Spontaneous complete regression of hypothalamic pilocytic astrocytoma after partial resection in a child, complicated with Stevens-Johnson syndrome: a case report and literature review

• Published in: Neurosurgical review 2016-04, Vol.39 (2), p.335-340
• Main Authors: Samadian, Mohammad, Bakhtevari, Mehrdad Hosseinzadeh, Haddadian, Karim, Alavi, Hossein Afshin, Rezaei, Omidvar
• Format: Article
• Language: English
• Subjects: Astrocytoma - diagnosis; Astrocytoma - pathology; Astrocytoma - surgery; Case Report; Cerebellar Neoplasms - diagnosis; Cerebellar Neoplasms - pathology; Cerebellar Neoplasms - surgery; Child; Humans; Magnetic Resonance Imaging - methods; Male; Medicine; Medicine & Public Health; Neoplasm, Residual - surgery; Neurosurgery; Stevens-Johnson Syndrome - diagnosis; Stevens-Johnson Syndrome - pathology; Stevens-Johnson Syndrome - surgery; Treatment Outcome
• ISSN: 0344-5607; 1437-2320
• DOI: 10.1007/s10143-015-0672-9

Pilocytic astrocytoma (PA) is the most common pediatric central nervous system glial neoplasm and the most common pediatric cerebellar tumor. The spontaneous regression that occurs after partial/subtotal resection is multifactorial, depending on multiple factors, as for the case of humoral and cell-mediated immune responses of the host to the implanted tumor. A 7-year-old boy was referred to a neurosurgery clinic with headache. Further imaging workup revealed hypothalamic PA. Partial resection of the lesions was performed with right-side pterional approach. The patient developed a severe panmucositis [Stevens-Johnson syndrome (SJS)] and respiratory failure plus conjunctivitis, due to phenytoin allergy. During the patient’s 6-month follow-up, postoperative magnetic resonance imaging (MRI) revealed a residual tumor, and about 9 months later (at 15 months postoperatively), the MRI showed total regression of the tumor. Clinically, symptomatic PA may undergo spontaneous regression after partial resection. We report a well-documented case of spontaneous regression hypothalamic PA after partial resection that complicated with SJS. Immune system reaction in SJS may have a role in tumor behavior and spontaneous regression. Multiple studies confirmed spontaneous regression in PA after partial/subtotal resection. This phenomenon occurs due to humoral and cell-mediated host immune responses to the implanted tumor. The immune system reaction in SJS may have a role in tumor behavior and spontaneous regression.