Heterogeneous size of the parathyroid glands in familial multiple endocrine neoplasia type 1

Marx, Stephen J.; Menczel, J; Campbell, Gregory; Aurbach, G. D.; Spiegel, Allen M.; Norton, Jeffrey A.

doi:10.1111/j.1365-2265.1991.tb00938.x

Cited by 66 publications

(26 citation statements)

References 16 publications

(8 reference statements)

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“…Therefore, parathyroid adenoma and hyperplasia could be the same disease entity, representing opposite ends of the spectrum of phenotypic expression. The prevalence of hyperplasia varies considerably in different studies, and there appears to be a strong influ- Single adenomas and parathyroid cancer have also been described in the setting of MEN 1 (49,50). One patient in our study with MEN 1 had an adenoma posterior to the superior pole of the right thyroid lobe and a hyperplastic parathyroid gland posterior to the inferior pole of the contralateral thyroid lobe.…”

Section: Discussionmentioning

confidence: 54%

Accuracy of parathyroid imaging: comparison of planar scintigraphy, spect, spect/ct and c-11 methionine pet for the detection of parathyroid adenomas and glandular hyperplasia

Öksüz

Dittmann²,

Wicke³

et al. 2010

Diagn Interv Radiol

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Sporadic primary hyperparathyroidism is the most common cause of hypercalcemia in the outpatient population, with a prevalence of one per 500 women and one per 2000 men over 40 years of age. Both increased cell proliferative activity and a decreased sensitivity of cells to secretory inhibition by calcium occur in hyperparathyroidism (1). The diagnosis of hyperparathyroidism is usually made by the demonstration of an inappropriately elevated parathyroid hormone level compared to the simultaneously measured serum calcium level (2). In 80% to 85% of patients, primary hyperparathyroidism is caused by one or more parathyroid adenomas, and in 15% to 20% of cases, it is the result of parathyroid hyperplasia. A rare cause of primary hyperparathyroidism, accounting for less than 1% of all cases, is parathyroid carcinoma. Persistent hyperparathyroidism occurs in 5% to 10% of all patients who undergo surgery for primary hyperparathyroidism, with a continuation of the pre-operative hypercalcemia in the immediate post-operative period. Hyperparathyroidism that presents after a period of more than six months of normocalcemia following surgery is called "recurrent hyperparathyroidism" and is commonly due to continuing growth of the remaining parathyroid glands (3). Secondary hyperparathyroidism is a compensatory hypertrophy of all parathyroid glands due to hypocalcemia, as occurs in renal failure or with vitamin D deficiency (4), whereas tertiary hyperparathyroidism describes the development of autonomous function of parathyroid tissue after longstanding secondary hyperparathyroidism (5-8).In most cases (80%-85%), parathyroid adenomas are found adjacent to the thyroid gland, which is the normal location for these adenomas. However, in up to 20% of cases, they are ectopically placed, e.g., in the anterosuperior, posterosuperior, or very rarely in the mid-lower mediastinum. Occasionally, parathyroid adenomas may be found within, or lateral to, the carotid sheath. Rarely, the lower parathyroid glands fail to migrate, remaining in the high neck anterior to the carotid bifurcation. Finally, 1.4% to 3.2% of all parathyroid adenomas are intrathyroidal, i.e., embedded completely within the thyroid gland (9).Among the several imaging procedures that have been developed for the detection and localization of parathyroid adenomas, dual-phase scintigraphy with Tc-99m sestamibi combined with ultrasonography is considered the imaging method of choice for pre-operatively localizing parathyroid adenomas. The overall accuracy of dual-phase scintigraphy combined with ultrasonography has been found to be superior to that of other scintigraphic or radiological techniques (10, 11). Studies comparing planar imaging and single photon emission computed tomography (SPECT) have shown significantly increased sensitivity for Tc-99m sestamibi SPECT, thus supporting its routine use prior to surgery (12-14). HEAD AND NECK IMAGING PURPOSETo compare the accuracy of planar scintigraphy, single photon emission computed tomography (SPECT), SPECT-CT, and positron...

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Section: Discussionmentioning

confidence: 54%

Accuracy of parathyroid imaging: comparison of planar scintigraphy, spect, spect/ct and c-11 methionine pet for the detection of parathyroid adenomas and glandular hyperplasia

Öksüz

Dittmann²,

Wicke³

et al. 2010

Diagn Interv Radiol

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“…In our patients pHPT was diagnosed between 7 years prior to, and 25 years after the development of other MEN 1-associated manifestations. Contrary to sporadic pHPT, which is expressed as single adenoma in the majority of cases, MEN 1-associated enlargement of the parathyroid is generally multiglandular with an asymmetric and metachronous evolution of disease [79], commonly with one or two glands of normal or minimally enlarged size [46]. In the Armed Forces Institute of Pathology fascicle [14] and the World Health Organisation classification [91] as well as in many case reports [23,27,33,61,66] MEN 1-associated parathyroid lesions are typed histopathologically as diffuse or nodular chief cell hyperplasia of all four glands.…”

Section: Morphological and Clinical Features Of Men 1 Lesionsmentioning

confidence: 99%

“…The MEN 1 gene still remains unidentified but is most likely considered a tumour suppressor gene [10,11,23,36,46,83], the inactivation of which gives rise to cell proliferation. The assumption that some heritable tumours (retinoblastomas, familial polyposis coli) result from mutational events is known as the two-hit hypothesis of carcinogenesis first introduced by Knudson [30].…”

Section: Pathogenesismentioning

confidence: 99%

Multiple endocrine neoplasia type 1 (MEN 1) revisited

Padberg

Schröder

Heitz

et al. 1995

Vichows Archiv A Pathol Anat

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Multiple endocrine neoplasia type 1 (MEN 1) is an inherited disease of the neuroendocrine cell system affecting primarily the parathyroids, pancreas, duodenum and the anterior pituitary. The pancreatic and duodenal turnouts may metastasize, but generally have a low malignant potential. The diagnosis of MEN 1 is usually made in the second decade of life and based on the involvement of at least two organs and a family history. The recent discovery of the MEN 1 locus on the centromeric region of the long arm of chromosome 11 may become a further diagnostic criterion. The use of flanking DNA markers permits presymptomatic testing for MEN 1 in affected families.

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“…Tumours in MEN 1 are thus monoclonal in origin but occur against a background of polyclonal hyperplasia. The heterogeneity of parathyroid glands has been demonstrated in several reports by comparing gland volume [10], ratio of size of the largest to the smallest gland [11], and gland weights [12,13]. One or more parathyroids may therefore appear macroscopically normal but account for the persistence of hypercalcaemia in a proportion of patients in whom inadequate resection is performed.…”

Section: Introductionmentioning

confidence: 96%

Primary hyperparathyroidism in MEN 1 – how radical should surgery be?

Hubbard

Sébag

Maweja

et al. 2002

Langenbeck’s Arch Surg

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Primary hyperparathyroidism is the most common manifestation of MEN 1 syndrome. The management of these patients is complex due to the underlying disease process, which predisposes patients to persistent and recurrent disease. The surgical treatment of patients with MEN 1 and hyperparathyroidism can therefore be considered to be palliative in nature. The basic principles of surgery include (1) obtaining and maintaining normocalcaemia for the longest time possible, avoiding persistent/recurrent hypercalcaemia, (2) avoiding surgically induced hypocalcaemia, and (3) facilitating future surgery for recurrent disease. Two approaches have been described as the best practice for patients with hyperparathyroidism in MEN 1: subtotal parathyroidectomy, leaving a remnant of no more than 60 mg in the neck, and total parathyroidectomy with immediate autotransplantation of 10-20 1 mm(3) pieces of parathyroid tissue. Both approaches should be combined with efforts to exclude supernumerary glands and ectopic parathyroid tissue by including resection of fatty tissue from the central neck compartment and thymectomy in all patients. Cryopreservation of parathyroid tissue should be performed whenever facilities are available. In patients with persistent or recurrent disease, an attempt to obtain total elimination of cervical parathyroid tissue is justified, combined with cryopreservation of parathyroid tissue. As radical as surgery is for hyperparathyroidism in MEN 1, the surgeon must take steps to avoid permanent hypoparathyroidism, which in young patients may be worse than the disease itself.

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Heterogeneous size of the parathyroid glands in familial multiple endocrine neoplasia type 1

Abstract: There is a wide heterogeneity in size of the parathyroid glands in the patients with FMEN1 and primary hyperparathyroidism.

Cited by 66 publications

References 16 publications

Accuracy of parathyroid imaging: comparison of planar scintigraphy, spect, spect/ct and c-11 methionine pet for the detection of parathyroid adenomas and glandular hyperplasia

Accuracy of parathyroid imaging: comparison of planar scintigraphy, spect, spect/ct and c-11 methionine pet for the detection of parathyroid adenomas and glandular hyperplasia

Multiple endocrine neoplasia type 1 (MEN 1) revisited

Primary hyperparathyroidism in MEN 1 – how radical should surgery be?

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