenase level had fallen from 6969U/L to 1827U/L, supporting the conclusion that the response of gastrointestinal metastases to dacarbazine had resulted in the perforation of the patient's bowel wall. A laparotomy was discussed with the patient and his family but he decided to go home with symptomatic treatment. He died 11 days later.When his skin was re-examined, an irregular pigmented lesion over the left clavicle measuring 15 mm × 8 mm with partial depigmentation was found. Histological examination of a duodenal lesion was consistent with a diagnosis of metastatic melanoma. The patient deteriorated and his level of lactate dehydrogenase rapidly increased. The patient was started on systemic treatment with dacarbazine 800 mg/mMelanoma can originate in, as well as metastasize to, the gastrointestinal tract. Gastrointestinal perforations due to responding tumors are a well-known complication of systemic treatment of gastrointestinal lymphomas. However, as the response rate of metastatic melanoma to dacarbazine is only 10% to 20%, and responses are usually only partial, perforation due to treatment response in metastatic melanoma is rare.Medical oncologists should be aware of the risk of bowel perforation after starting cytotoxic chemotherapy on patients with gastrointestinal metastases. The incidence of melanoma is increasing worldwide. In The Netherlands 19.4 cases per 100,000 persons were diagnosed in 2005. For the treatment of widespread metastatic diseases, single agent dacarbazine (DTIC) chemotherapy is still the standard of care. Combination regimens with other cytotoxic agents, cytokines and tyrosine kinase inhibitors do not result in a survival benefit .Treatment with high-dose interleukin-2 (IL-2) has induced a durable complete remission in a minority of patients with metastatic melanoma, but this treatment is associated with severe toxicity and it is not widely available .Treatment with dacarbazine results in response rates of 10% to 20%. Responses are usually partial and generally last for only four to six months, although prolonged remissions are occasionally seen. A survival benefit of treatment with dacarbazine over best supportive care has not been proven definitively . CompareIn November 2007, a 52-year-old Caucasian man of Dutch origin presented with upper abdominal pain, anorexia, nausea, dyspnea on exertion, and a general decline in condition for the past few weeks. His medical history revealed a subarachnoid hemorrhage eight years prior to presentation, from which he recovered completely, and essential hypertension that was well-controlled.On physical examination, a lymphadenopathy in the patient's left axilla and neck was found, in combination with a distended abdomen with shifting dullness and an enlarged irregular liver. Laboratory tests showed a slight leukocytosis and thrombocytosis, normal haemoglobin, creatinine and electrolytes levels, a lactate dehydrogenase (LDH) level of 373IU/L that increased to 6969IU/L in eight days, normal alkaline phosphate, normal transaminases and bilirubins. A computed tomography (CT) scan of his chest and abdomen revealed lymphadenopathy in the mediastinum, lung hili and left axilla, as well as ascites with an omental cake and multiple lesions in an enlarged liver. Ascitic fluid was sent to pathology, and a gastroduodenoscopy was also performed. Multiple dark gastric and duodenal lesions were found, which were suspect for metastatic melanoma or Kaposi's sarcoma Figure . A biopsSubsequently, on re-examination of the skin, a 1.5 cm irregular lesion over the left clavicle was found. The lesion was partially pigmented and partially depigmented, which was consistent with a melanoma in regression Figure .2) dacarbazine intravenously. He was discharged the next day.Two days after an uncomplicated paracentesis, our patient received 1560 mg (800 mg/mFive days after chemotherapy he was readmitted with severe abdominal pain. On physical examination, an acute abdomen was found. A chest X-ray showed the presence of free intraperitoneal air Figure  and a clMelanoma can originate anywhere in the digestive tract, but the majority of digestive tract lesions are metastatic in nature. The primary lesion can be discrete, especially when, as in this case, regression has occurred. In patients with end stage melanoma, gastric metastases and small bowel metastases are quite common. In two large autopsy series, gastric metastases were found in about 20% of the patients studied, and small bowel metastases in 35.6% and 58% of the patients .Differentiation between a primary gastrointestinal tract lesion and metastasis from an occult cutaneous melanoma can be difficult in cases with solitary gastrointestinal localization. However, due to the lack of convincingly effective adjuvant regimens for melanoma, the clinical consequences are marginal.Surgery for melanoma lesions that are metastatic to the gastrointestinal tract is quite effective for controlling symptoms but it rarely leads to long-term survival ,9.The systemic treatment of metastatic disease has been met with limited success. Over the decades, single-agent treatment with dacarbazin has remained the standard of care. An incentive for using this drug even in the end stage of the disease is its limited toxicity, with easily controllable nausea as its main clinical side effect. The generally mild hematological toxicity of dacarbazine compares favorably to that of drugs used in other advanced stages of cancer . AlthougPerforation of a gastrointestinal tumor as a result of chemotherapy is rare, as remissions are usually partial and occur gradually. An exception in this regard is intra-abdominal lymphoma. This tumor is highly sensitive to cytotoxic therapy and responds with rapid necrosis. Abdominal non-Hodgkin's lymphoma in children, with otherwise favorable prognosis, is associated with a very poor outcome if gastrointestinal perforation occurs ,12.We could only identify one other case of fatal gastrointestinal perforation in a patient with metastatic melanoma during treatment with dacarbazine, but bowel perforation was not attributed to chemotherapy by the authors . In our Chemotherapy in end stage melanoma is aimed at a rarely achievable goal of prolonged remission in the context of limited toxicity. In the presence of extensive digestive tract involvement, a remission could lead to perforation, resulting in excess toxicity and probably death. When perforation is recognized as a possible threat, a prolonged administration of the drug in its oral formulation might be advisable. This does not seem to compromise treatment efficacy .Written informed consent was obtained from the partner of 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.SO treated the patient with chemotherapy and drafted the manuscript. FP performed the endoscopy and made the diagnosis of metastatic melanoma. GH conceived of the manuscript and participated in its design. NM supervised this patients treatment and drafted the manuscript. All authors read and approved the final manuscript.


Solitary osteochondromas, which are the most common benign bone tumors of long bones, are rarely found in the vertebral column. A 16-year-old female patient presented with a hard palpable mass at lower lumbar region like a congenital deformity. Plain radiography illustrated a well-defined solid mass arising from the posterior elements of the L3 and ruled out any congenital anomalies. A computed tomography scan further determined a mass that arose from the spinous process of L3. The tumor was excised en bloc through a posterior approach and histopathological examination verified the diagnosis of osteocondroma.Osteochondromas are rarely found in the spine, when present in the spine, however, have a predilection for cervical or upper thoracic region arising usually from lamina of vertebrae and are rare in lumbosacral region and very rare at spinous process of the vertebrae.We present a case of osteochondroma locates in lumbar region and spinous process of vertebrae with unusual presentation and was considered clinically as congenital lumbar kyphosis. Osteochondroma is the most common primary benign bone tumor. However, this tumor rarely involves the spine and even more rarely involves the lower lumbar region . They rePresentation of these tumors in spine is usually circumscribed to the cervical and upper thoracic regions with few tumors presenting in the lumbosacral vertebrae -5.In this report, the osteochondroma was located at the spinous process of L3.A 16-year-old girl from Khorasan state of IRAN was referred to our spine clinic as a congenital lumbar kyphosis with a palpable non tender mass in the midline of lower lumbar region which was noticed by her parents two years prior to presentation to our hospital  further determined a mass that arose from the spinous process of L3 Figure .The tumor was excised en bloc through a posterior approach Figure . HistopaOsteochonroma is more frequent in male and patients are usually under the age of 20 -5.From 1-4% of osteochondromas involve the spine and are commonly included in the posterior elements of the vertebrae. Osteochondroma has a predilection for the cervical and upper thoracic regions and, as it is in our case, rarely involve Lumbosacral region of the spine .As this case, a simple painless mass may be the only presenting symptom , but if In all of the reported cases, the localization of osteocondroms are the posterior arch of the vertebrae more commonly the lamina and involvement of the spinous process specially in lumbar region as this case is very rare and has not been reported .Plain radiographs may be nondiagnostic in certain cases, and computed tomography scan is necessary for evaluating the origin, size and characteristic of the tumor ,5. In caOsteochondromas that consist the largest portion of benign bone tumors, usually occur in the long bones and are rarely found in the spine when present in the spine, however, have a predilection for cervical or upper thoracic region arising usually from lamina of vertebrae. Therefore we presented an osteochondroma locate in Lumbar region and spinous process of vertebrae with unusual presentation was considered clinically as Lumbar kyphosis.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 author declares that he has no competing interests.


Venous malformations of the jugular veins are rare findings. Aneurysms and phlebectasias are the lesions most often reported. We report on an adult patient with an abruptly appearing large tumorous mass on the left side of the neck identified as a jugular vein aneurysm. Upon clinical examination with ultrasound, a lateral neck cyst was primarily suspected. Surgery revealed a saccular aneurysm in intimate connection with the internal jugular vein. Histology showed an organized hematoma inside the aneurysmal sac, which had a focally thinned muscular layer. The terminology and the treatment guidelines of venous dilatation lesions are discussed. For phlebectasias, conservative treatment is usually recommended, whereas for saccular aneurysms, surgical resection is the treatment of choice. While an exact classification based on etiology and pathophysiology is not possible, a more uniform taxonomy would clarify the guidelines for different therapeutic modalities for venous dilatation lesions. The most common lesions of venous malformations in thehead and neck region are internal jugular vein aneurysms and phlebectasias. Although these terms are frequently used as synonyms, they pertain to twodivergent conditions, likely of different etiology and pathophysiology. Macroscopically, aneurysms are described as saccular , 2 andpWhile phlebectasia in the neck region is considered tobe congenital in origin or to arise from a primary congenital weakness withinthe muscular layer of the venous wall –6, venouWe report on an adult patient with a large tumorous mass on the leftside of the neck identified as a jugular aneurysm.Three days prior to admission to the University Central Hospital in Helsinki, a 71-year-old woman noticed a rapidly growing lump on the left side of her neck. Physicalexamination revealed a mass underneath the anterior border of thesternocleidomastoid muscle in the supraomohyoid region of the neck. The tumorwas slightly tender and warm upon palpation, but the patient had no infectioussymptoms. There was no known trauma to this area. Ear, nose, and throat statuswas normal. C-reactive protein and blood white cell count were normal. Pastmedical history included elevated blood pressure and bronchial asthma withmedication. No previous operations or arterial/venous catheterizations had beenperformed.Ultrasound revealed a smooth, rounded cysticconfiguration (diameter 3.5 × 1.9 cm) filled with a thick fluid. In differentialdiagnosis, either an infected cyst or a necrotized lymph node was suspected.The patient was treated with intravenouskefuroxime and metronidazole for one day and thereafter with per oralkefalexine and metronidazole for ten days because of a suspected infectedlateral neck cyst. During a six week follow-up, the mass had not regressed. Amalignant involvement could not be excluded and the surgery was performed.Upon surgery under generalanesthesia, a saccular venous aneurysm was detected. A short, wide-basedconnection was present between the venous wall of the jugular vein and thecyst-like aneurysm, which had a smooth and rounded contour. The pathologicalsegment of the jugular vein was resected and the free ends of the vesselligated . The patPathological examination showeda mass of 3.5 × 2.0 × 1.8 cm in diameter with a macroscopically normal venouswall encapsulating an organizing hematoma inside the lumen . FibrotiNo established classification ofvenous malformations can be found in vascular pathology handbooks, and theterms used for venous dilatation lesions vary. Two of these terms—aneurysmand phlebectasia—can be differentiated based on macroscopicclinicopathological findings of the lesions . The paOne of the first descriptions of an internal jugularphlebectasia by Gerwig Jr.  defines Opinions on the etiology of aneurysms and phlebectasias vary. Jugularfusiform phlebectasia is thought to be a childhood disease , 10–12. Pathological changes of vein walls varyhistologically , 5, 8. IInconsistency in the terms used is reflected in thenonuniform recommendations for treatment strategies of venous dilation lesions. The outcome of venous dilatation lesions is dependent on their anatomicallocation , 17. ThJugular venous aneurysms may beclinically confused with branchial cysts, laryngoceles, hemangiomas, andlymphangiomas, or other cystic lesions, such as carcinoma arising in primarylateral neck cyst or metastatic squamous cell carcinoma. Ultrasonography canassist in making the correct diagnosis but was not useful in this case. Dopplersonography, computed tomography, or angiographies are helpful investigations toincrease accuracy in diagnosis of acervical mass . GadolinThe etiopathology of the aneurysm here remains totally open, itsmanifestation appeared to be spontaneous and acquired, and histologicalexamination yielded no special pathological clues of its etiology.The rare presentation and inconsistent terminology ofvenous dilatation lesions can cause confusion in choosing an optimal treatment. The treatment options depend on the location and the form of the lesion. Forjugular phlebectasias, conservative treatment is usually recommended, whereasfor saccular aneurysms, which often present with thrombosis, surgical resectionis the treatment of choice. Although an exact taxonomy based on etiology orpathophysiology is not yet possible, a more uniform nomenclature would clarifythe guidelines for different therapeutic modalities for venous dilatationlesions.


Renal oncocytomas are benign neoplasms derived from cells of the distal renal tubule, and comprise 5% to 7% of primary renal neoplasms. Oncocytomas are mostly asymptomatic, and the majority of tumors are discovered incidentally. In this case report, we present a case of a patient with a giant oncocytoma arising from her left kidney.We describe a 25-year-old Turkish woman who was admitted to our hospital with abdominal pain and a 3-year palpable abdominal mass, which was found present since her second pregnancy. Examination revealed a 15 × 20-cm mass in her abdominal cavity. Computed tomography revealed a mass with regular outlines, measuring 18 × 11 × 12 cm, associated with the left kidney, and causing marked hydroureteronephrosis. We excised the mass and performed a left nephrectomy on our patient. The immunohistopathology of the mass was consistent with renal oncocytoma. No local or distant metastasis was seen at 6 months postoperatively.To the best of our knowledge, this is the second largest renal oncocytoma described in the English language literature. This is also the first reported giant oncocytoma that presented during pregnancy. Oncocytomas are benign, nonurothelial, epithelial tumors that constitute 5% to 7% of primary renal neoplasms. Classically, an oncocytoma is a solid mass that develops in the renal parenchyma and has a central fibrous scar ,2. Most A 25-year-old Turkish woman was admitted to our hospital in October 2008. She was suffering from abdominal pain, weakness, anorexia, and a 7-kg to 8-kg weight loss. It was discovered that she had a palpable abdominal mass three years prior to presentation. Physical examination revealed a 15 × 20-cm mass with regular contours in the her periumbilical region , 36 IU/L; and alanine aminotransferase (ALT), 41 IU/L. On abdominal X-rays, all of her intestines were seen shifted to the right. An intravenous pyleogram (IVP) showed a mass associated with her left kidney. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) revealed an 18 × 11 × 12-cm mass located in the middle of her left kidney and extending inferiorly, with diffuse contrast enhancement -stained sections at low power showed a neoplasm with a pseudocapsule and a tubulocystic pattern divided by thin fibrous septa. At higher magnification, the cells appeared to be round to polygonal with abundant granular eosinophilic cytoplasm, a regular round nucleus, and a central nucleolus. There were no foci of clear cell changes, necrosis, or mitotic figures.Renal oncocytoma was initially described in 1942 by Zippel. Oncocytomas are benign tumors of the kidney that are usually diagnosed postoperatively, after an erroneous diagnosis of renal cell carcinoma (RCC).et al. [et al. [et al. [et al. [A summary of the studies about giant oncocytomas published in the English literature is presented as Additional File et al.  have rep [et al.  reported [et al.  reported [et al.  reportedRenal oncocytomas are uncommon and consist of a pure population of oncocytes, which are large well-differentiated neoplastic cells with intensely eosinophilic granular cytoplasm due to the large number of mitochondria -10. LikeThe most useful marker for the differentiation of renal tumors are vimentin , CK7 , RCC marker and CD10 , and Hale's colloidal iron staining with diffuse reticular pattern and perinuclear halo  -16. The Oncocytomas can appear on CT and ultrasound as a central scar located within a homogeneous, well-circumscribed solid mass . HoweverRenal oncocytomas are almost invariably benign and no cases of metastasis have been reported. Even when very large, they are generally well encapsulated and are rarely invasive or associated with metastases . In our Renal oncocytoma has a benign clinical course with excellent long-term outcomes. Surgical treatment of renal tumors is still unclear. Although radical surgery has been employed in the past as principal therapy, more precise preoperative and peri-operative diagnosis should allow more frequent use of conservative surgery, such as partial nephrectomy or tumor excision. Conservative management such as organ-sparing surgery or partial nephrectomy should be reserved for bilateral tumors or where the tumor occurs in a solitary kidney ,19.Although there have been many improvements in the histopathological diagnosis of giant oncocytoma, more case reports and studies are needed to understand the behavior, prognosis and treatment of this tumor.RCC: renal cell carcinoma; EpCAM: epithelial cell adhesion molecule; CT: computed tomography; IVP: intravenous pyleogram; MRI: magnetic resonance imaging.Written informed consent was obtained from our patient for publication of this case report and any accompanying image. 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.SA and BC performed the surgical procedure, contributed in writing the article and review of the literature, and undertook a comprehensive literature search. SA contributed in the design of the study and preparation of the manuscript. ARS provided the histopathological information and AS provided the radiological information. All authors read and approved the final manuscriptTable S1. The summary of the studies about giant oncocytoma published in the English language literature.Click here for file