Background: Cardiac paraganglioma is a rare entity of an uncommon neuroendocrine tumor. Clinically, non-secreting tumors are often diagnosed because of their growth effects, secreting tumors present symptoms related to catecholamine. Correct diagnosis of a paraganglioma can be reached by biochemical investigations and imaging. Surgical resection is the treatment of choice and has to be planned carefully and interdisciplinarily. Aim: On the basis of a patient with a vague clinical presentation and an unclear situation after primary investigations, we highlight the diagnostic challenge of this rare subset of paragangliomas. Case presentation: We present the case of a 42-year-old woman whose unspecific symptoms and further investigations revealed a paracardiac mass with unknown local behavior and dignity. Surgical resection and histopathological examination led to the diagnosis of a cardiac paraganglioma. Conclusion: Cardiac paragangliomas are extremely rare, but may be treated curatively by resection after careful preoperative investigation and interdisciplinary planning.
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