Patel S R, Burgess M A, Papadopoulos N E, Linke K A, Benjamin R S
Department of Melanoma/Sarcoma Medical Oncology, University of Texas M.D. Anderson Cancer Center, Houston.
Am J Clin Oncol. 1995 Apr;18(2):161-3. doi: 10.1097/00000421-199504000-00014.
Extraskeletal myxoid chondrosarcoma (EMC) is a rare low-grade soft tissue sarcoma that has been reported to have an indolent nature history, and relatively good prognosis. The majority of primary tumors are located in the extremities and they tend to be bulky at presentation. Studies with long-term follow-up have revealed the development of distant metastases in virtually all patients, eventually resulting in death. We reviewed our experience with EMC over the last three decades. The patient population was identified through a search of the database maintained by the Departments of Patient Studies, Pathology, and Melanoma-Sarcoma Medical Oncology. Eleven patients with histologically confirmed diagnosis of EMC were identified. The median age was 59 (37-81 years), and there were nine males and two females. Nine patients had an extremity location and the remaining two had a chest wall and abdominal wall primary, respectively. The median size of the primary tumor was 10 cm (range: 4-17 cm) in maximum dimension. Ten of the eleven patients received chemotherapy, mainly with doxorubicin- and dacarbazine-based regimens. One patient is currently on beta-interferon. No objective responses were noted, to a median of 4 (2-6) cycles of chemotherapy. Three patients were treated with ifosfamide as a second-line chemotherapy without any benefit. Three patients have expired, two patients are alive with no evidence of disease, and six patients are alive with disease. The median follow-up is 5 years (range: 1.33-17 years) from diagnosis. Although small numbers preclude adequate assessment, there is no evidence of efficacy of standard soft-tissue sarcoma chemotherapy in patients with EMC.
骨外黏液样软骨肉瘤（EMC）是一种罕见的低级别软组织肉瘤，据报道其自然病程进展缓慢，预后相对较好。大多数原发性肿瘤位于四肢，就诊时往往体积较大。长期随访研究显示，几乎所有患者都会发生远处转移，最终导致死亡。我们回顾了过去三十年中我们治疗EMC的经验。通过检索患者研究部、病理科和黑色素瘤-肉瘤医学肿瘤科维护的数据库确定了患者群体。确定了11例经组织学确诊为EMC的患者。中位年龄为59岁（37 - 81岁），其中男性9例，女性2例。9例患者肿瘤位于四肢，其余2例分别以胸壁和腹壁为原发部位。原发性肿瘤的最大径中位大小为10 cm（范围：4 - 17 cm）。11例患者中有10例接受了化疗，主要采用以阿霉素和达卡巴嗪为基础的方案。1例患者目前正在使用β干扰素。化疗中位周期数为4（2 - 6）个周期，未观察到客观缓解。3例患者接受异环磷酰胺作为二线化疗，未获任何益处。3例患者已死亡，2例患者存活且无疾病证据，6例患者存活但有疾病。自诊断起的中位随访时间为5年（范围：1.33 - 17年）。尽管病例数较少无法进行充分评估，但尚无证据表明标准软组织肉瘤化疗对EMC患者有效。
