Metastatic Malignant Paraganglioma: A Case Report and Review of Literature

Abstract

Metastasis is a rare presentation of non-secretory paraganglioma. Consequently, there is no standard of care for the treatment of metastatic malignant paraganglioma. The most widely used chemotherapy regimen for non-resectable cases includes cyclophosphamide, vincristine, and dacarbazine (CVD). CVD has been previously studied with variable therapeutic response. However, yttrium-90 (Y90) radioembolization has not been previously studied in the treatment of hepatic metastasis in patients diagnosed with malignant paraganglioma. This case report follows the treatment of a patient with stage IV mediastinal paraganglioma with metastasis to the liver. Treatment consisted of the CVD chemotherapy regimen and Y90 radioembolization of the hepatic lesions. After 10 cycles of CVD, the tumor size has decreased from 6.0 × 8.8 to 5.5 × 3.0 × 3.4 cm on computed tomography scan. The prominent metastatic liver lesions responded after Y90 radioembolization of both the right and left hepatic arteries. The prominent right hepatic lobe lesion has decreased in size from 2.6 × 3.4 × 3.0 cm to 2.6 × 2.8 × 2.9 cm. The prominent left hepatic lobe lesion originally measuring 1.6 cm in diameter completely resolved on follow-up imaging studies. After completion of 12 cycles of chemotherapy, the most recent positron emission testing scan determined no evidence of disease regarding both the primary mass and the hepatic lesions. This study demonstrates the first case of combination chemotherapy and Y90 radioembolization with a complete response per response evaluation criteria in solid tumors criteria. The approaches toward diagnosis and treatment corresponding to this case of malignant metastatic paraganglioma are also reviewed in this study.

Metastatic Malignant Paraganglioma Presenting as a Neck Mass Treated with Radiolabeled Somatostatin Analog

“…Several studies have demonstrated that measurement of urinary metanephrines allows for greater diagnostic sensitivity than measurement of serum metanephrines [ 1 ]. Histological studies are then performed for definitive diagnosis [ 12 ].…”

Cuando la piel avisa

“…Los sitios más comunes de diseminación metastásica pueden ser cercanos al tumor primario; aunque esto no siempre sucede, localizándose con mayor frecuencia en pared torácica anterior, pared abdominal, cabeza, cuero cabelludo, y ombligo [6][7][8][9] . En relación con el tipo tumoral presentado por nuestra paciente, los paragangliomas son tumores neuroendócrinos que derivan de células cromafines de la cresta neural y se originan en los ganglios espinales del sistema autónomo, los cuales se localizan desde la base del cráneo hasta la pelvis, pudiendo ser o no secretores de sustancias 3,12,13 . El paraganglioma metastásico cutáneo es infrecuente, pero en caso de ocurrir la localización más frecuente es en cabeza 3,4 .…”

“…Pueden tener actividad mitótica atípica, invasión intravascular y necrosis. Las MC en general no afectan epidermis y pueden tener un patrón histológico similar al primario que les dio origen 3,7,13 . La IHQ es un complemento de gran importancia en estos tumores puesto que es positiva para cromogranina, sinaptofisina y enolasa neuronal específica 3,4,7,10,13,14 .…”

Multiple cutaneous metastases on 99mTC-HYNIC-TOC scan in a rare case of malignant laryngeal paraganglioma

“…Such patients can either be managed with chemotherapy or with radionuclide therapy such as 177 Lu labeled SSTR peptide analogs or with 131 I-meta-iodobenzylguanidine. [ 12 13 ] After our scan findings, this patient was planned for chemotherapy, because the laryngeal lesion, lung nodules, and metastatic cervical lymph nodes were not concentrating 99m Tc-HYNIC-TOC.…”