|
SDHB-related PGL/PCC
| Surgical resection is recommended due to risk for metastases. Prompt resection is particularly important for extra-adrenal sympathetic PGLs because of their tendency to metastasize. Perioperative alpha-adrenergic blockade is typically required.
| There may be no difference in metastatic potential between SDHB-assoc & sporadic HNPGLs [Richter et al 2022]. |
|
Nonsecretory HNPGL
| Treatment options:
Active observation Surgical resection Radiation therapy
Because most HNPGLs are nonsecretory, persons w/HNPGLs should be evaluated for catecholamine excess before surgical resection; if present, this can suggest an additional primary PGL/PCC. | Early detection allows for a timely decision re treatment or surveillance. Active observation & radiation therapy are often equally beneficial or better approaches. |
|
Carotid body, jugulotympanicum, & vagal PGL
| Treatment options:
Active observation Surgical resection Radiation
Treatment choice should be based on extent of tumor (e.g., Shamblin I & II carotid body tumors are good candidates for surgery), assoc risks (e.g., resection of glomus vagal tumors almost invariably leads to loss of ipsilateral vagal & recurrent laryngeal nerve), & presumed metastatic potential (e.g., SDHB-assoc tumors could be considered for more aggressive therapy). | Radiation therapy is an option, & there is currently no evidence for ↑ incidence of secondary malignancies in this population due to underlying genetic condition. 1 |
|
Jugular paragangliomas
| Treatment options:
| Small tumors may potentially be removed w/o complications or permanent nerve injuries. Resection of larger tumors is often assoc w/CSF leak, meningitis, stroke, hearing loss, cranial nerve palsy, or even death. Close observation w/symptomatically guided surgery may be prudent. Gamma knife stereotactic surgery is a good option to prevent morbidity from resection.
|
|
Catecholamine-secreting tumors
| Treatment is directed toward containing the effect of catecholamines through antagonism of catecholamine excess w/pharmacologic adrenergic blockade prior to surgical removal. 2 | |
|
Pheochromocytomas
| Preoperative:
Alpha-adrenergic blockade (w/prazosin/doxazosin) starting ≥7-10 days preoperatively to normalize BP & allow volume expansion. The dose of the α-blocker is adjusted for a low-normal systolic BP for age. Calcium channel blockers (e.g., amlodipine, nicardipine) as needed for second-line treatment of BP control 2 A liberal sodium diet & fluid intake to allow for plasma volume expansion. Once adequate α-adrenergic blockade or BP control w/calcium channel blockers is achieved, initiation of beta-adrenergic blockade may be required to control reflex tachycardia. The dose of the β-blocker is adjusted for a target heart rate of 80 beats per minute.
| Treat chronic & acute effects of catecholamine excess. Alpha-adrenergic blockade is required to control BP & prevent intraoperative hypertensive crises. The Endocrine Society guidelines have an algorithm for medication titration. 1 |
| Surgical resection, preferably laparoscopic, is the treatment of choice. 1 | |
Postoperative:
~2-8 wks after surgery, assess 24-hour urine fractionated metanephrines &/or plasma-free metanephrines. If the levels are normal, resection of the biochemically active PCC should be considered complete. If the levels are ↑, an unresected 2nd tumor &/or occult metastases should be suspected.
| |
|
Metastatic PGL/PCC
| Treatment options:
BP control w/α-blocker to ↓ symptoms from high catecholamine levels in persons w/sympathetic tumors Surgical debulking to ↓ tumor burden due to mass effect or catecholamine secretion Active observation for nonprogressing, nonsecreting disease Radiation therapy, esp for bony lesions Liver-directed therapy Systemic therapy w/chemotherapy (e.g., cyclophosphamide, vincristine, dacarbazine) I-131-MIBG therapy
| |
|
Metastatic or unresectable PGL/PCC – radionucleotide therapies
| I-131-MIBG; reserved for those requiring systemic therapy who have uptake in sites of disease on MIBG imaging. | |
| Lutathera® (Lu-177-DOTATATE therapy; peptide receptor radionuclide therapy [PRRT]) | Lutathera® is FDA approved for gastroenteropancreatic neuroendocrine tumors but not yet FDA approved for PGL/PCC (see Therapies Under Investigation). |
|
GIST
| Surgical resection of localized disease, particularly if tumor is bleeding, causing obstruction, >2 cm, or ↑ in size Tyrosine kinase inhibitor (TKI) for adjuvant therapy after surgical resection or as first-line therapy in those w/metastatic disease. However, SDH-deficient GISTs are largely resistant to TKIs.
| There are no standard recommendations other than expert opinion. 3 |
|
Pulmonary chondroma
| Active observation for these benign tumors unless they cause bronchial compression. | |
|
Clear cell renal cell carcinoma
| Early surgical intervention Partial nephrectomy in persons w/solitary tumor at early stage Standard treatment for metastatic disease
| There are no standard recommendations other than expert opinion. 4 |
