Paraganglioma with highly malignant potential involving the rib - Case report and review of the literature

Abstract Paragangliomas are rare neuroendocrine tumors arising from paraganglion cells in sympathetic or parasympathetic chains, which may develop in the abdomen, chest, skull base, and neck. As paragangliomas have a wide range of imaging features, the diagnosis often requires tissue sampling. We present a unique case of a paraganglioma which originally presented as a rib tumor. A 64-year-old male with right flank pain for 2 months’ was referred for a noncontrast renal colic CT. He was found to have a 3.7 × 3.5 cm soft tissue mass invading the left posterior 9th rib and paraspinal muscle. This was fluorodeoxyglucose F 18, (18F-FDG) avid, with no other distant metabolic activity. He underwent ultrasound-guided core biopsy which revealed a diagnosis of paraganglioma. A right thoracotomy with chest wall resection of 8, 9, and 10 ribs were subsequently performed. The tumor was removed along with a small portion of adherent lung. The tumor was positive for CD56, synaptophysin and chromogranin. S-100 highlighted occasional sustentacular cells, consistent with a pathologic diagnosis of a paraganglioma. The patient remains symptom free for 6 months’ after the operation. Our case highlights that, when paragangliomas occur within the chest wall, they may present as a rib tumor and can mimic metastasis, myeloma or other primary neoplastic etiologies originating from ribs. Both imaging and pathologic diagnosis can be challenging.