Vulvar Adenocarcinoma of Intestinal Type: A Case Report of an Uncommon Entity
| Autor: | Santiago Ramón y Cajal, Adela Saco, Josep Castellví, Ángel García, Natalia R. Gómez-Hidalgo, Olivier Moscoso, Armando Reques |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Pathology
medicine.medical_specialty Keratin-20 Adenocarcinoma Vulva Pathology and Forensic Medicine Lesion Cytokeratin Dermis medicine Humans CDX2 Transcription Factor Papillomaviridae Aged Pruritus vulvae Vulvar Neoplasms integumentary system business.industry Obstetrics and Gynecology Vulvar cancer medicine.disease Immunohistochemistry Dissection medicine.anatomical_structure Carcinoma Squamous Cell Female medicine.symptom business Rare disease |
| Zdroj: | International Journal of Gynecological Pathology. 41:98-103 |
| ISSN: | 0277-1691 |
| DOI: | 10.1097/pgp.0000000000000784 |
| Popis: | Vulvar cancer is rare and accounts for only 5% of all gynecologic cancers. Squamous cell carcinoma is the most common and makes up 90% of the cases. Vulvar adenocarcinoma usually arises in Bartholin and other vulvar glands. Primary vulvar intestinal-type adenocarcinoma is an extremely rare disease with an unclear prognosis and treatment. Its origin is still unknown, the most accepted theory suggests cloacal remnants as the source of origin. Only a few cases have been reported in the literature. We present a case of a 66-yr-old female who presented with vulvar pruritus and local discomfort, showing a 2 cm tumor located in the left labium minor in the region of vulvar fourchette. Wide vulvar excision and bilateral lymph nodes dissection were performed. Other concomitant lesions and distant extension of tumor were ruled out by positron emission tomography. Pathologic study revealed a colonic-type adenocarcinoma with typical villoglandular architecture with an irregular glandular structure composed of atypical columnar epithelium. The lesion had direct contact with epidermal surface and mainly was external without involving the dermis. Immunohistochemical analysis revealed positive staining for cytokeratin 20 and CDX2. p16 showed an abnormal diffuse and strong immunoexpression. The presence of a low-risk human papillomavirus was detected by polymerase chain reaction, therefore, the expression of p16 cannot be explained in this case by the presence of human papillomavirus. Additional studies are needed to be performed in further cases to clarify the role of human papillomavirus in this kind of tumors. |
| Databáze: | OpenAIRE |
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