Primary intra and extradural solitary fibrous tumor/hemangiopericytoma of thoracic spine with paravertebral intrathoracic spread: Case report and review of the literature

• Main Authors: Vito Fiorenza, Francesco Ascanio, Francesca Ferlito, Benedetto Lo Duca, Damiano Librizzi
• Format: Article
• Language: English
• Published: Elsevier 2020-09-01
• Series: Interdisciplinary Neurosurgery
• Subjects: Dumbbell Tumors; Primary Spine Tumors; Solitary Fibrous Tumor/Hemangiopericytoma
• Online Access: http://www.sciencedirect.com/science/article/pii/S2214751920300074

Solitary fibrous tumors/hemangiopericytomas (SFTs/HPCs), constitute 1% of all CNS tumors. Spinal SFTs/HPCs are extremely rare. To date, few retrospective studies and case reports of primary spinal SFTs/HPCs have been published in the literature. We report clinical and radiological presentation, surgical treatment, and post-operative outcome at three years follow-up of a rare case of primary spinal intra and extradural SFT/HPC of thoracic spine with dumbbell shaped paravertebral intrathoracic spread and multidirectional erosion of the bone. A 73-year-old female presented with progressive lower limbs weakness and hypoesthesia below the rib cage. MRI showed an irregular isointense T5-T7 dumbbell shaped tumor. Tumor resection was successfully carried out through posterior and antero-lateral approach. Histological examination showed a grade II SFT/HPC. No local recurrence nor systemic metastases were observed at three years follow-up. A literature review has been performed to describe epidemiology, radiographic features, treatment, recurrence rate and mean disease-free survival of primary spinal SFTs/HPCs. No radiographic pathognomonic findings have been reported for these tumors. Differential diagnosis must be made with meningioma, schwannoma, chordoma, aggressive hemangioma, metastases, angiosarcoma. Surgical resection is the first choice of treatment, and total resection should be attempted whenever possible in all cases. Postsurgical radiotherapy does not change significantly recurrence rate after GTR, nonetheless it increases mean disease-free survival, especially in patients with extradural SFTs/HPCs. After subtotal resection, adjuvant radiotherapy is necessary to reduce progression of disease. The efficacy of chemotherapy has yet to be determined. Pathological degree and total surgical resection are the most important predictive factors of recurrence.