Brain Tumor Res Treat.  2024 Jan;12(1):80-86. 10.14791/btrt.2023.0047.

Central Nervous System Dissemination of Solitary Sporadic Supratentorial Hemangioblastoma: A Case Report and Literature Review

• Jung SC
• Kim IY
• Jung S
• Jung TY
• Moon KS
• Kim YJ
• Park SJ
• Lee KH

• KMID: 2552331
• DOI: http://doi.org/10.14791/btrt.2023.0047

Abstract

We report a patient with whole neuroaxis dissemination of a sporadic supratentorial hemangioblastoma (HB) for more than 15 years. A 68-year-old female patient presented with severe radiating pain in the right leg. Gadolinium-enhanced lumbar spine MRI showed an intradural mass (2.5 cm in diameter) at the L4 level. The patient had been severely disabled for 22 years after a previous intraventricular brain tumor resection. At that time, the diagnosis was angioblastic meningioma, which was thought to be incorrect. At 14 years after the brain surgery, gamma knife radiosurgery was performed three times for newly developed or recurred supratentorial and infratentorial tumors in the cerebrospinal fluid pathway. The patient underwent lumbar spinal surgery, and a gross total removal of the mass was performed, which confirmed the histopathological diagnosis of HB. We reexamined the old histopathological specimen of the intraventricular tumor from 20 years ago and changed the diagnosis from angioblastic meningioma to supratentorial HB. Six months after spinal surgery, the patient underwent a second spinal surgery and brain surgery, and the histopathological diagnosis was HB following both surgeries, which was the same following the first spinal surgery. Here, we report a sporadic supratentorial HB patient who showed cranial and spinal disseminations for more than two decades along with a literature review

Keyword

Dissemination; Hemangioblastoma; Spine; Surgery; Magnetic resonance image; Recurrence

Figure

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  Fig. 1 Gadolinium (Gd)-enhanced sagittal lumbar spine MRI (July 2022) (A) of an intradural mass (2.5 cm in diameter) at the L4 level with well-homogeneous enhancement, which occupied the whole spinal canal at this level. Gd-enhanced whole-spine MRI of other masses at the cervical and thoracic spines (B). The tumor showed histological features with irregularly formed vessels interspersed with large stromal cells (C; H&E, ×100). The tumor cells were slightly positive for alpha-inhibin (D; immunohistochemistry, ×200) but strongly positive for EGFR (E; ×200).

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  Fig. 2 Gadolinium-enhanced axial brain MRI (December 2000) of an intraventricular mass (4 cm in diameter) at the third and lateral ventricles.

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  Fig. 3 Gadolinium-enhanced axial brain MRIs (April 2014) of the tumors in the cerebellomedullary cistern (A) and fourth ventricle (B).

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  Fig. 4 The tumor exhibited irregularly dilated blood vessels and extensive hemorrhage at low magnification (A; H&E staining, original magnification ×40). At high magnification, the tumor had vacuolated stromal cells characterized by large hyperchromatic nuclei and an eosinophilic foamy cytoplasm (B; H&E, ×200).

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  Fig. 5 Gadolinium (Gd)-enhanced sagittal spine MRI (January 2023) of a progressed 2 cm-sized vividly enhancing intramedullary mass at the T9 level (A), and Gd-enhanced axial brain MRI (May 2023) of the tumor in the right cerebellopontine angle (B). Histopathologically, the second recurrent brain tumor was composed of plump tumor cells characterized by a clear cytoplasm (C; H&E, ×100) with immunopositivity for alpha-inhibin (D; ×200) and EGFR (E; ×200).

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