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.
An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].