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

• Published in: The American journal of case reports Vol. 21; p. e923176
• Main Authors: Lugo Millan, Edrick G, Hess, Hans W, Carballo-Santiago, Juan Carlos, Velez-Rosario, Roman, Labat, Eduardo J
• Format: Journal Article
• Language: English
• Published: United States International Scientific Literature, Inc 07.11.2020
• Subjects: Central Nervous System Neoplasms; Female; Hemangiopericytoma - diagnostic imaging; Humans; Infant; Magnetic Resonance Imaging; Middle Aged; Solitary Fibrous Tumors - diagnostic imaging; Spinal Neoplasms; United States; United States
• ISSN: 1941-5923; 1941-5923
• DOI: 10.12659/AJCR.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.