Cerebellar pilocytic astrocytoma in children

see Recurrence of cerebellar pilocytic astrocytoma in children.

Among child patients, up to 67% of them present with cerebellar lesions, whereas among adult patients, about 55% present with supratentorial lesions ¹⁾.

Seven patients with cerebellar pilocytic astrocytoma were associated with hemorrhage and all these cases under 15 years of ages ²⁾.

Case reports underscore the importance of considering in the differential diagnosis the possibility of spontaneous hemorrhage in a posterior fossa PA in a child presenting with a sudden neurological ictus and raised ICP ³⁾.

The purpose of a study was to identify the optimal frequency and duration of magnetic resonance imaging follow-up in children who had gross totally resected cerebellar pilocytic astrocytomas (CPAs). The hypothesis was that following two MR examinations, separated by at least 3 months, showing no evidence of tumor, gross totally resected CPAs did not recur and no further imaging follow-up was necessary. A retrospective review of the Neuro-Oncology database from 1/2000 to 7/2013 yielded 53 patients with CPAs that had preoperative imaging and >2 years post-operative imaging follow-up available. Pilocytic astrocytomas with brainstem involvement and patients with neurofibromatosis type I were excluded. Preoperative tumor volumes were calculated. The dates and reports of the examinations were tabulated. The median number of follow-up examinations was 9 over a median follow-up time of 6.05 years (2.07-12.28 years). Two consecutive MR examinations over at least a 3-month span demonstrated the smallest negative likelihood ratio of future recurrence (0.15). There was no association of recurrence with preoperative tumor volume. Among the 35 patients with gross total resection of their tumor and greater than two negative follow-up examinations, one recurrence (2.9 %) was identified, occurring 6.4 years after initial resection. Gross totally resected pediatric CPAs can recur, but this is exceedingly rare. Frequent surveillance (every 3-6 months) is suggested in patients with CPAs until the absence of a tumor is concluded on imaging and documented on two consecutive studies spaced at least 3 months apart. The likelihood of recurrence thereafter is low ⁴⁾.

Fourteen cerebellar pilocytic astrocytomas patients were compared to the age norm and to a group of 14 high-achieving peers regarding cognitive functioning, health-related quality of life (HRQoL), and stress regulation. Mean follow-up time after diagnosis was 13.29 years (range: 3-21 years).

Patients showed satisfactory academic achievement and did not differ from the norm except for the bodily dimension of HRQoL. However, there were marked differences in specific neurocognitive functions between patients and high achievers. Age at diagnosis did not influence the neurocognitive outcomes.

CPA patients treated with surgery only seem to have a favorable long-term outcome, yet, in comparison with high achievers, specific cognitive impairments become apparent ⁵⁾.

Among the 35 patients with gross total resection of their tumor and greater than two negative follow-up examinations, one recurrence (2.9 %) was identified, occurring 6.4 years after initial resection. Gross totally resected pediatric CPAs can recur, but this is exceedingly rare. Frequent surveillance (every 3-6 months) is suggested in patients with CPAs until absence of tumor is concluded on imaging and documented on two consecutive studies spaced at least 3 months apart. The likelihood of recurrence thereafter is low ⁶⁾.

While uncommon radiologic features of PA, including non-enhancing cystic tumors, have been previously described, we present a unique case of a patient with a non-enhancing solid cerebellar PA. The main clinical, radiologic, and pathologic findings are discussed and the relevant literature reviewed. To our knowledge, this is the first reported patient with these radiologic features of PA, highlighting the need for awareness of uncommon presentations when discussing differential diagnosis and pre-operative planning for cerebellar tumors in the relevant age group ⁷⁾.

Spontaneous aneurysmal subarachnoid hemorrhage in a child with multiply recurrent posterior fossa juvenile pilocytic astrocytoma ⁸⁾.

An 8-year-old girl complained of decreased hearing and difficulty hearing from her right ear while on the telephone. Pure-tone and speech audiometry, immittance (tympanometry, acoustic reflex thresholds), auditory brainstem response, and transient click-evoked otoacoustic emissions were administered. The results were suggestive of a space-occupying lesion, and the patient was referred to a pediatric neurologist and neurosurgeon. A cerebellar pilocytic astrocytoma was found. The patient's audiologic profile is described, along with implications for pediatric neurologic evaluations ⁹⁾.