Clinical outcomes of primary surgical treatment for acquired vulvar lymphangioma circumscriptum

Gun Yoon,Chel-Hun Choi,Tae-Joong Kim,Byoung-Gie Kim,Ji Hye Hwang,Hyun-Soo Kim,Yoo-Young Lee,Jeong-Won Lee,Duk-Soo Bae

doi:10.1007/s00404-015-3801-3

Abstract

ObjectiveTo assess the clinical outcomes of surgical treatment for acquired vulvar lymphangioma circumscriptum in patients who received radical surgery and/or adjuvant radiation therapy for cervical cancer.MethodsA retrospective chart review of eight patients was performed to assess the demographic information, chief complaints, treatment modality for cervical cancer, location, and primary treatment modality for vulvar LC, postoperative changes in symptoms, and/or signs, the development of local recurrence and the outcome of patients.ResultsAll eight patients were previously diagnosed with cervical cancer FIGO clinical stage IA to IIA and received surgery, radiation therapy, or concurrent chemoradiation therapy. Microscopic examination revealed multiple, dilated, D2-40-positive dermal vascular channels containing eosinophilic proteinaceous material, consistent with LC. Most chief complaints showed considerable improvements on assessment at the outpatient clinic after the primary surgery. No patient showed aggravation of symptoms. Two patients developed local recurrences. One patient developed recurrence on the opposite side 13 months after local excision. We performed a second wide local excision. Another patient developed recurrence 47 months after the primary surgery. Since the lesion was very small and localized, we decided to manage it conservatively, but monitor it very closely. The remaining six patients remained free of recurrence.ConclusionIt is not easy for gynecologists to have an initial clinical diagnosis of LC, because there are a number of diseases that exhibit similar clinical manifestation to that of vulvar LC. Even if it is diagnosed correctly, local recurrence often occurs. Relevant symptoms associated with LC are not only distressing, but also affect patients’ quality of life. Based on our data, we propose that surgical treatment could provide a more long-lasting answer compared to other treatment modalities, since it is beneficial in terms of clinical outcomes. In the future, a long-term follow-up investigation is required to assess the prognosis and to compare the efficacy and side effects of each modality.

Highlights

Lymphangioma circumscriptum (LC) is a benign disease of nonspecific origin
All eight patients were previously diagnosed with cervical cancer FIGO clinical stage IA to IIA and received surgery, radiation therapy, or concurrent chemoradiation therapy
We performed a thorough search for previous literature on the treatment of vulvar LC using the US National Library of Medicine’s PubMed database and the Royal College of Obstetricians and Gynecologists database using the term ‘‘lymphangioma circumscriptum’’ and the word ‘‘vulva’’

Summary

Introduction

Lymphangioma circumscriptum (LC) is a benign disease of nonspecific origin It occurs in the lymphatic vascular system in the deep dermal and subcutaneous layer. It often occurs in the proximal part of limbs, which is rich in lymphatic vasculature such as shoulders, axilla, groin, buttock, and so on. Even though primary vulvar LC can occur congenitally (or primarily) due to the developmental defect of the vulvar lymphatic system, it is a very rare condition [3]. It can be acquired (or secondarily) in case of cervical cancer patients who received radical hysterectomy, pelvic lymphadenectomy, or pelvic radiation that can damage the lymphatics [4]. The linkage between the presentation of LC as superficial verrucous vesicles and deep lymphatic vasculature was suggested [5]

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Conclusion