Recurrent multiple spinal paragangliomas as a manifestation of a metastatic composite paraganglioma-ganglioneuroblastoma

Abstract

Dear editor,We describe the occurrence of multiple recurrent, aggressivespinal paragangliomas as the initial manifestation of a met-astatic composite paraganglioma-ganglioneuroblastoma in a51-year-old patient.Thepatientdevelopedprogressiveweaknessandhypesthesiaof both lower extremities. MRI demonstrated two intraspinalextradural lesions (Fig. 1a). A T4 and T6 hemilaminectomywas performed, and both intraspinal tumors were resected.During the postoperative period, the patient’s neurological def-icitsignificantlyimproved.Hi stologyrevealedaparaganglioma(Fig. 1b, c).Six months later, the patient presented with low back pain,and radicular and progressive ataxia. MRI scan revealedmultiple intraspinal extradural lesions. Hemilaminectomy wasperformed at the T6-T9 and L2-L3 level, and tumor debulkingwascarriedout.Histologicalexaminationwasconsistentwithaparaganglioma. Due to the clinical course, a staging CT wasperformed. It revealed a large tumor of the left adrenal gland,and extensive spinal and pelvic tumor dissemination (Fig. 1d).Biopsies from the adrenal tumor and from the bone marrowrevealed a composite paraganglioma-ganglioneuroblastoma(Fig. 1e). The patient received cisplatin, doxorubicin,etoposide, and endoxane chemotherapy for 9 months afterresection of the left adrenal gland, kidney, spleen, and leftcolonicflexure.Twoyearsaftertheinitialdiagnosis,thepatientdied of pneumonia after progression of the bone marrow infil-tration and subsequently compromised immune status.Paragangliomas are tumors that originate from extra-adrenal chromaffin cells or paraganglia lying adjacent tothe sympathetic ganglia [5, 9] and occur most often in thehead and neck and rarely in the retroperitoneum [6, 9].Though benign and malignant paragangliomas may have asimilar microscopic appearance, aggressive paragangliomasare histologically characterized by necrosis and vascularinvasion [5]. However, prognosis is predicted best by theclinical behavior of paragangliomas in the form of recur-rence or metastasis.Neuroblastomas are the more aggressive tumors andthe most common extracranial tumor in childhood, andthey are derived from primitive neural crest cells [4].Ganglioneuroblastomas are transitional tumors containing el-ements of malignant neuroblasts and benign ganglion cells.All neurogenic tumors except ganglioneuroblastomas andneuroblastomas are common in adults. Surgical resection ofthe primary tumor followedbyradiotherapyis recommended.Composite tumors of the adrenal medulla or paragangliaareuncommon,andtheirbiologicbehavior,treatment,andtheoutcomehasnotbeenwellelucidatedbecauseoftheirrelativerarity[4, 5, 9].These tumorshavetwo histologically different