Primary Cutaneous Mucinous Carcinoma of the Eyelid in a Young Male

Abstract

Primary cutaneous mucinous carcinoma of the eyelid is an adenocarcinoma of the eccrine glands. It is rare and locally aggressive but the prognosis following local excision, confirmed with tumour-free margins, is good. This tumour is usually described in the elderly. We present the occurrence, clinical and histological features, and management of this tumour in a young male.A 36-year-old male presented with a small cystic right lower lid lesion, which had increased in size and pigmentation over two years. He underwent excision biopsy for diagnostic purposes followed by Moh's micrographic surgical removal. The defect was repaired with an upper eyelid skin graft. A full oncological screen including whole-body computed tomography scan excluded the presence of primary mucinous carcinoma elsewhere and any metastatic spread. There has been no recurrence of tumour 18 months following excision. Ophthalmologists should be aware of the occurrence of this tumour in a younger age group than previously described. Moh's micrographic surgery is the most suitable method of treatment following exclusion of both distant primaries and metastases.

Unusual Eyelid, Periocular, and Periorbital Cutaneous Malignancies

“…25 When involving the eyelids, the lesion has been described as a solid nodule (4 to 25 mm in diameter) or cystic lesion with a bluish or darkened appearance. 23,25,30,31 Telangiectasia or madarosis may be present but ulcerative features are unusual and the overlying skin is typically intact. 34 Associated inflammation in the adjacent eyelid skin is almost never seen.…”

“…21,23,35,38,39 In the periocular area, deep orbital invasion requiring orbital exenteration has been reported, 39 and there are several examples of distant metastasis in the literature, although this is considered extremely rare for periocular MCS. 40,41 Once a metastatic lesion has been ruled out, the primary treatment for eyelid MCS is local excision with wide surgical margins.…”

“…31 Immunohistochemistry staining of MCS demonstrates positivity with low molecular cytokeratin, S-100, epithelial membrane antigen, carcinoembryonic antigen (CEA), and estrogen and progesterone receptors. 23,31,37 This pattern of staining can be used to differentiate MCS from metastatic mucinous carcinoma arising from other sites such as the ovary or colon, 37 but not the breast, which has a virtually identical immunohistochemistry profile. A possible exception to this rule is staining to cytokeratin 20, which has been reported to be positive in metastatic breast carcinoma but absent in MCS.…”

“…In critical areas such as the upper eyelid and the medial canthus, Mohs micrographic surgery is recommended to ensure clean margins whereas maximizing tissue preservation. 23,33,42 Recurrence rates for MCS after Mohs seem to be extremely low, 33,42 and assessment of margins can be aided by immunohistochemistry using low-molecular weight cytokeratin. 42 Despite the risk of regional spread, prophylactic lymph node dissection is not recommended for periocular MCS, mainly because of the significant morbidity associated with this procedure.…”

“…22 Primary MCS is indistinguishable by light microscopy from metastatic mucinous carcinomas arising from the gastrointestinal tract, ovary, salivary glands, or breast. 23 Mucinous or colloid carcinoma of the breast is particularly difficult to distinguish from MCS, and the diagnosis of a primary sweat gland MCS is usually determined only after carefully examining the patient's past medical history and performing a systemic workup to rule out metastatic disease. Recently, a variant of mucinous sweat gland carcinoma with neuroendocrine differentiation has been designated by the term endocrine mucin-producing sweat gland carcinoma (EMPSGC).…”