Malignant spindle cell tumors of the posterior fossa in children: case series and review of management

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  • 1 Department of Neurosurgery, Penn State Health Milton S. Hershey Medical Center, Hershey;
  • | 2 Department of Pathology, Penn State Health Milton S. Hershey Medical Center, Hershey; and
  • | 3 College of Medicine, Penn State Health Milton S. Hershey Medical Center, Hershey, Pennsylvania
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OBJECTIVE

The WHO Classification of Tumours of the Central Nervous System (2016) classifies nonmeningothelial malignant spindle cell tumors involving the extraaxial tissues of the posterior fossa as melanocytic tumors and malignant mesenchymal tumors (sarcomas). The objective of this study was to conduct a review of the literature pertaining to the management strategies of posterior fossa malignant spindle cell tumors in the pediatric population.

METHODS

The authors performed an institutional search of their pathology database for patients younger than 18 years of age who presented with posterior fossa malignant spindle cell tumors. A literature review was also performed using the PubMed database, with “posterior fossa” or “spindle cell tumors” or “Ewing sarcoma” or “high-grade” or “spindle cell sarcoma” or “leptomeningeal melanocytoma” as keywords. The database search was restricted to pediatric patients (age ≤ 18 years). Parameters reported from the literature review included patient age, tumor location, presenting symptoms, treatment modalities (resection, chemotherapy, and/or radiotherapy), leptomeningeal spread at or after the time of treatment, and follow-up length and resulting outcome.

RESULTS

The authors report 3 rare cases of posterior fossa malignant spindle cell tumors, including Ewing sarcoma in a 13-year-old male; high-grade spindle cell sarcoma, not otherwise specified in a 10-year-old male; and primary leptomeningeal melanocytoma in a 16-year-old female. All 3 patients underwent resection and radiotherapy and either chemotherapy or targeted immunotherapy. At the last follow-up, all patients were alive with either resolution or stable disease.

CONCLUSIONS

A review of these 3 cases and the existing literature support managing patients with intracranial malignant spindle cell tumors with multimodal therapy that can include a combination of resection, radiotherapy, and chemotherapy or immunotherapy to prolong progression-free and overall survival.

ABBREVIATIONS

EVD = external ventricular drain; Fx = fractions; NOS = not otherwise specified; PBRT = photon-beam radiation therapy; PNET = primitive neuroectodermal tumor.

Supplementary Materials

    • Supplemental Figs. 1-3 (PDF 6,330 KB)

Illustration from Soleman et al. (pp 544–552). Copyright Lucille Solomon.

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