Introduction: Mediastinal masses represent a difficult challenge in terms of their diagnosis and treatment. We present a case of a patient clinically mimicking as achalasia.
Case report: A 61-year-old man, with symptoms interpreted as achalasia, presented a huge mediastinal mass suspicious for sarcoma. Only definitive pathological examination after radical surgical resection evidenced a high-grade pleomorphic sarcoma of uncertain origin.
Discussion: Mediastinum is a rare primary site of sarcoma, clinical diagnosis and pathological classification remain challenging. Surgery is still the only treatment affecting survival, even if it is not always possible. In our experience, surgical radical removal was possible after a multicompartmental approach and the patient is alive with no evidence of disease after nine months from surgery.
Conclusion: Diagnosis of mediastinal sarcoma is still very difficult because of lack of pathognomonic symptoms and diagnostic work-up, clinical and pathological heterogeneity. Surgical aggressive approach is the only therapeutic option to extend survival.

Keywords: Mediastinal sarcoma; Huge mass; Rare tumor