Massive Localised Lymphedema of the Male External Genitalia: A Clinicopathologic Study of 7 Cases
Stephen Lee, Jonathan I Epstein. The Johns Hopkins Hospital, Baltimore
Background: Massive localised lymphedema is a clinical and pathologic mimic of sarcoma strongly associated with morbid obesity. The tumor most commonly involves the lower limbs as large pendulous masses. Only 4 case of massive localised lymphedema involving external male genitalia have been reported. In this study we report an additional 6 cases localised to the external male genitalia and 1 involving the spermatic cord.
Design: 7 cases were retrospectively identified from our surgical pathology database, 4 of which were from the consult files of one of the authors.
Results: Of the 7 cases, 5 were in morbidly obese patients (4 presented with diffuse scrotal edema and 1 with a penile mass). A further case of diffuse scrotal edema was associated with penile Crohn's disease in a non-obese patient. The remaining case presented as an inguinal mass associated with an inguinal hermia and spermatic cord lipoma in a non-obese patient. In all cases the clinical impression was of a benign chronic process developing over 3 months to 1 year. Four cases from outside institutions were all referred with benign pathological diagnoses. Grossly, the lesions associated with obesity were large (range 10.5 – 55 cm) in comparison to the non-obese cases (4 – 4.2 cm).
Microscopically, all cases exhibited varying degrees of stromal fibrosis and edema, multinucleated stromal cells, perivascular chronic inflammation, lymphangiectasia and microvascular proliferation. In contast to lesions in soft tissue locations, the presence of entrapped fat was a minor feature (and seen in only 4 cases including the case involving spermatic cord lipoma). Prominent dartos smooth muscle hyperplasia was noted in six lesions.
Conclusions: Massive localised lymphedema occurring in the male external genitalia may present as either diffuse massive enlargement or less commonly as localised smaller masses. Almost all cases are associated with obesity although localised lesions may be associated with local pathology. Lesions in the male external genitalia share many microscopic findings with massive localised lymphedema in other body regions although entrapped adipose tissue is not a prominent feature. A useful additional diagnostic feature is the presence of entrapped hyperplastic dartos muscle bundles within the fibrous areas.
Category: Genitourinary (including renal tumors)
Monday, March 19, 2012 1:00 PM
Poster Session II # 142, Monday Afternoon