Failure to thrive and hematochezia in children may be alarm signs warranting endoscopy. In contrast, vascular malformations of the small intestine are uncommon in this age group. We report on a female toddler in whom various imaging techniques revealed an unusually large segmental vascular malformation of the ileum as the cause of the child’s main clinical symptoms.A 19 months old girl presented with severe anemia (Hb 3 mmol/l), failure to thrive and chronic diarrhea. Diagnostics for intestinal blood loss and pathogens were negative. The child had duodenoscopy, also for histological diagnosis of celiac disease, with negative results. A dietary protocol was suggestive for inadequate iron intake and she was supplemented. After symptomless four-months the child presented again, now with mild abdominal pain and, for the first time, hematochezia. An orienting abdominal ultrasound (US) study showed a suspicious tumorous bowel condition. A subsequent detailed abdominal US supplemented by a saline enema during investigation  revealed a large circumferential cystiform vascular mass of the ileum causing segmental ileal obstruction.Complementing preoperative abdominal hydro-MRI, planned based on the findings of the US study, confirmed the suspected vascular malformation of the ileum and exquisitely outlined the extent, location and anatomy.The patient was successfully operated laparoscopically, the affected ileum segment with the mass was completely removed as proven by histology, and the child recovered well.The huge segmental vascular malformation of the distal ileum described here is an extreme rarity in young children. Although the reported child’s presenting symptoms malabsorption and malnutrition could have been responsible for its severe anemia, this was obviously caused by blood losses from the ileal vascular malformation. It was due to incipient abdominal pain rather than hematochezia that abdominal US was performed and proved crucial for correctly diagnosing this rare malformation. Even in this extensive case detailed imaging work-up including adapted MRI added all information necessary for minimal invasive laparoscopic en bloc resection. Both failure to thrive with chronic diarrhea as well as hematochezia are clinical alarm signs in children potentially warranting endoscopy. And while hematochezia has a great variety of causes, vascular malformations of the small intestine - as one of them - are uncommon in this age group. In particular, there are only very few reports on this disease in children younger than two years-4.Although infants and children suffering from intestinal vascular malformations may present with symptoms such as abdominal pain, gastrointestinal bleeding, diarrhea or vomiting, some cases are asymptomatic and thus remain hidden from the diagnostician’s eye for quite some time. On the other hand, very few cases may lay a false trail for the clinician because of unaccompanied anemia. Early iThis is demonstrated by our report of a 19 months old female toddler who presented first with failure to thrive and signs of malabsorption and much later with abdominal pain and hematochezia as the clinical symptoms leading to the diagnosis of an unusual large, segmental, circumferential, low-flow vascular malformation of the ileum.A 19 months old girl presented with severe anemia (Hb 3 mmol/l). Her parents had noticed her failure to thrive and chronic diarrhea. There was no visible blood in her stools, and markers for intestinal blood loss as well as assays for intestinal pathogens were negative. Also because of inconclusive celiac serology the child underwent duodenoscopy with normal macroscopic and histologic findings. Since a detailed dietary protocol kept by her parents pointed towards (iron) malnutrition, she was adequately supplemented.After a symptomless four-month interval the child presented again. It was only now that her parents had noted hematochezia and also suspected occasional abdominal pain. Physical examination showed no abnormalities, in particular no abdominal mass on palpation. Laboratory tests were normal apart from mild neutropenia and slighty elevated d-dimer values. However, guaiac fecal occult blood test  was positive.Abdominal sonography was performed and revealed a 10 to 15 cm long circular lobulated infiltration of the ileum wall, the aboral end almost extending to Bauhin’s valve  was performed and revealed a purple bacciform vascular malformation of the mid-ileum completely encircling the affected intestinal segment  provided the definitive diagnosis of a vascular malformation (due to late and diffuse contrast enhancement) of the ileum, which was confirmed histologically. It furthermore helped to outline preoperatively relevant information such as location, length, anatomical details and the absence of large feeding or draining vessels which might have caused surgical complications.The decision to perform surgery was made because the girl suffered from gastrointestinal bleeding (hematochezia), which required prompt diagnosis and therapy. Although the diagnostic workup immediately revealed a vascular anomaly of the distal ileum, the exact differentiation between a hemangioma and a complex vascular malformation could not be made. Thus medical treatment with propranolol or corticosteroid did not seem justified. Furthermore the MRI could not delineate the exact position or its extent to initiate sclerotherapy or embolization which would also have endangered the vitality of the affected bowel segment. In contrast, laparoscopy identified the affected segment of small bowel as well as the macroscopic borders of the lesion and thus lead to definite surgical therapy. After delivery through a minimally extended umbilical port sire the lesion could be resected completely, the pathology report revealed that the margins of resection were free of disease. Since then, the postoperative course of more than two years has been uneventful.In conclusion, this case shows that common symptoms may be present even in an extremely rare pathology. Furthermore, it demonstrates that even early invasive diagnostics may still require additional less- or non-invasive methods to enable the correct diagnosis; maybe an initial US study can be considered compulsory before going for more invasive studies.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.The authors declare that they have no competing interests.All authors 1) have made substantial contributions to conception and design, or acquisition of data, or analysis and interpretation of data 2) have been involved in drafting the manuscript or revising it critically for important intellectual content and 3) have given final approval of the version to be published.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2431/14/55/prepub


This classification has accepted management implications due to different risks of haemorrhage and malignant transformation of the four subtypes. Imaging guided biopsy and/or surgical resection very important in obtaining definitive characterization; nevertheless, MRI with intra-extravascular and hepatobiliary  agents, is an important tool not only in differential subtypes definition but even in surveillance with early identification of complications and discovery of some signs of HCA malignant degeneration. Inflammation, abnormal rich vascularisation, peliotic areas, and abundant fatty infiltration are pathologic findings differently present in the HCA subtypes and they may be detected by multiparametric MRI approach. Lesion enlargement and heterogeneity of signal intensity and of contrast enhancement are signs to be considered in malignant transformation. The purpose of this paper is to present the state of the art of MRI in the diagnosis of HCA and subtype characterization, with particular regard to morphologic and functional information available with dual phase contrast agents, and to discuss differential diagnosis with the most common benign and malignant lesions mimicking HCAs.Hepatocellular adenomas (HCAs) are currently categorized into distinct genetic and pathologic subtypes as follows: inflammatory hepatocellular adenoma, hepatocyte-nuclear-factor-1-alpha (HNF-1 Hepatocellular adenoma (HCA) is a rare benign tumour  that is mainly found in women of child-bearing age . There is evidence that HCA is strongly related to current and recent  oral contraceptives (OC) use. Recent, low-dose OCs appear less strongly, at all, related to HCA –6. Small HCA is generally asymptomatic. Right upper abdominal quadrant fullness or discomfort is present in 40% of cases due to mass effect. Typical clinical manifestation is spontaneous rupture or haemorrhage leading to acute abdominal pain with possible progression to hypotension and even death. HCA rarely undergoes malignant transformation to hepatocellular carcinoma (HCC). Laboratory tests for liver function are usually normal. Because of the different therapeutic options HCA must be distinguished from other hypervascular lesions , and metastases) that may occur in young adults without cirrhosis. Steroid related adenoma is typically detected when it reaches about 5 cm in diameter but the size could be 6–30 cm (large and multiple lesions are more prone to spontaneous haemorrhage). In 75% of cases the lesion involves the subcapsular region of right lobe of liver, in 10% it is intraparenchymal or pedunculated. HCA is usually solitary 70–80%) and multiple in 20–30%. A so-called adenomatosis is present in subjects observed to have more than 10 lesions: this entity is independent of gender or hormone therapy .–37.35–37Large regenerative hyperplasia (LRH) is another generally asymptomatic rare condition (0.5–2.5% on autopsy series) characterized by diffuse micronodular transformation of the hepatic parenchyma, without fibrous septa between nodules. Disorders in the hepatic microcirculation (chronic ischemia) with hyperplastic parenchyma response seem to be the primary cause of LRH. Myeloproliferative and lymphoproliferative disorders, chronic vascular and rheumatologic syndromes, and some drugs used are related to LRH. Adults (mean age 50), without gender predilection known, are more affected by LRH, rarely reported in childhood . Acute abdominal pain occurs in case of rupture of a large subcapsular nodule with hemoperitoneum. If central scar is present nodules can be indistinguishable from FNH. Portal vein obstruction and secondary hepatic arterial dilatation can be observed. In case of splenomegaly and increased flow in portal vein, several nodules can be observed with patent portal branches. When thrombosis of a large portal branch occurs, remnant portal flow is redirected in the perihilum parenchyma with large regenerative nodules  near the large portal tracts and small nodules with atrophy in the peripheral parenchyma. No malignant degeneration of LRH has been observed , 39. Prognosis depends on underlying disease. Treatment is indicated in progressive hepatic failure related to underlying disease.On unenhanced T1w MR images LRH is generally almost isointense or slightly hyperintense compared to the surrounding liver parenchyma while on unenhanced T2w images the nodule appears iso- or slightly hypointense. A pheripheral hypointense rim is often visible in large lesions on T1- and T2-weighted images. On dynamic MR imaging, LRH is usually hyperintense in the arterial phase and iso- or slightly hyperintense in the portal-venous and equilibrium phases. In the delayed, liver specific phase after Gd-BOPTA or GD-EOB-DTPA the lesion appears isointense or hyperintense because it consists of benign hepatocytes with abnormal biliary system drainage  40, 41], 4140, 4The main diagnostic dilemma is between LRH and inflammatory HCA; the key elements to achieve the differential diagnosis are the different signal on T2 images ; the dissimilar contrast behaviour in dynamic study.HCC in noncirrhotic patients and fibrolamellar hepatocellular carcinoma (FL-HCC) are rare malignant primary liver tumors which arise in young healthy patients of both sexes (M : F = 1 : 1). Hepatomegaly, malaise, pain in right upper quadrant, fever, and/or weight loss may be present. The lesion becomes symptomatic only when the mass reaches very big size and compresses adjacent structures or invades vascular or biliary vessels .Both tumors can present with metastatic disease (lymph nodes and lungs are the most frequent sites). Alpha-fetoprotein is usually elevated in conventional HCC but often normal in FL-HCC.Generally there is a large (average mean size >10 cm), single (80–90%), well-demarcated, lobulated, noncapsulated mass (incomplete capsule in 1/3 of cases); in 20% of cases it can be pedunculated. In FL-HCC coarse calcifications had been depicted in more than 50% of lesions and, at cut section, lobular arrangement fibrous septa with radial disposition and central scar are seen in 60–70% of cases; abdominal lymphadenopathy had been detected in >60% of patients. Central scar, fibrosis, and calcification are rare in conventional HCC whereas necrosis hemorrhage and intratumoral fat are much more common than in FL-HCC.The surgical resectability is high (50%); multiple or too large tumors can be treated with liver transplantation. FL-HCC is frequently recurrent, but 5-year survival is about 50–60%.On MR imaging, HCC in non-cirrhotic patients depends largely on tumor size that is generally large because the lesion is not detected in early stage. Generally, most HCCs, because of their hypervascular nature, are hyperintense compared to the liver in the arterial phase and hypointense in the portal-venous and equilibrium phases. Irregular mosaic-like or pheripheral enhancement is usually seen in large neoplasms, depending on the internal architecture. In the delayed liver-specific phases after Gd-BOPTA or Gd-EOB-DTPA well-differentiated and moderately differentiated HCCs may show in a small percentage of cases superior signal enhancement ratios to poorly differentiated HCCs .β-catenin, is very difficult: both adenomas may be heterogeneously hyperintense on T2-weighted images and hypointense on T1-weighted sequences; they may show intense and heterogeneous enhancement in the arterial phase of dynamic study, subsequent wash out, and/or hypointense signal in hepatobiliary phase. The larger component of fat is more typical for HNF-1α-mutated HCA.Differential diagnosis between HCC and HCA, subtypes inflammatory and FL-HCC is usually either hypointense or, rarely, isointense compared to the liver on T1-weighted images. On T2-weighted images, 90% of the lesions are hyperintense and the remaining 10% are isointense. The purely fibrous nature of the scar means that it is hypointense on both T1- and T2-weighted images. FL-HCC becomes heterogeneously hyperintense during the arterial phase after the administration of gadolinium and appears as isointense or slightly hypointense during the portal-venous and equilibrium phase. The central scar shows minimal or no enhancement in arterial and portal-venous phase images but may sometimes show persistent enhancement in equilibrium phase. In hepatobiliary phases, FL-HCC usually appears as heterogeneously hypointense with areas of low signal intensity due to necrosis or, less frequently, haemorrhage  42–44].–44.42–44Besides the key elements already described for HCC in non cirrhotic patients, FL-HCC may be differentiated from HCA for areas of fibrotic tissue which are hypointense on T1 and T2w images and hyperintense in the delayed phases of the dynamic studies. Signs of malignancy (vascular and biliary invasion) present in HCC and FL-HCC are other helpful elements in differential diagnosis.Cholangiocarcinoma (CC) is the primary malignancy arising from the bile duct epithelium and it is the second most common liver malignancy after HCC.Clonorchis sinensis and Opisthorchis viverrini, hepatolithiasis, congenital bile ducts anomalies, familiar polyposis, Thorotrast deposition, and Caroli's syndrome. Recent findings indicate that HCV-HBV infection conferred a more than twofold elevated risk of ICC.Incidence of CC among primary liver tumor ranges from 5 to 30%, with an average age of 50–60 years, seen slightly more often in men (M : F = 3 : 2). Risk factors are primary sclerosing cholangitis, bile stasis, recurrent cholangitis, infections with According to the site of origin CC can be classified into two types as follows: intrahepatic or peripheral (PCC) and extra-hepatic.PCC presents as large mass because tumor does not cause clinical symptoms in early stages. The initial symptoms are abdominal pain, malaise, anorexia, weight loss, fever, palpable mass, and jaundice (rare).The peripheral mass appears as a large white-grey lesion characterized by fibrosis (fibrotic core) and associated with capsular retraction; calcifications are rare. Sometimes there is concomitant dilatation of adjacent bile ducts and atrophy of corresponding liver segments.Lymph node involvement is present in 60–70% of PCC.Metastatic spread is common: lung, bone, pancreas, adrenals, kidney, spleen, and peritoneum.According to the Liver Cancer Study Group of Japan (LCSGJ) and based on the gross appearance ICC can be categorized in three patterns of growth that can be present alone or in to combination: mass-forming type (MF), intraductal growth (IG) type, and periductal infiltrating type (PI). The prognostic significance of such classification has been confirmed in several clinical studies , 46.Curative resection is the most effective treatment and the only therapy associated with prolonged disease-free survival; the rate of radical resection is extremely variable in the literature, ranging between 30% and 80%. Prognosis of ICC is poor due to late presentation and limited resectability. Five-year survival is less than 30% of surgically treated patients.On MR imaging PCC is either isointense or hypointense relative to the normal liver on T1w MR images but may range from mildly to markedly hyperintense on T2w images. The signal intensity of the tumour is variable and depends on the amount of mucinous material, fibrous tissue, haemorrhage, and necrosis within the lesion. On dynamic study after injection of Gd-BOPTA or Gd-EOB-DTPA, minimal or moderate incomplete enhancement is seen at the periphery on early images, whereas progressive central contrast enhancement is seen on later images. Generally, on delayed phase images lesions show peripheral hypointensity and central iso- or hyperintensity due to central pooling of contrast medium within central desmoplastic reaction. Satellite nodules are seen in about 10–20% of PCC cases and are chiefly responsible for the poor prognosis of this tumour  47, 48], 4847, 4Primary lymphoma (PL) of the liver (confined to the liver without involvement of lymph nodes or spleen or bone marrow) is very rare . PL can arise at every age but it is more frequent in childhood-adolescence or in middle-old age (M : F = 4 : 1). It can be associated with Epstein-Barr infection (EBV), and more frequently in patients with chronic hepatitis or cirrhosis (by HBV and/or HCV infections), with autoimmune disorders or with AIDS manifestations; it can also be associated with immunosuppression in transplanted patients. Liver involvement (50% of cases) in haematological diseases (secondary liver lymphoma) is more common.Hepatomegaly, presence of hepatic mass or masses, and pain in upper right quadrant are the most frequent signs and symptoms in primary lymphoma. Fever, weight loss, and perspiration are associated in 50% of cases.PL can present as multiple, small nodular lesions (well-differentiated B-cell lymphoma) or as diffuse infiltration of hepatic parenchyma (undifferentiated subtypes); in some patients with Hodgkin's lymphoma peliosis hepatis can be associated. The outcome, with appropriate treatment, is favourable. Surgical resection followed by chemotherapy and/or radiation gives the best prognosis in primary lymphoma , 50. On MR imaging, PL is generally seen as homogenously/heterogeneously hypointense compared to the normal parenchyma on unenhanced T1-weighted images and hyperintense on T2-weigthed images. Dynamic imaging after Gd-BOPTA or Gd-EOB-DTPA typically reveals a hypointense appearance on arterial phase images, followed by homogeneous, delayed enhancement on portal-venous phase images, and isointensity on equilibrium phase images. However, some hypervascular PLs are described. In most cases, PL is hypointense in the delayed hepatobiliary phases  51]. . 51]. α-mutated HCA may be differentiated for the fatty intralesional component which determines signal patterns on plain MR and dynamic study.The equivocal behavior of PL does not offer important elements for differential diagnosis with HCA. However, HNF-1Metastases are the most common cause of malignant focal liver lesions . At autopsy hepatic metastases occur in 30 to 55% of patients dying from malignant disease. Liver metastases originate predominantly from primary tumors localized in gastrointestinal tract , by hematogenous spread, via portal vein. Other frequent secondary lesions are from breast and lung cancers : primary neoplasms probably give metastases by hematogenous spread, via the arterial blood supply to the liver. Lymphogenous spread occurs along bile ducts from near organs (pancreas) or from haematological diseases . Direct invasion from nearest organs  can also be the cause of liver metastases. Liver involvement by intra-abdominal dissemination can also be observed (ovarian cancer) .Most patients with metastases to the liver present with symptoms related only to the primary tumor; the asymptomatic hepatic involvement is discovered in the course of clinical evaluation. Sometimes there are no specific symptoms: weakness, weight loss, fever, and loss of appetite. Rarely, hepatomegaly, hepatic mass, or pain in upper right quadrant can be the first symptoms of liver involvement mostly when lesion/s are huge and multiple.Metastases can vary in size, number, consistency, uniformity of growth, vascularity, and stromal response. The outcome depends not only on primitive tumor, but even on number, size, and tissue component of metastases. If resectable, hepatic metastases have 20–40% survival rate at 5 years (colon cancer metastases).The differential diagnostic dilemma between HCA and metastases is mainly in the case of hypervascular, solitary lesion in patients with unknown primary cancer.Generally, on unenhanced T1w images metastases have low signal intensity compared to the surrounding parenchyma. On T2w sequences the lesions demonstrate high signal intensity, although the signal is generally lower than that typically observed in hemangiomas. Hypervascular metastases tend to reveal strong transient enhancement in the arterial phase followed by hypointensity in the portal and equilibrium phases; they appear hypointense in hepatobiliary phase  41, 53,,53, 54.The most important elements to achieve the differential diagnosis between HCA and each type of hypervascular lesions in non cirrhotic patients are summarized in α-mutated) hepatocellular adenomas, β-catenin-mutated hepatocellular adenomas, and unclassified adenomas. This classification has definitive management implications. MR imaging plays an important role in diagnosis and characterization, particularly in inflammatory and steatotic subtypes, as well as in identification of complications and surveillance.According to recent studies HCAs are currently categorized in to four distinct genetic and pathologic subtypes: inflammatory hepatocellular adenomas, hepatocyte-nuclear-factor-1-alpha (HNF-1Image-guided biopsy or surgical resection with histopathologic and immunohistochemical analysis is necessary for complete characterization of HCAs and in some differential diagnostic dilemmas.


Metastasis of rectal adenocarcinoma develops by lymphatic or hematogenous spread. The usual sites of metastasis from rectal adenocarcinoma include local and distant lymph nodes, the liver and the lungs. The current case report presents a unique case of a mass that was identified in the tonsil by positron emission tomography-computed tomography (PET-CT), indicating a metastasis from rectal adenocarcinoma. Metastatic tumor to the tonsil is extremely rare and to the best of our knowledge, no previous studies have reported a case of tonsil metastasis from rectal adenocarcinoma. PET-CT scanners represent an important evolution in technology that is helping to bring anatomical imaging togeother with functional imaging in cancer diagnosis and therapy. Written informed consent was obtained from the patient. Rectal cancer is the common cause of cancer-related mortality in the United States, with ~40,290 new cases in 2012 . By deteA 67-year-old male, diagnosed with stage III rectal adenocarcinoma, was admitted to West China Hospital  for abdominal rectal resection in May 2010. Postoperative pathological examination indicated mucosal adenocarcinoma, invading the whole intestinal wall and tumor cells were identified in 4/4 mesenteric lymph nodes.The patient was treated by chemotherapy with oxaliplatin, calcium folinate and fluorouracil every 14 days for seven cycles. In March 2011, PET-CT revealed a left lower lobe nodule with mildly increased glucose metabolism. The largest standardized uptake value (SUV) was 2.38, which exhibited metastatic possibility. The pulmonary nodule was surgically removed and the postoperative pathological diagnosis was a poorly differentiated adenocarcinoma arising from primary rectal adenocarcinoma. The tumor was diagnosed as invading the visceral pleura and immunohistochemical staining showed caudal-type homeobox transcription factor 2 (CDX-2)(+), mucin-1(+), cytokeratin 7 (CK7)(+) and thyroid transcription factor-1 (TTF-1)(−) in the tumor cells .Subsequently, the patient was treated by chemotherapy with 50 mg TS-1 twice daily. In November 2011, PET-CT revealed that the tonsil was swollen and the largest SUV was 8.08 . PatholoPET-CT scanning is important in oncology; functional and anatomical images are captured in a single scanning process. The PET-CT approach offers extensive possibilities for improving the diagnosis of primary and metastatic tumors . In the Identification of the primary tumor site is a challenging issue, which has prognostic and therapeutic significance. In the current case report, PET-CT revealed a mass in the left side of the tonsil. To identify the original site of the tumor, immunohistochemistry was performed and the results indicated a CK7(−)/CK20(+) phenotype, which confirmed colorectal carcinoma .Malignant tumors seldom metastasize to the oral cavity. When this occurs, such metastasis is usually from various organs, including the lung, breast and rectum. According to previous studies, a few cases of metastatic localization have been identified from the colon and rectum, including the gingival, alveolar mucosa and tongue –9. The cSince inflammatory and reactive lesions are common in the oral cavity, it is challenging to diagnose the rare case of rectal metastatic tumors in the tonsil. Occasionally, patients with tonsil metastasis clinically mimic patients with the primary tonsil neoplasm or tonsillitis. Therefore, it must be considered in the differential diagnosis of patients with unexplained pharyngalgia and antiadoncus. Careful examination, as well as a multidisciplinary approach, is recommended to validate the clinical suspicion. In conclusion, the current case report presents a unique case of tonsil metastasis from rectal adenocarcinoma and the oncologist must recognize the possibility of tonsil metastasis from malignant disease, such as colorectal cancer.


A case of lipomatous tubular adenoma (LTA) with syringocystadenom papilliferum (SCAP) arising from the external auditory canal in a 25-year-old man is described and to the best of our knowledge through literature review, this kind of morphologic entity has not been reported before. Herein we reported the first case in the English literature in the world. Tumors arising from the external ear canal are extremely rare. Exostosis and squamous cell carcinomas are the most commonly-encountered benign and malignant tumors, respectively. Tubular adenoma (TA), also named as tubular apocrine adenoma, tubular syringoadenoma, or tubulopapillary hidradenoma ,2, was fA 25-year-old male was referred for hearing impairment and discharge on and off from left ear for a period of time. Family history is unremarkable and laboratory data is with normal limit. Pure tone audiometry evaluation was arranged and conductive hearing loss of left ear was identified. A mass noted in the left external auditory canal. High resolution computed tomography showed an approximately 0.8 × 0.6 cm soft tissue mass occupying within the left external ear canal near the opnening, without eroding canal wall. Tumor excision was performed and the resected tumor was submitted for pathological examination. The patient recovered uneventfully without evidence of recurrence nine months after operation.Section of the tumor shows circumscribed lobules of well-differentiated double-layered tubular structures located in the deep dermis Figure . The strTA is a rare entity and sometimes occurs with SCAP. SCAP was previously reported to be often associated with pre-existing nevus sebaceous , and morTA or SCAP was reported to have certain relationship with human papillomavirus infection  and a huImmunohistochemical study may be useful in puzzling cases. S-100 protein was reported to be detected in the peripheral myoepithelial cells in the TA and failed to be present in the SCAP ,10, whicTreatment of LTA with SCAP includes careful preoperative evaluation and surgical resection with safe margin. Computed tomography is useful in evaluating the tumor and surrounding tissue.Our case shows the characteristic SCAP and TA with abundant adipocytes in the upper and the deeper portion, respectively. In a thorough research for PUBMED, this is the first reported case with LTA and SCAP occurring in the external auditory canal. Although Lee at al  publisheWritten 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 journalLTA: Lipomatous tubular adenoma; SCAP: syringocystadenom papilliferum; TA: tubular adenoma.The authors declare that they have no competing interests.TS: wrote, aligned and drafted the manuscript. KS participated in the collection of the data, literature review. HW: collected clinical and pathological records. PC: took the pictures for this manuscript. MC: modified this manuscript and make and final approval.All authors read and approved the final manuscript.


An embryonic paratesticular rhabdomyosarcoma is a very rare mesenchymal tumor. It is an intrascrotal tumor that is localized in paratesticular structures such as the epididymis or spermatic cord. Rhabdomyosarcoma is most often observed in children and adolescents, presenting as a painless scrotal mass.Our patient was an 18-year-old Moroccan man who presented with a painless left scrotal mass that had evolved over four months. An inguinal orchiectomy was performed. A histological examination of the excised tissue revealed an embryonic rhabdomyosarcoma.Our patient had three sessions of chemotherapy with vincristine, actinomycin C and cyclophosphamide. Each chemotherapy session was conducted over five days, with a cycle of 21 days. Our patient was assessed two months after the last chemotherapy session and demonstrated good clinical improvement.Paratesticular rhabdomyosarcoma is a rare aggressive tumor manifesting in children and very young adults. Localized forms have a good prognosis whereas metastatic tumors show very poor results. A well-defined treatment based on surgery and chemotherapy yields good results. An embryonic paratesticular rhabdomyosarcoma is a very rare mesenchymal tumor. It is an intrascrotal tumor that is localized in paratesticular structures such as the epididymis or spermatic cord. Rhabdomyosarcoma is most often observed in children and adolescents where it is usually presents as a painless scrotal mass.We report the case of an 18-year-old man who presented with a large mass that had developed over four months. An investigation into tumoral markers was negative. The surgical intervention consisted of left orchiectomy. A histological examination of the surgical specimen confirmed embryonic paratesticular rhabdomyosarcoma. Adjuvant chemotherapy was initiated.Our patient was an 18-year-old Moroccan man who presented with a painless left scrotal mass that had evolved over four months. A clinical examination revealed a hard testicular mass in his left side with a diameter of 10cm; the mass was renitent, static and suspicious.Clinical examinations of his lymph nodes and abdomen were normal. An ultrasound revealed an expansive process characterized by intrascrotal heterogeneous tissue density. A computed tomography (CT) scan of his thorax, abdomen and pelvis showed a left heterogeneous testicular tumoral process with high adherence measuring 7cm to 8cm. However, neither pelvic nor lombo-aortic lymphadenopathy was revealed. The thoraco-abdomino-pelvic CT scan did not show any metastases.Our patient’s levels of tumoral markers alpha-fetoprotein, beta-human chorionic gonadotropin and angiotensin-converting enzyme were normal. An inguinal orchiectomy was performed. A histological examination of the surgical specimen demonstrated tumoral proliferation with unorganized architecture in sheets of pleomorphic clear tumoral cytoplasmic cells and eosinophils with atypical nuclei; eccentric aspects with strong nucleoli and multi-nucleoli with rhabdomyoblastic aspects were observed. This confirmed the diagnosis of an embryonic rhabdomyosarcoma.Immunohistochemistry demonstrated focal myogenic expression in less than half of the tumoral cells Figures  and2.2, actinomycin C 1.5mg/m2 and cyclophosphamide 500 mg/m2 were performed. Each chemotherapy session was conducted over five days, with a cycle of 21 days. Our patient was assessed two months after his last chemotherapy session and demonstrated good clinical improvement.Three chemotherapy sessions of vincristine 1.5 mg/mRhabdomyosarcoma usually occur in the extremities. A paratesticular localization is rare; the tumor develops from mesenchymal tissues of the spermatic cord, and epididymis.There are two frequency peaks found for the development of rhabdomyosarcoma, the first at the age of 4 years and the second at the age of 18 years, as in our case. There is no predilection for race.The consensus is that this tumor derives from mesenchymal elements of the testis envelope, epididymis and spermatic cord. The tumor manifests as a hard painless inguinoscrotal swelling, the size and duration of development are varied and it rarely invades the scrotal skin. The mass might evolve within the external inguinal ring away from the scrotal contents,3. A cliA testicular ultrasound is routinely performed for a scrotal mass. This imaging modality shows a mass with heterogeneous echogenicity and inguinoscrotal extension in 80% of cases. This alA thoraco-abdomino-pelvic CT scan allows for any deep invasion of the lymph nodes to be investigated, especially lombo-aortic and pelvic metastases as well as possible metastases to the liver or lung. Most authors prefer the use of thoraco-abdomino-pelvic CT compared to ultrasound for lymphomas.Magnetic resonance imaging is an efficient imaging modality when using surface coils. The tumor appears homogeneous in T1-weighted images and heterogeneous in T2-weighted images with signal intensity similar to that in a normal testis. The low signal intensity of the tunica albuginea in T2-wieghted images allows the visualization of a clear separation of the mass from the testis,6.In rhabdomyosarcoma, tumoral markers including alpha-fetoprotein, beta-human chorionic gonadotropin and carcinoembryonic antigen are usually normal. This was the case with our patient.A malignant tumor might be suspected in masses sitting in the distal cord with a hard and irregular form adhering to surrounding structures; a rapid increase in tumor volume might be noted. But the diagnosis is mainly made using histology. The macroscopic features include a lobed tumor surrounded by whitish pseudocapsules, hemorrhagic array are sometimes revealed.There are four histological types of rhabdomyosarcoma: pleomorphic, alveolar, botryoidal, and embryonal, such as found in our patient. The characteristic cell is rhabdomyoblast, which is not necessary for the diagnosis. Whenever rhabdomyoblasts are not present, immunohistochemical investigations are conducted using a panel of antibodies including myosin and desmin,8,9.Rhabdomyosarcoma may be included in the differential diagnosis for other paratesticular sarcomas such leiomyosarcoma, liposarcoma and fibrosarcoma. However, these pathologies occur more often in adults. Imaging cannot discriminate between these tumors. The final diagnosis can be established by histological study after surgical excision of the tumoral mass,7.Radical orchidectomy by the inguinal route with first cord ligation remains the essential act for histological diagnosis and constitutes the first step of treatment regardless of the stage of the disease. Hemiscrotectomy associating inguinal treatment is indicated first in scrotal cases whenever local invasion or presence of lymph are clinically evidenced,7. An inChemotherapy should be routinely administered since rhabdomyosarcoma is chemosensitive. This therapeutic approach consists of administrating actinomycin D, vincristine and cyclophosphamide-7,10. RaOur patient benefited from an inguinal orchiectomy. Three sessions of chemotherapy were performed, resulting in no signs of metastasis or residual foci.Paratesticular rhabdomyosarcoma is a rare aggressive tumor manifesting in children and very young adults. Localized forms have a good prognosis whereas metastatic tumors show very poor results. A well-defined treatment based on surgery and chemotherapy yields good results. Radiotherapy is indicated in cases of residual foci and retroperitoneal lymphnodes. Strict follow-up has to be instituted for all patients.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.AAB was the principal author and major contributor in writing the manuscript. SM, MFT, RK, KL, KB, JE, AK and YK analyzed and interpreted the patient data and reviewed the literature. MJE and MHF read and corrected the manuscript. All authors read and approved the final manuscript.


Synchronous multiple colorectal cancers are defined as multiple malignant colorectal tumors that occur simultaneously. All tumors are distant from each other, and none are the result of metastasis from other tumors. Here, we present a case of a 79-year-old man who was admitted to our hospital because of a 3-month history of abdominal pain associated with anemia, loss of appetite, and body weight loss. The patient did not have a family history of cancer. Computed tomography revealed bowel wall thickness and mesentery inflammation at the hepatic flexure of the colon and cecum. Colonoscopy revealed a tumor located 10 cm from the anal verge. Colonoscopic examination of the large bowel was not possible because of bowel obstruction due to the rectal tumor. Synchronous triple adenocarcinoma of the colon and rectum was confirmed by pathologic examination. The tumor was surgically resected by two-segment resection of the colon, low anterior resection, and right hemicolectomy. We used intraoperative colonoscopy to confirm that there were no other lesions after the resection of the three tumors. To the best of our knowledge, this is the first case of synchronous triple carcinoma of the colon and rectum in Taiwan. We consider that comprehensive preoperative study, extensive intraoperative exploration, and radical resection can increase the survival rate of patients with synchronous multiple colorectal cancers. The colon is the organ most frequently involved in synchronous malignancy, especially in patients of advanced age-3. SynchA 79-year-old man with a history of hypertension presented with a 3-month history of abdominal pain associated with hematochezia, poor appetite, and body weight loss. The patient did not have a family history of cancer. Physical examination revealed tenderness at the right upper quadrant of the abdomen. Laboratory studies revealed a hematocrit value of 24.9%, a hemaglobin level of 7.5 g/dl, and a CA-199 value of 486.2 U/ml. Other cancer markers such as á-FP, CEA, and PSA were within the normal range. No anomalies were detected on chest X-ray, abdominal sonography and fiber-optic gastroscopic examination. Computed tomography (CT) of the abdomen revealed obvious bowel wall thickness and mesentery inflammation in the hepatic flexure of the colon and cecum  colonography are two mini-invasive imaging methods that were described in 1994 and 1997, respectively, and were used to examine the entire colon on the basis of the respective CT and MR data sets,16. The Operative techniques for managing multiple lesions or cancers need to be tailored to the individual, based on the location, status of invasion, and patient’s health condition. The initial operative procedure for multiple colorectal carcinomas is controversial. Some studies have suggested total or subtotal colectomy to remove any potential existing synchronous tumors or polyps that have not been detected-29. It iTo the best of our knowledge, the present report is the first case of synchronous multiple colorectal cancers in Taiwan. The patient had no family history of colon cancer. The first tumor measuring 3 cm in diameter was in the rectal region, the second tumor measuring 7 cm in diameter was in the hepatic flexure of the colon, and the third tumor measuring 4 cm in diameter was in the cecum. For treatment of synchronous multiple colorectal cancers, identification of concurrent lesions is vital to improve prognosis. Therefore, a thorough evaluation of the entire colon is very important. Treatment of synchronous colorectal cancers is not different from that of single colorectal cancers. Comprehensive preoperative study, extensive intraoperative exploration, and radical resection allow for early diagnosis and treatment of synchronous multiple colorectal cancers, which can increase the survival rate of patients with these cancers.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.CT: computed tomography; MR: magnetic resonance; PET: positron emission tomography.The authors declare that they have no competing interests.C-CY wrote the main manuscript and performed the operation. S-CH, C-PC, and Y-HK revised the manuscript for important intellectual content, and provided the final approval for the version to be submitted for publication. All authors read and approved the final manuscript.


Recurrent spontaneous rupture of the urinary bladder has rarely been reported in English articles. This condition may be difficult to diagnosis before a laparotomy due to acute peritonitis. Herein we describe a case of recurrent spontaneous rupture of the bladder in a 39-year-old woman with human T-lymphotropic virus type 1 (HTLV-1) -associated myelopathy/topical spastic paraparesis (HAM/TSP). Recurrent rupture of the urinary bladder is a rare condition, it is usually the result of an underlying disease which may be difficult to diagnose, and the diagnosis is almost always confirmed after a laparotomy . In the Idiopathic bladder rupture in some patients may be due to a neurogenic bladder dysfunction, which has not been discovered in a clinical and paraclinical evaluation . An undeWe present a case of recurrent rupture of the urinary bladder in a patient with HTLV-1–associated myelopathy which has not previously been reported in the literature.A 39-year-old woman presented with a sudden onset of generalized abdominal pain and decreased urine volume. No other lower urinary tract symptoms including; dysuria, or hematuria, were present. In her past medical history there was a history of multiple laparotomies due to different etiologies, including; appendicitis, cesarean section, PID peritonitis and two episodes of spontaneous bladder rupture. The bladder ruptures had occurred two and five years ago, although the initial evaluation did not reveal any etiology and she had not received any special treatment. Her medical records showed that the bladder ruptures had occurred at the dome of the bladder, and it had been repaired with absorbable sutures. She also complained of gradually progressing voiding problems, such as: dysuria, hesitancy, straining to urinate and a sensation of incomplete emptying, for a prior period of about 20 years. This had eventually resulted in an inability to void and she had been using CIC (clean intermittent catheterization) for bladder emptying since this time. No other associated risk factors such as trauma, malignancy or radiotherapy were found in her past medical history. However, she did have a suspicious medical history with a positive HTLV-1 test, without any treatment or follow-up.On physical examination, there was generalized abdominal tenderness and guarding with positive rebound tenderness. All of the initial blood tests  were normal. A Foley catheter was inserted, but only a small amount of urine was detected. On the basis of her symptoms, the patient underwent an urgent sonography, which showed free fluid in the abdomen, but it did not reveal any hydronephrosis or other urinary pathologies. Based on the clinical and radiological findings, the patient underwent a laparotomy which showed an intraperitoneal rupture of the urinary bladder dome. The site of the perforation was sutured in two layers. The patient remained asymptomatic after the operation. During her follow-up, a cystography study was carried out which showed no pathology, and a subsequent cystoscopy also revealed no pathology. A bladder biopsy was conducted and this showed chronic cystitis. A detailed neurologic examination was performed by a neurologist, which revealed spastic paraparesis. On the basis of the patient’s past medical history and the neurologic examination, a quantitative real time PCR assay was carried out to measure the proviral load of HTLV-1 in her peripheral mononuclear blood cells (PMBCs). It showed a proviral load of 409 copies in 104 PMBCs indicating that 4% of the PMBCs were infected. Therefore, according to her neurologic examination, this lab data was suggestive of HAM/TSP. Further evaluation was done using urodynamic studies, which revealed detrusor hyposensitivity and hypoactivity, and also detrusor sphincter dyssynergia (DSD) . In a cySpontaneous bladder rupture is a rare event with an incidence of 1:126 000. A total of 79% of all reported cases were in men and the overall mortality was 47% . DiffereA diagnosis of urinary bladder rupture is difficult to make, and it requires a high index of suspicion. The most common presentation is; diffuse abdominal pain and lower abdominal tenderness, which is often seen due to chemical peritonitis. Confirmation of the diagnosis is almost always done with a laparotomy. Treatment and repair of the perforation is usually performed during a laparotomy, but some patients, especially those with a history of radiation, may be managed conservatively using antibiotic therapy and prolonged bladder drainage .HTLV-1 is a retrovirus that infects about 10 to 20 million people worldwide. Southern Japan, the Caribbean, Central and South Africa, South America and the north east of Iran, are the endemic foci of this virus . In 1989Urodynamic testing includes the assessment of both detrusor and external sphincter functions. Patients with moderate to severe incontinence, those suspected of having a neurological disease, and those with urge incontinence, as well as patients with obstructive problems, may need urodynamic evaluation for diagnosis and management . Castro In the present case, detrusor hyposensitivity and hypoactivity, along with DSD in the urodynamic evaluation, were diagnosed. It appears that rupture of the urinary bladder was caused mainly by bladder overdistention and thinning of the dome wall, secondary to a large volume of urine. Therefore, treatment of the underlying disease and careful regulation of CIC were recommended in order to avoid similar episodes of bladder rupture.


Fusion is a developmental anomaly characterized by the union of two adjacent teeth. In this paper we report a rare case of fusion involving permanent mandibular second molar with supernumerary tooth. The rarity with which this entity appears, along with its complex characteristics, often makes it difficult to treat. The use of high-end diagnostic imaging modalities such as spiral computed tomography can help the clinician in making a confirmatory diagnosis and determining the treatment plan before undertaking the actual treatment. In the present case, we have used spiral computed tomography (SCT) for better understanding the complicated root canal morphology of the fused tooth and successful management of this rare case. Teeth with morphoanatomic changes pose a challenge in endodontic therapy. It is imperative that aberrant anatomy is identified before and during root canal treatment of such teeth. Morphoanatomic changes in teeth may be divided according to the site of their occurrence, that is, tooth crown, roots, and root canals. Fusion and gemination are irregularities in tooth development . PindborThe degree of fusion depends on the stage of tooth development that has occurred at the time of fusion, with the union of dentin being the main criterion. Fused teeth may contain separate pulp canals or share a common pulp canal. Fusion may occur between two normal teeth or between a normal tooth and a supernumerary tooth. Supernumerary tooth is an additional entity to the normal series and is seen in all the quadrants of the jaw . When thWhen fusion occurs between a molar and a supernumerary tooth as a result of their abnormal morphology and excessive crown width, problems with crowding, alignment, and occlusal functions can occur . These tRadiographic examination is an essential component in the management of endodontic problems. However, the conventional intraoral periapical views produce only a 2-dimensional image of a 3-dismensional object, resulting in the superimposition of structures. Conventional multidetector computed tomography (CT) imaging has been widely used in medicine since the 1970s and was introduced in the endodontic field in 1990 , 11. This paper presents the endodontic management of a fused mandibular second molar with a supernumerary tooth with the use of SCT as a diagnostic aid.A 20-year-old male patient presented to the department of conservative dentistry and endodontics with pain to hot and cold food in the left mandibular posterior region. On clinical examination, the mandibular first molar exhibited caries on the distoocclusal aspect and second molar exhibited abnormal crown morphology with 2 large extra cusps on the buccal aspect of the tooth . Distinc There was no previous history of trauma or any hereditary conditions. Medical history was noncontributory. All vital signs were found to be within normal limits. Oral examination revealed a normal set of dentition. The teeth 20 and 21 were not sensitive to palpation or percussion. The tooth responded to an electric pulp tester  and showed early response to the cold test . On radiographic examination, tooth 21 showed resorption with the distal root and the exact anatomy of tooth 20 that could not be clearly identified, but it was possible to recognize the complex structure of the pulp . TherefoThe images revealed that the mandibular left second molar had 3 root canals and that there was connection between the paramolar and second molar in the pulpal chamber. The paramolar exhibited a type II canal (according to Wiene's classification). Local anesthesia was administered and a rubber dam was applied. The prongs of the rubber dam retainer had to be trimmed to accommodate the unusual anatomy of the tooth. Endodontic access cavity was done on the occlusal surface using a no. 2 round bur and EX 24 bur  . Pulp ex After a week, the tooth was asymptomatic and the root canal was obturated using thermoplastic obturation technique  and AH PLUS as a sealer. The access cavity was then sealed with resin composite .Routine endodontic treatment was carried out with tooth 21. The tooth was totally asymptomatic at a 1-year recall visit Figures  and 4.Fused teeth afford a striking clinical manifestation of the differentiable and morphogenetic processes of tooth development. Fusion between supernumerary and permanent teeth occurs less frequently than fusion between other types of teeth. The incidence of unilateral occurrence is estimated in the literature to be 0.5% in the deciduous and 0.1% in the permanent dentition. The incidence of bilateral occurrence is estimated at around 0.02% for both types of dentition , 13. As  Fusion is a developmental anomaly with inherently unusual and bizarre anatomy. When it occurs, it commonly results in caries, periodontal disease, and crowding . Endodon A new CT technique, SCT or volume acquisition CT, has been developed that has its inherent advantage. Current CT scanners have a linear array of multiple detectors, allowing multiple slices to be taken simultaneously, resulting in faster scan times and often less radiation exposure to the patient. The slices of data are then “stacked” up and can be reformatted to obtain three-dimensional images . SCT can get a large volume of data in seconds and offer more rapid examination time and with an effective dose in the range of about 1–30 mSv, which is much less than the conventional CT . Christo In this specific case, a SCT could confirm the complicated morphology of the root canal system of the fused tooth. This technique seems to have the potential to visualize the topography of root canals and offer new perspectives for dental imaging of special clinical cases. With the advent of newer tomographic scanners like cone beam computed tomography (CBCT) or digital volume tomography (DVT) specifically for maxillofacial and dental use, conventional scanners like SCT will be less preferred for dental imaging purposes. CBCT is advantageous in that it has a low effective dose in the same order of magnitude as conventional dental radiographs . AlthougThis paper demonstrates a predictable and successful solution towards the endodontic management of a fused mandibular second molar with a supernumerary tooth by using SCT as a diagnostic tool to a great effect in understanding the complex root canal anatomy, thus helping a great deal in rendering successful endodontic therapy. Proper diagnosis and treatment planning for endodontic management of fused teeth by using SCT can ensure predictable and successful results.


Introduction. Synovial sarcomas (SS) are aggressive malignant soft tissue tumours that are thought to arise from pluripotent mesenchymal cells. Clinical Report. A 20-year-old male presented with an acute onset of respiratory stridor. Computer tomography scanning confirmed a mass arising from the left supraglottic larynx and an emergency tracheostomy was performed. A diagnosis of biphasic synovial sarcoma was formed. A total laryngectomy and left hemithyroidectomy was performed in conjunction with a left modified radical neck dissection. The patient received adjuvant chemotherapy followed by a course of radiotherapy and remains alive and disease free at 18 months after treatment. Discussion. Prognosis for patients with SS is related to primary tumour extent, grade, and size. The presence of the diagnostic translocation, t, is being targeted and hopefully will lead to the development of new therapeutics . Conclusion. Laryngeal SS remains a rare and poorly understood entity. A multidisciplinary approach to treatment is essential and long-term followup is imperative. Sarcomas represent 1% of all head and neck malignancies . The mosAn otherwise fit and well nonsmoking 20-year-old male presented with an acute onset of respiratory stridor associated with a sore throat and odynophagia. Flexible nasendoscopy revealed a large well circumscribed mass in the left aryepiglottic fold which was causing some degree of laryngeal inlet obstruction. Computer tomography (CT) scanning confirmed a large cystic mass measuring 7.5 cm × 3.6 cm arising from the left supraglottic larynx extending through the cricothyroid membrane into the left thyroid lobe . There wA positron emission tomography with fluoro-deoxyglucose (FDG-PET) scan revealed avid isotope uptake in the left larynx and thyroid, with some mild uptake in several upper left cervical lymph nodes. There was no evidence of distant metastatic disease.A total laryngectomy and left hemithyroidectomy were performed in conjunction with a left modified radical neck dissection. The final histopathology confirmed SS showing characteristic biphasic histology  with cleThe American Cancer Society estimates that in 2012 there will be about 12,360 new cases of laryngeal cancer diagnosed . SquamouSS acquired its name due to its microscopic resemblance to developing synovium but is immunophenotypically and ultrastructurally distinct from normal synovium, only rarely arising in joint cavities, and usually occurs in association with para-articular regions of the extremities, with no relation to synovial structures . Except Histologically, SS can be divided into two main groups: biphasic and monophasic. Both variants contain a population of monomorphic spindle cells arranged in fascicles with tapering nuclei and pale, ill-defined cytoplasm set in a variably collagenous stroma. In addition, classic biphasic lesions contain glandular structures lined by well-differentiated cuboidal to columnar epithelium. A branching hemangiopericytoma-like vascular pattern is characteristic and a common finding in both types is the presence of stromal calcification, which ranges from focal to extensive and is an important diagnostic clue . Biphasic lesions generally pose no diagnostic difficulty but those in unusual locations may raise a differential diagnosis of other biphasic tumours such as carcinosarcoma, malignant mesothelioma, and malignant peripheral nerve sheath tumour (MPNST), which rarely has glandular elements, particularly those arising in patients with neurofibromatosis type 1 . Immunohistochemically, SS is characterised by coexpression of mesenchymal and epithelial markers  . About 3Cytogenetics contribute greatly to the diagnosis of SS as 90% harbour a specific translocation between the SYT gene on chromosome 18 and either the SSX1 or SSX2 gene on the X chromosome .Prognosis for patients with SS is related to primary tumour extent, tumour grade, and size . The 5-yDisease recurrence is a significant problem, with up to 45% of patients with head and neck SS developing a local recurrence and 33% developing distant metastatic disease . The preOur case adds to current literature of laryngeal SS which remains a rare and relatively poorly understood entity. There are no well-established risk factors to enable a screening plan to be recommended or management protocols to be constructed. A multidisciplinary approach to diagnosis and treatment is essential to improve the management of SS. Long-term followup is imperative due to the relatively high locoregional and metastatic recurrence rates . Future Synovial sarcomas are aggressive malignant soft tissue tumours that usually involve large joints within the lower extremities. They are extremely rare within the head and neck.Our case report describes a young male with a biphasic laryngeal synovial sarcoma who remains disease free 18 months after treatment.Cytogenetics contribute greatly to the diagnosis of synovial sarcoma as 90% have the  trans-location. This is being targeted and will hopefully lead to the development of new therapeutics.A multidisciplinary approach to management and long-term followup is essential due to the high locoregional and metastatic recurrence rates.


An unusual multinodular and vacuolating neuronal tumour (MVNT) has been described in the cerebral hemispheres of ten patients with adult-onset seizures. We report the findings in two cases with similar features, a surgical resection and the other an autopsy specimen.Case 1, a 34-year-old female, underwent surgical resection for a multinodular non-enhancing frontal white matter lesion causing intractable epilepsy. Case 2, presented with motor neurone disease (MND) at the age of 71 and MRI scanning revealed extensive multinodular non-enhancing white matter lesions in the temporal lobe. There was no history of epilepsy and post mortem histology confirmed MND.Macroscopically multiple small grey well-formed, discrete and coalescent nodules were seen in the deep cortex and subcortical white matter. On histology, mature-looking neurons with large cytoplasmic vacuoles were distributed in a fibrillary background, where vacuoles were also noted. In the resected tumour scattered oligodendroglia-like cells were present. No ganglion cells were seen. The vacuolated cells exhibited immunopositivity for synaptophysin, HuC/HuD and p62 but were negative for NeuN, neurofilament, GFAP, IDH1, nestin and CD34. Electron microscopy showed non-membrane bound cytoplasmic vacuoles in the neurons and in some neuronal processes. The seizures recurred in Case 1.Some clinicopathological features of this lesion suggest a possible relationship with dysembryoplastic neuroepithelial tumour (DNT) although the morphological features are not typical of DNT. Case 2 demonstrates that MVNT may remain asymptomatic. The improving imaging techniques and the availability of surgery for the treatment of chronic, drug-resistant epilepsy have lead to the recognition of indolent brain tumours that appear to be largely restricted to this clinical setting. Many are of glio-neuronal lineage of which dysembryoplastic neuroepithelial tumour (DNT) and ganglioglioma are to be the most frequent . HoweverA 34-year-old female, who suffered from intractable epilepsy since age 8, was found on MRI to have multiple small T2-weighted hyperintense lesions within the subcortical white matter of the antero-medial aspect of the left superior frontal gyrus Figure , suggestA 71-year-old female was diagnosed with a motor neuron disease (MND) with predominant bulbar involvement in July 2010 following one year history of dysarthria and increased difficulty with swallowing. She had no history of epilepsy or dementia. EMG was reported as consistent with anterior horn cell disease and treatment with Riluzole 50 mg was initiated. MRI brain scan in July 2010 showed a lesion with a high T2 signal in the left lateral temporal lobe  was fixed in buffered formalin for 24–36 hours. All sampled tissue blocks were processed and embedded in paraffin wax and stained by conventional haematoxylin and eosin (H&E). Selected blocks showing histological abnormalities were further stained with Luxol-fast blue/Nissl (LFB/N) and PAS-D. Immunohistochemistry was carried out with antibodies against neuronal nuclear protein (NeuN), neuronal protein HuC/HuD (HuC/HuD), pan-neurofilament (pan-NF), neurofilament 200 KD (NF200 KD), synaptophysin, chromogranin, glial fibrillary acidic protein (GFAP), nestin, CD3, CD45, CD68, CD34, human leukocyte antigen-DR (HLA-DR), isocitrate dehydrogenase1 (IDH1) R132H, calbindin, parvalbumin, calretinin, Ankyrin-G, p62 and Ki67.The brain and the spinal cord (Case 2) were consented to research and donated to the MRC London Neurodegenerative Disease Brain Bank at King’s College Hospital. After fixation in buffered formalin for 12 weeks, the whole brain and the spinal cord were sampled as per our departmental protocol for MND. All sampled blocks were stained with H&E and selected blocks were stained with LFB/N, p62, TDP-43, tau, CD34, CD68, PAS, Sudan black, Nestin, NeuN, pan-NF, NF200 KD, Ki67, CD68, HLA-DR, CD45, CD3, α-synuclein, amyloid precursor protein (APP).Sections of 7 μm thickness (14 μm for LFB/N) were cut from paraffin-embedded blocks. Immuno-staining was performed using a Bond-max™ automated staining system . The immunolabelling was visualised using the Bond Polymer Refine Detection kit . The negative controls were treated identically except that primary antibody was omitted. The sources of the antibodies, and respective dilution and pre-treatment are described in Table Electron microscopy was performed on material re-embedded from paraffin block in Case 1 and from formalin fixed wet tissue in Case 2 .Genomic DNA was extracted from formalin fixed paraffin embededd tissue using the QIAamp DNA FFPE Tissue kit . Bisulphite conversion was performed using an EpiTect kit . MGMT promoter methylation status and BRAF V600 mutation were determined using the therascreen MGMT Pyro Kit and therascreen BRAF Pyro Kit respectively using a Q24 MDx Pyrosequencer . Sequencing of IDH1 and IDH2 was also performed by pyrosequencing methods .The specimen consisted of 4 fragments measuring 30 × 30 × 20 mm, 20 × 10 × 5 mm, 20 × 10 × 3, and 15 × 10 × 3 mm. On slicing at 5 mm intervals, two grey nodules of 2 -3 mm were identified in the subcortical white matter  discrete, well-demarcated, focally coalescent nodules, which were found predominantly within the white matter and in the deep cortex traversing the white matter – cortical junction Figure . The lesCase 2 showed typical histological features of MND in the brain and spinal cord.GFAP highlighted the reactive astrocytes within the nodules. There was marked microglial cell activation throughout the lesions as demonstrated by up-regulated HLA-DR and CD68 but the vacuolated cells were negative Figure A-B. The Some antibodies did not work on the overfixed autopsy material; therefore additional immunohistochemistry was carried out on Case 1 only. NeuN was negative within the vacuolated cells Figure A, but noIn Case 2, the immunophenotype confirmed the diagnosis of MND with limbic and mild neocortical TDP-43 pathology. Occasional TDP43 positive oligodendroglial inclusions were seen within the lesions, as frequently as in the rest of the white matter.There was no evidence of mutations of BRAF V600E, IDH1 and IDH2 in either case. MGMT promoter showed borderline methylation in Case 1, while Case 2 was unmethylated.The preservation was better in Case 1. In both cases, the vacuolated cells showed ultrastructural features of mature neurons with round to oval frequently indented nuclei with nucleoli and cytoplasm which was rich in mitochondria and rough endoplasmic reticulum Figure . In someWe have described two cases, a surgical resection and the other an autopsy specimen, with histological features similar to those recently described as MVNT by Huse et al. . The radUntil the neuronal nature of the strikingly vacuolated cells could be established, the uniform greyness of the lesions and the loss of myelin staining raised the differential diagnosis of demyelinating disease. In Case 2 the nodules were numerous but there were none in a periventricular location as usually observed in multiple sclerosis. There was prominent microglial activation with some foamy macrophages which sharply demarcated the abnormal areas from the surrounding white matter. Microglial marker upregulation has been found in tumours from patients with epilepsy, such as diffuse and nodular DNT, ganglioglioma and pleomorphic xanthoastrocytoma, and has also been described in focal cortical dysplasia (FCD) ,9. HowevThe vacuolated cells failed to express NeuN (available in Case 1). It appeared to label only the unaltered normal mature neurons of the white matter but not the vacuolated neurons. There was also no chromogranin or neurofilament positivity in the cytoplasm of those cells but axonal immunoreactivity with neurofilament was detected in the axons running through the lesion. We were able to confirm consistent labelling of the vacuolated cells by HuC/HuD as reported by Huse et al. . In addiElectron microscopy confirmed the neuronal nature of the vacuolated cells and the presence of a neuropil, which also showed some vacuolation. The cause of the vacuolation of neurons could not be established nor could it be related to a specific cell organelle. There was no accumulated material. A selective artefact affecting the neurons within the lesion is unlikely.The question remains unanswered as to whether the nodules of markedly vacuolated neurons represent definite neoplastic transformation or aberrant development. Nodular heterotopia has similar naked eye appearances although the nodules are usually more well demarcated from the cortex and do not show coalescence ,15. HoweMost importantly, the differential diagnosis includes the well known glioneuronal neoplastic lesions. We saw no ganglion cell morphology or atypia characteristic of ganglioglioma and gangliocytoma. Although vacuolar alteration is not uncommon in ganglion cell tumours, those are rarely and inconsistently seen in the ganglion cell component, and more likely to manifest as microcystic or myxoid change. There was also no typical accompanying glial component in these lesions, such as Rosenthal fibres, eosinophilic granular bodies, microcalcification or lymphocytic inflammatory reaction -18. The Molecular genetic testing showed no mutation of BRAF V600E, which may be found up to 50% of gangliogliomas and pleomorphic xanthoastrocytomas , in eithThe presence of an oligodendroglial/OLC component is suspected in this lesion. Huse et al. described OLIG2 expression in all of their lesions ,23. TherMVNT was reported as a lesion associated with adult onset seizures. Of the ten cases reported by Huse et al., five cases had adult onset epilepsy, most with a short history of seizures or episodic confusion (between 1 to 5 years) . Three pThe biological behaviour of MVNT appears to be benign, similar to DNT. Regardless of the extent of excision, no tumour recurrence was noted in the 8 cases available for follow up in the series of Huse et al. . Our casMany of the tumour-like glioneuronal lesions associated with chronic epilepsy appear to be clearly neoplastic, others continue to confound, straddling the boundary between malformation and neoplasm. The ten MVNT cases recently reported by Huse et al. were named as tumour but share features with malformation. On the other hand, this type of lesion shows similarities at least in behaviour with DNT and it is possible that the MVNT may even form part of the spectrum of DNT. MVNT may also present as an incidental lesion without seizures.Written informed consent for publication were obtained for case report using accompanying MRI and histological images without identifiable patient details.The authors declare they have no competing interests.IB, OC, SAS and MH made the pathological diagnosis. RS performed the surgery and RE performed the electrophysiology. Molecular genetic testing was performed by RL. IB and MH drafted the manuscript. All authors contributed to the manuscript and approved the final manuscript.


The discovery of adrenal incidentalomas due to the widespread use of sophisticated abdominal imaging techniques has resulted in an increasing trend of adrenal gland specimens being received in the pathology laboratory. In this context, we encountered three uncommon adrenal incidentalomas.The aim of this manuscript is to report in detail the three index cases of adrenal incidentalomas in the context of a 13-year retrospective surgical pathology review.The three index cases were investigated and analyzed in detail with relevant review of the English literature as available in PubMed and Medline. A 13-year retrospective computer-based histopathological surgical review was conducted in our laboratory and the results were analyzed in the context of evidence-based literature on adrenal incidentalomas.A total of 94 adrenal specimens from incidentalomas were identified, accounting for 0.025% of all surgical pathology cases. In all 76.6% were benign and 23.4% were malignant. A total of 53 females (56.4%) and 41 males (43.6%) aged 4 to 85 years were identified. The benign lesions included cortical adenoma (43.1%), pheochromocytoma (29.3%) and inflammation/fibrosis/hemorrhage (8.3%). Metastatic neoplasms were the most common malignant lesions (50%) followed by primary adrenocortical carcinomas (31.8%) and neuroblastoma (13.6%). These cases were discovered as adrenal incidentalomas that led to surgical exploration.The three index cases of adrenal incidentalomas with unusual pathologies were encountered that included (a) adrenal ganglioneuroma, (b) periadrenal schwannoma and (c) primary adrenal pleomorphic leiomyosarcoma. These cases are discussed, with a literature and clinicopathological review.Adrenal lesions are uncommon surgical specimens in the pathology laboratory. However, higher detection rates of adrenal incidentalomas aided by the ease of laparoscopic adrenalectomy has resulted in increased adrenal surgical specimens leading to unsuspected diagnostic and management dilemmas. Accurate pathological identification of common and uncommon adrenal incidentalomas is essential for optimal patient management. At autopsy, adrenal masses are among the most common tumors, reported in 3% of the population over 50 years of age ,2. Such The aim of this manuscript was to review and document the trend of adrenal specimens received in our surgical pathology laboratory over a 13-year period with special emphasis on three uncommon pathologies of adrenal ‘incidentalomas’ that were encountered, including ganglioneuroma, periadrenal schwannoma, and primary pleomorphic leiomyosarcoma.Pathological diagnosis of three unusual lesions resulting from the analysis of adrenal incidentaloma specimens prompted a detailed review of all adrenal lesions over the past 13 years (1997 to 2010) available in the Laboratory Information System (LIS). The LIS, operational since 1996, is a robust, reliable, centralized electronic data repository, which is administered by the Saskatoon Health Region for the Department of Pathology and Laboratory Medicine in the city of Saskatoon, Canada. All surgical material was retrieved and analyzed to confirm the histomorphological diagnosis. Histopathological review and clinicopathological correlation with the recorded patient information, including sex, age, laterality, and final diagnosis, was conducted.A complete review using the keywords ‘adrenal’ with ‘ganglioneuroma’, ‘schwannoma’, and ‘leiomyosarcoma’ was completed within the scope of the English language as available in PubMed/Medline databases. Primary references were read and analyzed, and their reference lists were used to retrieve secondary sources. PubMed’s ‘Related Articles’ feature was an additional tool in the identification of relevant articles. Additionally, a literature review on the current status of adrenal incidentalomas was undertaken.This study was conducted with ethics approval from the University of Saskatchewan Biomedical Research Ethics Review Committee.The surgical pathology review of 381,200 cases over the 13-year period (1997 to 2010) retrieved 94 adrenal lesions (incidence of 0.025%). Patient ages ranged from 4 to 85 years, with a mean of 51.6 years (median 55 years). The majority (54%) were between the ages 40 to 60. Fine-needle aspiration specimens were rare (4/94). Females (56.4%) had a slightly higher preponderance than males (43.6%). There was a slight predominance for left-sided lesions, with 55 (58.5%) pathologies detected in the left adrenal gland and 39 (41.5%) in the right in our series. Over three-quarters (76.6%) of the pathologies diagnosed were benign  with renal cell carcinoma as the commonest site of origin  followed by lung adenocarcinoma  and solitary cases  of colorectal adenocarcinoma, gastroesophageal adenocarcinoma, B cell lymphoma and urinary bladder carcinoma. Primary malignant lesions included seven adrenocortical carcinomas , three neuroblastomas  and one pleomorphic leiomyosarcoma   scintiscan, which were negative, ruling out pheochromocytoma. Urinary free cortisol and demonstration of a normal diurinal rhythm of cortisol secretion by the administration of the dexamethasone suppression test were also negative ruling out subclinical cortical functional tumors. A laparoscopic left adrenalectomy was carried out. Her recovery was uneventful, and she was discharged 48 h later.The left adrenal tumor was firm, tan, non-encapsulated and measured 3.5 × 2.2 × 2.4 cm in maximum dimension. On cross-sectioning, the mass was well demarcated with a stretched rim of normal-appearing adrenal tissue on either side Figure . On micrA 64-year-old non-smoker with uncontrolled hypertension (average 220/90 mmHg), poorly controlled type II diabetes, atrial fibrillation, depression and bilateral idiopathic blepharospasm underwent abdominal CT scanning for the investigation of abdominal wall swelling. A soft tissue mass on the medial aspect of the left adrenal gland measuring 3.1 × 2.7 × 4.1 cm was visualized incidentally. No additional mesenteric or retroperitoneal lymphadenopathy was identified, and the right adrenal gland, kidneys, spleen, and pancreas appeared normal. A renal Doppler ultrasound confirmed enlargement of the left adrenal gland, and further imaging with CT and MRI showed a solid enhancing mass with imaging features inconsistent with a benign adenoma. Based on his elevated blood pressure, and a 1-month history of biweekly episodes of sweating that lasted 5 minutes without associated chest pain, headache, shortness of breath or palpitations he was clinically suspected to have a pheochromocytoma. At two examinations, urine catecholamines were modestly elevated though metanephrines, vanillyl mandelic acid (VMA), homovanillic acid (HVA) and 5-hydroxyindoleacetic acid (5-HIAA) were normal on repeated testing. Due to the larger size of the tumor (4 to 4.5 cm), he underwent surgical exploration with a left laparoscopic radical adrenalectomy. Postoperatively his blood pressure remained stable and as there were no complications, the patient was discharged in 48 h.The specimen weighed 65 g and consisted of the left adrenal gland wrapped in mature adipose tissue. A large nodule was seen protruding adjacent to the adrenal gland, measuring 3.8 × 2.8 × 3.5 cm. Cut sections of the specimen revealed a well demarcated, fibrous mottled whitish-yellow lesion that grossly appeared to be directly adjacent to the adrenal gland with no direct involvement. On microscopic examination, the nodule was composed of (a) hypercellular areas of spindle cells with abundant eosinophilic cytoplasm, indistinguishable cell borders, and wavy nuclei with focal palisading areas Figure  and (b) A 28-year-old female who smoked half a pack of cigarettes a day with no significant medical history was being investigated for abdominal pain. A CT scan was ordered, which revealed a large left adrenal mass adherent to the kidney. Preoperative investigations including hormonal studies to evaluate catecholamine excess by the assessment of plasma metanephrines, urinary catecholamines and a MIBG scintiscan were negative, ruling out pheochromocytoma. Urinary free cortisol and demonstration of a normal diurinal rhythm of cortisol secretion by the administration of the dexamethasone suppression test were also negative ruling out subclinical cortical functional tumors. The larger size of the mass was highly suspicious for malignancy; therefore an open surgical intervention was planned. At surgery, via a left thoracoabdominal incision an en-bloc resection of the left adrenal mass was carried out including left nephrectomy, partial diaphragmatic resection with reconstruction and placement of a left thoracostomy. Postoperatively, the only significant complication was an asymptomatic left-sided pneumothorax that resolved spontaneously upon removal of the chest tube. She was discharged with an outpatient referral to the Cancer Center in 6 weeks. No further postoperative information is available for review in the current health record system.The gross specimen weighing 1,492 g consisted of the left kidney and adrenal gland, with portions of the diaphragm. A large 16.5 × 13.5 × 12.5 cm ragged, irregular, pale-tan rubbery multinodular mass with hemorrhage and necrosis was seen completely replacing the left adrenal gland with no residual normal tissue being identified. On microscopic examination, the tumor was surrounded by a pseudocapsule distinct from the adjacent renal parenchyma Figure  and compIn situ hybridization for Epstein-Barr virus was negative. A diagnosis of primary pleomorphic leiomyosarcoma was suggested due to the cells being strongly positive to SMA. The final diagnosis of high-grade primary pleomorphic leiomyosarcoma of the adrenal gland was confirmed with corroborative external consultation due to the rarity of this lesion.Immunohistochemical examination showed the neoplastic cells to be strongly positive for vimentin and smooth muscle actin (SMA) Figure  with negImprovements in non-invasive sophisticated abdominal imaging techniques coupled with their growing popularity and acceptance have resulted in the increasing detection of clinically silent adrenal lesions, ‘incidentalomas’ . Such trin vivo functional characterization of the adrenal glands due to the uptake and accumulation of the radio tracer. In this context, MIBG and N-59 scans for functioning adrenal medullary and cortical tissues, respectively, are undertaken to rule out any subclinical medullary/cortical functional tumors.An optimal diagnostic approach for the management of adrenal incidentalomas has yet to be determined; however, certain recommendations have been proposed ,2,10. A et al., preoperative CT and MRI tend to significantly underestimate the actual histological size of adrenal tumors larger than 3 cm by 18.1% and 20%, respectively. In this context, it is recommended that three-dimensional reconstruction of spiral CT or MRI images may overcome this problem of size underestimation [Preoperative radiological imaging including CT scans and MRI are used to determine size of the adrenal tumor, which dictates the need for surgical intervention and influences the surgical approach employed by the surgeon. However, as seen in the study by Kouriefs timation ,13. CT itimation . Similartimation . Additiotimation . The siztimation . Factorstimation . These ptimation . The firtimation . This prtimation .Cystic masses in the adrenal are rare and documented incidence varies between 0.064% to 0.18% in autopsy series ,18. FourThe following section focuses on the discussion of three rare lesions encountered as adrenal incidentalomas in our surgical pathology practice.Ganglioneuromas are benign, slow-growing neoplasms that arise from primordial neural crest tissue containing mature Schwann cells, ganglion cells, and nerve fibers . These tMRI and CT features vary between tumors due to the mixed composition of ganglion and Schwann cells . In thisDefinitive diagnosis of ganglioneuroma of the adrenal gland is only by microscopic examination. Ganglioneuromas are encapsulated on gross examination, with a solid, homogeneous gray-white cut surface as seen in Figure Schwannomas are slow-growing benign nerve sheath tumors that originate from neural crest cells and most commonly arise in the head, neck, upper and lower extremities, and trunk ,25. RareSchwannomas arising specifically within the adrenal gland are extremely rare ,29. It iOn gross examination adrenal/periadrenal schwannomas often appear as a solitary, well circumscribed tumor enveloped in a fibrous capsule with a solid firm white-tan and homogeneous cut surface that may contain cystic changes or hemorrhagic regions ,32. PeriLeiomyosarcoma is a mesenchymal malignancy of the smooth muscle that most commonly arises in the myometrium, retroperitoneum, and the respiratory tract . Primaryin situ hybridization of the tumor.The exact etiology of these neoplasms remains unclear. Immunocompromised patients including transplant recipients, those on chemotherapy and those with HIV/AIDS are predisposed to the development of smooth muscle tumors (SMT). They are also reported to have an increased risk for developing adrenal leiomyosarcoma . In thisOptimal management of this rare lesion recommends complete resection with a minimum of 3 cm margins, though this is often not feasible due to local invasion . Venous Primary adrenal pleomorphic leiomyosarcoma is an extremely rare lesion, with only three cases reported in the English literature as listed in Table Adrenal lesions are rare specimens in the surgical pathology laboratory. With increased and improved use of sophisticated diagnostic imaging techniques it is predicted that the number of adrenal incidentalomas detected will continue to rise. Laparoscopic or open surgical exploration of such lesions will result in increased specimens for pathological analysis. In this context, awareness of common and uncommon benign and malignant lesions of the adrenal medulla and cortex is vital for accurate pathological diagnosis to guide optimal patient management.The authors of this article have no relevant financial relationships with commercial interests to disclose.All authors participated in the creation and revisions of this manuscript. All authors read and approved the final manuscript.


Lymphoepithelioma-like carcinoma is a distinct entity among Epstein-Barr virus (EBV)-associated gastric carcinomas, characterized by the presence of a lymphoid stroma with small nests of cancer cells uniformly distributed throughout. Epithelioid granulomas with multinucleated giant cells are extremely rare in the tumor tissue. The presence of epithelioid granulomas with prominent lymphocyte infiltration is associated with a favorable prognosis. This lesion tends to form a bulging mass in the submucosa. In the present study, we report a lymphoepithelioma-like carcinoma of the stomach with epithelioid granulomas which appeared as a bulging mass in CT scanning and correlate it with the pathology. Clinicians and radiologists should recognize the features of such tumors. Lymphoepithelioma-like gastric carcinoma (LELC) is a rare type of gastric carcinoma with characteristic clinicopathological features –3. The mA 53-year-old Chinese male was admitted to Nanfang Hospital, Southern Medical University, Guangzhou, China, with a dull epigastric pain. Gastic endoscopy revealed a submucosal mass in the lesser curvature of the upper body with scattered ulcers on the mucosal surface . The labA contrast-enhanced CT of the abdomen using a CT scanner  was performed at the hospital. The contrast-enhanced CT scan and coronal reformatted image showed a bulging mass at the lesser curvature wall of the gastric upper body near the cardia, with a large tumor thickness-to-width ratio (see arrows on In situ hybridisation for EBV-encoded RNA-1 (EBER-1) revealed strong nuclear staining in the tumor cells, while the background lymphocytes were negative (Macroscopically, the resected specimen appeared as a 7.0×5.0×2.0-cm mass with ulcerations on the mucosa. Using light microscopy, a cross-section of the resected specimen at low magnification showed a circumscribed, superficially depressed ulcerated mass. The majority of the lesion was located in the submucosa . The tumnegative .EBV-associated gastric carcinoma is defined by the presence of EBV in gastric carcinoma cells and its absence in normal epithelium or dysplastic lesions . EBV-assLELC, which constitutes ∼4% of all gastric carcinomas, is a relatively rare type of gastric carcinoma, characterized by morphological features similar to undifferentiated nasopharyngeal carcinoma . It occuLELCs are characteristically accompanied by prominent lymphocyte infiltration, particularly in the submucosa. EBV is considered to be the main cause of the lymphocytic response ,14. The et al reported that EBV-associated gastric carcinomas had various appearances in a CT study, including focal mucosal thickening, marked wall thickening with contrast enhancement and bulky portions projecting from the gastric wall (Although LELCs have distinct clinicopathological features, they are not familiar to most radiologists. The CT appearance of LELCs with epithelioid granulomas has not been reported previously. In the present case, the advanced lesion was located in the gastric upper body near the cardia and appeared as a bulging mass with a large thickness-to-length ratio. The low-density stripe of the normal gastric wall abruptly terminated at the edge of the lesion. The majority of the lesion was revealed to be located in the submucosa by pathological investigation. It has been reported that the growth pattern with a larger thickness-to-length ratio is the characteristic appearance of EBV-associated lesions . Variousric wall . It is dric wall . HoweverIn summary, the CT findings of an LELC with epithelioid granulomas that developed in the gastric body revealed that it appeared as a bulging mass with abundant lymphoid stroma. The LELC tumor is a distinct entity associated with a good prognosis. The case in the present study is under follow-up at present. Therefore, understanding the radiological and clinical features of LELC is important in the preoperative diagnosis and in differentiating this entity from other tumors.


An asymptomatic issueless young staff nurse underwent pre-employment health screening and USG abdomen showed multiple hypodense lesions in liver. Further screening with whole body positron emission tomography-computed tomography (PET-CT) scan showed significantly FDG avid mass involving most of the right lobe of liver with multiple large FDG avid lymph nodal metastases. Unsuspected focal abnormal, FDG avid, hyperdense mural nodule was seen in uterus, which is the site of primary. With the advent of sophisticated medical instrumentation in the diagnostic workup for malignancies, detailed investigations fail to reveal the primary site of origin for a subset of patients with metastatic carcinoma. This is often referred to as Carcinoma of Unknown Primary (CUP) or occult primary malignancy.The exact incidence of CUP in the United States is not precisely known, but it is definitely underreported. Its actual incidence is most probably between 2 and 6%. In 15–25Clinical presentation of cancer of unknown primary origin is extremely variable and depends on the extent and type of organ involvement. Investigations are usually guided by any positive findings on initial evaluation. Patients have early dissemination of their cancer without symptoms at the primary site. The symptoms often depend on the site of metastases, like ascites may be the initial presentation in a patient with a GI or an ovarian malignancy, etc.The patient was an issueless, 32-year-old staff nurse undergoing pre-employment health checkup. USG abdomen showed a large heterogenous lobulated mass with predominant hypoechogenicity suggestive of possible atypical hemangioma or possible evolving abscess. Magnetic resonance imaging (MRI) of abdomen showed a large heterogenous enhancing hypodense lesion with altered signal intensity with a large exophytic component in the right lobe of liver suggestive of possible hepatic adenoma/atypical hemangioma/possible neoplasm. Fine needle aspiration cytology (FNAC) followed by trucut biopsy was suggestive of possible metastatic rhabdomyosarcoma.18F FDG (Fluoro Deoxyglucose) intravenously in euglycemic status. After 1 hour, the patient was imaged. Images showed large hypodense, significantly FDG avid, non-enhancing mass involving most of the right lobe of liver [Standard Uptake Value (SUV) max 30 g/ml]. Multiple large FDG avid, left axillary, porta hepatic, celiac and retropancreatic lymph nodal metastases were also present. Portal vein was seen encased by the tumor at porta hepatis. Inferior venacava was obstructed below the liver and collateralized via azygos and hemiazygous system. [Whole body positron emission tomography-computed tomography (PET-CT) scan was done using 8 mCi on  system. . Inferio system. .The patient underwent further pelvic USG and was biopsy proven. The patient was started on chemotherapy and is doing well for the past 6 months. Follow-up CT of abdomen showed recession in size of liver metastases.Rhabdomyosarcoma is primarily a disease affecting children; it rarely affects adults. Common sites of involvement are the head and neck 28%), extremities 24%), and genitourinary tract (18%).8%, extre Uterine %, and geMost uterine sarcomas fall into the category of leiomyosarcoma, endometrial stromal sarcoma, or undifferentiated sarcoma.5 MetastaRhabdomyosarcoma is subdivided into three general types histologically as follows.Embryonal rhabdomyosarcoma: This usually occurs in head and neck locations with small round or oval tumor cells and a finely granular eosinophilic cytoplasm. Well-differentiated tumors demonstrate elongated, strap-shaped or tadpole-shaped rhabdomyoblasts.Alveolar rhabdomyosarcoma is the next variety comprising relatively small, poorly differentiated round and oval cells aggregated into irregular clusters or nests separated by fibrous septa. An occasional variant, referred to as the botryoid type, shows a diffuse myxoid or mucoid matrix with thinly scattered primitive mesenchymal cells. The characteristic feature of this type is a peripheral zone of increased cellularity, sometimes known as the “cambium layer.”Pleomorphic rhabdomyosarcoma shows randomly arranged eosinophilic cells with considerable variation in cell size and shape, as well as variation in nuclear size and shape. The pleomorphic cells are often admixed with small, primitive mesenchymal cells. This tumor is often so undifferentiated that the identification of the cell of origin is difficult or impossible. Positive immunostains for desmin and myoglobin are helpful as in our case.Regardless of the histologic subtype, special stains are often quite useful for differentiating rhabdomyosarcoma from other neoplasms. The trichrome stain is especially useful because it colors rhabdomyoblasts bright red while myofilaments and cross-striations have fuchsinophilic properties, also highlighted by PTAH (deep purple color). Myxoid stroma may be positive for hyaluronidase with acid mucopolysaccharide staining, although many other tumors also have positive stroma with these stains. The most418F FDG PET-CT is an important screening tool for the evaluation of unknown primaries as well as for staging and follow-up of several malignancies.[In patients with metastatic disease, prognosis is poor with a 5-year event-free survival rate of less than 30%. 18F FDG gnancies.8 FDG PETOur patient was unique, as she was a young asymptomatic lady presenting with multiple large liver and nodal secondaries, which is relatively an uncommon site for metastases. The patient had no previous history of menorrhagia or metrorrhagia as usually expected in uterine rhabdomyosarcomas. Incidentally detected mural nodule in uterus helped in finding the site of hidden primary malignancy.