AI Article Synopsis
- Primary spindle cell and pleomorphic sarcomas of bone are rare types of cancers, which include malignant peripheral nerve sheath tumors, particularly noted outside the head and neck area.
- The study involved eight cases of these tumors from archives, detailing their clinical, radiological, and morphological characteristics.
- Findings indicate that these tumors are aggressive, with a median survival of only seven months for the majority of patients, emphasizing the need for further research into more effective treatment options.
Article Abstract
Primary spindle cell and pleomorphic sarcomas of bone represent an exceedingly rare group of mesenchymal malignancies that include soft tissue histotypes, as malignant peripheral nerve sheath tumor. Outside the head and neck region, only 36 cases of primary malignant peripheral nerve sheath tumor of bone have been described. We retrieved from our archives eight cases of primary malignant peripheral nerve sheath tumor of bone arising outside the head and neck region, describing their clinical, radiological, and morphologic features. Our series, in which all but one patient died of diseases after a median of seven months, confirms that primary malignant peripheral nerve sheath tumors of bone are aggressive tumors. Pathologists should be aware of this rare histotype. More aggressive and active adjuvant treatments should be investigated.
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| http://dx.doi.org/10.1016/j.humpath.2022.02.003 | DOI Listing |
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