Two-Stage Surgical Management of Intramedullary Holocord Astrocytoma in an Adult: A Case Report and Literature Review

TL;DR

This case report describes a rare spinal tumor in an adult and shows that a two-stage surgery can lead to significant neurological recovery.

Contribution

The report presents a successful surgical approach for a rare adult spinal tumor and contributes new clinical data.

Findings

A two-stage surgical approach enabled safe removal of a holocord astrocytoma with neurological improvement.

Only five adult cases of holocord astrocytoma have been reported, highlighting the rarity of this tumor.

Postoperative recovery included restored sphincter control and improved mobility.

Abstract

Holocord astrocytomas are extremely rare spinal cord tumors, and only a few cases have been reported in adults. Because these tumors may extend along the entire spinal cord, diagnosis and treatment are often challenging. In this report, we describe a young adult who presented with severe neurological symptoms caused by such a tumor. We treated him using a planned two-stage surgical approach, allowing for safe removal of tumor tissue across multiple spinal levels. After surgery, the patient showed marked neurological improvement, regaining mobility and sphincter control. To contextualize this case, we also reviewed all published reports of holocord astrocytomas. Due to the small number of adult cases, clear treatment recommendations are limited. Our findings show that with careful planning, meaningful recovery can be achieved, and this report adds valuable clinical data to a very rare and understudied tumor entity.

Background/Objectives: Holocord astrocytomas are exceptionally rare intramedullary tumors, especially in adults, and often present with extensive longitudinal growth. Because only a small number of cases have been described, management strategies remain insufficiently defined. This report presents an adult patient treated with a staged surgical approach and provides an updated review of the literature. Methods: A 31-year-old male presented with progressive paraparesis, sensory deficits, and sphincter dysfunction. MRI demonstrated an intramedullary tumor extending from T3 to the conus medullaris. The patient underwent a planned two-stage resection with intraoperative neurophysiological monitoring. Histopathological and DNA-methylation analyses were performed. Additionally, a systematic review of previously reported holocord astrocytoma cases was conducted. Results: The two-stage surgical strategy enabled extensive debulking across multiple spinal segments while preserving neurological function. The patient demonstrated marked postoperative improvement, including restoration of sphincter control, improved motor function, and better mobility. Histopathological analyses confirmed a high-grade astrocytoma with piloid features. The literature review identified 28 previously reported cases, including only 5 in adults. Reported neurological outcomes across adult cases are variable, reflecting the heterogeneity and rarity of this tumor entity. Conclusions: Holocord astrocytomas in adults are extremely rare and pose particular diagnostic and therapeutic challenges. This case demonstrates that a carefully planned, staged surgical approach can achieve meaningful neurological recovery, even in patients presenting with severe preoperative deficits. The report expands the limited body of evidence available for adult holocord astrocytomas and may support future management strategies.

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