Pathological Features and Clinical Course in Patients With Recurrent or Malignant Orbital Solitary Fibrous Tumor/Hemangiopericytoma

Abstract

Purpose: A risk assessment score for metastasis based on age, tumor size, and mitotic figures has been suggested for nonorbital solitary fibrous tumor (SFT)/hemangiopericytoma. The authors herein examine the clinicopathological features of recurrent and metastatic orbital SFT and evaluate the existing risk assessment score for orbital SFT. Methods: The American Society of Ophthalmic Plastic and Reconstructive Surgery Oncology Database was queried for patients with recurrent or malignant orbital hemangiopericytoma/SFT. The medical records were reviewed for clinical and pathologic findings, treatments, and outcomes. Results: Eight patients from 3 institutions were identified with recurrent orbital hemangiopericytoma/SFT. Median age at diagnosis was 59 years, and 4 patients were women. The mean size of tumor was 2.1 ± 1.1 cm. All patients were initially treated with surgery and experienced local recurrence after a median of 4 (range 0.5–10) years. Five patients were treated with orbital radiation. Two patients also developed distant metastases and eventually died of their disease. Median Ki-67 was 5% (range 1–65%) and 5 mitotic figures/10 high-power fields (range 2–30). The previously described risk stratification model for nonorbital SFT did not correlate with the propensity to develop metastases in this cohort; however, both patients with distant metastasis had > 4 mitotic figures /10 high-power fields. Conclusions: In this cohort of recurrent orbital hemangiopericytoma/SFT, median time to recurrence was 4 years underscoring the importance of careful continued follow-up. The current risk stratification models have limited use for orbital lesions, mostly due to the fact that orbital SFTs are smaller than even the smallest size criteria in this risk assessment model.

Clinicopathological Characteristics of Three Cases with Recurrent Orbital Solitary Fibrous Tumors: A Retrospective Study and Literature Review

“…In the present research, clinical, imaging, and pathological features of 3 patients with recurrent orbital SFT were retrospectively compared. We also used the PubMed database to search for recurrent orbital SFT, and 21 related literatures were selected to explore their clinical and pathological features [3,4,[6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23].…”

“…The MRI ndings of orbital SFT showed that lesions had equal density with the gray matter on T1WI, and values of signal intensity on T2WIs were controversial [30][31][32][33]. Previous studies reported that orbital SFT is rich in brous matrix and T2WI should be dominated by a low signal intensity, however, in several reported cases of malignant orbital SFT, cystic degeneration could be detected with a high signal intensity, suggesting that cystic degeneration could be related to malignant transformation [4,34]. In addition, the contrast-enhanced MRI showed enhanced SFT, while the enhancement can be uniform or uneven, and it may be associated with the distribution of blood vessels and capillary permeability [35][36][37].…”

“…emphasized that the number of mitotic gures higher than 4/10 HPFs can be a predictor of malignant SFT [4]. In addition, nuclear atypia, cell proliferation, and necrosis were also considered as manifestations of malignant SFT [35].…”

“…The hemosiderotic brolipomatous tumor is histologically characterized by the presence of mature adipose tissue and spindle cell component accompanied by hemosiderin pigment deposition within macrophages, in the cytoplasm of some of the spindle cells and also within the extracellular stroma. The spindle cell component of these lesions is generally positive for CD34 and negative for CD68, S100, SMA and desmin [4,5]. The biological course of SFT tends to be benign in nature, while some cases with recurrent SFT and its malignant transformation have been reported in recent years.…”

The Diagnosis and Treatment of Orbital Solitary Fibrous Tumor

“…In our case, 2 patients were found to have relapsed, with the lesions growing more rapidly and more invasive after recurrence. In the cohort of recurrent orbital hemangiopericytoma/SFT, median time to recurrence was 4 years underscoring the importance of careful continued follow-up [18] . This group were followed up from 7 months to 8 years, and the median was 2 years 10 months.…”