Lymphoma may occasionally involve the adrenal glands, but primary adrenal lymphoma (PAL) is very rare and only few cases have been reported. We present a case of a 60-year-old, otherwise healthy, woman, with bilateral PAL presenting with adrenal insufficiency. The patient responded initially upon administration of large doses of intravenously hydrocortisone with total remission of symptoms. An abdominal computerized tomography scan demonstrated bilateral adrenal lesions but did not demonstrate any other pathology. Since metastatic malignant disease was suspected a positron-emission-tomography scan was performed only showing significant uptake in the adrenal glands. Endocrine evaluation did not reveal abnormal function of any hormonal system and the patient was scheduled for bilateral adrenalectomy. However the clinical condition deteriorated rapidly and the patient was readmitted to hospital before surgery was performed. A new computerized tomography scan showed rapid progression of disease with further enlargement of the adrenal masses and both pulmonary and hepatic metastasis. Needle biopsy was performed but the patient refused further treatment and died before a diagnosis was obtained. The immuneohistochemical diagnosis was large B-cell lymphoma. This case should remind clinicians that PAL may be a cause of bilateral adrenal incidentaloma especially if the patient presents with adrenal insufficiency. Primary adrenal lymphoma (PAL) is rare whereas secondary involvement of the adrenal glands in nodal non-Hodgkin lymphoma occurs more often and is present in approximately 25% of cases .Primary extranodal lymphoma occurs in 1/3 of patients but primary involvement of the endocrine system is not frequent (3%) and most often involves the thyroid gland .The rare cases of PAL may present with bilateral (bPAL) involvement and is then more often histologically of diffuse large B-cell type. Immunodysfunction, as observed with human immunodeficiency virus infection or autoimmunity, may predispose to the disease but is not obligate .Adrenal insufficiency may be the primary symptom of presentation, especially with bilateral involvement as in bPAL .A high degree of suspicion is important in order to obtain a diagnosis quickly and initiate treatment since prognosis is poor.A 60-year-old woman previously diagnosed with systemic lupus erythematosus, but without symptoms of disease or need of treatment for the last 10 years, was admitted to our hospital due to a month's history of nausea, vomiting, fatigue, and fever. According to the patient an unintended weight loss of 5 kilograms had occurred. The patient took no prescribed medications and had no other medical history.Primary physical evaluation revealed a normal blood pressure and temperature. Clinical examination was unremarkable especially no lymphadenopathy, hepatosplenomegaly, or skin pigmentation was observed.9/L (145–390 × 109/L). Blood sugar was normal. Laboratory examination showed severe hyponatremia with a sodium level of 106 mmol/L (137–145 mmol/L) and a potassium level of 4.5 mmol/L (3.6–5.0 mmol/L). Liver parameters were slightly affected, coagulation factor II, VII, X was 0.55 (0.7–1.3), and lactate dehydrogenase was 292 U/L (105–205 U/L). C-reactive protein was 35 mg/mL (0.2–8 mg/mL) and the patient had slight thrombocytopenia 92 × 10 Due to the symptoms and the severe hyponatremia, adrenal insufficiency was suspected and an ACTH stimulation test was performed. The test demonstrated an insufficient response with an increase from baseline plasma cortisol of 222 pmol/L (190–600 pmol/L) to 239 pmol/L (>500 pmol/L) after 30 minutes and an elevated plasma ACTH of 75 pmol/L (2–11 pmol/L), indicating a primary adrenal insufficiency.The patient was treated with high doses of intravenous hydrocortisone and rehydrated with sodium chloride infusion resulting in complete remission of symptoms and normalization of biochemistry.Further biochemical evaluation showed no antibodies against the adrenal cortex. Plasma renin was 46 miU (8.8–36 miU) and plasma aldosterone was 38 pmol/L (38–490 pmol/L). Urine sodium was subnormal <20 mmol/L (50–150 mmol/L) and urine osmolarity was also low as expected 286 mmol/kg (>800 mmol/kg).A Quantiferon test ruled out Mycobacterium tuberculosis infection and phaechromocytoma was ruled out as well by the measurement of plasma metanephrine of 79 ng/L (0–170 ng/L) and normetanephrine <20 ng/L (0–72 ng/L). A contrast enhanced computerized tomography (CT) scan of the chest and abdomen revealed solid bilateral adrenal masses of 4 cm × 7 cm in the left adrenal gland and 6 × 3 cm in the right adrenal gland . Masses Adrenal metastasis from unknown primary cancer was suspected and a positron-emission-tomography scan was performed. The scan demonstrated significant uptake in the adrenal glands no primary tumor was found and no enlarged lymph nodes were detected.The patient preferred diagnostic adrenalectomy over needle biopsy and was referred to the Department of Urology in order to perform diagnostic bilateral adrenalectomy, but the clinical condition deteriorated and the patient was readmitted to our hospital before the surgical procedure could be performed. A new enhanced CT scan showed rapid progression of the adrenal masses as well as pulmonary and hepatic metastases. In order to be able to obtain a diagnosis and possibly treat the patient, a needle biopsy was performed. Initially a histological analysis was done on a frozen section in order to be able to offer some empiric treatment to the patient, but tentative diagnosis was too unspecific to offer chemotherapy. Subsequently the patient refused further treatment and died before a final pathological diagnosis was known. Pathological examination showed biopsy consisting of solid tumor tissue only, with no organ specific structures. The tumor cells were large with abundant basophilic cytoplasm and irregular nuclei, many with a nucleolus. The tumor cells expressed CD20, CD45, CD79a, Bcl-2, vimentin, CD38, and Kappa the diagnosis was thereby established as diffuse Large B-cell lymphoma . The proAn increasing number of adenomas in the adrenal glands are now diagnosed due to an increasing number of abdominal CT scans being performed. Incidentalomas are defined as tumors in the adrenal glands found by accident when performing diagnostic imaging for nonadrenal symptoms .Less than 5% of the incidentalomas are malignant and only 1 in 7 shows excessive production of any adrenal hormone . Incidentalomas are often unilateral .Diagnostic imaging with nonenhanced CT scans is used primarily to characterize the tumor. Low Hounsfield score, homogeneous morphology, and small size indicate that tumor is benign. Further evaluation with contrast enhanced CT of the abdomen and calculation of percentage wash-out can be used in need for further evaluation. Fine needle biopsy or adrenalectomy is used when a histological diagnosis is needed. If the tumor is hormone producing or if the tumor is large adrenalectomy is performed . The patLymphoma may spread to any part of the body and involvement of the adrenal glands in malignant lymphomas is reported in 25% of autopsies. Bilateral adrenal tumors often represent metastasis. Primary lung or stomach tumors are the cause in 50% of the cases and metastasis from lung, breast, stomach and lymphoma is the most common course of adrenal metastasis giving rise to adrenal insufficiency. PAL on its own is an extremely rare disease entity and less than 100 cases have been reported in the last 40 years .PAL is rare and often presents with bilateral tumor masses (70%) and can results in adrenal insufficiency. Survival time is short and a high degree of suspicion is needed in order to obtain a quick diagnosis. Advancing age, tumor size, level of LDHs and the presence of adrenal insufficiency are poor prognostic signs .Most patients with PAL have a limited time of survival. Complete remission of disease after initiation of chemotherapy have been described in a few patients, with a followup of 12 months in one patient without remission of disease and a followup of 7 years in another patient with no signs of remission –12.Primary adrenal lymphoma is rare but most often present with bilateral tumors and symptoms of adrenal insufficiency. A high degree of suspicion is needed in order to obtain a quick diagnosis since prognosis is extremely poor.


Intussusception is an uncommon cause of abdominal pain in adults and poses diagnostic challenges for emergency physicians, due to its varied presenting symptoms and time course. Diagnosis is thus often delayed and results in surgical intervention due to the development of bowel ischaemia. We report on a young patient who presented with an ileo-ileal intussusception in whom there were no underlying lesions identified as a causal factor. A 26-year-old male, with no prior medical history, presented to the emergency department with a 24-h history of bouts of severe colicky abdominal pain, worse in the left lower quadrant. The symptom had initially started with vomiting just prior to the abdominal pain. He subsequently developed some diarrhoea, further episodes of vomiting and began to feel unwell with a fever. He was unable to tolerate even oral fluids, which had prompted his presentation to the ED that morning. There was no episode of rectal bleeding. His temperature was 38°C, pulse of 120 beats per minute and a respiratory rate of 28 breaths per minute. He remained normotensive and maintained good oxygen saturations. Examination revealed a soft abdomen but gross tenderness in the lower quadrants, worse in the left iliac fossa. There was no palpable mass, and rectal examination did not demonstrate any blood. Bowel sounds were present and there were no clinical signs of peritonitis. After blood investigations were sent, he was treated with intravenous paracetamol, hyoscine butylbromide and intravenous fluids. He was sent for an abdominal x-ray, which revealed a single dilated loop of small bowel (3 cm) in the central abdomen with scanty bowel gas elsewhere . He had a raised lactate level of 3.5 mmol/l. Other blood tests illustrated raised acute inflammatory markers such as C-reactive protein of 231.7 ng/ml, and a white cell count of 15.9 × 109/l with a neutrophil count of 13.7 × 109/l. Given his extreme pain, fever and raised lactate level, a clinical diagnosis of intra-abdominal sepsis secondary to gut ischaemia was made and the patient referred to the surgical team. A preoperative CT scan of his abdomen revealed an ileo-ileal intussusception with several loops of dilated small bowel proximal to the intussusception  and a primary end-to-end anastomosis was performed. He was transferred to the intensive care unit postoperatively for optimisation. He made a good recovery and was discharged from the hospital 5 days later. Histopathology results of the removed specimen confirmed an intussuscepted segment of small bowel, which demonstrated a spectrum of changes from mucosal ischaemia/infarction to transmural haemorrohagic infarction. There was no evidence of malignancy or any other pathological trigger/nidus, and therefore the aetiology of his intussusception was unknown.Abdominal pain, which comprises about 5 to 10 percent of emergency department (ED) visits, continues to pose diagnostic challenges for emergency physicians because of the wide range of differential diagnoses, including gastrointestinal, gynaecological, genitourinary and cardiopulmonary causes .Intussusceptions occur when one segment of the gastrointestinal tract (intussusceptum) telescopes into the lumen of an adjacent distal segment of the gastrointestinal tract (intussuscipiens). Adult intussusceptions represent only about 5% of all intussusceptions  and thusIntussusception remains a rare clinical entity in adults. The mean age is 54.4 years, and the male-to-female ratio is 1:1.3 . This wiA classification system exists according to the location of the intussusception. The four types are ileo-colic, ileo-ileo-colic, colo-colic and small bowel intussusception  . In adulAbdominal CT is the most useful diagnostic tool not only for detecting an intussusception with a diagnostic yield of around 78%, but also helps in identifying the underlying cause . The CT Treatment is almost always surgical in adults when compared to children and invariably leads to resection of the involved bowel segment with subsequent primary anastomosis. Gastroduodenal and coloanal intussusceptions are extremely rare and may require innovative surgical techniques . IntermiAlthough, intussusceptions themselves have a good prognosis, it is often the nature of the lesion causing the intussusception on which the decisive factor is expected. Mortality for adult intussusceptions increases from 8.7% for the benign lesions to 52.4% for the malignant variety [Adult intussusception is a rare but well-recognized condition. A high index of suspicion and early diagnosis with a CT scan will identify patients requiring emergent surgery and thus prevent serious complications such as haemorrhage, intestinal gangrene and perforation.Consent was obtained from the patient for publication of this case report and accompanying images.The authors declare that they have no competing interests.SS - Wrote the first draft of the paper and coordinated the review of all the drafts. PM - Reviewed all drafts of the paper. TJ - Reviewed and commented on all the drafts of the paper and on all radiographic images. All authors read and approved the final manuscript.


Primary central nervous system lymphomas (PCNSLs) are mainly B-cells lymphomas. A risk factor for the development of PCNSL is immunodeficiency, which includes congenital disorders, iatrogenic immunosuppression, and HIV. The clinical course is rapidly fatal; these patients usually present signs of increased intracranial pressure, nausea, papilledema, vomiting, and neurological and neuropsychiatric symptoms. PCNSL may have a characteristic appearance on CT and MR imaging. DWI sequences and MR spectroscopy may help to differentiate CNS lymphomas from other brain lesions. In this paper, we report a case of a 23-year-old man with T-primary central nervous system lymphoma presenting with a mass in the right frontotemporal lobe. We describe clinical, CT, and MRI findings. Diagnosis was confirmed by stereotactic biopsy of the lesion. PCNSL causes approximately 3%-4% of all primary brain tumors. PCNSL is defined as lymphoma in the central nervous System (CNS) without primary tumor elsewhere. PCNSL is mainly diffuse large B-cell lymphoma; less common PCNSL histological types are Burkitt's lymphoma and T-cell lymphoma. Our case is about a 23-year old man with T-cell central nervous system lymphoma (T-PCNSL).A 23-year-old man was referred to our emergency room because of nausea, vomiting with associated acute confusional state. Neurological examination revealed slow response, postural instability without rigidity or tremor in any of the four extremities, and normal sensation. No remarkable abnormality was observed at the physical examination. Laboratory tests were within normal limits, in exception for LDH 61,1 mU/mL . Past medical history was negative for considerable pathologies. HIV status of the patient was positive. Computed tomography (CT) of the brain revealed a 6 × 5 cm mild hyperdense mass in the right frontotemporal region with associated perilesional oedema. Mild mass effect on the homolateral ventricle was observed with 1 cm midline shift. CT also revealed the presence of another similar lesion measuring 3,5 cm localized in the right cerebellar lobe . In the next, CT scan was integrated with brain MRI and single-voxel (1)H MR spectroscopy. Diffusion weighted imaging (DWI) showed hyperintense lesions at the right fronto-temporal area and at the right cerebellar lobe, respectively ; apparenMasses appeared mildly hyperintense also in T2-weighted MR images with peri-lesional oedema . T1-weigGadolinium-DTPA enhanced T1-weighted images revealed mild heterogenous enhancement throughout the masses . Single-voxel (1)H MR spectroscopy was then performed to evaluate metabolic information of the tumor. The (1)H MR spectra were obtained with long TE acquisition, a single-voxel point resolved spin-echo sequence for localization  and a three-pulse chemical shift selective saturation sequence to provide water suppression. (1)H MRS was characterized by a lactate/lipid peak, increased Choline/creatine, and reduced N-acetylaspartate/creatine ratios in the lesion . Staging of the disease was performed using CT scan with no distant metastases shown at different levels. μL, glucose 80 mg/dL (blood glucose 110 mg/dL), proteins 45.1 mg/dL (reference: 8–32 mg/dL), lactate 15.3 mg/dL (reference: 10.8–18.9 mg/dL), and IgG index 0,95 . Cerebrospinal fluid examination revealed white cell count 1/Stereotactic biopsy of the right frontal lobe was performed showing medium-sized lymphoid cells with a perivascular pattern. Immunohistochemical showed positive staining of CD3, CD8, and CD56 indicating that tumor cells were T-cells lymphoma in origin. EBV (EBER) status of the lesion was not included. Flow cytometry study of the peripheral blood and bone marrow supported the notion of no systemic involvement. The diagnosis was T-primary central nervous system lymphoma (T-PCNSL). In light of the lack of any potentially effective radiation option, the patient underwent chemotherapy with methotrexate at high dose of 3500 mg/mq and intrathecal administration. The radiation oncologists were consulted and proposed radiotherapy consolidation with a total of 45 Gy divided in 1.8 Gy/die on the entire brain, extended to C2. Primary central nervous system lymphomas (PCNSLs) are rare non-Hodgkin tumors without any evidence of systemic lymphoma. PCNSL represents 3%-4% of primary brain tumors and 1%-2% of all lymphomas. In most cases are diffuse B-cell lymphoma, T cell is very rare constituting of 1,7% of all PCNSLs [The overall incidence rate of PCNSL is 4 cases per million persons per year. The peak incidence is between 60 and 70 years old for immunocompetent patients. The male: female ratio is 1.5 : 1 , 4.Young age of the patient should be noted, because it is not typical for lymphoma. Significant increment of incidence rate over time is associated with increased incidence of AIDS and advanced age. Prominent risk factor for the development of PCNSL is immunodeficiency, which includes congenital disorders, iatrogenic immunosuppression and HIV , 6. PCNSL usually presents, solitary or multiple lesions mainly located at supratentorial level, usually in the periventricular regions, infiltrating the corpus callosum and the basal ganglia. Multiple lesions are reported in 38%–55% of non-AIDS PCNSLs. Multifocal intraparenchymal lesions without a dural involvement are very uncommon. Frontal lobe is affected in 20%–43% of PNCLs, brain stem, or cerebellum in 13%–20%. Other localitations are leptomeninges, spinal cord, and eyes . Our patient had multifocal lesions located in right frontotemporal lobe and in homolateral cerebellar lobe with dural involvement.Patients with PCNSL usually present with neurological and neuropsychiatric symptoms. Neurological examination reports pyramidal signs or sensory abnormalities associated with extrapyramidal symptoms, including rigidity, bradykinesia, and masked face. Headache is frequent (56%) as like as other signs of increased intracranial pressure, such as nausea (35%), vomiting (11%), and papilloedema (32%). Changes of personality, irritability, restlessness, and inappropriate behavior are common, since the tumor shows a tendency to be located at the frontal lobes . In the literature, multifocal tumors present symptoms at an earlier stage because multiple lesions are likely to have a larger effect than single lesions . CT has been the primary method for the evaluation of the PCNSL. Tumor results in hyperdensity, but it may also appear isodense . HoweverMagnetic resonance imaging offers several potential advantages in the evaluation of these lesions by using DWI sequences and MR spectroscopy for differential diagnosis. T-cell PCNSL usually appears with a subcortical distribution, peripheral nervous system involvement, and leptomeningeal spread. On unenhanced T1-weighted imaging, lesions are typically hypo- or isointense and on T2-weighted MR imaging iso- to hyperintense to gray matter . Most leTypical features of PCNSL are “notch sign,” an abnormally deep depression at the tumor margin and “open ring” enhancement. There could be mild or marked perilesional oedema . HemorrhBecause CNS lymphomas are highly cellular tumors, water diffusion is often restricted, making them appear hyperintense on DWI and hypointense on ADC maps . DiffereADC is inversely associated with tumor cellularity with lymphoma lower than glioblastoma .In addition to morphological MRI, (1)H-MRS provides noninvasively a wide spectrum of biochemical information with the lesion, which can be used for differential diagnosis of expansive lesions, estimation of the tumor type, and therapeutic response monitoring –14.In PCNSL, proton MR spectroscopy was characterized by predominance of lipid peaks combined with high Cho/Cr ratios and reduced N-acetylaspartate  1, 7, 1, 17, 15.These findings are reported on glioblastoma multiforme and some metastates with definitive diagnosis reliant on histopathology. Histology showed an infiltrate comprised of small, intermediate, or large-sized lymphocytes with surrounding plasma cells in a perivascular configuration with associated background reactive atrocities and Rosenthal fibers. Lymphocytes can show nuclear membranes irregularities, moderately dispersed chromatin, inconspicuous nucleoli, and perinuclear halo. Necrosis can be noted. On immunohistochemistry the lymphocites show positive staining for CD3, CD8, and CD56 but negative staining with CD2, CD5, CD7, CD4, CD20, and CD30. Unfortunately T-cell gene rearrangement study was not included. PCNSL has a 5-year survival rate between 4% and 40%. The clinical course is rapidly fatal if treatment is not promptly started. Surgery has not improved the survival, with an average of 3.5–5 months and a deterioration in the quality of life . The standard treatment for PCNSL has not been defined yet for the lack of adequate randomized studies. Retrospective series have shown a very significant survival advantage for the combination chemoradiotherapy. First-line chemotherapy consists in high-dose methotrexate followed by radiotherapy. This strategy allows a 5-year survival of 25%–40% versus 3%–24% with the radioboost alone . T-PCNSLs are extremely rare brain tumors that affect the elderly and immunocompromised patients DWI MRI sequences have some pathognomonic aspects which may help differential diagnosis between brain lymphoma and other glial tumors. Contrast-enhanced MRI can actually be considered the gold standard imaging technique. In addition to morphologic MRI, (1)H-MRS provides non-invasively a wide spectrum of biochemical information of the lesion. CT-guided biopsy, with immunohistochemical sample studies, should be thoroughly performed.


Littoral cell angioma (LCA) is a rare vascular tumor of the spleen. Generally thought to be benign, additional cases of LCA with malignant features have been described. Thus, its malignant potential seems to vary and must be considered uncertain. The etiology remains unclear, but an immune dysregulation for the apparent association with malignancies of visceral organs or immune-mediated diseases has been proposed.We report a case of LCA in a 43-year old male patient who presented with a loss of appetite and intermittent upper abdominal pain. Computed tomography showed multiple hypoattenuating splenic lesions which were hyperechogenic on abdominal ultrasound. Lymphoma was presumed and splenectomy was performed. Pathological evaluation revealed LCA.LCA is a rare, primary vascular neoplasm of the spleen that might etiologically be associated with immune dysregulation. In addition, it shows a striking association with synchronous or prior malignancies. With about one-third of the reported cases to date being co-existent with malignancies of visceral organs or immune-mediated diseases, this advocates for close follow-ups in all patients diagnosed with LCA. To our knowledge, this report is the first one of LCA associated with previous pulmonary sarcoidosis and hypothesizes a TNF-α related pathogenesis of this splenic tumor. Vascular tumors are the most common primary neoplasms of the spleen. Among these, LCA is a very rare tumor which arises from the littoral cells lining the sinuses of the splenic red pulp. The tumor displays a unique immunohistochemical phenotype of dual endothelial and histiocytic differentiation but is difficult to differentiate from other benign and malignant splenic lesions preoperatively. Thus, diagnosis is usually established after elective splenectomy. Currently, both etiology as well as biological behavior remain uncertain. Increasing numbers of LCA in association with autoimmune disorders or visceral organ tumors have been reported which hypothesizes an immunological association of this entity.A 43-year old male presented with non-specific clinical symptoms such as loss of appetite and intermittent upper abdominal pain which improved slightly with antacid medication. His medical history was unremarkable except for pulmonal sarcoidosis in his twenties which had been treated with corticosteroid medication. Both physical examination and laboratory tests were without pathological findings. An ulcerous lesion in the duodenum was detected gastroscopically and computed tomography was performed to exclude an external compressing tumor. CT scans Figure  did not Streptococcus pneumoniae, Haemophilus influenca B and Neisseria meningitidis, laparoscopic splenectomy was performed. The cut surface of the 12 × 8 × 4 cm specimen  is a rare vascular tumor that occurs exclusively in splenic tissue and was first described by Falk et al in 1991  to hyperSince the littoral cells have features intermediate between those of endothelial cells and macrophages, they show a hybrid endothelial-histiocytic phenotype on immunohistochemical staining. Expression of endothelial marker factor VIII-related antigen and also of histiocytic markers such as CD68 and lysozyme is thought to be characteristic for LCA ,6,7 and Generally thought to be benign, there are additional reports of LCA with malignant features which were divided into a low-grade variant  and theThe etiology of this neoplasm also remains unclear, but for its apparent association with visceral organ tumors and immune-mediated diseases in one-third of the reported cases to date -16, an eSince our patient had a history of pulmonal sarcoidosis, we concentrated on possible immunological links between these two entities.In sarcoidosis, a multisystemic granulomatous disorder of unknown origin, the inflammatory response is characterized by the increased production of several pro-inflammatory cytokines which mainly belong to the tumor necrosis factor family. Tumor necrosis factor-alpha (TNF-α) is considered the pivotal factor in the formation of granulomas by mediating inflammation and cellular immune response among the cytokines involved . TNF-α iLCA is a rare, primary vascular neoplasm of the spleen. Currently, both etiology and biological behavior remain unclear, but an underlying immune dysregulation has been proposed for LCA's association with malignancies of visceral organs or immune-mediated disorders in about one-third of the reported cases. Our case presentation supports the assumption of an association of LCA and an altered immune status, hypothesizing a TNF-α-related pathogenesis of this splenic tumor. Close follow-ups of patients diagnosed with LCA for subsequent development of additional tumors is mandatory.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.SC reviewed relevant literature and wrote the initial draft. MP reviewed the draft and contributed the CT scans. UT contributed the histological images. HP provided clinical expertise and reviewed the manuscript. MWH performed the surgery and reviewed the manuscript. All authors read and approved the final manuscript.


Tumors of the pancreas that contain substantial cystic components include mainly mucinous cystic neoplasm, intraductal papillary mucinous neoplasm, solid pseudopapillary tumor, and cystadenomas . Microcystic adenoma of the pancreas is a tumor that is benign in nature. Malignant transformation in the tumor with metastases is rare and only about 26 cases have been reported so far. Here we present a giant microcystic adenoma of the pancreas, possibly the largest ever malignant type in this group ever reported in the literature with extensive metastases to the liver and causing extensive compression and encasement on surrounding structures. Pancreatic cystic tumors fall into the serous or mucinous category. The latter most prominently shows malignant transformation in the long run, while the former is considered benign . MicrocyA 70-year-old woman, hailing from Kashmir in India, presented to us with progressively increasing abdominal mass since 11 years. This was associated with progressive appearance of jaundice and dull aching pain in abdomen since 3 months with pruritus for one month that worsened nocturnally. She neither complained of fever, bleeding diathesis, chest pain, cough or palpitations, and altered bowel habits nor did she suffer from anorexia. There was significant unintentional weight loss secondary to early satiety since last one year. There was no history of swelling of legs or puffiness of face during this period. There was no history of swelling over other parts of the body or associated skin changes. The patient is a known case of diabetes mellitus on oral hypoglycemic agents with adequate glycemic control since the last 20 years. Eleven years ago, the patient underwent an open laparotomy for complicated symptomatic gall stone disease and was incidentally detected to have a space occupying lesion of the head of pancreas. The lesion was highly vascular on intraoperative analysis and was left alone at that time with a fine needle aspiration cytology evaluation, which was later confirmed to be that of benign microcystic adenoma. The patient thereafter started noticing progressive enlargement of an epigastric mass which was initially seen 8 years ago. This mass progressed to involve the whole of the abdomen with associated lethargy and early satiety and difficulty in performing routine works. Multiple fine needle aspiration cytology of the mass lesion was done at various hospitals outside, before presentation to our facility. All those reports were noncontributory and confirmed the presence of only blood and cystic elements. On examination, at our center, the patient was found to be conscious and alert but emaciated with temporal hollowing and facial muscle wasting, with good performance status (ECOG-PS 0). Pallor was evident, as were icterus and scratch marks over the abdomen and arms. There was no cyanosis, clubbing, lymphadenopathy, or edema. The abdominal examination revealed the presence of a large epigastric mass, extending towards the right iliac fossa and trespassing on the left lateral aspect of umbilicus with a rough nodular surface, being firm in consistency, nontender, and without visible pulsations. A palpable bruit and a venous hum were also evident. There were areas of engorged, nontortuous veins over the stretched skin surface of the mass. No other organomegaly was appreciable and free fluid in abdomen was difficult to ascertain. The rest of the systemic examination was essentially normal. On further evaluation, the patient was found to have hemoglobin levels of 10 g/dL  with normal total leukocyte and platelet counts. The ESR was 30 mm in the first hours . The liver function tests revealed normal bilirubin levels with normal transaminases, in the presence of alkaline phosphatase of 326 IU/L  and gamma glutamyl transpeptidase of 228 U/L . The kidney function tests were normal and so were all the viral markers and autoimmune markers. The serum tumor markers, including CA19-9, CEA, AFP, tumoral Beta-HCG, Chromogranin A, and CA-125, were all within normal limits. Computed tomography imaging of the abdomen subsequently followed by angiographic studies in triple phase and magnetic resonance imaging in triple phase revealed the presence of a large lobulated heterogenous mass in the retroperitoneum extending from the undersurface of the liver to the whole of lower abdomen Figures . The masMultiple hypodense septation (which was T2 hypointense) was noted within the heterogenous mass. Contrast administration revealed moderate heterogenous enhancement with enhancing septation. Compression and displacement of structures at the porta and compression of common bile duct with upstream dilatation of the CBD and bilobar intrahepatic biliary radical dilatation were also evident. The CBD measured 2.7 cm. The inferior vena cava was compressed posteriorly with displacement of the bowel laterally. The tumor derived its supply mostly from the celiac axis with extensive tumoral angiogenesis . MultiplThese cells also showed features of monomorphic round hyperchromatic nuclei and moderate amount of clear cytoplasm with inconspicuous nucleoli. The intervening stroma contained several vascular channels with hyalinised stroma. The tumor cells were positive for CK-7, SMA, CD34, and focal EMA. They were negative for focal S-100, CD10, HMB45, MUC1/2, and calretinin Figures . The glyWith these clinical, imaging, and histopathological features, a diagnosis of microcystic adenoma was made and the carcinomatous transformation was confirmed by the presence of liver metastases. The patient was not a candidate for surgical or interventional therapy and hence was managed medically for symptom relief. According to the patient's family's wishes, she was then allowed to follow up at her hometown at a nearby center and was lost to follow-up from our side.Cystadenomas of the pancreas are mainly microcystic, oligo- or macrocystic, and solid serous in type. The cystadenomas are almost always considered benign . The malCalretinin, CEA, chromogranin, MUC1, MUC2, S-100, HMB-45, CD-31, and vimentin are usually negative . In our


Epitheloid leiomyoma is a very rare subtype of benign mesothelial tumors of the bladder. A 46-year-old female patient presented to our hospital with prolonged dysuria, frequency, and recurrent urinary tract infections. Bimanual examination revealed a mobile, round mass in bladder. There was a round hyperdense intravesical mass near bladder neck in computed tomography (CT) scan that was compatible with her magnetic resonance imaging (MRI). A well defined 3 × 4 centimeter mass was seen in superolateral part of bladder neck during cystoscopy. The patient underwent partial cystectomy and histopathologic findings confirmed the diagnosis of epithelioid leiomyoma. The patient's followup was uneventful in a period of 2 years. Size and anatomic location of this tumor were major factors that affect on treatment. Benign mesothelial tumors are rare cause of bladder neoplasms , 2. LeioA 46-years-old woman presented to our hospital with complaining of dysuria, frequency, and recurrent urinary tract infections from one year ago. She had no hematuria, suprapubic pain, or family history of such problems. She had history of hysterectomy three years before this admission due to uterine leiomyoma. Physical examination revealed a mobile, round mass in bimanual examination. Abdominal ultrasonography revealed a round mass measuring 4 × 5 cm . There wShe underwent exploratory laparotomy with low midline incision. The mass was identified in the posterior aspect of the bladder wall so partial cystectomy was performed for her. The pathology of mass was epithelioid leiomyoma. The postoperative period was uneventful, and the patient was discharged from hospital after 4 days.The patient's followup with clinical examination, abdominopelvic CT scan, and cystoscopy was normal in period of 2 years. Benign bladder tumors consist of myomas, fibromyomas, leiomyomas, rhabdomyomas, fibromas, angiomas, myxomas, and osteomas. The most common histologic type of benign bladder tumor is leiomyoma which does not have any predilection for gender or age . In 1994In imaging such as intravenous urography (IVU) or cystourethrogram usually a smooth filling defect in the bladder was identified. Abdominal ultrasound may be helpful in differentiating cystic lesion from solid one , 9. FernThese tumors are easily visualized in cystoscopy that covered the normal bladder mucosa . In histThe pathophysiology of leiomyoma of the bladder is unclear. However, four major theories have been proposed for this tumor: 1) inflammatory resection of the bladder wall to an infection of the bladder musculature; (2) metaplastic reaction around the perivascular walls from vascular inflammation of the bladder; (3) neoplasm controlled by hormonal influences; (4) dysontogenesis: the result of embryologic rest within the bladder wall developing into a smooth muscle tumor. Despite of mysterious cause of it, the diagnostic examination and surgical management are easy  inflamma.The treatment of this rare condition is primarily determined by its size and anatomic location . In GoluIn our patient, irritative voiding symptoms were predominant and intramural lesion was diagnosed by imaging modalities. The normal bladder mucosa over the mass confirmed intramural situation of lesion. A straightforward partial cystectomy was performed for her.


Background. To our knowledge, this is the sixth reported case in the literature of fourth ventricular schwannoma. The etiology and natural history of intraventricular schwannomas is not well understood. A thorough review of potential etiopathogenic mechanisms is provided in this case report. Case Description. A 69-year-old man presented with an incidentally found fourth ventricular tumor during an evaluation for generalized weakness, gait instability, and memory disturbance. Magnetic resonance imaging (MRI) revealed a heterogeneously enhancing lesion in the fourth ventricle. A suboccipital craniotomy was performed to resect the lesion. Histopathological examination confirmed the diagnosis of schwannoma (WHO grade I). Conclusions. Schwannomas should be considered in the differential diagnosis of intraventricular tumors. Although the embryologic origins may be different from nerve sheath-derived schwannomas, the histologic, clinical, and natural history appear identical and thus should be managed similarly. Intraventricular schwannomas are rare tumors with only 17 cases reported in the literature . The etiWe present a rare case of fourth ventricular schwannoma and provide a review of the current literature.A 69-year-old man presented with progressive worsening generalized weakness, gait instability, and memory disturbances. His neurological examination was unremarkable.An MRI with contrast of the brain revealed a 1.8 cm × 1.8 cm × 2.0 cm heterogeneously enhancing lesion in the posterior fourth ventricle . Given tA standard suboccipital craniotomy with telovelar approach was used to access the tumor. A firm and yellowish appearing tumor originating from the floor of the fourth ventricle was encountered. The inside of the tumor, however, was purple in color. The tumor did not appear to arise from any of the lower cranial nerves, and actually appeared to arise from the area postrema on the right side. A complete microsurgical resection was performed. Microscopic examination showed a spindle cell neoplasm characterized by Antoni A and Antoni B areas with Verocay bodies characteristic of schwannomas . Areas oIntraventricular schwannomas are very rare tumors with only 17 previously reported cases in the literature  see Tab. The vasThe natural history and growth patterns of the intraventricular schwannoma, unlike its extra-axial counterpart, are not completely understood given the rarity of its occurrence. Based on the previously reported literature, this neoplasm appears to assume a benign course with no recurrence when there is no histological evidence of malignancy. Barbosa et al. described a case of cystic intraventricular schwannoma without recurrence for 10 years after resection .Similar to other intracranial schwannomas, intraventricular schwannomas radiographically demonstrate heterogeneous or homogeneous contrast enhancement with cystic components and occasional calcification. It is often not considered in the differential diagnosis of fourth ventricular tumors due to the rarity of occurrence. It is usually difficult to identify intraventricular schwannomas on neuroimaging because it lacks specific imaging characteristics to differentiate them from other intraventricular tumors , 9, 16. As reported in 5 other cases of fourth ventricular schwannomas, a midline suboccipital craniotomy was implemented for surgical access to the lesion. Most of these tumors arise from the ventricular floor, but in 2 reported cases, the tumor appeared to originate from the roof of the fourth ventricle , 12. In The histological appearance of intraventricular schwannomas is similar to the commonly encountered extra-axial schwannomas, characteristically demonstrating spindle cell architecture with Antoni A and B areas. Antoni A areas are highly cellular with nuclear palisading and associated Verocay bodies. Antoni B areas consist of loosely organized tissue with myxomatous and cystic changes. The proliferation marker MIB-1 is typically low, although focal areas of high MIB-1 activity have been detected in some neuropathological specimens of intraventricular schwannomas . ImmunohDespite this similar histological appearance, intraventricular schwannomas and extra-axial schwannomas may differ in their source of origin. It is well accepted that extra-axial schwannomas are derived from the myelinated cell of the peripheral nervous system . SchwannInterestingly, the exact source of origin for intraventricular schwannomas is currently not known, as there are several proposed etiopathogenic mechanisms. One such theory is neoplastic transformation of neural crest cells that were displaced during embryogenesis , 15, 16.Another hypothesis is transformation of autonomic nervous tissue accompanying intrinsic arteries and choroid plexus , 15, 16.Another proposed theory is schwannomas arising from aberrant peripheral nerve fibers. This proliferation may arise from localized traumatic injury; for example, focal axonal proliferation in the pons adjacent to infarcted brain tissue  or traumOthers have proposed transformation of multipotent cells into Schwann cells and proliferation of foci of Schwann cells (“schwannosis”) as potential etiologies , 7, 16. Lastly, there is now much evidence that cancer stem cells may be responsible for maintenance and growth of tumors. The cancer stem cell hypothesis centers on the idea that tumors arise from a small subpopulation of stem cells, which have the ability to self-renew, produce daughter tumorigenic cells, and can give rise to nontumorigenic cancer cell phenotypes. Stem cells, for example, have been detected in neural tumors, including glioblastoma, medulloblastoma, and ependymoma , 19. TheFourth ventricular schwannomas are rare. Although their etiopathologic origin is different from extra-axial schwannomas, their imaging, histology, ability to achieve a gross total resection, and clinical course appear identical with other schwannomas and should be managed similarly. Although rare, schwannomas should be considered in the differential diagnosis of contrast-enhancing fourth ventricular lesions in both children and adults.


A 53-year-old Taiwanese male had several episodes of left epididymitis with hydrocele refractory to antibiotic treatment. Partial epididymectomy plus preventive vasectomy were planned, and, incidentally, an ill-defined nodule was found lying on the tunica vaginalis near the epididymal head. The pathological diagnosis was malignant mesothelioma of the tunica vaginalis testis. Radical orchiectomy with wide excision of the hemi-scrotal wall was performed. So far, there is no evidence of recurrence after more than 3 years of follow-up. Malignant tumor should be considered in the case of recurrent epididymitis refractory to empirically effective antibiotic treatment. Although the nature of this tumor is highly fatal, the malignancy can possibly be cured by early and aggressive surgical treatment. Malignant mesothelioma of the tunica vaginalis testis is a rare tumor. Since the first case report, fewer than 100 cases have been reported to date. Most ofAbout 3 years ago, a 53-year-old man was referred to our hospital with the chief complaint of a repetitive painful nodule over the left scrotum. He had visited a local medical clinic and had been treated with empirical antibiotics with the diagnosis of epididymitis. The patient was sexually active and reported no lower urinary tract symptoms. He denied any history of exposure to asbestos or scrotal trauma. First, scrotal ultrasonography was arranged to rule out an abscess. The ultrasonographic pictures FigureA showed One year later the patient returned with the problem of several episodes of scrotal pain and recurrent epididymitis. This time, antibiotic therapy had a limited therapeutic effect, and the inflammatory symptoms fluctuated, but never subsided. Thus, we decided to perform partial epididymectomy plus a preventive vasectomy. Prior to surgery, follow-up scrotal ultrasonography demonstrated a hypoechoic lesion of about 0.5 cm in size over the left epididymal head FigureB, and inThe pathological report showed unusual neoplastic change of the tunica vaginalis. Under microscopic examination, the nodule showed a picture of malignant mesothelioma, well-differentiated, accompanied by tubulopapillary morphological features. These tumor cells were rounded or a cuboidal pattern with nuclear atypia, prominent nucleoli and a diffuse infiltrating pattern FigureA. By immOnce the pathologic diagnosis was established, complete workup of the tumor staging was arranged. By computerized tomography, no evidence of local invasion or metastasis in the adjacent lymph nodes was demonstrated. Subsequently, radical orchiectomy with wide excision of the hemi-scrotal wall was performed. The patient was discharged and so far (after over 3 years of follow-up) there is no evidence of local or distant recurrence.Malignant mesothelioma of the tunica vaginalis testis is an uncommon disease and is usually described as originating from the pleura, pericardium, and peritoneum. Malignant mesothelioma of the tunica vaginalis testis is extremely rare. Since the first case was described in 1957, about 1The main clinical symptom of these patients with malignant mesothelioma of the tunica vaginalis testis is a painless scrotal mass with hydrocele. Diagnosis is mostly made intraoperatively or postoperatively owing to the nonspecific symptoms and the absence of a tumor marker before surgery. By reviewing evidence obtained by searching PubMed for articles on malignant mesothelioma of the tunica vaginalis testis between 1998 and August 2012,8-33, anIn general, there are several causes of epididymal inflammation: infection, trauma, autoimmune disease, vasculitis, and idiopathic. The pathophysiology of epididymitis remains unclear, although it is postulated to occur secondary to retrograde flow of infected urine into the ejaculatory duct. Epididymitis is characterized by inflammation of the epididymis presenting as pain and swelling, generally occurring on one side and developing over several days. Multiple objective findings of epididymitis have been identified and, in variable degrees may include positive urine cultures, fever, erythema of the scrotal skin, leukocytosis, urethritis, hydrocele, and involvement of the adjacent testis. The infBecause there is no precise diagnostic modality available, several methods have been applied for early detection prior to surgery. The most popular diagnostic tool is scrotal ultrasound. A series of sonographic features in scrotal mesothelioma were reviewed for preoperative diagnosis. In addition to hydrocele being commonly presented, scrotal mesothelioma could be associated with an extratesticular mass with atypical sonographic features. Color DTreatment for malignant mesothelioma of the tunica vaginalis testis is still not established. Some authors have adopted the treatment of pleural mesothelioma for patiIn this case report, we have demonstrated that malignant mesothelioma of the tunica vaginalis testis might mimic epididymitis. We also proposed a hypothetical correlation between epididymitis and paratesticular malignant mesothelioma. Malignancy should be considered in the differential diagnoses, especially in cases of recurrent epididymitis refractory to empirically effective antibiotic treatment. Definitive diagnosis warrants surgical exploration. Once a pathological diagnosis is confirmed, radical orchiectomy with hemiscrotectomy should be performed as soon as possible. Although the nature of this tumor is highly fatal, the malignant disease could be potentially cured by early and aggressive surgical treatment.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.CHY and HCL were responsible for delivering patient care. CTL and CJS provided opinions in diagnosis and treatment. CHY contributed towards to drafting the manuscript while HCL provided overall supervision and edited the final version of the manuscript. All authors read and approved the final manuscript.Division of Urological Surgery, Department of Surgery, Song-Shan Armed Forces General Hospital, Taipei, Taiwan.


The common presentations of lymphoma are widespread lymphadenopathy or development of constitutional symptoms. This paper presents a case of a patient who presented with a solitary mass detected on chest X-ray and underwent FDG-PET for further evaluation of this mass. FDG-PET is a commonly utilised technique to assess solitary nodules as it not only allows characterisation of the lesion but can also detect nodal and extra-thoracic disease with greater accuracy than the standard CT. In this case, FDG-PET demonstrated abnormal activity in the lung nodule and at the gastro-oesophageal junction. Biopsies confirmed Non-Hodgkin’s Lymphoma at both sites. The value of FDG-PET in this case was the determination of previous unsuspected disease in an unusual presentation of lymphoma and as a useful tool for monitoring the therapeutic effect post chemotherapy. FDG-PET:is of clinical benefit in the assessment of the solitary pulmonary nodule;can often detect occult metastatic disease; andis a sensitive modality for monitoring therapeutic efficacy in lymphoma.A 71-year-old lady had an incidental finding of an opacity in her left lower lobe on a chest radiograph. Thoracic CT confirmed the presence of a 1.0 cm solitary pulmonary nodule but did not identify any lymphadenopathy or evidence of distant metastatic disease.The patient was referred for an F18 FDG-PET study  for inveThe patient underwent upper gastrointestinal endoscopy. Biopsy of the gastric wall during endoscopy revealed non-Hodgkin’s lymphoma (NHL). The patient proceeded to thoracotomy for investigation of her lung lesion to definitively ascertain the nature of this lesion. Pathology again confirmed lung NHL. Therefore, the patient was treated with systemic chemotherapy for lymphoma.A follow-up post therapy FDG-PET study  showed aNon-Hodgkin’s lymphoma is a known but relatively rare cause of solitary pulmonary nodules . GastroiFDG-PET is used throughout the world in the assessment of solitary pulmonary nodule. It has been shown to have high sensitivity >95%) and high specificity (>75%) in determining whether the nodule is benign or malignant based on high uptake in the lesion [% and higFDG-PET in this case also showed a total metabolic response to treatment following appropriate chemotherapy. In the authors' institution, post-therapy assessment in NHL is one of the most common indications for FDG-PET. The findings may be of prognostic significance as total metabolic response has been associated with improved long term outlook in NHL . FDG-PETThis case thus illustrates the usefulness of FDG-PETas teaching points:in the assessment of the solitary pulmonary nodulein the detection of previously occult disease andas a sensitive modality for monitoring therapeutic efficacy in lymphoma.


Trichobezoar is a rare condition that may pose a diagnostic challenge. Patients with this condition often have an underlying psychiatric illness, and history may not be easily forthcoming. The condition should be entertained especially in young females. Delay in diagnosis may lead to futile complications. We report a classic case of trichobezoar in terms of patient profile, presentation, and investigative findings. Badzehr, which means antidote [trich which means hair [A bezoar is a mass of undigested material within the gastrointestinal tract. The term bezoar derives from the Arabic word antidote . Bezoarsantidote . Trichobans hair . A trichans hair , 5. In uans hair . Trichobans hair . The sitans hair .A 16-year-old female was referred to our surgical services with a problem of epigastric pain associated with an epigastric mass. The patient resided in a local children's home due to poor social circumstances. On questioning, patient reported nonspecific pain of approximately 3 months duration with no dyspeptic symptoms; the patient reported no early satiety and no history of weight loss. Examination revealed a well looking girl, with a nondistended abdomen. Palpation of her abdomen revealed a large, firm mobile epigastric mass that was minimally tender. Ultrasound and Computed Tomography (CT) scan of the abdomen both confirmed the presence of a large gastric mass with internal air loculi involving the entire stomach with extention into the duodenum but not into the jejunum . At endoTrichobezoars commonly occur in adolescent females, often with an underlying psychiatric or social problem. Clinical presentation of these patients may be confusing as often they are not forthcoming with a history of trichophagia either due to embarrassment or the unintentional nature of the problem. Although this is a rare condition, numerous case reports and series have been reported as high mortality may follow complications associated with this condition. Trichobezoars in humans were first described from a post mortem by Swain in 1854 . The posPresentation ranges from nonspecific abdominal or epigastric pain, to a range of complications as mentioned. Clinical examination often reveals a large mobile epigastric mass that may be indentable, the so-called Lamerton's sign . EndoscoManagement options include endoscopic removal, laparoscopic removal, or via laparotomy. Gorter et al., in a retrospective review of 108 cases of trichobezoar, evaluated the available management options ; it was Trichobezoars should be considered as a differential diagnosis in a young female patient with a mobile epigastric mass. Diagnosis can be easily made with the use of CT scan and endoscopy. Management almost always requires surgical removal. It is emphasized that the majority of these patients have an underlying psychiatric or social disorder. A multidisciplinary approach is essential to prevent recurrence of the problem.


A 59-year-old woman was referred with nausea, vomiting and generalized abdominal pain after colonoscopy. She is a known case of amyloidosis for 10 years that resulted in renal failure. Amyloidosis is a rare disease in which extracellular fibrillar proteins deposit in tissues and organs interfering with the normal functioning of the affected organ.This disease is defined based on the biochemical nature of fibril proteins. The collection of abnormal proteins may be localized in one organ or may be systemic and involve many organs of the body. Secondary amyloidosis refers to a group of diseases in which a composition of the acute phase reactant serum amyloid A protein accumulates in tissues and organs in association with chronic inflammatory disease. AlthoughThe gastrointestinal tract  is usually involved by systemic amyloidosis and deposition of amyloid in the intestinal wall leads to a variety of signs and symptoms such as abdominal pain, dysmotility, pseudoobstruction, GI bleeding and malabsorbtion. However, small bowel infarction and perforation is also a rare complication of intestinal involvement. It seemsSome authors believe that because of the poor prognosis of spontaneous intestinal perforation in amyloidosis and the fact that the same results are achieved in conservative and operative management, surgical procedure should be limited to acute abdomen.5]5]Our case was a 59-year-old woman with a history of secondary amyloidosis on an underlying psoriasis disease for 10 years who presented with severe generalized abdominal pain, nausea and vomiting 12 hours following colonoscopy. This procedure was performed as the standard procedure for determining the cause of anemia in this patient after positive fecal occult blood exam. She had undergone living donor renal transplantation because of end stage renal disease 2 years ago.On physical examination, she had unstable vital signs  and fever. Abdominal examination revealed generalized abdominal tenderness and there was an abdominal wall hernia near the transplant kidney scar.Laboratory tests showed normal limit levels except for leukocytosis .Ultrasound showed a small amount of free fluid in the peritoneal cavity with mildly dilated bowel loops. There was a fascial defect near the recent surgical scar and fluid-filled small-bowel loop and a small amount of free fluid was seen in this hernia.Plain abdominal graphy showed dilatation of bowel loops with air in the subdiaphragmatic and retroperitoneal spaces.Contrast enhanced abdominopelvic CT scan revealed dilatation of the bowel loops  with pneumatosis intestinalis and free air in the retroperitoneal, intraperitoneal and posterior mediastinal spaces with hepatic portal venous gas. In addition, atrophic native kidneys and transplant kidney in the right pelvic cavity and adjacent incisional hernia containing bowel loops were evident. Bilateral pleural effusion and ascites were detected.According to the above mentioned findings, spontaneous perforation due to bowel necrosis secondary to amyloidosis or perforation following colonoscopy were our differential diagnoses.In this case, regarding the history of recent colonoscopy, it was doubtful that perforation was due to bowel wall involvement by amyloidosis or occurred after the colonoscopy procedure; however, the second diagnosis was more probable.As a result of this finding and the unstable condition of the patient, conservative treatment was started initially before surgery, but the patient did not tolerate and unfortunately expired after a few hours.This case was interesting because of not only the rarity of secondary amylodosis due to psoriasis, but also severe probable involvement of the bowel loops by the disease and perforation following colonoscopy (not primary).


The authors report on the case of a 67-year-old man with longstanding breathlessness, which was eventually attributed to a fixed mass in the upper third of the trachea causing upper airway obstruction. The lesion was amenable to loop electrocautery resection via flexible bronchoscopy that led to prompt resolution of patient symptoms. Biopsy was consistent with tracheal hamartoma, an exceedingly rare benign tracheal tumor. All the cases of tracheal hamartomas in the literature to date, the application of electrocautery and other methods of interventional bronchoscopy for resection of selected tracheal tumors are discussed. Primary tracheal tumors are rare in adults and usually malignant (80–90%). Reports regarding the histological distribution of the benign tracheal tumor are scarce, and little information is available about their natural history and behavior. The rarity of cases results in low levels of suspicion among physicians thus leading to substantial delay in correct diagnosis and treatment , 2. A 67-year-old exsmoker man presented with a 5-year history of progressive breathlessness on exertion and no other associated symptoms whatsoever. He had been empirically treated as for chronic obstructive lung disease in the past without any improvement.Flow-volume loop test revealed a variable extrathoracic airway obstruction . Chest XThe patient underwent flexible bronchoscopy under local anesthesia following an oral approach which demonstrated a white pendulated tumor covered with smooth mucosa attached with a narrow base of 2-3 mm to the right antero-lateral tracheal wall between the second and the third cartilaginous rings form the vocal cords. The lesion was obliterating the tracheal lumen by 80%. An electrocautery loop was passed through the bronchoscope, positioned around the base of the tumor, and tightened to gather the tissue at the point of its attachment to the tracheal wall. The tumor was completely resected by delivering electrocautery. Following resection, the tumor remained attached to the electrocautery loop, and it was removed with the simultaneous withdrawal of the bronchoscope, the electrocautery loop, and the hamartoma en bloc. The procedure was well tolerated, without hemorrhage from the tumor base or other complications, and offered the patient immediate normalization of his breathing and resolution of his stridor . HistoloThe patient was discharged the next day being completely asymptomatic. A repeat flow-volume loop at three months showed normalization of the inspiratory limb. There had been no recurrence of dyspnea during that period .Hamartomas are the most common benign tumor of the lung. Grossly, they occur endobronchially  in about 10% of the cases and intraparenchymally in the remaining 90% . Endotracheal hamartomas are excitingly rare amongst other benign tracheal tumors. On reviewing the PubMed database, approximately 20 cases of tracheal hamartoma have been reported from 1959 onwards, including cases in adults and children. One case of each of the followings was found: an endotracheal hamartoma coexistent with an intrapulmonary hamartoma , an endoTracheobronchial hamartomas commonly manifest with varying degree of respiratory distress, depending on the degree of luminal obstruction, but manifestations ranging between episodes of pneumonia and acute onset of respiratory failure, cough, hemoptysis, chest pain, and paucity of symptoms have been described –11. MacrTracheal tumors are amenable to direct visualization and sufficient sampling by flexible bronchoscopy. Surgical resection has been the treatment of choice in malignant tracheal tumors and benign lesions invading the tracheal wall or attached to the tracheal lumen with a broad base . When deWhen added to other published data, this case report of tracheal hamartoma brings the total reported number of this rare localization of a common condition to only around twenty. It also highlights the challenges relating to the timely correct diagnosis of central airway lesions. Based on our experience from the management of this case and on reviewing the relevant literature, we propose that, in the hands of an experienced manipulator, bronchoscopic techniques such as electrocautery may serve as the method of choice for the resection or debulking of selected endotracheal tumors in certain patients; they are safe and straightforward techniques, provide immediate effect, are cost-effective, increase patient satisfaction, and reduce length of hospital stay.


P < 0.01). While the radiographic diagnosis of lipoma or well-differentiated liposarcoma cannot be made with 100% certainty, experienced observers have a 69% chance of rendering a correct diagnosis.Well-differentiated liposarcoma represents a radiographic diagnostic dilemma. To determine the accuracy, interrater reliability, and relationship of stranding, nodularity, and size in the MRI differentiation of lipoma and well-differentiated liposarcoma, MRI scans of 60 patients with large (>5 cm), deep, pathologically proven lipomas or well-differentiated liposarcomas were examined by 10 observers with subspecialty training blinded to diagnosis. Observers indicated whether the amount of stranding, nodularity, and size of each tumor suggested a benign or malignant diagnosis and rendered a diagnosis of lipoma or well-differentiated liposarcoma. The accuracy, reliability, and relationship of stranding, nodularity, and size to diagnosis were calculated for all samples. 69% of reader MRI diagnoses agreed with final pathology diagnosis (95% CI 65–73%). Readers tended to err choosing a diagnosis of liposarcoma, correctly identifying lipomas in 63% of cases (95% CI 58–69%) and liposarcomas in 75% of cases (95% CI 69–80%). Assessment of the relationship of stranding, nodularity, and size to correct diagnosis showed that the presence of each was associated with a decreased likelihood of a lipoma pathological diagnosis ( Well-differentiated liposarcoma presents a diagnostic dilemma to the treatment team due to the inaccuracy of biopsy and the difficulty in differentiating these low-grade tumors from benign lipomas on imaging alone –4. This MRI serves as an excellent modality to discern certain soft tissue tumors like lipomas given their specific imaging characteristics , 3. Low-Several authors have evaluated the sensitivity and specificity of specific MRI sequences in the diagnosis of well-differentiate liposarcoma; however, the diagnostic reliability between specialties  has not been well characterized –4, 10. IThe purpose of this study was therefore to evaluate the accuracy, reliability, and association between the variables of size, stranding, and nodularity in the diagnosis of lipoma versus well-differentiated liposarcoma based on MRI imaging alone. In addition, the accuracy and reliability of trained musculoskeletal radiologists and orthopaedic oncologists in the radiographic diagnosis of these tumors were assessed to see if any differences existed between specialties. Our hypothesis was that experienced readers in both specialties blinded to diagnosis would be unable to differentiate between a series of fatty lesions and that the variables of size, nodularity, and stranding would show no predictive association with these diagnoses. If true, this study would support the argument to treat all low-grade fatty lesions with marginal excision regardless of the MRI interpretation or concern regarding variables of size, nodularity, or stranding.After research ethics board approval, MRI scans of 60 patients with large (>5 cm in greatest dimension), deep, pathologically proven lipomas (31 patients) or well-differentiated liposarcomas (29 patients) were identified from a prospectively collected database. Pathological review and diagnosis were made by one of three pathologists with subspeciality training in musculoskeletal oncology and sarcoma pathology following WHO criteria for the classification of sarcoma. Informed consent was obtained from all research subjects as a part of their enrolment into this database. Patient MRI scans were retrospectively examined by 10 observers with subspecialty training in musculoskeletal radiology or orthopaedic oncology, blinded to diagnosis. There were 2 attending staff physicians and 3 fellows from each orthopaedic oncology and musculoskeletal radiology who participated.After reviewing each patient MRI on a PACS viewing module, observers indicated whether the amount of stranding, nodularity, and size of each tumor was concerning for well-differentiated liposarcoma and rendered a diagnosis of lipoma or well-differentiated liposarcoma. To simulate a clinical scenario, each reader was given the ability to use whatever image sequences available for diagnostic purposes (see later). Stranding  was defiAs the variables of size, stranding, and nodularity are used clinically by radiologists without standardization criteria to interpret such images and render a diagnosis, these variables were intentionally not standardized for individual readers for the purpose of this study. This was done to simulate a clinical scenario and therefore better evaluate the ability of readers to render a correct diagnosis and to determine the reliability and validity of reader diagnoses towards these tumors.All imaging was performed with 1.5-T MRI system  using a surface coil or body coil depending on the anatomic location. Specific imaging protocols included at least two planes of T1-weighted images (TR 500 ms/TE 10 ms) and fast spin echo T2-weighted images (TR 4800 ms/TE 100 ms) with selective fat saturation. STIR sequences (inversion time 110–150 msec) were also occasionally available. In all of the cases reviewed, either T2W fast spin echo imaging with spectral fat suppression or STIR imaging was performed. Imaging sequences used by readers of this study for diagnostic purposes were performed without contrast agents.P values for comparison to “no agreement” were generated from an exact test [P values were two-sided and any P-values less than 0.05 were considered statistically significant. Sixty MRI studies resulted in 95% power of 0.8 based on a type I error of 5%. Data analysis was performed using SAS version 9.2 computer software.Kappa statistics along with 95% confidence limits were used to determine the reliability of each categorical measurement . The intact test . All P v413/600 (69%) reader diagnoses agreed with the final pathological diagnosis. The 95% confidence limits for the accuracy percentage were (65%–73%). If the pathology diagnosis was lipoma, readers correctly identified lipoma in 196/310 (63%) cases (95% CI 58%–69%). If the pathology diagnosis was well-differentiated liposarcoma, readers correctly identified liposarcoma in 217/290 (75%) cases (95% CI 69%–80%). All readers chose liposarcoma more frequently than lipoma. There was no difference regarding diagnostic accuracy when compared between the specialties of orthopaedic oncology and musculoskeletal radiology . Attending physicians had a slightly high rate of diagnostic accuracy when compared to fellows .P < 0.01). Although this combination had a higher probability of a true liposarcoma diagnosis, readers overdiagnosed liposarcoma by a large margin in this instance. The inverse is true if readers choose “no” for both stranding and nodularity, which was associated with a lipoma diagnosis. A positive response for size was also associated with increased likelihood of a liposarcoma diagnosis (P < 0.01), although the misdiagnosis gap was not as large as for stranding/nodularity, as readers were overall more likely to choose a diagnosis of liposarcoma. There was no difference regarding interpretation of these categorical variables or their relationship towards the ultimate diagnosis when compared between specialties.Tables Well-differentiated liposarcomas are low-grade fatty tumors with negligible metastatic potential and less than 10% chance of local recurrence even after positive margin excision , 8, 13. The goal of this study was to determine whether experienced observers in musculoskeletal radiology and orthopaedic oncology could differentiate between lipomas and well-differentiated liposarcomas on imaging alone and to determine the relative association of variables such as stranding, nodularity, and size to this diagnosis. Our hypothesis was that even experienced observers would be unable to differentiate between lipomas and well-differentiated liposarcomas. Therefore, if treating physicians were unable to accurately diagnose these tumors on imaging or via biopsy, an argument suggesting that all symptomatic low-grade fatty lesions may be treated with marginal excision could be supported. While the results presented here are better than chance alone, they do reaffirm that a definitive diagnosis cannot be made on imaging alone until better imaging criteria are identified.We also present data here that supports several other variables surrounding the radiographic diagnosis of low-grade fatty lesions. First, there is a substantial interrater reliability based on kappa statistics towards making the radiographic diagnosis. These data are based on a high number of readers and the categorical variables of “lipoma” versus “liposarcoma.” In addition, the relative accuracy of observers from musculoskeletal radiology and orthopaedic oncology with various levels of training (fellow versus staff) was not statistically different. Finally, the variables of size, nodularity, and stranding did show an association with the diagnosis of well-differentiated liposarcoma and as such based on these data should continue to be used in the radiographic interpretation of these tumors.Other authors have evaluated the utility of MRI in the differentiation of well-differentiated liposarcoma from lipoma and determined the value of fluid-sensitive imaging sequences in making this distinction , 3, 10. The accuracy of diagnosis presented here 69%) is lower than that documented in previous reports –3. In on% is loweData presented here suggests that the variables of size, nodularity, and stranding show an association with the ultimate pathological diagnosis and as such should continue to be used in the radiographic interpretation . It has Recently, the identification and gene amplification of the MDM2 gene have been identified to supplement the pathological diagnosis of liposarcoma , 15. UnfAt tertiary referral sarcoma centers, where the treatment team understands the low metastatic potential of these lesions and the appropriateness of a marginal excision, the inability to differentiate these lesions on MRI alone does not represent a major treatment dilemma, as symptomatic tumors require marginal excision without adjuvant treatment , 6, 16. Experienced observers in musculoskeletal radiology and orthopaedic oncology can differentiate between lipomas and well-differentiated liposarcomas in 69% of cases. This level of accuracy needs to be improved upon with accepted and validated mechanisms to differentiate between these two entities. The variables of nodularity, stranding, and relative size do show an association with the diagnosis of well-differentiated liposarcoma and therefore should continue to be used in the radiographic impression. A better understanding of the biology of well-differentiated liposarcomas, appropriate treatment, and risk of local recurrence/overall survival related to this diagnosis is important to appropriately counsel patients and avoid unnecessary referral.


Adult intussusception is a rare entity accounting for only 5% of all intussusceptions and causes approximately 1% of all adult intestinal obstructions. Unlike paediatric intussusceptions which are usually idiopathic, there is usually a lead point pathology which might be malignant in up to 50% cases. We present an unusual case of adult intussusception which was not diagnosed on any investigation including computerized tomographic (CT) scan and magnetic resonance imaging (MRI). It was a case of ileo-ileo-cecal intussusception caused by a large lipoma 38 mm × 43 mm × 61 mm. It was treated by emergency laparotomy for acute intestinal obstruction. A conservative resection with ileostomy was performed with good postoperative recovery. Intussusception means telescoping of a proximal segment of bowel (intussusceptum) into the lumen of the adjacent distal segment (intussuscipiens). Rarely, a distal segment of the bowel telescopes into the lumen of the adjacent proximal segment, which is known as retrograde intussusception . IntussuThe patient was a 55-year-old male, employed as a manual laborer in Indian Railways. Patient presented in emergency with severe abdominal pain, multiple episodes of vomiting, abdominal distension, and obstipation. He had a history of recurrent attacks of colicky abdominal pain, nausea, and vomiting over the last 2 years. There was a history of hospitalization for occasional episodes of severe abdominal pain and distension and obstipation over this period. The frequency of these attacks had been progressively increasing over the last 2 years with the patient requiring weekly admission for the preceding two months before coming to our centre. There was history of loss of weight and appetite. There was no associated history of fever. There was no contributory past or family history. He was a chronic smoker of 80 packet years and a social alcoholic. He was investigated by CT scan of the abdomen and Thorax and MRI of the abdomen during his previous admissions and was diagnosed as a case of hiatus hernia with retroperitoneal fibrosis/diffuse lymphoproliferative disorder. CT chest showed nodularity indicative of infective pathology. However, these investigations were not available with the patient at the time of admission to our hospital. He was normotensive and afebrile but was having tachycardia and tachypnoea (pulse: 108/minute and respiratory rate: 22/minute). His nutritional status appeared adequate. His general physical examination was essentially normal.His systemic examination of chest, CVS, and CNS revealed no obvious abnormality. On abdominal examination he had a distended abdomen with stretched umbilicus. There were no visible scars. Abdomen was diffusely tender and guarded with no palpable organomegaly. Examination of external genitalia, hernial orifices and renal angles revealed no abnormality. On a digital rectal examination the rectum was empty and ballooned. A provisional diagnosis of acute intestinal obstruction with impending strangulation was made and confirmed on plain X-ray of the chest and abdomen erect and supine which showed grossly dilated small bowel loops and absence of any free intraperitoneal air. His haemogram, biochemical parameters, and urinanalysis done in emergency were within normal limits. The patient was taken up for emergency laparotomy without any other investigations as clinically there was an impending strangulation. The abdomen was opened by a midline incision. There was a dilatation of small bowel till the terminal ileum where there was an ileo-ileo-caecal intussusception. The telescoping had started in the terminal ileum and gone through the ileo-caecal valve with the apex extending up to midascending colon . There wSince its first description in 1674 by Barbette  intussusIntussusceptions are classified according to location into: enteric, colonic, and ileocaecal or ileocolic . EntericSmall intestinal tumors are rare, accounting for 1-2% of all gastrointestinal tract tumors . Among tAlthough they are usually asymptomatic, lipomas larger than 2 cm may cause bowel obstruction, intermittent nonspecific abdominal pain, diarrhea, or bleeding. Furthermore, some lipomas by forming a lead point may cause intussusception, as well , 16. AduSince the clinical picture is vague, varied, and nonspecific, preoperative diagnosis of adult intussusception is rare and essentially radiological. Plain skiagram of the abdomen may reveal features of acute intestinal obstruction . On bariUltrasonography is often used to evaluate suspected intussusception as it is cheap, readily available, and noninvasive. The classic features include the “target and doughnut sign” on transverse view and the “pseudokidney sign” in longitudinal view. The major disadvantages are operator dependency and difficulty in image interpretation in presence of air, which is often present in cases of obstruction , 20. TheCT scan has been reported to be the most useful imaging technique, with a diagnostic accuracy of 58%–100% and a specificity of 57–71% , 20. On In view of the uncertain aetiology and diagnosis and high incidence of malignancy (approaching 50%), the treatment of intussusception in adults is invariably surgical resection. However, the extent of bowel resection and the manipulation of the intussuscepted bowel during reduction remain controversial , 8, 15. Adult intussusception is a rare entity which is distinct from paediatric cases in incidence, aetiology, and management. Ileo-colic intussusception is often caused by lead point pathology which can be a submucous lipoma but may be a malignant lesion thereby necessitating resection and histopathology. Our case had a long history, age of 55 years, and no bowel pathology on CT. Intraoperatively there was viable healthy bowel, and absence of any free fluid, lymph nodes, or nodules in liver. Therefore we attempted reduction by gentle traction and did a limited resection with ileostomy. In adult patients with long history and investigations and intraoperative findings favoring a benign pathology, it is possible to do avoid sacrificing unnecessary length of terminal ileum, more so where it is possible to save the ileo-caecal valve.


Background. Castleman's disease is a benign lymphoproliferative disorder. The disease may be localized or multicentric. Three histologic variants are recognized: hyaline vascular, plasma cell, and mixed types. Case. A 20-year-old man presented with large left axillary mass. The histologic examination of the resected mass showed follicular pattern with large nodules of mantle cells arranged concentrically around atrophic and vascularized germinal centers. There was also some benign-appearing bone trabeculae interspersed with lymphoid tissue. The diagnosis of Castleman's disease, hyaline-vascular type with osseous metaplasia, was made. Castleman's disease (CD) is first described by Castleman in 1956 in a mediastinal lymph node . It is cThe disease may be unicentric or multicentric. The hyaline-vascular type is usually unicentric and pauci symptomatic, but plasma cell type and mixed type are usually multicentric, associated with fever, anemia, weight loss, and skin rash with a more aggressive behavior , 2.The exact etiology of CD is unclear, but immunosuppression, viral infection, autoimmunity, and chronic inflammation are proposed to have a role in disease development . Among tHerein we present a case of localized hyaline-vascular type of CD presented as an axillary lymphadenopathy showing osseous metaplasia.A 20-year-old man referred to surgery department of Faghihi hospital, affiliated to Shiraz University of medical science, due to painless left axillary mass for 2 months duration. His past medical history was unremarkable. On physical examination he had stable vital signs. A firm mass was palpated at the left axillary area measuring about 8 × 7 centimeter (cm). There was no evidence of hepatosplenomegaly and generalized lymphadenopathy.Left axillary ultrasound examination showed a well-defined superficial mass with area of punctate calcification with no evidence of hemorrhage and necrosis, suggestive of an enlarged lymph node .The patient's lab data including complete blood count with differential and biochemical tests were within normal limits. Chest X-ray and abdominal ultrasound were normal. The left axillary mass was excised. On gross inspection, there was a firm creamy brown lymph node, measuring 8 × 7 × 5 cm with focal areas of white discoloration and hard consistency . HistoloThe diagnosis based on histologic examination was Castleman's disease, hyaline-vascular type with osseous metaplasia.CD is a benign lymphoproliferative disorder –6. It is In 1972 Keller et al. . The hyaAccording to clinical presentation CD may be unicentric or multicentric –6, 9, 10The etiology of CD is unclear. There is a strong evidence of HHV-8 participation in development of multicentric CD , 6. It iβ (TGF-β) which stimulates differentiation of mesenchymal cells to osteoblasts through binding to appropriate receptor [In our case osseous metaplasia was observed in a lymph node involved by hyaline-vascular type of CD. Among hematologic malignancies, amyloid-producing dyscrasia and diffuse large B-cell lymphoma were reported to produce heterotopic bones . Two thereceptor . Bull etreceptor  demonstrreceptor , 14 it mreceptor , 13. Alsreceptor , and thiThe differential diagnosis of CD is non-Hodgkin's lymphoma , Hodgkin's lymphoma , and a vTo our knowledge this is the first case of CD with osseous metaplasia although the exact osteogenic substance is not clear.


Hemangioma is the most common benign tumor of liver and is often asymptomatic. Spontaneous rupture is rare but has a catastrophic outcome if not promptly managed. Emergent hepatic resection has been the treatment of choice but has high operative mortality. Preoperative transcatheter arterial embolization (TAE) can significantly improve outcome in such patients. We report a case of spontaneous rupture of giant hepatic hemangioma that presented with abdominal pain and shock due to hemoperitoneum. Patient was successfully managed by TAE, followed by tumor resection. TAE is an effective procedure in symptomatic hemangiomas, and should be considered in such high risk patients prior to surgery. Liver hemangiomas have prevalence between 0.4–7.4% in autopsy studies. One of t1A 31-year-old man experienced sudden onset of abdominal pain and tenderness in the right hypochondrium. There was no history of trauma, and no history of significant disease in the past. He underwent laparotomy at a primary health care centre, owing to a misdiagnosis of ruptured liver abscess with peritonitis. On exploration, hemoperitoneum with active bleeding from the liver surface was found. Due to lack of diagnosis of the possible cause of bleeding, perihepatic packing was done with sponges, and the patient was referred to our tertiary care institute. The patient received three units of packed cells prior to shifting to our center. On arrival, patient was pale and in hemorrhagic shock. The blood pressure was 76/50 mmHg, pulse was 124 beats per min and feeble. Abdomen was distended and diffusely tender. Laboratory investigation revealed hemoglobin of 6.8 g/dl, hematocrit 26%, and high transaminase levels. After initial resuscitation with intravenous fluids and packed red blood cells (RBC), the patient underwent a contrast enhanced computerized tomography (CECT) scan on a spiral computerized tomography (CT) scanner. CT scan demonstrated a peripherally enhancing lesion in right lobe of liver, located in segments VI and VII . The larDigital Subtraction Angiography (DSA) revealed typical angiographic findings of a liver hemangioma with pooling and puddling of contrast in the right lobe lesion. Arterial contrast extravasation was noted on the celiac angiogram, after which a selective right hepatic arteriogram was done that confirmed active contrast extravasation from the hemangioma .The hemangioma was embolized in a single session, first using PVA particles for embolisation of tumor interstices, and then the principal arteries were occluded using two steel coils . PVA parThe patient received a total of six transfusions perioperatively. When the general condition of the patient stabilized, he underwent laparotomy for removal of surgical packs. After pack removal, surgeons did not note any active bleed, and a large hemangioma involving segments VI and VII, which was ruptured on the posterior surface was noted . The hemCavernous hemangiomas are the most common benign neoplasm of liver and are often incidentally detected during abdominal imaging done for unrelated clinical indications. Most of 6et al in 1991.[14Recent studies have emphasized the role of TAE in the effective treatment of symptomatic hemangiomas, progressively growing hemangiomas and those at risk of bleeding.11 Howeve in 1991. Since th in 1991.1415 The In our case, the lesion was subcapsular and located in segment VII of right lobe. The likelihood of spontaneous ruptures is unknown but large, subcapsular lesions are considered to be at greater risk. Abdominaet al. observed a significant improvement in coagulative factors and a decrease in intraoperative blood loss, in patients with consumptive coagulopathy related to intravascular coagulation in hemangioma, that were treated with preoperative TAE.[Tumor resection after TAE resulted in minimal per-operative blood loss in our patient. In cases of rupture, TAE results in stanching or reducing the hemorrhage, thereby improving the general condition of patient, thus making subsequent hepatic resection a safer procedure. Suzuki etive TAE.To conclude, in a patient presenting with acute abdominal pain, with hitherto no known abdominal problem, spontaneous rupture of a hepatic tumor like hemangioma should also be kept as a rare differential. In addition, our case substantiates the limited available literature regarding validity of preoperative transcatheter arterial embolization and its usefulness in significantly improving the outcome in patients undergoing surgery for a ruptured hepatic hemangioma.


Ectopic pancreas in the mediastinum is extremely rare. We are reporting on a case of a twenty two year old woman who presented to our clinic with a large cervical mass. The CT scan revealed a cystic lesion in the anterior mediastinum. The patient underwent surgical resection by cervical approach. A Cystic mass with pseudocysts, cysts and complete pancreatic tissue were found in pathology. There were no signs of pancreatitis or malignancy. No recurrence was observed after a follow up of four years. We reviewed the case reports describing this rare condition in the medical literature.We conclude that the possibility of ectopic pancreatic tissue should be included in the differential diagnosis of anterior mediastinal cystic mass, though as a remote possibility. Surgery is probably needed for the diagnosis and treatment. Posterior mediastinal pseudocyst is a different entity associated with acute pancreatitis. In those cases surgery is not recommended. Our third conclusion is that pancreatic tissue should be actively sought, if a structure resembling a pseudocyst is found in an unexpected location.http://www.diagnosticpathology.diagnomx.eu/vs/1849369005957671The virtual slide(s) for this article can be found here: Congenital ectopic pancreas is a known phenomena found in the gastrointestinal tract in about 2% of autopsies. An ectoThe first reported case was published in 1957 by Shillitoe and Wilson. Herein An otherwise healthy twenty two year old woman was sent to our hospital because of a left cervical mass. The mass was soft without tenderness and with no symptoms like chest pain, dyspnea or cough. The physical examination was unremarkable except the cervical mass. Blood tests including complete blood count, electrolytes and thyroid function tests were within the normal range. Cervical ultrasound revealed a left supraclvicular hypoechogenic lesion 2.4×3.8 cm). Chest computed tomography demonstrated a cystic mass in the anterior mediastinum protruding to the left cervical region, close to the left common carotid artery Figure . Fine ne.4×3.8 cmWe performed a Pub med search in order to find all the published cases of ectopic pancreatic tissue in the Mediastinum. We reviewed the cases in order to describe this rare condition Table .In addition to our patient we found twenty one case reports of ectopic pancreas in the mediastinum. Fifteen case reports were written in English three in Chinese, two in German and one in Spanish. Most of the cases were described in young adults; Average age was thirty four and ages ranged from 5 to 66. The prevalence was higher in females . The clinical presentation was usually nonspecific including, chest pain, shoulder pain, shortness of breath, fever, neck swelling, night sweats, heart murmur, fatigue, chronic pulmonary infiltrate and tamponade. Four patients were asymptomatic at presentation. All the cases showed cystic lesions located in the anterior mediastinum. Most of the lesions were large ranging from 3.5 cm to 20 cm in diameter. Large lesions encompassed adjacent structures such as the great vessels. Associated findings were pleural effusions and pericardial effusions. There were no specific findings on the CT scan that could distinguish ectopic pancreatic tissue from other diagnosis. The cyst wall and the solid portion of the lesions usually showed mild to moderate enhancement with contrast material. The radiological appearance could not be distinguished from Thymoma, Lymphoma or Teratoma. In all cases the diagnosis was done only after surgery. Twenty one cases were benign and fully recovered after the operation and in a single case pancreatic carcinoma arising from the mediastinum was found. In the benign cases no recurrence was reported. The only patient with pancreatic adenocarcinoma died 15 months after the operation. This case strengthens the importance of surgery.Ectopic pancreas in the mediastinum is a very rare condition. There are two theories on the embryogenesis of this anomalous development,8. The fA different entity is a pseudocyst arising from the posterior mediastinum as a result of acute pancreatitis-26. In tEctopic tissue in the mediastinum is a rare phenomenon. The anomalous development of ectopic tissue may occur due to abnormal differentiation of pluripotent cells, migration of ectopic cells or malignant transformation. A few case reports described this rare phenomenon. Hong Li et al. described a rare liposarcoma in the superior mediastinum. The oriThe first reported case of ectopic mediastinal pancreas was published in 1957 by Shillitoe and Wilson. They deWe conclude that ectopic pancreas should be considered in the differential diagnosis of anterior mediastinal lesions. Surgery is probably needed for the diagnosis and treatment. Pancreatic tissue should be actively sought, if a structure that looks like a pseudocyst is found in an unexpected location.Written informed consent was obtained from the patient for the publication of this report and any accompanying images.


In this report, we describe a rare case of papillary thyroid carcinoma with adrenocortical carcinoma without excess hormone production.A 40-year-old Turkish man was admitted to our institution with a large left adrenal mass that was identified during the work-up for shortness of breath. The patient did not have specific signs and symptoms of hormone excess. The mass was removed surgically. The pathological findings were consistent with adrenocortical carcinoma. The patient was also found to have a multicentric papillary thyroid microcarcinoma.Most adrenocortical carcinomas and papillary thyroid carcinomas are sporadic; however, the occurrence of two different endocrine neoplasms during the same period of time is a rare situation, but it is possible, as in our patient. When an endocrine tumor is diagnosed, endocrinologists must be consider the possibility of the existence of another endocrine tumor. Adrenocortical carcinomas (ACCs) are an extremely rare type of cancer with an incidence of less than 2 per 1 million worldwide [There are few case reports in the literature that describe comorbid ACC and PTC. The available reports attribute the concomitant appearance of the tumors to coincidence and do not discount a potential genetic or hereditary link -5. Herei+, K+,Cl–), fasting blood glucose, and fasting insulin levels were normal. Plasma cortisol was suppressed after administration of 1mg dexamethasone. The patient was diagnosed with a non-functional adrenal mass, and a left adrenalectomy was performed. The resected tumor was 11×9×6cm in size and weighed 339g. The tumor was of high nuclear grade and had diffuse architecture, focal necrotic areas, more than 5 mitoses per 50 high-power fields, and an infiltrated capsule. Sinusoidal vascular infiltration was observed, but clear cells were less than 25%. Immunohistochemical studies showed that melan-A and inhibin were positive, and thyroid transcription factor-1 (TTF-1) and thyroglobulin were negative. The pathological findings were consistent with ACC. Treatment with adjuvant radiotherapy with concurrent mitotane was planned for the patient.A 40-year-old Turkish man who presented to our institution with shortness of breath was evaluated by computed tomography. A mass in his left adrenal gland was diagnosed incidentally. A magnetic resonance imaging (MRI) scan showed a large mass measuring 8×9.5×10.4cm in diameter in the left adrenal gland area  with a heterogeneous presentation and generally have a poor prognosis . Women aPatients present with evidence of adrenal steroid hormone excess in approximately 60% of cases. Rapidly progressing Cushing’s syndrome with or without virilization is the most frequent presentation. Hormonal inactive ACC usually presents with abdominal discomfort  or back pain caused by a mass effect of the large tumor.PTC comprises about 85% of cases with differentiated thyroid carcinoma . PTC usuet al. reported sensitivity of 97% and specificity of 100% for a score of 4 or 5 as being predictive of malignancy [The ultrasonographic features suggesting the presence of malignancy in a thyroid nodule have been described clearly. These include microcalcifications, marked hypoechogenicity, absent “halo” sign, extraglandular extension, an irregular or microlobulated margin, and a heterogeneous echo structure ,16. Indilignancy .PTC generally metastasizes to the lymph nodes. Distant organ metastasis is rare, but lung, bone, brain, liver, and adrenal gland metastases have been reported ,20. ACC et al. reported a case of a middle-aged woman with virilizing adrenocortical adenoma complicated with Cushing’s syndrome, PTC, and hypergastrinemia [et al. reported the case of a patient with synchronous presentation of an aldosterone-secreting metastatic ACC and PTC [p53 and menin gene mutations were absent.In the literature, there are few case reports of patients with with comorbid ACC and PTC. Fukushima trinemia . They di and PTC . That paHerein we present a case of a patient with PTC and ACC without clinical hormone excess. Most ACCs and PTCs are sporadic; however, the possibility of a hereditary cancer syndrome could not be ruled out because of the co-occurrence of two different endocrine neoplasms within the same period of time. Therefore, when an endocrine tumor is diagnosed in a patient, endocrinologists must consider the possibility of the existence of another endocrine tumor.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.MK analyzed and interpreted the patient’s data. The surgery was performed by OH. EC and TD contributed to the writing of the manuscript’s Discussion section. All authors read and approved the final manuscript.