Siringoma condróide maligno: relato de caso e revisão da literatura / Malignant chondroid syringoma: case report and literature review

O siringoma condróide maligno, também designado tumor misto maligno da pele, é lesão extremamente rara com aproximadamente 40 casos descritos até o presente momento. Trata-se de neoplasia derivada das glândulas sudoríparas que acomete preferencialmente tronco e extremidades distais em pacientes na sexta década e predomina no sexo feminino. Relatamos um caso com os aspectos clínicos e histológicos característicos para familiarizar o patologista com esse diagnóstico, visto que essa neoplasia com freqüência apresenta metástases locais e/ou regionais (cerca de 60 por cento), sobretudo para linfonodos, pulmões e ossos; além disso, possui taxa de mortalidade de aproximadamente 25 por cento após curso evolutivo prolongado.

Malignant chondroid syringoma (malignant mixed tumor of the skin) is an extremely rare tumor. Approximately 40 cases have been described to date. It is a neoplasm originated from sweat gland. The tumor predominantly affects the trunk and distal extremities, arises in the sixth decade and shows a predilection for females. We relate one case with clinical and histopathologic typical features to alert the pathologist to this diagnosis, since this neoplasm has a metastasic rate of approximately 60 percent (lymph nodes, lungs and bones are predominantly affected) and a mortality of roughly 25 percent.

A Case of Apocrine Mixed Tumor / 대한피부과학회지

Mixed tumor of the skin, so called chondroid syringoma, is a benign epithelial neoplasm with glandular or ductal differentiation surrounded by myxoid stroma. It is most frequently located on the head and neck and presents as an asymptomatic, firm, subcutaneous nodule. Mixed tumor with follicular, sebaceous and apocrine differentiation is considered as an expression of the common embryologic origin of elements of the folliculo-sebaceous-apocrine unit. We report a case of mixed tumor of the skin with follicular, sebaceous and apocrine differentiation.

Apocrine Mixed Tumor with Follicular Differentiation

Mixed tumor of the skin (chondroid syringoma) is a rare benign tumor composed of epithelial elements intermingled with myxoid or cartilagenous stroma which is not separated by basement membrane. It had been believed to originate from the eccrine gland but recently, it was described to be of apocrine gland origin in case of showing apparent apocrine secretion. We report on a 63-year-old man with a tumor on the right upper eyelid showing typical microscopic features of mixed tumor of the skin. Many apocrine decapitation secretions were seen in tubular structures and follicular differentiations were also seen, which represents the common origin of folliculo-sebaceous-apocrine unit.

Mixed Tumor of the Skin: Clinicopathological Study of Seven Cases

BACKGROUND: Mixed tumor of the skin or chondroid syringoma is a benign neoplasm characterized by histological features of a mixture of epithelial and mesenchymal components. It is a rare and benign appendageal tumor, found mostly on the head and neck, and present as an asymptomatic, firm, subcutaneous nodule. There have only been a few reported cases in Korea. OBJECTIVES: This study was aimed to characterize the clinical and histopathlological features of mixed tumor of the skin. METHODS: We reviewed the clinical data and histologic slides of seven patients who have been diagnosed with mixed tumor of the skin by histopathological examination. RESULTS: There were six male patients and only one female. Age of onset of mixed tumor of the skin varied from 26 to 65 years. A11 patients had the lesion on the head: perioral area (3 cases), nose (2 cases), cheek (1 case), and temple area (1 case). Each tumor was a solitary, asymptomatic, and firm, about 0.5-1.5 cm sized, subcutaneous nodule. Histopathologically, all 7 cases presented apocrine differentiation. Two cases showed follicular differentiation, and 2 cases showed sebaceous differentiation. Every tumor showed myxoid stroma except two with typical chondroid matrix. Adipose metaplasia of the matrix was present in 2 cases. In 3 cases, the so-called hyaline cells were rich in the stroma. CONCLUSION: Mixed tumor of the skin was most commonly seen as an asymptomatic, firm subcutaneous nodule on the head. Tumors showing apocrine differentiation were more common than that of eccrine differentiation, All 7 cases presented apocrine differentiation. Follicular and sebaceous differentiation might occur in apocrine type of mixed tumors of the skin. The stroma of mixed tumor of the skin might be myxoid, chondroid, or adipose.