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Joshua M. Ammerman, Russell R. Lonser, James Dambrosia, John A. Butman and Edward H. Oldfield

Object

In the course of their lives most patients with von Hippel–Lindau (VHL) disease require treatment for several symptom-producing hemangioblastomas of the cerebellum, brainstem, or spinal cord. However, many tumors never produce symptoms and do not require treatment. Detection at an early stage of lesions that will later produce symptoms and ultimately require treatment would allow for earlier excision of hemangioblastomas of the spinal cord, brainstem, or cerebellum, and may identify cerebellar hemangioblastomas that can be treated with radiosurgery at a stage before treatment is contraindicated because of tumor size or the presence of an associated cyst.

Methods

To identify features predictive of symptom development that might allow for earlier treatment of smaller, presymptomatic hemangioblastomas in patients with VHL disease, the authors reviewed and analyzed the serial clinical and imaging findings in all patients with VHL disease who were followed up at the National Institutes of Health for more than 10 years. Features predictive of symptom formation were determined by recursive partition and regression analyses.

Nineteen patients (10 men and nine women; mean age 32.6 ± 11.6 years) harboring a total of 143 hemangioblastomas were identified (mean follow-up duration 12.4 ± 1.4 years). Hemangioblastomas were located in the cerebellum (68 hemangioblastomas, 48% of patients), brainstem (17 hemangioblastomas, 12% of patients), and spinal cord (58 hemangioblastomas, 40% of patients). Despite measurable growth in almost all hemangioblastomas (138 lesions, 97% of patients), only 58 (41% of patients) became symptomatic. Hemangioblastomas grew in a stuttering pattern. (mean growth period 13 ± 15 months, mean quiescent period 25 ± 19 months). Twenty-six (45%) of the hemangioblastomas that eventually produced symptoms were not among the tumors that were apparent on the initial MR imaging study. Depending on location, the hemangioblastoma size and/or tumor and cyst growth rates predicted symptom development and the need for treatment (p < 0.05). Cerebellar hemangioblastomas growing faster than 112 mm3/ month or larger than 69 mm3 with associated tumor and cyst growth rates greater than 14 mm3/month became symptomatic (100% sensitivity, 72% specificity). Brainstem hemangioblastomas larger than 245 mm3 with growth rates greater than 0.1 mm3/month became symptomatic (75% sensitivity, 89% specificity). Spinal hemangioblastomas larger than 22 mm3 became symptomatic (79% sensitivity, 94% specificity).

Conclusions

Because hemangioblastomas exhibit a stuttering growth pattern, frequently remain asymptomatic, and do not require treatment for long intervals, unqualified radiographic progression is not an indication for treatment. Basing the decision to intervene in individual tumors solely on radiographic progression would have resulted in approximately four additional procedures per patient during the 10-year study period. Threshold values are presented for tumor size and/or tumor and cyst growth rates that can be used to predict symptom formation and future need for treatment.

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Alfredo Pompili, Andrea Pace and Emanuele Occhipinti

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Joshua M. Ammerman, Russell R. Lonser and Edward H. Oldfield

Object. To overcome the limitations associated with surgical approaches that have been described for accessing intraparenchymal lesions of the anteromedial region of the superior cerebellum, the authors used a posterior subtemporal transtentorial approach to remove tumors in this region. In this paper they describe the surgical technique that they used as well as the operative findings and clinical outcomes observed in patients who underwent resection of tumors in the anteromedial superior cerebellum.

Methods. The consecutive patients with anteromedial superior cerebellar tumors who underwent resection performed using the posterior subtemporal transtentorial approach at the National Institutes of Health were included in this study. Clinical, neuroimaging, and operative results were analyzed.

Three patients (two men and one woman) with anteromedial superior cerebellar tumors (two hemangioblastomas and one pilocytic astrocytoma) underwent resection via this approach. All the tumors were larger than 3 cm in diameter (range 3.1–3.5 cm). This approach provided excellent surgical access and permitted complete tumor resection in each case. The patients remained neurologically unchanged compared with preoperative baseline findings at the last follow-up examination (conducted at 4, 18, and 42 months postoperatively). One patient displayed a mild transient confusion immediately after surgery, but it resolved within 6 days.

Conclusions. The posterior subtemporal transtentorial approach provides excellent access to the anteromedial superior cerebellar region. This approach permits resection of large lesions in this location, while avoiding many of the limitations associated with other approaches to this site.

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Roberto C. Heros