Acquired Vulvar Lymphangioma Circumscriptum: A Comparison between Crohn's Associated and Radiation Therapy Induced Tumors
JA Papalas, SJ Robboy, MA Selim. Duke University Medical Center, Durham, NC
Background: Lymphangioma circumscriptum is a tumor of lymphatic origin which occurs throughout the body. Vulvar involvement may occur in a variety of clinical settings.
Design: We review 11 cases, with an average follow-up time of 6 years with an emphasis on comparison between cases that occur in patients with Crohn's disease and those with a history of pelvic radiation exposure.
Results: The average age at presentation was 52 years old. 64% of patients had lesions in multiple anatomically distinct regions. 27% of patients had a history of Crohn's disease, 64% had a history of radiation therapy for abdomino-pelvic malignancy, and 10% had no significant past medical history. 4/11 patients had lower extremity edema and 4/11 patients had fistula tracts. Average time to onset after first documentation of Crohn's disease or after undergoing radiation therapy was 17.8 years. Common presenting complaints were vulvar puritus, wetness, and edema. Clinically, the tumors ranged from tan-white pedunculated papules to sessile nodules (2.0 cm on average) or were weeping vesicular patches, most often found on the labia majora followed by the mons pubis. The tumors were composed of subcutaneous dilated lymphatics located at the junction of the reticular and papillary dermis with some demonstrating focal extension into the epidermis. No lesions involved the subcutaneous tissues. Cytologically, cells protruded into the dilated lymphatic channels in a hobnail pattern with minimal cytologic atypia and mitotic activity. All lesions so examined were positive for D240. Patients were most often treated with surgical excision followed by excisional biopsy and laser ablation. 4/11 patients would experience disease progression necessitating additional excisions.
Conclusions: When comparing patients with a history radiation therapy to those with Crohn's disease, those treated with radiation present almost 20 years sooner, are more likely to have associated co-morbidities, and are more likely to have disease progression requiring additional surgeries.
|Number of patients||3||7|
|Years to onset||32||15|
|Associated lower extremity edema||0||4|
|History of fistula tract formation||3||1|
|Progression requiring additional surgery||0||4|