This Article Order Full text via Infotrieve 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 HighWire Citing Articles via Google Scholar Google Scholar Articles by Quade, B. J. Articles by Morton, C. C. Search for Related Content PubMed PubMed Citation Articles by Quade, B. J. Articles by Morton, C. C.

American Journal of Pathology, Vol 150, 2153-2166, Copyright © 1997 by American Society for Investigative Pathology

REGULAR ARTICLES

Disseminated peritoneal leiomyomatosis. Clonality analysis by X chromosome inactivation and cytogenetics of a clinically benign smooth muscle proliferation

BJ Quade, CM McLachlin, V Soto-Wright, J Zuckerman, GL Mutter and CC Morton
Department of Pathology, Brigham and Women's Hospital, Boston, Massachusetts 02115, USA.

Disseminated peritoneal leiomyomatosis (DPL, leiomyomatosis peritonealis disseminata) is a rare condition in which multiple histologically benign smooth muscle tumorlets diffusely stud peritoneal and omental surfaces in females, predominantly of reproductive age. Although the distribution of these lesions suggests a metastatic process, DPL generally has a benign clinical course and has been regarded as a metaplastic process. We assessed clonality of 42 tumorlets and 15 normal tissues from four females with DPL by analyzing X chromosome inactivation as indicated by the methylation status of the androgen receptor gene (HUMARA). In each of the four patients, the same parental X chromosome was nonrandomly inactivated in all tumorlets, consistent with a metastatic unicentric neoplasm, or alternatively, selection for an X-linked allele in clonal multicentric lesions. Anomalous demethylation of the marker for X inactivation (HUMARA) was associated with loss of heterozygosity for markers spanning the X chromosome, or monosomy X, in part of one leiomyomatous lesion. Biallelic demethylation of the HUMARA microsatellite polymorphism was also found in one intramural leiomyoma. Two of six DPL lesions karyotyped had cytogenetic abnormalities involving chromosomes 7, 12, and 18, suggesting a pathogenesis in common with uterine leiomyomas.

This article has been cited by other articles:

				A. Astrinidis and E. Petri Henske Aberrant Cellular Differentiation and Migration in Renal and Pulmonary Tuberous Sclerosis Complex J Child Neurol, September 1, 2004; 19(9): 710 - 715. [Abstract] [PDF]

				M. Karbowniczek, A. Astrinidis, B. R. Balsara, J. R. Testa, J. H. Lium, T. V. Colby, F. X. McCormack, and E. P. Henske Recurrent Lymphangiomyomatosis after Transplantation: Genetic Analyses Reveal a Metastatic Mechanism Am. J. Respir. Crit. Care Med., April 1, 2003; 167(7): 976 - 982. [Abstract] [Full Text] [PDF]

				J. YU, A. ASTRINIDIS, and E. P. HENSKE Chromosome 16 Loss of Heterozygosity in Tuberous Sclerosis and Sporadic Lymphangiomyomatosis Am. J. Respir. Crit. Care Med., October 15, 2001; 164(8): 1537 - 1540. [Abstract] [Full Text] [PDF]

				S. J. Diaz-Cano, M. de Miguel, A. Blanes, R. Tashjian, and H. J. Wolfe Germline RET 634 Mutation Positive MEN 2A-related C-Cell Hyperplasias Have Genetic Features Consistent with Intraepithelial Neoplasia J. Clin. Endocrinol. Metab., August 1, 2001; 86(8): 3948 - 3957. [Abstract] [Full Text] [PDF]

				D. Danikas, V. T. Goudas, Ch. V. Rao, and D. K. Brief LUTEINIZING HORMONE RECEPTOR EXPRESSION IN LEIOMYOMATOSIS PERITONEALIS DISSEMINATA Obstet. Gynecol., June 1, 2000; 95(6): 1009 - 1011. [Abstract] [Full Text] [PDF]

				T. Carsillo, A. Astrinidis, and E. P. Henske Mutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis PNAS, May 23, 2000; 97(11): 6085 - 6090. [Abstract] [Full Text] [PDF]