Adnexal Clear Cell Carcinoma with Comedonecrosis (ACCCC)

     

    The patient is a 61-year-old female who presented with a vulvar lesion in July 2005. At that time, she was diagnosed as having squamous cell carcinoma with clear cell features. At the time of diagnosis, the patient declined complete treatment. Nearly 1 year later, she underwent an extensive radical hemivulvectomy with bilateral groin lymph dissection, which showed multiple positive lymph nodes, along with adjuvant radiation therapy.

    She continued to do well till May 2008, when she noticed having groin discomfort. This time the recurrent lesion was biopsied and was reclassified as an adnexal clear cell carcinoma with comedonecrosis, based on newer literature and supporting histologic and immunohistochemical features.