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Splenic metastases from mucinous neoplasms of the appendix and colon.

AIMS AND BACKGROUND: Splenic metastases associated with mucinous intraabdominal tumors have been an enigma in the radiologic and oncology literature. These focal parenchymal defects from a non-metastasizing malignancy within an organ that rarely develops metastatic foci, even with high-grade cancer, were studied. METHODS: Information on 9 patients who underwent splenectomy with intraparenchymal splenic masses associated with appendiceal or colorectal mucinous tumors with peritoneal dissemination was collected. The CT scan, the histopathology and the clinical parameters of these patients were studied. A literature review searching for prior reports of this subject was performed. RESULTS: Eight of these patients had mucinous appendiceal tumors and 1 a mucinous sigmoid colon cancer. All patients had mucinous carcinomatosis at some time in their clinical course. These splenic tumor masses had a CT image compatible with metastases and not compatible with mucinous tumor layered out of the splenic capsule. None of the patients had evidence of metastases to other sites such as liver or lymph nodes. All patients had a mucinous histopathology. Splenectomy may be associated with prolonged survival. CONCLUSIONS: From our review of the clinical information available on these 9 patients, these splenic lesions were thought to be an entrapment of mucinous tumor within splenic surface trabeculae, which expand into the splenic parenchyma resembling metastatic disease. These CT findings may be more accurately referred to as splenic pseudometastases.

Adenocarcinoma, Mucinous↗

Cytogenetic analysis of several pseudomyxoma peritonei lesions originating from a mucinous cystadenoma of the appendix.

Epithelial proliferative lesions of the appendix are rare and have never been studied cytogenetically. We present the chromosomal banding analysis of four successfully short-term cultured samples from pseudomyxoma peritonei lesions originating from a cystadenoma of the appendix. All four sample contained clonal chromosome abnormalities. In three of them, the clone 46,XX,der(6)?del(6)(q16q21)?del(6)(q27) was found, whereas a clone with the karyotype 46,XX,t(2;17)(p21;p13),t(6;12)(p21;q13),t(12;15)(q24;q15) was detected in the fourth sample. Our findings support the view that pseudomyxoma peritonei originates by spreading from a primary mucinous neoplasm of an intraperitoneal organ rather than through mucinous metaplasia or multifocal primary neoplastic transformation of the peritoneum.

Adult↗

[An appendicular villous adenoma].

Villous adenomas of the vermiform appendix are uncommon. Their malignant potential is unknown and their treatment is controversial. Because of a reported association between adenomas of the appendix and other gastrointestinal neoplasms, long-term surveillance is recommended. A new case of appendicular villous adenoma is reported.

Adenoma↗

Pseudomyxoma peritonei et ovarii associated with sequential ovarian and appendicular tumors and acromegaly.

Pseudomyxoma peritonei associated with highly differentiated mucinous, intestinal-type ovarian tumor is reported in a 46-year-old acromegalic patient. Five years after its discovery a second operation revealed a mucinous cystadenoma of the appendix. The coexistence of pseudomyxoma peritonei with ovarian and appendicular tumors is rare, raising questions about the primary was origin of the peritoneal tumor. In this case the primary was ovarian while the appendicular tumor occurred later. Since acromegaly is associated with a high risk for the development of colorectal tumors, it could also have elicited a similar response in the intestinal-type ovarian tumor and the appendicular neoplasm found in this patient. The role of appendectomy in the surgical treatment and staging of ovarian tumors is stressed.

Acromegaly↗

Composite carcinoma-carcinoid tumors of the gastrointestinal tract. A morphologic, histochemical, and immunocytochemical study.

A morphologic, histochemical, and immunocytochemical study of 20 cases of pure gastrointestinal carcinoids, adenocarcinomas, and mixed neoplasms composed of both elements, so-called composite carcinoma-carcinoid tumors (CCC), was undertaken in order to correlate the morphologic patterns with the immunocytochemical localization of carcinoembryonic antigen (CEA), serotonin, and a battery of polypeptide hormones (calcitonin, glucagon, insulin, gastrin, somatostatin, and adrenocorticotropin [ACTH]). Paraffin sections from five pure carcinoids, seven pure adenocarcinomas, and eight CCC from the stomach, small bowel, appendix, and colon were studied with mucicarmine, silver impregnation stains, and a peroxidase-anti-peroxidase technic. Of the eight CCC, all were mucin positive, four were argyrophilic, and three were argentaffin positive. CEA was present in all eight, serotonin in seven, and calcitonin in one. No other neurohormonal peptides were demonstrated. The distribution of serotonin and CEA generally corresponded to the morphologic pattern, but discordance was observed in two cases, i.e., serotonin was not always localized to areas of carcinoid and CEA not always confined to areas of carcinoma. All five pure carcinoids demonstrated intracytoplasmic localization of serotonin, whereas none contained intracytoplasmic CEA. In two cases, CEA was present within acinar lumens only. The seven colonic adenocarcinomas were argyrophil and argentaffin negative. All contained CEA within the cytoplasm and in gland lumens. None contained serotonin. None of the neurohormonal peptides was localized in either pure adenocarcinomas or carcinoids. This study reveals that among gastrointestinal neoplasms displaying morphologic patterns of adenocarcinoma and carcinoid, immunocytochemical localization of CEA and serotonin confirms their bidirectional differentiation and justifies the designation "composite carcinoma-carcinoid."

Appendiceal Neoplasms↗

Carcinoid disease.

We reviewed 28 cases of carcinoid tumor arising in the small intestine (11 cases), appendix (eight), rectum (three), and lung (six), which were diagnosed during a ten-year period ending in 1977. Components of the carcinoid syndrome occurred in only three patients, all of whom had hepatic metastases from tumors of the small bowel. Most of the small intestinal lesions, which usually were multicentric, metastasized. When metastases were absent or confined to regional lymph nodes, surgical excision alone provided satisfactory long-term therapy. When the disease involved the liver, the course until death was usually long but inexorable unless chemotherapy was used. During follow-up, which averaged more than six years, no carcinoid of the appendix, rectum, or lung recurred or metastasized after surgical extirpation. Other neoplasms, including adenocarcinomas of the rectum, appendix, prostate, and nasopharynx and acute granulocytic leukemia, appeared in six patients, which extends previous observations that patients with carcinoids have a neoplastic diathesis.

Adult↗

Metastasis from small cell carcinoma of the lung producing acute appendicitis.

A case of acute gangrenous appendicitis with perforation caused by metastatic small cell carcinoma of the lung in a 65 year old man is reported. The manifestation of appendicitis occurred more than 4 years after the diagnosis of the bronchogenic carcinoma. With longer survival of patients with disseminated tumors it is probable that new manifestations of those malignancies will be discovered. Acute appendicitis due to metastasis from a distant neoplasm should be considered in the differential diagnosis of right lower abdominal pain in the oncology patient.

Abdomen, Acute↗

Epidemiology, tumour biology and histopathological classification of neuroendocrine tumours of the gastrointestinal tract.

The diffuse neuroendocrine cell gives rise to a heterogeneous population of tumours which differ in their morphological and functional features. The term 'carcinoid', although well established in medical terminology, is therefore no longer adequate to cover the entire spectrum of neuroendocrine neoplasms. Here we use the term neuroendocrine tumours (NET), which was suggested in the WHO classification of 2000, and review the most important NET entities that are currently recognised in the gastrointestinal tract, highlighting their distinguishing features.

Appendiceal Neoplasms↗

Mucinous cystadenoma of the appendix.

Mucinous neoplasms of the Appendix are rare conditions, usually diagnosed intraoperatively or postoperatively on the pathology report. They have an association with colonic and ovarian neoplasms, and spillage can result in pseudomyxoma peritonei. While appendicectomy is adequate treatment for cystadenomas, cystadenocarcinomas require a right hemicolectomy. Open approach is recommended for the surgical treatment of these lesions.

Aged↗

Mucinous cystadenoma and cystadenocarcinoma of the vermiform appendix with particular reference to mucocele and pseudomyxoma peritonei.

Ten cases of mucocele of the vermiform appendix are described. Eight cases were of mucinous cystadenoma of the appendix and six cases showed acute inflammation. Two of the six cases showed pseudoinvasion of the appendix and in a further case the appendix had perforated with extrusion of a misplaced neoplasm. Two cases were of mucinous cystadenocarcinoma and one of these was diagnosed as ;pseudomyxoma peritonei'. ;Pseudomyxoma peritonei' is a misnomer and is caused by dissemination of a mucinous cystadenocarcinoma within the peritoneal cavity. The special problems of histological diagnosis are discussed.

Aged↗

Pseudomyxoma peritonei in women: a clinicopathologic analysis of 30 cases with emphasis on site of origin, prognosis, and relationship to ovarian mucinous tumors of low malignant potential.

Pseudomyxoma peritonei (PMP) is a poorly understood condition characterized by the accumulation of abundant mucinous material within the peritoneal cavity and associated with a mucinous tumor of the gastrointestinal tract or ovaries. Recently there has been considerable debate over the primary site of origin of the tumor associated with PMP in women. Some investigators have proposed a primary site in the ovaries, whereas others favor the gastrointestinal tract or the peritoneum. Another confusing issue has been the nature of the ovarian mucinous tumors associated with PMP. Although these neoplasms may be frankly malignant, more often they show minimal cytologic atypia and epithelial proliferation and have been classified as borderline or low malignant potential tumors. In order to address the issues of site of origin and nature of the associated ovarian mucinous tumors, we studied 68 cases of PMP in women, 30 of whom had mucinous tumors involving the ovaries. All 30 of these cases had an associated mucinous appendiceal or intestinal tumor. The PMP cases with ovarian tumors were compared with 30 ovarian mucinous tumors of low malignant potential (LMP). Based on the analysis of the primary ovarian mucinous LMP tumors, a set of criteria was formulated and used to determine the probable site of origin of PMP in the 30 women with mucinous tumors involving the ovaries. The following gross and microscopic features of the ovarian tumor were considered to be inconsistent with a primary ovarian origin: (1) surface involvement with or without superficial stromal involvement only; (2) adenocarcinoma with signet ring cell differentiation, with a previously diagnosed or concurrent appendiceal tumor of similar morphology; (3) bilateral adenocarcinoma consistent with colonic or appendiceal morphology; and (4) unilateral adenocarcinoma consistent with colonic or appendiceal morphology with a history of a colonic or appendiceal adenocarcinoma. When any one of these features was present the ovarian tumor was diagnosed as secondary. The following additional features also were considered to be more typical of secondary ovarian involvement: (1) normal or only slightly enlarged ovaries; (2) bilateral ovarian involvement; (3) simple or only focally proliferative mucinous epithelium with abundant extracellular mucin in cases with predominantly surface involvement of the ovaries, with or without a history of/or concurrent appendiceal adenoma; (4) multifocal or extensive pseudomyxoma ovarii in cases with stromal involvement, with or without a history of/or concurrent appendiceal adenoma; (5) ruptured appendiceal adenoma and unruptured ovarian tumor of similar histology; and (6) presence of an associated mucinous intestinal tumor.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗

Primary adenocarcinoma of the appendix invading the urinary bladder.

We report a case of adenocarcinoma of the appendix invading the urinary bladder in a 75-year-old man. Although cystoscopic examination and computed tomography suggested a primary or secondary bladder tumor, repeated transurethral bladder biopsy could not confirm the neoplasm. At operation a primary neoplasm of the appendix invading the bladder was discovered and en bloc resection of the urinary bladder with the adherent cecum followed by an ileocolonic anastomosis and ureterocutaneostomy was performed. The patient died of carcinoma 13 months later.

Adenocarcinoma↗

Pseudomyxoma peritonei et ovarii with an occult neoplasm of appendix: case report.

Although pseudomyxoma peritonei is comparatively rare, the simultaneous occurrence in this condition of mucinous tumours of ovary and appendix is common and has been termed pseudomyxoma peritonei ovarii et appendicis. Four closely followed patients in a small series showed only 25% 6-year survival. This paper presents a further case with a 14-month follow-up.

Appendiceal Neoplasms↗

Synchronous serrated adenoma of the appendix and high-grade ovarian carcinoma: a case demonstrating different origin of the two neoplasms.

Association of mucinous adenomas of the appendix and mucinous ovarian tumors is well known. The origin of the ovarian tumor (metastasis from the appendix vs independent primary) is still debated. Serrated adenoma is a rare neoplasm of the distal gastrointestinal tract, and its precancerous role in the colorectum was recently postulated. A 74-year-old patient was subjected to hysterectomy with routine appendectomy due to a 17-cm tumor of her right ovary. Histological examination revealed a high-grade ovarian adenocarcinoma with peritoneal involvement. The appendix, grossly unremarkable, harbored a serrated adenoma with no evidence of invasion or malignant transformation. Immunohistochemical examination revealed CD7+, CK20-phenotype of the ovarian and reverse (CK7-, CK20+) phenotype of the appendiceal tumor. Microsatellite analysis demonstrated microsatellite instability (MSI-high) within the serrated adenoma (4/5 markers with positive amplification) and no MSI (0/6 amplified markers) in the samples from the ovarian carcinoma, its metastases and the uninvolved uterine cervix. There were also differences in LOH pattern between the ovarian adenocarcinoma and the serrated adenoma. The findings suggest two independent primaries with profound differences in tumorigenetic pathways of both lesions. To the best of our knowledge this is the first report of synchronous serrated adenoma of the appendix and ovarian carcinoma.

Adenocarcinoma↗

Primary adenocarcinoma of the vermiform appendix: a case report.

Primary adenocarcinoma of the appendix is rare, and since Berger first recognised the neoplasm in 1882, fewer than 250 cases have been recorded. Adenocarcinoma of the appendix is never suspected pre-operatively, being usually first discovered by histological examination. Ileocaecal resection during the first operation and right hemicolectomy for a carcinoma diagnosed after appendicectomy remain the main stay of treatment.

Adenocarcinoma↗

Strumal carcinoid of the ovary.

A 45-year-old woman presented with an apparently nonfunctioning strumal carcinoid of the ovary. On ultrastructural examination the cells in the solid parts of the tumor as well as in the "follicular" areas contained dense-core secretory granules, indicating that this neoplasm is a pure carcinoid tumor. The thyroid-like appearance in areas is due to the accumulation of secretory material within unusually dilated acinar spaces. This study raises the question whether any strumal carcinoid contains thyroid tissue since the cells forming the follicle-like spaces in this tumor contain dense-core granules.

Appendiceal Neoplasms↗

Carcinoma in situ of the vermiform appendix associated with adenomatosis of the colon.

A case is reported of carcinoma in situ of the vermiform appendix associated with adenomatosis of the colon. Histologic examination revealed the presence of tubulovillous adenocarcinoma in an adenoma. Other lesions associated with this neoplasm were multiple adenomatous polyps in the colon and duodenum and two fibromas in the neck. Since the vermiform appendix is a part of the large intestine, it should be expected that careful examination may reveal the adenomatous involvement and may lead to the discovery of carcinoma in situ.

Adenocarcinoma↗