Gland and Hormone Related Diseases

All Conditions

Nutritional and Metabolic Diseases

Neoplasms

Diseases and Abnormalities at or Before Birth

Symptoms and General Pathology

Hyperparathyroidism

Hypoglycemia

Endocrine Gland Neoplasms

Parathyroid Neoplasms

Multiple Endocrine Neoplasia

Hypercalcemia

Parathyroid Diseases

Endocrine System Diseases

Metabolic Diseases

Calcium Metabolism Disorders

Water-Electrolyte Imbalance

Neoplasms, Multiple Primary

Syndrome

Neoplastic Syndromes, Hereditary

Genetic Diseases, Inborn

Head and Neck Neoplasms

All Drugs and Chemicals

Dihydroxyphenylalanine

Parathyroid Hormone

Patients with confirmed or suspected primary hyperparathyroidism or complications

Patients scanned using imaging agents 68GALLIUM-DOTATATE and 18F-DOPA

68Ga-Dotatate PET/CT will be administered prior to a PET/CT scan to detect known and occult primary and metastatic bronchial, gastrointestinal and pancreatic neuroendocrine tumors.

68Ga-Dotatate

18F-DOPA PET/CT will be administered prior to a whole-body scan to detect known and occult primary and metastatic bronchial, gastrointestinal and pancreatic neuroendocrine tumors.

18F-DOPA

Smita Jha, M.D.

Observational Phase: Patients whose parathyroid activity is elevated above normal are referred to as having hyperparathyroidism. This study will help researchers better understand the causes of hyperparathyroidism and to evaluate and improve methods for diagnosis and treatment. Patients diagnosed with or suspected of having hyperparathyroidism will be selected to participate. In addition, patients with related conditions, such as parathyroid tumors, will also be selected.

Subjects will be asked to provide blood and urine for testing to confirm their condition. They will then be surgically treated by removal of the parathyroid gland(s) (parathyroidectomy). Subjects with parathyroid tumors will undergo several diagnostic tests to determine the exact location of the tumor as well as the tumor's activity. The tests may include; ultrasounds, nuclear scanning, CT scans, MRI, and specialized blood testing.

Sometimes parathyroidectomy leads to hypoparathyroidism. Options for treating the patients after the surgical procedure will also be addressed. Calcium and Vitamin D supplements are typically the mainstay of post parathyroidectomy therapy. Other potential treatments include transplanting the parathyroid gland(s) to other areas of the body.

Clinical Trial: An imaging substudy was added to this protocol in 2018. Patients with multiple endocrine neoplasia type 1 (MEN1) will have 68Gallium-Dotatate Positron Emission Tomography (PET) - Computed Tomography (CT), 18F-DOPA PET/CT, MRI, and CT scans and the number of lesions detected by each of these types of scans will be compared.

Observational: Patients with confirmed or suspected primary hyperparathyroidism or complications therefrom (such as postoperative hypoparathyroidism) will be admitted for diagnosis and treatment. The principal diagnostic components are calcium in serum and urine, parathyroid hormone in serum, and mutation tests on germline or tumor DNA. Patients with moderate to severe primary hyperparathyroidism will be treated. Treatment will be mainly by parathyroidectomy. Other options are medications or no intervention. Patients with a hyperparathyroid syndrome may be managed for their extraparathyroid features. Preoperative testing to localize parathyroid neoplasm(s) will be used usually and with more extended methods in cases with prior neck surgery. Preoperative tumor localization tests will be selected according to clinical indications from the following: ultrasound, technetium-thallium scan, computerized tomography, magnetic resonance imaging, fine needle aspiration for parathyroid hormone assay, selective arteriogram, selective venous catheterization for parathyroid hormone assay. Options for management of postoperative hypocalcemia include calcium, vitamin D analogs, parathyroid autografts and synthetic parathyroid hormone. Research specimens may consist of blood or tumors.

Clinical Trial: An imaging substudy was added to this protocol in 2018. Patients with MEN1 will have 68Ga-Dotatate PET/CT, 18F-DOPA PET/CT, MRI, and CT scans and the number of lesions detected by each of these types of scans will be compared.

Studies of Elevated Parathyroid Activity

Studies of Hyperparathyroidism and Related Disorders

The purpose of this study is to understand the causes of primary hyperparathyroidism, to evaluate and improve methods for diagnosis and treatment, and to provide insight into the mechanisms of normal parathyroid function. Hereditary causes of primary hyperparathyroidism will be characterized. Patients were categorized as follows:

1. MEN1: Diagnosed by demonstration of a germline variant in MEN1 gene or one of the following: a) two of three primary MEN1 manifestations b) one primary MEN1 manifestation with a family member with MEN1.
2. Other familial: Non-MEN1 patients who had a positive family history of hyperparathyroidism suspicious for underlying germline predisposition syndrome.
3. Sporadic: Patients who did not have a positive family history of hyperparathyroidism.
4. Unknown: No data to help categorize the patients in any of the above categories.

For each organ, agreement between 68Ga-DOTATATE and 18F-DOPA

The total number of lesions identified by each imaging modality

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

Patients with confirmed or suspected primary hyperparathyroidism or complications who are missing age and other baseline data because of changes in data management system

Primary Hyperparathyroidism - Incomplete Data

Primary Hyperparathyroidism

Patients scanned using imaging agents 68GALLIUM-DOTATATE and 18F-DOPA

68Ga-Dotatate: 68Ga-Dotatate PET/CT will be administered prior to a PET/CT scan to detect known and occult primary and metastatic bronchial, gastrointestinal and pancreatic neuroendocrine tumors.

18F-DOPA: 18F-DOPA PET/CT will be administered prior to a whole-body scan to detect known and occult primary and metastatic bronchial, gastrointestinal and pancreatic neuroendocrine tumors.

DOTATATE and F-DOPA

Total

These data are not available because of changes to the data management system. Most were enrolled before the year 2000.

Age, Continuous

Sex: Female, Male

Ethnicity (NIH/OMB)

Race (NIH/OMB)

United States

Region of Enrollment

Age is not available for 634 participants in the primary hyperparathyroidism arm because the necessary records are not in the current computer system

Dr. Smita Jha

Type of Hyperparathyroidism

All locations

Lung

Gastrointestinal

Pancreas

Duodenum

Liver

Adrenal

Lymph nodes

Patients who received both DOTATATE and F-DOPA scans

Imaging was only conducted in the "DOTATATE and F-DOPA" Arm/Group and therefore results are only reported for this arm

Organs With Identified Lesions

Imaging was only conducted in the "DOTATATE and F-DOPA" Arm/Group and therefore results are only reported for this arm. This outcome is only reported for the 16 patients who had both scans

Number of Lesions Identified

Overall Study

Recurrence

Adenoma

Neoplasms, Glandular and Epithelial

Neoplasms by Histologic Type

subjects who are diagnosed with parathyroid adenomas. There will be 6 subjects who have not had surgery and 25 subjects who have had surgery.

Michele Lisi, MD

The purpose of this study is to design a method to better localize parathyroid adenomas. This study will include approximately 6 patients who have not had surgery and another 25 patients who have already had surgery over the course of one year.

While a successful neck exploration for PHP is primarily dependent on the experience of the surgeon, there are currently several localizing studies available to increase success. Although the necessity of localizing studies pre-operatively for first time neck explorations is still under debate, there is no question of their importance for persistent or recurrent PHP that requires re-operation. Localizing studies in these patients have been clearly shown to reduce operating time, avoid unnecessary dissection, reduce morbidity, and improve success rate.

Several modalities have been employed for localization such as ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI). The sensitivity of ultrasound for the preoperative detection of parathyroid adenomas has been reported to be 65-90% (1). There are however, limitations to US. Because of the sonolucent appearance of parathyroid adenomas, it is impossible to distinguish an ectopic cervical parathyroid adenoma from a pathologic lymph node. Furthermore, mediastinal parathyroid adenomas are difficult, if not impossible to visualize due to acoustic shadowing from the sternum and clavicles (2). Also, this method is very operator-dependent. MRI has a slightly higher sensitivity of 62.5-94% while CT has a poor sensitivity of only 40-44% (1).

We are using dual-phase single photon emission tomography (SPECT) imaging with technetium 99m sestamibi and visual thyroid subtraction with technetium 99m as pertechnetate. This method can achieve sensitivities of 68-95% and specificities of 75-100% (1). SPECT imaging is dependent on the differential washout of sestamibi between normal thyroid tissue and abnormal parathyroid tissue. Theoretically, sestamibi will washout of normal thyroid tissue much more quickly than it will from abnormal parathyroid tissue. Therefore, SPECT imaging is usually performed immediately and then at 90 and 180 minutes after sestamibi injection. SPECT imaging, however, has several limitations, not the least of which is its inability to provide discrete anatomic detail.

In addition, we will use a control group of patients who have parathyroid adenomas but who have not yet had surgery to test the efficacy of our fusion software.

Our goal is to acquire MRI and SPECT imaging with fiducial markers and utilize fusion software to create a detailed anatomic map of the neck for more accurate localization of the lesion.

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization	Link
Studies of Elevated Parathyroid Activity	NCT00001277	Hyperparathyroi... Hypercalcemia Parathyroid Neo... Multiple Endocr... MEN1	68Ga-Dotatate 18F-DOPA	2 Months -	National Institutes of Health Clinical Center (CC)
Evaluation of MRI and SPECT Fusion Software to Localize Parathyroid Adenomas	NCT00639405	Parathyroid Neo... Parathyroid Dis...		18 Years - 80 Years	State University of New York - Upstate Medical University

Spots Global Cancer Trial Database for parathyroid neoplasm