Carcinoid tumor of the duodenum and accessory papilla associated with polycythemia vera

Carcinoid tumors have been reported in a wide range of organs but most frequently involve the gastrointestinal tract; however, duodenal carcinoid tumors are rare. We report a 50-year-old male patient complaining of multiple melenas for 3 wk. The panendoscopy and endoscopic retrograde cholangiopancreaticography revealed swelling accessory papilla with an ulcer. The biopsy taken showed a carcinoid tumor. The lesion was removed by wide resection. Patient was found to have an abnormal blood cell count during the follow-up period with elevated levels of hemoglobin and hematocrit of 21.2 g/dL and 63.5%, respectively, thrombocytosis of 501 000/μL, and leukocytosis of 20 410/μL. He was diagnosed as a polycythemia vera by a hematologist after further evaluation. He received periodic phlebotomy and hydroxyurea treatment. The response was good and his hematocrit was stabilized by periodic phlebotomy in the range of 44-49% during the last 2 years. The possible origin of UGI bleeding by a duodenal carcinoid tumor, although rare, should be considered. There has been one case report of a duodenal carcinoid tumor that involved accessory papilla of the pancreas divisum and one case report of metastatic carcinoid tumor associated with polycythemia vera. It is different in our patient as compared with the latter report, which mentioned a polycythemia vera patient who was found to have a metastatic carcinoid in the 17 years follow-up period. Chemotherapy had been given before the carcinoid tumor was revealed. Our patient had no previous chemotherapy for polycythemia vera before he was found to have duodenal carcinoid tumor; this excludes the possibility of chemotherapy induced carcinoid tumor, although it had been suspected in the previous report. In our patient, the existence of both diseases may be by predisposition of each other since both diseases have an increased incidence of other neoplasm, or they may be coexistent incidentally.     

Gastrointestinal stromal tumor of the stomach with a giant abscess penetrating the gastric lumen

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. In large GISTs, cystic degeneration, necrosis and focal hemorrhage that occur inside the tumor can result in gastrointestinal bleeding. We describe a case of a 74-year old male with GIST of the stomach accompanied with a giant abscess that penetrated the gastric lumen. The patient experienced undiagnosed fever for two months prior to hospitalization. Gastrointestinal endoscopy, X-ray series and computed tomography of the patient’s abdomen revealed a gastric submucosal tumor in the fornix, with a fistula to the gastric lumen that was inundated with a great deal of pus. The mass was diagnosed as a GIST from biopsy specimens. The patient was treated by endoscopic drainage of the abscess and intravenous administration of antibiotics. Eventually, a partial gastrectomy was performed. He was also administered Imanitib mesylate as adjuvant therapy. He was followed up for 2 years and no metastasis or recurrence was recognized at the follow- up examinations. This is the first report of a patient with clearly diagnosed GIST with endoscopic evidence of an abscess penetrating into the gastric lumen.     

Large primary hepatic gastrinoma in young patient treated with trisegmentectomy: A case report and review of the literature

Primary hepatic gastrinoma is a rare disease, with fewer than 40 cases reported in the medical literature. Because it is located in an organ in which metastases are common, its diagnosis is difficult. We report a case of a 19 years old male patient with a history of gastric ulcers since the age of nine. Following gastric surgery, an antrectomy and a vagotomy, there was some alleviation of symptoms. Subsequently, the patient reported various intermittent episodes of diarrhea, diffuse abdominal pain, and vomiting. The patient underwent tomography, which revealed the presence of a hepatic mass measuring 19.5 cm × 12.5 cm × 17 cm. Primary hepatic gastrinoma was diagnosed based on laboratory examinations that indicated hypergastrinemia and a positron emission tomography/magnetic resonance study with somatostatin analogue that confirmed the liver as the primary site. After hepatic trisegmentectomy(Ⅱ, Ⅲ, Ⅳ, Ⅴ, Ⅷ), the patient's symptoms improved. The case is notable for the presence of a rare tumor with uncommon dimensions.     

Solitary plexiform neurofibroma of the stomach:A case report

Plexiform neurofibroma(PN)of the digestive tract is very rare and usually part of the generalized syndrome of neurofibromatosis type 1(von Recklinghausen disease).Solitary PN of the stomach is extremely rare and has not been reported in the literatures.Here we present a case of solitary PN of the stomach,which was not associated with von Recklinghausen disease.A38-year-old male presented abdominal pain and distention for 7 d.The patient underwent endoscopy of the upper gastrointestinal tract,which revealed a 3.5 cm protruding and cauliflower-shaped mass with a shallow1 cm central ulcer in the greater curvature of the stomach.The lesion was removed by laparoscopic surgery.Histological examination demonstrated characteristic histological findings of spindle-shaped cells.Immunohistochemical analysis showed that the tumor cells were positive for S-100 protein,but negative for CD34,KI-67,CD117,and actin.Based on histological findings,gastrointestinal stromal tumor could be excluded,and thus the case was confirmed as PN.We described the clinical features,physical examination,endoscopic findings,and histopathological examination of this case.     

A case report of anaplastic carcinoma of the pancreas with remarkable intraductal tumor growth into the main pancreatic duct

We herein report a case of anaplastic carcinoma of the pancreas with remarkable intraductal tumor growth into the main pancreatic duct.A 76-year-old male was referred to our hospital for treatment of a pancreatic tumor.Preoperative examinations revealed a poorly defined tumor in the main pancreatic duct in the body of the pancreas,accompanied with severe dilatation of the main pancreatic duct,which was diagnosed as an intraductal papillary-mucinous neoplasm.We performed distal pancreatectomy and splenectomy.The pathological examination revealed that the tumor consisted of a mixture of anaplastic carcinoma(giant cell type)and adenocarcinoma in the pancreas.There was a papillary projecting tumor composed of anaplastic carcinoma in the dilated main pancreatic duct.The patient is now receiving chemotherapy because liver metastasis was detected 12 mo after surgery.In this case,we could observe a remarkable intraductal tumor growth into the main pancreatic duct.We also discuss the pathogenesis and characteristics of this rare tumor with specific tumor growth.     

Perforation of metastatic melanoma to the small bowel with simultaneous gastrointestinal stromal tumor

The gastrointestinal tract (GIT) is a common site of metastases for malignant melanoma. These metastatic tumors are often asymptomatic. We describe a case of a 58-year-old male who presented with a sudden onset of generalized abdominal pain. The patient's past medical history was significant for lentigo melanoma of the right cheek. Laparotomy was performed and two segments of small bowel, one with a perforated tumor, the other with a non-perforated tumor, were removed. Histology and immunohistochemical staining revealed the perforated tumor to be a metastatic malignant melanoma and the non-perforated tumor was found to be a gastrointestinal stromal tumor (GIST). The patient was discharged 7 d postoperatively. To the best of our knowledge, this is the first reported case in the literature of a simultaneous metastatic malignant melanoma and a GIST. Surgical intervention is warranted in patients with symptomatic GIT metastases to improve the quality of life or in those patients with surgical emergencies.     

Ampullary somatostatinomas and jejunal gastrointestinal stromal tumor in a patient with Von Recklinghausen＇s disease

Von Recklinghausen’s disease is an autosomal dominant hereditary disease associated with a wide number of neoplasms. We report a case of a 47-year-old Caucasian male affected by Von Recklinghausen’s disease who developed a malignant somatostatinoma of the papilla major and minor associated with jejunal gastrointestinal stromal tumour with uncertain behaviour. At laparotomy, multiple hepatic metastases were evident. Whipple pancreaticoduodenectomy, jejunal resection, extensive lymphadenectomy and multiple hepatic wedge resections were performed. The patient was alive without recurrence after 24 mo. This is the fourth case reported in the world literature of a patient with Von Recklinghausen’s disease associated with periampullary somatostatinomas and jejunal stromal tumor. In patients with Von Recklinghausen’s disease who complain of gastrointestinal symptoms, a high suspicion index for periampullary endocrine tumours and/or gastrointestinal stromal tumour is required. An aggressive surgical approach seems to give long term survival also in metastatic patients.     

PEComa of the colon resistant to sirolimus but responsive to doxorubicin/ifosfamide

A 23-year-old male presented with a three-week-history of crampy abdominal pain and melaena.Colonoscopy revealed a friable mass filling the entire lumen of the cecum;histologically,it was classified as perivascular epithelioid cell tumor(PEComa).An magnetic resonance imaging scan showed,in addition to the primary tumor,two large mesenteric lymph node metastases and four metastatic lesions in the liver.The patient underwent right hemicolectomy and left hemihepatectomy combined with wedge resections of metastases in the right lobe of the liver,the resection status was R0.Subsequently,the patient was treated with sirolimus.After 4 mo of adjuvant mammalian target of rapamycin inhibition he developed two new liver metastases and a local pelvic recurrence.The visible tumor formations were again excised surgically,this time the resection status was R2 with regard to the pelvic recurrence.The patient was treated with 12 cycles of doxorubicin and ifosfamide under which the disease was stable for 9 mo.The clinical course was then determined by rapid tumor growth in the pelvic cavity.Second line chemotherapy with gemcitabine and docetaxel was ineffective,and the patient died 23 mo after the onset of disease.This case report adds evidence that,in malignant PEComa,the mainstay of treatment is curative surgery.If not achievable,the effects of adjuvant or palliative chemotherapy are unpredictable.     

Central pancreatectomy for pancreatic schwannoma: A case report and literature review

Schwannomas are mesenchymal tumors originating from Schwann cells in peripheral nerve sheaths. Although the tumor can be located in any part of the human body, the most common locations are the head, neck, trunk and extremities. Pancreaticschwannomas are rare. To our knowledge, only 64 cases of pancreatic schwannoma have been reported in the English literature over the past 40 years. In this paper, we present a pancreatic schwannoma in a 59-year-old female. Ultrasound, computed tomography and magnetic resonance imaging revealed the tumor located in the pancreatic body; however, accurate diagnosis was hard to obtain preoperatively and a pancreatic cystadenoma was preliminarily considered. During laparotomy, the mass was found in the body of the pancreas. An enlarged gallbladder with multiple stones was also observed. We performed central pancreatectomy, end-to-side pancreaticojejunostomy and cholecystectomy. Notably, central pancreatectomy has been reported in only one case prior to this report. The gross specimen showed a mass with a thin capsule, 1.6 cm × 1.1 cm × 1.1 cm in size. Microscopic examination showed that the tumor was mainly composed of spindle-shaped cells with palisading arrangement and no atypia, which is consistent with a benign tumor. Both hypercellular and hypocellular areas were visible. Immunohistochemical staining revealed strongly positive results for protein S-100. Finally, the tumor was diagnosed as a schwannoma of the pancreatic body. Postoperatively, the patient recovered well and left the hospital 6 d later. During the 53-mo follow-up period, the patient remained well and free of complications.     

Glomus tumor in the stomach:Computed tomography and endoscopic ultrasound findings

A 57-year-old man presented with intermittent dull abdominal pain after a period of 1 year.Abdominal computed tomography(CT) was performed.Except for the endoscopy,the work-up for possible medical causes remained inconclusive.An open-abdomen,partial surgical excision of the stomach was performed after the unsuccessful endoscopic resection.The pathology report revealed a glomus tumor of the stomach.Importantly,glomus tumors of the stomach are rare and are almost always benign.Therefore,the most important current role of imaging associated with the diagnostic approach and therapeutic plan for a glomus tumor is to differentiate it from other gastric submucosal tumors(SMTs).We report this case with representative radiologic findings,including CT and endoscopic ultrasound(EUS) reports,and also correlate them with clinical and pathologic presentations that can help in the early detection and differentiation of gastric SMTs from other SMTs.As such,the purpose of this report is to provide a better understanding of relevant CT and EUS features.Alternative treatments should be considered carefully according to the imaging results.     

Giant primary liposarcoma of the stomach: a case report

Liposarcoma is the most common soft tissue sarcoma and accounts for 15%–20% of all mesenchymal malignancies. The tumor occurs most frequently in the limbs, retroperitoneum and rarely has a visceral location. We report a case of a gastric liposarcoma in a male patient who was admitted to hospital for abdominal discomfort and feeling fullness lasting for a month. The CT examination revealed a giant epigastric mass (36 cm × 34 cm) involving the wall of the stomach. A subtotal gastrectomy together was performed. The histopathological diagnosis was a myxoïd liposarcoma. The patient did not undergo any adjuvant treatment. He was under close follow up and nine months after surgery he represented with tumor recurrence. We report this case due to the rarity of this tumor in the stomach (fifteen cases reported in the literature).     

Liposarcoma of the stomach: Report of two cases and review of the literature

Liposarcoma of the stomach is extremely rare, and only 37 cases have been reported worldwide. We herein report two cases of liposarcoma of the stomach. The first patient was referred to our hospital with upper abdominal discomfort. The endoscopic examination revealed a tumor mass about 3 cm in diameter. The patient underwent a partial gastrectomy and had an uneventful recovery. The histopathological examination revealed a welldifferentiated liposarcoma. The second patient had symptoms of upper abdominal discomfort combined with nausea and anorexia. Several palpable masses were found with endoscopy. Endoscopic submucosal dissection was the treatment used, and the postoperative course was uneventful. The histopathological diagnosis was a well-differentiated liposarcoma. The two patients did not undergo any adjuvant therapy. They are both currently in good condition without recurrence. Therefore, we believe that the outcome of liposarcoma of the stomach is positive, and surgical resection may be the first choice for treatment at present.     

Hepatocellular adenoma in a male with familial adenomatous polyposis coli

Hepatocellular adenoma (HA) is a benign liver tumor most frequently occurring in young women using oral contraceptives. We report a rare case of HA in a 27-year-old male patient with familial adenomatous polyposis (FAP). The patient underwent a total colectomy and ileo-rectal anastomosis for FAP in 2003. A preoperative computed tomography scan of the abdomen disclosed a tumor in the left-lobe of the liver, 5.8 cm in diameter. Pathologic examination of a needle biopsy disclosed HA, but he had never used anabolic steroids or other known inducers of HA. The size of the liver mass gradually increased to 8.5 cm during a follow-up period of 38 months, and a left hepatectomy was performed in 2006. Pathology of the resected specimen confirmed the diagnosis of HA. Although FAP is known to be complicated with neoplasia in various extracolonic organs, only five reported cases of HA have developed in patients with FAP, including this case. This is the first report of HA to develop in a male FAP patient.     

Myxoid Liposarcoma in the Abdominal Wall: A Case Report

A liposarcoma is the most common type of soft tissue sarcoma, and most liposarcomas are malignant. The extremities are the most common site for liposarcomas. There are 5 histologic types of liposarcoma, as follows: well differentiated; myxoid; round cell; pleomorphic; and dedifferentiated. Myxoid liposarcomas (MLSs) represent a subgroup of liposarcomas. There has been no report of MLSs in the abdominal wall.We report a rare case of a MLS of a 43-year-old male who presented with tensile force on the abdominal wall. Computed tomography (CT) found a tumor in abdominal wall. There was no other abnormal symptom and the laboratory testing was also unusual.At last, the tumor was successfully excised, which was diagnosed MLSs in pathology. Following standard principles, after complete excision, the patient received radiotherapy. The patient was followed up for 8 month and no disease recurrence was identified.MLSs are rarely seen in the clinic, irrespective of the presenting signs, but also based on histologic features. The aim of this report was to present the differential diagnosis of an abdominal wall mass, and to remind us of MLSs.     

Primary Pedunculated Giant Esophageal Liposarcoma: Case Report

Liposarcoma is one of the most common soft tissue sarcomas in adults, but the incidence of esophageal liposarcoma is extremely low. To our knowledge there are only 18 cases of esophageal liposarcoma reported in the English-language literature. Here we report a new case of primary esophageal liposarcoma in a 50-year-old male patient who complained of aggravating dysphagia with intermittent vomiting for 6 months. The tumor was located in the upper esophagus. Polypectomy was performed and histologic examination showed a well-differentiated liposarcoma. Diagnostic and therapeutic tools are discussed and the results of a literature review are discussed.     

Primary omental liposarcoma presenting with torsion: a case report.

An 83 year-old man was admitted because of abdominal pain and distention. A mass measuring 20x10 cm and associated with tenderness and guarding was palpable in the right lower abdomen. Ultrasonography and computed tomography showed a tumor with solid and multiple cystic areas. Celiac angiography showed slight tumor vascularity fed by the right gastroepiploic artery. A malignant tumor of the omentum was suspected. At laparotomy, torsion of the omental pedicle of the tumor was found. Histological examination of the resected tumor revealed characteristics of round-cell liposarcoma, which usually has a poor prognosis. The patient has been alive and well for 2 years. Primary liposarcoma of the omentum has been reported in only seven previous cases. None of these patients presented with torsion, and no report has thus far included a documented survival.     

A rare case of iron deficiency

Giant lipomas of the stomach are very rare, accounting for less than 3% of all benign tumors of the stomach. A clear-cut endoscopic differentiation between gastric lipomas and other submucosal neoplasms is not feasible, because routine endoscopic gastric biopsies do not reach the submucosal layer. Gastric submucosal lipomas can cause gastric ulceration as in the case presented below and in rare instances this may in turn promote gastric cancer. Therefore, complete pretreatment diagnostic evaluation is needed. We present a 52-year-old man with a 6-month history of epigastric discomfort, early satiety, decreased appetite, and dyspepsia. His weight was noted to be stable and he was iron deficient (hemoglobin 11.5 g/dl and ferritin of 5 g/dl). His past history included a gastric ulcer found on endoscopy 5 years ago for which he was on omeprazole 40 mg once a day, hypertension, hypercholesterolemia, and diabetes. Clinical examination revealed central obesity with divarification of recti muscles. He underwent a colonoscopy that was normal, and an oesophago-gastro-duodenoscopy that revealed a smooth extrinsic indentation of the anterior aspect of the distal stomach at around 50 cm. Biopsies of this were normal. A computed tomography scan was obtained () that demonstrated a 14 by 15-cm fatty tumor arising from the distal stomach with a couple of 5-mm nodes adjacent to tumor and no distant metastasis representing either a lipoma, liposarcoma or gastrointestinal stromal tumours. He subsequently underwent a subtotal gastrectomy. Macroscopically, the antrum was distorted by a huge submucosal intramural tumor mass. The antral mucosa was stretched over its surface and bore a central 15-mm ulcer surrounded by a raised border (). Microscopic examination confirmed an ulcerated benign submucosal lipoma. Our patient was symptomatic with a large gastric lipoma that necessitated surgical excision. Following surgery his postoperative recovery was uneventful, and he was asymptomatic when reviewed 4 weeks later. This case demonstrates a rare case of gastric lipoma causing gastric epithelial ulceration leading to iron deficiency.     

Liposarcoma of the pleural cavity

We report a case of pleural liposarcoma in a 58-year-old male patient with a twenty-year history of smoking, who was admitted for chest pain and chronic cough. Pleural liposarcoma is a rare, highly malignant intrathoracic tumor arising from the soft tissue of the pleural cavity. Chest radiograph and CT scan revealed a homogeneous mass with subtotal opacification of the left hemithorax. Tumor diagnosis was based on histopathological examination of a transparietal biopsy and revealed a mixed type liposarcoma. Surgical resection was performed followed by adjuvant irradiation. No recurrence was diagnosed during the follow-up period of ten months and the patient continues to do well at the time of writing this paper.     

Solitary gastric Peutz-Jeghers type stomach polyp mimicking a malignant gastric tumor

Most cases of Peutz-Jeghers type polyps of the stomach are associated with mucocutaneous pigmentation and multiple intestinal polyposis. A solitary Peutz-Jeghers type polyp of the stomach is rare. We here report a case of a 71-year-old woman with a solitary Peutz-Jeghers type polyp of the stomach who presented with intolerable epigastric pain and weight loss of 5 kg over the prior two months. During the hospital treatment course for this patient, endoscopic examination revealed a bulging lesion with a central hole, mucosal ulceration, an asymmetrical wall thickness and a narrowing of the gastric lumen. A gastric biopsy further revealed ulceration with moderate dysplasia. The patient received endoscopic ultrasonography which showed a second subepithelial lesion that measured 4 cm × 3 cm. Computed tomography of the abdomen subsequently showed a thickened gastric wall with three visibly enlarged lymph nodes, all greater than 1 cm. The suspected diagnosis was malignant gastric cancer with lymph node metastases. The other lesion, which measured 2 cm × 2 cm × 1 cm was noted in the submucosa of the jejunum during surgery. The patient was treated using a subtotal gastrectomy and partial resection of the jejunal tumor. The final pathological report indicated a gastric Peutz-Jeghers type polyp with proliferation of smooth muscle bundles in the submucosal layer, and hyperplastic glands in the mucosal layer and ectopic pancreas of the jejunum. This is the first reported clinical case of a solitary Peutz-Jeghers type polyp of the stomach accompanying a lymph node enlargement and ectopic pancreas in the jejunum that simulates stomach cancer with lymph node metastases.