Neuroendocrine tumors are a heterogeneous group of tumors that originate from the neuroendocrine cells in the diffuse neuroendocrine system anywhere in the body but more commonly in the gastrointestinal tract and the respiratory system.
Pheochromocytoma and Paraganglioma
Pheochromocytoma and paraganglioma are rare neuroendocrine tumors that originate from the chromaffin cells of the adrenal glands.Chromaffin cells produce catecholamine neurotransmitters, such as epinephrine, norepinephrine, and dopamine. Compared to the normal chromaffin cells, pheochromocytomas and paraganglioma express high levels of the norepinephrine transporter on their cell surfaces. The excess amount of norepinephrine causes the clinical signs and symptoms like hypertension, headache, sweating, tremor, and palpitation. While most pheochromocytoma and paraganglioma are non-malignant (non-metastatic), about 10% of pheochromocytoma are malignant and about 25% of paraganglioma are malignant (metastatic) which can spread to other parts of the body, such as the liver, lungs, bone, or distant lymph nodes.
Coverage is subject to the specific terms of the member’s benefit plan.
Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the www.fepblue.org website.
Lutetium 177 (Lu 177) dotatate treatment is considered medically necessary when conditions 1 through 8 are met:
Continuation of Treatment
Continuation of Lu 177 dotatate is considered medically necessary when conditions 1 through 5 are met:
Lu 177 dotatate treatment is considered investigational in all other situations in which the above criteria are not met
Lu 177 dotatate treatment greater than a total of 4 doses as per the Food and Drug Administration-approved regimen is considered investigational
Iobenguane I 131
Iobenguane I 131 is considered medically necessary when conditions 1 through 5 are met:
Iobenguane I 131 treatment is considered investigational for all other indications including neuroblastoma and gastroenteropancreatic neuroendocrine tumors
Use of iobenguane I 131 not in accordance with FDA approved dosing (first dosimetric dose followed by two therapeutic doses administered 90 days apart) is considered investigational
Practice Guidelines and Position Statements
The National Comprehensive Cancer Network guidelines (v.1.2019) for neuroendocrine and adrenal tumors added key eligibility criteria for individuals treated with lutetium 177 dotatate for neuroendocrine tumors. Eligibility criteria include low or intermediate grade neuroendocrine tumor (proliferation index Ki-67 < 20%), detection of somatostatin receptor expression using somatostatin-based receptor imaging, and adequate bone marrow, renal and hepatic function. Table 13 summarizes the National Comprehensive Cancer Network guidelines for neuroendocrine and adrenal tumors.
Table 13. Recommendations for Use of Lutetium 177 Dotatate for Neuroendocrine Tumors
|Mid-gut locoregional advanced or distant metastases gastrointestinal neuroendocrine tumors after disease progression on somatostatin analogues||1|
|Bronchopulmonary/thymic locoregional advanced or distant metastases neuroendocrine tumors if there is clinically significant tumor burden and low grade (typical) or evidence of progression or intermediate grade (atypical)||2A|
|Locoregional advanced or distant metastases gastrointestinal neuroendocrine tumors after disease progression on somatostatin analogues||2A|
|Locoregional advanced or distant metastases pancreatic neuroendocrine tumors after disease progression on somatostatin analogues||2A|
The National Comprehensive Cancer Network guidelines (v.1.2019) for neuroendocrine and adrenal tumors gives iobenguane I 131 category 2A recommendation for treatment of individuals with locally unresectable or distant metastatic tumors with positive MIBG (iobenguane) scan.
Internal Medical Policy Committee 3-16-2020
Current medical policy is to be used in determining a Member's contract benefits on the date that services are rendered. Contract language, including definitions and specific inclusions/exclusions, as well as state and federal law, must be considered in determining eligibility for coverage. Members must consult their applicable benefit plans or contact a Member Services representative for specific coverage information. Likewise, medical policy, which addresses the issue(s) in any specific case, should be considered before utilizing medical opinion in adjudication. Medical technology is constantly evolving and the Company reserves the right to review and update medical policy periodically.