Article Text
Abstract
Introduction/Background Leiomyosarcoma of the Vulva is very rare. It accounts for 1–2% of gynaecological cancers. 1,2,3 Leiomyosarcoma originates from smooth muscles, blood vessels, round ligaments, and erector-pili muscles.2,3 On the Bartholin's area it may show similarities to cysts therefore causing delayed diagnosis. 3
Methodology We report the case of a 40-year-old woman who was referred to our tertiary hospital following a routine marsupialization for Bartholin's cyst. The histology confirmed incomplete excision of leiomyosarcoma - smooth muscle tumour of uncertain potential (STUMP). The management of her case was referred to the central Gynaecological Oncology Multidisciplinary Team (MDT). She underwent staging CT and MRI investigations, followed by radical wide local re-excision. Post-surgery adjuvant radiotherapy was considered. However, the patient refused due to insufficient literature support. Instead, close clinical and radiological surveillance was commenced.
Results Pre-surgery CT and MRI investigations did not reveal metastatic disease. Re-excision performed confirmed clear margins with no residual tumour. Eighteen months later she remains tumour free.
Conclusion Atypical presentation of Vulval lesions including at the area of the Bartholin's gland should raise suspicion. Appropriate investigations and referral to a central cancer MDT ought to be considered. Radical wide local excision is the recognized treatment. Adjuvant radiotherapy and/or chemotherapy needs to be considered but individualized to the patient. Intense long term surveillance is required due to possibility of delayed recurrence.
References
Nielsen GP, et al: Smooth muscle tumors of the vulva: A clinicopathological study of 25 cases and review of the literature. Am J Surg Pathol 1996;20:779–793
Losch A, et al: Leiomyosarcoma of the vulval A case report; J Repro Med 2001;46(6):209–612
Chokoeva AA, et al: Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. International J Immuno Pharm 2015;28(2):168–177.
Disclosure Nothing to disclose