Familial clustering of Leiomyomatosis peritonealis disseminata: an unknown genetic syndrome?

Abstract

Background: Leiomyomatosis peritonealis disseminata (LPD) is defined as the occurrence of multiple tumorous intraabdominal lesions, which are myomatous nodules. LPD is a rare disease with only about 100 cases reported. The usual course of LPD is benign with the majority of the patients being premenopausal females. Only two cases involving men have been reported, no syndrome or familial occurrence of LPD has been described.

A Rare Case of Parasitic Myoma: Possible Etiologies and Review of the Literature

“…[5][6][7] Third proposed theory is based on genetic analysis that emphasizes the importance of somatic mutations particularly affecting MED 12, a subunit of mediator complex regulates transcription initiation and elongation in gene promoters to the RNA polymerase II initiation complex. 8,9 All these theories are suggested as possible etiologies for spontaneous parasitic myomas. In our case, the parasitic myoma may be explained by the first theory proposed by Kelly and Cullen, because the patient had no history of uterine surgery.…”

“…Cullen, most of these cases are reported as a consequence of laparoscopic morcellation procedures. [4][5][6][7][8][9][10] In this study we aimed to present a very rare case of spontaneous parasitic myoma in a patient without history of uterine surgery and examine the proposed etiologies together with possible prevention methods.…”

Leiomyomatosis Peritonealis Disseminata: A Case Report

“…Literatures reported few case of LPD presented during pregnancy. One case report shows that LPD presented in postmenopausal period [4,5].…”

Leiomiomatosis Peritoneal Diseminada

“…Estos tumores expresan receptores hormonales, desarrollándose generalmente en pacientes en edad reproductiva, siendo su aparición independiente en muchos casos de la condición de leiomiomatosis uterina previa (Bekkers et al, 1999;Halama et al, 2005). Suele ser un hallazgo radiológico o quirúrgico, ya que la mayoría de los pacientes son asintomáticos.…”