Primary Intracranial Rhabdomyosarcoma in the Cerebellopontine Angle Resected After Preoperative Embolization

Yoshida, Keisuke; Miwa, Takaki; Akiyama, Takahiro; Nishimoto, Masaaki; Kamamoto, Dai; Yoshida, Kazunari

doi:10.1016/j.wneu.2018.05.054

Cited by 9 publications

(8 citation statements)

References 29 publications

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“…CPA lesions are uncommon in pediatric population with an incidence rate of less than 10%. [ 5 ] Unlike adult population where the vast majority of CPA lesions are vestibular schwannomas and meningiomas, pediatric patients experience more various tumor types comprising 35%–45% of CPA lesions (e.g., arachnoid cyst, lipoma, and cavernous hemangioma). [ 6 ] Malignant sarcomas constitute less than 1% of pediatric CPA tumors.…”

Section: Discussionmentioning

confidence: 99%

“…Intracranial RMS is an aggressive, rapidly growing, and vascular tumor that carries a very poor prognosis with only nine reported case survived beyond 2 years. [ 5 ] Unfortunately, RMS cannot be distinguished from other primary or metastatic tumors of the brain with imaging alone. 4 …”

Section: Discussionmentioning

confidence: 99%

“…[ 4 ] Primary intracranial types are even more rare, with only 50 reported cases of primary intracranial RMS in the literature. [ 5 ]…”

Section: Introductionmentioning

confidence: 99%

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Intracranial rhabdomyosarcoma of the cerebellopontine angle in a 6-year-old child: A case report

et al. 2020

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A BSTRACT Rhabdomyosarcoma (RMS) is the most common soft-tissue malignancy in children under 15 years of age. Approximately, 35% of RMS cases originate from the head and neck region. Among various sites in the head and neck region, intracranial extension is more likely to occur with parameningeal tumors, which is also considered an unfavorable prognostic factor in children with RMS. About 20% of RMS occurs in a parameningeal site. Intracranial RMS are rare tumors that usually arise from parameningeal sites or metastasis from an extracranial site. Primary intracranial types are even rarer, with only 50 reported cases of primary intracranial RMS in the literature. Hereby, we report the case of a 6-year-old boy who presented with clinical and radiologic features of a cerebellopontine angle lesion, which turned out to be a RMS.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Intracranial rhabdomyosarcoma of the cerebellopontine angle in a 6-year-old child: A case report

et al. 2020

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“…In general, preoperative embolization benefits in resection of deep-seated skull base tumors, where feeders cannot be cauterized until the later stages of the operation. [11][12][13] When considering resection of hypervascular lesions through EEA, reducing tumor vascularity beforehand by embolization would be highly beneficial because hemostatic maneuvers under endoscopy is often more challenging than those under microscopy. [4][5][6][7][8][9][10] Even proximal feeder occlusions can be effective when combined with devascularization under endoscopy.…”

Section: Discussionmentioning

confidence: 99%

Pterygovaginal artery as a target of embolization before endoscopic skull base surgery

et al. 2021

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Background The pterygovaginal artery (PtVA), a recurrent branch of the internal maxillary artery (IMA), can be a feeder of skull base tumors. Preoperative embolization can help endoscopic resection of hypervascular lesions, which is performed under a narrow surgical space with restricted instrumental maneuverability. Methods We performed preoperative embolization in five cases with hypervascular skull base lesions supplied by the PtVA, four of which were resected via endoscopic endonasal approach. In two cases, selective PtVA embolization through the distal IMA was successfully conducted. Results In all the cases, intraoperative bleeding during endoscopic resection was easily controlled. The medial and lateral origins of the PtVA from the IMA were demonstrated by cone-beam CT images reconstructed from three-dimensional rotational angiography, and anastomoses around the eustachian tube and soft palate were visualized by superselective angiography. Conclusions The PtVA embolization can be an effective strategy before endoscopic skull base tumor resection. When embolizing through the PtVA, clinicians should be aware of its anatomical variations and dangerous anastomoses. Understanding the surrounding angioarchitecture by angiographic techniques helps ensure safe embolization.

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“…MRI of the spine was negative for metastasis and disseminated disease. Selective catheter embolization of the left anterior inferior cerebellar artery (AICA) that was feeding the tumor was performed as previously described [14]. Then, the patient underwent gross total resection (GTR) of the tumor.…”

Section: Our Casementioning

confidence: 99%

A case of primary CNS embryonal rhabdomyosarcoma with PAX3-NCOA2 fusion and systematic meta-review

et al. 2021

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Purpose: Primary central nervous system (CNS) rhabdomyosarcomais a rare mesenchymal tumor predominantly seen in children and associated with a poor outcome. We report a case of primary CNS rhabdomyosarcoma with PAX3-NCOA2 fusion and presenta systematic meta-review of primary CNS rhabdomyosarcoma to characterize this rare tumor. Methods: We present the case of a 6-year-old boy with primary CNS rhabdomyosarcoma in the posterior fossa. In a systematic meta-review, we compare the demographic data of primary CNS rhabdomyosarcoma with data of rhabdomyosarcoma at all sites from the SEER database and analyze clinical factors associated with survival outcome.Results: Our patient underwent gross total resectionand receivedvincristine, actinomycin-D, cyclophosphamide with early introduction of concurrent focal radiation and remained alive with no evidence of disease for 2 years after the end of therapy. Histopathological review revealed embryonaltype rhabdomyosarcoma, and whole-transcriptome analysis revealed PAX3 (EX6)-NCOA2 (EX12) fusion. In all, 77 cases of primary CNS rhabdomyosarcoma were identi ed through the meta-review. The demographic data of primary CNS rhabdomyosarcoma were similar to dataof rhabdomyosarcoma at all sites. Overall and event-free survival outcomes were available for 64 and 56 patients, respectively, with a 3-year OS of 29.5%and a 3-year EFS of 26.2%. The group that received trimodal treatment exhibited better survival outcomes, with a 3-year OS of 57.4%and a 3-year EFS of 46.3%.Conclusions: Primary CNS rhabdomyosarcoma shares common histological, molecular, and demographic features with non-CNS rhabdomyosarcoma. A trimodal treatment approach with early introduction of radiation therapy may result in favorable survival outcomes.

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Primary Intracranial Rhabdomyosarcoma in the Cerebellopontine Angle Resected After Preoperative Embolization

Cited by 9 publications

References 29 publications

Intracranial rhabdomyosarcoma of the cerebellopontine angle in a 6-year-old child: A case report

Intracranial rhabdomyosarcoma of the cerebellopontine angle in a 6-year-old child: A case report

Pterygovaginal artery as a target of embolization before endoscopic skull base surgery

A case of primary CNS embryonal rhabdomyosarcoma with PAX3-NCOA2 fusion and systematic meta-review

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