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A Case Report of Rare Sacral Solitary Fibrous Tumor

By Admin | July 31, 2022


Abstract

Huge primary epidural solitary fibrous tumors in the sacrum are a rare clinical entity. The purpose of this article is to present our experience in treating such large and complex neoplasms in a 31-year-old woman. The patient complained of constant nocturnal bilateral hip and lower back pain and unilateral radicular symptoms (numbness, paresthesias) in the left S1/S2 dermatomal distribution. Diagnostic imaging, biopsy, preoperative endovascular embolization, two-staged tumor resection, and lumbosacroiliac fusion were performed. The treatment resolved the patient’s neurological symptoms and resulted in overall good postoperative functionality. The patient has been in remission for more than five years despite her refusal of adjuvant radiotherapy.

Introduction

Solitary fibrous tumor (SFT), also previously known as hemangiopericytoma, is a rare mesenchymal neoplasm of fibroblastic origin, accounting for less than 2% of all soft tissue tumors [1]. The 2013 edition of World Health Organization (WHO) Classification of Tumors of Soft Tissue and Bone [2] and the updated 2016 edition, Classification of Tumors of the Central Nervous System, introduced a new combined entity of soft tissue and meningeal hemangiopericytomas and SFTs [3], but WHO in 2021 edited this entity in the Classification of Tumors of the Central Nervous System, leaving only the term of SFT [4]. CNS SFT can be classified/graded into three grades by the presence of necroses and amount of mitoses: CNS WHO grade 1, < 2.5 mitoses/mm2 ([4]. Most commonly SFTs occur in the pleura and less often in extrapleural locations, such as the abdomen, pelvis, and retroperitoneal space. They can seldom be found in soft tissues of extremities, head and neck, and central nervous system. Adults between 50 and 70 years of age with no gender predilection are usually affected [1]. Several large studies in the literature had analyzed and tried to predict the local recurrence and metastasis of SFTs. In a large European multicentric cohort study, local and metastatic recurrence had occurred in 20 (12.3%) and 27 (16.7%) out of 162 patients, respectively. The calculated local recurrence incidence rates at 10 and 20 years were 19.2% and 38.6%, respectively. The metastatic recurrence incidence rates at 10 and 20 years were 31.4% and 49.8%, respectively [5]. Demicco et al. proposed the risk assessment score for predicting the development of metastasis in SFTs, which included the patient age, tumor size, mitotic activity, and the presence of necrosis. According to this study, low-risk lesions had 0% risk of metastasis at 10 years, intermediate-risk cases had 10% risk of metastasis at 10 years, and high-risk cases had 73% risk of metastasis at 5 years [6]. Modified Demicco et al.’s risk stratification system was applied to our patient. This article presents a rare primary epidural SFT at the L5/S3 levels, immunohistochemical results, and treatment strategy.

Case Presentation

A 31-year-old woman presented to the Department of Obstetrics and Gynecology in Vilnius University Hospital Santaros Klinikos for an elective laparoscopical diagnostic procedure due to a suspected ovarian cyst. She also complained of constant nocturnal bilateral hip and lower back pain and unilateral radicular symptoms (numbness, paresthesias) in the left S1/S2 dermatomal distribution that worsened over the last two years. No gynecological abnormalities were found, only a large retroperitoneal mass. Then she was referred to the Department of Neurosurgery in the same hospital where one of the authors gave further plan. Initial differentials involved more common masses in the sacral area, such as chordoma, chondrosarcoma, and giant cell tumor. There was no history of weight or appetite changes. Pelvic magnetic resonance imaging (MRI) was performed, which demonstrated...(More)

For more info please read, A Case Report of Rare Sacral Solitary Fibrous Tumor, by Cureus

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