A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.
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.