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A Rare Presentation of Recurrent Malignant Peripheral Nerve Sheath Tumor with Glandular Differentiation—A Case Report

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Abstract

Malignant peripheral nerve sheath tumors (MPNST) are rare heterogeneous group of soft tissue neoplasms. In most cases, they originate within the pre-existing neurofibromatosis. The emergence of glandular structures in MPNST is curious and enigmatic. We report a case of recurrent MPNST with glandular differentiation arising in the background of neurofibroma in a 20-year-old lady. By immunohistochemistry, MPNST showed focal positive staining for S100 and negative staining for SOX10 while adjacent neurofibroma showed diffuse positivity for S100 and SOX10. The glandular tumor cells showed positive staining for CDX2, Cam5.2, CK19, and CK7 (focal), while negative for SOX10 and S100. MPNST with glandular differentiation is quite rare which may pose a diagnostic challenge. The glandular differentiation in MPNST should be excluded from the metastasis from second primary with the aid of clinical and radiological correlation.

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The authors received no financial support for the research, authorship, and/or publication of this article.

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Authors and Affiliations

  1. Department of Pathology, Laboratory Services, Sultan Qaboos Comprehensive Cancer Care and Research Center, Muscat, Oman

    Asmanaz Nadaf, Ramesh Babu Telugu, Zahida Niaz, Ibrahim Hassan Al Haddabi & Babikir Ismail

  2. Department of Surgical Oncology, Sultan Qaboos Comprehensive Cancer Care and Research Center, Muscat, Oman

    Maha Al-Shaibi

  3. Department of Medical Oncology, Sultan Qaboos Comprehensive Cancer Care and Research Center, Muscat, Oman

    Boris Itkin

  4. Department of Radiation Oncology, Sultan Qaboos Comprehensive Cancer Care and Research Center, Muscat, Oman

    Salim Chaib Rassou

  5. Department of Radiodiagnosis, Sultan Qaboos Comprehensive Cancer Care and Research Center, Muscat, Oman

    Javeria Munir

Authors
  1. Asmanaz Nadaf
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  2. Ramesh Babu Telugu
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  7. Boris Itkin
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Correspondence to Ramesh Babu Telugu.

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Nadaf, A., Telugu, R.B., Niaz, Z. et al. A Rare Presentation of Recurrent Malignant Peripheral Nerve Sheath Tumor with Glandular Differentiation—A Case Report. Indian J Surg Oncol 15, 414–419 (2024). https://doi.org/10.1007/s13193-024-01914-0

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  • DOI: https://doi.org/10.1007/s13193-024-01914-0

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