Rare Diseases Symptoms Automatic Extraction

The long-term outcomes of radiosurgery for intracranial hemangioblastomas.

[von hippel-lindau disease]

Optimal timing in the treatment of intracranial hemangioblastoma (HB) remains controversial, particularly for patients of von Hippel-Lindau disease (VHL) with multiple small lesions. We evaluated efficacy of stereotactic radiosurgery (SRS) for intracranial HB based on the longer follow-up data with a larger number of patients and lesions.Twenty-one patients (11 men, 10 women) initially underwent SRS for 57 intracranial HBs. Seven patients had sporadic lesions and 14 had VHL-related lesions. During the follow-up, 40 lesions were additionally treated in VHL patients in 10 SRS treatments. Thus, a total of 97 lesions were included in this study. Median tumor volume was 0.13 cm(3) (range, 0.004-9.5 cm(3)), and median margin dose was 18 Gy (range, 14-20 Gy).Median duration of follow-up was 96 months (range, 3-235 mo) after initial SRS treatment. Ten tumors in 7 patients showed progression after SRS. Actuarial tumor control rates after SRS at 5 and 10 years were 94% and 80%, respectively. Factors associated with longer control were solid lesion (P = .03), smaller volume (P = .01), and lesions associated with VHL (P = .0005) in univariate analysis. Five- and 10-year tumor control rates were 67% and 44% for sporadic patients and 97% and 83% for VHL patients.SRS could be offered as an effective treatment for small, solid, and VHL-associated HBs. If the tumors show apparent enlargement in size or can possibly become symptomatic along with a slight increase in size, SRS should be recommended before they present with the clinical symptoms.

Diseases presenting "enlargement in size" symptom

  • von hippel-lindau disease

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