Malignant Chondroid Syringoma With Central Nervous System Involvement

Abstract

Malignant chondroid syringoma is a mixed cutaneous tumor, with epithelial and mesenchymal components, which compromises principally the trunk and extremities. This lesion is quite rare, with few cases related in the literature and no publications demonstrating its involvement of the central nervous system. Histologically, owing to its mixed origin, it represents a lesion that is difficult to recognize, often being confused with basocellular carcinoma. We report the case of a female patient, carrier of malignant chondroid syringoma in the occipital region, with invasion of the central nervous system, who was submitted to surgical excision of the lesion at our service. We also made a brief revision of the literature on the theme.

Benign chondroid syringoma (mixed tumor of skin) on the flank with satellites: case report and literature review

“…Their survival period was from 6 months to 7 years (mean: 4.9 years). The most common site for distance metastasis was lung, followed by bone and brain [5,8,14,16]. Nine of the 49 (18 %) patients died from their disease.…”

“…Although most mixed tumors of skin are benign, malignant forms have been reported. Unlike its benign counterpart, the malignant form occurs predominantly in females [5][6][7][8][9][10][11][12][13][14][15].…”

Surgical treatment and dilemmas in the treatment of basal cell carcinomas with intracranial propagation

“…Despite the fact that basal cell carcinomas are affected by radiation therapy (except for the morpheaform type), cryotherapy, curettage, local cytostatic therapy, retinoids or electrodisection, surgical treatment is the method of choice in the treatment of this type of tumor [5][6][7][8][9][10] . Surgically, the tumor can be entirely removed with safe histopathological checkup and confirmation 11,12 .…”

Spinal cord compression secondary to metastasis of malignant chondroid syringoma: case report

“…18 Complementing surgical treatment with radiotherapy for this entity is still controversial. 2,6,16 Although local radiotherapy is often unsuccessful, skeletal metastases have shown a response to radiotherapy. If we take into account all malignancies, Level I evidence suggests that surgery followed by radiotherapy remains the treatment of choice for selected patients presenting with spinal cord compression caused by a metastatic lesion.…”

“…15 Although most reported cases of chondroid syringoma have been benign, several cases of malignancy have been reported. [1][2][3][7][8][9]11,14,17,18,[20][21][22] Malignant chondroid syringoma is the least common variety of sweat gland carcinoma. 10,22 In contrast to its benign counterpart, the malignant form occurs predominantly in women of any age and is observed more commonly in the trunk and extremities.…”