Seeding of Abdomen with Primary Intracranial Hemangiopericytoma by Ventriculoperitoneal Shunt: Case Report

Background

Ventriculoperitoneal shunt (VPS) placement has been implicated in extraneural metastasis of many primary central nervous system tumors. Reported cases include, but are not limited to, medulloblastoma, germ cell tumor, astrocytoma, oligodendroglioma, lymphoma, ependymoma, melanoma, and choroid plexus tumors. However, a literature review reveals no reported cases of extraneural metastasis of solitary fibrous tumor/hemangiopericytoma (SFT/HPC).

Case Description

Here we report the case of a 34-year-old man with recurrent intracranial malignant SFT/HPC who had undergone surgical tumor resection and subsequent placement of a VPS for obstructive hydrocephalus in 2004. Subsequently, the patient presented in 2011 and again in 2013 with abdominal SFT/HPC metastasis likely caused by the presence of the VPS.

Conclusion

The case raises concern regarding placement of a VPS in patients with obstructive hydrocephalus caused by SFT/HPC. To avoid spread of SFT/HPC to the abdomen, we propose that patients with intracranial SFT/HPC and obstructive hydrocephalus be treated primarily by endoscopic third ventriculostomy.

Introduction

Hemangiopericytoma (HPC) is a rare malignancy known to occur in the musculoskeletal system while less frequently affecting the central nervous system (CNS).¹ HPC arises from fibroblasts with pathologic features resembling those of solitary fibrous tumors (SFT).2, 3, 4, 5 Previously, in the World Health Organization (WHO) classification of CNS tumors, meningeal HPCs were classified as distinct entities from SFTs.2, 6 Meningeal SFT was considered a benign WHO I tumor, whereas an intracranial HPC was classified as a malignant (WHO II or III) tumor, likely requiring adjuvant treatment, typically radiotherapy.1, 2 More recently, the 2016 WHO classification system groups intracranial HPCs with SFTs, using the terminology SFT/HPC.⁵

Meanwhile, radiographically, SFT/HPC can resemble meningioma.¹ It can be of primary intracranial origin in both adult and pediatric populations, with average age of presentation ranging from 38 to 42 years.1, 7, 8 Surgical resection is standard treatment.¹ Some SFT/HPC patients suffer from obstructive or postsurgical hydrocephalus requiring cerebral spinal fluid diversion, and there is known delayed metastasis to many organ systems.⁷

It has been reported that neurosurgical procedures including ventriculoperitoneal shunt (VPS) placement are implicated in extraneural metastasis of primary intracranial tumors.⁹ As such, the authors performed a U.S. National Library of Medicine PubMed search seeking publications regarding abdominal metastases in the setting of a VPS. The goal was to further identify trends in tumor pathology, extent of resection, timing of VPS placement in relation to development of abdominal metastases, and overall outcome. The search was limited to human studies and reports with 1 or more patients.

Reported cases have included medulloblastoma, germ cell tumor, astrocytoma, oligodendroglioma, lymphoma, ependymoma, melanoma, and choroid plexus tumors, with both pediatric and adult populations affected.10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26 However, to our knowledge, there has been no previously reported case of extraneural metastasis of HPC in the setting of a VPS.