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[A case of a patient with multiple primary neoplasms: ileal leiomyosarcoma and bronchial carcinoid].

Small bowel leiomyosarcoma and lung carcinoid are uncommon neoplasms, and the association of these two neoplasms has never been reported in the literature. In the present article we report the case of a man who developed carcinoid of the lung three years after being operated for leiomyosarcoma of the small intestine. The short time elapsed between the two diagnoses adds further interest to this case.

Bronchial Neoplasms↗

Primary jejuno-ileal neoplasms in eastern Nigeria.

Small intestinal neoplasms are uncommon with reported incidences of less than 1% of GI neoplasms. A retrospective review of cases of small intestinal neoplasms seen by the authors in a ten-year period is presented. Ten cases were seen during the period (8 females and 2 males). Seven patients were aged less than 20 years while the rest were aged above 2 years. Six patients presented with intestinal obstruction, 3 with features of chronic ill-health while 1 was an incidental finding. The ileum was involved in 5 patients, the jejunum in 4 while 1 showed multiple gut involvement. One patient had a benign lesion (Peutz-Jeghers Syndrome). The rest consisted of lymphosarcoma [5],adenocarcinoma [3] while 1 patient had leiomyo-sarcoma. Treatment offered included resection of small gut in 7 patients and ileo-colectomy in 3 patients. Three patients with lymphosarcoma had a full course of cytotoxic chemotherapy. The outcome was poor; 2 patients were alive after 3 years, 3 died within 6 months of surgery while the rest were lost to follow-up at variable periods after surgery. Neoplasms of the small gut presents late in our environment. Lymphosarcoma seems commoner in childhood and carries a better prognosis.

Adolescent↗

Recurrent lower gastrointestinal hemorrhage: ileal neoplasm diagnosed by scintigraphy with Tc 99m red blood cells and angiography.

A 71-year-old man with recurrent lower gastrointestinal bleeding had negative colonoscopic findings on three occasions. Preoperative diagnosis of a large stromal tumor in the right lower quadrant was based on scintigraphy with tagged red blood cells and mesenteric arteriographic features. The use of nuclear scintigraphy in the diagnosis and evaluation of stromal tumor of unknown malignant potential has not been previously described.

Aged↗

[Morphology, DNA cytophotometry and prognosis in gastrointestinal neuroendocrine tumours (carcinoids). A clinico-pathological study of 95 patients].

A total of 123 manifestations (97 primary tumours and 26 metastases) of neuroendocrine tumours of the gastrointestinal tract observed in 95 patients was investigated for the prognostic value of clinical, histological and DNA cytophotometric parameters. Metastases almost exclusively occurred among ileal carcinoids, which also were responsible for all 14 cases of lethal outcome observed during the follow-up period of mean 42 months. Aneuploid DNA values could be determined significantly more frequently among ileal than in non-ileal carcinoids and showed - upon analysis of the total group of gastrointestinal neuroendocrine tumours - a significant correlation to lethal course of disease. In addition, among 18 cases with primary and secondary carcinoid manifestations available for DNA cytophotometry, an association between the DNA content of metastatic neuroendocrine tumours and prognosis came to light. When applied to the group of ileal neoplasms, however, the parameter DNA content did not allow a better prognostic assessment.

Adult↗

[Our experience in the surgical treatment of gastrointestinal stromal tumors].

The authors carry out a retrospective review of 30 patients with gastrointestinal stromal tumours (GISTs) who underwent surgical treatment over the period from 1974 to 2001. Sixteen were male and 14 female, with an average age of 60.9 years. Histologically, 19 tumours showed evidence of differentiation towards smooth muscle elements (10 benign and 9 malignant), 9 towards neural elements (3 benign and 6 malignant) and 2 iacked differentiation towards either cell type. Twenty-one tumours were located in the stomach, 1 in the duodenum, 3 in the jejunum and 5 in the ileum. The main symptoms were abdominal pain and abdominal masses, and the most sensitive diagnostic techniques were abdominal CT scan and endoscopy in gastroduodenal locations. In 21 gastric GISTs, the surgical procedures were local resection (15 cases), partial gastric resection (3 cases), subtotal gastrectomy (2 cases) and total gastrectomy (1 case). In 8 small bowel GISTs, we performed a typical intestinal resection while duodenal undifferentiated GIST was managed by pancreatico-duodenectomy. There was no operative mortality or morbidity. Among the 13 patients with benign GISTs, 1 died of causes unrelated to the disease, while 12 patients are still alive and in good health after a mean follow-up of 148.5 months (range: 6-262). Among patients with malignant muscular GISTs (6 gastric and 3 ileal), 3 with gastric tumours were lost to follow-up, 3 with gastric and 1 with ileal neoplasms are alive and free from disease after a median follow-up of 58 months (range 3-108), while 2 patients with ileal neoplasms died of metastatic disease 39 and 29 months after the surgical procedure. Among 6 patients with malignant neural GISTs (2 gastric, 2 jejunal and 2 ileal) 1 with a gastric tumour and 1 with a jejunal tumour were lost to follow-up, while 3 are still alive and in good health after a median follow-up of 67.6 months (range 19 to 94); another with jejunal disease developed liver metastases 14 months after small bowel resection and died 12 months later. The two patients with undifferentiated GIST both died of liver metastasis 38 months after pancreatico-duodenectomy and 43 months after total gastrectomy. The most frequent symptoms were abdominal pain and a palpable mass, but no specific signs were detected. In gastroduodenal lesions endoscopy plays a very important diagnostic role and CT scan is the most sensitive diagnostic technique in the evaluation of location, size, invasion of adjacent organs and metastases. Prognostic prediction on the basis of histological findings is difficult and in our experience undifferentiated tumours are always malignant.

Aged↗

[Stromal tumor of the ileum (GIST) at the same time as a renal carcinoma. Description of a case and review of the literature].

The gastrointestinal mesenchymal tumors from a heterogenous group that include several entities: leiomyomas, schwanomas and less differentiated tumors often referred as GIST. These neoplasm are uncommon and their clinical behaviour is most difficult to predict. We describe a malignant gastrointestinal stromal tumor of the ileum coexisting with renal cell carcinoma. The neoplasms were fixed in formaldehyde, embedded in paraffin and stained with hematoxylin-eosin. For immunohistochemical studies deparaffinized tissue sections were incubated with antibodies against vimentin, desmin, muscle specific actin, S100, CD34, GFAP, NSE and keratin. The epithelioid and spindle cells of ileal neoplasm were arranged in interlacing fascicle with occasional palisading and were positive for vimentin and CD34. Positivity for muscle specific actin was focally found. The renal neoplasm required differential diagnosis from metastatic GIST. The morphological and immunohistochemical investigations in our case were consistent with GIST coexisting with primitive renal cell carcinoma. One of the problems connected to the anatomo-clinical evaluation of GIST consist in the difficulty of making a prognosis. An almost complete review of the literature and view point on the topic has been performed. As a conclusion judging from papers regarding this argument, no clear parameters of biological behaviour exist excluding mitotic index.

Adenocarcinoma, Clear Cell↗

Care of patients and their families with familial adenomatous polyposis.

Familial adenomatous polyposis (FAP) is a dominantly inherited disorder that is typically characterized by the appearance of multiple colorectal adenomas usually by the teenage years, with a risk of early colorectal cancer approaching 100%. Genetic testing can help determine which family members have the disorder and require surveillance endoscopy. Astute physicians may detect unsuspected FAP in patients with extraintestinal manifestations such as hard or soft cutaneous tumors. Colectomy will prevent cancer but is often necessary before the patient is 20 years old. Postoperative lifelong surveillance is indicated to screen for associated duodenal, thyroid, and rectal or ileal neoplasms. Attenuated FAP variants are less typical and may be confused with other types of familial colorectal neoplasia. Chemoprevention, regression, and other treatment strategies are being developed to improve the management of extracolonic neoplasms and desmoid tumors. A better understanding of the natural history of these FAP-associated phenomena will facilitate the rational selection of interventions. Management guidelines that were recently developed at Mayo Clinic Rochester to provide for uniform care and surveillance are discussed.

Adenomatous Polyposis Coli↗

Neuroendocrine tumors of the gastrointestinal tract.

Neoplastic proliferations of neuroendocrine cells (NE) may occur throughout the entire GI tract but affect particularly appendix and ileum ("midgut carcinoids"), rectum ("hindgut carcinoids"), as well as stomach and the duodenum ("foregut carcinoids"). Only more exceptionally, they arise in the esophagus, jejunum and colon. The NE tumors encompass a heterogeneous gross and microscopic structural spectrum, ranging from inconspicuous microproliferations ("mucous membrane nevi") to bulky tumor masses. Their growth patterns are usually characteristic and easily recognized. In doubtful cases their NE differentiation becomes established by a characteristic silver affinity, by the ultrastructurally observed presence of characteristic "endocrine" secretion granules, and by immunohistochemically detectable occurrence of "pan-NE markers" (neuron-specific enolase, chromogranins, and synaptophysin), biogenic amines (mainly serotonin), and neurohormonal peptides. Foregut carcinoids usually contain serotonin, gastrin, and somatostatin, midgut carcinoids often only serotonin and tachykinins, whereas the hindgut carcinoids as a rule are multihormonal with a wide spectrum of hormonal peptides, including even insulin. Most GI NE tumors are found in the appendix (50%) and the ileum (30%). Practically all (98%) of the appendiceal NE tumors are benign. They have recently been proposed as arising from apparently Schwann-cell-related NE cells in the submucosa, whereas the ileal--and probably also all the other non-appendiceal NE tumors--are derived from the totipotential cells in epithelial crypts of the mucosa. Among the ileal NE neoplasms a large number can metastasize and result in a fatal outcome. The ability to metastasize is related to the size and to the multiplicity of the primary tumors at the time of initial diagnosis and, to some extent, to their histopathologic growth pattern. Now, some relationship between the prognosis and the cytochemically assessed nuclear DNA content of the NE tumor cells has also been established; not less than about 1/4 to 1/3 seem to be aneuploid. Almost 90% of the rectal carcinoids are benign. Exceptionally, a highly malignant NE neoplasms can arise from the colon/rectum--as well as from the esophagus--composed of NE cells of small and intermediate size. The NE tumors of the stomach are often composed of ECL (enterochromaffin-cell-like) cells; such ECL cell carcinoids are related to atrophic gastritis with pernicious anemia; experimentally, they can be induced by hypergastrinemia in rats. Duodenal carcinoids often contain psammoma bodies and can be associated with neurofibromatosis.

Appendiceal Neoplasms↗

Gastrointestinal stromal tumor with skeinoid fibers of the ileum.

Gastrointestinal stromal tumor (GIST) is not uncommon among gastrointestinal nonepithelial tumors, but there are few reports describing the cytologic findings. We report a case of GIST with skeinoid fibers in scrape cytology preparation. The patient was a 53-year-old man with a tumor in the small intestine. Scrape preparations from the cut surface of the resected tumor revealed cellular material composed of spindle cells showing loose clusters or single cells. The nuclei were spindled, elongated or cigar-shaped, and relatively uniform. The cytoplasm was fragile and demonstrated a finely fibrillar material. Dense hyaline materials with irregular outline were observed within the loose clusters composed of the tumor cells. The hyaline materials were also observed in the background. Histologic preparation showed spindle cells arranged in a fascicular or storiform pattern. Most eosinophilic globules were distributed between the tumor cells. The globules were positive in periodic acid-Schiff reaction, and were stained blue with Masson's trichrome stain. Immunohistochemically, the tumor cells were strongly and diffusely positive for c-kit, focally and weakly positive for alpha-smooth muscle actin, and negative for CD34 and S-100 protein. We emphasize that skeinoid fibers are characteristic of GIST arising in the small intestine, and their presence predicts a good prognosis, even in malignant GIST.

Gastrointestinal Neoplasms↗

Lectin expression in carcinoid tumours of the gastrointestinal tract.

The binding of peroxidase-conjugated Dolichos biflorus (DBA), Triticum vulgaris (WGA), Canavalia ensiformis (Con A), Arachis hypogaea (PNA), Lotus tetragonolobus, and Bandeiraea simplicifolia I (BSAI) to gastrointestinal carcinoid tumours was studied. The results indicate that carcinoid tumour cells express certain carbohydrates similar to those present in the adjacent surface epithelium. The differences in the lectin-binding properties of carcinoid tumours of different sites of the gastrointestinal tract are closely related to the regional differences in the lectin binding of adjacent surface epithelium. These observations therefore form a useful basis for further studies in the application of lectin histochemistry to elucidate the histogenesis of carcinoid tumours.

Appendiceal Neoplasms↗

Non-Hodgkin's lymphomas presenting with gastrointestinal involvement.

Between 1978 and 1983 a total of 33 patients with non-Hodgkin's lymphoma (NHL) involving the gastrointestinal tract were seen in our institution. Pathological classification was performed according to Kiel. Low grade NHL was diagnosed in 17, high grade NHL in 16 patients. The most frequent histological entity was lymphoplasmocytoid immunocytoma (11 patients). The most common sites of origin were the stomach (23 patients) and the ileocecal region (6 patients). The majority of patients presented with stage I and II disease (20 of 33 patients). As a rule primary therapy consisted of surgery with curative intent. Most of the patients received additional chemotherapy or radiotherapy. Patients with limited disease and complete tumour resection showed long-term survival from 12+ to 57+ months (mean 32.9+ months). Patients with advanced disease (stage III and IV) and only palliative surgery or with lymphoblastic lymphoma had a probability of survival of less than 12 months.

Adolescent↗

Diffuse ganglioneuromatosis with plexiform neurofibromas limited to the gastrointestinal tract involving a large segment of small intestine.

A rare case of diffuse ganglioneuromatosis with plexiform neurofibromas limited to the gastrointestinal tract in a 20-year-old man is reported. The patient had no cafe-au-lait spots or any apparent tumorous lesions affecting other organs. A fan-shaped resection of the mesentery, including 200cm of the ileum, together with ileocecal resection was done, and the diagnosis was histologically confirmed. The relationship between this benign tumor and other neurogenic disorders is discussed, along with a review of the literature. Long-term follow up of this patient is required because of the possible development of von Recklinghausen's disease or multiple endocrine neoplasia type IIb.

Adult↗

A case of primary amyloidosis confined to the small intestine.

A 60 year-old man with primary amyloidosis confined to the small intestine was reported. Multiple polyps of the small intestine were found by an upper GI series and enteroscopic polypectomy revealed massive deposition of amyloid in the lamina propria and the submucosa. No predisposing disorder or other sites of deposition were found, and the diagnosis of primary amyloidosis of the small intestine was confirmed.

Amyloidosis↗

Superficial spreading adenocarcinoma of appendix, cecum, and terminal ileum.

A case of superficial spreading adenocarcinoma involving terminal ileum, appendix and cecum is presented. The tumor might primarily arise from the proximal part of the appendix with extension to the cecum and terminal ileum, although multicentric origin of the tumor cannot be excluded. The mechanism underlying superficial infiltration of the tumor is through close contact and intramucosal lymphatic spread.

Adenocarcinoma↗

Gastrointestinal autonomic nerve tumors (plexosarcomas). is A radiological diagnosis possible?

The gastrointestinal autonomic nerve (GAN) tumor, or plexosarcoma, is a very uncommon stromal tumor of the gastrointestinal tract and retroperitoneum. Distinction of GAN tumors from other stromal tumors is not possible based on imaging studies, and needs specific ultrastructural and inmunohistochemical techniques. There are some morphologic characteristics that the radiologist should know, so as to include these rare tumors in the differential diagnosis of an abdominal mass. We report two new cases: small bowel (jejunum-ileum) and stomach GAN tumors.

Aged↗