Chirurgie maxillo-faciale, stomatologie, chirurgie orale et chirurgie plastique de la face, hospices civils de Lyon-université Claude-Bernard-Lyon 1, centre hospitalier Lyon-Sud, 165, chemin du Grand-Revoyet, 69310 Pierre-Bénite, France.
Chirurgie maxillo-faciale, stomatologie, chirurgie orale et chirurgie plastique de la face, hospices civils de Lyon-université Claude-Bernard-Lyon 1, centre hospitalier Lyon-Sud, 165, chemin du Grand-Revoyet, 69310 Pierre-Bénite, France.
J Stomatol Oral Maxillofac Surg. 2018 Jun;119(3):232-235. doi: 10.1016/j.jormas.2018.02.008. Epub 2018 Feb 22.
Malignant chondroid syringomas, also known as cutaneous malignant mixed tumors, are rare neoplasms that most frequently occur on the torso or extremities of women. Here, we present an illustrated case of a facial malignant chondroid syringoma.
A 32-year-old female patient with no notable medical history presented with an approximately 1cm-wide, painless, palpably-mobile subcutaneous nodule, suggestive of a sebaceous cyst, just above the middle third of the right eyebrow. The nodule had grown steadily over six months. She had no palpable cervical lymphadenopathies.
Anatomic pathology of the enucleated nodule found an adnexal sudoriparous tumor measuring 6×10mm and indicative of a malignant chondroid syringoma. Cervicofacial computed tomography and positron emission tomography scans showed no near or distant lymph node involvement. A second intervention for wide excision around the original enucleation lesion (+1cm) was validated in a multidisciplinary, cancerology-dermatology consultation. The eyebrow was reconstructed with a temporally-harvested fasciocutaneous island flap.
Malignant chondroid syringomas are very rare and thus no standardized treatment has been established for them. Only 12 craniofacial localizations have been described to date. Radiation therapy and chemotherapy have not been shown effective for this malignancy, leaving only wide excision as a therapeutic option. A high and sustained (as much as 20 years after the initial diagnosis) risk of recurrence or metastasis necessitates prolonged patient follow-up.
恶性软骨样汗管瘤，也称为皮肤恶性混合瘤，是一种罕见的肿瘤，最常发生在女性的躯干或四肢。在这里，我们介绍了一个面部恶性软骨样汗管瘤的病例。
一名 32 岁的女性患者，无明显病史，于右眉中三分之一上方出现一个约 1cm 宽、无痛、可触及的皮下结节，提示为皮脂囊肿。该结节在六个月内稳定生长。她没有可触及的颈部淋巴结病。
切除的结节的解剖病理学发现一个附属的汗腺肿瘤，大小为 6×10mm，提示为恶性软骨样汗管瘤。头颈部计算机断层扫描和正电子发射断层扫描显示无邻近或远处淋巴结受累。在多学科癌症学-皮肤科咨询中，对原始切除病变（+1cm）周围进行广泛切除的第二次干预得到了验证。眉毛用颞部筋膜皮瓣重建。
恶性软骨样汗管瘤非常罕见，因此尚未为其制定标准化的治疗方案。迄今为止，仅描述了 12 个颅面定位。放射治疗和化疗对这种恶性肿瘤无效，仅广泛切除是一种治疗选择。高且持续（甚至在初始诊断后 20 年）的复发或转移风险需要对患者进行长期随访。
