Ileal carcinoid tumor complicating carcinoid heart disease and secondary retroperitoneal fibrosis

A 70‐year‐old man with metastatic liver tumors showed carcinoid syndrome with clinical symptoms of facial flushing, palpitation, dyspnea, and an itching sensation. Regurgitation of the tricuspid and aortic valves was observed by echocardiography. An elevated serum level of serotonin and a high urine...

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Veröffentlicht in:Pathology international 2000-05, Vol.50 (5), p.404-411
Hauptverfasser: Sakai, Daisuke, Murakami, Masato, Kawazoe, Kazuya, Tsutsumi, Yutaka
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Murakami, Masato
Kawazoe, Kazuya
Tsutsumi, Yutaka
description A 70‐year‐old man with metastatic liver tumors showed carcinoid syndrome with clinical symptoms of facial flushing, palpitation, dyspnea, and an itching sensation. Regurgitation of the tricuspid and aortic valves was observed by echocardiography. An elevated serum level of serotonin and a high urine excretion of serotonin metabolites were confirmed. Autopsy confirmed a serotonin‐immunoreactive 1 cm ileal carcinoid tumor with metastasis to the liver, bone and peritoneum. The tumor cells were argyrophilic but not argentaffin, and showed erythrophagocytosis in the primary lesion. All the four heart valves and bilateral atrial endocardium showed fibromyxoid thickening, indicating the association of carcinoid heart disease. Desmoplastic reaction with deposition of sulfated acid mucopolysaccharides was also observed in the retroperitoneal space (secondary retroperitoneal fibrosis). Stenosis of the bilateral ureters and inferior mesenteric artery provoked hydronephrosis and lethal ischemic necrosis of the left‐sided colon, respectively. Rarity of functioning ileal carcinoid tumor and pathogenesis of systemic fibroplasia are discussed.
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Regurgitation of the tricuspid and aortic valves was observed by echocardiography. An elevated serum level of serotonin and a high urine excretion of serotonin metabolites were confirmed. Autopsy confirmed a serotonin‐immunoreactive 1 cm ileal carcinoid tumor with metastasis to the liver, bone and peritoneum. The tumor cells were argyrophilic but not argentaffin, and showed erythrophagocytosis in the primary lesion. All the four heart valves and bilateral atrial endocardium showed fibromyxoid thickening, indicating the association of carcinoid heart disease. Desmoplastic reaction with deposition of sulfated acid mucopolysaccharides was also observed in the retroperitoneal space (secondary retroperitoneal fibrosis). Stenosis of the bilateral ureters and inferior mesenteric artery provoked hydronephrosis and lethal ischemic necrosis of the left‐sided colon, respectively. 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Regurgitation of the tricuspid and aortic valves was observed by echocardiography. An elevated serum level of serotonin and a high urine excretion of serotonin metabolites were confirmed. Autopsy confirmed a serotonin‐immunoreactive 1 cm ileal carcinoid tumor with metastasis to the liver, bone and peritoneum. The tumor cells were argyrophilic but not argentaffin, and showed erythrophagocytosis in the primary lesion. All the four heart valves and bilateral atrial endocardium showed fibromyxoid thickening, indicating the association of carcinoid heart disease. Desmoplastic reaction with deposition of sulfated acid mucopolysaccharides was also observed in the retroperitoneal space (secondary retroperitoneal fibrosis). Stenosis of the bilateral ureters and inferior mesenteric artery provoked hydronephrosis and lethal ischemic necrosis of the left‐sided colon, respectively. Rarity of functioning ileal carcinoid tumor and pathogenesis of systemic fibroplasia are discussed.</abstract><cop>Melbourne, Australia</cop><pub>Blackwell Science Pty</pub><pmid>10849330</pmid><doi>10.1046/j.1440-1827.2000.01053.x</doi><tpages>8</tpages><oa>free_for_read</oa></addata></record>
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subjects Aged
Bone Neoplasms - metabolism
Bone Neoplasms - secondary
carcinoid heart
Carcinoid Heart Disease - etiology
Carcinoid Heart Disease - pathology
carcinoid syndrome
Carcinoid Tumor - complications
Carcinoid Tumor - metabolism
Carcinoid Tumor - secondary
erythrophagocytosis
Humans
ileal carcinoid
Ileal Neoplasms - complications
Ileal Neoplasms - metabolism
Ileal Neoplasms - pathology
Immunoenzyme Techniques
Liver Neoplasms - metabolism
Liver Neoplasms - secondary
Male
Peritoneal Neoplasms - metabolism
Peritoneal Neoplasms - secondary
Retroperitoneal Fibrosis - etiology
Retroperitoneal Fibrosis - pathology
secondary retroperitoneal fibrosis
serotonin
Serotonin - metabolism
title Ileal carcinoid tumor complicating carcinoid heart disease and secondary retroperitoneal fibrosis
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