Acquired vulvar lymphangioma circumscriptum: a comparison of 12 cases with Crohn's associated lesions or radiation therapy induced tumors.
BACKGROUND
Lymphangioma circumscriptum (LC) is a benign lesion of lymphatic origin. Vulvar involvement occurs in various clinical settings.


METHODS
We present 12 cases, and compare lesions in patients with Crohn's disease and those associated with pelvic radiation.


RESULTS
The average age at presentation was 49 years. Thirty-three percent of the patients had Crohn's disease, 58% had radiation therapy and 9% had no significant medical history. Sixty-seven percent of the patients had multifocal lesions in anatomically distinct regions. Patients presented on average 16 years after onset of predisposing factors. Presenting complaints were pruritus, wetness and vulvar edema. Lesions were clinically heterogeneous, often found on the labia majora. Lesions consisted of dilated lymphatic channels at the junction of the reticular and papillary dermis. The cells lining these spaces lacked cytologic atypicality or mitotic activity. All lesions so examined were immunoreactive for D240. Patients were most often treated with surgical excision followed by laser ablation. Four of twelve patients, all with radiation-associated lesions, experienced disease progression necessitating additional surgery.


CONCLUSIONS
Patients with LC secondary to radiation, when compared to those with Crohn's disease, were 10 years younger, more likely to have associated co-morbidities, and frequently experienced disease progression needing additional surgeries. Acquired vulvar LC has multiple causes with differing prognosis.
Papalas
John A
JA
Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA. papal002@mc.duke.edu
Robboy
Stanley J
SJ
Burchette
James L
JL
Foo
Wen-Chi
WC
Selim
M Angelica
MA
D000328:Adult; D000368:Aged; D000911:Antibodies, Monoclonal / Q000378:metabolism; D058846:Antibodies, Monoclonal, Murine-Derived; D014408:Biomarkers, Tumor / Q000378:metabolism; D015897:Comorbidity; D003424:Crohn Disease / Q000453:epidemiology / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006801:Humans; D008202:Lymphangioma / Q000453:epidemiology / Q000378:metabolism / Q000473:pathology*; D008875:Middle Aged; D009381:Neoplasms, Radiation-Induced / Q000453:epidemiology / Q000378:metabolism / Q000473:pathology*; D011878:Radiotherapy / Q000009:adverse effects; D014846:Vulvar Neoplasms / Q000453:epidemiology / Q000378:metabolism / Q000473:pathology*; D055815:Young Adult
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D014408:Biomarkers, Tumor; C500987:monoclonal antibody D2-40