Longest survival by the combination of radiation-therapy and resection in patient with metastatic spinal paragangliomas from primary-neck lesion with succinate dehydrogenase subunit B (SDHB) mutation
| Autor: | Kazuhiro Takekoshi, Takehiro Kitagawa, Nobuharu Nishihara, Masakazu Nishigaki, Junkoh Yamamoto, Tatsuya Ohtonari |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Male
medicine.medical_specialty SDHB Endocrinology Diabetes and Metabolism medicine.medical_treatment 030209 endocrinology & metabolism Paraganglioma Lesion 03 medical and health sciences Myelopathy 0302 clinical medicine Endocrinology Biopsy medicine Humans Spinal canal Aged Spinal Neoplasms medicine.diagnostic_test business.industry Middle Aged medicine.disease Spinal cord Combined Modality Therapy Progression-Free Survival Succinate Dehydrogenase Radiation therapy Treatment Outcome medicine.anatomical_structure Codon Nonsense Head and Neck Neoplasms 030220 oncology & carcinogenesis Radiotherapy Adjuvant Radiology medicine.symptom business |
| Zdroj: | Endocrine Journal. 68:81-86 |
| ISSN: | 1348-4540 0918-8959 |
| DOI: | 10.1507/endocrj.ej20-0292 |
| Popis: | Metastatic paraganglioma (MPG) of the spine is a rare condition, with no established management. Herein, we report the longest survival case of a primary neck tumor that caused spinal MPG with a succinate dehydrogenase subunit B (SDHB) mutation (c.470delT, p.L157X) which could have promoted its malignancy. This male patient initially presented with a left neck PG which was diagnosed by a biopsy when he was 54 years-old. Simultaneously performed additional examinations revealed the spinal metastatic tumors on the T5-7 vertebrae and L3 vertebra-sacrum. These primary neck and metastatic spinal tumors' growths were once suppressed under the radiation therapy. Nineteen years later, he developed acute progressive paraparesis due to a mass located at the T2-3 level, tightly compressing the spinal cord, and protruding into the left thoracic cavity. We resected the maximum possible area of tumor in the spinal canal, confirmed MPG by histological examination, and then, we administered radiation therapy of 40 Gy in 20 fractions. Eventually, the patient was able to walk unaided with no evidential tumor recurrence for 3 years after treatment. Generally, clinical feature of MPG with SDHB mutation from abdominal lesion is thought to be poor prognosis. However, our case suggests the possibility of long-term control of spinal MPG with the adequate combination of radiation therapy and resection if metastatic lesions from primary-neck lesion with an SDHB mutation are remained to spine. |
| Databáze: | OpenAIRE |
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