原发性骨内孤立性纤维性肿瘤:一例极为罕见的病例报告及文献回顾。

IF 4.4 Q1 PATHOLOGY PATHOLOGICA Pub Date : 2022-10-01 DOI:10.32074/1591-951X-524
Giulia Coppola, Carmine Zoccali, Jacopo Baldi, Alessio Annovazzi, Thedora Daralioti, Mariavittoria Vescovo, Renato Covello
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引用次数: 0

摘要

孤立性纤维性肿瘤(SFT)是一种罕见的纤维母细胞间质肿瘤,最初发现于纵隔胸膜,后来在许多胸膜外部位也有报道。原发性骨孤立性纤维性肿瘤的报道极为罕见,文献中只报道过少数病例,且多发生在扁平骨和短骨。在这里,我们提出的情况下,53岁的女性,谁被转介到急诊室的外围医院意外跌倒后。影像学检查显示转子间骨折伴潜在髓内溶解性病变。活检后初步诊断为尤文氏肉瘤。她来到我们医院,我们重新评估了这个病例。我们回顾了活检并提出了骨内SFT的诊断。她接受了右股骨近端整块切除。像我们的病例一样,由于影像学研究的低特异性和定位的极端罕见,原发性骨SFTs很容易被误诊。准确的诊断和早期切除是非常重要的,仔细的长期随访是必不可少的,特别是对于那些有恶性行为的患者,早期发现可能的复发或转移。
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Primary intraosseous solitary fibrous tumor: an extremely rare case report and brief review of the literature.

Solitary fibrous tumor (SFT), a rare mesenchymal neoplasm of fibroblastic origin, was initially discovered in the mediastinal pleura and then described in many extra-pleural sites.

The reports of primary solitary fibrous tumor of bone are extremely rare and only a few cases have been previously mentioned in the literature, most of which in flat and short bones.

Here we present the case of a 53-year-old female, who was referred to the emergency department of a peripheral hospital after an accidental fall. Imaging studies revealed an intertrochanteric fracture with an underlying intramedullary lytic lesion. A biopsy was performed and a diagnosis of Ewing sarcoma was initially suggested. She arrived at our hospital where we reevaluated the case. The biopsy was reviewed and a diagnosis of intraosseous SFT was proposed. She underwent en-block resection of the proximal right femur.

Primary SFTs of the bone are, like in our case, easily misdiagnosed due to the low specificity of the imaging studies and the extreme rarity of the localization. An accurate diagnosis and early resection are very important and with careful long-term follow-up is essential, particularly in those who with malignant behavior, for the early detection of possible recurrence or metastasis.

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来源期刊
PATHOLOGICA
PATHOLOGICA PATHOLOGY-
CiteScore
5.90
自引率
5.70%
发文量
108
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