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胫骨神经滑膜肉瘤——罕见部位罕见肿瘤的病例报告,需要早期诊断。

Synovial sarcoma of the tibial nerve - case report of a rare tumor in a rare location requiring early diagnosis.

机构信息

Department of Neurology, University Hospital Hradec Králové, Hradec Králové, Czech Republic.

The Fingerland department of Pathology, University Hospital Hradec Králové, Hradec Králové, Czech Republic.

出版信息

BMC Neurol. 2023 Feb 10;23(1):65. doi: 10.1186/s12883-023-03061-5.

Abstract

BACKGROUND

We present the case of a patient with a rare synovial sarcoma (SS) of the tibial nerve. So far, only 4 cases of patients with SS originating from the tibial nerve have been described in the literature, and our patient is only the second patient whose limb was saved during treatment. Synovial sarcomas are malignant mesenchymal tumors, i.e., tumors arising from connective tissue. Synovial sarcomas account for 5-10% of all soft tissue sarcomas. However, the name synovial sarcoma is misleading, because the tumor does not originate from synovial cells, but rather from primitive mesenchymal cells. The name most likely originated from the localization around the large joints on the limbs, more often on the lower ones, in the area of the knee joints. We point out the aspects of correct and quick diagnosis and subsequent treatment, which has very important effect on the patient's prognosis. Primary less radical excision without prior biopsy verification leads to a higher risk of local recurrence, even if a proper reexcision was performed immediately after biopsy verification of the sarcoma.

CASE PRESENTATION

A woman born in 1949 began to suffer at the end of 2020 with escalating pain under the left inner ankle with a projection to the sole and fingers. Her personal, family work and social history were insignificant. After the initial neurological examination, the patient was sent for an ultrasound examination of the ankle, which showed a lobular mass measuring 50 × 22 × 16 mm and according magnetic resonance imaging, the finding appeared to be a suspicious neurinoma of the tibial nerve. The tumor was surgically excised, without prior biopsy verification: a 50 × 20 mm tumor was dissected in the distal part of the tarsal canal, which grew through the structure of the tibial nerve and in some places into the surrounding area and appeared intraoperatively as a neurofibroma. But histologically the tumor was classified as monophasic synovial sarcoma. The patient was indicated for a wide reexcision of the skin with the subcutaneous tissue of size 91 × 20 × 15 mm. Now the patient is being treated with external radiotherapy to the tumor bed and she is able to walk.

CONCLUSION

This report draws attention to a rare type of malignant nerve tumor, which both clinically and radiologically can mimic benign peripheral nerve sheath tumors. Synovial sarcoma should be considered in very painful resistances, typically located around the joints of the lower limbs, the growth of which can be slow. Because the size of the tumor is a negative prognostic factor, it is necessary to make a timely diagnosis using MR imaging and a biopsy with histological examination and to start treatment quickly. Surgical treatment should take place only after a biopsy with histological examination of the tumor so that it is sufficiently radical and does not have to undergo an additional reoperation, as happened in the case of our patient.

摘要

背景

我们报告了一例罕见的胫骨神经滑膜肉瘤(SS)病例。迄今为止,文献中仅描述了 4 例起源于胫骨神经的 SS 患者,而我们的患者是治疗过程中唯一肢体得以保留的第二位患者。滑膜肉瘤是一种恶性间叶组织肿瘤,即源自结缔组织的肿瘤。滑膜肉瘤占所有软组织肉瘤的 5-10%。然而,滑膜肉瘤这个名称具有误导性,因为肿瘤并非起源于滑膜细胞,而是起源于原始间充质细胞。这个名称可能源于四肢大关节附近的定位,更常见于下肢,膝关节区域。我们指出了正确和快速诊断以及随后治疗的重要方面,这对患者的预后有非常重要的影响。未经活检证实的初步非根治性切除会导致局部复发风险增加,即使在肉瘤活检后立即进行适当的再次切除。

病例介绍

一位 1949 年出生的女性,于 2020 年底开始出现左内踝下疼痛加剧,向足底和脚趾放射。她的个人、家庭、工作和社会史无足轻重。在进行初始神经学检查后,患者接受了踝关节超声检查,显示出一个 50×22×16mm 的分叶状肿块,根据磁共振成像,该发现似乎是胫骨神经可疑神经瘤。肿瘤被手术切除,未经活检证实:在跗管远端切除了一个 50×20mm 的肿瘤,该肿瘤穿过了胫骨神经的结构,在某些地方进入了周围区域,术中表现为神经纤维瘤。但组织学上,肿瘤被分类为单相滑膜肉瘤。患者被指示进行广泛的皮肤和皮下组织切除,大小为 91×20×15mm。现在患者正在接受肿瘤床外放射治疗,她能够行走。

结论

本报告提请注意一种罕见的恶性神经肿瘤,其在临床和影像学上均可模拟良性周围神经鞘肿瘤。在位于下肢关节周围、生长缓慢且非常疼痛、抵抗治疗的肿瘤中,应考虑滑膜肉瘤。由于肿瘤大小是一个负预后因素,因此需要使用磁共振成像和组织学检查进行活检,以便及时诊断,并迅速开始治疗。只有在对肿瘤进行组织学活检后才能进行手术治疗,以便手术足够彻底,无需进行额外的再次手术,就像我们的患者那样。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a88d/9912578/f98ede28ea05/12883_2023_3061_Fig1_HTML.jpg

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