Vulvar lymphangioma circumscriptum presenting periodic symptoms

Abstract

The authors report a case of acquired lymphangioma circumscriptum (LC), localised in the right labium majus of vulva and characterised by periodic symptoms for 8 years; however, 3 months after the right major labiectomy, the same symptoms developed in both the left labium majus and the right labium minus. To the best of our knowledge, this is the first case of acquired LC with this type of localisation and symptoms without any known aetiologic factor.

Acquired lymphangiectasia (lymphangioma circumscriptum) of the vulva: Clinicopathologic study of 11 patients from a single institution and 67 from the literature

“…Our search of the English language literature found 67 patients with ALV reported as of June 2014, and the mean age of affected patients was 50 years. Overall, 41 patients (61%) had a history of malignancy,,,,,,,,,,, 27 of whom had cervical cancer ,,,,,,,,,,,,. Other malignancies represented included endometrial cancer ( n = 3), breast cancer ( n = 3), Hodgkin lymphoma ( n = 3), vulvar cancer ( n = 2), rhabdomyosarcoma ( n = 2), and melanoma ( n = 1) .…”

Acquired Vulvar Lymphangioma Circumscriptum: Two Cases Reports

“…5,9 Vulvanın kazanılmış LS'si uzun dönemde, pelvik lenfatik sistemin obstrük-siyonunun komplikasyonu sonucu ortaya çık-maktadır. 3,4 Vulvar bölgede lokalize kazanılmış LS en sık, serviks kanseri nedeni ile yapılan cerrahi, radyoterapi, enfeksiyon (tüberküloz, erizipel vb. ), Crohn hastalığı, konjenital displastik anjiyopati ve konjenital lenfödem sonrası görülmektedir.…”

Acquired vulvar lymphangioma circumscriptum after cervical cancer treatment: Case report

“…Acquired LC is a rare entity which results from lymphatic damage; and can arise in the vulvar region after radiation therapy for cervical cancer, as seen in this case. Other causes include previous surgery, infection (tuberculosis, filariasis, recurrent cellulitis), and Crohn's disease ( Kokcu et al, 2010 , Stewart et al, 2009 , Vignes et al, 2010 ). Architectural disruption causes dilatation and sequestration of previously normal lymphatic channels ( Stewart et al, 2009 ).…”

“…Common presenting symptoms include pain, oozing of lymphatic fluid, sexual dysfunction, and infection. Patients may also have periodic labial swelling, pruritus, and recurrent cellulitis ( Sinha et al, 2015 , Kokcu et al, 2010 , Vignes et al, 2010 ). Grossly, the lesions can be wart-like or papulovesicular; and may be confused clinically for fungating squamous cell carcinoma, molluscum contagiosum, condyloma acuminatum, or other viral/fungal infections( Sinha et al, 2015 , Kokcu et al, 2010 , Stewart et al, 2009 , Mu et al, 1999 ).…”

“…LC usually presents as a warty appearing lesion which may or may not weep lymphatic fluid, and is often painful. The clinical differential diagnosis includes malignant tumor, condyloma acuminatum, molluscum contagiosum, and genital herpes ( Sinha et al, 2015 , Kokcu et al, 2010 ). The lesion has been classified as either 1) congenital which presents in childhood, usually before the age of 5 years, due to improper development of lymphatic channels with no connection to the main lymphatic drainage ( Roy et al, 2006 ); or 2) acquired which presents in adulthood as a consequence of surgery, infections, or radiation resulting in a disruption of a previously normal lymphatic system ( Sinha et al, 2015 , Kokcu et al, 2010 , Stewart et al, 2009 , Vignes et al, 2010 , Buckley and Barnes, 1996 ).…”