Recommendations for Diagnosing and Treating Rare Histiocytic Neoplasms

Ronald S. Go, MD, hematologist/oncologist at the Mayo Clinic in Rochester, Michigan, discusses what community oncologists should know regarding the guidelines on managing patients with histiocytic neoplasms.

At the end of 2021, The National Comprehensive Cancer Network (NCCN) released Clinical Practice Guidelines in Oncology for histiocytic neoplasms which provide recommendations on assisting, diagnosing, and treating adult patients with histiocytic neoplasms. The NCCN compiled data from small retrospective studies, case series, and case reports to help create the guidelines.

According to Go, diagnosing this rare group of heterogeneous cancers can be challenging. He recommends experts keep in mind that molecular studies, including next-generation sequencing for mutations that are common in these diseases, can be very helpful when performed in addition to microscopic diagnosis, and immunohistochemistry staining.

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0:08 | One thing that we have learned about these conditions is that the diagnosis can be challenging. For example, it is not uncommon for patients to require multiple biopsies in order to arrive at a histopathologic diagnosis. This is because the tumor cells or the cancer cells are quite scanty in these lesions, whether it's in the bone or the lymph node, or the kidneys, and therefore, multiple biopsies are required.

0:41 | The other thing that we have learned about these conditions is that in addition to microscopic diagnosis, and immunohistochemistry staining, molecular studies, such as next-generation sequencing for mutations that are common in these diseases, like BRAF, or MAP kinase pathway mutations are very helpful. Oncologists should perform next-generation sequencing, and if common mutations are found, it helps solidify the diagnosis even though under the microscope or the histopathologic diagnosis case, the pathologic features may not be completely diagnostic. Finally, it helps to have the pathology slides be reviewed. Expert centers can help with this.