Metastatic Spindle Epithelial Tumor with Thymus Like Differentiation in a 10 year old male

Purpose: This case study highlights a rare experience of late recurrent pulmonary metastatic Spindle Epithelial Tumor with Thymus-Like Differentiation (SETTLE) following an initial diagnosis of SETTLE of the thyroid. The case illustrates the clinical features of metastatic SETTLE and emphasizes the importance of long term patient follow up after a diagnosis of childhood cancer. Methods: Chart and literature reviews were performed with an emphasis on clinical presentation and treatment protocol for metastatic SETTLE. Records were obtained of a 10-year-old male who presented to Cook Children’s Medical Center with multiple bilateral pulmonary nodules on x-ray and a history significant for a SETTLE tumor of the thyroid 5 years prior. Due to situational adversities, the patient was lost to follow up and was not seen by an oncologist following his initial diagnosis with SETTLE. Results: Cook Children’s Medical Center Oncologist reviewed the radiologic reports and proceeded with core needle biopsy of the pulmonary masses to identify an etiology. The pulmonary biopsies were compared to the patient’s initial thyroid tumor specimen and a diagnosis of metastatic SETTLE was established. Due to the rare nature of this disease, no formal treatment protocols currently exist for metastatic SETTLE. Cook Oncologist decided to proceed with a chemotherapy protocol consistent with literature recommendations for metastatic pulmonary SETTLE. Conclusions: The present case is an example of a rare tumor that has the potential to demonstrate late onset metastatic disease. With little known about the disease course, subsequent follow-up with an oncologist after diagnosis is of the utmost importance to help monitor for tumor recurrence and metastatic disease. As demonstrated by this case, failure to identify secondary metastatic disease at an earlier stage resulted in multiple, non-resectable pulmonary tumors. This case further highlights the importance of patient enrollment in childhood cancer survivorship programs, which can help identify late onset metastatic cancer and secondary malignancies.