Pediatric sellar solitary fibrous tumor/ hemangiopericytoma: A rare case report and review of the literature
- Department of Neurosurgery, Riverside University Health System, Moreno Valley, United States.
- Department of Neurosurgery, Kaiser Permanente, Los Angeles, United States.
- Department of Neurosurgery, Desert Regional Medical Center, Palm Springs, United States.
- Department of Neurosurgery, Kaiser Permanente, Fontana, California, United States.
Department of Neurosurgery, Kaiser Permanente, Fontana, California, United States.
DOI:10.25259/SNI_234_2020Copyright: © 2020 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Hammad Ghanchi1, Tye Patchana1, Eisha Christian2, Chao Li3, Mark Calayag4. Pediatric sellar solitary fibrous tumor/ hemangiopericytoma: A rare case report and review of the literature. 08-Aug-2020;11:238
How to cite this URL: Hammad Ghanchi1, Tye Patchana1, Eisha Christian2, Chao Li3, Mark Calayag4. Pediatric sellar solitary fibrous tumor/ hemangiopericytoma: A rare case report and review of the literature. 08-Aug-2020;11:238. Available from: https://surgicalneurologyint.com/surgicalint-articles/10188/
Background: Solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) is a rare tumor which originates from the walls of capillaries and has historically been thought to be able to occur anywhere in the body that blood vessels are found. It is rarely found in the sellar region.
Case Description: InS this report, we present the first case of this tumor occurring in the sellar region of a pediatric patient. This 12-year-old male presented with progressive vision loss which prompted surgical resection after a sellar lesion was discovered on imaging. The initial transsphenoidal approach resulted in subtotal resection and the patient experienced reoccurrence within 3 months. He underwent an orbitozygomatic craniotomy to achieve gross total tumor resection.
Conclusion: We conducted a literature review of intracranial SFT/HPC in the pediatric population and found it to be an extremely rare occurrence, with
Keywords: Orbitozygomatic craniotomy, Pediatric hemangiopericytoma, Pediatric sellar tumor, Pediatric tumors
Solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) is a rare tumor derived from the wall of capillaries and has historically been thought to be able to occur anywhere in the body, though it is rarely seen in the sellar region. The term “hemangiopericytoma” was first introduced in 1942 by Stout and Murray as a vascular tumor originating from Zimmermann’s pericytes, modified smooth muscle cells within the capillary walls that form endothelial tubes and sprouts.[
Head-and-neck cases make up approximately 25–33% of total HPC with <1% of these lesions occurring intracranial. HPC is rare in adults, and even more so in children, as less than 10% of HPC occur in children:[
Our patient is a 12-year-old male who was initially being evaluated by optometry for headaches and progressive visual impairment over a 2-month period. On assessment of the patient’s vision, a diagnosis of bitemporal hemianopsia was made, prompting magnetic resonance imaging (MRI) of brain and orbits. As demonstrated in [
On neurological examination, the patient was neurologically intact with the exception of bitemporal visual field loss. Visual acuity was noted to be 20/20 on the right and 20/100 on the left. Serum laboratory assessment for hormonal irregularities was unremarkable. The patient was also noted to have polydipsia without overt signs of diabetes insipidus (i.e., stable serum sodium levels and remaining euvolemic). He subsequently underwent transnasal transsphenoidal craniotomy for resection and decompression of the optic chiasm. Postoperatively, the patient recovered well and was subsequently discharged home without any evident complications, with plan for possible further surgery after pathology finalized.
Intraoperatively, the tumor was noted to be firm and fibrous. Complete resection was not feasible from the trans-nasal approach as the tumor was noted to be extremely adherent to surrounding structures. Frozen section pathology from the initial surgery was inconclusive. However, final pathology from this surgery was reported to be SFT/HPC, World Health Organization (WHO) Grade II.
On 3-month follow-up, imaging demonstrated progressive and enlarging lesion, as demonstrated in [
Postoperative imaging, as visualized in [
Extracranial SFT/HPCs have been reclassified as a spectrum of SFTs, whereas the HPC continues to be used among neuropathologists. Both entities share the 12q3 inversion and fusion of the NGFI-A-binding protein 2 (NAB2) and STAT6 genes with the STAT6 nuclear expression visible on immunohistochemistry.[
HPCs have the potential to occur anywhere in the body as they originate from blood vessels but the most common location is in the lower extremity in the pediatric population.[
Two forms of HPC have been identified during childhood; infantile type which occurs during the first 12 months of life and the adult type which occurs after the age of 12 months old.[
A recent study[
STAT6 for diagnosis of SFT/HPC
STAT6 immunohistochemistry has been found to be an exclusively nuclear immunostaining signal with a strong ability to verify SFTs and HPCs, with recent studies showing all non-SFT/HPCs unanimously STAT6 negative and 98.7% agreement among observers.[
Sellar location SFT/HPC
To date, a total of 15 cases of sellar region SFT/HPC were identified in the English literature, as listed in [
Given the rarity of HPC, information about management specific to intracranial HPC for pediatric population is scarce. Moreover, the distinction between infantile and adult HPC is paramount to the clinical course. The infantile variant, occurring <12 months of age, is chemoresponsive. There are reports of extracranial infantile HPC exhibiting spontaneous regression.[
On the contrary, adult HPC requires a more aggressive approach. Surgical resection followed by adjuvant radiation therapy has been recommended in the past for treatment from adult literature.[
However, a recent meta-analysis of 563 cases found that GTR alone provided superior survival, with or without adjuvant radiation therapy; patients receiving >50 Gy of radiation had worse survival outcomes.[
Data specific to pediatric population for intracranial disease is lacking; however, given the similar clinical behavior of extracranial disease,[
The authors present a report of a SFT/HPC occurring in a 12-year-old male patient in the sella. This lesion mimicked a nonfunctioning pituitary adenoma both clinically and on imaging, with a surprising final pathological result; this is the first report of HPC occurring in the sellar region in the pediatric population. Moreover, recent literature with regard to STAT6 positivity suggests that many prior diagnoses of HPC may be false positives, making it an even rarer entity. Data are lacking for evidence-based treatment recommendations for pediatric HPC; from adult data, goals favor GTR for best overall survival for adult-type HPC, which occurs in the pediatric population after the age of 1 year. The role for adjuvant radiation therapy is unclear at this time, with benefits seen with in patients only able to undergo STR with regard to the progression-free interval. Prospective trials are needed to further characterize specific treatment modalities.
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