Emerging Treatments for Advanced/Metastatic Pheochromocytoma and Paraganglioma.
Opinion Statement: The incidence of metastatic pheochromocytoma (PHEO) and paraganglioma (PGL) may occur in as many as 35% of patients particularly with PGL and even more frequently in those with specific mutations. Biochemical, morphological, and molecular markers have been investigated for use in...
| Published in: | Current Treatment Options in Oncology Vol. 21; no. 11; pp. 1 - 19 |
|---|---|
| Main Authors: | , , , |
| Format: | review Journal Article |
| Published: |
Springer Nature
Nov2020
|
| Online Access: | View this record in EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=146431600&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 146431600 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 15272729 3KHC jtl: Current Treatment Options in Oncology issn: 15272729 maglogo: N pubinfo: dt: Nov2020 vid: 21 iid: 11 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 146431600 146431600 NLM32862332 146431600 10.1007/s11864-020-00787-z NLM32862332 146431600 ppf: 1 ppct: 18 formats: fmt: – @attributes: type: T – @attributes: type: P tig: atl: Emerging Treatments for Advanced/Metastatic Pheochromocytoma and Paraganglioma. aug: au: Ilanchezhian, Maran Jha, Abhishek Pacak, Karel Del Rivero, Jaydira affil: Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD, USA sug: subj: Paraganglioma Therapy Adrenal Gland Neoplasms Therapy Pheochromocytoma Therapy Antineoplastic Agents Therapeutic Use Radiopharmaceuticals Therapeutic Use Cyclophosphamide Therapeutic Use Octreotide Acetate Therapeutic Use Surgery, Operative Paraganglioma Pathology Angiogenesis Inhibitors Therapeutic Use Vincristine Therapeutic Use Adrenal Gland Neoplasms Pathology Pheochromocytoma Antineoplastic Agents, Combined Therapeutic Use Liver Neoplasms Benzene Derivatives Therapeutic Use Octreotide Acetate Analogs and Derivatives Bone Neoplasms Dacarbazine Therapeutic Use Pheochromocytoma Pathology Lung Neoplasms Cytoreduction Surgical Procedures Liver Neoplasms Therapy Paraganglioma Bone Neoplasms Therapy Lung Neoplasms Therapy Clinical Assessment Tools ab: Opinion Statement: The incidence of metastatic pheochromocytoma (PHEO) and paraganglioma (PGL) may occur in as many as 35% of patients particularly with PGL and even more frequently in those with specific mutations. Biochemical, morphological, and molecular markers have been investigated for use in the distinction of benign from malignant PHEO/PGL. PHEO/PGL metastasizes via hematogenous or lymphatic routes and shows differences based on mutational status. The most common sites of involvement in patients that have an SDHB mutation are the bone (78%), lungs (45%), lymph nodes (36%), and liver (35%). In patients with sporadic PHEO/PGL, the most common sites of metastasis are the bones (64%), lungs (47%), lymph nodes (36%), and liver (32%). Metastases may be present at presentation or may occur later. Metastases to the liver and lungs are associated with a shorter survival. Overall, the estimated 5-year survival rates are between 34 and 74%. Currently, treatments for metastatic PHEO/PGL are essentially palliative. Surgery is potentially curative; however, tumor dissemination limits the chance for a curative resection. When surgical intervention is not amenable, the therapeutic options include radiolabeled MIBG (Azedra®-iobenguane 131 was recently FDA-approved for patients > 12 years and older with iobenguane scan positive) or systemic chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) with an overall objective response rate (ORR) of less than 40%; however, it is not clear if the administration of CVD impacts overall survival, as nearly all patients develop progressive and ultimately fatal disease. Other treatment modalities under investigation include cytoreductive techniques, novel radiopharmaceuticals, chemotherapy, radiotherapy, immunotherapy, and experimental therapies. Here we are discussing emerging treatment for advanced/metastatic PHEO/PGL. pubtype: Academic Journal doctype: review Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
|---|