Case Report: Congenital Perineal Lipoma Associated With Additional External Genitalia Anomalies

Abstract

Perineal lipoma is an uncommon congenital benign tumor sometimes associated with genitourinary or anorectal malformations. Accessory scrotum and accessory labioscrotal fold are infrequent features, often concurrent with perineal tumors. We describe a single institution experience with three consecutive cases of perineal lipoma associated with external genital anomalies, and a literature review.

Keywords: accessory scrotum; children; labioscrotal fold; lipoma; perineal mass.

FIGURE 1

Case 1. (a,b) Soft and spherical mass under the right hemiscrotum, with rugated pigmented scrotal skin on its upper part associated with incomplete penoscrotal transposition and right penoscrotal fusion. (c) Abdominal MRI: exophytic adipose tissue mass. (d) Surgery: excision of the mass and (e) perineal closure through interrupted resorbable stitches. (f) One-month follow-up. (g,h) Histological examination of specimens showing an area characterized by smooth muscle bundles dispersed in dermal collagen and a contiguous area with an abundant mature adipose tissue in the deep dermis and hypodermis.

FIGURE 2

Case 2. (A,B) Soft and spherical mass arising from the caudal aspect of the right labium major and from the perineum with a hollow and wrinkled skin area on its center side. (C) Surgery: excision of the mass. (D) Surgical specimen. (E) Perineal and labium skin closure through interrupted resorbable stitches. (F) One-month follow-up. (G,H) Histological examination: abundant mature adipose fat cells and superficially intra-lesional entrapped adnexal structures.

FIGURE 3

Case 3. (a) Soft and spherical mass arising from the central and caudal aspects of the left labium major with wrinkled unpigmented skin above. Surgery: (b) excision of the mass and (c) the labium major after mass excision. (d) Suture edges conform to continuous resorbable stitches. (e) Surgical specimen. (f) One-month follow-up. (g,h) Histological examination: abundant mature adipose fat cells both in the deep dermis and hypodermis and smooth muscle bundles in the dermis (arrows).