Parathyroid Disease and Neoplasms

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Parathyroid Disease and Neoplasms

Head and Neck Surgery
Author Credentials 

Russell B. Smith, MD
Baptist MD Anderson Cancer Center
Baptist Health
Jacksonville, FL

Module Summary

The parathyroid glands are important in maintaining calcium homeostasis in the body.  Abnormal parathyroid gland function can be related to intrinsic disease of a parathyroid gland/-s or be a physiologic response to other conditions. An understanding of the different parathyroid diseases will allow one to complete the appropriate patient evaluation to establish the diagnosis. Once the diagnosis is established, options for treatment may include observation, medical therapy, or surgery. When surgical therapy is required, preoperative imaging for localization is frequently utilized. Depending on the diagnosis and findings on preoperative imaging, minimally invasive surgical approaches with the use of intraoperative adjuncts can result in high cure rates. While rare, complications from parathyroid surgery can occur and may require immediate recognition and therapy.  Recurrent disease is not common but can occur. Salvage surgery is much more complex and accurate localization of recurrent disease is critical to success.

Module Learning Objectives 
  1. Review the embryologic development of the parathyroid glands and the implications for anatomic relationships encountered during surgical exploration.
  2. Describe the mechanisms for the different types of hyperparathyroidism.
  3. State the symptoms for the different types of hyperparathyroidism.
  4. Differentiate primary hyperparathyroidism from other causes of hypercalcemia.
  5. Recognize the uncommon types and syndromic etiologies of primary hyperparathyroidism.
  6. Explain the diagnostic evaluation for a patient with hyperparathyroidism.
  7. Review the differentiate options for preoperative imaging for parathyroid localization.
  8. Determine the appropriate extent of surgery and use of intraoperative adjuncts for patients with hyperparathyroidism.
  9. Recognize and treat the complications that may occur following parathyroid surgery.
     

Embryology

Learning Objectives 
  1. Explain how the parathyroid glands develop from the 3rd and 4th branchial pouches and how this affects the typical and ectopic locations of the superior and inferior parathyroid glands.
References 

Baloch, ZW, LiVolsi, VA. Pathology of parathyroid glands in hyperparathyroidism. Semin Diagn Pathol. 2013;30:165-77.

Anatomy

Learning Objectives 
  1. Describe the appearance, size, shape, and weight of a normal parathyroid gland relative to a parathyroid adenoma.
  2. Review the most common location of the superior and inferior parathyroid glands.
  3. Recognize the most common ectopic locations of the superior and inferior parathyroid glands.
  4. Explain the blood supply to the parathyroid glands.
  5. Review the relationship of the superior and inferior parathyroid glands relative to the recurrent laryngeal nerve.

Pathogenesis

Learning Objectives 
  1. Know that the vast majority of patients with primary hyperparathyroidism do not have a definable etiology.
  2. Understand MEN1 and MEN2a and the other associated diseases as an etiology of primary hyperparathyroidism.
  3. Be familiar with the conditions associated with secondary hyperparathyroidism and the changes that occur in patients with renal failure that results in tertiary hyperparathyroidism.

Basic Science

Learning Objectives 

Describe parathyroid hormone and the role it plays in calcium homeostasis.

Genetics

Learning Objectives 
  1. Be familiar with the genetic abnormalities in MEN1 and MEN2a.
  2. Be familiar with the genetic abnormalities in familial hypocalciuric hypercalcemia.
References 
  1. DeLellis, RA, Mazzaglia, P, Mangray, S. Primary Hyperparathyroidism: a current perspective. Arch Pathol Lab Med. 2008;132:1251-62.
  2. Herfarth KK, Wells SA Jr. Parathyroid glands and the multiple endocrine neoplasia syndromes and familial hypocalciuric hypercalcemia. Semin Surg Oncol. 1997;13:114-24.
  3. Christensen SE, Nissen, PH, Vestergaard P, Mosekilde, L. Familial hypocalciuric hypercalcemia: a review. Curr Opin Endocrinol Diabetes Obes. 2011;18:359-70.

Patient Evaluation

Learning Objectives 
  1. Determine that the etiology of hypercalcemia is hyperparathyroidism.
  2. Understand that atypical variants of primary hyperparathyroidism.
  3. Know the differential diagnosis of hypercalcemia that must be excluded when evaluating a patient for potential primary hyperparathyroidism.
References 
  1. Amin, AL, Wang, TS, Wade, TJ, Yen, TWF. Normal PTH Levels in Primary Hyperparathyroidism: Still the Same Disease? Ann Surg Oncol. 2011;18:3437-3442.
  2. Lowe H, McMahon DJ, Rubin MR, Bilezikian JP, Silverberg SJ. Normocalcemic primary hyperparathyroidism: further characterization of a new clinical phenotype. J Clin Endocrinol Metab. 2007;92:3001-5.

Imaging

Learning Objectives 
  1. Understand the different imaging modalities that can be utilized for preoperative localization.  
  2. Develop a cost-effective approach to preoperative imaging based upon the surgical approach being utilized.
References 
  1. Smith RB, Evasovich M, Girod DA, Jorgensen JB, Lydiatt WM, Pagedar NA, Spanos WC. Ultrasound Localization in Primary Hyperparathyroidism. Otolaryngol Head Neck Surg. 2013;149:366-71.
  2. Perez-Monte JE, Brown ML, Shah AN, Ranger NT, Watson CG, Carty SE, Clarke MR. Parathyroid adenomas: Accurate detection and localization with Tc-99 m sestamibi SPECT. Radiology. 1996;201:85-91.
  3. Chazen JL, Gupta A, Dunning A, Phillips CD. Diagnostic accuracy of 4D-CT parathyroid adenomas and hyperplasia. Am J Neuroradiol. 2012;33:429 –33. 
  4. Ishibashi M, Nishida H, Hiromatsu Y, Kojima K, Tabuchi E, Hayabuchi N. Comparison of technetium-99 m-MIBI, technetium-99 m-tetrofosmin, ultrasound and MRI for localization of abnormal parathyroid glands. J Nucl Med. 1998;39:320-4.

Pathology

Learning Objectives 
  1. Understand the differences between single gland and multi-gland disease in primary hyperparathyroidism.
  2. Understand the specific pathology findings to distinguish parathyroid adenomas from parathyroid carcinoma in patients with a suspicious clinical presentation.
References 

Baloch, ZW, LiVolsi, VA. Pathology of parathyroid glands in hyperparathyroidism. Semin Diagn Pathol 2013;30:165-77.

Treatment

Learning Objectives 
  1. Describe the patient with hyperparathyroidism in which observation is appropriate.
  2. Describe the indications for surgery in a patient with primary hyperparathyroidism.
  3. Describe the indications for surgery in a patient with secondary and tertiary hyperparathyroidism.
References 

Silverberg SJ, Shane, E, Jacobs, TP, Siris, E, Bilezikian, JP. A 10-Year Prospective Study of Primary Hyperparathyroidism with or without Parathyroid Surgery. NEJM 2000;341:1249-55.
Bilezikian JP, Khan A, Potts JT Jr. Guidelines for the management of asymptomatic primary hyperparathyroidism: summary statement from the third international workshop. J Clin Endocrinol Metab. 2009;94:335-9.  
Pitt, SC, Sippel, RS, Chen, H. Secondary and Tertiary Hyperparathyroidism, State of the Art Surgical Management. Surg Clin North Am. 2009;89:1227-1239.

Medical Therapies

Learning Objectives 
  1. Understand the acute medical management of severe symptomatic hypercalcemia.
  2. Understand the long-term medical management of chronic hypercalcemia.
References 
  1. Bilezikian JP. Management of acute hypercalcemia. N Engl J Med. 1992;326:1196-203.

Pharmacology

Learning Objectives 
  1. Describe the use of diuretics to manage hypercalcemia.
  2. Describe the use of cinacalcet to manage hypercalcemia. 
  3. Describe the use of calcium and vitamin D to manage postoperative hypocalcemia.

Surgical Therapies

Learning Objectives 
  1. Understand the surgical technique of 4-gland exploration.
  2. Understand the surgical technique of minimally invasive parathyroidectomy in appropriately selected patients.
    • Intraoperative PTH assays.
    • Radio-guided surgery.
  3. Determine the appropriate surgical approach for a given patient based upon clinical presentation and preoperative imaging if obtained.
  4. Be familiar with 3 ½ gland excision vs. 4 gland excision with autotransplantation in patients with 4-gland hyperplasia.
References 
  1. Krempl GA, Medina JE, Bouknight AL. Surgical management of the parathyroids. Otolaryngol Clin North Am. 2003;36:217-28.
  2. Boggs JE, Irvin GL 3rd, Molinari AS, Deriso GT. Intraoperative parathyroid hormone monitoring as an adjunct to parathyroidectomy. Surgery 1996;120:954-8.
  3. Murphy C, Norman J. The 20% rule: a simple, instantaneous radioactivity measurement defines cure and allows elimination of frozen sections and hormone assays during parathyroidectomy. Surgery. 1999;126:1023-9.

Case Studies

  1. A 72 year-old female with incidentally detected mild asymptomatic hyperparathyroidism. 
  2. A 60 year-old male with hyperparathyroidism and a history of nephrolithiasis.
  3. A 48 year-old female with fatigue and myalgias with a mildly elevated calcium and a PTH in the normal range.

Complications

Learning Objectives 
  1. Know the potential acute complications of parathyroidectomy and their treatment.
  2. Know the potential chronic complications of parathyroidectomy and their treatment.
  3. Describe the evaluation of patients with persistent hypercalcemia following parathyroidectomy. 

Review

Review Questions 
  1. What is the diagnostic evaluation to establish a diagnosis of primary hyperparathyroidism?
  2. Which patients with primary hyperparathyroidism are appropriate to observe and how should they be monitored?
  3. What are the indications for surgery in the different forms of hyperparathyroidism?
  4. Which imaging studies should be considered for localization in primary hyperparathyroidism?
  5. Which patients are appropriate for minimally invasive parathyroidectomy? 
References 
  1. Wilhelm SM, Wang TS, Ruan DT, Lee JA, Asa SL, Duh, QY, Doherty GM, Herrera, MF, Pasieka JL, Perrier ND, Silverberg SJ. Solarzano CC, Sturgeon C, Tublin ME, Udelsman R, Carty SE. The American Association of Endocrine Surgeons Guidelines for Definitive Management of Primary Hyperparathyroidism. JAMA. 2016 Oct 1;151(10):959-968.
  2. Randolph, G (2013)Surgery of the Thyroid and Parathyroid Glands, 2ed, Saunders. Chapters 56-70.
Resources 

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