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Leiomyomatosis peritonealis disseminata in association with Currarino syndrome?

Nappi C, Di Spiezio Sardo A, Mandato VD, Bifulco G, Merello E, Savanelli A, Mignogna C, Capra V, Guida M

Department of Gynecology and Obstetrics, and Pathophysiology of Human Reproduction, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy. nappi@unina.it

BACKGROUND: Leiomyomatosis peritonealis disseminata (LPD) is a rare disease in which multiple smooth muscle or smooth muscle-like nodules develop subperitoneally in any part of the abdominal cavity. No reports of multiple congenital malformations associated with LPD have been found in the English literature. CASE PRESENTATION: A 27 year-old patient referred to our gynaecology unit for pelvic pain, amenorrhoea, stress incontinence, chronic constipation and recurrent intestinal and urinary infections. Multiple congenital malformations had previously been diagnosed. Most of these had required surgical treatment in her early life: anorectal malformation with rectovestibular fistula, ectopic right ureteral orifice, megadolichoureter and hemisacrum. An ultrasound scan and computed tomography performed in our department showed an irregular, polylobate, complex 20 cm mass originating from the right pelvis that reached the right hypochondrium and the epigastrium. The patient underwent laparotomy. The three largest abdominal-pelvic masses and multiple independent nodules within the peritoneum were progressively removed. The histological diagnosis was of LPD. CONCLUSION: The case we report is distinctive in that a rare acquired disease, LPD, coexists with multiple congenital malformations recalling a particular subgroup of caudal regression syndrome: the Currarino syndrome.

Published 22 June 2006 in BMC Cancer, 6: 127.
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