Syringocystadenoma Papilliferum of the Anogenital Area and Buttocks: A Report of 16 Cases, Including Human Papillomavirus Analysis and HRAS and BRAF V600 Mutation Studies

Abstract

Syringocystadenoma papilliferum (SCAP) is a benign tumor most commonly located on the head and neck area often associated with nevus sebaceus. In its usual location, the human papillomavirus (HPV) DNA and mutations in the RAS/mitogen-activated protein kinase signaling pathway have been detected in SCAP. We studied 16 cases of SCAP in the anogenital areas and buttock where this neoplasm is rare and attempted to find out whether SCAP in these sites have different histopathological and molecular biological features. It seems that there is no significant difference between the morphology of anogenital SCAP and SCAP in other locations. Several tumors in our cohort demonstrated features resembling those seen in warts, but HPV DNA was not found in these lesions. On the contrary, we identified DNA of HPV high-risk types in some tumors without HPV-related morphology. Our study confirms the role of HRAS and BRAF V600 mutations in the pathogenesis of SCAP, including SCAP in the anogenital areas and buttock.

Contiguous squamous proliferations in syringocystadenoma papilliferum: A retrospective study of 14 cases

“…The role of human papilloma virus 16/18 infection has also been suggested. 2,9 In this study, we found that the adenomatous component of contiguous squamous proliferations expressed CK7 and GATA3, whereas these markers were not expressed by hyperplastic contiguous squamous proliferation component. Interestingly, the neoplastic contiguous squamous proliferations showed concordant expression of CK7 and GATA3 in both adenomatous and contiguous squamous proliferation components while the normal adjacent epidermis was negative.…”

“…Syringocystadenoma papilliferum is a benign adnexal neoplasm which can either occur sporadically or within a pre-existing nevus sebaceous. 2 Without background nevus sebaceous, syringocystadenoma papilliferum not uncommonly demonstrates contiguous squamous proliferations in syringocystadenoma papilliferum and to explore their pathogenesis.…”

“…1 Konstantinova et al studied syringocystadenoma papilliferum of anogenital region where 10/16 cases (62.5%) had contiguous squamous proliferations. 2 The clinical appearance of the lesion is unable to predict underlying contiguous squamous proliferation. The mean age of patients of syringocystadenoma papilliferum with contiguous squamous proliferation in this study was higher than patients without contiguous squamous proliferation, although this difference was not statistically significant.…”

“…Contiguous squamous proliferations are squamous proliferations which appear adjacent to syringocystadenoma papilliferum. 2 The exact pathogenesis of contiguous squamous proliferations in syringocystadenoma papilliferum is unclear and this study is aimed to identify the morphological spectrum and frequency of contiguous squamous proliferation in syringocystadenoma papilliferum and to explore its pathogenesis.…”

Current Diagnosis and Treatment Options for Cutaneous Adnexal Neoplasms with Apocrine and Eccrine Differentiation

“…Adnexal adenocarcinoma NOS can occur in any skin region, but the most common localization is the head and neck [4]. The primary importance is to rule out the adenocarcinomas metastatic to the skin [37]. There is no consensus on the immunohistochemical local excision (WLE) may be performed, and 2 cm peripheral margins with a minimum deep margin to fascia are suggested because of the high risk of recurrence (up to 30-47%), especially if the surgical margin is 4 mm or less.…”