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Simplex (differentiated) type VIN: absence of p16INK4 supports its weak association with HPV and its probable precursor role in non‐HPV related vulvar squamous cancers

any histological resemblance to tumours of Müllerian origin support such a diagnosis. The most effective therapy is complete surgical excision, while the role of adjuvant radiotherapy and chemotherapy remains controversial. The FATWO should be distinguished from granulosa cell tumours, mainly of the diffuse or the infantile type, Sertoli-Leydig cell tumours and adenomatoid tumours and endometrioid carcinomas of the Fallopian tubes. The resemblance to these tumours, however, is only superficial and, if sufficient tissue is examined, the Wolffian origin of the tumour becomes apparent. Endometrioid carcinoma of the Fallopian tube usually occurs as an intralumenal mass and has abnormal hyperchromatic nuclei and high mitotic activity. Intralumenal mucin is common, and reticulin is arranged around groups of neoplastic glands that are EMA-positive. Sertoli-Leydig cell tumours more commonly occur in young females, aged 20–30 years, and develop in the ovaries, rather than the broad ligaments or the mesosalpinx. More importantly, they contain typical Leydig cells with abundant eosinophilic granular cytoplasm, and may be associated with endocrine manifestations due to androgen secretion. In addition, Sertoli-Leydig cell tumours often express a-inhibin. Granulosa cell tumours are composed of typical cells exhibiting longitudinal nuclear grooving and a strong diffuse inhibin positivity. Adenomatoid tumours also contain cyst-like spaces, but these are set in a connective tissue stroma that is often hyalinized and contains smooth muscle fibres. Nonetheless, it has been reported that among 25 FATWO seen in consultation, the correct diagnosis was considered by the referring pathologist in only 17%. The biological behaviour of these tumours is unpredictable but is, in general, benign. This is only the seventh malignant tumour described thus far, with distinctive morphological features being the large size of the tumour, larger than 100 mm, apparent hypercellularity, capsular invasion, and rupture of the capsule with demonstrable tumour implants and metastases. This report serves to remind us of the existence of female adnexal tumours of probable Wolffian origin and, in particular, their malignant potential.