LETTER OF MEDICAL NECESSITY FOR HEREDITARY PHEOCHROMOCYTOMA/PARAGANGLIOMA
GENETIC TESTING (PGLNext)
Date:
Date of service/claim
To:
Utilization Review Department
Insurance Company Name, Address, City, State
Re:
Patient Name, DOB, ID #
ICD-9 Codes: (quick reference as suggestions: 194.6 malignant neoplasm of aortic body and other
paraganglia; 227.6 benign neoplasm of aortic body and other paraganglia; 237.3 neoplasm of uncertain
behavior of paraganglia; V18.19 family history of other endocrine and metabolic diseases)
This letter is in regards to my patient and your subscriber, First, Last Name to request full coverage
of medically-indicated genetic testing for hereditary paraganglioma/pheochromocytoma to be
performed by Ambry Genetics Corporation.
Paragangliomas (PGL) and pheochromocytomas (PCC) are endocrine tumors thought to have a
hereditary component in up to 40% of cases. Those with hereditary PGL/PCC are at risk for
multiple PGL/PCC, some of which have a high risk of becoming malignant. Those with hereditary
conditions related to PGL/PCC have an increased lifetime risk of developing tumors and/or cancers
(such as up to a 70% risk of developing renal cancer in those with von Hippel-Lindau disease, and
up to a 100% risk of developing medullary thyroid cancer in those with multiple endocrine
neoplasia type 2). Some of these gene mutations also increase the lifetime risk for additional
cancers/tumors (like pancreatic tumors, hemangioblastomas, neurofibromas, optic gliomas,
acoustic neuromas, and other neuroendocrine tumors).1,2,3
Significant aspects of my patient’s personal and/or family medical history that suggest a
reasonable probability of hereditary PGL/PCC are below:
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Based on the above history, I am requesting coverage for this test (PGLNext), which analyzes 12
high-risk genes associated with increased risks for PGL/PCC: FH, MAX, MEN1, NF1, RET, SDHA,
SDHAF2, SDHB, SDHC, SDHD, TMEM127, VHL. Due to the history stated above, there is a reasonable
probability of detecting a mutation in my patient. The clinical overlap with mutations in the abovementioned genes makes this multi-gene test the most efficient and cost-effective way to analyze
these genes.4 Therefore, germline genetic testing is warranted.1,2,4
This genetic testing will help estimate my patient’s risk to develop tumors/cancer. It will
also directly impact my patient’s medical management. An aggressive approach following
guidelines is indicated in those that carry a mutation found by this test. Management guidelines to
reduce the morbidity and mortality associated with these tumors/cancers may include:
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Consideration of CT/MRI-based screening/technologies
Annual biochemical screening
More prompt removal of tumor due to increased malignant potential (especially if a
SDHB mutation is found by this test)
Prophylactic thyroidectomy
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Annual ophthalmology and audiology examinations
Other: ____________________________________
Due to the tumor/cancer risks associated with these mutations and the interventions available, this
genetic testing is medically indicated. As such, I am ordering this testing as medically
necessary and affirm that my patient has provided informed consent for genetic testing.
A positive test result would confirm a genetic diagnosis and/or risk in my patient, and would
ensure my patient is being managed appropriately. I am specifying Ambry Genetics Corporation
because this laboratory has highly-sensitive and cost-effective testing for hereditary PGL/PCC,
along with a large database of previously tested patients to ensure highly validated, accurate, and
informative test interpretation.
I recommend that you support this request for coverage of diagnostic genetic testing for hereditary
PGL/PCC in my patient. Genetic testing can take several weeks to complete, and the laboratory will
not bill until testing is concluded. Therefore, I am requesting that the authorization be valid for 3
months.
Thank you for your time, and please don’t hesitate to contact me with any questions.
Sincerely,
Ordering Clinician Name (Signature Provided on Test Requisition Form)
(MD/DO, Clinical Nurse Specialist, Nurse-Midwives, Nurse Practitioner, Physician Assistant, Genetic
Counselor*)
*Authorized clinician requirements vary by state
Test Details
CPT codes:
81228x1, 81403x1, 81404x1, 81405x1, 81406x1, 81408x1
Laboratory:
Ambry Genetics Corporation (TIN 33-0892453 / NPI 1861568784), a CAPaccredited and CLIA-certified laboratory located at 15 Argonaut, Aliso Viejo, CA
92656
References:
1. Fishbein L, et al. Inherited mutations in pheochromocytoma and paraganglioma: why all
patients should be offered genetic testing. Ann Surg Oncol. 2013 May;20(5):1444-50.
2. Lenders JW, et al. Endocrine Society. Pheochromocytoma and paraganglioma: an endocrine
society clinical practice guideline. J Clin Endocrinol Metab. 2014 Jun;99:1915–42.
3. Giusti F, Marini F, Brandi ML. Multiple Endocrine Neoplasia Type 1. 2005 Aug 31 [updated
2015 Feb 12]. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews® [Internet].
Seattle (WA): University of Washington, Seattle; 1993-2015.
4. Karasek D, et al. An update on the genetics of pheochromocytoma. J Hum Hypertens. 2013
Mar;27(3):141-7.