Primary parathyroid hyperplasia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Hyperplasia of the parathyroid gland

Overview

Historical Perspective

Classification

  • Primary parathyroid hyperplasia
  • Secondary parathyroid hyperplasia

Pathophysiology

  • Parathyroid hyperplasia is the diffuse enlargement of the parathyroid glands and is a cause of primary hyperparathyroidism[1]
  • In ~50% of cases there is asymmetric enlargement of the parathyroid glands.

Genetics

Associated Conditions

  • Multiple endocrine neoplasia I (MEN I)
  • Multiple endocrine neoplasia II A (MEN IIA)
  • Isolated familial hyperparathyroidism

Gross Pathology

  • Classically all parathyroid glands are involved; however, some may be spared making it difficult to differentiate this from parathyroid adenoma.

Microscopic Pathology

  • Classically have abundant adipose tissue[2]
  • +/-Water-clear cells ("water-clear cell hyperplasia").
Hyperplasia of parathyroid gland HE-staining, 20× magnification.[3]

Causes

  • Primary parathyroid hyperplasia
  • Sporadic (80%): associated with exposure to radiation and lithium
  • Familial (20%): associated with MEN 1 and MEN 2a
  • Secondary parathyroid hyperplasia
  • Renal failure

Differentiating type page name here from other Diseases

  • Parathyroid adenoma
  • Parathyroid carcinoma

Epidemiology and Demographics

Age

Females are more commonly affected with parathyroid hyperplasia than males. The females to male ratio is approximately 3:1.

Risk Factors

  • Multiple endocrine neoplasia I
  • Multiple endocrine neoplasia II A
  • Isolated familial hyperparathyroidism

Screening

Natural History, Complications and Prognosis

  • Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as excellent.

Complications

  • Kidney stones
  • Osteitis fibrosa cystica

Diagnosis

Diagnostic Criteria

If available, the diagnostic criteria are provided here.

History

A directed history should be obtained to ascertain

Symptoms

  • Constipation
  • Bone fractures or bone pain
  • Nausea
  • Lethargy
  • Muscle pain

Past Medical History=

Family History

Social History

Occupational

Alcohol

The frequency and amount of alcohol consumption should be characterized.

Drug Use

Smoking

Allergies

Physical Examination

Appearance of the Patient

Vital Signs

Skin

Head

Eyes

Ear

Nose

Mouth

Throat

Heart

Lungs

Abdomen

Extremities

Neurologic

Genitals

Other

Laboratory Findings

  • Calcium
  • Phosphorus
  • Magnesium
  • PTH
  • Vitamin D levels
  • A 24-hour urine test

Electrolyte and Biomarker Studies

Electrocardiogram

Chest X Ray

  • Bone x-rays

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

  • Bone density test (DXA)
  • Nuclear medicine (Tc-99m MIBI) is the current modality of choice.

Other Diagnostic Studies

Treatment

Surgery

  • Surgery is the mainstay of treatment for parathyroid hyperplasia. Three and a half part of the glands are removed during surgery.[4]

Parathyroidectomy

Patients with primary hyperparathyroidism and concordant localisation to a single site can undergo minimally invasive parathyroidectomy. Patients with primary hyperparathyroidism where localisation has not been successful are more likely to have multiple gland disease and should undergo open parathyroidectomy and four-gland exploration. Patients with secondary and tertiary hyperparathyroidism require either subtotal parathyroidectomy or total parathyroidectomy with or without forearm autotransplantation. Successful detection and removal of the involved parathyroid tissue will occur in 98% of patients. In the small percentage of patients in whom the gland is not detected at the time of primary surgery, it is likely to lie in an ectopic position, e.g. pericardium or middle mediastinum and additional, localisation studies such as CT scanning and selective venous sampling will be required prior to a second operation.

Complications of parathyroidectomy

The complications of parathyroidectomy include all the general complications of any operation, such as bleeding, wound infection, and reaction to the anaesthetic agent. In addition, there are specific complications, including: damage to the recurrent laryngeal nerves and to the external branch of the superior laryngeal nerves failure to locate abnormal parathyroid tissue hypoparathyroidism Recurrent nerve palsy leads to a hoarse voice that usually recovers but may require procedures such as vocal cord medialisation. If the external branch of the superior laryngeal nerve is damaged, the patient may lose the ability to sing, shout or project their voice. Failure to locate abnormal parathyroid tissue may be due to the adenoma being in an ectopic site such as the mediastinum. Further localisation studies and surgery will be required. If more than one parathyroid gland is involved, subtotal parathyroidectomy may lead to hypoparathyroidism, which may require short-term administration of oral calcium and 1,25-dihydroxyvitamin D for several weeks.

Primary Prevention

Secondary Prevention

  • Genetic screening for patients with family history of MEN syndrome.

Cost-Effectiveness of Therapy

Future or Investigational Therapies

References

  1. Parathyroid hyperplasia. Radiopedia (2016). http://radiopaedia.org/articles/parathyroid-hyperplasia Accessed on January 7, 2016
  2. Parathyroid hyperplasia. Librepathology (2016). http://librepathology.org/wiki/index.php?title=Parathyroid_hyperplasia&redirect=no Accessed on January 7, 2016
  3. Image courtesy of wikipedia. Biomedcentral (original file ‘’here’’.Creative Commons BY-SA-NC
  4. Parathyroid. Surgwiki (2016). http://www.surgwiki.com/w/index.php?title=Parathyroid&oldid=5933 Accessed on January 7, 2016