Primary Villoglandular Mucinous Adenocarcinoma of the Vulva

Matsuzaki, Akiko; Saio, Masanao; Kosuge, Noritake; Aoyama, Hideyasu; Tamaki, Tomoko; Matsumoto, Hirofumi; Yoshimi, Naoki

doi:10.1155/2017/1765460

Cited by 8 publications

(5 citation statements)

References 21 publications

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“…Immunohistochemistry plays a determinant role in the diagnosis. Unlike most Müllerian tumors of female genital tract, adenocarcinoma of intestinal type very often overexpresses CK20 and less often CK7 (23). However, a few cases have also reported expression simultaneously of CK 7 and CK20 (8).…”

Section: Discussionmentioning

confidence: 99%

Vulvar Adenocarcinoma of Intestinal Type: A Case Report of an Uncommon Entity

Moscoso

Reques

Saco

et al. 2021

International Journal of Gynecological Pathology

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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 in additional cases to clarify the role of human papillomavirus in this kind of tumor.

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Section: Discussionmentioning

confidence: 99%

Vulvar Adenocarcinoma of Intestinal Type: A Case Report of an Uncommon Entity

Moscoso

Reques

Saco

et al. 2021

International Journal of Gynecological Pathology

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“…Actually, in this case, the results of high-risk-HPV testing were not provided [ 29 ]. Other authors reported the association with high-risk HPV (HPV 16, detected by in situ hybridization) in one case of vestibular adenocarcinoma in situ [ 20 , 36 ]. However, none of the above-mentioned research reported the connection of HPV with specific vulvar cancer subtype VAIt.…”

Section: Resultsmentioning

confidence: 99%

“Intestinal-Type” Vulvar Adenocarcinoma: A Review of the MITO Rare Tumors Group

Dellino

Cicogna

Falcone

et al. 2022

Cancers

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Intestinal-type adenocarcinoma (VAIt) represents a sporadic variant of vulvar carcinoma. It appears frequently localized to epithelial glands in the vulvar region, and it probably derives from cloacal remnants persisting in the adult. We performed a systematic review of the limited cases reported in the literature, with the intent to assess the specific peculiarities of this rare neoplasia and to state consistent management recommendations. The principal histological VAIt characteristic is that it resembles mucinous colonic carcinomas. Therefore, immunohistochemical workup, with different tumor markers including CK20, CDX2, and CK7 staining, is needed. To confirm vulvar origin, a thorough diagnostic, and radiological examination is required to rule out other primary malignancies. The gold standard of treatment for VAIt is surgery, with local excision with tumor-free margins. Lymph node staging is an option advised if the tumor size is >2 cm or if lymph node metastases are suspected on imaging. On the other hand, the role of neoadjuvant therapy is still in doubt, but a good response to adjuvant chemotherapy treatments has been described in both advanced and recurrent diseases. Sometimes, VAIt behavior can be unpredictable, with relapses even after many years, so more experiences and longer follow-up periods are needed to elucidate the best therapeutic management and its long-term prognosis.

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“…We reviewed previous reports of adenocarcinoma of intestinal-type of the vulva, and the report by Willén et al is likely a villous adenoma with an uncertain degree of dysplasia in the context of a colorectal adenoma [10]. Matsuzaki et al reported villoglandular adenocarcinoma of the vulva without stromal invasion [24], which could also be considered tubulovillous adenoma with high-grade dysplasia. The cases of adenocarcinoma of intestinal-type of the vulva are considered to have a good prognosis, but it may be possible to rede ne some of them as "adenoma.…”

Section: Discussionmentioning

confidence: 98%

Tubulovillous adenoma with high-grade dysplasia of the vulva harboring high tumor mutational burden and cancer-associated mutations: a case report

Sato

Murakami

Otani

et al. 2022

Preprint

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Background Vulvar cancer is a rare disease, accounting for approximately 5% of gynecological malignancies. Primary adenocarcinoma of intestinal-type of the vulva or its precancerous lesion is extremely rare, and details regarding its origin, evolution and related genetic mutations are unknown. Treatment options for this cancer have not been defined. Case presentation A 63-year-old Japanese woman came to the hospital because she was aware of a vulvar mass. There was a 1 cm mass on the dorsal side of the vulva, just outside the remains of the hymen. Biopsy revealed suspected adenocarcinoma, and wide local excision was performed. From histopathology and immunohistochemistry, the specimen was diagnosed as tubulovillous adenoma with high-grade dysplasia of the vulva. No other primary lesions were found, and the vulva was considered the primary site. A gene panel test (FoundationOneCDx assay) showed a high tumor mutational burden and mutations in TP53, KEL, RB1, RNF43, PTEN, GNAS, and PIK3CA. Conclusions The current case of tubulovillous adenoma with high-grade dysplasia of the vulva had a variety of cancer-associated mutations, despite being a precancerous lesion. In cases of intestinal-type neoplasms of the vulva, it may be helpful to check tumor mutational burden and gene mutations for treatment selection.

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Primary Villoglandular Mucinous Adenocarcinoma of the Vulva

Cited by 8 publications

References 21 publications

Vulvar Adenocarcinoma of Intestinal Type: A Case Report of an Uncommon Entity

Vulvar Adenocarcinoma of Intestinal Type: A Case Report of an Uncommon Entity

“Intestinal-Type” Vulvar Adenocarcinoma: A Review of the MITO Rare Tumors Group

Tubulovillous adenoma with high-grade dysplasia of the vulva harboring high tumor mutational burden and cancer-associated mutations: a case report

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