Pilocytic astrocytoma (PA) is one of the most common central nervous system (CNS) tumors in childhood, accounting for 21-23% of all pediatric CNS tumors. PA is a low grade glioma often characterized by solid and cystic components. Current treatment strategies include surgery, chemotherapy, and/or radiation. Although chemotherapy and radiation are effective against the solid component, they show limited activity on the cystic component. Management of cystic tumor progression often involves surgical intervention and can be problematic. Bevacizumab is a monoclonal antibody that binds vascular endothelial growth factor (VEGF) and prevents formation of new blood vessels. In this case series, we present three patients with PAs and cystic progression, who were treated with bevacizumab from 2012 to 2015. Patient 1 has an optic pathway glioma (OPG) treated with two chemotherapy regimens, multiple surgical procedures for cyst fenestration and placement of an Ommaya reservoir. Patient 2 has a midbrain PA treated with subtotal surgical resection, multiple chemotherapy regimens, and gamma knife radiation. Patient 3 has an OPG treated with two chemotherapy regimens, tumor debulking, and cyst fenestration. All three patients were treated with bevacizumab every two weeks with improvement in the cystic components of their tumors as shown by magnetic resonance imaging. In this case series, we report three patients with significant history of refractory disease in which bevacizumab was well-tolerated and showed substantial improvement in the cystic component of their tumor. We believe bevacizumab is a promising agent for cyst control in patients with pilocytic astrocytoma.
Collision tumors are rare tumors comprised of two distinct histologies. In this case report, we discuss a suprasellar collision tumor consisting of adamantinomatous craniopharyngioma and supratentorial ependymoma in a pediatric patient. Case Presentation: Our patient was a two-year-old female with progressive craniopharyngioma status post cyst decompression with Ommaya reservoir placement, subcutaneous peginterferon, Ommaya taps, and subtotal resection. An MRI three months post-resection showed progression and treatment was started with subcutaneous interferon alfa. After eight weeks, she presented with new onset headaches and vomiting. MRI demonstrated tumor progression with associated obstructive hydrocephalus. She underwent a subtotal resection. Pathology revealed recurrent adamantinomatous craniopharyngioma and a 0.5cm ependymoma with classic histomorphology lacking anaplasia features. The ependymoma was positive for GFAP immunostain and EMA immunohistochemistry highlighted a ‘dot-like’ reaction. The Ki-67 proliferation index was very low (<1%). The limited diagnostic material precluded further genomic characterization of the ependymoma. The previous pathology was reviewed and no ependymoma was identified. Spine MRI was negative for metastatic disease. CSF cytology was negative for malignant cells. Following recovery from surgery, she received 54Gy (RBE) focal proton radiation. Eight months from completion of therapy, surveillance MRI shows stable residual tumor. Genetic work-up for cancer predisposition syndrome is in process despite no strong family history of cancer. Discussion: Due to the patient’s young age at diagnosis, our initial treatment strategy was to delay radiotherapy and utilize other treatment options. Following diagnosis with a collision tumor, the patient proceeded to radiotherapy to manage both tumor components. The role of interferon in the development of a collision tumor in this patient in unknown, but we suspect it to be unrelated. Conclusion: To our knowledge, this is the first documented case of a suprasellar collision tumor comprised of craniopharyngioma and ependymoma. Discovery of the collision tumor impacted the patient’s treatment plan.
Current strategies for managing craniopharyngioma result in significant morbidity. Successful treatment with interferon alfa(INFα) after progression is reported in the literature. This retrospective review details our institutional experience with INFα in craniopharyngioma patients. Method: Between 2000-2021, we treated 81 craniopharyngioma patients. Twenty-two patients received 26 treatment courses of subcutaneous INFα. Twenty-three courses were evaluable for response. Results: Ten patients received upfront INFα after cyst decompression +/- ommaya placement. Progression free survival(PFS) ranged between 7-38mo. Three patients continue on treatment (10+, 12+, 14+mo); seven progressed (four on treatment (7, 9, 25, 38mo), three after treatment (13, 19, 32mo)). At progression, three underwent surgery alone, three underwent surgery and radiation, one resumed INFα. Thirteen patients received INFα after progression. Prior to INFα, eight patients had had surgery, five surgery and radiation. Two in each group had INFα, previously. PFS ranged between 5-82+mo. One patient remains on treatment (5+mo); four continue in follow-up without progression (23+,40+,64+,82+mo) with two patients avoiding radiation to date; eight progressed (three on treatment (6-8mo), five after treatment (16,24,26,46,71mo)). At progression, two underwent surgery alone, three underwent surgery and radiation, one received re-irradiation, two resumed INFα. While receiving INFα, two patients experienced serious adverse events (one intra-tumoral hemorrhage (not attributed to INFα), one suicidal ideation). Both recovered. One tolerated retreatment with INFα. Three additional patients stopped INFα for intolerance, but two received INFα at subsequent progression. No other unanticipated side effects were reported. Conclusion: INFα therapy in patients with both newly diagnosed and progressive craniopharyngioma delayed the need for aggressive surgical resection and/or radiotherapy in some cases. In some patients, INFα resulted in prolonged stabilization of disease delaying or avoiding radiation. Overall, INFα side effects were manageable. These results are encouraging regarding INFα therapy for patients with craniopharyngioma and warrant further evaluation with a clinical trial.
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