KCMC-FCCT Tumor Board
After a several month hiatus, the KCMC-FCCT Tumor Board resumed January 2025 with the presentation of a fascinating case of a small-round blue cell tumor in a 23 year-old man. The case highlights on-going issues with access to immunohistochemistry for accurate diagnosis. KCMC has an outstanding pathology department with the ability to develop high quality specimens for H&E and also has telepathology capabilities. However, reagents for complex immunohistochemistry, a mainstay of modern pathologic diagnosis, are often difficult to obtain.
A 23 year-old man presented with a 19cm adrenal mass with intra-abdominal and lung metastases. One year prior, he had undergone right eye enucleation for an orbital mass clinically felt to represent an optic nerve glioma. A CT-guided biopsy of the adrenal mass revealed a small-round blue cell tumor. This histologic description refers to high grade tumors with small round cells with blueish appearance under the microscope. Given the location, neuroblastoma was in the differential diagnosis. Adult adrenal neuroblastomas are very rare tumors with a dismal prognosis. Other malignancies with this histology can include high grade neuroendocrine tumors (small cell carcinoma), Ewing’s sarcoma and high-grade lymphomas. Typically, a battery of immunohistochemical studies would be used to clarify the diagnosis including neuroendocrine markers, cytokeratins and hematologic markers; however, these reagents were not available. Given the dire situation, it was elected to begin multi-agent chemotherapy directed at neuroblastoma (which also has activity in high grade lymphomas and Ewing’s). Meanwhile, urinary catecholamine studies were obtained and sent to an international laboratory. We received an update at the February tumor board that the patient has had a dramatic response to chemotherapy and that the catecholamine results returned as normal suggesting that this was not a neuroblastoma. He will continue with multi-agent chemotherapy for a likely diagnosis of high-grade lymphoma.
Randy Hurley MD, cTropMed
HealthPartners and Regions Hospital Cancer Care Centers
Global Health Faculty, University of Minnesota
5101 Vernon Ave S, Suite 501
Edina, MN 55436
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