Depth and Patterns of Adnexal Involvement in Primary Extramammary (Anogenital) Paget Disease: A Study of 178 Lesions From 146 Patients

Abstract

Extramammary Paget disease (EMPD) is a rare neoplasm usually presenting in the anogenital area, most commonly in the vulva. Adnexal involvement in primary EMPD is a very common feature and serves as a pathway for carcinoma to spread into deeper tissue. The depth of carcinomatous spread along the appendages and the patterns of adnexal involvement were studied in 178 lesions from 146 patients with primary EMPD. Hair follicles and eccrine ducts were the adnexa most commonly affected by carcinoma cells. The maximal depth of involvement was 3.6 mm in this series. When planning topical therapy or developing novel local treatment modalities for EMPD, this potential for significant deep spread along adnexa should be taken into account.

Spectrum of Changes in Anogenital Mammary-like Glands in Primary Extramammary (Anogenital) Paget Disease and Their Possible Role in the Pathogenesis of the Disease

“…In contrast, we have previously shown that cutaneous adnexa including hair follicles, sebaceous glands, and apocrine and eccrine units may present a path for carcinomatous spread into deeper tissues in cases of EMPD, as they are involved in almost all cases. 7,22 A similar situation may be presumed with respect to AGMLG. We noticed several patterns of colonization of AGMLG by neoplastic cells.…”

“…If our proposition is confirmed in subsequent studies, this may result in a reevaluation of current therapeutic approaches to primary EMPD, especially with respect to local surgical and nonsurgical treatment, which should take into consideration both the depth of secondary adnexal involvement (up to 3.6 mm) and the depth of location of AGMLG (up to 3.9 mm) as a possible source of disease, including recurrences, which have a rate that ranges currently from 12% to 57%. 3,22,32…”

“…2,7 Further, AGMLG may represent a path for carcinomatous spread into deeper tissues. 2,7,22 The goals of our study were to identify and describe the spectrum of involvement of AGMLG and their ducts in cases of primary anogenital EMPD, and to determine whether a subset of primary EMPD may originate in AGMLG, analogous to breast carcinoma.…”

“…All included patients were the subjects of previous publications addressing various other clinicopathologic aspects of AGMLG and EMPD. 7,22,[27][28][29]…”

Difficulties of the diagnosis of the extramammary Paget disease

“…Опухоль развивается чаще у женщин белой расы, обычно в период менопаузы [2]. Возраст заболевших варьирует от 40 до 95 лет и в среднем составляет 71 год [14]. Хотя возраст пациентки, которую мы наблюдали, не достигал 70 лет, она находились в менопаузе.…”