Spinal astroblastoma: a rare tumour in an unusual location

Rao, Shilpa; Nufina, T A; Sugur, Harsha; Arumalla, Kirit; Devi, Bhagavatula Indira; Santosh, Vani

doi:10.1007/s00381-022-05468-w

Cited by 4 publications

(6 citation statements)

References 12 publications

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“…17 Both of the previously described MN1 altered spinal astroblastomas responded well to intensity-modulated radiation therapy and temozolamide. 4,5 In our case, 3 months after GTR, no residual disease was found in postcontrast scan. As the role of adjuvant radiotherapy and chemotherapy is not properly defined, there is little literature regarding spinal astroblastomas; gross total removal of the tumor was achieved, and the child was given only supportive care and kept under close follow-up.…”

Section: Discussionsupporting

confidence: 47%

“…Among the previously reported cases of spinal astroblastoma, one, reported by Yamada et al 4 occurred in a 20-year-old female involving the T1-T2 level of the spinal cord, and another, reported by Rao et al, 5 occurred in a 16-year-old boy involving the T11-T12 level of the spinal cord; both harbored MN1 alterations. While the third case, reported by Yamasaki et al, 6 which occurred in a 3-month-old child involving the lower portion of the medulla to the C4 level, was classified as a high-grade neuroepithelial tumor (HGNET) with MN1 alteration based on methylation assay, despite having EWSRI-BEND2 fusion detected on RNA sequencing and lack of MN1 alteration demonstrated by both FISH and RNA sequencing.…”

Section: Discussionmentioning

confidence: 97%

“…Among the previously reported cases of spinal astroblastoma, one, reported by Yamada et al 4 . occurred in a 20‐year‐old female involving the T1–T2 level of the spinal cord, and another, reported by Rao et al ., 5 occurred in a 16‐year‐old boy involving the T11–T12 level of the spinal cord; both harbored MN1 alterations.…”

Section: Discussionmentioning

confidence: 97%

“…3 Astroblastomas in the spine are extremely rare, and only three cases have been described in the literature to date. [4][5][6]…”

Section: Introductionmentioning

confidence: 99%

“…No definite grade is assigned to these tumors in recent central nervous system (CNS) World Health Organization (WHO) classification as both low‐ and high‐grade tumors are known to occur 3 . Astroblastomas in the spine are extremely rare, and only three cases have been described in the literature to date 4–6 …”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Spinal astroblastoma, MN1 altered in 3‐year‐old child: An uncommon tumor at an unusual site

Panda,

Das,

Singh

et al. 2024

Neuropathology

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Astroblastoma is an uncommon circumscribed glial tumor mostly involving the cerebral hemisphere. The characteristic molecular alteration is meningioma (disrupted in balanced translocation) 1 (MN1) rearrangement. No definite World Health Organization grade has been assigned as both low‐ and high‐grade tumors are known to occur. Tumors in the spine are extremely rare; to date only three cases have been reported in the literature. A vigilant microscopy and ancillary testing aid in diagnosis when the tumors present in unusual locations, as in our case. The prompt differentiation of this tumor from its mimickers is a mandate as modalities of management are different and not clearly established.

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Section: Discussionsupporting

confidence: 47%

Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 97%

“…3 Astroblastomas in the spine are extremely rare, and only three cases have been described in the literature to date. [4][5][6]…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Spinal astroblastoma, MN1 altered in 3‐year‐old child: An uncommon tumor at an unusual site

Panda,

Das,

Singh

et al. 2024

Neuropathology

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MN1 altered astroblastoma with APC and LRP1B gene mutations: a unique variant in the cervical spine of a pediatric patient

et al. 2023

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Spinal Cord Astroblastoma With EWSR1-BEND2 Fusion in Female Patients

Fu,

Lao,

Huang

et al. 2024

American Journal of Surgical Pathology

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Astroblastoma is an extremely rare central nervous system tumor characterized by astroblastic pseudorosettes and vascular hyalinization. Despite these histologic hallmarks, its morphology can vary, occasionally resembling other central nervous system tumors such as ependymoma. A novel tumor entity, astroblastoma, meningioma 1 (MN1)-altered, has been identified, featuring MN1 gene rearrangements typically involving BEN-domain containing 2 (BEND2) as a fusion partner. Most astroblastomas arise in the cerebral hemisphere. Here, we report 4 cases of spinal cord astroblastoma in female patients, all showing Ewing sarcoma RNA-binding protein 1 fusion with BEND2, rather than MN1. These tumors displayed growth patterns akin to traditional intracranial astroblastomas, with three cases demonstrating high-grade histology, including elevated mitotic activity and necrosis. Interestingly, some cases exhibited positive staining for pan-cytokeratin and hormone receptors. DNA methylation profiling clustered three of the four cases with the reference “AB_EWSR,” whereas one case exhibited an independent methylation signature near the reference methylation group “AB_EWSR” and “pleomorphic xanthoastrocytoma.” Together with the existing literature, we summarized a total of eleven cases, which predominantly affected children and young adults with female predilection. Eight of 10 patients experienced recurrence, underscoring the aggressive nature of this disease. We suggest recognizing a new molecular subgroup of spinal astroblastoma and recommend testing newly diagnosed infratentorial astroblastomas for Ewing sarcoma RNA-binding protein 1-BEND2 fusion.

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Spinal astroblastoma: a rare tumour in an unusual location

Cited by 4 publications

References 12 publications

Spinal astroblastoma, MN1 altered in 3‐year‐old child: An uncommon tumor at an unusual site

Spinal astroblastoma, MN1 altered in 3‐year‐old child: An uncommon tumor at an unusual site

MN1 altered astroblastoma with APC and LRP1B gene mutations: a unique variant in the cervical spine of a pediatric patient

Spinal Cord Astroblastoma With EWSR1-BEND2 Fusion in Female Patients

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