Background

Synovial sarcoma is a soft tissue sarcoma, of uncertain histological origin, usually located near large joints and concerning mainly young adults. Intracranial presentation in the form of metastasis from a primitive body sarcoma has been rarely reported. However, intracranial primitive synovial sarcoma (IPSS) is extremely rare and only a few cases have been reported in the literature.

Case description

We present the case of a 48-year-old man, with no particular medical history, that was referred to our hospital for severe headache with a normal neurological exam and a CT cerebral scan showing a left frontal lobe hematoma. The initial cerebral CT scan didn’t show any vascular malformation and the body CT scan was negative for a primitive lesion. A close follow-up with a cerebral MRI three months later, demonstrated a T1 enhanced lesion with an important volume progression. The patient underwent a complete surgical removal of this lesion and the first pathology diagnosis was compatible with a meningioma. After further proofreading by an expert and molecular analysis, the diagnosis of monophasic synovial sarcoma was confirmed. Nine months after the first surgery, the follow-up MRI showed the progressive recurrence of the lesion and in this context the patient underwent a second surgery with total resection of the tumor and frontal thin margin excision. Afterwards, the patient was treated with adjuvant radiotherapy, with a good clinical evolution, and till now the follow-up shows no recurrence.

Conclusion

IPSS is an extremely rare sarcoma, with challenging diagnosis and difficult management. Specific molecular analysis is necessary. Complete resection followed by radiotherapy seem to be the most appropriate therapeutic approach. However, the prognosis is still poor. Our case is even rarer because of the initial presentation as a cerebral hematoma.

中文翻译：

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类似自发性脑血肿的颅内原发性滑膜肉瘤一例报告及文献复习

背景

滑膜肉瘤是一种软组织肉瘤，组织学起源不确定，通常位于大关节附近，主要涉及年轻人。很少报道原始身体肉瘤以转移形式出现的颅内表现。然而，颅内原始滑膜肉瘤（IPSS）极为罕见，文献中仅报道了少数病例。

案例说明

我们介绍了一名 48 岁男性的病例，没有特别的病史，他因严重头痛被转诊到我们医院，神经系统检查正常，CT 脑扫描显示左额叶血肿。最初的脑 CT 扫描未显示任何血管畸形，全身 CT 扫描对原始病变呈阴性。三个月后对脑部 MRI 进行了密切随访，结果显示 T1 增强的病灶具有重要的体积进展。该患者接受了该病灶的完全手术切除，第一次病理诊断与脑膜瘤相符。经专家进一步校对和分子分析，确诊为单相滑膜肉瘤。第一次手术九个月后，随访 MRI 显示病变进行性复发，在这种情况下，患者接受了第二次手术，全切除肿瘤和额叶薄缘切除术。随后，患者接受了辅助放疗，临床进展良好，至今随访未见复发。

结论

IPSS 是一种极为罕见的肉瘤，诊断难度大，治疗困难。特定的分子分析是必要的。完全切除后放疗似乎是最合适的治疗方法。然而，预后仍然很差。我们的病例更罕见，因为最初表现为脑血肿。