The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.
A 71-year-old man visited our hospital with a raised nodular mass of size 2×3×1 cm in his left groin and an erythematous area of 9×10 cm over the left groin (). He had first noticed the erythema and nodules 6 months before he visited our hospital. The erythema had gradually expanded in size. Ulceration with intermittent bleeding was noted within the nodular mass. On physical examination, edema of the left leg was observed, and lymph nodes in both inguinal regions were palpable. The results of routine hematological and biochemical examinations were normal. Examination of skin biopsies from the inguinal folds revealed features of EMPD in the epidermis and underlying glandular neoplastic foci in the reticular dermis and in the subcutaneous adipose tissue (). Moreover, focal angiovascular invasion was noted. Typical Paget cells were scattered in the epidermis (). The glandular neoplastic foci resembled carcinoma in situ. Some foci showed accumulation of secretion in central regions (). The tumor cells exhibited abundant eosinophilic cytoplasm and large vesicular nuclei.\nThe tumor and Paget cells showed strong positive immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen, cytokeratin 7 (CK7), and gross cystic disease fluid protein-15 (GCDFP-15) () but were negative for melanin-A, S-100, and CK20 (). The patient was diagnosed with apocrine carcinoma related with extensive EMPD. Chest radiography, abdomino-pelvic computed tomography (CT), a gallium scan, a bone scan, and positron emission tomography-CT were performed to exclude the presence of internal malignancy. The results showed multiple lung, bone, and lymph node metastases. After consultation with a urologist and a surgeon, we concluded that this patient was inoperable, and opted for conservative treatment; however, the patient refused any treatment.