Malignant hidradenoma of vulva in pregnancy-A rare case

Introduction: Nodular malignant hidradenoma (NH) of vulva is a rare entity. It is a malignant, aggressive tumor of the eccrine glands that was first reported in literature as a clear cell eccrine carcinoma in 1954. It is a rare tumor with predilection for the head face and extremities. It usually present in sixth decade of life with equal male/female distribution but cases have also been reported in children and neonates. Here we present a case of malignant nodular hidradenoma vulva in a 20yr pregnant woman. Case Presentation: A 20yrs old primi patient at 38weeks pregnancy presented to our outpatient department with a painless vulval swelling since 3 months which had increased rapidly in size. On examination the swelling was arising from upper one third of right labia minora. The mass was 4 x 4 cm in size and was displacing the urethral meatus towards left and involving the vagina upto the fornices. The patient had history of excision of similar vulvar swelling 7 months back and the histopathology was suggestive of hidradenoma vulva. Patient delivered vaginally after 2 days. She was thoroughly investigated.CT scan lower abdomen and pelvis and chest x-ray revealed that it was a locally invasive tumor with distant metastasis. Patient was given neoadjuvant chemotherapy for 3 cycles after which surgical excision of the residual disease with bilateral inguinal lymphadenectomy done post operatively 3 more cycles of chemotherapy given. Patient was kept in regular follow up. Conclusion: Malignant hidradenoma of vulva is a locally aggressive tumor with rapid progression, poor prognosis and high recurrence. Early diagnosis and excision with adjuvant chemotherapy is imperative. Keywords: Malignant, Hidradenoma, Vulva, Metastasis.