- Department of Pathology, University of Miami Miller School of Medicine, Miami, Florida, United States.
- Department of Neurosurgery, University of Miami Miller School of Medicine, Miami, Florida, United States.
Khadeja Khan, Department of Pathology University of Miami Miller School of Medicine, Miami, Florida, United States.
DOI:10.25259/SNI_423_2021Copyright: © 2021 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Khadeja Khan, Evan Luther, Alexis A. Morrell, Sze Kiat Tan, Daniel G. Eichberg, Ashish H. Shah, Victor M. Lu, Sakir H. Gultekin, Jacques J. Morcos. Recurrent adult pilocytic astrocytoma presenting with intraventricular and leptomeningeal spread. 19-Jul-2021;12:359
How to cite this URL: Khadeja Khan, Evan Luther, Alexis A. Morrell, Sze Kiat Tan, Daniel G. Eichberg, Ashish H. Shah, Victor M. Lu, Sakir H. Gultekin, Jacques J. Morcos. Recurrent adult pilocytic astrocytoma presenting with intraventricular and leptomeningeal spread. 19-Jul-2021;12:359. Available from: https://surgicalneurologyint.com/surgicalint-articles/10979/
Background: Infratentorial pilocytic astrocytomas are uncommon tumors in adulthood but are thought to be prognostically similar to their pediatric counterparts with excellent overall survival following gross total resection. However, given the relative rarity of these tumors, no management guidelines exist for recurrent adult pilocytic astrocytomas (APAs). This lack of consensus is especially problematic for inoperable recurrences or those with aggressive features concerning for malignant transformation.
Case Description: In 2017, a 26-year-old female presented with headaches, nausea, vomiting, and blurry vision. A brain magnetic resonance imaging (MRI) demonstrated a large, well-circumscribed mass within the fourth ventricle causing obstructive hydrocephalus. She underwent near-total resection through a midline suboccipital transtonsillar approach. Pathology demonstrated a World Health Organization Grade 1 pilocytic astrocytoma. Despite initial improvement in her symptoms, she developed worsening headaches and lethargy 10 months after surgery and repeat MRI demonstrated recurrent tumor within the entire ventricular system and the subarachnoid spaces of the left cerebellopontine angle suggesting leptomeningeal spread. Due to the unresectable nature of the recurrence, the patient declined any further intervention and succumbed to her disease 6 months later.
Conclusion: We present the first case of a recurrent APA presenting with intraventricular and leptomeningeal spread. Although thought to be a benign neoplasm, close interval follow-up with serial imaging is of essential, especially in those patients with known residual tumor, to prevent aggressive recurrences such as this.
Keywords: Adult pilocytic astrocytoma, Inoperable recurrence, Leptomeningeal spread, Malignant transformation, Subtotal resection
Pilocytic astrocytomas are one of the most common benign brain neoplasms of childhood but remain exceedingly rare in adults accounting for only 0.8% of central nervous system tumors in patients >20 years of age.[
An otherwise healthy 26-year-old female presented in 2017 with severe headaches, nausea, vomiting, and visual changes. After fundoscopic examination demonstrated Grade 5 papilledema, contrast-enhanced brain magnetic resonance imaging (MRI) was performed revealing a 4.8 × 4.4 × 4.1-cm mass centered in the fourth ventricle resulting in obstructive hydrocephalus [
Imaging performed in April 2017. (a) Preoperative T1 contrast enhancing MRI demonstrating a well-circumscribed lesion within the fourth ventricle. (b) Preoperative T2 MRI demonstrating no significant surrounding edema or infiltration of the normal brain parenchyma. (c) Preoperative DWI demonstrating no diffusion restriction. (d) Postoperative T1 contrast enhancing MRI demonstrating no obvious residual tumor.
Imaging performed in March 2018. (a) T1 contrast-enhanced MRI demonstrating tumor recurrence within the frontal horns of the lateral ventricle. (b) T1 MRI demonstrating recurrent tumor within the third ventricle. (c) T1 MRI demonstrating leptomeningeal enhancement in the left cerebellopontine angle and temporal horns.
Pilocytic astrocytomas are one of the most common gliomas of childhood. However, in adults, they are much less frequent with an incidence of 3.4/1 million.[
Several studies have shown that increasing patient age is correlated with lower rates of overall survival in pilocytic astrocytomas with 5-year survival dropping from 96.5% in pediatric patients to approximately 53% in patients older than 60.[
In the pediatric population, several retrospective series examined the benefit of surveillance imaging for pilocytic astrocytomas and concluded that patients with a gross total resection have a very low likelihood of recurrence and may not benefit from long-term surveillance.[
Little data exist to help guide postsurgical adjuvant therapy for subtotally resected APAs. Radiation therapy (XRT) has been evaluated in several studies with mixed findings. A recent study evaluating overall survival following subtotal resection of APAs found that those patients who received postoperative XRT had significantly higher mortality rates compared to those that received surveillance alone.[
Although no standard chemotherapeutic regimen exists for APAs, various drug combinations, with or without XRT, have been used in recurrent/inoperable pilocytic astrocytomas with varying degrees of effectiveness.[
Leptomeningeal disease in recurrent APAs remains significantly difficult to manage because, by definition, it is unresectable. Biopsy may be warranted to determine if the lesion has malignantly transformed. In addition, molecular testing may also increase diagnostic and prognostic accuracy as DNA methylation has been shown to help determine tumor grade when histologic features alone remain ambiguous.[
In our patient, the tumor involved the fourth ventricle and resection may have led to leptomeningeal spread within the CPA. However, this does not fully explain how the tumor casted the entire ventricular system. Given that the patient was shunted for hydrocephalus, this may have led to ventricular seeding yet this would likely have tracked along the catheter rather than involving even the contralateral lateral ventricle.[
We report a rare case of a recurrent APA presenting with diffuse intraventricular and leptomeningeal spread. This atypical and fatal case highlights the need for improved management guidelines and better diagnostic criteria for these tumors in the adult population. Novel molecular profiling may help detect subtle differences in tumor grade and, in turn, better direct adjuvant therapy in patients with known residual or recurrent disease. Although thought to be a benign neoplasm, close interval follow-up with serial imaging may be useful in those patients with known residual tumor.
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