ABSTRACT
Chondroid syringomas are similar to mixed parotid tumors and present in both benign and malignant forms. Malignant chondroid syringoma (MCS) is a rare skin tumor that has a predilection for extremities, particularly in young women. It is even rarer to present as a scalp tumor with very few reported cases in the literature. We present a middle-aged woman, with a history of increasing fatigability of her right arm for the past 3 months who, on examination, was found to have scalp swelling and matted right posterior triangle lymph nodes. The working diagnosis on her was a large sebaceous cyst with secondary in the neck from an occult primary/non-Hodgkin抯 lymphoma. Preliminary fine-needle aspiration was inconclusive. Imaging followed by wide excision of the tumor and the nearby occipital node was done. The final histopathological diagnosis was MCS with secondary in the lymph node. These tumors are aggressive and metastasize early. Radical surgery is the only hope of cure as adjuvant treatment is yet to be standardized
ABSTRACT
Introducción: El siringoma condroide maligno es un tumor cutáneo muy infrecuente pero que debemos tener en cuenta en el diagnóstico diferencial de los tumores axilares. Objetivos: Se realiza el análisis y la presentación del siguiente caso clínico con el objetivo de considerar esta entidad como diagnostico diferencial de los tumores axilares.Presentación de Caso: Paciente de sexo femenino de 82 años que refiere la presencia de una tumoración cutánea en axila derecha de 3 años de evolución, con realización de biopsia (PUNCH) que informa infiltración por carcinoma moderadamente diferenciado. Se decide su exéresis quirúrgica.Discusión: El siringoma condroide maligno es un tumor infrecuente, pero puede dar metástasis, pero con prolongados tiempos de sobrevida. Prefiere las extremidades y el tronco. Su pronóstico es bueno cuando la exéresis es completa, aunque es necesario realizar un seguimiento completo de los pacientes.
Introduction: Malignant chondroid syringoma is a very rare skintumor but must be considered in the differential diagnosis of axillary tumors. Objectives: We performed the analysis and presentation of thefollowing clinical case in order to consider this entity in the differential diagnosis of axillaries tumors. Case Presentation:Female 82 years old patient referring the presence of a skin tumor in the right axilla for three years standing. Biopsy (PUNCH) reports moderately differentiated carcinoma infiltration. S urgical excision is decided. Discussion: Malignant chondroid syringoma is a rare tumor, but can metastasize, with prolonged survival times. It prefers limbs and trunk. Its prognosisis good when the removal is complete, but anyway it is necessary to follow up patients.
Subject(s)
Humans , Female , Aged, 80 and over , Adenoma, Pleomorphic/diagnosis , Adenoma, Pleomorphic/pathology , Adenoma, Pleomorphic/therapyABSTRACT
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.
Subject(s)
Humans , Male , Middle Aged , Adenoma, Pleomorphic/diagnosis , Adenoma, Pleomorphic/pathology , Rare Diseases/diagnosis , Sweat Gland Neoplasms/pathology , ImmunohistochemistryABSTRACT
Malignant chondroid syringoma, previously called "mixed tumor of the skin of the salivary gland type"is a fairly uncommon type of sweat gland tumor. Malignant chondroid syringoma frequently arises from the trunk and extremities, whereas the benign tumor is common to the head region. The present case occurred in a female. The malignant nature of the tumor was evident from repeated recurrences after excision of the mass and histopathological study. Lack of response to radiotherapy and chemotherapy led to widespread metastasis. We report a case of malignant chondroid syringoma with lung metastasis in a 39-year-old female patient and response to chemotherapy. We also reviewed the literatures of malignant chondroid syringoma.