Parathyroid carcinoma

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Parathyroid carcinoma
Parathyroid es.svg
Parathyroid gland anatomy(green marks)
Specialty Oncology, ENT surgery

Parathyroid carcinoma is a rare cancer resulting in parathyroid adenoma to carcinoma progression. [1] It forms in tissues of one or more of the parathyroid glands (four pea-sized glands in the neck that make parathyroid hormone (PTH). PTH helps the body maintain normal levels of serum calcium by promoting calcium reabsorption from bone. It is antagonized by the hormone calcitonin, which prompts calcium storage.).

Contents

It is rare, with documented cases of less than one thousand since its first discovery in 1904; [2] [3] [4] [5] [6] [7] and much less common than parathyroid adenoma. It can be difficult to excise. [8] The rate of occurrence of parathyroid carcinoma is between 0.5% to 5% [9] [10] [6] [11]

Signs and symptoms

Most patients experience moderate to severe hypercalcemia and high parathyroid hormone levels. A large mass in the neck is often seen, and kidney and bone abnormalities are common. [1]

Risk factors

Parathyroid cancer occurs in midlife at the same rate in men and women. [12]

Conditions that appear to result in an increased risk of parathyroid cancer include multiple endocrine neoplasia type 1, [13] autosomal dominant familial isolated hyperparathyroidism [13] and hyperparathyroidism-jaw tumor syndrome [1] (which also is hereditary). [1] Parathyroid cancer has also been associated with external radiation exposure, but most reports describe an association between radiation and the more common parathyroid adenoma. [13]

Diagnosis

On Sestamibi parathyroid scan, intense radioactivity greater than submandibular gland on delayed image, no washout between early and delayed images, and high concentration of parathyroid hormone concentration in blood in those who age more than 40 years is suggestive of parathyroid carcinoma. [14] Some authors suggest high levels of HCG as a marker for parathyroid carcinoma in the right context. [15] However, other thyroid diseases such as multinodular goitre, Hashimoto thyroiditis, thyroid adenoma, and thyroid carcinoma also retains the radiotracer because of high metabolic nature of these diseases. [16] Thus, the final diagnosis always requires pathological examination of the tissue in question.

Treatment

Parathyroid carcinoma is sometimes diagnosed during surgery for primary hyperparathyroidism. If the surgeon suspects carcinoma based on severity or invasion of surrounding tissues by a firm parathyroid tumor, aggressive excision is performed, including the thyroid and surrounding tissues as necessary. [1]

Agents such as calcimimetics (for example, cinacalcet) are used to mimic calcium and are able to activate the parathyroid calcium-sensing receptor (making the parathyroid gland "think" we have more calcium than we actually do), therefore lowering the calcium level, in an attempt to decrease the hypercalcemia.[ citation needed ]

Related Research Articles

<span class="mw-page-title-main">Multiple endocrine neoplasia</span> Medical condition

Multiple endocrine neoplasia is a condition which encompasses several distinct syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. In some cases, the tumors are malignant, in others, benign. Benign or malignant tumors of nonendocrine tissues occur as components of some of these tumor syndromes.

<span class="mw-page-title-main">Parathyroid gland</span> Endocrine gland

Parathyroid glands are small endocrine glands in the neck of humans and other tetrapods. Humans usually have four parathyroid glands, located on the back of the thyroid gland in variable locations. The parathyroid gland produces and secretes parathyroid hormone in response to a low blood calcium, which plays a key role in regulating the amount of calcium in the blood and within the bones.

<span class="mw-page-title-main">Calcitonin</span> Amino acid peptide hormone secreted by the thyroid gland

Calcitonin is a 32 amino acid peptide hormone secreted by parafollicular cells (also known as C cells) of the thyroid (or endostyle) in humans and other chordates in the ultimopharyngeal body. It acts to reduce blood calcium (Ca2+), opposing the effects of parathyroid hormone (PTH).

Hypercalcemia, also spelled hypercalcaemia, is a high calcium (Ca2+) level in the blood serum. The normal range is 2.1–2.6 mmol/L (8.8–10.7 mg/dL, 4.3–5.2 mEq/L), with levels greater than 2.6 mmol/L defined as hypercalcemia. Those with a mild increase that has developed slowly typically have no symptoms. In those with greater levels or rapid onset, symptoms may include abdominal pain, bone pain, confusion, depression, weakness, kidney stones or an abnormal heart rhythm including cardiac arrest.

Disorders of calcium metabolism occur when the body has too little or too much calcium. The serum level of calcium is closely regulated within a fairly limited range in the human body. In a healthy physiology, extracellular calcium levels are maintained within a tight range through the actions of parathyroid hormone, vitamin D and the calcium sensing receptor. Disorders in calcium metabolism can lead to hypocalcemia, decreased plasma levels of calcium or hypercalcemia, elevated plasma calcium levels.

<span class="mw-page-title-main">Parathyroid chief cell</span>

Parathyroid chief cells are one of the two cell types of the parathyroid glands, along with oxyphil cells. The chief cells are much more prevalent in the parathyroid gland than the oxyphil cells. It is perceived that oxyphil cells may be derived from chief cells at puberty, as they are not present at birth like chief cells.

<span class="mw-page-title-main">Hyperparathyroidism</span> Increase in parathyroid hormone levels in the blood

Hyperparathyroidism is an increase in parathyroid hormone (PTH) levels in the blood. This occurs from a disorder either within the parathyroid glands or as response to external stimuli.

Technetium (<sup>99m</sup>Tc) sestamibi Pharmaceutical drug

Technetium (99mTc) sestamibi (INN) is a pharmaceutical agent used in nuclear medicine imaging. The drug is a coordination complex consisting of the radioisotope technetium-99m bound to six (sesta=6) methoxyisobutylisonitrile (MIBI) ligands. The anion is not defined. The generic drug became available late September 2008. A scan of a patient using MIBI is commonly known as a "MIBI scan".

<span class="mw-page-title-main">Parathyroidectomy</span> Surgical removal of one or more of the parathyroid glands

Parathyroidectomy is the surgical removal of one or more of the (usually) four parathyroid glands. This procedure is used to remove an adenoma or hyperplasia of these glands when they are producing excessive parathyroid hormone (PTH): hyperparathyroidism. The glands are usually four in number and located adjacent to the posterior surface of the thyroid gland, but their exact location is variable. When an elevated PTH level is found, a sestamibi scan or an ultrasound may be performed in order to confirm the presence and location of abnormal parathyroid tissue.

<span class="mw-page-title-main">Endocrine gland</span> Glands of the endocrine system that secrete hormones to blood

Endocrine glands are ductless glands of the endocrine system that secrete their products, hormones, directly into the blood. The major glands of the endocrine system include the pineal gland, pituitary gland, pancreas, ovaries, testicles, thyroid gland, parathyroid gland, hypothalamus and adrenal glands. The hypothalamus and pituitary glands are neuroendocrine organs.

<span class="mw-page-title-main">Primary hyperparathyroidism</span> Medical condition

Primary hyperparathyroidism is a medical condition where the parathyroid gland produce excess amounts of parathyroid hormone (PTH). The symptoms of the condition relate to the resulting elevated serum calcium (hypercalcemia), which can cause digestive symptoms, kidney stones, psychiatric abnormalities, and bone disease.

<span class="mw-page-title-main">Multiple endocrine neoplasia type 1</span> Medical condition

Multiple endocrine neoplasia type 1 (MEN-1) is one of a group of disorders, the multiple endocrine neoplasias, that affect the endocrine system through development of neoplastic lesions in pituitary, parathyroid gland and pancreas. Individuals suffering from this disorder are prone to developing multiple endocrine and nonendocrine tumors. It was first described by Paul Wermer in 1954.

<span class="mw-page-title-main">Multiple endocrine neoplasia type 2</span> Medical condition

Multiple endocrine neoplasia type 2 is a group of medical disorders associated with tumors of the endocrine system. The tumors may be benign or malignant (cancer). They generally occur in endocrine organs, but may also occur in endocrine tissues of organs not classically thought of as endocrine. MEN2 is a sub-type of MEN and itself has sub-types, as discussed below. Variants in MEN2A have been associated with Hirschsprung disease. Screening for this condition can begin as young as eight years old for Pheochromocytoma.

<span class="mw-page-title-main">Osteitis fibrosa cystica</span> Medical condition

Osteitis fibrosa cystica is a skeletal disorder resulting in a loss of bone mass, a weakening of the bones as their calcified supporting structures are replaced with fibrous tissue, and the formation of cyst-like brown tumors in and around the bone. Osteitis fibrosis cystica (OFC), also known as osteitis fibrosa, osteodystrophia fibrosa, and von Recklinghausen's disease of bone, is caused by hyperparathyroidism, which is a surplus of parathyroid hormone from over-active parathyroid glands. This surplus stimulates the activity of osteoclasts, cells that break down bone, in a process known as osteoclastic bone resorption. The hyperparathyroidism can be triggered by a parathyroid adenoma, hereditary factors, parathyroid carcinoma, or renal osteodystrophy. Osteoclastic bone resorption releases minerals, including calcium, from the bone into the bloodstream, causing both elevated blood calcium levels, and the structural changes which weaken the bone. The symptoms of the disease are the consequences of both the general softening of the bones and the excess calcium in the blood, and include bone fractures, kidney stones, nausea, moth-eaten appearance in the bones, appetite loss, and weight loss.

<span class="mw-page-title-main">Tertiary hyperparathyroidism</span> Medical condition

Tertiary hyperparathyroidism is a condition involving the overproduction of the hormone, parathyroid hormone, produced by the parathyroid glands. The parathyroid glands are involved in monitoring and regulating blood calcium levels and respond by either producing or ceasing to produce parathyroid hormone. Anatomically, these glands are located in the neck, para-lateral to the thyroid gland, which does not have any influence in the production of parathyroid hormone. Parathyroid hormone is released by the parathyroid glands in response to low blood calcium circulation. Persistent low levels of circulating calcium are thought to be the catalyst in the progressive development of adenoma, in the parathyroid glands resulting in primary hyperparathyroidism. While primary hyperparathyroidism is the most common form of this condition, secondary and tertiary are thought to result due to chronic kidney disease (CKD). Estimates of CKD prevalence in the global community range from 11 to 13% which translate to a large portion of the global population at risk of developing tertiary hyperparathyroidism. Tertiary hyperparathyroidism was first described in the late 1960s and had been misdiagnosed as primary prior to this. Unlike primary hyperparathyroidism, the tertiary form presents as a progressive stage of resolved secondary hyperparathyroidism with biochemical hallmarks that include elevated calcium ion levels in the blood, hypercalcemia, along with autonomous production of parathyroid hormone and adenoma in all four parathyroid glands. Upon diagnosis treatment of tertiary hyperparathyroidism usually leads to a surgical intervention.

<span class="mw-page-title-main">Endocrine disease</span> Medical condition

Endocrine diseases are disorders of the endocrine system. The branch of medicine associated with endocrine disorders is known as endocrinology.

<span class="mw-page-title-main">Parathyroid adenoma</span> Medical condition

A parathyroid adenoma is a benign tumor of the parathyroid gland. It generally causes hyperparathyroidism; there are very few reports of parathyroid adenomas that were not associated with hyperparathyroidism.

<span class="mw-page-title-main">Parathyroid disease</span> Medical condition

Many conditions are associated with disorders of the function of the parathyroid gland. Some disorders may be purely anatomical resulting in an enlarged gland which will raise concern. Such benign disorders, such as parathyroid cyst, are not discussed here. Parathyroid diseases can be divided into those causing hyperparathyroidism, and those causing hypoparathyroidism.

Familial hypocalciuric hypercalcemia (FHH) is an inherited condition that can cause hypercalcemia, a serum calcium level typically above 10.2 mg/dL; although uncommon. It is also known as familial benign hypocalciuric hypercalcemia (FBHH) where there is usually a family history of hypercalcemia which is mild, a urine calcium to creatinine ratio <0.01, and urine calcium <200 mg/day.

<span class="mw-page-title-main">Sestamibi parathyroid scan</span> Procedure in nuclear medicine

A sestamibi parathyroid scan is a procedure in nuclear medicine which is performed to localize parathyroid adenoma, which causes Hyperparathyroidism. Adequate localization of parathyroid adenoma allows the surgeon to use a minimally invasive surgical approach.

References

  1. 1 2 3 4 5 Hu MI, Vassilopoulou-Sellin R, Lustig R, Lamont JP. "Thyroid and Parathyroid Cancers" in Pazdur R, Wagman LD, Camphausen KA, Hoskins WJ (Eds) Cancer Management: A Multidisciplinary Approach. 11 ed. 2008.
  2. ↑ Givi B, Shah JP (August 2010). "Parathyroid carcinoma". Clinical Oncology. 22 (6): 498–507. doi:10.1016/j.clon.2010.04.007. PMC   3781923 . PMID   20510594.
  3. ↑ Wei CH, Harari A (March 2012). "Parathyroid carcinoma: update and guidelines for management". Current Treatment Options in Oncology. 13 (1): 11–23. doi:10.1007/s11864-011-0171-3. PMID   22327883. S2CID   39872789.
  4. ↑ Dudney WC, Bodenner D, Stack BC (April 2010). "Parathyroid carcinoma". Otolaryngologic Clinics of North America. Thyroid and Parathyroid Surgery. 43 (2): 441–53, xi. doi:10.1016/j.otc.2010.01.011. PMID   20510726.
  5. ↑ Beus KS, Stack BC (August 2004). "Parathyroid carcinoma". Otolaryngologic Clinics of North America. Parathyroids. 37 (4): 845–54, x. doi:10.1016/j.otc.2004.02.014. PMID   15262520.
  6. 1 2 Healy SJ, Soe KK (2016-06-01). "Parathyroid Cancer Cases: A Single Center's Experience". AACE Clinical Case Reports. 2 (3): e221–e227. doi: 10.4158/EP15857.CR . ISSN   2376-0605.
  7. ↑ Lee JE (July 2005). "Predicting the presence of parathyroid carcinoma". Annals of Surgical Oncology. 12 (7): 513–514. doi:10.1245/ASO.2005.03.904. PMID   15952075. S2CID   195245925.
  8. ↑ "Endocrine Pathology" . Retrieved 2009-05-08.
  9. ↑ Wynne AG, van Heerden J, Carney JA, Fitzpatrick LA (July 1992). "Parathyroid carcinoma: clinical and pathologic features in 43 patients". Medicine. 71 (4): 197–205. doi: 10.1097/00005792-199207000-00002 . PMID   1518393. S2CID   25457018.
  10. ↑ Mohebati A, Shaha A, Shah J (December 2012). "Parathyroid carcinoma: challenges in diagnosis and treatment". Hematology/Oncology Clinics of North America. Rare Cancers. 26 (6): 1221–1238. doi:10.1016/j.hoc.2012.08.009. PMID   23116578.
  11. ↑ Givi B, Shah JP (August 2010). "Parathyroid carcinoma". Clinical Oncology. 22 (6): 498–507. doi:10.1016/j.clon.2010.04.007. PMC   3781923 . PMID   20510594.
  12. ↑ Givi B, Shah JP (August 2010). "Parathyroid carcinoma". Clinical Oncology. 22 (6): 498–507. doi:10.1016/j.clon.2010.04.007. PMC   3781923 . PMID   20510594.
  13. 1 2 3 "Parathyroid Cancer Treatment". National Cancer Institute. 11 March 2009.
  14. ↑ Cheon M, Choi JY, Chung JH, Lee JY, Cho SK, Yoo J, et al. (December 2011). "Differential findings of tc-99m sestamibi dual-phase parathyroid scintigraphy between benign and malignant parathyroid lesions in patients with primary hyperparathyroidism". Nuclear Medicine and Molecular Imaging. 45 (4): 276–284. doi:10.1007/s13139-011-0103-y. PMC   4043053 . PMID   24900018.
  15. ↑ Valdes-Socin H, Betea D, Daly A, Delanaye P, Souberbielle JC, Beckers A, Cavalier E (2018-05-08). "Human Chorionic Gonadotrophin (hCG) as a diagnostic test to differentiate between Parathyroid Carcinoma, Primary Benign Hyperparathyroidism and Secondary Hyperparathyroidism". Endocrine Abstracts. Bioscientifica. 56. doi:10.1530/endoabs.56.P214.
  16. ↑ Nguyen BD (May 1999). "Parathyroid imaging with Tc-99m sestamibi planar and SPECT scintigraphy". Radiographics. 19 (3): 601–614. doi:10.1148/radiographics.19.3.g99ma10601. PMID   10336191.

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