PARATHYROID

Primary Paraganglioma of the Parathyroid: A Case Report and Clinicopathologic Review.

Levy MT,  Braun JT, Pennant M, Thompson LDR.

Head and Neck Pathol J. 2009-12-24.

Paragangliomas are relatively uncommon neoplasms that arise in adrenal and extra-adrenal paraganglia of the autonomic nervous system. Parasympathetic paraganglioma develop predominantly in the head and neck. It is exceedingly uncommon to develop a primary intraparathyroid paraganglioma. There is only a single case report in the English literature. The information from the single previous case report (Medline 1960–2009) was combined with this case report. Our patient was a 69 year old woman who presented with a thyroid gland mass, with extension into the substernal space. The patient had a history of renal cell carcinoma removed 18 months before. At surgery, a thyroid lobectomy and a parathyroidectomy were performed. The parathyroid tissue showed a very well defined zellballen arrangement of paraganglion cells within the parenchyma of the parathyroid gland. The cells had ample basophilic, granular cytoplasm. The nuclei were generally round to oval with ‘salt-and-pepper’ nuclear chromatin distribution. There was a richly vascularized stroma. Mitotic figures, necrosis, invasive growth, and profound nuclear pleomorphism were absent. The neoplastic cells were strongly and diffusely immunoreactive with chromogranin, synaptophysin, CD56, and focally with cyclin-D1. The paraganglioma showed a delicate S-100 protein positive supporting sustentacular framework. Keratin, CD10, PTH, calcitonin and RCC markers were negative. The patient showed no stigmata of Multiple Endocrine Neoplasia (MEN) and has no paraganglioma in any other anatomic site. She is alive without any additional findings 12 months after surgery. Isolated paraganglioma within the parathyroid is rare, and should be separated from parathyroid adenoma, hyperplasia or metastatic disease to assure appropriate management.

PMID: to be determined

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Parathyroid carcinoma.

Thompson LDR.

Ear Nose Throat J. 2008 Sep; 87(9):502-504.

FIRST PARAGRAPH:  Parathyroid carcinoma is rare, comprising less than 1% of all cases of primary hyperparathyroidism. Parathyroid carcinoma occurs in patients of all ages, and there is no predilection for either sex. Its clinical features are primarily attributable to the effects of hypercalcemia and excessive secretion of parathyroid hormone (PTH). Most of its symptoms--weakness, fatigue, anorexia, weight loss, and nausea--are nonspecific, but an excessively high serum calcium level (>16 mg/dl) can be associated with nephrolithiasis, renal insufficiency, and bone "brown tumors." A palpable neck mass suggests a parathyroid carcinoma. Parathyroid carcinoma is a suggested component of hyperparathyroidism-jaw tumor syndrome.

PMID: 18800320

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Primary parathyroid hyperplasia.

Penner CR, Thompson LD.

Ear Nose Throat J. 2003 May;82(5):363.

FIRST PARAGRAPH: Parathyroid hyperplasia is classified as either primary, secondary, or tertiary. Primary parathyroid hyperplasia occurs in approximately 15% of patients with hyperparathyroidism. Most cases are sporadic, and they usually occur in patients who are middle-aged and older. Approximately 20% of all cases of primary chief-cell hyperplasia are associated with one of the multiple endocrine neoplasia syndromes. Symptoms are referable to the level and duration of serum calcium elevation, although routine biochemical testing has led to an increase in the identification of asymptomatic patients. Biochemically, ionized serum calcium levels are elevated, and serum phosphorus concentrations are lowered. Technetium-99m sestamibi imaging successfully localizes as many as 60% of hyperplastic glands, although this technique is significantly more effective in localizing adenomas and carcinomas. Therefore, at least two glands should be examined histologically to confirm the diagnosis.

PMID: 12789760

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Parathyroiditis.

Thompson LD.

Ear Nose Throat J. 2005 Oct;84(10):636.

FIRST PARAGRAPH: Parathyroiditis is a rare and poorly understood condition. Chronic parathyroiditis may occur in patients with hypoparathyroidism, as well as in those with primary chief-cell hyperplasia. Most patients are asymptomatic. Antibodies to parathyroid tissue are seen in only a few cases of parathyroiditis. It is thought that parathyroiditis represents an autoimmune process similar to thyroid gland Hashimoto’s disease. The presence of seronegative cases of parathyroiditis does not rule against an autoimmune etiology, because a similar phenomenon is observed in Hashimoto’s thyroiditis. Based on this assumption, it is believed that the lymphocytic infiltration is an ongoing destructive process.

PMID: 16382744

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Parathyroid carcinoma occurring in multiple endocrine neoplasia type 1.

Kodroff KS, Lieber C, Thompson LDR

Endocr Pract 1999;5:347-349

Objective: To describe a patient with multiple endocrine neoplasia type I (MEN-I) in whom parathyroid carcinoma developed.

Methods: A case report is presented, including detailed history, laboratory findings, and pathologic features.

Results: A 49-year-old man with a known history of MEN-I syndrome had hypercalcemia and profoundly increased parathyroid hormone levels.  Seven years earlier, he had been diagnosed with a gastrinoma, prolactinoma, and primary hyperparathyroidism. A neck exploration at that time consisted of resection of a right inferior parathyroid gland and parathyroid tissue in the thyroid gland as well as biopsies of two left-sided glands, after which microscopic examination revealed parathyroid hyperplasia and his serum calcium levels normalized. On reexploration of the neck, resection revealed pathologic f~ndings consistent with parathyroid carcinoma.

Conclusion: Parathyroid carcinoma has rarely been reported in the setting of MEN-I. This case illustrates the need for near-complete resection of parathyroid tissue in the patient with MEN-I. Subtotal or total parathyroidectomy in conjunction with forearm autotransplantation should be performed, not simply to prevent recurrence of benign disease but also to prevent the potential development of carcinoma over time.

PMID: 0

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