Rhabdomyosarcoma of the duodenum: report of a case.

This report presents a case of primary pleomorphic rhabdomyosarcoma arising in the duodenum. A 63-year-old male with persistent melena was referred for a solid tumor in his right upper abdomen detected using ultrasonography. Gastrofiberscopy revealed a protrusion in the upper part of the duodenum, with a large ulcer on the top of it. Enhanced computed tomography showed that the tumor extended to the pancreas. Pancreaticoduodenectomy was performed, despite the absence of malignant cells in the biopsy specimen, with a preoperative diagnosis of duodenal cancer. The tumor consisted of multiple cell types, and immunohistochemical staining was positive for desmin, HHF-35 and alpha smooth muscle actin. Electron microscopy revealed primitive Z-band structures in the tumor. The final diagnosis was pleomorphic rhabdomyosarcoma of the duodenum. This is the first report of primary rhabdomyosarcoma occurring in the duodenum, confirmed by immunohistochemical staining and electron microscopy.

Primary Ewing's sarcoma of the suodenum: a case report.

We report a case of Ewing's sarcoma arising from the duodenum in a 20-year-old woman who presented with a rapidly progressive ulcerative lesion. The surgical specimen obtained via Whipple's operation revealed a small round-cell tumor (SRCT) in the first and second portion of the duodenum. The tumor cells revealed strong immunore-activity for CD 99 and vimentin and focal paranuclear dot-like immunoreactivity for cytokeratin. Electron microscopy showed primitive tumor cells with few cytoplasmic organelles, but neither neurosecretory granules nor specific cell junctions were present. On Western blot study, 68-kDa EWS/FLI1 fusion protein was detected. The occurrence of Ewing's sarcoma in the gastrointestinal hollow viscus has recently been recognized, and this case expands the known anatomic sites that can harbor Ewing's sarcoma by demonstrating primary duodenal involvement.

[Ultrastructural phenotypes of tumor cells of endocrine-cellular neoplasms of hepatopancreatoduodenal organs and their role in determining the degree of malignancy and prognosis of these diseases]. / Ul'trastrukturnye fenotipy opukholevykh kletok éndokrinno-kletochnykh novoobrazovanii organov gepatopankreatoduodenal'noi zony i ikh znachenie v opredelenii stepeni zlokachestvennosti i prognoza étikh zabolevanii.

78 tumors of the hepatopancreoduodenal system were studied clinically, cytologically and ultrastructurally. Hormonal disturbances were observed in 36% of the patients. The 5-year survival after radical surgery did not depend on the tumor size, tumor cell atypia or metastases to the regional lymph nodes. The prognosis was worse when the tumor was located in the liver. Ultrastructural features of the tumor cells were reliable criteria of the malignancy degree and tumor prognosis. The prognosis, recurrence-free interval and survival improve with an increase in the number of ultrastructurally differentiated cells and organoids in cytoplasm, and with a reduction in nuclear polymorphism and number of dark cells. The degree of histologic and ultrastructural differentiation of tumor cells may not coincide.

Numerous eosinophilic globules (skeinoid fibers) in a duodenal stromal tumor: an exceptional case showing smooth muscle differentiation.

A unique case of duodenal stromal tumor in a 51-year-old man is reported. The tumor histologically showed spindle cell proliferation and numerous eosinophilic globules. Most globules were composed of tangled 45 nm thick fibrils, which were ultrastructurally identical to 'skeinoid fibers'. The presence of glycogen granules in the tumor cells and the immunoreactivity for alpha-smooth muscle actin suggested smooth muscle differentiation. Focal ultrastructural findings also supported the smooth muscle nature of this tumor. There were no immunohistochemical and ultrastructural features indicating neural differentiation. In previous studies, the presence of such 'skeinoid fibers' was suggested to be a histological marker for neural differentiation in gastrointestinal stromal tumor. However, the findings in the present case suggest that numerous 'skeinoid fibers' can be identified in duodenal stromal tumor with smooth muscle differentiation, although this condition may be rare.

Gangliocytic paraganglioma of the duodenum: report of a case with immunocytochemical and ultrastructural investigation.

A case of gangliocytic paraganglioma is reported in a 70-year-old female presenting as a polypoid tumor of the second portion of the duodenum. Immunohistochemical and ultrastructural features of the tumor indicate that gangliocytic paraganglioma of the duodenum (GPD) represents an unique tumor originating from the neuroectodermal derivative exhibiting dual phenotypic expression toward paraganglionic and epithelial (neuroendocrine) cells. These findings correlate well with the literature and it is further suggested that GPD belongs to the histopathologic spectrum of tumors derived from the neural crest. The findings offer a plausible explanation for histogenetic possibilities of the occurrence of pure epithelial tumors (i.e., carcinoid-like tumors) in the paraganglionic systems. The patient remains with no evidence of disease 4 years following a simple polypectomy.

Sarcomatoid carcinoma of the small intestine.

A case of sarcomatoid carcinoma of the small intestine in a 76 year old Chinese woman is presented. The diagnosis was confirmed by light microscopic, immunohistochemical and ultrastructural findings. It is a rare neoplasm, with only 13 previous reported cases in the literature. Patients with the disease usually had a poor prognosis. The nomenclature, histological and immunohistochemical findings of these tumours were diverse. To our knowledge, this case is the first report of this disease entity in a Chinese patient, and has its distinctive features. The patient was the oldest one in literature with this disease, and had the shortest survival time after the presentation of symptoms.

Aberrant insulinoma of the duodenal bulb.

A rare insulin-immunoreactive neuroendocrine tumor of the duodenum in a 54 year old male is reported. The incidentally identified tumor was located on the anterior free wall of the duodenal bulb and measured approximately 6 mm in diameter. Uncomplicated endoscopic resection of the tumor was carried out. The lesion exhibited classic histologic features of insulinoma of the beta-islet cell type with stromal amyloid deposition. In addition to positive reactivities of chromogranin A, neuron-specific enolase, synaptophysin, Leu 7 (CD57), cystatin C, CA15-3 and cytokeratin, the non-argyrophilic tumor cells were strongly immunoreactive for insulin and C-peptide. The stromal amyloid was clearly labeled for amylin. A few cells were stained for somatostatin, whereas other hormones were negative. Interestingly, a few isolated insulin-positive cells were identified in the non-neoplastic duodenal mucosa in the proximity of the tumor. Immunoelectron microscopy using paraffin sections disclosed insulin-immunoreactive secretory granules in the cytoplasm. The patient exhibited no signs or symptoms of hypoglycemia. Serum insulin levels were not measured prior to resection. No tumors were demonstrated in the pancreas. Magnetic resonance imaging revealed a 1 cm asymptomatic pituitary mass, in association with moderately elevated serum prolactin levels. The patient is currently being followed up in the outpatient clinic.

Cytogenetically detected clonal heterogeneity in a duodenal adenocarcinoma.

A primary duodenal adenocarcinoma, a tumor type for which no previous chromosome data existed, was cytogenetically analyzed after short-term culture. The main tumor mass was localized in the pancreatic head, but the histopathologic examination revealed its duodenal origin. A total of six abnormal, karyotypically unrelated, clones were identified. The largest exhibited clonal evolution and consisted of two subclones with massively rearranged karyotypes in the hypodiploid and hypotetraploid range. Chromosome imbalances brought about by these complex changes were gain of 1q, losses of chromosomes 6 and 9, and total or partial losses of 1p, 3p, 3q, 9p, 10p, 17p, 17q, 18q, 20p, and 20q. The remaining five smaller clones had 1-2 numerical or balanced structural chromosome aberrations. The present study thus revealed yet another epithelial tumor type characterized by karyotypically unrelated clones. For this as for other tumors, the pathogenetic significance of such cytogenetic polyclonality remains uncertain.

Von Recklinghausen's disease associated with duodenal somatostatinoma: contrast of duodenal versus pancreatic somatostatinomas.

A case of Von Recklinghausen's disease with duodenal somatostatinoma is reported. A search of the world's literature revealed 27 patients with Von Recklinghausen's disease associated with an immunohistologically proved duodenal somatostatinoma. Twenty-nine cases of duodenal somatostatinoma not associated with Von Recklinghausen's disease and 32 cases of pancreatic somatostatinomas have been identified for comparison. While their histology may be similar in many respects, the duodenal and pancreatic somatostatinomas show significant differences, especially in hormonal and growth behaviors. In contrast to its pancreatic counterpart, the duodenal somatostatinoma is frequently associated with Von Recklinghausen's disease, is seldom associated with a recognizable "somatostatin syndrome," often contains psammoma bodies, and is less frequently associated with demonstrable metastases at the time of operation.

Bombesin receptors in a human duodenal tumor cell line: binding properties and function.

The bombesin family of peptides elicit numerous biological responses in the gut, including stimulation of cell proliferation, and have been implicated as growth factors in a variety of gastrointestinal tumors. Even though these peptides and their receptors are distributed throughout the gastrointestinal tract, there are few cell lines available as model systems to study bombesin action in gastrointestinal cells. In this study, we have characterized functional bombesin receptors in a human duodenal cancer cell line, HuTu-80. The binding of [125I-Tyr4]bombesin to intact cells at 4 degrees C reached equilibrium by 6 h. Scatchard analysis of [125I-Tyr4]bombesin binding showed that HuTu-80 cells contained a single class of high affinity binding sites (5900 +/- 1960/cell; Kd = 80 +/- 20 pM). [125I-Tyr4]bombesin binding was inhibited by bombesin receptor agonists and antagonists with the following order of potencies: gastrin-releasing peptide (GRP) = GRP-(14-27) = bombesin > [DPhe6]bombesin(6-13)ethylamide > [Leu13 psi-(CH2NH)Leu14]bombesin > neuromedin B. Photoaffinity cross-linking studies, in which N-5-azido-2-nitrobenzoyloxysuccinimide was used to covalently couple [125I]GRP(14-27) to cells at 4 degrees C, resulted in the specific labeling of a broad band with an apparent molecular mass of 66,000 daltons. Consistent with the presence of high affinity receptors, bombesin increased the formation of inositol phosphates in HuTu-80 cells in a dose-dependent manner (concentration eliciting half-maximal effect, 290 +/- 70 pM). However, under conditions where both insulin and serum increased [3H]thymidine incorporation into DNA, 10 nM bombesin had no effect either alone or in the presence of insulin. Bombesin also had no effect on colony formation by HuTu-80 cells in soft agar. Furthermore, the bombesin receptor antagonist, [Leu13 psi(CH2NH)Leu14]bombesin, did not inhibit [3H]thymidine incorporation or clonal growth either in the absence or in the presence of serum. Together, these results show that HuTu-80 cells contain high affinity bombesin receptors of the GRP subtype. These receptors are functionally coupled to second messenger production but do not stimulate cell proliferation.

Tripartite differentiation in a carcinoma of the duodenum.

BACKGROUND: Carcinomas containing three distinctly different cell lines have been encountered in the colon and rectum, but a tripartite malignancy in the small intestine has not been reported previously. METHODS: A duodenal carcinoma was studied by light and electron microscopic examination and immunohistochemistry. RESULTS: The duodenal carcinoma was found to have tripartite glandular, squamous, and neuroendocrine differentiation. Histologically, an adenocarcinoma, which originated in a villous adenoma, was continuous with squamous cell carcinoma and small cell carcinoma components. Tumor cells of the squamous cell carcinoma component had conspicuous intercellular bridges but did not form keratin pearls. Immunohistochemical analysis showed strong expression of carcinoembryonic antigen (CEA) by the adenocarcinomatous component. The squamous cell carcinoma component demonstrated focal weak CEA and neuron specific enolase (NSE) reactivity. Ultrastructurally, tumor cells of this component had frequent desmosomes and free tonofilaments. The small cell carcinoma had clusters of dense core granules in tumor cell cytoplasmic processes, which are indicative of neuroendocrine differentiation. This neuroendocrine component was immunoreactive for somatostatin and NSE. CONCLUSIONS: This case of tripartite duodenal carcinoma supports the theory of an origin from an intestinal pluripotential stem cell capable of differentiating into multiple cell types.

An immunohistochemical and ultrastructural study of case of small-cell neuroendocrine carcinoma in the ampullary region of the duodenum.

One case of small-cell neuroendocrine carcinoma in the ampullary region of the duodenum is reported. The histological appearance of the tumor was identical to pulmonary small-cell carcinoma. Neuroendocrine differentiation was demonstrated immunohistochemically by positive immunoreaction for neuron specific enolase, Leu-7 and chromogranin, and ultrastructurally by the presence of scanty dense-core neurosecretory type granules. Small-cell neuroendocrine carcinoma in the ampulla of Vater is extremely rare. To our knowledge, this is the sixth reported case.

Argyrophil-negative G-cell duodenal carcinoid.

A case of mono-hormonal-producing and functionally active G-cell carcinoid tumor of the duodenum is described. The tumor, found to be argyrophil negative, was investigated immunohistochemically and ultrastructurally. We report our results, then discuss the possible histochemical causes that can lead to the failure of argyrophilia, and direct attention to the possible misdiagnosis that can result from that failure.

Small intestinal stromal tumors with skeinoid fibers. Clinicopathological, immunohistochemical, and ultrastructural investigations.

Microscopic appearances of spindle cell tumors of the gastrointestinal tract are suggestive of smooth muscle origin; however, they usually lack specific muscle cell features by electron microscopy and immunohistochemistry, thus justifying their designation as stromal tumors. The present report describes nine cases of small intestinal stromal tumors with eosinophilic stromal globules composed of tangles of curved fibers with crossbands simulating an appearance of skeins, designated as skeinoid fibers. Patients' ages ranged from 28 to 87 years; and four were male. The tumors presented as well-delineated mural nodules ranging from 1.8 to 13 cm in size, causing intestinal obstruction or hemorrhage. Four were in the duodenum, three in the jejunum, and two unspecified. Microscopically, seven were benign; one, to the largest, was definitely malignant and metastasized to the liver. Another, the second largest (7.5 cm), showed moderate atypia with two mitoses per 10 high-power fields. The light microscopic appearance, including immunohistochemistry, were typical for small intestinal stromal tumors. Skeinoid fibers were strongly periodate-Schiff's procedure-positive and stained blue with the trichrome stain. They appeared as a few micra-sized specks to large globules reaching a few millimeters. Skeinoid fibers were also found in three neurogenic spindle cell tumors (an acoustic neuroma, a neurofibroma, and a plexosarcoma of the mesentery), suggesting that such fibers are possible ultrastructural markers for neurogenic tumors and thus small intestinal stromal tumors with skeinoid fibers are neurogenic in origin.

Exocrine pancreatic insufficiency and pancreatic fibrosis due to duodenal somatostatinoma in a patient with neurofibromatosis.

A case of duodenal somatostatinoma is described in a patient with Von Recklinghausen neurofibromatosis. The patient presented with exocrine pancreatic insufficiency, probably due to distal obstruction of the pancreatic duct by the tumor. Preoperative evaluation with calcium-pentagastrin and tolbutamide stimulation tests were nondiagnostic. At laparotomy, local excision of the tumor was performed. Pathological findings were compatible with duodenal somatostatinoma, causing pancreatic fibrosis. Somatostatin extracted from the tumor coeluted with the somatostatin-14 standard on high performance liquid chromatography (HPLC).

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.

Pathology and clinical features of gastro-intestinal lymphoma in Saudi Arabia.

The histopathology and clinical presentation of 19 cases of primary gastrointestinal lymphoma is described. Our patients are similar to others in the Middle East but have a lower incidence of diarrhoea and malabsorption. All revealed a widespread chronic inflammatory background. Four patients with primary gastric lymphoma had endoscopic biopsies from the duodenum; these biopsies were completely free from tumour but showed a moderate to severe diffuse lymphoplasmacytic infiltrate. Electron microscopy shows that tumour cells penetrate basement membranes and invade the epithelium which becomes thin and attenuated. Substances which inhibit lymphocyte tropism might be useful in preventing intestinal ulceration. The MALT concept has been found useful in classification of the tumours. Two patients with unusual mesenteric node histology are described and it is thought that the appearances may indicate a substantial capacity for differentiation in the group of tumours.

Cronkhite-Canada syndrome. Light and electron microscopy of the cutaneous pigmentary abnormalities.

This article describes the light and electron microscopic studies from a macule and the surrounding lightly hyperpigmented skin of a patient with the Cronkhite-Canada syndrome. Increased numbers of melanin granules in keratinocytes, increased numbers of melanosomes in melanocytes, and areas with increased numbers of melanocytes were found in the macular lesion. Skin from both the macule and surrounding area were also characterized by compact hyperkeratosis as well as perivascular inflammation and exocytosis not previously reported in these patients.