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A Rare Case of a Primary Spinal Solitary Fibrous Tumor/Hemangiopericytoma in a 9-Month-Old Patient

Challenging differential diagnosis, Rare disease

Edrick G. Lugo Millan, Hans W. Hess, Juan Carlos Carballo-Santiago, Roman Velez-Rosario, Eduardo J. Labat

Puerto Rico Department of Diagnostic Radiology, University of Puerto Rico School of Medicine, San Juan, Puerto Rico

Am J Case Rep 2020; 21:e923176

DOI: 10.12659/AJCR.923176

Available online: 2020-09-28

Published: 2020-11-07


#923176

BACKGROUND: Solitary fibrous tumors (SFTs)/hemangiopericytomas (HPCs) are mesenchymal tumors commonly found in middle-aged patients, usually localized to thoracic pleurae. Spinal tumor involvement is rarely seen, and its imaging findings are largely inconsistent because of the rarity of these cases. We present a case report of a 9-month-old girl with a rare intraspinal tumor with histologic evidence of SFT/HPC, but no STAT6 nuclear immunoreactivity.
CASE REPORT: A 9-month-old girl, born at term with good prenatal care, presented to the emergency room with regression of developmental milestones. The patient was in good health until 2 months, when she developed decreased spontaneous leg movements. Physical exam revealed diffuse muscular atrophy, with no deep tendon reflexes, sensation, or spontaneous movements of the lower extremities. Computed tomography and magnetic resonance imaging showed a heterogeneous irregular mass filling the lumbosacral spinal canal, extending through the neural foramina to the prevertebral/perivertebral and presacral regions. The tumor was biopsied and referred to the National Institutes of Health for consultation and the diagnosis of SFT/HPC was confirmed on the basis of its histologic features, despite the fact that the tumor was negative for STAT6 immunoreactivity.
CONCLUSIONS: Although the tumor histology was consistent with SFT/HPC, it was negative for STAT6 nuclear immunoreactivity, which is unusual and may exclude the diagnosis. To our knowledge, this is the youngest patient to present with a spinal SFT with these features.

Keywords: Hemangiopericytoma, Solitary Fibrous Tumors, STAT6 Transcription Factor



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