Efficacy of oral metformin in a patient with metastatic adrenocortical carcinoma: Examination of mechanisms and therapeutic implications
| Autor: | Racy Dj, Sousa Tt, Gomes Lm, Nagourney Ra, Shigenaga M, Peixoto Rd |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
Oncology medicine.medical_specialty Adrenocortical carcinoma Histology lcsh:RC254-282 Article 03 medical and health sciences 0302 clinical medicine Internal medicine medicine Neoplasm Doxorubicin Mitotane Li-Fraumeni syndrome neoplasms Etoposide Cisplatin business.industry nutritional and metabolic diseases medicine.disease lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens Metformin 030104 developmental biology Li–Fraumeni syndrome 030220 oncology & carcinogenesis business metformin medicine.drug |
| Zdroj: | Rare Tumors Rare Tumors, Vol 10 (2018) |
| ISSN: | 2036-3613 2036-3605 |
| Popis: | Although rare, adrenocortical carcinoma is among the most common tumors found in children with Li-Fraumeni syndrome and Li-Fraumeni-like syndrome, associated with germ-line mutations in the TP53 gene. In southern Brazil, one form of Li-Fraumeni syndrome, associated with childhood adrenocortical carcinoma, is caused by a mutation in the R337H TP53 tetramerisation domain and is attributed to a familial founder effect. Adrenocortical carcinoma is considered an aggressive neoplasm, usually of poor prognosis and is generally unresponsive to systemic chemotherapy. Optimal treatment regimens remain to be established. We report the case of a young woman with metastatic adrenocortical carcinoma, who achieved stable disease with mitotane, cisplatin, doxorubicin, and etoposide as first-line therapy, but then had an objective response to oral metformin that lasted 9 months. The presence of the R337H TP53 mutation suggests a mechanism for the observed response to metformin. |
| Databáze: | OpenAIRE |
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