myelin-basic-protein and Solitary-Fibrous-Tumors

Solitary fibrous tumor is a rare, spindle-cell benign mesenchymal neoplasm and has a high recurrence rate. In this study, we reviewed our experience in the diagnosis and treatment of 24 patients with central nervous system solitary fibrous tumors.. Clinical data were retrieved from the medical records. Prognosis was assessed by clinic service and telephone interview. The specimens were stained with hematoxylin and eosin. Immunohistochemistry for CD34, CD99, EMA, HMB-45, Bcl-2, vimentin, GFAP, S-100, MBP, CK and MIB-1 was performed in all cases. Distributions of time to progression and recurrence were estimated using the Kaplan-Meier method and compared using the log-rank test.. The 24 patients included 13 men and 11 women with a median age of 49.0 years. The most frequent initial symptoms were headache, dizziness, unstable walk and hearing loss. The most common location was cerebellar pontine angle (n = 6). Surgery reached gross total removal for 18 patients but subtotal removal for six patients on initial operation. Histopathologic examination showed spindle to oval cells were disposed in wavy fascicles between prominent, eosinophilic bands of collagen. Dense bands of collagen appeared in cross section as minute nodules that separated individual tumor cells. Cellular areas with a partial hemangiopericytoma pattern were noted in six cases. Atypical presentations were shown on initial operation in three cases. CD34, CD99 and vimentin were 100% positive; but EMA, CK, MBP, HBM-45 and GRAP were 100% negative. The positive in Bcl-2, RF and S-100 was 89%, 85% and 26%, respectively. Follow-up information was available for 23 patients. The median follow-up period was 36.0 months. Nine patients recurred and one patient died from the progression. Incomplete surgical resection was significantly associated with recurrence (p = 0.010). MIB-1 labeling index in recurrence was higher than in no recurrence (6.0% versus 3.4%, p = 0.029). All treated with subtotal removal only had subsequent tumor recurrence or progression; however, the two patients who were administered adjuvant radiosurgery after subtotal removal did not recur or progress. Adjuvant radiosurgery seemed to improve the prognosis (p = 0.028).. Solitary fibrous tumor is a rare mesenchymal tumor with a propensity to recur. The most affected area is the cerebellopontine angle. Immunohistochemistry should be used to differentiate solitary fibrous tumor from other tumors. The extent of resection, MIB-1 labeling index and some anaplastic features might be predictive for recurrence. Postoperative radiosurgery might be an option in incompletely resected solitary fibrous tumor. Regular and long-term follow-up remains mandatory to monitor recurrence.

Topics: 12E7 Antigen; Adolescent; Adult; Aged; Antigens, CD; Antigens, CD34; Biomarkers, Tumor; Cell Adhesion Molecules; Central Nervous System Neoplasms; Child; Female; Humans; Immunohistochemistry; Kaplan-Meier Estimate; Ki-67 Antigen; Male; Middle Aged; Myelin Basic Protein; Neoplasm Recurrence, Local; Prognosis; Proto-Oncogene Proteins c-bcl-2; S100 Proteins; Solitary Fibrous Tumors; Vimentin; Young Adult

Solitary fibrous tumor (SFT) is a rare spindle-cell benign neoplasm and located in orbit. The present research represents case reports of ten patients with orbital SFT.. Prognosis was assessed by clinic service and telephone interview. Clinical data were retrieved from the medical records. Immunohistochemistry for CD34, CD99, EMA, HMB-45, Bcl-2, GFAP, S-100, MBP, CK, and MIB-1 was performed. Distributions of time to progression and recurrence between different operative methods were estimated using the Kaplan-Meier method and compared using the log-rank test.. The ten patients included six men and four women (range: 19-75 years). The most common initial symptom was painless proptosis (n = 6). After the ten initial surgeries, with four requiring gross total resection (GRT) and six requiring subtotal resection (STR), an additional nine were required to remove recurrent tumors. Eighteen samples were obtained for pathological examination (the third sample for case 4 was not achieved). Cellular areas with partial hemangiopericytoma pattern were noted in four samples (4/18); scarce mitosis was noted in six samples (6/18). All SFTs (18/18) were positive for CD34, CD99 and vimentin, while all SFTs (18/18) were negative for EMA, CK, MBP, HBM-45 and GFAP. Bcl-2 was positive in 13/18 cases, while S-100 was negative in 14/18 cases. The MIB-1 labeling index varied from 1% to 3%. Follow-up was available for nine patients with a median of 88 months. All four patients who underwent GTR on initial operation did not recur; while the five patients who underwent STR recurred. The log-rank test showed that the incomplete surgical resection was significantly associated with recurrence (p = 0.015).. Orbital SFT is a rare mesenchymal tumor and painless proptosis is the most common initial symptom. Immunohistochemistry should be used to differentiate SFT from other tumors. Orbital SFT usually has a non-aggressive histological characteristic, and has a good prognosis if GTR is performed. Postoperative regular and long-term follow-up remains mandatory to monitor recurrence.

Topics: 12E7 Antigen; Adult; Aged; Antigens, CD; Antigens, CD34; Biomarkers, Tumor; Cell Adhesion Molecules; Diagnosis, Differential; Disease Progression; Female; Follow-Up Studies; Glial Fibrillary Acidic Protein; gp100 Melanoma Antigen; Histiocytoma, Benign Fibrous; Humans; Immunohistochemistry; Kaplan-Meier Estimate; Ki-67 Antigen; Male; Melanoma-Specific Antigens; Middle Aged; Mitotic Index; Mucin-1; Myelin Basic Protein; Neoplasm Recurrence, Local; Orbital Neoplasms; Prognosis; Reoperation; Reticulin; S100 Proteins; Solitary Fibrous Tumors; Survival Analysis; Vimentin; Young Adult