Serac G, Maubec E, Laissy J-P, Marinho E, Abgrall S, Avril M-F, Le Cesne A, Crickx B
Service de dermatologie, hôpital Bichat, université Paris-VII-Denis-Diderot, 46, rue Henri-Huchard, 75877 Paris, France.
Ann Dermatol Venereol. 2008 Jun-Jul;135(6-7):499-502. doi: 10.1016/j.annder.2007.11.033. Epub 2008 Jun 2.
Extraskeletal osteosarcoma is a rare mesenchymatous tumour occurring in adults aged over 50 years and is located mainly in the limbs or retroperitoneum. We report a case of metastatic extraskeletal osteosarcoma revealed by a cutaneous occipital tumour site.
CASE-REPORT: A 53-year-old woman was admitted for dyspnea and weight loss. An occipital tumour, noticed for one year by the patient, was discovered. It was freely movable on the bone, of hard consistency and responsible for alopecia. In addition to left-sided pleural effusion, a chest CAT revealed a large mass in the left lung, including areas of necrosis and calcifications with intracardiac extension. Histological examination of biopsies of the skin and of pulmonary and intracardiac lumps showed an osteosarcomatous proliferation. No primary osteosarcoma was found in the bones. A diagnosis was made of metastatic extraskeletal osteosarcoma. Intravenous chemotherapy was given followed by radiotherapy. After a six-month stabilization period, the disease progressed.
Extraskeletal cutaneous locations of osteosarcoma are extremely rare. They may comprise either the primary tumour or a metastatic lesion. In this patient, the immediately metastatic nature of the disease was a poor prognostic factor for this high-grade sarcoma.
骨外骨肉瘤是一种罕见的间叶组织肿瘤，发生于50岁以上的成年人，主要位于四肢或腹膜后。我们报告一例以枕部皮肤肿瘤为表现的转移性骨外骨肉瘤病例。
一名53岁女性因呼吸困难和体重减轻入院。发现患者已注意到一年的枕部肿瘤。该肿瘤在骨上可自由移动，质地硬，导致脱发。除左侧胸腔积液外，胸部计算机断层扫描显示左肺有一个大肿块，包括坏死和钙化区域，并向心内延伸。对皮肤、肺部和心内肿块活检的组织学检查显示为骨肉瘤增殖。在骨骼中未发现原发性骨肉瘤。诊断为转移性骨外骨肉瘤。给予静脉化疗，随后进行放疗。经过六个月的稳定期后，疾病进展。
骨肉瘤的骨外皮肤部位极为罕见。它们可能是原发性肿瘤或转移性病变。在该患者中，疾病的直接转移性是这种高级别肉瘤的不良预后因素。
