Reis-Filho Jorge S, Paiva Maria E, Lopes José M
Institute of Molecular Pathology and Immunology, University of Porto (IPATIMUP), Portugal.
Brain Pathol. 2004 Jan;14(1):111-2, 115. doi: 10.1111/j.1750-3639.2004.tb00505.x.
The August 2003 COM. A 47-year-old woman presented with a long history of muscle pain, weakness, and visual disturbances. Over the last year, she developed diplopia and left sixth nerve palsy. No other neuro-ophthalmologic abnormalities were found. Past medical and family history was unremarkable. Laboratory investigation disclosed hypophosphatemia, phosphaturia, elevated serum alkaline phosphatase activity, and normal serum calcium levels. CT scans showed a lobulated mass arising on the meningeal surface of the cavernous sinus, measuring 3x 2 x 2 cm. The lesion was partially resected and microscopic examination revealed a highly vascularized tumor composed of primitive mesenchymal cells arranged whether in a patternless-pattern or surrounding thin-walled, branching vascular spaces and thick-walled hyalinized vessels. Other eye-catching features were microcystic areas, multinucleated osteoclastic-like giant cells, scattered islands of mature adipocytes, foci of hemorrhage, thrombosed medium-sized-to-large vessels, and hemosiderin-laden macrophages. After surgery, the patient recovered from the muscle pain and weakness, with a significant improvement of serum calcium and alkaline phosphatase levels and phosphate blood levels. Taken together, the clinical and pathologic features were consistent with a diagnosis of phosphaturic mesenchymal tumor (mixed connective tissue variant) of the cavernous sinus in a setting of oncogenic osteomalacia. No further treatment was carried out. The patient has been followed for the last 4 years with no evidence of recurrent disease. Oncogenic osteomalacia is a rare clinical entity characterized by hypophosphatemia, phosphaturia, and a low concentration of 1,25-dihydroxyvitamin D(3) caused by a neoplasm. Pathologists should be aware of this entity, because surgical excision of the tumor is usually curative.
2003年8月病例。一名47岁女性，有长期肌肉疼痛、无力及视觉障碍病史。在过去一年里，她出现了复视和左侧第六脑神经麻痹。未发现其他神经眼科异常。既往病史和家族史无异常。实验室检查发现低磷血症、磷酸盐尿、血清碱性磷酸酶活性升高，血清钙水平正常。CT扫描显示在海绵窦脑膜表面有一个分叶状肿块，大小为3×2×2厘米。病变部分切除，显微镜检查显示为高度血管化肿瘤，由原始间充质细胞组成，这些细胞呈无规律排列或围绕薄壁分支血管腔隙和厚壁玻璃样变血管。其他显著特征包括微囊区、多核破骨细胞样巨细胞、散在的成熟脂肪细胞岛、出血灶、中到大血管血栓形成以及含铁血黄素巨噬细胞。手术后，患者的肌肉疼痛和无力症状缓解，血清钙、碱性磷酸酶水平及血磷水平显著改善。综合来看，临床和病理特征符合海绵窦磷酸尿性间叶肿瘤（混合结缔组织型）伴肿瘤性骨软化的诊断。未进行进一步治疗。患者在过去4年一直接受随访，无疾病复发迹象。肿瘤性骨软化是一种罕见的临床病症，其特征为低磷血症、磷酸盐尿以及由肿瘤导致的1,25 - 二羟维生素D(3)浓度降低。病理学家应了解这种病症，因为肿瘤的手术切除通常可治愈。
