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腹壁前部孤立性纤维瘤。病例报告及文献复习。

Solitary fibrous tumor of the anterior abdominal wall. A case report and review of the literature.

作者信息

Testa Domenica Carmen, Selvaggi Federico, Andreano Tiziana, Mazzola Lorenzo, Cortellese Roberto

出版信息

Ann Ital Chir. 2019 Jun 13;8:S2239253X19030068.

PMID:31366738
Abstract

BACKGROUND

Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm affecting soft tissues with a not well defined biological behavior. SFT occurs mostly in the pleura and the thorax, while extra-thoracic localization is uncommon and abdominal localization is very rare. Histologically, SFT is a well defined mass with splindle-cell proliferation in collagenous matrix with staghorn vascular network and CD34 reactive.

CASE REPORT

A 64 years-old man with a history of recurrent gastric cancer previously treated with total gastrectomy, was admitted with contrast enhanced CT-scan diagnosis of a well demarcated oval mass of 4.8 cm with microcysts, vascularized in the arterial phase and with wash out in the tardive phase, located in the peritoneal side of right rectus abdominis muscle, suspected for metastatic gastric tumor. The patient underwent minilaparotomy and en-bloc excision of the lesion. Histologically the tumor was characterized by a hemangiopericitoma like growth pattern and the immunostaining was positive to CD34, CD99, BCL-2 and Vimentin. The definitive diagnosis was SFT with a proliferation index (Ki-67/MIB-1) <3%. In our case, chemotherapy was not indicated. At the 6-month follow-up, the patient is in good clinical conditions with no recurrence or metastasis.

CONCLUSIONS

We reported a rare case of primitive SFT located in peritoneal side of the of right rectus abdominis muscle treated surgically, in a patient previously affected by gastric adenocarcinoma. In this case, SFT showed a benign behaviour during a short term follow-up. Dimensional pattern, histopathological features and curative surgery remain the most important indicators of clinical outcome.

KEY WORDS

Abdominal wall, Hemangiopericitoma, SFT, Solitary fibrous tumorSpindle cell.

摘要

背景

孤立性纤维性肿瘤(SFT)是一种罕见的间叶性肿瘤,可累及软组织,其生物学行为尚不明确。SFT大多发生于胸膜和胸部,而胸外定位不常见,腹部定位则极为罕见。组织学上,SFT是一种边界清晰的肿块,在胶原基质中有梭形细胞增生,伴有鹿角状血管网且CD34呈阳性反应。

病例报告

一名64岁男性,有复发性胃癌病史,此前接受过全胃切除术,因增强CT扫描诊断为右腹直肌腹膜侧有一个边界清晰的椭圆形肿块,大小为4.8 cm,有微囊肿,动脉期血管化,延迟期有廓清,怀疑为转移性胃肿瘤而入院。患者接受了小切口剖腹术并整块切除病变。组织学上,肿瘤表现为血管外皮细胞瘤样生长模式,免疫组化对CD34、CD99、BCL-2和波形蛋白呈阳性。最终诊断为增殖指数(Ki-67/MIB-1)<3%的SFT。在我们的病例中,未指示进行化疗。在6个月的随访中,患者临床状况良好,无复发或转移。

结论

我们报告了一例罕见的原发性SFT,位于右腹直肌腹膜侧,对一名先前患有胃腺癌的患者进行了手术治疗。在该病例中,SFT在短期随访中表现出良性行为。大小模式、组织病理学特征和根治性手术仍然是临床结果的最重要指标。

关键词

腹壁;血管外皮细胞瘤;SFT;孤立性纤维性肿瘤;梭形细胞

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