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Older woman presents with chronic right upper lid cystic lesion
Pathology of the lesion showed a well-circumscribed papillary, solid nodular proliferation of basophilic epithelial cells, with the central area and microcysts filled with mucin.
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A 71-year-old woman presented to the New England Eye Center Oculoplastics Department for a right upper lid lesion that was present for approximately 1 year. She said that it did not change in appearance or size and was tender to touch. The lesion did not reduce in size despite weeks of warm compresses. Her medical history included diabetes type 2, gout, cardiac murmur, hypertension, hyperlipidemia and hypothyroidism, all of which were being treated with medications.
Examination
The patient’s best corrected visual acuity was 20/20 in both eyes. The pupils had no evidence of an afferent pupillary defect or anisocoria in light or dark. Extraocular movements were full, and IOPs were normal. On external exam, the right upper lid had a white cystic-type non-ulcerated lesion with minimal telangiectasia and no madarosis (Figure 1). On slit lamp biomicroscopy, the anterior segment was significant for posterior chamber IOLs in both eyes, and posterior segment examination of the right eye revealed an epiretinal membrane with few peripheral drusen.
Due to the eyelid lesion causing the patient irritation, it was removed and sent to pathology (Figures 2 to 4). The hematoxylin and eosin stain of the lesion showed a well-circumscribed papillary, solid nodular proliferation of basophilic epithelial cells, with the central area and microcysts filled with mucin. This lesion also stained positively with mucicarmine, synaptophysin, cytokeratin 7, estrogen and progesterone stains.
Figure 1. Right upper lid with a white cystic-type non-ulcerated lesion with minimal telangiectasia and no madarosis.
Images: Sodhi AK, Kapadia M
Figure 2. H&E stain of a well-circumscribed cystic, papillary, solid nodular proliferation of basophilic epithelial cells, with a central area of mucin with microcysts containing mucin.
Figure 3. Mucicarmine stains mucus pink or red, which helps identify mucus-secreting adenocarcinomas.
Figure 4. Lesion staining positive with synaptophysin stain, which is a neuroendocrine marker for synaptic vesicle glycoproteins in neuroendocrine cells and all neurons taking part in synaptic transmissions.
What is your diagnosis?
Chronic cystic lesion
The diagnosis of mucin-producing eccrine carcinoma is difficult without histopathology because this lesion has nonspecific clinical features. Based on the cystic appearance, the differential includes benign and malignant lesions. Benign cystic lesions include epidermal inclusion cyst, apocrine or eccrine hidrocystoma, trichoepithelioma, syringoma or pleomorphic adenoma. Malignant cystic lesions include cystic basal cell carcinoma or cystic squamous cell carcinoma.
Mucin-producing eccrine carcinoma is a rare, low-grade sweat gland carcinoma found in older individuals (mean age: 70 years), mostly women. There are no well-identified risk factors to date. It typically presents as a slow-growing nodule and nonspecifically appears as a flesh-colored non-ulcerated growth with focal telangiectatic vessels with or without madarosis. Size ranges from 1 mm to 1.2 cm. It has a tendency to occur on the eyelids, although there is no predilection for upper or lower eyelid involvement.
Histopathologically, the lesion is characterized by a solid, cystic, papillary architecture with well-differentiated nests of cuboidal or round cells suspended in the pools of mucin. The nuclei have diffusely stippled chromatin, giving a “salt and pepper” appearance.
Mucicarmine stain confirms the presence of intracellular mucin in these cells. There are also small pools of extracellular mucin, but it is never abundant compared with mucinous adenocarcinoma. Immunohistochemical expressions of cytokeratin 7, carcinoembryonic antigen, estrogen receptor and progesterone receptor confirm sweat gland differentiation, and synaptophysin, chromogranin and CD56 are the neuroendocrine markers needed for diagnosis. Notably, there is no statistically significant relationship between the number of neuroendocrine markers expressed or architectural type with the tumor growth pattern.
Jennifer Renz
Avneet K. Sodhi
Complete tumor excision is recommended to manage mucin-producing eccrine carcinoma, either with Mohs micrographic surgery or wide local excision with frozen section control of margins. Adjuvant external beam radiation and sentinel lymph node biopsy are typically reserved for reoccurrences. Zembowicz et al observed that 50% of their case series of 12 patients with mucin-producing eccrine carcinoma had coexisting invasive mucinous adenocarcinoma by histology, suggesting that mucin-producing eccrine carcinoma could be a precursor to invasive mucinous adenocarcinoma. Fortunately, mucinous adenocarcinoma is a locally aggressive tumor with low metastatic potential. In 2013, Hoguet et al reported a recurrence rate of 12% over 20 months, but previous literature reported at least a 30% to 40% recurrence rate, with follow-up ranging from 3 months to 12 years.
Due to possible association with invasive mucinous adenocarcinoma, patients should be followed for recurrence.
Follow-up
Our patient underwent Mohs surgery with subsequent lid reconstruction and will be followed closely.