gms | German Medical Science

Objective: To describe two pediatric cases of holocord pilocytic astrocytoma with completely different clinical and biological behavior. The surgical indication and management strategy of this rare tumor entity are critically discussed.

Methods: Two cases of young female patients (11 and 13 years) harboring a holocord pilocytic astrocytoma are described. Clinical, imaging, surgical and histopathological, data were analyzed in the preoperative, postoperative and follow-up period. A Pubmed analysis was performed and the literature was reviewed.

Results: In both cases a gross total resection of the tumor in its whole extension was achieved. The neuropathological work-up of both tumors revealed a pilocytic astrocytoma (WHO I). In both cases ostentatious presence of Rosenthal fibers and eosinophilic granular bodies effectively excluded pilomyxoid astrocytoma. Moreover, both showed an almost identical textbook morphology of pilocytic astrocytoma with a low proliferative activity. However, in the first case the holocord pilocytic astrocytoma regrew rapidly after surgery and further progressed despite chemotherapy. In the second case the tumor remnants were totally stable in MRI surveillance over 18 months and even regressed in some areas with regard to contrast enhancement.

Conclusions: Although pilocytic spinal cord astrocytoma is a neuropathological entity, it can present with very different biological courses. Nevertheless, early, often multistaged, IOM-guided microsurgical removal should be performed as long as the patient shows only little neurological deficits. Excellent results may be achieved by surgery alone, but if the tumor behaves clinically aggressive and recurs within a short time period, the patient should undergo additional chemotherapy. In case of chemoresistance, radiotherapy has to be discussed.