- Department of Neurosurgery, Mount Sinai School of Medicine, New York, NY, USA
- Department of Pathology, Mount Sinai School of Medicine, New York, NY, USA
Department of Pathology, Mount Sinai School of Medicine, New York, NY, USA
DOI:10.4103/2152-7806.127968Copyright: © 2014 Skovrlj B. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Skovrlj B, Pain M, Bederson JB, Fowkes M. Pilomyxoid astrocytoma of the cerebellar vermis in an elderly patient. Surg Neurol Int 27-Feb-2014;5:29
How to cite this URL: Skovrlj B, Pain M, Bederson JB, Fowkes M. Pilomyxoid astrocytoma of the cerebellar vermis in an elderly patient. Surg Neurol Int 27-Feb-2014;5:29. Available from: http://sni.wpengine.com/surgicalint_articles/pilomyxoid-astrocytoma-of-the-cerebellar-vermis-in-an-elderly-patient/
Background:Pilomyxoid astrocytoma (PMA) has recently been accepted as an aggressive variant of pilocytic astrocytoma with distinct histopathological features. PMAs have been frequently described in the pediatric population with a predilection for the hypothalamic/chiasmatic region.
Case Description:A 72-year-old African American male presented with 6 months of memory loss, difficulty expressing himself, and a progressively worsening gait. Magnetic resonance imaging of the brain demonstrated a heterogeneously enhancing cystic mass centered within the cerebellar vermis with mass effect on the fourth ventricle and ventriculomegaly. The patient underwent placement of a ventriculoperitoneal shunt followed by a surgical resection of the lesion, which after immunohistopathologic evaluation, was diagnosed as a World Health Organization grade II PMA. The patient refused further treatment of the lesion and expired 11 months after initial symptom presentation and 4 months after surgery.
Conclusion:To our knowledge, this is the first report of PMA of the cerebellar vermis in a previously unreported age group. This case report describes the natural history of this type of tumor in a patient who refused adjuvant therapy following surgical resection.
Keywords: Cerebellar vermis, elderly patient, natural history, pilomyxoid astrocytoma
Pilomyxoid astrocytoma (PMA) represents a new clinical entity within neuro-oncology. First described in 1999, Tihan et al. identified a subset of 18 pilocytic astrocytomas with a distinctive pilomyxoid histological pattern as well as a higher recurrence rate than similar tumors with more classical pilocytic astrocytoma characteristics.[
To our knowledge, this is the first report of PMA of the cerebellar vermis in an elderly patient. Moreover, this case report describes the natural history of this type of tumor in a patient who refused additional medical treatment following surgical resection.
A 72-year-old male with a distant history of alcohol abuse presented with approximately 6 months of memory loss, difficulty expressing himself, and a progressively worsening gait. A magnetic resonance imaging (MRI) of the brain revealed a 2.5 × 3.7 × 2.5 cm heterogeneously enhancing cystic mass centered within the cerebellar vermis with mass effect on the fourth ventricle and mild ventriculomegaly [Figure
Preoperative (a) axial and (b) sagittal T1-weighted contrast enhanced magnetic resonance imaging demonstrating a 2.5 × 3.7 × 2.5 cm heterogeneously enhancing cystic mass centered within the cerebellar vermis causing marked mass effect on the fourth ventricle. Preoperative (c) coronal and (d) axial T1-weighted contrast enhanced magnetic resonance imaging demonstrating an 8 × 6 × 4 mm satellite lesion within the right superior cerebellar peduncle
Routine preoperative testing found the patient to be in rapid atrial fibrillation and he was sent to the emergency room. While undergoing evaluation of the arrhythmia, his rhythm converted to a junctional bradycardia, necessitating placement of transvenous pacemaker. In the absence of other identifiable etiologies for his sudden bradycardia, his cardiac symptoms were attributed to intracranial hypertension secondary to the obstructive hydrocephalus caused by the vermian lesion. A computed tomography (CT) scan of the head revealed ventriculomegaly but no frank hydrocephalus and the patient was taken to the operating room for placement of a ventriculoperitoneal (VP) shunt. Intraoperatively, he was found to have elevated intracranial pressure. His postoperative course was notable for immediate resolution of the cardiac arrhythmia.
Two days following placement of the VP shunt, the patient underwent a suboccipital craniectomy for resection of the cerebellar lesion. Intraoperatively, the lesion was found to be soft and mildly vascular with clean margins relative to the surrounding brain. Pathology specimens were sent for frozen histological analysis and were thought to be most consistent with a diagnosis of glioma. A postoperative MRI revealed a subtotal resection of the lesion. Permanent section of the lesion demonstrated a glial neoplasm composed of astrocytes with elongated, bipolar cytoplasmic processes, forming fascicles and focally, loose perivascular patterns, dispersed within an abundant myxoid background [Figure
Specimen resected at surgery demonstrating: (a) piloid tumor cells within a myxoid background (hematoxylin and eosin, ×10); (b) tumor cells with a perivascular orientation, lack of Rosenthal fibers identified (arrow) (hematoxylin and eosin, ×20); (c) nonreactive blood vessels surrounded by reactive tumor cells (glial fibrillary acidic protein (GFAP), ×20); (d) tumor cells with low proliferative index (Ki-67 (MIB-1), ×10)
PMAs have been recently described and accepted as a more aggressive variant of classical PA. Although both the PAs and PMAs present throughout childhood and can occur anywhere in the neuraxis, PMAs have a predilection for the hypothalamic region and tend to afflict very young children.[
Clinically, PMAs have been shown to behave more aggressively than PAs. Komotar et al.[
There is currently no consensus on the standard of care for the management of PMAs. At present, PMAs are managed in a similar manner to PAs. Gross total resection (GTR), when possible, is the primary treatment strategy. GTR has been demonstrated to be the most reliable predictor of outcome in children with low-grade gliomas when surgery can be performed without excessive morbidity.[
To our knowledge this is the first report of PMA presenting in the cerebellum of an elderly patient. Our patient underwent subtotal surgical resection of the lesion and refused postoperative adjuvant therapy. He died from complications secondary to disease progression 11 months after initial diagnosis and 4 months following surgery. This report outlines the occurrence of PMA in the adult population and emphasizes the need for aggressive postoperative adjuvant therapy in adults with subtotal tumor resection. The current treatment recommendations based on a small sample of case reports suggests that aggressive surgical resection followed by adjuvant therapy with either chemotherapy and/or radiation therapy should be tailored to individual patients based on their age and disease characteristics.
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