KCMC-FCCT Tumor Board July 2023

Again, very challenging cases were presented in July 2023.

The first case involved a 24-year-old woman with a widely metastatic adenocarcinoma with papillary features with lung liver and lymph node metastases. A biopsy was obtained from a large left supraclavicular lymph node. Immunohistochemistry on the pathology specimen is not readily available in Tanzania to confirm the site of origin of the cancer. However, imaging studies also suggested a very large exophytic right kidney mass suggestive of a renal cell carcinoma as the site of origin. Renal cell carcinoma in young adults is very rare in the United States. One of our Mayo Clinic oncologists, participating in the conference, suggested this may be a translocation renal cell carcinoma. (https://bmcurol.biomedcentral.com/articles/10.1186/s12894-022-01060-1). A rare variant of renal cell carcinoma is the 46xp11.2 chromosomal translocation variant of renal cell carcinoma. Recently recognized, this variant is primarily seen in children and young adults and has a female predisposition. Histologically it often has papillary features. Advance molecular techniques are not available in Tanzania to confirm this diagnosis but the case illustrates the wide spectrum of pathology seen at KCMC. Fortunately, first line tyrosine kinase inhibitor therapy with sunitinib is available at KCMC and could be an option for palliative treatment for this young woman.

Two other cases of soft tissue sarcoma were presented. One case involved a 50-year-old with neurofibromatosis who had a 5cm leiomyosarcoma resected from the scalp. She also had a large mass along the chest wall eroding into a rib. This had not been biopsied yet. The concern was that this also represented a soft tissue sarcoma such as a malignant peripheral nerve sheath tumor. The second case was of a 19-year-old young man with a large rhabdomyosarcoma of the right shoulder causing pain and bleeding. Shoulder disarticulation surgery was required to manage the bleeding. The discussion revolved around the need for post-operative chemotherapy and the role of radiotherapy. A recent correspondence in the Journal of Global Oncology (https://ascopubs.org/doi/full/10.1200/GO.23.00110?bid=289236149&md5=43a87e97c86eacd1b1abf77f5daec6e9&cid=DM14281) highlights the need to consider neoadjuvant and adjuvant radiation and chemotherapy in the management of soft tissue sarcomas. Thru our public-private partnership with FCCT, KCMC, the Tanzanian government and USAID, a radiation therapy facility is currently being constructed at KCMC and will improve the ability to manage sarcomas and other cancers at this institution