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David W. Pincus, Erich O. Richter, Anthony T. Yachnis, Jeffrey Bennett, M. Tariq Bhatti and Amy Smith

Object

Although it is widely accepted that biopsy sampling is not indicated for the diagnosis and empiric treatment of diffuse pontine glioma, it is common to encounter patients with brainstem lesions that cannot be diagnosed on the basis of imaging studies alone. In cases not amenable to resection, a tissue diagnosis may still be necessary to make appropriate treatment recommendations. The authors retrospectively reviewed their institutional experience with stereotactic biopsy procedures in pediatric patients during a 4-year period.

Methods

A three-dimensional graphics workstation was used for trajectory planning to obtain biopsy samples of brainstem lesions in 10 patients. One patient experienced mild diplopia postoperatively. No other morbidity was noted; no patient died as a result of the procedure. The biopsy procedure yielded a pathological diagnosis in all cases. A later resection in one patient resulted in a change in diagnosis. Overall, the pathological findings were varied, and in some cases the tissue diagnosis altered the treatment recommendations.

Conclusions

The findings in this small series suggest that brainstem stereotactic biopsy sampling in children is a safe procedure with a high diagnostic yield. In patients in whom radiographic findings are not consistent with diffuse pontine glioma and resection is not appropriate, stereotactic biopsy sampling should be considered.

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Matthew A. Saxonhouse, Anthony T. Yachnis, David J. Burchfield, Ronald Quisling, Michael P. Sullivan and David W. Pincus

✓The authors report on a patient with a large hypothalamic hamartoma with a cleft lip and palate and seizures. Neuroimaging revealed a large extraaxial, intradural mass in the prepontine and interpeduncular cisterns with significant distortion of the brainstem. A stereotactic transfontanel needle biopsy revealed a cellular lesion that contained immature-appearing neuroepithelial cells consistent with prior descriptions of hypothalamic hamartoblastoma. While having a low level of proliferation by Ki67 (MIB-1) labeling, the lesion also contained evidence of neuronal maturation, with many cells expressing neuronal nuclear antigen as observed during immunohistochemical analysis. Further clinical evaluation revealed no other significant congenital abnormalities, and the patient was discharged home. Outpatient follow up has continued for 2 years and the patient has been doing well, requiring no further treatment. This case illustrates that, despite its immature and proliferative histological appearance, this rare neonatal mass can be regarded as a “differentiating” hypothalamic hamartoma and can have a favorable prognosis.

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Patricia T. Molloy, Anthony T. Yachnis, Lucy B. Rorke, James J. Dattilo, Michael N. Needle, William S. Millar, Joel W. Goldwein, Leslie N. Sutton and Peter C. Phillips

✓ Medulloepithelioma is an uncommon childhood tumor of the central nervous system (CNS) whose histopathological appearance has been confused with medulloblastoma and other childhood primitive neuroectodermal tumors (PNETs), but which has a vastly different clinical course. The authors have reviewed the clinical features and treatment responses of eight children with these rare tumors, the largest series to date.

In this series, the medulloepitheliomas were equally distributed between supratentorial and infratentorial primary sites. Four patients underwent gross- or near-total resections, one patient's tumor was partially resected, and one patient had biopsy only. Biopsy and ablative surgery were not attempted in two children with pontine tumors. Treatment included both radiation and chemotherapy (four patients), radiation alone (one patient), chemotherapy alone (one patient), and no postoperative treatment (two patients). Six patients died with a mean survival of 10 months and two are disease free with neurological impairment. Both long-term survivors underwent gross-total resections of their tumors. Postmortem examination revealed diffuse CNS tumor dissemination in four patients.

Medulloepithelioma, often confused with less aggressive PNETs, can mimic intrinsic brainstem glioma, responds poorly to treatment, and is prone to CNS dissemination at the time of tumor progression.

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Ellen G. Shaver, Susan L. Rebsamen, Anthony T. Yachnis and Leslie N. Sutton

✓ Sinus histiocytosis with massive lymphadenopathy was first described in 1969 by Rosai and Dorfman. The typical clinical characteristics of this disease include painless cervical lymphadenopathy, fever, and weight loss. The condition can present with an extranodal mass in about 25% of patients, and isolated masses without lymph node involvement occur rarely. The authors describe a 5-year-old boy with cavernous sinus syndrome due to an isolated extranodal form of sinus histiocytosis with massive lymphadenopathy in the temporal fossa. Several cases of this disease involving the central nervous system are reviewed. The histopathological and magnetic resonance imaging characteristics are discussed.