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Adenocarcinoma of the thymus (AT) is a rare and aggressive neoplasm that can be difficult to diagnose and treat. Recently, an enteric type of AT with unique histopathological features was identified, which can be confused with gastrointestinal carcinomas, leading to delays in diagnosis and treatment. This poses a challenge for medical oncologists and pathologists. We present the case of a 54-year-old woman who was diagnosed with enteric-type adenocarcinoma of the thymus and treated with induction chemotherapy, followed by concurrent chemotherapy and radiotherapy. This resulted in excellent outcomes, highlighting the importance of precise characterization and molecular profiling in guiding treatment.