[Eccrine porocarcinoma of the cheek skin with parotid gland infiltration]. / Ekkrines Porokarzinom der Wangenhaut mit dem klinischen Bild eines primären Gl. parotis-Tumors.

BACKGROUND: Eccrine porocarcinoma is a rare malignant tumor, arising from the intraepithelial ductal parts of the sweat glands. Although the clinical appearance is variable, some tumors present with a diameter of 10 cm or more. In most cases the tumor presents as a slowly growing painless mass. PATIENTS AND METHODS: A case of a 61 year old male patient is presented, who was admitted because of a peripheral facial nerve palsy. The patient reported about a sting in this area some ten years ago with a persistent swelling. Four years ago this lesion began to grow slowly. RESULTS: Clinically a huge mass in the left cheek extended to the parotid gland was obvious. The patient underwent radical tumor excision with histologically clear margins. The defect was reconstructed by a microvascular forearmflap, the facial nerve was reconstructed using either a graft from the suralis nerve (for the upper part of the facial nerve) along with a hypoglossal facial nerve anastomosis for the lower part of the facial nerve. Postoperative radiation therapy was added. Histologically an eccrine porocarcinoma was confirmed with an infiltration of the facial nerve and the parotid gland. Lymph nodes in the neck were negative. DNA image cytometry achieved a DNA malignancy grade of 0.73, the immunohistochemical assessment using a monoclonal antibody against the proliferation antigen MIB-1 achieved a proliferation rate of < 10 %. CONCLUSIONS: Although very rare eccrine porocarcinoma should be considered in the differential diagnosis of slowly growing skin masses in the face. Therapeutically a radical resection with histological clear margins is recommended, in case of lymph node metastases with additional postoperative radiotherapy.

[Eccrine porocarcinoma with extensive cutaneous metastasis]. / Porocarcinome eccrine avec large extension métastatique de voisinage.

BACKGROUND: Porocarcinoma is a malignant tumour of the eccrine sweat duct, arising from acrosyringium. The tumoral lesions involve the deep dermal tissue. CASE REPORT: We report the case of an 84 year-old woman, suffering from a porocarcinoma, extensively involving the major part of the left lower limb. The first symptoms appeared two years ago. Hundreds of metastatic papules and small nodules were present, isolated or confluent into large plaques. The clinical picture was very close to lymphangioma. Diagnosis was confirmed by histopathologic examination. Radiotherapy was useful short-term, allowing partial flattening of the lesions and improving lymphatic drainage, thus providing comfort for the patient. It did not prevent a later progression of the tumoral process. DISCUSSION: Porocarcinoma is a rare tumour that usually appears as a single nodule or a plaque, arising from a preexistent eccrine poroma, or developing de novo. Two histopathological variants are described: trabecular or epidermotropic. This latter form, observed in the present case, is more aggressive, leading to frequent local recurrences and/or metastases. Our report is exceptional: the literature shows only one other case with such widespread cutaneous involvement. The clinical course of our case is discussed.

Recurrent malignant acrospiroma. Treatment by chest wall excision.

Malignant acrospiroma is an uncommon tumor of the eccrine sweat gland. In contrast to its relatively more common benign counterpart, malignant acrospiroma is highly invasive, often with significant lymphatic and distant metastasis. The establishment of this diagnosis is difficult on both clinical and histopathologic grounds. After diagnosis, wide surgical excision is warranted to completely extirpate these lesions. We report the case of a 66-year-old female with a recurrent malignant acrospiroma. This patient's tumor was removed by wide radical resection, including chest wall excision, followed by reconstructive surgery and radiotherapy. After 16 months there is no evidence of local recurrence or distant metastasis.