Distinctive Characteristic Features of Intramedullary Hemangiopericytomas

Abstract

Study DesignThe retrospective analysis of intramedullary hemangiopericytomas (HPCs) was performed, and the entity was discussed in accordance with the literature findings.PurposeThis study aimed at defining distinctive characteristic features of intramedullary HPC with respect to surgical approach and prognosis.Overview of LiteratureIntramedullary HPCs are extremely rare tumors. They originate from capillary pericytes, supposedly follow the vessels over the spinal cord, and infiltrate deep into the spinal cord without a distinct plane. Their treatments and prognosis are not well-defined in the literature.MethodsOur database was retrospectively reviewed for the cases of HPCs. Later on, a literature search was performed to reveal all reported cases of intramedullary HPCs. The following key words were searched in PubMed databases: "hemangiopericytoma and intramedullary," "hemangiopericytoma and spine (spinal) and intradural," and "hemangiopericytoma and spinal cord." The articles were reviewed for patients' demographics features, imaging characteristics, tumor-specific factors (surgical technique, pathological descriptions, and world health organization grades), and postoperative course and prognosis (adjuvant therapies, recurrences, complications, and mortalities).ResultsA total of seven patients (three male and four female) was reached, with their ages ranging from 15 to 80 years (mean, 32.5 years). The tumors were located majorly in thoracic region (5/7, 71.4%), and only two cases were in the cervical region (2/7, 28.6%). All tumors were completely removed, and only two cases received radiotherapy. No recurrence was reported.ConclusionsComplete resection of the intramedullary HPCs seems to be the best management strategy for long-term and recurrence-free survival and in alleviating further need for radiotherapy.

A Rare Case of a Primary Spinal Solitary Fibrous Tumor/Hemangiopericytoma in a 9-Month-Old Patient

“…Before the World Health Organization (WHO)’s revision of SFT/ HPC, some studies attempted to characterize the imaging features of SFT and HPC separately, with conflicting results demonstrating a wide range of tumoral MR signal intensities and enhancement patterns [ 9 ]. Current unified WHO guidelines state that SFT/HPC demonstrate isointensity on T1-weighted MRI and high or mixed signal intensity on T2-weighted MRI.…”

A Rare Case of a Thoracic Spinal Hemangiopericytoma

“…Complete resection of the tumor is the main objective. The extent of tumor resection correlates with a low rate of long-term tumor recurrence [10]. However, the vascular nature of the tumor may be responsible for significant intraoperative bleeding thus making complete excision difficult.…”

Spinal intradural solitary fibrous tumor/ hemangiopericytoma with intramedullary invasion mimicking a hemangioblastoma

“…Total patients [1][2][3][4][5][6][7][8] 34 Age (years) Mean 48.1 Range 15-83 Sex Male 21 Female 13 Original diagnosis [1][2][3][4][5][6][7][8] SFT 23 HPC 11 Location [1][2][3][4][5][6][7][8] Cervical 12 Cervicothoracic 2 oracic 18 Lumber 2 Preoperative diagnosis [1][2][3][4][5][6][7][8]…”

“…We found 34 cases of SFT/HPC cases (i.e., including this case) in our review of case studies from the literature [Supplemental Table 1]. [1][2][3][4][5][6][7][8] Patients averaged 48.1 years of age, and there was a 1.6:1 male-to-female ratio [Table 1]. [1][2][3][4][5][6][7][8] Tumors were typically located in the lower cervical region followed by the upper and midthoracic regions, but were only rarely encountered in the lumbar spine [Figure 6].…”

“…[1][2][3][4][5][6][7][8] Patients averaged 48.1 years of age, and there was a 1.6:1 male-to-female ratio [Table 1]. [1][2][3][4][5][6][7][8] Tumors were typically located in the lower cervical region followed by the upper and midthoracic regions, but were only rarely encountered in the lumbar spine [Figure 6]. T1 MR findings for SFT/HPC were nonspecific.…”