scholarly journals Ectopic Intact PTH Secretion Causing Humoral Hypercalcemia of Malignancy

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A191-A192
Author(s):  
Mohammad Talha Rauf ◽  
Winnie Nhan ◽  
Gregory A Clines ◽  
Shafaq Khairi
Keyword(s):  
Primary Hyperparathyroidism ◽  
Somatostatin Receptor ◽  
Liver Lesion ◽  
Receptor Expression ◽  
Parathyroid Glands ◽  
Pathological Examination ◽  
Venous Sampling ◽  
Humoral Hypercalcemia Of Malignancy ◽  
Intact Pth ◽  
Subtotal Parathyroidectomy

Abstract Background: Tumor-generated ectopic intact PTH is difficult to diagnose and should be suspected in patients with apparent primary hyperparathyroidism but with normal parathyroid glands. Clinical Case: A 72-year-old man presented with symptoms of hypercalcemia including generalized weakness, polyuria, and polydipsia. Initial labs were consistent with primary hyperparathyroidism: calcium 12.1 mg/dL (n 8.6–10.3 mg/dL, albumin-corrected 12.5 mg/dL), intact PTH (iPTH) 115.6 pg/mL (n 10–65 pg/mL), low normal 25-OH vitamin D (25 ng/mL, n 25–100 ng/mL), and relatively high normal 1,25 dihydroxyvitamin D (52 pg/mL, n 18–78 pg/mL). 24-hour urine calcium was 381 mg/day (n 100–300 mg/day) and PTHrP was 1.6 pmol/L (n <4.2 pmol/L). Neck ultrasound demonstrated a 0.5 x 1 cm hypoechoic mass near right thyroid inferior pole, though sestamibi SPECT/CT scan did not reveal scintographic evidence of a parathyroid adenoma. He underwent subtotal parathyroidectomy with largest excised gland weighing 0.262 grams. The left inferior parathyroid gland appeared normal intraoperatively, thus was clipped and left in place. PTH decreased from 194 pg/mL to 98 pg/mL postoperatively. Pathological examination revealed three normocellular parathyroid glands with enlargement of only the right superior gland. Venous sampling of the parathyroid vasculature failed to identify the source of autonomous iPTH post operatively. Due to refractory hypercalcemia, cinacalcet was initiated. However, hypercalcemia as high as 12.6 mg/dl and hyperparathyroidism to 672 pg/mL persisted despite dose escalation. He eventually received pamidronate with subsequent transition to denosumab due to declining renal function. A  68Ga DOTATATE scan was performed to locate occult ectopic parathyroid, which reported multiple foci of presumed somatostatin receptor expression involving the liver and intra-abdominal lymph nodes without significant uptake in the neck concerning for metastatic disease. Liver lesion biopsy was consistent with pancreato-biliary adenocarcinoma. Surprisingly, the biopsy was negative for iPTH and neuroendocrine tumor markers on staining/immunohistochemistry. Given his poor prognosis and multiple comorbidities, the patient opted not to pursue any further workup or therapy for his malignancy. Conclusion: Occult malignancy should be suspected for a patient with persistent hyperparathyroidism after parathyroidectomy. Treatment of the malignancy may lead to an improvement in hypercalcemia and iPTH levels. Employment of iPTH mRNA testing or intra-abdominal venous sampling to prove ectopic iPTH secretion would be ideal, as iPTH staining could be falsely negative. Further testing was not completed as the patient declined further evaluation.

scholarly journals Relationship between parathyroid calcium-sensing receptor expression and potency of the calcimimetic, cinacalcet, in suppressing parathyroid hormone secretion in an in vivo murine model of primary hyperparathyroidism

2005 ◽  
Vol 153 (4) ◽  
pp. 587-594 ◽  
Author(s):  
Takehisa Kawata ◽  
Yasuo Imanishi ◽  
Keisuke Kobayashi ◽  
Takao Kenko ◽  
Michihito Wada ◽  
...  
Keyword(s):  
Parathyroid Hormone ◽  
Primary Hyperparathyroidism ◽  
Secondary Hyperparathyroidism ◽  
Murine Model ◽  
Hormone Secretion ◽  
Suppressive Effect ◽  
Receptor Expression ◽  
Parathyroid Glands ◽  
Calcium Sensing Receptor ◽  
Calcium Sensing

Cinacalcet HCl, an allosteric modulator of the calcium-sensing receptor (CaR), has recently been approved for the treatment of secondary hyperparathyroidism in patients with chronic kidney disease on dialysis, due to its suppressive effect on parathyroid hormone (PTH) secretion. Although cinacalcet’s effects in patients with primary and secondary hyperparathyroidism have been reported, the crucial relationship between the effect of calcimimetics and CaR expression on the parathyroid glands requires better understanding. To investigate its suppressive effect on PTH secretion in primary hyperparathyroidism, in which hypercalcemia may already have stimulated considerable CaR activity, we investigated the effect of cinacalcet HCl on PTH-cyclin D1 transgenic mice (PC2 mice), a model of primary hyperparathyroidism with hypo-expression of CaR on their parathyroid glands. A single administration of 30 mg/kg body weight (BW) of cinacalcet HCl significantly suppressed serum calcium (Ca) levels 2 h after administration in 65- to 85-week-old PC2 mice with chronic biochemical hyperparathyroidism. The percentage reduction in serum PTH was significantly correlated with CaR hypo-expression in the parathyroid glands. In older PC2 mice (93–99 weeks old) with advanced hyperparathyroidism, serum Ca and PTH levels were not suppressed by 30 mg cinacalcet HCl/kg. However, serum Ca and PTH levels were significantly suppressed by 100 mg/kg of cinacalcet HCl, suggesting that higher doses of this compound could overcome severe hyperparathyroidism. To conclude, cinacalcet HCl demonstrated potency in a murine model of primary hyperparathyroidism in spite of any presumed endogenous CaR activation by hypercalcemia and hypo-expression of CaR in the parathyroid glands.

scholarly journals Persistent primary hyperparathyroidism: an uncommon location for an ectopic gland - Case report and review

2012 ◽  
Vol 56 (6) ◽  
pp. 393-403 ◽  
Author(s):  
Sofia Gouveia ◽  
Dírcea Rodrigues ◽  
Luísa Barros ◽  
Cristina Ribeiro ◽  
Anabela Albuquerque ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Gland ◽  
Median Sternotomy ◽  
Parathyroid Glands ◽  
Aortopulmonary Window ◽  
Genetic Syndromes ◽  
Subtotal Parathyroidectomy ◽  
Common Causes ◽  
Ectopic Parathyroid ◽  
99Mtc Sestamibi

Primary hyperparathyroidism (PHPT) is a common endocrine disorder that mainly affects middle-aged women. Patients are usually asymptomatic. The disease might be ascribable to hyperplasia, carcinoma, and single or multiple adenomas. PHPT may be sporadic or familial, the latter comprising multiple endocrine neoplasia type 1 or 2A, familial benign hypocalciuria hypercalcemia, and hyperparathyroidism-jaw tumor syndrome. The most common causes for persistent PHPT are multiglandular disease, and missed abnormal ectopic or orthotopic parathyroid glands. Imaging localization studies should precede a new surgical intervention. Ectopic parathyroid glands are rarely located at the aortopulmonary window. For diagnosis confirmation, 99mTc-sestamibi SPECT/CT seems to be an advantageous test. Another possibility is to perform 99mTc-sestamibi followed by thoracic CT or MRI. Parathyroidectomy may be performed by means of median sternotomy, thoracotomy, or video-assisted thoracoscopy. We describe a case of persistent primary hyperparathyroidism due to the presence of an ectopic parathyroid gland found at the aortopulmonary window. As the investigation necessary to clarify the etiology of recurrent nephrolithiasis proceeded, the diagnosis of PHPT was determined. The patient underwent subtotal parathyroidectomy; nevertheless, PHPT persisted. Genetic syndromes that could account for this condition were excluded. Imaging studies available at that time were not able to locate abnormal glands; moreover, the patient refused to undergo surgical exploration. Later, the patient underwent 99mTc-sestamibi SPECT/CT, which revealed a parathyroid gland at the aortopulmonary window.

scholarly journals Somatostatin receptor expression in parathyroid neoplasms

2019 ◽  
Vol 8 (8) ◽  
pp. 1213-1223 ◽  
Author(s):  
Sara Storvall ◽  
Helena Leijon ◽  
Eeva Ryhänen ◽  
Johanna Louhimo ◽  
Caj Haglund ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Carcinoma ◽  
Somatostatin Receptor ◽  
Somatostatin Receptors ◽  
Receptor Expression ◽  
Benign Tumors ◽  
Receptor Subtypes ◽  
Receptor Imaging ◽  
Somatostatin Receptor Subtypes ◽  
Parathyroid Adenomas

Introduction Parathyroid carcinoma represents a rare cause of primary hyperparathyroidism. Distinguishing carcinoma from the benign tumors underlying primary hyperparathyroidism remains challenging. The diagnostic criteria for parathyroid carcinoma are local and/or metastatic spreading. Atypical parathyroid adenomas share other histological features with carcinomas but lack invasive growth. Somatostatin receptors are commonly expressed in different neuroendocrine tumors, but whether this also holds for parathyroid tumors remains unknown. Aim Our aim is to examine the immunohistochemical expression of somatostatin receptor 1–5 in parathyroid typical adenomas, atypical adenomas and carcinomas. Methods We used a tissue microarray construct from a nationwide cohort of parathyroid carcinomas (n = 32), age- and gender-matched typical parathyroid adenomas (n = 72) and atypical parathyroid adenomas (n = 27) for immunohistochemistry of somatostatin receptor subtypes 1–5. We separately assessed cytoplasmic, membrane and nuclear expression and also investigated the associations with histological, biochemical and clinical characteristics. Results All parathyroid tumor subgroups expressed somatostatin receptors, although membrane expression appeared negligible. Except for somatostatin receptor 1, expression patterns differed between the three tumor types. Adenomas exhibited the weakest and carcinomas the strongest expression of somatostatin receptor 2, 3, 4 and 5. We observed the largest difference for cytoplasmic somatostatin receptor 5 expression. Conclusions Parathyroid adenomas, atypical adenomas and carcinomas all express somatostatin receptor subtypes 1–5. Somatostatin receptor 5 may serve as a potential tumor marker for malignancy. Studies exploring the role of somatostatin receptor imaging and receptor-specific therapies in patients with parathyroid carcinomas are needed.

scholarly journals MON-912 Undiagnosed Chronic Eczema as a Presentation of Glucagonoma in MEN 1 Syndrome

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Siroj Dejhansathit ◽  
Parinya Samakkarnthai ◽  
Subhanudh Thavaraputta ◽  
Ana Marcella Rivas Mejia ◽  
Kenneth Nugent ◽  
...  
Keyword(s):  
Somatostatin Receptor ◽  
Skin Lesions ◽  
Parathyroid Glands ◽  
Pancreatic Tumors ◽  
Topical Steroids ◽  
Erythematous Plaque ◽  
Subtotal Parathyroidectomy ◽  
Normal Ranges ◽  
Necrolytic Migratory Erythema ◽  
Chronic Eczema

Abstract Background: Glucagonomas are pancreatic tumors arising from the islets cell of Langerhans that over secrete glucagon. Necrolytic migratory erythema (NME) is an important feature for the recognition of glucagonomas. Glucagonomas occurring in MEN1 is infrequent and seen in less than 3% of all glucagonomas. Clinical Case: A 51-year-old male presented to the clinic multiple visits for rash affecting the legs and genital area of two months. His medical history include type 2 DM and HT. The rash was attributed to subacute eczema and treated with topical steroids but showed no improvement. The skin eruption initially appeared on lower extremities progressed to trunk, and face. The skin lesions were associated with weight loss and stomatitis. On physical examination, skin showed ill-defined erythematous plaque exhibiting annular pattern, scale, and erosion on all extremities and perioral area. When the skin lesions healed, the new cutaneous eruptions occurred. Laboratory testing revealed plasma glucose of 185 mg/dL. The skin biopsy reported vacuolated keratinocytes in the epidermis with eosinophil cytoplasm, compatible with NME leading to further workup for pancreatic tumor. CT abdomen revealed tumor mass 9.6x6 cm at the pancreatic tail and multiple nodules in the liver. Somatostatin receptor scintigraphy showed an area of increased radiotracer uptake at the tail of the pancreas and multiple liver nodules corresponding to the previous CT scan. Serum glucagon was 923 pg/mL, confirming the diagnosis for glucagonoma. The patient was treated with distal pancreatectomy, and enucleation of liver metastases. Histopathological reported grade 2 well-differentiated NET. During the admission, the patient was found to have a parathyroid level of 79.3 pg/mL and increased uptake in the left and right lower regions of the thyroid gland from parathyroid MIBI scan, indicating hyperfunctioning parathyroid glands. All pituitary hormones were within normal ranges and no pituitary tumor was detected by the MRI brain. BMD showed osteoporosis at the lumbar spine and left femoral neck. The patient was referred to general surgery for subtotal parathyroidectomy. Pathological of resected parathyroid glands reported parathyroid hyperplasia. Postoperative PTH level and calcium were returned to normal range. Genetic testing focused on MEN1 gene, and the mutation was identified. After four months follow up plasma glucagon decreased to 425 pg/dL, patient had complete resolution of the cutaneous lesions. Conclusion: Glucagonomas are a rare pancreatic tumors and often difficult to recognize. Chronic eczema may misdiagnose for NME and delay diagnosis. NME can be challenging for physicians to recognize NME which is an important key to diagnose glucagonoma. Even though MEN1 association with glucagonoma is infrequent, awareness of such is important to allow appropriate testing for MEN1 in patients with glucagonoma.

scholarly journals Severe bone disease caused by primary hyperparathyroidism: a case report and review of the literature

2020 ◽  
Vol 48 (10) ◽  
pp. 030006052096648
Author(s):  
Yu Wang ◽  
Jie Liu
Keyword(s):  
Case Report ◽  
Primary Hyperparathyroidism ◽  
Bone Disease ◽  
Distal Humerus ◽  
Left Lobe ◽  
Pathological Examination ◽  
Hypoechoic Mass ◽  
Brown Tumors ◽  
Restricted Mobility ◽  
The Right

Bone disease is an important complication of hyperparathyroidism. We herein report a rare case of severe bone disease caused by primary hyperparathyroidism. A 33-year-old man presented with pain and restricted mobility in his right upper limb and right hip due to a fall 3 days previously. X-ray examination showed a fracture of the proximal and distal humerus. Computed tomography examination showed a supracondylar fracture of the right humerus, a fracture of the right femoral neck, a fracture of the right sciatic branch, and multiple brown tumors. Ultrasonography showed a 3.5- × 1.6-cm hypoechoic mass below the left lobe of the thyroid. The patient was diagnosed with primary hyperparathyroidism based on increased serum calcium and parathormone concentrations, pathological fractures, and multiple brown tumors. He therefore underwent bilateral lower parathyroidectomy. Pathological examination revealed a parathyroid adenoma. The patient recovered well after surgery and was followed up for 6 months with no symptoms of hyperparathyroidism. This case report suggests that clinicians should be aware of the possibility of severe bone disease secondary to primary hyperparathyroidism. Active and early diagnosis and surgical treatment are important in such cases.

Parathyroid glands in primary hyperparathyroidism: An ultrastructural study of 50 cases

1986 ◽  
Vol 17 (10) ◽  
pp. 1036-1046 ◽  
Author(s):  
Saverio Cinti ◽  
Giacomo Colussi ◽  
Ernesto Minola ◽  
G. Richard Dickersin
Keyword(s):  
Primary Hyperparathyroidism ◽  
Ultrastructural Study ◽  
Parathyroid Glands

scholarly journals Advances in the diagnosis and the management of primary hyperparathyroidism

2021 ◽  
Vol 12 ◽  
pp. 204062232110159
Author(s):  
Ana Kashfia Islam
Keyword(s):  
Renal Function ◽  
Parathyroid Hormone ◽  
Bone Loss ◽  
Primary Hyperparathyroidism ◽  
Kidney Stones ◽  
Parathyroid Glands ◽  
Definitive Treatment ◽  
Indications For Surgery ◽  
Musculoskeletal Complaints ◽  
Parathyroid Cancer

The parathyroid glands, one of the last organs to be discovered, are responsible for maintaining calcium homeostasis, and they continue to present the clinician with diagnostic and management challenges that are reviewed herein. Primary hyperparathyroidism (PHPT) comprises the vast majority of pathology of the parathyroid glands. The classic variant, presenting with elevated calcium and parathyroid hormone levels, has been studied extensively, but the current body of literature has added to our understanding of normocalcemic and normohormonal variants of PHPT, as well as syndromic forms of PHPT. All variants can lead to bone loss, kidney stones, declining renal function, and a variety of neurocognitive, gastrointestinal, and musculoskeletal complaints, although the majority of PHPT today is asymptomatic. Surgery remains the definitive treatment for PHPT, and advances in screening, evolving indications for surgery, new imaging modalities, and improvements in intra-operative methods have greatly changed the landscape. Surgery continues to produce excellent results in the hands of an experienced parathyroid surgeon. For those patients who are not candidates for surgery, therapeutic advances in medical management allow for improved control of the hypercalcemic state. Parathyroid cancer is extremely rare; the diagnosis is often made intra-operatively or on final pathology, and recurrence is common. The mainstay of treatment is normalization of serum calcium via surgery and medical adjuncts.

S178 – Utility of CT-Sestamibi Fusion in Parathyroid Exploration

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P135-P136
Author(s):  
Michael J Clark ◽  
Phillip Pellitteri
Keyword(s):  
Primary Hyperparathyroidism ◽  
Minimally Invasive ◽  
Image Fusion ◽  
Fusion Imaging ◽  
Parathyroid Glands ◽  
Preoperative Localization ◽  
Minimally Invasive Surgical ◽  
Sestamibi Imaging ◽  
Invasive Surgical Approach ◽  
Operative Findings

Objectives 1) Delineate the role of CT-technetium 99m sestamibi (CT-MIBI) fusion in directed parathyroidectomy. 2) Determine the clinical situations where CT-MIBI fusion would be strongly recommended. Methods Charts from 190 patients with primary hyperparathyroidism who underwent CT-MIBI mage fusion as a part of a scan directed, minimally invasive parathyroid exploration protocol were reviewed. The results of conventional sestamibi imaging and CT-MIBI image fusion were compared with operative findings. Results CT-MIBI image fusion accurately localized solitary hyperfunctional parathyroid glands in 70% of patients imaged; 55% of patients were localized with conventional sestamibi imaging. CT-MIBI fusion imaging was most accurate and predictive when conventional images suggested that the solitary gland was separated from the thyroid or when the adenoma was located in the retro-thyroidal/ retro-esophageal plane or mediastinum. Conclusions CT-MIBI image fusion is not superior to conventional sestamibi imaging when utilized for routine localization of hyperfunctional parathyroid glands. CT-MIBI fusion is of greatest benefit in guiding the directed approach to solitary glands, which are separate from the thyroid or ectopically located, regions where conventional imaging has proven to be less accurate. This imaging technique will augment the minimally invasive surgical approach in selected patients with primary hyperparathyroidism in order to further refine the focused technique. Its utility as the standard preoperative localization modality is not yet established and requires further investigation. Evaluation of differences in facility utilization with CT-MIBI image fusion and conventional sestamibi imaging may be helpful in determining its role in preoperative localization for hyperparathyroidism.

scholarly journals Imaging of the parathyroid glands in primary hyperparathyroidism

2016 ◽  
Vol 174 (1) ◽  
pp. D1-D8 ◽  
Author(s):  
Salvatore Minisola ◽  
Cristiana Cipriani ◽  
Daniele Diacinti ◽  
Francesco Tartaglia ◽  
Alfredo Scillitani ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Minimally Invasive ◽  
Invasive Procedure ◽  
Parathyroid Glands ◽  
Definitive Treatment ◽  
Preoperative Imaging ◽  
Disease Etiology ◽  
Minimally Invasive Surgical ◽  
Asymptomatic Patients ◽  
Surgical Expertise

Primary hyperparathyroidism (PHPT) is one of the most frequent endocrine diseases worldwide. Surgery is the only potentially curable option for patients with this disorder, even though in asymptomatic patients 50 years of age or older without end organ complications, a conservative treatment may be a possible alternative. Bilateral neck exploration under general anaesthesia has been the standard for the definitive treatment. However, significant improvements in preoperative imaging, together with the implementation of rapid parathyroid hormone determination, have determined an increased implementation of focused, minimally invasive surgical approach. Surgeons prefer to have a localization study before an operation (both in the classical scenario and in the minimally invasive procedure). They are not satisfied by having been referred a patient with just a biochemical diagnosis of PHPT. Imaging studies must not be utilized to make the diagnosis of PHPT. They should be obtained to both assist in determining disease etiology and to guide operative procedures together with the nuclear medicine doctor and, most importantly, with the surgeon. On the contrary, apart from minimally invasive procedures in which localization procedures are an obligate choice, some surgeons believe that literature on parathyroidectomy over the past two decades reveals a bias towards localization. Therefore, surgical expertise is more important than the search for abnormal parathyroid glands.

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