Cytomorphologic Features of Extramammary Paget’s Disease of The Vulva in a patient with history of Squamous Cell Carcinoma of The Cervix, Status Post Chemoradiation and Neoadjuvant Therapy. The Importance of Basic Principles

Abstract

Abstract Introduction/Objective Extramammary Paget disease (EMPD) is a rare neoplasm commonly affects postmenopausal women. It usually presents in the anogenital area where apocrine sweat glands are abundant, most commonly in the vulva. The disease is characterized by slow grow and high local recurrence rates. Clinically, EMPD present as well demarcated erythematous lesion or plaques that may ulcerated. Microscopically, it shows a group of atypical cells with abundant clear cytoplasm and nuclear pleomorphism. Methods/Case Report Here in we present a 58-year-old female with history of vulvar intraepithelial neoplasia III (VIN III) status post wide local excision, and poorly differentiated squamous cell carcinoma status post radical hysterectomy and bilateral salpingo-oopherectomy and chemoradiation who presented for perineal pain, itching and discomfort. She also noticed skin changes on her left labia without bleeding or discharge. Punch biopsies of the vulva and periurethral areas revealed acanthosis of the epidermis with intraepidermal scattering of single or clusters of large cells with round/ovoid nuclei and abundant clear cytoplasm. The cells are positive for p16, CK19, CK7, PAX8 supporting the diagnosis of EMPD without evidence of dysplasia. The concurrent PAP smear shows hypercellular specimen composed of hyperchromatic fragments of tissue with high nuclear-to-cytoplasmic rations, and apoptotic bodies. The presence of intracytoplasmic mucin and the tridimensionality of the fragments supported the diagnosis of adenocarcinoma. The HPV testing was positive for HPV-16. Results (if a Case Study enter NA) N/A Conclusion This study compares the histological and cytomorphological features of EMPD with high-grade squamous intraepithelial lesion (HSIL), since the molecular pathways, precursor lesions, etiologic associations, staging, clinical treatment, and prognosis differ substantially and may have a significant clinical impact for the patient’s treatment.