Painless eyelid nodule

A 72-year-old female presented for evaluation of a lower eyelid lesion. She reported progressive enlargement of a skin colored papule over a year with rapid increase over the last several months. There was no reported inciting event, associated pain, bleeding, drainage, or visual changes. Her past medical history was notable for remote breast carcinoma with subsequent bilateral mastectomy. Physical examination revealed a firm 2.0 cm skin-colored nodule of the lower eyelid (Fig 1). An excisional biopsy was performed (Figs 2 and 3).Fig 2View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig 3View Large Image Figure ViewerDownload Hi-res image Download (PPT) Question 1: Based on the clinical presentation and histologic findings, what is the most likely diagnosis?A.Mucinous carcinomaB.Sebaceous carcinomaC.Basal cell carcinomaD.Endocrine mucin-producing sweat gland carcinomaE.Hidrocystoma Answers:A.Mucinous carcinoma – Correct. Primary cutaneous mucinous carcinoma is a rare adnexal tumor of the sweat gland that most commonly affects the eyelid. Histology shows a dermal tumor consisting of fibrous septae delineating pools of mucin with collections of basophilic epithelioid cells.1Tillit S.M. Iyer S.S.R. Grieser E.J. LiVecchi J.T. Treatment of recurrent primary cutaneous mucinous carcinoma of the eyelid with modified wide local excision.Case Rep Ophthalmol Med. 2020; 2020: 6668640https://doi.org/10.1155/2020/6668640Crossref PubMed Google ScholarB.Sebaceous carcinoma – Incorrect. While sebaceous carcinoma may clinically appear similar to mucinous carcinoma, the 2 can be differentiated histologically. In sebaceous carcinoma, histology reveals lobules of basaloid cells within the dermis with mitotic figures and necrosis.2Kyllo R.L. Brady K.L. Hurst E.A. Sebaceous carcinoma: review of the literature.Dermatol Surg. 2015; 41: 1-15https://doi.org/10.1097/DSS.0000000000000152Crossref PubMed Scopus (72) Google ScholarC.Basal cell carcinoma – Incorrect. Basal cell carcinoma is the most common cutaneous neoplasm and commonly occurs on the eyelid; however, nodes of uniform basaloid cells with stromal clefting arising from the epidermis would be present.D.Endocrine mucin-producing sweat gland carcinoma – Incorrect. Endocrine mucin-producing sweat gland carcinoma is a rare neuroendocrine derived tumor that most commonly presents on the periorbital skin. Histologically this is analogous to papillary breast carcinoma consistent with the similar embryologic origins of both sweat and mammary glands, and would display areas of solid and papillary growth.E.Hidrocystoma – Incorrect. Hidrocystomas are benign cystic proliferations of sweat glands that appear as blue to skin-colored dome-shaped papules or nodules. Characteristic histopathologic findings include a single or multiple cystic cavities within the dermis. Question 2: Additional workup or management should include which of the following?A.Immunohistochemical stainingB.ColonoscopyC.No further management is indicatedD.Warm compressE.Enucleation Answers:A.Immunohistochemical staining – Correct. When evaluating mucinous carcinoma, the major diagnostic concern is determining metastatic or primary cutaneous origin. Given this patient’s history of breast cancer, review of previous breast pathology and workup with immunohistochemical staining is warranted. Primary cutaneous mucinous carcinoma is positive for CK7 and negative for CK20, thyroid transcriotion factor-1, and D2-40,3Javaid H. Raza N. Ejaz U. Sarfraz T. Unusual skin mass (primary cutaneous mucinous carcinoma).BMJ Case Rep. 2018; 2018bcr2017222546PubMed Google Scholar which could help to exclude metastases from colon or other gastrointestinal sites and the lung. However, breast carcinoma displays a similar immunophenotype, and both primary cutaneous and metastatic breast mucinous carcinomas may also express estrogen receptor, progesterone receptor, GATA3, and p63, warranting the need for additional diagnostic imaging to exclude metastasis.B.Colonoscopy – Incorrect. Colonoscopy would be included in the diagnostic workup of sebaceous carcinoma when there is concern for associated Muir–Torre syndrome.C.No further management is indicated – Incorrect. This would not be an appropriate choice for mucinous carcinoma, as further workup and definitive management with surgical excision or radiation therapy is warranted.D.Warm compress – Incorrect. Warm compress is utilized for the conservative management of chalazion.E.Enucleation – Incorrect. Enucleation is used to treat uveal melanoma. Question 3: What histological findings would be seen in this patient?A.Collections of periodic acid–Schiff positive mucinB.Lobular dermal based sebaceous cellsC.Cystic space lined by a double layer of epithelial cellsD.Peripheral palisading with retraction from surrounding stromaE.Well demarcated papillated or cribriform nodules Answers:A.Collections of periodic acid–Schiff positive mucin – Correct. Mucinous carcinomas contain large collections of mucin that are periodic acid–Schiff positive and stains with colloidal iron, mucicarmine, and Alcian blue at a pH of 2.5. The mucin is classified as a sialomucin, a nonsulfated mucopolysaccharide that is resistant to hyaluronidase and diastase.4Chavez A. Linos K. Samie F.H. Primary cutaneous mucinous carcinoma of the eyelid treated with Mohs surgery.JAAD Case Rep. 2015; 1: 85-87https://doi.org/10.1016/j.jdcr.2015.02.002Abstract Full Text Full Text PDF PubMed Scopus (7) Google ScholarB.Lobular dermal based sebaceous cells – Incorrect. Lobular dermal based sebaceous cells with cytoplasmic lipid granules that stain positive with Oil-Red O and Sudan black are found in sebaceous carcinoma.C.Cystic space lined by a double layer of epithelial cells – Incorrect. Hidrocystomas appear histologically as unilocular or multilocular cystic spaces lined by a double layer of epithelial cells.D.Peripheral palisading with retraction from surrounding stroma – Incorrect. Peripheral palisading with retraction from surrounding stroma is a finding present in basal cell carcinomas.E.Well demarcated papillated or cribriform nodules – Incorrect. Cystic or solid well demarcated papillated or cribriform nodules are seen with Endocrine mucin-producing sweat gland carcinoma. Additionally, these tumors will most commonly stain positively for neuroendocrine markers including synaptophysin and chromogranin. Neuroendocrine immunohistochemistry helps to further differentiate from mucinous carcinoma.5Agni M. Raven M.L. Bowen R.C. et al.An update on endocrine mucin-producing sweat gland carcinoma: clinicopathologic study of 63 cases and comparative analysis.Am J Surg Pathol. 2020; 44: 1005-1016https://doi.org/10.1097/PAS.0000000000001462Crossref PubMed Scopus (21) Google Scholar None disclosed.