Primary cutaneous adenoid cystic carcinoma in the United States: Incidence, survival, and associated cancers, 1976 to 2005

Abstract

Background-Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare appendageal tumor of uncertain origin. Details on epidemiologic features of PCACC are sparse and largely based on clinical reports.

Primary cutaneous adenoid cystic carcinoma with MYB aberrations: report of three cases and comprehensive review of the literature

“…The PubMed‐based search revealed a total of 114 PCACC published cases the majority of which were part of case reports, with only a couple of small series and four relatively large series first authored by Seab & Graham, Urso et al, Ramakrishnan et al and North et al, which included 10, 5, 27 and 19 PCACC cases, respectively. After including our three cases, complete clinical information was found in 116 cases with 1 case that was reported as part of an immunohistochemical study did not include any clinical data .…”

“…This list includes the first report by Boggio, as well as three tumors that predate that publication and were reported under different rubrics and were later retrospectively deemed to be PCACCs . An epidemiologic study utilizing data from the United States Surveillance, Epidemiology and End Results (SEER) program indicated that 152 cases of PCACC were diagnosed (not necessarily published) in the 30‐year period spanning from 1976 to 2005, yielding an incidence rate of 0.23 cases per 1 million people per year . This SEER‐based study also pointed out that PCACC arises in older patients with a median age of 64 years (mean: 63 years, range: 23–94 years), has a predilection for the head and neck/face region and a low‐grade yet protracted clinical course including multiple recurrences .…”

“…An epidemiologic study utilizing data from the United States Surveillance, Epidemiology and End Results (SEER) program indicated that 152 cases of PCACC were diagnosed (not necessarily published) in the 30‐year period spanning from 1976 to 2005, yielding an incidence rate of 0.23 cases per 1 million people per year . This SEER‐based study also pointed out that PCACC arises in older patients with a median age of 64 years (mean: 63 years, range: 23–94 years), has a predilection for the head and neck/face region and a low‐grade yet protracted clinical course including multiple recurrences . Although very useful and important, SEER‐based studies could be afflicted by potential ‘contamination’ from misdiagnosed cases, as the actual pathology of the cases is usually not reviewed.…”

Primary cutaneous adenoid cystic carcinoma of the chest wall: A rare entity

“…[3] However, a US-based study comprising a larger population group shows the incidence to be equal in both the sexes. [6] Perineural invasion, although an important feature is noted only in 76% of cases. [7] In comparison to the analogous tumor of the salivary glands, PCACC shows less aggressive behavior.…”

Cryotherapy as a method of choice in the treatment of cylindromas on the elderly

“…It is often observed on the scalp and face (usually in the vicinity of the ear). Cylindromas are rarely found on the trunk or extremities [1] . It is also known as "turban tumor", as the extensive scalp involvement can create a clinical morphology that resembles a headpiece.…”