Giant intracranial congenital hemangiopericytoma/solitary fibrous tumor: A case report and literature review
- Department of Neurosurgery, University of Athens Medical School, “Evangelismos” General Hospital, National and Kapodistrian University of Athens, Athens, Greece
- Department of Imaging, National and Kapodistrian University of Athens, Athens, Greece
- Department of Pathology, Children’s Hospital “Aghia Sofia”, Athens, Greece
- Department of First Department of Pediatrics, National and Kapodistrian University of Athens, Athens, Greece
- Department of Neurosurgery, Children’s Hospital “Aghia Sophia”, Athens, Greece
Department of Neurosurgery, Children’s Hospital “Aghia Sophia”, Athens, Greece
DOI:10.25259/SNI-85-2019Copyright: © 2019 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: Dimitrios Giakoumettis, Ioannis Nikas, Kalliopi Stefanaki, Antonis Kattamis, George Sfakianos, Marios S. Themistocleous. Giant intracranial congenital hemangiopericytoma/solitary fibrous tumor: A case report and literature review. 24-Apr-2019;10:75
How to cite this URL: Dimitrios Giakoumettis, Ioannis Nikas, Kalliopi Stefanaki, Antonis Kattamis, George Sfakianos, Marios S. Themistocleous. Giant intracranial congenital hemangiopericytoma/solitary fibrous tumor: A case report and literature review. 24-Apr-2019;10:75. Available from: http://surgicalneurologyint.com/surgicalint-articles/9278/
Background:Hemangiopericytoma and solitary fibrous tumor (HPC/SFT) are considered to be one category according to the WHO 2016 classification of central nervous system tumors. HPC/SFT are subdivided into infantile (congenital) and adult type. Both are extremely rare entities, with little knowledge about etiology, prognosis, and optimal therapeutic strategy.
Case Description:A 10-day-old girl was referred to our neurosurgical department due to hypotonia, palsy of the right oculomotor nerve, and prominent frontal fontanel. Imaging studies revealed a large occupying mass in the right middle cerebral fossa and the suprasellar cisterns. Only a subtotal resection of the tumor was possible, and postoperatively, she underwent chemotherapy (CHx). After a 3-year follow-up, the girl has minimum neurologic signs and receives no medications, and she can walk when she is supported.
Conclusion:Congenital HPC/SFT is considered to have a benign behavior with a good prognosis. Treatment with gross total resection, when it is feasible, is the key to a good prognosis and low rates of recurrence. However, there is no consensus on the therapeutic strategy of a HPC/SFT, which is difficult to be completely resected. Literature lacks a therapeutic algorithm for these tumors, and thus, more clinical studies are needed to reach a consensus.
Keywords: Congenital, hemangiopericytoma, intracranial, solitary fibrous tumor
It is known that congenital brain tumors are very rare with an incidence of 1.1–3.6/100,000 newborns.[
A 10-day-old girl was referred to our neurosurgical department from the neonatal intensive care unit where it was being treated since her 3rd day after birth due to jaundice. She presented with hypotonia, palsy of the right oculomotor nerve, and prominent frontal fontanel; a cerebral ultrasound and subsequently a computed tomography (CT) scan were performed and revealed a large hyperdense space-occupying mass in the right middle cerebral fossa and the suprasellar cisterns. Magnetic resonance imaging (MRI) demonstrated a tumor with marked inhomogeneous enhancement, with mixed cystic and solid components with dimensions of 6.7 cm × 6.2 cm × 6.1 cm [
The incidence of congenital tumors is 0.34 per one million births, and infantile HPCs are extremely rare with an incidence of <1% of all CNS tumors.[
In our case, the neoplasm shows morphological heterogeneity and is composed of cellular areas of the short bundles of spindle, stellate, and ovoid cells with eosinophilic cytoplasm and a round nucleus with fine chromatin without nucleolus and with mild-to-moderate nuclear atypia. A moderate-to-brisk mitotic activity of 12–40 mitoses/10 high-power fields (hpf) × 40 and 2–6 mitoses/hpf × 40 was recognized [
(a) Cellular areas of short bundles of spindle cells with an eosinophilic cytoplasm and a round nucleus with fine chromatin without nucleolus and with mild-to-moderate nuclear atypia. (b) Heterogeneous cellular density with cellular heterogeneity and obvious mitoses in a cellular area of the neoplasm. (c) Immunohistochemical expression of CD34 in the neoplastic cells mainly membranous and cytoplasmic. (d) A heterogeneous expression of smooth muscle actin in bundles of spindle cells.
Infantile HPC/SFT is considered to have a benign behavior with a good prognosis. Treatment with GTR is the key to a good prognosis and low rates of recurrence. Nevertheless, GTR is not always feasible. There is no consensus on the therapeutic strategy of a HPC/SFT, which is difficult to be completely resected. CHx before surgery has been proven useful, to make the tumor operable. Moreover, it has been successfully applied as an adjuvant therapy after surgery, or in case of recurrence. RTx has also been used in treating these tumors, but there have been studies that support its ineffectiveness. Literature lacks a therapeutic algorithm for these tumors, and thus, more clinical studies are needed to reach a consensus. In our case, a subtotal resection was performed, which was postoperatively complicated with obstructive hydrocephalus. After a ventriculoperitoneal shunt operation, the patient received CHx.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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