Spectrum of Changes in Anogenital Mammary-like Glands in Primary Extramammary (Anogenital) Paget Disease and Their Possible Role in the Pathogenesis of the Disease. Am J Surg Pathol 2017 Aug;41(8):1053-1058
Date
06/15/2017Pubmed ID
28614205DOI
10.1097/PAS.0000000000000857Scopus ID
2-s2.0-85020714728 (requires institutional sign-in at Scopus site) 9 CitationsAbstract
To determine whether a subset of primary extramammary Paget disease (EMPD) may originate in anogenital mammary-like glands (AGMLG), the authors studied 181 specimens of EMPD, detailing alterations in AGMLG. The latter were identified in 33 specimens from 31 patients. All patients were women, ranging in age from 38 to 93 years (median, 65 y). In all cases, lesions involved the vulva and in 1 patient the perianal skin was affected. Histopathologically, AGMLG manifested changes identical to columnar cell change (CCC) (87.1%), usual ductal hyperplasia (22.6%), columnar cell hyperplasia (CCH) (9.7%), oxyphilic (apocrine) metaplasia (6.5%), and atypical duct hyperplasia (3.2%). Four cases (12.9%), in addition to intraepidermal carcinoma, harbored invasive carcinoma. In all 4 of these, AGMLG displayed a range of alterations including ductal carcinoma in situ, CCC, and CCH. Three further cases (9.7%) showed ductal carcinoma in situ without any definite invasive carcinoma. Colonization of AGMLG by neoplastic Paget cells was noted in 6 cases. As CCC and CCH may be encountered in normal AGMLG, these alterations are unlikely to play a significant role in the pathogenesis of the disease. However, by analogy with mammary Paget disease, rare cases of primary EMPD may originate in AGMLG with a subsequent upward migration of the neoplastic cells into the epidermis and possible later breach through the basal membrane. Usual ductal hyperplasia and atypical duct hyperplasia can then be regarded as earlier precursor lesions, linking both ends of the spectrum.
Author List
Konstantinova AM, Spagnolo DV, Stewart CJR, Kacerovska D, Shelekhova KV, Plaza JA, Suster S, Bouda J, Kyrpychova L, Michal M, Belousova IE, Kerl K, Kazakov DVMESH terms used to index this publication - Major topics in bold
AdultAged
Aged, 80 and over
Anal Canal
Anus Neoplasms
Female
Humans
Middle Aged
Paget Disease, Extramammary
Vulva
Vulvar Neoplasms