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 39-year-old Japanese male presented with a 3 months history of a skin tumor on the left side of the jaw. Past medical history revealed lung tuberculosis at age 20 years old. Physical examination showed a dome-shaped, slightly erythematous nodule measuring approximately 2 cm in diameter (Fig. ). Preoperative examination revealed no lymph node or distant metastasis. Punch biopsy was performed, and it was diagnosed as mucinous adenocarcinoma of the skin.\nThe tumor was excised with 5-mm surgical margin and was reconstructed with local rotational flap. The definitive histopathology showed small cellular nests of adenocarcinoma lying in pools of extracellular mucin that were separated by fibrocollagenous septae without involvement of underlying muscle. (Figure a,b) The immunohistochemical staining revealed that the tumor cells were positive for CK7, epithelioid membrane antigen (EMA), MUC1, MUC2, estrogen receptor (ER), and progesterone receptor (PR).\nThe patient again noticed a small tumor on the left side of the jaw in the area of the previous operation site 2 years after the primary operation. However, the patient had chosen not to be examined by a physician and left it alone for 4 years. Because of the rapid growth of the tumor, the patient visited our clinic. At this point, the tumor measured approximately 6 cm in diameter (Fig. a). Preoperative examination revealed invasion of the mandible and one left neck lymph node metastasis. The lesion was excised with at least 1-cm border of normal skin including the mandible (Fig. ). A left radical neck dissection was performed, and the mandible was reconstructed with a free scapula flap. The definitive pathology revealed MCS recurrence with no residual tumor cells in the excised normal skin area. One of 20 regional lymph nodes contained metastasis. Postoperatively, the patient was free of tumor and was followed up by the physician in charge every 3 months for 5 years and every 6 months for the next 5 years. The patient was disease-free for ten consecutive years on physical examination, chest X-ray, neck echogram, and ordinary blood test. Although the patient did not have any symptoms, the chest X-ray at the 11th year follow-up revealed multiple lung metastases (Fig. a,b). Additional chest computed tomography (CT), magnetic resonance imaging (MRI), and RI showed cervical vertebrate (Th5) tumor, and biopsy revealed metastasis of MCS. The patient received radiotherapy for pain control and died 3 year after the diagnosis of metastasis.