Malignant gastrointestinal stromal tumor of the ampulla of Vater presenting with obstructive jaundice

Malignant gastrointestinal stromal tumor (GIST) consists a rare neoplasm, developing in small intestine and stomach. The presenting manifastations include weakness, weight loss, nausea, melena and anaemia. The present case refers to a 65 years old female patient with a GIST of the ampulla of Vater presenting with obstructive jaundice. Diagnosis was achieved pre-operatively by biopsies collected through diagnostic ERCP. The tumour was locally excised, with preservation of the ampulla. The histological analysis suggested low grade GIST positive for both CD 117 (c-kit) and CD34. Two years after the surgery the patient remains free of disease. Malignant GIST of the ampulla of the Vater is extremely rare as only few similar cases have been described in the literature. This is the first time a GIST being presented as obstructive jaundice ever reported. Despite the unavailability of EUS-FNA, the diagnosis was set preoperatively and the tumor was resected.     

Mucinous adenocarcinoma with superficial stromal invasion and villous adenoma of urachal remnants: a case report

This report describes a case of mucinous adenocarcinoma with superficial stromal invasion and villous adenoma originating in the dome of the urinary bladder. Although no urachal remnants were identified, the location suggested urachal derivation. Only two previous cases of urachal adenocarcinoma with features of early stromal invasion associated with a villous tumour have been described.     

Mixed acinar-endocrine carcinoma arising in the ampulla of Vater

We present a case of mixed acinar-endocrine carcinoma arising in the periampullary region of the duodenum. The patient was a 78-year-old male with a periampullary mass diagnosed during upper endoscopy. On gross dissection, the mass was 2.3 cm in diameter, noncystic, and confined to the duodenal submucosa. Microscopically, the tumor formed nests that were positive for amylase, trypsin (weakly), and synaptophysin (diffusely). Ultrastructurally, the tumor had 2 populations of granules with mean diameters of 175 nm and 540 nm, consistent with endocrine and zymogen granules, respectively. These studies were consistent with a mixed acinar-endocrine carcinoma that arose in the duodenum. A review of the literature revealed 1 report of an acinar cell carcinoma arising in jejunal pancreatic heterotopia. The present article is the first reported case of an acinar cell carcinoma arising in the periampullary region of the duodenum, possibly in a focus of pancreatic heterotopia.     

Adenocarcinoma arising in gastric heterotopic pancreas: a case report

A heterotopic pancreas in the gastrointestinal tract is mostly found incidentally and its malignant transformation is extremely rare. We describe the second case of adenocarcinoma arising in a gastric heterotopic pancreas of an asymptomatic 35-yr-old man in Korea. Esophagogastroduodenoscopy revealed a submucosal tumor with an irregular central umbilication in the gastric antrum. A wedge resection specimen demonstrated a submucosal oligolocular cystic mass (1.7 x 1.4 x 1.2 cm) with a solid portion. Microscopically, the cystic portion was composed of dilated pancreaticobiliary type ducts with adjacent small foci of periductal glandular structures. The adenocarcinoma components in the solid area infiltrated the proper muscle and the overlying mucosa of the stomach. The transitional area between the benign ductal structures and the adenocarcinoma component was found. The follow-up course was uneventful 5 months postoperatively.     

Gastrointestinal stromal tumor of the duodenum: a case report.

The report describes a malignant gastrointestinal stromal tumor occurring in the duodenum in a 71-year-old woman. The neoplasm showed both epithelioid and spindle cell patterns by light microscopy. The ultrastructural features were diagnostic of nerve sheath origin. The tumor had numerous wrapping processes joined by junctions and surrounded by axons. No features of smooth muscle differentiation were identified. Immunocytochemistry was inconclusive. The differential diagnosis of such neoplasms is discussed.     

Paneth-like cells in an adenoma and adenocarcinoma in the ampulla of Vater

A case of adenocarcinoma with significant Paneth cell differentiation arising in a villoglandular polyp at the ampulla of Vater is presented. Paneth-like cells, containing distinct fuchsinophilic granules, were a prominent component of the nonglandular invasive adenocarcinoma and were also seen in the associated adenomatous polyp. Lysozyme, trichrome, and periodic acid-Schiff digest stains, and electron microscopy confirmed that the granules were lysosomal in nature. This case confirms that cells with Paneth-like features can be a significant component in invasive neoplasms and can occur at unusual sites such as the ampulla of Vater.     

Oncocytic adenocarcinoma of the rectum arising on a villous adenoma with oncocytic features

Rectal adenocarcinoma with diffuse oncocytic features is a very rare lesion, having been reported only once in the English literature. We describe a case of oncocytic adenocarcinoma of the rectum, associated with a villous adenoma, arising on a 66-year-old man. On histological examination, the adenocarcinoma was composed of neoplastic glands lined by a strongly eosinophilic, granular epithelium that deeply infiltrated the rectal wall. Some basophilic calcifications were present in the gland lumina. Superficially, a villous adenoma with high-grade dysplasia was evident; adenomatous cells showed focal eosinophilic changes, consisting of a large granular cytoplasm, an oval atypical nucleus, and a prominent nucleolus. Immunohistochemically, neoplastic glands reacted strongly with antimitochondria antibody, carcinoembryonic antigen, cytokeratin 20, p53, and CDX2. Molecular alterations observed in oncocytic changes and their significance with regards to neoplastic transformation are briefly discussed.     

Primary squamous cell carcinoma of the colon arising in a villous adenoma

The first case of pure squamous cell carcinoma arising in a villous adenoma of the cecum is reported, and the literature of squamous cell carcinoma of the colon is reviewed. The possible origin of this neoplasm in adenomas of the colon, similar to that of adenocarcinoma, is discussed, and the site distributions of adenocarcinoma and squamous cell carcinoma are compared. The distribution of primary colonic squamous cell carcinoma is found to be predominantly right-sided in comparison to adenocarcinoma (p less than 0.05). Although this result does not preclude adenomas as the origin of most squamous cell carcinomas, it suggests the influence of as yet unknown site-asymmetric factors that are different from those for adenocarcinoma.     

肠系膜低度恶性子宫内膜间质肉瘤一例

患者女,46岁。因反复腹泻2年余,加重伴腹痛1d于2003年8月7日入院。患者分别于2年前,5个月前出现腹泻,持续3～5d后可缓解,一天多达10余次,大便呈黄色糊状,里急后重,无黏液脓血,腹痛,呕吐,与月经无明确关系。     

前列腺恶性胃肠道间质瘤一例

患者男,49岁。因无明显诱因感会阴部酸痛不适10d。无尿急、尿频及尿痛,无血尿,在当地医院就诊,B超检查发现盆腔前列腺部位实性占位性病变,CT检查发现前列腺增大约8·03cm×6·58cm,提示前列腺肿瘤。实验室检查:前列腺特异性抗原1·11ng/ml,血清游离前列腺特异性抗原0·2ng/ml,为进一步诊治于2005年3月入本院治疗。直肠指诊:前列腺增大约6cm×8cm,质坚硬,无压痛,中央沟消失,表面不平。X线和ECT检查未发现转移病灶。临床初步诊断为:前列腺肿瘤,肉瘤可能。患者入院1周后行前列腺肿瘤根治术,手术顺利,术中未发现盆腔肿大淋巴结。病理检查:带…     

Malignant gastrointestinal stromal tumor of intestine: a case report

Smooth muscle tumors of the alimentary tract are uncommon.Cancer of small intestine comprises less than 20% of all malignant tumors. A 65-year-old male patient was admitted with complain of pain in abdomen since 7 days. He was diagnosed as a case of acute intestinal obstruction and on laparotomy an extraluminal mass was found at jejunoileal junction. Histopathology revealed a malignant gastrointestinal stromal tumor (GIST) which was confirmed by immunohistochemistry. The case is reported with review of literature and criteria for differentiation between benign and malignant tumors are enumerated.     

Cytological features of mixed adenoneuroendocrine carcinoma of the ampulla of Vater: A case report with immunocytochemical analyses

Mixed adenoneuroendocrine carcinoma (MANEC) is defined as a tumor that has morphologically recognizable both adenocarcinoma and neuroendocrine carcinoma components comprising at least 30% of either components. MANEC occurring in the ampulla of Vater is extremely rare, and only 16 cases have been reported in the English language literature. In the present report, we describe the first case of MANEC of the ampulla of Vater with immunocytochemical analyses. An 82‐year‐old Japanese male was incidentally found to have a tumorous lesion in the ampulla of Vater. Endoscopic ultrasound‐fine needle aspiration (EUS‐FNA) of the tumor was performed. The Papanicolaou smear demonstrated the presence of different three components. The most dominant component was cohesive clusters of small round cells with round to oval nuclei with powdery chromatin and scant cytoplasm, which corresponded to small cell carcinoma. The second component was an adenocarcinoma, which was composed of irregularly overlapping clusters of tall columnar cells with large round to oval nuclei containing conspicuous nucleoli. The third component was an adenoma, which was comprised of flat cohesive clusters of columnar cells without atypia. Immunocytochemical analyses demonstrated that synaptophysin was expressed in the small round cells, and cdx‐2 was expressed in all three components. Accordingly, a cytodiagnosis of MANEC with adenoma component was made. Preoperative diagnosis of ampullary MANEC is difficult. However, this report clearly demonstrates three different components in the EUS‐FNA cytological specimen. Therefore, we suggest that cytological examination is a useful method for diagnosis of MANEC of the ampulla of Vater.     

Presacral carcinoid tumour arising in a tailgut cyst--a case report

Primary carcinoid tumours of the presacral region are extremely infrequent with just a handful of such cases reported in literature. Tailgut cysts(retrorectal cystic hamartoma) are also very uncommon lesions which are usually identified in adult life. They are developmental abnormalities and consist of multiloculated cysts lined by squamous, transitional or glandular epithelium. Malignant transformation within tailgut cysts is rare; the tumours which arise include carcinoid tumours and adenocarcinomas. We report the unusual occurrence of a carcinoid tumour developing within a tailgut cyst.     

Significance of GLUT1 expression in adenocarcinoma and adenoma of the ampulla of Vater

The expression of glucose transporter protein 1 (GLUT1) in malignant tumors is increased due to the higher metabolic needs of the proliferating cell populations. Aberrant GLUT1 expression is exhibited in a wide spectrum of epithelial malignancies and their precursors, which occur with low frequency and intensity; aberrant GLUT1 expression does not occur in normal epithelial cells. The expression of GLUT1 in tumors of the ampulla of Vater was evaluated on immunohistochemistry, and the relationships of GLUT1 expression to histological parameters and p53 expression were analyzed. Twenty-one (58.3%) of 36 adenocarcinomas and three (17.6%) of 17 adenomas had GLUT1 immunoreactivity. None of the regenerating or normal epithelia had any immunoreactivity. No significant relationships were found between GLUT1 expression and histological parameters or p53 expression. It was found that histological subtypes originated from different epithelium were strongly related to different macroscopic types. In the ampulla of Vater, GLUT1 expression was associated with malignant change, and might be a useful marker of malignancy.     

A case of adenocarcinoma with enteroblastic differentiation of the ampulla of Vater

Cancer of the ampulla of Vater is rare, though it has various histological types and its histogenesis is fascinating in view of the anatomically complex nature of the ampulla. Fetal gut‐like adenocarcinoma, usually found in the stomach, can also develop in the ampullary region in extremely rare cases. Here we present a case of ampullary adenocarcinoma with enteroblastic and neuroendocrine differentiation. A 55‐year‐old woman presented with an epigastric pain. Endoscopic examination revealed a 2‐cm submucosal tumor‐like lesion in the ampulla. The surgical specimen showed that an exposed protruding type of tumor appeared as a well‐demarcated whitish‐yellow solid mass. Microscopically, the tumor had proliferated in the common channel and invaded the duodenal submucosa with mucosal lesion of intestinal‐type adenocarcinoma. The main tumor consisted of three different histological types showing transitional areas: adenocarcinoma with enteroblastic differentiation (ENT), neuroendocrine carcinoma (NEC), and well differentiated adenocarcinoma (WEL). Morphologically the ENT resembled fetal gut and immunohistochemically expressed SALL4 and glypican 3. The WEL was positive for CK20 and CDX2, revealing an intestinal‐type phenotype. AFP and HepPar1 were not evident in any part of the lesion. We speculated this tumor had arisen from intestinal‐type adenocarcinoma of the common channel and acquired enteroblastic and neuroendocrine differentiation during growth.     

Adenocarcinoma arising in a traditional serrated adenoma of the rectosigmoid colon with osseous metaplasia

Within the gastrointestinal tract, osseous metaplasia is an extremely rare phenomenon. It has only recently been described within a traditional serrated adenoma. Serrated colorectal carcinoma is the end point of the serrated neoplasia pathway. Left sided lesions typically arise from traditional serrated adenomas and represent only 8% of colorectal carcinomas. Herein reported is a case of colorectal adenocarcinoma arising from a traditional serrated adenoma with the rare occurrence of osseous metaplasia within the adenoma. The significance of the finding is discussed.     

Paratesticular malignant peripheral nerve sheath tumour: a case report

Primary malignant peripheral nerve sheath tumours (MPNST) of intrascrotal extra testicular site are extremely rare with only few cases reported in literature. These are highly malignant tumours most often associated with neurofibromatosis. The incidence in general population is 0.001%. A 35-year-old male presented with swelling of the scrotal sac of three months duration. There were no features of neurofibromatosis. The lesion was excised and was diagnosed as MPNST. The patient developed recurrence within two months.     

Adenocarcinoma arising in gastric inverted hyperplastic polyp: a case report and review of the literature

A 54-year-old man, found to have a submucosal tumor in the stomach by double contrast roentgenography, endoscopy, and endoscopic ultrasonography, underwent a laparoscopic partial gastrectomy. The pathological examination revealed that the lesion, measuring 45 mm x 35 mm, was an inverted hyperplastic polyp (IHP) located in the submucosal layer and consisting mostly of columnar cells resembling foveolar epithelium and pyloric gland cells. Notably, adenocarcinoma with adjacent dysplasia was observed in the submucosal glands. Transition from hyperplasia to dysplasia and from dysplasia to adenocarcinoma was noted. The adenocarcinoma component was intensely and diffusely positive for p53 overexpression, while the dysplasia component showed only weak and focal positivity, suggesting a role of p53 mutation in the dysplasia-carcinoma sequence. Gastric IHP is very rare, and only 31 cases (in 29 patients) have been reported. Five of these IHPs coexisted with gastric adenocarcinomas, which had all developed separately from the IHP lesions. Therefore, this is the first case of adenocarcinoma arising within gastric IHP itself.