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

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Abstract

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.

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Author information

Authors and Affiliations

  1. Department of Neurosurgery, Loghman e Hakim Hospital, Shahid Beheshti University of Medical Sciences, Kamali Avenue, Kargar Jonubi Street, Tehran, Iran

    Mohammad Samadian, Mehrdad Hosseinzadeh Bakhtevari, Karim Haddadian & Omidvar Rezaei

  2. Department of Pathology, Day General Hospital, Tavanir Avenue, Vali Asr Street, Tehran, Iran

    Hossein Afshin Alavi

Authors
  1. Mohammad Samadian
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  2. Mehrdad Hosseinzadeh Bakhtevari
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  3. Karim Haddadian
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  4. Hossein Afshin Alavi
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  5. Omidvar Rezaei
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Correspondence to Mehrdad Hosseinzadeh Bakhtevari.

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Farahnaz Bidari, Theran, Iran

This paper has the great merit to focus on the role of interfering immunological mechanisms which induces tumoral cell apoptosis and may be a hopeful therapeutic challenge in the future.

Ramin Mahmoodi, Hannover, Germany

Whenever complete resection of the low-grade glioma is feasible, it may result in satisfactory long-term outcome. In clinical practice, critically located tumors such as hypothalamic gliomas are always considered as a challenging issue in surgical treatment. Owing to the proximity to the vital structures, a complete tumor removal is not often achievable without the risk of development of neurological complications. Partially resected low-grade gliomas may exhibit various clinical courses in follow-up. The most expected scenario is gradual growth of the tumor residue, which needs surgical intervention. Sometimes the remnants stay stable without clinical or radiological progression.

An uncommon but known behavior of a partially resected low-grade glioma is regression. Little is known about the detailed mechanism of tumor regression. No single immunological or biological mechanism could explain every aspect of this clinical condition, but it seems that induction of apoptosis is playing an important role in the regression of tumors.

In the present case report, the authors described an unusual behavior of a partially resected hypothalamic tumor (pilocytic astrocytoma) in a young boy, who experienced an immunological reaction (Stevens-Johnson syndrome) postoperatively. By reviewing and discussing the relevant articles, the authors hypothesized that immunological processes resulted in tumor regression.

Finding an effective alternative treatment has always been a desire in the management of critically located tumors. Recent advances in immunotherapy, gene therapy, and molecular biology potentially propose new treatment strategies in the management of such tumors. The importance of the current case report is to describe spontaneous complete regression of a hypothalamic tumor in a non-neurofibromatosis patient and propose an immunological reaction as a probable reason. The present case can highlight the possible role of immunologic processes in planning new treatment modalities.

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Samadian, M., Bakhtevari, M.H., Haddadian, K. et al. Spontaneous complete regression of hypothalamic pilocytic astrocytoma after partial resection in a child, complicated with Stevens-Johnson syndrome: a case report and literature review. Neurosurg Rev 39, 335–340 (2016). https://doi.org/10.1007/s10143-015-0672-9

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  • DOI: https://doi.org/10.1007/s10143-015-0672-9

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