HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Darvishian, F. Articles by Basham, K. Search for Related Content PubMed PubMed Citation Articles by Darvishian, F. Articles by Basham, K.

Familial Visceral Myopathy with Carcinoma of Unknown Primary

Address correspondence to Farbod Darvishian, M.D., Department of Pathology, North Shore University Hospital, 300 Community Drive, Manhasset, New York 11030, USA; tel 516 562 4180; fax 516 562 4591; e-mail: farbodarv{at}cs.com.

Abstract

We report an autopsy case of a 35-yr-old man with familial visceral myopathy, a cause of primary intestinal pseudo-obstruction. The patient died from complications of familial visceral myopathy, sepsis, and generalized signet-ring cell carcinomatosis. The patient had massive distension of the large and small intestines, a dilated thickened esophagus, and fibroneoplastic adhesions between intra-abdominal and thoracic structures. This case provides an observation, not previously described in cases of familial visceral myopathy, which is fibrosis and atrophy of the outer longitudinal smooth muscle of the small bowel, alternating to involve only the inner smooth muscle layer of the large bowel. This case shows how a malignant neoplasm can compound the pathology of familial visceral myopathy.

Keywords: familial visceral myopathy, signet ring cell carcinoma, neoplasia