Microcystic adnexal carcinoma of the orbit mimicking pagetoid sebaceous gland carcinoma

Abstract

Microcystic adnexal carcinoma (MAC) is a rare malignancy of sweat glands that has been reported most often on the face in the form of a cutaneous lesion, with the potential for deeper invasion. The synonyms of MAC include sclerosing sweat duct carcinoma, syringomatous carcinoma, and malignant syringoma. Clinically, MAC in the periocular area has been misdiagnosed as basal cell carcinoma, squamous cell carcinoma, or even chalazia. We report a case of MAC presenting clinically as sebaceous gland carcinoma with pagetoid spread for the first time in literature.

Periocular Microcystic Adnexal Carcinoma: A Case Report and a Major Review

“…Review of the literature identified 92 patients fulfilling the inclusion criteria for this study in addition to our current patient, for a total of 93 patients with MAC. 2,3,5,8,9,11–69 Patient demographics and presenting characteristics are summarized in Table 1.…”

“…Information on the duration of follow-up was available on 64 of 93 patients (69%). 2,3,5,8,9,11–16,18,20–22,24–33,36–51,53–55,57–59,61–69 The average total period of observation from the time of initial presentation to the final documented care was 8 years (median 5, range 0.2–70 years), and the average follow-up after last documented treatment was 3 years (median 2, range 0.2–20 years). Follow-up information was available on 87 of 93 (94%) patients.…”

“…[32][33][34]39,43,45,46,48,62,63 Six patients (6%) underwent orbital exenteration and 1 patient (1%) underwent enucleation. 8,19,31,50,[64][65][66] Biopsy/debulking without definitive resection was performed in 9 (10%) patients. 2,8,24,29,30,40,55,61 Regional lymph node dissection was undertaken in 4 (4%) patients.…”

“…In this review, 22% (20/ 93) of patients at any point of the clinical course demonstrated orbital involvement, requiring orbital exenteration in 14% (13/93) of patients. 2,3,5,8,13,24,31,38,41,44,[48][49][50]55,57,[64][65][66]68 Initial presentation with an orbital mass was seen in 18% (12/68) of cases.…”

Microcystic adnexal carcinoma: report of rare cases

“…MAC is characterized by pilar and eccrine biphasic differentiation and sclerosing stroma [6]. The gross microscopic appearance usually comprises keratin cysts, nests, and cords of bland keratinocytes, and well-differentiated ducts [7]. Furthermore, numerous immunological markers have been reported to be positive in MAC, including CK5/6, CK8/18, EMA, and CK7 [8,9].…”