Unresectable Primary Enteric-Type Thymic Adenocarcinoma Treated With FOLFOX Chemotherapy: A Case Report

Background

Enteric-type thymic adenocarcinomas are an extremely rare and distinct subtype of thymic malignancies, as classified by the 2021 World Health Organization classification of thymic tumors. These tumors exhibit close molecular and morphologic similarity to primary gastrointestinal malignancies. To date, there are no tailored treatment guidelines for enteric-type thymic adenocarcinoma.

Case

A 65-year-old woman was admitted to the Oncology unit of a Melbourne Metropolitan Hospital after presenting with progressive symptoms of thoracic outlet syndrome, where a CT scan identified a localized anterior mediastinal mass measuring up to 7.5 × 6.0 × 6.0 cm (transverse × Anterior–Posterior × Superior–inferior). The mass was deemed unresectable. A core biopsy diagnosed the patient with a primary enteric-type thymic adenocarcinoma, based on positive immunohistochemical staining for the intestinal markers CK20 and CDX2, positive staining for CD5—a marker commonly associated with thymic carcinoma, and positive staining for CK7—a marker not classically expressed in colonic adenocarcinoma. The patient received five fractions of 20 Gray palliative radiotherapy for superior vena cava syndrome, followed by four cycles of Oxaliplatin/5-fluorouracil/leucovorin (FOLFOX) chemotherapy. FOLFOX, a common systemic therapy for gastrointestinal adenocarcinomas, was chosen due to the tumor's enteric profile and was selected over the then-NCCN-recommended carboplatin/paclitaxel regimen, which did not have trial-backed evidence of efficacy in thymic adenocarcinomas, particularly adenocarcinomas of the enteric type. After starting on frontline systemic therapy with FOLFOX, restaging scans after Cycle 3 showed a reduction in the size of her anterior mediastinal mass, which further decreased in size after cycle 4. Treatment was then discontinued per the patient's goals of care; however, follow-up imaging showed ongoing disease stability for 6 months. The patient died of disease 8 months after treatment discontinuation and 13 months after her initial diagnosis.

Conclusion

We report the first case of primary enteric-type thymic adenocarcinoma treated at an Australian institution, and the first published case using FOLFOX as frontline systemic therapy in a patient with unresectable primary enteric-type thymic adenocarcinoma. Noting the close molecular resemblance between enteric-type thymic adenocarcinoma and gastrointestinal malignancies, chemotherapy regimens commonly used in the treatment of gastrointestinal cancer may offer a more effective option than standard frontline therapies used in thymic carcinomas for the management of enteric-type thymic adenocarcinoma.