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Synchronous Epstein-Barr virus–associated skull base and adrenal smooth-muscle tumors in an 8-year-old girl with recent Epstein-Barr virus infection
- Source :
- Journal of Neurosurgery: Pediatrics. 22:283-287
- Publication Year :
- 2018
- Publisher :
- Journal of Neurosurgery Publishing Group (JNSPG), 2018.
-
Abstract
- Epstein-Barr virus–associated smooth-muscle tumors are rare tumors seen in immunocompromised patients. Most cases occur in the context of AIDS and organ transplantation, and very rarely in the setting of congenital immunodeficiency, with only 5 case reports of the latter published so far in the literature. The authors report the case of a previously healthy 8-year-old girl with headaches and precocious puberty who was found to have a large skull base lesion. There was a synchronous left adrenal lesion. She underwent resection of the skull base lesion and a left adrenalectomy. Thorough evaluation for immunodeficiency was negative for a known congenital immunodeficiency syndrome. She had a short course of intravenous immunoglobulin and has had no recurrence of disease or new lesions in the 17 months since presentation. Continued surveillance for the development of opportunistic infections and new or recurrent lesions is warranted in this case. Repeat surgery for surgically accessible tumors or chemoradiation would be recommended for any additional lesions.
- Subjects :
- 0301 basic medicine
Epstein-Barr Virus Infections
Herpesvirus 4, Human
medicine.medical_specialty
Pathology
Tomography Scanners, X-Ray Computed
Context (language use)
Organ transplantation
Viral Matrix Proteins
Lesion
Immunocompromised Host
03 medical and health sciences
0302 clinical medicine
Antigens, CD
medicine
Humans
Precocious puberty
Child
Epstein–Barr virus infection
Smooth Muscle Tumor
Immunodeficiency
Skull Base
business.industry
Blood Proteins
General Medicine
medicine.disease
Magnetic Resonance Imaging
Skull
030104 developmental biology
medicine.anatomical_structure
Positron-Emission Tomography
030220 oncology & carcinogenesis
Female
Headaches
medicine.symptom
business
Subjects
Details
- ISSN :
- 19330715 and 19330707
- Volume :
- 22
- Database :
- OpenAIRE
- Journal :
- Journal of Neurosurgery: Pediatrics
- Accession number :
- edsair.doi.dedup.....f943e239a4b1b204faa6ae1f944d2a3c