Prognostic Factors, Treatment, and Survival in Primary Cutaneous Mucinous Carcinoma: A SEER Database Analysis

Abstract

BACKGROUND Limited information exists on the demographics, tumor characteristics, and treatment in primary cutaneous mucinous carcinoma (PCMC). OBJECTIVE The authors sought to describe prognostic factors, incidence rates, and the subsequent primary malignancy (SPM) risk in patients with PCMC. METHODS Primary cutaneous mucinous carcinoma cases in the National Cancer Institute's Surveillance, Epidemiology, and End Results data (1972–2013) were analyzed to provide demographic, cancer-related, and treatment information and to calculate incidence and mortality. Patients were stratified by stage (local, regional, distant disease) for comparison. The risk of developing an SPM was calculated. RESULTS Four hundred eleven PCMC cases were identified. The age-adjusted incidence was 0.04 cases per 100,000-person years. Blacks were disproportionately affected by PCMC (0.048; 95% confidence interval, 0.034–0.065; p < .001). Approximately 67.4% of patients had local disease, 10.5% had regional disease, and 5.8% had distant disease. Primary cutaneous mucinous carcinoma–specific mortality was independent of sex, age, race, primary site, histologic tumor grade, tumor size, tumor stage, or treatment. The overall frequency of developing a second primary malignancy was not increased in patients with PCMC. CONCLUSION Although PCMC occurs with equally in both sexes, it may be more common in African Americans than previously recognized. Although eyelid PCMC may have a higher rate of distant metastasis, all patients need close follow-up.

Primary mucinous adenocarcinoma of the eyelid: A case with initial clinical misdiagnosis requiring surgical re-excision of the tumor

“…Regarding ethnicity, most cases reported were of Caucasian race [7] . On the other hand, Rismiller in a large series of 411 cutaneous PMA cases demonstrated equal occurrence in both sexes with more predilection among African Americans [1] . A thorough search of the relevant literature yielded only two reported cases from the Middle East [8] .…”

“…Primary mucinous adenocarcinoma (PMA) is considered a rare clinical entity with an incidence of 0.04 cases per 100,000-person years [1] . It most commonly involves the periorbital region, presenting as an asymptomatic, slow-growing, well-circumscribed mass that is often misdiagnosed as a benign lesion [2] .…”

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

“…It was believed to be of eccrine origin; however, this neoplasm demonstrates apocrine-type differentiation [ 92 ]. It is more widely postulated that endocrine mucin-producing sweat gland carcinoma may be a precursor to MC, despite a different MYC expression [ 93 ].…”

“…Neuroendocrine differentiation and immunophenotype can be identified locally. Immunohistochemical profile of MC includes positivity for CK7, CAM5.2, EMA, CEA, GCDFP-15 (gross cystic disease fluid protein 15), RCC (renal cell carcinoma) antigen, WT1 (Wilms’ tumor protein), ER, PR, and AR [ 93 , 98 , 99 ]. Myoepithelial markers’ staining (p63, calponin) may also be positive.…”

“…Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a low-grade primary cutaneous neoplasm. It was proposed that EMPSGC is an in situ precursor of primary mucinous adenocarcinoma with neuroendocrine features [ 93 ]. However, the more favorable prognosis of neuroendocrine-type mucinous sweat gland adenocarcinoma with reduced recurrence and absence of metastasis compared to other types has been propounded.…”