TY - JOUR
T1 - A Rare Presentation of Recurrent Malignant Peripheral Nerve Sheath Tumor with Glandular Differentiation—A Case Report
AU - Nadaf, Asmanaz
AU - Telugu, Ramesh Babu
AU - Niaz, Zahida
AU - Al Haddabi, Ibrahim Hassan
AU - Ismail, Babikir
AU - Al-Shaibi, Maha
AU - Itkin, Boris
AU - Rassou, Salim Chaib
AU - Munir, Javeria
N1 - Publisher Copyright:
© The Author(s), under exclusive licence to Indian Association of Surgical Oncology 2024.
PY - 2024/3/14
Y1 - 2024/3/14
N2 - 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.
AB - 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.
KW - Glandular differentiation
KW - MPNST
KW - Nerve sheath tumor
KW - S100, SOX10
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UR - https://www.mendeley.com/catalogue/9c42ef48-0211-3b34-8ba9-725f21987ce6/
U2 - 10.1007/s13193-024-01914-0
DO - 10.1007/s13193-024-01914-0
M3 - Article
AN - SCOPUS:85187643075
SN - 0975-7651
JO - Indian Journal of Surgical Oncology
JF - Indian Journal of Surgical Oncology
ER -