Malignant Chondroid Syringoma with Bone Invasion: A Case Report and Review of the Literature

Abstract

We describe a 27-year-old Japanese female with a recurrent nodule on the left big toe and local bone invasion. Histopathologically, the tumor consisted of nests of atypical cells with few mitotic cells, which partly formed gland-like structures. Areas of myxoid degeneration, positive for Alcian blue staining and that did not stain after they were digested with hyaluronidase, were prominent in the matrix among tumor cells. Positive staining was noted in tumor cells for cytokeratin (AE1+AE3), S-100 protein, neuron specific enolase (NSE), and glial fibrillary acidic protein (GFAP). These findings, especially positive GFAP staining were characteristic and very helpful for the diagnosis of the rare tumor-malignant chondroid syringoma. Based on the previous reports, 39% of cases were found to have metastatic lesions and 22% died of this malignant tumor. There have been no reports reporting effectiveness of chemotherapy and radiotherapy, and an early wide excision with a broad margin may be the most reliable treatment to date.

Cytologic Features of Chondroid Syringoma in Fine Needle Aspiration Biopsies

“…Wide local excision with or without radiotherapy appears to be the treatment of choice in malignant chondroid syringoma. 19,20 Chondroid syringomas, although rarely aspirated, have characteristic cytologic criteria identical to pleomorphic adenoma that allow a definitive cytologic diagnosis on FNA. …”

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.…”

“…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.…”

Siringoma condroide maligno: a propósito de un caso

“…El siringoma condroide maligno tiene un curso clí-nico impredecible, que puede cursar curación, recurrencias locales (hasta el 50% de los casos según las series) (6), metástasis locales y a distancia (39% y 36% de los casos respectivamente) (6). Las metástasis a distancia suelen localizarse en los ganglios linfáti-cos, hueso y órganos (pulmón, cerebro…) (15,16) y son más frecuentes en mujeres.…”

“…El tumor cutáneo mixto benigno es relativamente frecuente, sin embargo, el siringoma condroide maligno es extremadamente raro (6). Es más común en mujeres (mujer: hombre 3:2), su localización es más frecuente en tronco y extremidades que en cabeza y cuello; frecuentemente es de mayor tamaño que la forma benigna (7), se presenta como un nódulo firme, subcutáneo, de crecimiento lento, único simple o múltiple, sin predilección por una determinada edad (8).…”