title: Syringocystadenocarcinoma Papilliferum In Situ-Like Changes in Extramammary Paget Disease: A Report of 11 Cases.
abstract: The authors report 11 cases of extramammary Paget disease (EMPD), all of which also demonstrated a combination of histological changes highly reminiscent of syringocystadenocarcinoma papilliferum in situ. In addition to the classical features of EMPD, characterized by the intraepidermal spread of individually dispersed neoplastic cells with ample cytoplasm, many of which contained mucin, there were areas of acanthosis with the substitution of spinous layer keratinocytes by neoplastic cells, whereas the native basal cell layer was intact. In addition to acanthosis (and sometimes papillomatosis), the dermal papillae showed a prominent infiltrate of plasma cells, completing the resemblance to syringocystadenocarcinoma papilliferum in situ; this similarity was further enhanced in 2 cases, which showed conspicuous gland formation. One additional case showed multifocal dermal proliferations compatible with eccrine syringofibroadenoma (syringofibroadenomatous hyperplasia). The changes described herein seem to be relatively rare in EMPD, and they can represent a diagnostic pitfall, as evidenced by 2 cases that were originally misinterpreted as syringocystadenocarcinoma papilliferum in situ. Clinically, these microscopic changes sometimes corresponded to nodular lesions, which were specifically noted to have a papillated erosive surface.
authors: *Department of Pathology, Clinical Research and Practical Center for Specialized Oncological Care, Saint Petersburg, Russia; †Department of Pathology, Medical Faculty, Saint Petersburg State University, Russia; ‡Bioptical Laboratory, Pilsen, Czech Republic; §Sikl's Department of Pathology, Medical Faculty in Pilsen, Charles University in Prague, Pilsen, Czech Republic; ¶Department of Histopathology, King Edward Memorial Hospital, Perth, Australia; ‖Department of Pathology, Medical Faculty Hospital, Martin, Slovak Republic; **Pathology and Laboratory Medicine Service, Veterans Administration Medical Center, OK; ††Department of Pathology, University of Oklahoma College of Medicine, Oklahoma City, OK; ‡‡Private Pathology Laboratory, Chomutov, Czech Republic; §§Private Pathology Laboratory, Ostrava, Czech Republic; and ¶¶Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
authors: Anastasia M
authors: AM
authors: Konstantinova
authors: Denisa
authors: D
authors: Kacerovska
authors: Colin J R
authors: CJ
authors: Stewart
authors: Peter
authors: P
authors: Szepe
authors: Jan
authors: J
authors: Pitha
authors: Miroslav
authors: M
authors: Sulc
authors: Vladimir
authors: V
authors: Bencik
authors: Michal
authors: M
authors: Michal
authors: Barbara
authors: B
authors: Shideler
authors: Katrin
authors: K
authors: Kerl
authors: Dmitry V
authors: DV
authors: Kazakov
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001005:Anus Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D018283:Cystadenocarcinoma, Papillary / Q000737:chemistry / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D010145:Paget Disease, Extramammary / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D011237:Predictive Value of Tests; D013544:Sweat Gland Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D014846:Vulvar Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery
chemical_list: D014408:Biomarkers, Tumor