M H J van Rosmalen1, C Reijnen2, D Boll3, J M A Pijnenborg2, A A M van der Wurff4, J M J Piek3. 1. Department of Obstetrics & Gynaecology, Comprehensive Cancer Centre South Location Elisabeth-TweeSteden Hospital, Dr. Deelenlaan 5, Postbox 90107, 5000 LA Tilburg, The Netherlands. Electronic address: mhjvanrosmalen@gmail.com. 2. Department of Obstetrics & Gynaecology, Comprehensive Cancer Centre South Location Elisabeth-TweeSteden Hospital, Dr. Deelenlaan 5, Postbox 90107, 5000 LA Tilburg, The Netherlands. 3. Department of Obstetrics and Gynaecology, Comprehensive Cancer Centre South Location Catharina Hospital, Michelangelolaan 2, Postbox 1350, 5602 ZA Eindhoven, The Netherlands. 4. Department of Pathology, Elisabeth-TweeSteden Hospital, Hilvarenbeekseweg 60, Postbox 90151, 5000 LC Tilburg, The Netherlands.
Abstract
BACKGROUND: There are limited cases in literature of patients with mucinous adenocarcinoma of the vulva with neuroendocrine differentiation have. With this new case, we aim to provide an overview of the existing literature and present a tool with relevant markers for the pathologist in the differential diagnosis. CASE DESCRIPTION: A 92-year-old multiparous, Caucasian woman presented with a 8 cm spherical tumor of the left major labium. Since the initial punch biopsy was not conclusive, a local resection was performed. Histopathological examination showed mucus production, large pools of mucin with trabeculae and cribriform glandular structures with strongly atypical columnar epithelium. Additional immunohistochemical analysis demonstrated expression of: CEA, CK7, EMA, and the neuroendocrine markers synaptophysin and chromogranin supporting the diagnosis. CONCLUSION: In this report, we present a new case of a mucinous adenocarcinoma of the vulva with neuroendocrine differentiation based immunohistochemical analysis. Due to the indolent tumor behavior, partial vulvectomy is the therapy of choice.
BACKGROUND: There are limited cases in literature of patients with mucinous adenocarcinoma of the vulva with neuroendocrine differentiation have. With this new case, we aim to provide an overview of the existing literature and present a tool with relevant markers for the pathologist in the differential diagnosis. CASE DESCRIPTION: A 92-year-old multiparous, Caucasian woman presented with a 8 cm spherical tumor of the left major labium. Since the initial punch biopsy was not conclusive, a local resection was performed. Histopathological examination showed mucus production, large pools of mucin with trabeculae and cribriform glandular structures with strongly atypical columnar epithelium. Additional immunohistochemical analysis demonstrated expression of: CEA, CK7, EMA, and the neuroendocrine markers synaptophysin and chromogranin supporting the diagnosis. CONCLUSION: In this report, we present a new case of a mucinous adenocarcinoma of the vulva with neuroendocrine differentiation based immunohistochemical analysis. Due to the indolent tumor behavior, partial vulvectomy is the therapy of choice.