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Case Report
A giant occipital malignant peripheral nerve sheath tumor in non-neurofibromatosis person: A rare case and review of literature
1 Professor, Department of Surgery, Nnamdi Azikiwe University, Awka, Nigeria
2 Lecturer, Department of Surgery, Nnamdi Azikiwe University, Awka, Nigeria
3 Senior Resident, Department of Surgery, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Anambra State, Nigeria
4 Resident, Department of Surgery, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Anambra State, Nigeria
Address correspondence to:
JKC Emejulu
Department of Surgery, Nnamdi Azikiwe University, Awka,
Nigeria
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Article ID: 101157Z01JE2020
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Emejulu JKC, Nkwerem SPU, Ekweogwu OC, Okpalike IV, Nwosu RC, Okwunze CL. A giant occipital malignant peripheral nerve sheath tumor in non-neurofibromatosis person: A rare case and review of literature. Int J Case Rep Images 2020;11:101157Z01JE2020.ABSTRACT
Introduction: Malignant peripheral nerve sheath tumor (MPNST) is an aggressive tumor which is often associated with neurofibromatosis type-1 (NF-1). This rare tumor is often associated with nerve roots and bundles in the extremities. Occipital MPNST is rare. Giant size occipital scalp MPNST is extremely rare in literature. The size of tumor often becomes difficult to treat. We present one of the most gigantic occipital MPNSTs (14 × 27 × 44 cm) published in the literature.
Case Report: A 27-year-old lady who presented with a huge occipital mass and a history suggestive of bilateral pathological fracture of the tibia. At presentation her occipital mass measured 14 × 27 × 44 cm. Cranial computed tomography (CT) showed evidence of calvarial erosion. She had subtotal tumor resection with histology suggestive of MPNST while immunohistochemistry was positive for S-100. She was referred to the oncology unit for chemoradiation.
Conclusion: Huge occipital MPNST presents management challenges because treatment is difficult. This giant MPNST has been added to the body of knowledge while the search for the ultimate solution continues.
Keywords: Giant, Malignant peripheral sheath tumor, Non-neurofibromatosis 1, Occipital
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Author Contributions
JKC Emejulu - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
SPU Nkwerem - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
OC Ekweogwu - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
IV Okpalike - Conception of the work, Design of the work, Analysis of data, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
RC Nwosu - Acquisition of data, Analysis of data, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
CL Okwunze - Acquisition of data, Analysis of data, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2020 JKC Emejulu et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
