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Suprasellar Primitive Neuroectodermal Tumor in an Adult
Angelina Espino Barros Palau1, Khurrum Khan, Michael L Morgan
1Department of Ophthalmology (AEBP), Centro Médico Zambrano Hellion-Tec Salud, Monterrey, Mexico; Department of Ophthalmology (KK, AGL), Baylor College of Medicine, Houston, Texas; Department of Ophthalmology, Houston Methodist Hospital, Houston, Texas; Department of Pathology and Genomic Medicine (MLM, AGL), Houston Methodist Hospital, Houston, Texas; Departments of Ophthalmology (AGL), Neurology, and Neurosurgery, Weill Cornell Medical College, Houston, Texas; Department of Ophthalmology (AGL), UTMB Galveston, Texas; The UT M.D. Anderson Cancer Center (AGL), Houston, Texas; and The University of Iowa Hospitals and Clinics (AGL), Iowa City, Iowa.
Primitive neuroectodermal tumors (PNET) are rare brain cancers in adults. This case highlights successful treatment of a suprasellar PNET in a young woman, leading to significant visual recovery.
Area of Science:
- Neuro-oncology
- Pediatric neurosurgery
- Ophthalmology
Background:
- Primitive neuroectodermal tumors (PNET) are rare central nervous system (CNS) embryonal tumors.
- Supratentorial PNETs are common in children but rare in adults.
- PNETs originate from undifferentiated or poorly differentiated neuroepithelial cells.
Observation:
- A 31-year-old woman presented with bilateral vision loss and bitemporal hemianopia.
- Neuroimaging identified a suprasellar mass.
- Pathological examination confirmed the diagnosis of PNET.
Findings:
- The patient underwent surgical debulking of the suprasellar PNET.
- Post-operative treatment included radiation therapy and chemotherapy.
- The patient experienced significant visual recovery and remained stable for 14 months.
Implications:
- This case demonstrates the feasibility of aggressive treatment for adult suprasellar PNET.
- Multimodal therapy can lead to favorable outcomes and visual improvement in rare adult CNS embryonal tumors.
- Highlights the importance of considering PNET in the differential diagnosis of suprasellar masses in young adults.
