A Rare Case of a Solitary Fibrous Tumor in a Child, Presenting as a Congenital Mass, Displaying NAB2ex4::STAT6ex2 Gene Fusion with an Incidental Ganglioneuroblastoma

Abstract

A solitary fibrous tumor (SFT) is a ubiquitous tumor that occurs across all ages. It is rarely reported in pediatric patients, especially as a congenital mass. A 1 year 7-month old male child presented with a slow-growing gluteal mass since birth along with a recent episode of bleeding. Magnetic resonance imaging (MRI) showed a well-defined, T1-hypointense and T2-hyperintense mass in the skin and the subcutaneous plane. Subsequently, he underwent a surgical resection that revealed a 5.7 cm-sized fleshy mass. On histopathologic examination, there was a fairly well-circumscribed, cellular spindle cell tumor, with cells arranged in intersecting fascicles and hemangiopericytomatous pattern with areas of hemorrhage and fibrinoid necrosis. Cells revealed mild nuclear atypia. Mitotic figures were up to 8/10 hpf. Immunohistochemically, the tumor cells were diffusely positive for CD34 and STAT6. Furthermore, the tumor revealed NAB2 exon 4:: STAT6 exon 2 fusion by RT-PCR and Sanger sequencing. Post-excision, during follow-up, the patient developed an adrenal mass that was histopathologically proven as a neuroblastic tumor, not further specified on biopsy and finally as ganglioneuroblastoma, intermixed type on excision. This constitutes one of the rare cases of SFT in a pediatric patient, presenting as a congenital mass, confirmed by STAT6 immunostaining and further, molecular testing. A review of literature of similar cases, including treatment-related implications, is presented.