An Unusual Presentation of Primary Hyperparathyroidism: Ectopic Parathyroid Gland and Multiglandular Disease

Abstract Primary hyperparathyroidism (PHPT) can be associated as a single parathyroid adenoma in approximately 85% of patients, the remaining 15% of them correspond to individuals with hyperplasia. The multiglandular parathyroid disease varies in range 7–33% and contribute a persistent PHPT. We report a case of primary hyperparathyroidism with an unusual presentation of ectopic mediastinal parathyroid. The case was of a 54-year-old female, who started her illness 3 years ago, with frequent headache, regular fatigue and muscle pain, denied losing weight. She was admitted to emergency room for a urinary infection. During the hospitalization showed bilateral nephrocalcinosis, hypercalcemic crisis. She has a background of total thyroidectomy and subtotal parathyroidectomy because a thyroid adenomatous hyperplasia and parathyroid hyperplasia with removal of a total of three parathyroid glands. She had very high PTH level. Laboratory: serum calcium: 16.8mg/dl; ionic calcium: 2.37 mmol/L; P: 1.9 (2.7–4.5g/dL); Hb: 9 g/dL; Platelets: 558 000 per microliter; Leukocytes 9 600 cells/mcL; Albumin: 4g/dl; TSH: 0.06 mU/L; fT4: 1.29 (L-T4 doses were 100 ug). Four months after parathyroid surgery, the intact PTH level dropped from 1602 ng/l to 550 (15–65 ng/L). Computed tomography and Tc-sestamibi scintigraphy - SPECT revealed a residual cervicothoracic mass in retroesophageal region (D1-D2) (Fig1 -2). The patient underwent a new surgery and the intact PTH dropped 39ng/L. Histopathology revealed characteristic features of a parathyroid adenomatous (10 gram weight) (Fig.3); additionally a retroesternal ectopic thyroid tissue. There was not reappearance of high blood calcium and parathormone levels more than 6 months after second surgery for PHPT. An unusual case of PHPT caused by a multiglandular disease parathyroid varies (four glands and a ectopic gland), reliable histopathologic adenomatous and hyperplastic parathyroid disease, and persistent primary hyperparathyroidism with very high serum intact PTH level. Reference: (1) Masi L. Primary Hyperparathyroidism. Brandi ML (ed): Parathyroid Disorders. Focusing on Unmet Needs. Front Horm Res. Basel, Karger, 2019, vol 51, pp 1–12. (2) Pecheva M, Mahendran K, Kadlec J, Lofthouse M1, Van Tornout F. Mediastinal giant parathyroid adenoma-a minimally invasive mediastinal surgical approach for an emergency presentation. Ann Cardiothorac Surg. 2016 Jan;5(1):70–3. (3) Cakmak H 1, Tokat AO, Karasu S, Özkan M. Adenoma paratiroideo mediastínico gigante. Tuberk Toraks. 2011; 59 (3): 263–5. (4)Thier M, Daudi S, Bergenfelz A, Almquist M. Predictors of multiglandular disease in primary hyperparathyroidism. Langenbecks Arch Surg. 2018;403(1):103–109.

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An Unusual Presentation of Primary Hyperparathyroidism: Pathological Fracture

Case Reports in Orthopedics ◽

10.1155/2011/521578 ◽

2011 ◽

Vol 2011 ◽

pp. 1-3 ◽

Cited By ~ 1

Author(s):

Ben Abdelghani Khaoula ◽

Ben Abdelghani Kaouther ◽

Chelly Ines ◽

Turki Sami ◽

Leith Zakraoui ◽

...

Keyword(s):

Primary Hyperparathyroidism ◽

Female Patient ◽

Calcium Level ◽

Pathological Fracture ◽

Unusual Presentation ◽

Uneventful Recovery ◽

Brown Tumor ◽

Pathological Findings ◽

Pth Level

Primary hyperparathyroidism revealed by a pathological fracture is very uncommon. We present a case of a 54-year-old female patient who was admitted with fracture of her right femur. She underwent closed intramedullary reconstruction nailing with bipolar locking. The pathological findings confirmed the diagnosis of primary hyperparathyroidism with brown tumor. Further tests showed increased both calcium level and PTH level. A parathyroidectomy was performed. She made an uneventful recovery and was discharged to home.

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Night Sweats as the Presenting Symptom of Primary Hyperparathyroidism

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.421 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A207-A207

Author(s):

Vanessa Williams ◽

Hadoun Jabri ◽

Michael G Jakoby

Keyword(s):

Primary Hyperparathyroidism ◽

Parathyroid Adenoma ◽

Serum Calcium ◽

Vasomotor Symptoms ◽

Temperature Threshold ◽

Hot Flushes ◽

Parathyroid Surgery ◽

Intact Pth ◽

Patient Reported ◽

Night Sweats

Abstract Background: Approximately 25–40% of patients report night sweats in the previous month during appointments with their primary care clinicians. The differential diagnosis for night sweats is broad, with hyperthyroidism, carcinoid syndrome, pheochromocytoma, medullary thyroid carcinoma, insulinoma, and acromegaly as established endocrine causes. We present a case of primary hyperparathyroidism (PHPT) in which the patient’s chief complaint was night sweats and resolution occurred after parathyroidectomy. Case. A 39-year-old female reported one-year of daily night sweats that required changes of clothes and bedding. She denied excessive daytime sweating, frequent palpitations, tremors, nightmares, rashes, fevers, chills, cough, headaches, dizziness, abdominal pain, diarrhea, disrupted menses, or unintentional weight loss. Vital signs and examination were unremarkable. Hypercalcemia (11.0 mg/dL, 8.6–10.3) was noted and confirmed by additional serum calcium measurements. Intact PTH ranged from 27–33 pg/mL (12–88), and 24 h urine calcium (258 mg) excluded familial hypocalciuric hypercalcemia (FHH). Parathyroid scintigraphy and neck ultrasound identified a left neck mass, and the patient underwent successful resection of a left inferior parathyroid adenoma. Hypercalcemia and night sweats initially resolved after surgery, but the patient returned six weeks later with recurrence of night sweats. Reevaluation was notable for serum calcium 10.4 mg/dL, phosphorus 2.4 mg/dL (2.5–5.0), and intact PTH 104 pg/mL. A right superior parathyroid adenoma was identified on repeat parathyroidectomy, and the patient experienced durable resolution of night sweats and hypercalcemia following her second parathyroid surgery. She was screened for multiple endocrine neoplasia type 1 (MEN1) due to multiple parathyroid tumors, though no known pathogenic menin gene variants were identified. Conclusions: A title/abstract search in PubMed linking “hyperparathyroidism” and “hypercalcemia” to “night sweats,” “sleep hyperhidrosis,” “sweating,” “hot flashes,” “hot flushes,” “diaphoresis” and “vasomotor symptoms” yielded only one relevant case of a postmenopausal woman with hot flushes unresponsive to hormone replacement that resolved after parathyroidectomy for PHPT. Hypercalcemia is known to affect central nervous system function. It is possible that in rare cases hypercalcemia alters function of the medial preoptic area, lowering the temperature threshold above which peripheral vasodilatation and perspiration occur to dissipate heat. The patient’s predisposition to only night sweats is unclear, though unlike the first patient reported with PHPT and sweating, our patient is premenopausal. This case indicates that vasomotor symptoms may occur with PHPT and resolve after successful parathyroid surgery.

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Surgeon-Performed Ultrasound Improves Localization of Abnormal Parathyroid Glands

The American Surgeon ◽

10.1177/000313480507100704 ◽

2005 ◽

Vol 71 (7) ◽

pp. 557-563 ◽

Cited By ~ 9

Author(s):

Carmen C. Solorzano ◽

Theresa M. Lee ◽

Marcela C. Ramirez ◽

Denise M. Carneiro ◽

George L. Irvin

Keyword(s):

Parathyroid Hormone ◽

Primary Hyperparathyroidism ◽

Thyroid Tissue ◽

Parathyroid Tissue ◽

Initial Step ◽

Multiglandular Disease ◽

Venous Sampling ◽

Sporadic Primary Hyperparathyroidism ◽

Operative Findings ◽

Abnormal Tissue

With a secure diagnosis of hyperparathyroidism, preoperative localization of abnormal glands is the initial step toward limited parathyroidectomy (LPX). We investigated whether ultrasonography in the hands of the surgeon (SUS) could improve the localization of abnormal parathyroids when sestamibi scans (MIBI) were negative or equivocal. One hundred eighty patients with sporadic primary hyperparathyroidism (SPHPT) underwent preoperative SUS and MIBI scans before LPX guided by intraoperative parathormone assay. When the sestamibi scans were negative, SUS was used to localize the parathyroid, distinguish parathyroid from thyroid tissue, and to guide the intraoperative jugular venous sampling for differential elevation of parathyroid hormone (PTH). Operative findings, intraoperative hormone dynamics, and postoperative calcium levels determined successful localization. MIBI was negative or equivocal in 36/180 (20%) patients: 1) showed no parathyroid gland in 22 patients, 2) suggested an incorrect location for the abnormal gland in 9, and 3) was insufficient in recognizing multiglandular disease in 5. In these 36 patients, the addition of SUS led to the successful identification of the abnormal tissue in 19/36 (53%). In the remaining 17 patients with negative/equivocal scans, the parathyroid could not be clearly visualized by SUS. In these patients, SUS facilitated LPX by aiding preoperative transcutaneous jugular venous sampling for differentially elevated PTH (n = 3) and identifying questionable thyroid nodule versus parathyroid tissue (n = 1). Overall, SUS was useful in 23/36 (67%) patients with nonlocalizing MIBI scans, thus improving the rate of localization from 80 per cent to 93 per cent ( P < 0.01). Surgeon-performed cervical ultrasonography improved the localization of abnormal parathyroids by MIBI scan, adding to the success of limited parathyroidectomy.

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SAT-369 Parathyroid Microadenomas as a Cause of Normocalcemic Primary Hyperparathyroidism (PHPT) and a Surgical Challenge

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.1503 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Tiffany Nguyen ◽

Marc D Coltrera ◽

Jing H Chao

Keyword(s):

Primary Hyperparathyroidism ◽

Parathyroid Adenoma ◽

Serum Calcium ◽

Compression Fracture ◽

Extended Period ◽

Significant Loss ◽

Mineral Density ◽

Dxa Scan ◽

Normal Bone Mineral Density ◽

Pth Level

Abstract Background: Patients with normocalcemic primary hyperparathyroidism (PHPT) share similar risks of osteoporosis and nephrolithiasis with patients having hypercalcemic PHPT. The prevalence and natural history of normocalcemic PHPT is not well defined. Parathyroid microadenomas weighing < 100 mg may present as special clinical challenges in both the diagnosis and surgical resection. Clinical Case: A 55-year-old woman presented for an evaluation of osteoporosis following a non-traumatic L1 compression fracture at the age of 50 years. Noting an initial serum calcium of 10 mg/dL, we diagnosed her with normocalcemic PHPT based on a concurrent parathyroid hormone (PTH) level of 112 pg/mL (12-88 pg/mL) and a lack of other causes for an elevated PTH, including a normal eGFR, serum 25-hydroxyvitamin D, 24-hour urine calcium, and autoantibodies for celiac disease. Initial dual-energy x-ray absorptiometry (DXA) scan from 2015 showed normal bone mineral density (BMD) and T-scores in the L-spine, total hip, and femoral neck. Neck imagings, including ultrasound, sestamibi scan, and 4D CT, failed to localize a parathyroid adenoma. In the absence of a target and indications for parathyroidectomy other than osteoporosis, she was treated with bisphosphonates. While her serum calcium remained normal (9.6-10.2 mg/dL) despite an elevated PTH level (92-116 pg/mL) over a two-year period, subsequent DXA scans showed a significant loss in BMD on the bisphosphonates, prompting a peripheral DXA scan, which revealed severe osteoporosis at the distal third of the forearm (BMD 0.450 g/cm2, T-score -3.7), consistent with PTH-associated bone resorption. Following a 4-gland exploration with removal of 2 parathyroid microadenomas (63 and 100 mg), intra- and post-operative PTH levels normalized. She now awaits a repeat DXA scan at 1 year after a successful parathyroidectomy. Conclusion: Although many patients with normocalcemic PHPT may progress to develop hypercalcemia, some remain normocalcemic over an extended period. We report a patient who maintained normocalcemia for over 2 years. To correctly establish the diagnosis of normocalcemic PHPT, a comprehensive evaluation must be performed to exclude secondary causes of an elevated PTH (renal insufficiency, hypovitaminosis D, hypercalciuria, and malabsorption). Since significant osteoporosis may be preferentially seen in the cortical bone, BMD at the distal 1/3 of forearm should be evaluated in addition to the L-spine, hips and femoral necks. When a parathyroid adenoma cannot be localized in patients with normocalcemic PHPT, a careful 4-gland exploration for parathyroid microadenoma(s), paying special attention to the size, texture, and shape of the glands, and guided by intraoperative PTH levels, ensures the successful removal of the culprit gland(s).

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A Familial Case of a Whole Germline CDC73 Deletion Discordant for Primary Hyperparathyroidism

Hormone Research in Paediatrics ◽

10.1159/000495800 ◽

2019 ◽

Vol 92 (1) ◽

pp. 56-63

Author(s):

Naomi Hatabu ◽

Naho Katori ◽

Takeshi Sato ◽

Naonori Maeda ◽

Eri Suzuki ◽

...

Keyword(s):

Primary Hyperparathyroidism ◽

Phenotypic Variability ◽

Early Adulthood ◽

Familial Case ◽

Incomplete Penetrance ◽

Heterozygous Deletion ◽

Imaging Studies ◽

Intact Pth ◽

Pathogenic Variants ◽

Pth Level

Introduction: Primary hyperparathyroidism (PHPT) occurs as part of familial syndromes, including CDC73-related disorders caused by germline pathogenic variants of the CDC73 gene, particularly in early adulthood. Herein, we report a familial case of a whole germline CDC73 deletion discordant for PHPT. Case Description: A 15-year-old boy was admitted to our hospital because of persistent nausea and vomiting. Laboratory tests showed hypercalcemia (13.6 mg/dL), hypophosphatemia (2.4 mg/dL), and elevated intact PTH level (149 pg/mL). Imaging studies showed an enlarged single parathyroid gland. Thus, the diagnosis of PHPT was made. Microarray analysis of peripheral blood DNA showed a 3.4-Mb heterozygous deletion of 1q31 encompassing 11 genes, including CDC73. Total thyroidectomy/parathyroidectomy was performed; histology was compatible with parathyroid adenoma without any evidence of malignancy. DNA sequencing of the removed adenoma confirmed a hemizygous nonsense variant in the CDC73 gene in a mosaic manner, which was potentially involved in parathyroid tumorigenesis as the “second hit.” Importantly, the same deletion was identified in his 52-year-old father who had an unremarkable medical history. Conclusions: These data clearly demonstrate the Knudson two-hit theory from a molecular viewpoint. Phenotypic variability and incomplete penetrance of CDC73-related disorders, even if caused by a gross deletion, should be noted in a clinical setting.

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Abstract #838: A Case of Parathyroid Adenoma with Normal PTH Level: A Special Entity for Primary Hyperparathyroidism

Endocrine Practice ◽

10.1016/s1530-891x(20)45172-9 ◽

2016 ◽

Vol 22 ◽

pp. 181

Author(s):

Ilgin Yildirim Simsir ◽

Hatice Ozisik ◽

Banu Pinar Sarer Yureli ◽

Ilker Altun ◽

Mehmet Erdogan ◽

...

Keyword(s):

Primary Hyperparathyroidism ◽

Parathyroid Adenoma ◽

Pth Level

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ONCOCYTIC INTRATHYROID PARATHYROID ADENOMA MASQUERADING AS HURTHLE CELL NEOPLASM: FINE NEEDLE ASPIRATION CYTOLOGY OF TWO CASES

10.36106/ijsr/0430540 ◽

2021 ◽

pp. 57-58

Author(s):

Mega Lahori ◽

Hua Chen

Keyword(s):

Parathyroid Adenoma ◽

Thyroid Tissue ◽

Parathyroid Tissue ◽

Needle Aspiration ◽

Thyroid Lobe ◽

Cell Adenoma ◽

Hürthle Cell ◽

Hypoechoic Nodule ◽

Left Thyroid Lobe ◽

Pth Level

Ectopic parathyroid adenomas in thyroid tissue are uncommon (0.7 - 6%), and their oncocytic variants are exceedingly rare. We report two cases of intrathyroid parathyroid adenoma which were diagnosed as Hurthle cell adenoma on cytology. Case 1 is a 49-year-old female with a 2.4 cm hypoechoic nodule in the left lateral neck. Electrochemiluminescent immunoassay of the aspirate revealed PTH level of 422 pg/ml, conrming the presence of hyperfunctional parathyroid tissue. Subsequent resection of the left thyroid lobe revealed an enlarged intrathyroidal parathyroid. Case 2 is a 58-year-old female with a 2.1 cm hypoechoic nodule in the posterior-mid left thyroid lobe. Strong overexpression of parathyroid hormone and Chromogranin A genes and low expression of thyrocyte-related genes suggested parathyroid origin of the cells sampled. MEN1 mutation and multiple copy number alterations indicated the neoplastic nature of the nodule. Parathyroid oxyphil cells and oncocytic thyrocytes share cytomorphological ndings and distinguishing them by cytology alone is challenging, especially when the targeted lesion is intrathyroidal. Distinguishing intrathyroid oncocytic parathyroid adenoma from oncocytic thyroid follicular lesions has signicant clinical implications as Bethesda-IV category lesions have 20%–30% risk of malignancy. While stippled chromatin or intracytoplasmic fat vacuoles may be suggestive of parathyroid origin, these are not specic. Classifying the origin of a nodule as parathyroid vs thyroid rests upon the detection of PTH in aspirate material by ECL, immunocytochemistry or next-generation sequencing. In parathyroid aspirates, PTH level 100 pg/mL is suggestive of the presence of PTH-secre ≥ ting tissue at the site biopsied or along the needle track.

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A Parathyroid Adenoma: Benign Disease Presenting with Hyperparathyroid Crisis

Case Reports in Medicine ◽

10.1155/2010/596185 ◽

2010 ◽

Vol 2010 ◽

pp. 1-4

Author(s):

A. S. Tahim ◽

J. Saunders ◽

P. Sinha

Keyword(s):

Parathyroid Adenoma ◽

Epigastric Pain ◽

Cystic Mass ◽

Benign Disease ◽

Thyroid Lobe ◽

Ultrasound Studies ◽

Rare Manifestation ◽

Parathyroid Disease ◽

Endocrine Surgeon ◽

Pth Level

Hyperparathyroid crisis is a rare manifestation of parathyroid disease. We present the case of a 53-year-old gentleman with a review of the current literature. He presented in acute renal failure with epigastric pain and vomiting. His serum-corrected calcium (CCa2+) was raised at 5.2 mmol/L, in addition to a massively raised parathyroid hormone (PTH) level (3957 ng/L). Ultrasound studies of the neck revealed a 2 cm well-defined mass inferoposterior to right thyroid lobe. CT scans of the neck showed a normal mediastinum and confirmed no associated lymphadenopathy. Having undergone medical resuscitation for 9 days, a neck exploration revealed a cystic mass, which was excised. Histological investigations revealed a 9.25 g, cystic parathyroid adenoma with no features of malignancy. His PTH and CCa2+returned to normal postoperatively. This suspicious presentation of benign disease, including a marked elevation in PTH, highlights the challenges facing the endocrine surgeon in dealing with parathyroid disease.

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Parathyroid Elastography―Elastography Evaluation Algorithm

Timisoara Medical Journal ◽

10.35995/tmj20200105 ◽

2020 ◽

Vol 2020 (1) ◽

pp. 1

Author(s):

Laura Cotoi ◽

Daniela Amzăr ◽

Ioan Sporea ◽

Andreea Borlea ◽

Oana Schiller ◽

...

Keyword(s):

Primary Hyperparathyroidism ◽

Secondary Hyperparathyroidism ◽

Muscle Tissue ◽

Thyroid Tissue ◽

Parathyroid Tissue ◽

Parathyroid Glands ◽

Cervical Lymph Nodes ◽

Cardiovascular Morbidity And Mortality ◽

Parathyroid Lesions ◽

Parathyroid Disease

(1) Background: Primary hyperparathyroidism is a common disorder of the parathyroid glands and the third most frequent endocrinopathy, especially among elderly women. Secondary hyperparathyroidism is a common complication of chronic kidney disease, associated with high cardiovascular morbidity and mortality. In both primary and secondary hyperparathyroidism, the need to correctly identify the parathyroid glands is mandatory for a better outcome. Elastography can be an effective tool in the diagnosis of parathyroid lesions, by differentiating possible parathyroid lesions from thyroid disease, cervical lymph nodes, and other anatomical structures. There are currently no guidelines or recommendations and no established values on the elasticity of parathyroid lesions. (2) Material and Methods: In our studies, we have evaluated, by Shear Wave elastography (SWE), both primary and secondary hyperparathyroidism, determining that parathyroid glands have a higher elasticity index than both thyroid tissue and muscle tissue. (3) Results: For primary hyperparathyroidism, we have determined, using 2D-SWE, the parathyroid adenoma tissue (mean elasticity index (EI) measured by SWE 4.74 ± 2.74 kPa) with the thyroid tissue (11.718 ± 4.206 kPa) and with the surrounding muscle tissue (16.362 ± 3.829 kPa). For secondary hyperparathyroidism, by SWE elastographic evaluation, we have found that the mean EI in the parathyroid gland was 7.83 kPa, a median value in the thyroid parenchyma of 13.76 kPa, and a mean muscle EI value at 15.78 kPa. (4) Conclusions: Elastography can be a useful tool in localizing parathyroid disease, whether primary or secondary, by correctly identifying the parathyroid tissue. We have determined that an EI below 7 kPa in SWE elastography correctly identifies parathyroid tissue in primary hyperparathyroidism, and that a cut-off value of 9.98 kPa can be used in 2D-SWE to accurately diagnose parathyroid disease in secondary hyperparathyroidism.

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An Unusual Presentation of Calciphylaxis Due to Primary Hyperparathyroidism

Archives of Pathology & Laboratory Medicine ◽

10.5858/2001-125-1351-aupocd ◽

2001 ◽

Vol 125 (10) ◽

pp. 1351-1353

Author(s):

Imran Mirza ◽

Damanjeet Chaubay ◽

Himanshu Gunderia ◽

Winston Shih ◽

Hani El-Fanek

Keyword(s):

Primary Hyperparathyroidism ◽

Parathyroid Adenoma ◽

Parathyroid Gland ◽

Complete Recovery ◽

Skin Lesions ◽

Unusual Presentation ◽

Surgical Removal ◽

Review Of The Literature ◽

Clinical Recovery

Abstract We present the case of a 69-year-old woman with calciphylaxis due to primary hyperparathyroidism. A 0.5-g parathyroid adenoma was surgically removed, which resulted in complete recovery of the patient. Review of the literature revealed 7 other cases of calciphylaxis due to primary hyperparathyroidism and showed that prompt surgical removal of the autonomous parathyroid gland lesion results in clinical recovery of calciphylactic skin lesions.

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