Acute GI bleeding by multiple jejunal gastrointestinal autonomic nerve tumour associated with neurofibromatosis type I.

We describe a surgical emergency due to GI-bleeding caused by gastrointestinal autonomic nerve tumours (GANT s) in a patient with von Recklinghausen s disease. A 72 year old female patient with von Recklinghausen s disease was admitted with maelena. Endoscopy showed no active bleeding in the stomach and the colon. Therefore an angio-CT-scan was performed which revealed masses of the proximal jejunum as source of bleeding. Laparotomy was indicated and a 20 cm segment of jejunum which carried multiple extraluminal tumours was resected. The source of the bleeding was a 2 cm tumour which had eroded the mucosal surface. Immunohistologically, evidence of neuronal differentiation could be shown in the spindle-formed cells with positive staining for C-Kit (CD 117), CD 34, and a locally positive staining for synaptophysine and S100. This case report illustrates the association between neurofibromatosis and stromal tumours and should alert surgeons and gastroenterologist about gastrointestinal manifestations in patients with von Recklinghausen s disease.     

Jejunojejunal Intussusception in a Patient with von Recklinghausen's Disease

Abstract: A 63-year-old female with von Recklinghausen's disease was referred to us because of intermittent periumbilical pain and vomiting. Abdominal ultrasonography showed a segment of the bowel with intussusception in the left ripper quadrant. After intestinal decompression, small-intestinal endoscopy was performed. Endoscopy revealed multiple protruding lesions in the esophagus, stomach, and jejunum, the largest one markedly stenosing the proximal jejunum. We were able to identify a tumor as the lead point preoperatively. Segmental jejunectomy was performed for the resection of the tumor. Pathological studies revealed that the tumor was leiomyosarcoma. Gastrointestinal involvement is well documented in von Recklinghausen 's disease. Thus attention should be paid to gastrointestinal involvement in the management of these patients. The present case had intussusception, a rare complication of von Recklinghausen 's disease. Svnall-intestinal Endoscopy played an essential vole in the diagnosis of gastrointestinal involvement in our patient.     

A rare case of multiple myeloma initially presenting with pseudoachalasia

Pseudoachalasia is a rare clinical entity with clinical, radiographic, and manometric features often indistinguishable from achalasia. Primary adenocarcinomas arising at the gastroesophageal junction or a tumor of the distal esophagus are the most frequent causes of pseudoachalasia. Rarely, processes other than esophagogastric cancers including chronic idiopathic intestinal pseudo-obstruction, amyloidosis, sarcoidosis, Chagas' disease, vagotomy, antireflux surgery, pancreatic pseudocysts, von Recklinghausen's neuroinomatosis, gastrointestinal stromal tumor, and other malignancies and rare genetic syndromes, may lead to the development of pseudoachalasia. Secondary achalasia is extremely rare, with less than 100 cases reported in the literature so far. Gastrointestinal manifestations in primary or secondary amyloidosis include abdominal pain, diarrhea, constipation, malabsorption, obstruction, motility disturbance, intestinal infarction, perforation, and hemorrhage; however, gastrointestinal tract involvement is asymptomatic in most instances. We present here a rare case of multiple myeloma initially presenting with dysphagia because of esophageal amyloidosis and manometric findings typical of achalasia.     

Gastric calcifying fibrous tumor removed by endoscopic submucosal dissection

The World Health Organization describes calcifying fibrous tumors(CFTs) as rare, benign lesions characterized by hypocellular, densely hyalinized collagenization with lymphoplasmacytic infiltration. These tumors rarely involve the gastrointestinal(GI) tract. A routine endoscopic upper gastrointestinal screen detected a 10-mm submucosal tumor(SMT) in the lesser curvature of the lower corpus of the stomach of an apparently healthy, 37-year-old woman with no history of Helicobacter pylori infection. Endoscopic ultrasonography(EUS) localized the internally isoechoic, homogeneous SMT mainly within the submucosa. Malignancy was ruled out using endoscopic submucosal dissection(ESD). A pathological examination confirmed complete resection of the SMT, and defined a hypocellular, spindle-cell tumor with a densely hyalinized, collagenous matrix, scattered lymphoplasmacytic aggregates as well as a few psammomatous, dystrophic calcified foci. The mass was immunohistochemically positive for vimentin and negative for CD117(c-kit protein), CD34, desmin, smooth muscle actin(SMA) and S100. Therefore, the histological findings were characteristic of a CFT. To date, CFT resection by ESD has not been described. This is the first case report of a gastric calcifying fibrous tumor being completely resected by ESD after endoscopic ultrasonography.     

Two cases of gastrointestinal stromal tumor of the stomach with lymph node metastasis

Lymph node metastasis from gastrointestinal stromal tumor (GIST) is quite rare. We report two cases of gastric GIST with nodal metastases and results of their mutation analyses. In the first case (78-year-old male), a mass 4.0 cm in size was located at the gastric cardia. Proximal gastrectomy was performed. In the other case (40-year-old female), the gastric tumor was 2.5 cm in size. Computed tomography scan revealed a hepatic metastasis. Imatinib mesylate was administered as primary treatment, at the patient's preference, but the tumors exhibited no response. Wedge resection of the stomach and partial hepatectomy were performed. In both cases, histological examination revealed that the tumors consisted of spindle cells. In the former case, there was an isolated lymph node metastasis at the right cardia. In the latter, three of 5 sampled nodes adjacent to the tumor were positive. In both cases, immunohistochemical analyses showed that primary and metastatic tumors were diffusely positive for CD117 and CD34 and negative for desmin and S100-protein. In the former case, there was a deletion mutation in CD117 exon 11, the most common genotype in GIST. In the latter, there were no detectable mutations in CD117 or platelet-derived growth factor receptor alpha.     

A case of foregut gastric duplication cyst with pseudostratified columnar ciliated epithelium

Gastrointestinal duplication is a congenital rare disease. Duplication cyst of the stomach with pseudostratified columnar ciliated epithelium is extremely rare. A 44-year-old Japanese woman visited University of Yamanashi Hospital for evaluation of an abnormal tumor detected by abdominal ultrasonography at an annual general health examination. Abdominal computed tomography indicated a subserosal cystic lesion 6 cm in diameter on the posterior wall of the stomach. The cystic lesion was resected through partial resection of the stomach. Histopathology showed that the cyst did not communicate with the gastric lumen, was covered with gastric epithelium and pseudostratified columnar ciliated epithelium with circular muscle layers, and did not contain cartilaginous tissue. Consequently, the patient was diagnosed as having foregut duplication cyst of the stomach. Gastrointestinal duplication can occur in any region of the gastrointestinal tract, but foregut duplication cyst of the stomach is rare. The present case was a subserosal cyst on the greater curvature that did not communicate with the gastric lumen and was covered with gastric epithelium and pseudostratified columnar ciliated epithelium, suggesting a foregut cyst caused by an aberrant respiratory organ.     

Rectal GIST Presenting as a Submucosal Calculus

This case report presents an incidental finding of a rectal GIST (gastrointestinal stromal tumor) presenting as a submucosal calculus, not previously reported. A 53-year-old man without a significant medical history presented with abdominal pain in the left lower quadrant, and with constipation. Upon rectal examination, a hard submucosal swelling was palpated 4 cm from the anus, at 3 o'clock, in the left rectum wall. X-ray photos, computerized tomography (CT)-scan and a magnetic resonance imaging (MRI) scan clearly showed a calculus. Excision revealed a turnip-like lesion, 3.1 x 2.3 x 1.8 cm. Analysis showed it was a rectal GIST, a rare mesenchymal tumor of the gastrointestinal tract, which expressed CD117 (or c-kit, a marker of kit-receptor tyrosine kinase) and CD34. Calcification is not a usual clinicopathological feature of GISTs [1-3], and although a number of rectal GISTs have been reported [4-9], we have found no cases so far of rectal GIST presenting as a submucosal calculus.In general, GISTs are rare mesenchymal tumors of the gastrointestinal tract (nerve tissue, smooth muscle). Histology and immunohistochemistry discriminate gastrointestinal stromal tumors from leiomyomas and neurinomas. The most important location is the stomach; the rectal location is rare. Usually, the classic signs of malignancy such as cellular invasion and metastasis are missing. A set of histologic criteria stratifies GIST for risk of malignant behavior such as mitotic activity and tumor size, cellular pleomorphism, developmental stage of the cell and quantity of cytoplasma [7,13]. Tumors with a high mitotic activity and size above 5 cm are considered malignant. Recent pharmacological advances such as tyrosine kinase inhibitors have determined c-kit (i.e., CD117) as the most important marker, amongst others. C-kit positive tumors respond extremely well to chemotherapy with Imatinib (Glivec, Gleevec) [10-12].     

Clinicopathological features of gastric glomus tumor

AIM:To study the clinicopathological features of gastric glomus tumor and review the related Chinese literature published in 1990-2010.METHODS:A case of gastric glomus tumor was re-ported.Clinicopathological findings in 56 cases of gastric glomus tumor were analyzed.RESULTS:Gastric glomus tumor was far more common in women than in men with a female to male ratio of 1.6:1.The median age of the patients was 45 years(range 28-79 years).The patients often complained of epigastric pain and bloody stool.The tumor was located in antrum of the stomach.The greatest diameter of the tumor was 0.8-11cm.Histologically,the tumor was comprised of nests of glomus cells surrounding the capillaries.Glomus cells were small,uniform and round.Vimentin,smooth muscle actin and actin were expressed in the tumor.Other markers,including S-100 protein,CD34,CD117,desmin,CD56,synaptophysin,chromo-granin A,neuron specific enolase and cytokeratin were all negative.CONCLUSION:Gastric glomus tumor is a rare benign mesenchymal neoplasm.Its diagnosis depends on pathologic examination.Differential diagnosis includes gastrointestinal stromal tumor,paraganglioma and carcinoid tumor.     

Duodenal somatostatinoma associated with Von Recklinghausen’s disease

Somatostatinomas are rare, malignant, somatostatin-producing neuroendocrine tumors with a prevalence of one in 40 million. The coincidence of Von Recklinghausen’s disease and duodenal somatostatinoma has been known since 1982. We report the case of a 57-year-old female patient with Von Recklinghausen’s disease and a tumor of the pancreatic head that was diagnosed due to painless icterus. Histopathological examination after pylorus-preserving pancreatoduodenectomy revealed the existence of a duodenal somatostatinoma with lymph node metastases. Characteristics of the association of von Recklinghausen’s disease and somatostatinoma, and therapy and prognosis will be discussed. In patients with Von Recklinghausen’s disease and an ampullary tumor, a somatostatinoma should be considered. In contrast to its pancreatic counterparts, duodenal somatostatinoma is frequently associated with Von Recklinghausen’s disease, often contains psammoma bodies, is rarely associated with a recognizable “somatostatin syndrome”, and is hardly ever associated with demonstrable metastases at the time of diagnosis. Small tumors arising in the duodenum may be treated with local excision, whereas larger tumors should be treated by total excision, which may entail a partial pancreatoduodenectomy.     

Amyloidosis localized in the sigmoid colon

A 79-year-old woman was admitted to our hospital presenting with bloody stool. Colonoscopic examination showed many irregularly-shaped discrete ulcerations in the sigmoid colon extending for a length of 6cm, situated 16cm proximal to the anl ring. Histopathology of the biopsy specimen in and around the ulcerations revealed the deposition of amyloid materials in the proper mucosa and the submucosa. Similar ulcerations were not detected by endoscopy in other parts of the gastrointestinal tract, i.e., esophagus, stomach, and duodenum. Multiple biopsy specimens taken from various parts of the gastrointestinal tract other than the sigmoid colon showed no amyloid deposition. Further investigations showed no particular abnormality in any organs and the patient was free of such systemic disease as myeloma or collagen disease. The findings were consistent with those of localized sigmoid amyloidosis. Localized colonic amyloidosis is rare, this being the fifth patient reported in Japan in the past 15 years.     

A case of arteriovenous malformation in the submucosal layer of the stomach

Arteriovenous malformation (AVM) of the stomach is extremely rare. We report a patient with asymptomatic gastric AVM detected during mass screening of the upper gastrointestinal tract. The patient, a 69-year-old female, had no history of gastro-intestinal bleeding. Endoscopy revealed a gastric submucosal tumor (3 cm in diameter) at the posterior wall below the esophago-cardiac junction. Endoscopic ultrasonography (EUS) showed a hypoechoic structure in the third layer of the stomach, suggesting gastric AVM in the submucosal layer. Complete resection of the AVM tissue was performed by strip biopsy with surgical resection. Histological examination showed AVM in the submucosal layer. EUS examination was useful for the diagnosis of gastric AVM in this case.     

Inflammatory fibroid polyp, a rare tumor of the appendix

We report the rare occurrence of an inflammatory fibroid polyp of the appendix. The lesion was diagnosed in a 33-year-old woman presenting with abdominal pain, fever and localized tenderness in right iliac fossa on abdominal palpation. CT-scan showed an 8 cm appendiceal mass and a laparoscopic appendectomy was consequently performed. On microscopic examination, the tumor consisted of spindle cells dispersed in a loose fibromyxoid stroma containing numerous blood cells and inflammatory cells with abundant eosinophils. On immunohistochemistry, the spindle tumor cells were positive for vimentin, fascin and focally for CD34 and CD35. They were negative for smooth muscle actin, desmin, CD21, CD23, CD117 and S100 protein. Inflammatory fibroid polyp is a rare benign mesenchymal tumor of the gastrointestinal tract rarely reported in the appendix. This tumor shares some common pathologic features with the myofibroblatic inflammatory tumor but they are two different entities. The pathogenesis of this tumor remains unclear but fascin and CD35 immunoreactivity of the tumor cells suggests a probable dendritic cell origin.     

A case of duodenal carcinoid tumor accompanied by liver metastasis and lymph node metastases in a patient with von Recklinghausen disease

We report a case of duodenal carcinoid tumor accompanied by liver metastasis and lymph node metastases in a patient with von Recklinghausen disease. A 48-year-old woman with von Recklinghausen disease was referred to our hospital because of a submucosal tumor at the ampulla of Vater detected by upper gastrointestinal endoscopy. The lesion was diagnosed as a carcinoid tumor based on the pathology of the biopsy specimen. At operation, although there were liver metastasis on the surface of S3 the liver and regional lymph node metastases, we selected pancreatoduodenectomy with lymph node dissection and enucleation of the liver metastasis. The postoperative course was good and the woman was discharged on postoperative day 33. No recurrence has been seen at 24 months since surgery.     

Metastatic follicular dendritic cell sarcoma of the stomach: a case report and review of the literature

Follicular dendritic cell (FDC) sarcomas are rare tumours, typically seen in lymph nodes. However, in about one third of the reported cases, a FDC sarcoma presents as an extranodal mass. Involvement of the gastrointestinal tract is extremely rare, and only 3 cases have been described to date. We report on a 40-year-old female patient with a follicular dendritic cell sarcoma located in the stomach and the presence of a metastasis in the liver at the time of diagnosis. Severe asthenia, nausea, back pain and loss of weight were the presenting symptoms. A CT scan of the abdomen and an upper gastrointestinal endoscopy revealed a tumour mass in the stomach. The diagnosis of a FDC sarcoma was made on histological and immunohistochemical findings. We report the second case of a FDC sarcoma presenting in the stomach. Due to its rarity, a FDC sarcoma seldom enters the differential diagnosis of spindle cells neoplasms of the gastrointestinal tract. Complete surgical resection is the treatment of choice for FDC sarcoma.     

Invagination intestinale aiguë sur une pseudotumeur fibreuse calcifiante : entité non rapportée précédemment

Introduction

Calcifying fibrous pseudotumor is an unusual benign tumor whose unique histological features consist of thick collagen bundles, scattered fibroblasts, psammomatous and dystrophic calcification. We present a case of calcifying fibrous pseudotumor of the small bowel. To our knowledge, this is the second such case to be described in the gastrointestinal tract.

Case report

The present case is a 37-year-old woman with occlusive syndrome. A computed tomography scan revealed a sign of acute intestinal invagination. The macroscopic examination of the intestinal resection indicated the presence of a white wall polyp measuring 1 × 1 cm with calcification. Histologic examination posed the diagnosis of calcifying fibrous pseudotumor. In immunohistochemistry spindle cells tested positive for vimentin and negative for smooth muscle actin, desmin, S100 protein, CD34 and bcl2.

Discussion

Calcifying fibrous pseudotumor is one of the benign tumors and is located mostly in soft tissues, pleura and peritoneum. Its location in the gastrointestinal tract is extremely rare. The pathogenesis remains uncertain. We discuss through our observation, accompanied by a review of the literature, the histopathological features, the problems of differential diagnosis and the surgical treatment of this most unusual case.     

Giant solitary fibrous tumor arising from greater omentum

Extrathoracic solitary fibrous tumors (SFTs) have been described at almost every anatomic location of human body, but reports of SFT in the abdominal cavity are rare. We herein present a rare case of SFT originating from greater omentum. Computed tomography revealed a 15.8 cm × 21.0 cm solid mass located at superior aspect of stomach. Open laparotomy confirmed its mesenchymal origin. Microscopically, its tissue was composed of non-organized and spindle-shaped cells exhibiting atypical nuclei, which were divided up by branching vessel and collagen bundles. Immunohistochemical staining showed that this tumor was negative for CD117, CD99, CD68, cytokeratin, calretinin, desmin, epithelial membrane antigen, F8 and S-100, but positive for CD34, bcl-2, α-smooth muscle actin and vimentin. The patient presented no evidence of recurrence during follow-up. SFT arising from abdominal cavity can be diagnosed by histological findings and immunohistochemical markers, especially for CD34 and bcl-2 positive cases.     

RARE GASTRIC CALCIFYING FIBROUS PSEUDOTUMOR REMOVED BY ENDOSCOPIC SUBMUCOSAL DISSECTION

We report a case of a calcifying fibrous pseudotumor of the stomach that we resected using endoscopic submucosal dissection (ESD). A 61‐year‐old male with a gastric submucosal tumor was admitted to our hospital for treatment. By upper gastrointestinal tract endoscopy, a smooth‐surfaced submucosal tumor measuring 2 cm in diameter was observed in the anterior wall of the middle body of the stomach. By endoscopic ultrasonography, a mass was observed in the stomach submucosa; the mass had a well‐defined boundary. Internally, the mass was heterogeneous and hypoechoic; high spots were scattered throughout the mass. Continuity between the mass and the muscularis mucosae and muscularis propria was not observed. Strongly suspecting that this mass was a gastrointestinal stromal tumor arising from the stomach, we resected the mass by ESD for total biopsy. Histopathologically, the mass consisted of proliferation of eosinophilic collagen fibers with plasma cell infiltration and lymphoid follicle proliferation. Calcification was also observed in some parts of the mass. Thus, the mass was identified as calcifying fibrous pseudotumor. Calcifying fibrous pseudotumor of the stomach is extremely rare and its histogenesis remains unclear; however, its morphology became distinct by comparing endoscopic/radiological and histopathological findings.     

Eosinophilic gastroenteritis with eosinophilic infiltration of the gall bladder

Eosinophilic gastroenteritis (EG) is a rare disease of unknown etiology characterized by eosinophilic infiltration of the gastrointestinal tract wall with various gastrointestinal manifestations. Several organs in the digestive tract may be involved by EG, but biliary tract involvements are rare. We report here a case of EG with eosinophilic infiltration of the gall bladder. A 57-year-old man was admitted to the hospital complaining of abdominal pain and diarrhea. As ultrasonography and magnetic resonance imaging showed thickened gall bladder wall and debris in the neck of gall bladder, we performed cholecystectomy, but his symptoms were not improved. Esophagogastroduodenoscopy showed multiple erosions, redness and edema in the duodenum and stomach. Biopsy specimens from the duodenum and stomach showed infiltration of eosinophils, plasma cells and lymphocytes. Microscopic findings of resected gall bladder showed eosinophilic infiltration into the all layers of gall bladder wall without vasculitis. Based on these findings, EG with eosinophilic infiltration of the gall bladder was diagnosed. Following oral steroid administration, clinical symptoms were immediately improved. One month after the therapy, esophagogastroduodenoscopy did not show any findings of gastritis or duodenitis, and there were no eosinophilic infiltrations in biopsy specimens from duodenum and stomach.     

Coexistence of hepatocellular carcinoma and gastrointestinal stromal tumor： A case report

Malignant gastrointestinal stromal tumors(GIST)are raremesenchymal tumors originating from the wall of thegastrointestinal tract.Their coexistence with other tumorsoriginating from other germ layers is unique.We havereported a case of a 63-year-old GIST patient presentingas an epigastric mass associated with hepatic tumor.Histologically,the mesenteric tumor was composed ofspindle cells showing both neural and smooth muscledifferentiation.Immunohistochemical examinationshowed positive staining for CD117,vimentin,S-100,and SMA,while CD34 antigen was negative.The hepatictumor was diagnosed as hepatocellular carcinoma(HCC).To the best of our knowledge,this is the first case ofGIST and HCC coexistence.The rarity of the case,however,should not lead to ignoring such a possibility indifferential diagnosis.     

Giant primary angiosarcoma of the small intestine showing severe sepsis

Primary malignant tumors of the small intestine are rare,comprising less than 2%of all gastrointestinal tumors.An 85-year-old woman was admitted with fever of 40℃ and marked abdominal distension.Her medical history was unremarkable,but blood examination showed elevated inflammatory markers.Abdominal computed tomography showed a giant tumor with central necrosis,extending from the epigastrium to the pelvic cavity.Giant gastrointestinal stromal tumor of the small intestine communicating with the gastrointestinal tract or with superimposed infection was suspected.Because no improvement occurred in response to antibiotics,surgery was performed.Laparotomy revealed giant hemorrhagic tumor adherent to the small intestine and occupying the peritoneal cavity.The giant tumor was a solid tumor weighing 3490 g,measuring24 cm×17.5 cm×18 cm and showing marked necrosis.Histologically,the tumor comprised spindle-shaped cells with anaplastic large nuclei.Immunohistochemical studies showed tumor cells positive for vimentin,CD31,and factorⅧ-related antigen,but negative for c-kit and CD34.Angiosarcoma was diagnosed.Although no postoperative complications occurred,the patient experienced enlargement of multiple metastatic tumors in the abdominal cavity and died 42 d postoperatively.The prognosis of small intestinal angiosarcoma is very poor,even after volume-reducing palliative surgery.