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Endocrine Journal Jun 2022Water-clear cell parathyroid adenoma is an uncommon cause of primary hyperparathyroidism. Herein, we report an interesting case of a 56-year-old man who presented with... (Review)
Review
Water-clear cell parathyroid adenoma is an uncommon cause of primary hyperparathyroidism. Herein, we report an interesting case of a 56-year-old man who presented with weight loss, bone pain, fatigue, and a palpable right neck mass. Laboratory tests indicated hypercalcemia, elevated parathyroid hormone (PTH) levels, and normal thyroid function. Further examinations detected osteoporosis and kidney stones. The ultrasound of neck revealed bilateral extrathyroidal tumors, which were sestamibi-avid. The patient underwent resection of the large right inferior and left inferior parathyroid tumors. Histopathology revealed a double water-clear cell parathyroid adenoma. His serum calcium and PTH levels normalized after surgery. The literature review identified 37 cases of water-clear cell parathyroid adenoma between 1985 and 2021. The median age at diagnosis was 56 years. Classic complications were common, including nephrolithiasis in nine and skeletal presentations in 10 patients. Before surgery, the median calcium and PTH levels were 12.0 mg/dL and 290 pg/mL, respectively. Overall, 89% were localized on ultrasonography, and 60% were positive on scintigraphy. Four patients had double adenomas. The median maximum diameter was 3.8 cm, and the median weight of the resected adenoma was 5.27 g. In summary, water-clear cell parathyroid adenoma has certain unique features. These include larger tumor size, relatively indolent biochemical profile, high prevalence of complications and nonspecific symptoms, an isoechoic appearance on ultrasonography, and reduced scintigraphic sensitivity.
Topics: Adenoma; Calcium; Humans; Male; Middle Aged; Parathyroid Glands; Parathyroid Hormone; Parathyroid Neoplasms; Water
PubMed: 34987145
DOI: 10.1507/endocrj.EJ21-0590 -
EBioMedicine Apr 2024To date, because of the difficulty in obtaining normal parathyroid gland samples in human or in animal models, our understanding of this last-discovered organ remains...
BACKGROUND
To date, because of the difficulty in obtaining normal parathyroid gland samples in human or in animal models, our understanding of this last-discovered organ remains limited.
METHODS
In the present study, we performed a single-cell transcriptome analysis of six normal parathyroid and eight parathyroid adenoma samples using 10 × Genomics platform.
FINDINGS
We have provided a detailed expression atlas of parathyroid endocrine cells. Interestingly, we found an exceptional high expression levels of CD4 and CD226 in parathyroid endocrine cells, which were even higher than those in lymphocytes. This unusual expression of lymphocyte markers in parathyroid endocrine cells was associated with the depletion of CD4 T cells in normal parathyroid glands. Moreover, CD4 and CD226 expression in endocrine cells was significantly decreased in parathyroid adenomas, which was associated with a significant increase in Treg counts. Finally, along the developmental trajectory, we discovered the loss of POMC, ART5, and CES1 expression as the earliest signature of parathyroid hyperplasia.
INTERPRETATION
We propose that the loss of CD4 and CD226 expression in parathyroid endocrine cells, coupled with an elevated number of Treg cells, could be linked to the pathogenesis of parathyroid adenoma. Our data also offer valuable information for understanding the noncanonical function of CD4 molecule.
FUNDING
This work was supported by the National Key R&D Program of China (2022YFA0806100), National Natural Science Foundation of China (82130025, 82270922, 31970636, 32211530422), Shandong Provincial Natural Science Foundation of China (ZR2020ZD14), Innovation Team of Jinan (2021GXRC048) and the Outstanding University Driven by Talents Program and Academic Promotion Program of Shandong First Medical University (2019LJ007).
Topics: Humans; Parathyroid Glands; Parathyroid Neoplasms; Down-Regulation; Carcinogenesis; Cell Transformation, Neoplastic; Hyperplasia; Lymphocytes
PubMed: 38471398
DOI: 10.1016/j.ebiom.2024.105053 -
Medicine Nov 2022Most patients with parathyroid adenomas are asymptomatic and rarely present with chronic pancreatitis (CP). Several studies have reported a positive association between... (Review)
Review
BACKGROUND
Most patients with parathyroid adenomas are asymptomatic and rarely present with chronic pancreatitis (CP). Several studies have reported a positive association between primary hyperparathyroidism (PHPT) and pancreatitis. Parathyroidectomy is the definitive treatment for PHPT. IV bisphosphonates can be considered the drug of choice for bridge to surgery.
METHODS
We reported a 57-year-old female patient was admitted to the emergency room with left upper quadrant abdominal pain and a diagnosis of recurrent pancreatitis. Magnetic Resonance Cholangiopancreatography confirmed the diagnosis of CP. The patient had no common etiology of pancreatitis. Persistent hypercalcemia was noted despite administering intravenous fluids, and Calcitonin. Intravenous Pamidronate, a Bisphosphonate derivative, was also administered. Although calcium levels initially decreased, they were later found to rebound to previous levels.
RESULTS
A diagnosis of parathyroid adenoma and PHPT was made based on the elevated parathyroid hormone levels and cervical ultrasonography indicated right inferior parathyroid adenoma. Technetium-99m methoxy-isobutyl-isonitrile scintigraphy revealed a focal hot spot of tracer accumulation at the right lower thyroid bed. The patient underwent right lower parathyroidectomy smoothly and successfully. After right lower parathyroidectomy, she had normal serum calcium levels (9.2 mg/dL) and parathyroid hormone (16.1 pg/mL). There was no recurrent abdominal pain after the operation.
CONCLUSION
CP is a rare manifestation of parathyroid adenoma. When patients with a history of recurrent pancreatitis, without common causes of pancreatitis, present persistent elevated serum calcium levels, PHPT could be suspected.
Topics: Humans; Female; Middle Aged; Parathyroid Neoplasms; Calcium; Pancreatitis, Chronic; Hypercalcemia; Parathyroid Hormone; Abdominal Pain
PubMed: 36401406
DOI: 10.1097/MD.0000000000031750 -
Bulletin Du Cancer Mar 2006Parathyroid carcinoma is a rare disease accounting for 1 to 5% of parathyroid neoplasms. This malignant tumour must be suspected when a severe primary... (Review)
Review
Parathyroid carcinoma is a rare disease accounting for 1 to 5% of parathyroid neoplasms. This malignant tumour must be suspected when a severe primary hyperparathyroidism occurs with high hypercalcemia and elevated parathormon levels. At this time, a cervical mass is often palpable. Both head and neck ultrasonography and 99mTc-sestamibi scintigraphy are the best preoperative imaging tests to suspect and localize the tumour. Surgical approach with simultaneous tumorectomy and hemithyroidectomy, completed by selective neck dissection (level VI) is the treatment of choice. An elective lateral neck dissection should be performed if necessary. Tumour control should be monitored by regular measurement of calcium and parathormon levels. Local recurrence or metastasis risk is 30 to 70% and the 5 year overall survival about 50 to 80%. In case of recurrence, aggressive surgical management should be applied and adjuvant radiation therapy may be discussed.
Topics: Humans; Hypercalcemia; Hyperparathyroidism; Parathyroid Neoplasms
PubMed: 16567315
DOI: No ID Found -
Endocrine Journal 2013A 46 year-old male presented with persistently high level of serum parathyroid hormone (PTH), despite successful resection of an oxyphilic cell parathyroid adenoma of... (Review)
Review
A 46 year-old male presented with persistently high level of serum parathyroid hormone (PTH), despite successful resection of an oxyphilic cell parathyroid adenoma of the left lower gland. Renal function and serum calcium were normal, leading to vitamin D deficiency being considered. Tc99m-sestamibi parathyroid scintigraphy showed no capitation, but a cervical ultrasound demonstrated an increase in the lower parathyroids. Surgery confirmed that the right gland was normal but the left corresponded to parathyroid carcinoma. The patient developed severe hypocalcemia, with PTH values being consistent with hypoparathyroidism for a few months. However, a progressive increase in calcium and PTH serum levels indicated recurrence of disease. Tc99m-sestamibi scintigraphy demonstrated hyperfixation in topography of the left inferior parathyroid and the patient was subjected to a third and more extensive surgery, with removal of lymph nodes and adjacent thyroid tissue. Serum calcium and PTH remained elevated, requiring loop diuretics and intravenous bisphosphonates to control hypercalcemia. Cervical radiotherapy was implemented as adjuvant therapy. After two months the patient complained of dyspnea, and a CT scan of the chest demonstrated areas of parenchymal condensation, suggestive of actinic pneumonitis. At the 2-year follow-up no major issues were evident.
Topics: Adenoma, Oxyphilic; Brazil; Carcinoma; Delayed Diagnosis; Diagnostic Errors; Humans; Hyperparathyroidism, Primary; Hypocalcemia; Male; Middle Aged; Neoplasms, Multiple Primary; Parathyroid Neoplasms; Radiotherapy, Adjuvant; Recurrence; Treatment Outcome
PubMed: 23268928
DOI: No ID Found -
International Journal of Surgery... Jul 2016Multiple endocrine neoplasia type 1 (MEN1) is a genetic disorder characterized by usually benign tumors of the parathyroid glands, pancreatic islet cells, and anterior... (Review)
Review
BACKGROUND
Multiple endocrine neoplasia type 1 (MEN1) is a genetic disorder characterized by usually benign tumors of the parathyroid glands, pancreatic islet cells, and anterior pituitary. Hyperparathyroidism (HPT) occurs in 90% of MEN1 patients. In rare cases, it is associated with parathyroid carcinoma (PC) or atypical parathyroid neoplasm (APN). We present a cohort of 3 such patients.
METHODS
We performed a retrospective review of our institution's MEN1 database to identify patients who underwent operations for HPT and had a histopathologic diagnosis of PC or APN. Clinical features, genetics, and outcomes were summarized.
RESULTS
Of 291 MEN1 patients, 242 had HPT (83.2%). Two of the 242 patients (0.8%) had a histopathologic diagnosis of PC, and 1 (0.4%) had a diagnosis of APN. The patients with PC were male, ages 62 and 56 years at the time of surgery; the patient with APN was female, age 32 years. All patients also had a pancreatic endocrine tumor. The observed genetic mutations in the PC patients were c.703G > A (p.E235K) in exon 4 and c.1378C > T (p.R460X) in exon 10. All 3 patients had recurrence of hypercalcemia, and 2 patients underwent reoperation; pathologic analysis revealed the presence of a hyperplastic gland, not tumor recurrence. No cases had distant metastasis.
CONCLUSIONS
This is the first report of APN in an MEN1 patient. Although rare, the presence of PC or APN in MEN1 is noteworthy because it affects the management if hypercalcemia recurs, possibly requiring an open approach rather than the minimally invasive techniques used in the reoperative setting for benign disease.
Topics: Adult; Female; Humans; Hyperparathyroidism; Male; Middle Aged; Multiple Endocrine Neoplasia Type 1; Mutation; Parathyroid Glands; Parathyroid Neoplasms; Retrospective Studies
PubMed: 27212590
DOI: 10.1016/j.ijsu.2016.05.035 -
European Annals of Otorhinolaryngology,... Nov 2019Primary hyperparathyroidism (PHPT) during pregnancy can be responsible for serious maternal and foetal complications and should be treated by elective low-risk,...
INTRODUCTION
Primary hyperparathyroidism (PHPT) during pregnancy can be responsible for serious maternal and foetal complications and should be treated by elective low-risk, minimally invasive surgery. Preoperative assessment of hyperfunctioning parathyroid glands is classically based on high-resolution neck ultrasound (US) and Tc-sestamibi/I scintigraphy. However, administration of any radiopharmaceutical during pregnancy must be limited and justified and US alone may be sufficient to localize an abnormal parathyroid gland.
CASE REPORT
We report the case of a 4-month pregnant woman with severe primary hyperparathyroidism, in whom US failed to localize the abnormal parathyroid gland. Tc-sestamibi scintigraphy was performed in preference to -fluorocholine (FCH)-PET/CT on the basis of a multidisciplinary decision. As Tc-MIBI demonstrated an hyperfunctioning right inferior parathyroid, I was not administered. A large right paravertebral parathyroid adenoma was successfully removed, as confirmed by decreased postoperative serum parathyroid hormone and calcium levels. The eutrophic newborn infant was delivered at term, with normal serum calcium and TSH levels.
DISCUSSION
In pregnant women with primary hyperparathyroidism, US alone may be sufficient when it localizes the abnormal parathyroid gland, allowing elective low-risk minimally invasive surgery. Otherwise, a multidisciplinary approach is mandatory to select the radiopharmaceutical that can be safely used to identify the hyperfunctioning parathyroid gland with minimal risks for the foetus.
Topics: Adenoma; Adult; Female; Humans; Hyperparathyroidism; Parathyroid Glands; Parathyroid Neoplasms; Pregnancy; Radionuclide Imaging; Technetium Tc 99m Sestamibi
PubMed: 31378705
DOI: 10.1016/j.anorl.2019.05.009 -
BMC Endocrine Disorders May 2023Parathyroid carcinoma is a rare disease with a frequency of 0.005% of all malignancies [1, 2]. Various aspects of its pathogenesis, diagnosis, and treatment remain...
BACKGROUND
Parathyroid carcinoma is a rare disease with a frequency of 0.005% of all malignancies [1, 2]. Various aspects of its pathogenesis, diagnosis, and treatment remain poorly understood. Furthermore, cases with secondary hyperparathyroidism are fewer. In this case report, we describe a case of left parathyroid carcinoma with secondary hyperparathyroidism.
CASE PRESENTATION
The patient was a 54-year-old woman who had been on hemodialysis since the age of 40 years. At 53 years of age, her calcium levels were high, and she was diagnosed with drug-resistant secondary hyperparathyroidism and was referred to our hospital for surgical treatment. Blood tests revealed calcium levels of 11.4 mg/dL and intact parathyroid hormone (PTH) levels of 1007 pg/mL. Neck ultrasonography revealed a 22-mm large round hypoechoic mass, partially indistinct margins, and D/W ratio > 1 at the left thyroid lobe. Computed tomography scans revealed a 20-mm nodule at the left thyroid lobe. No enlarged lymph nodes or distant metastases were noted. Tc-hexakis-2-methoxyisobutylisonitrile scintigraphy revealed an accumulation at the superior pole of the left thyroid lobe. Laryngeal endoscopy revealed paralysis of the left vocal cord, signifying recurrent nerve palsy due to parathyroid carcinoma. Based on these results, a diagnosis of secondary hyperparathyroidism and suspected left parathyroid carcinoma was made, and the patient underwent surgery. Pathology results revealed hyperplasia in the right upper and lower parathyroid glands. The left upper parathyroid gland showed capsular and venous invasion, and the diagnosis was left parathyroid carcinoma. At 4 months post-surgery, calcium levels improved to 8.7 mg/dL and intact PTH levels to 20 pg/mL, with no signs of recurrence.
CONCLUSIONS
We report a case of left parathyroid carcinoma associated with secondary hyperparathyroidism. Concomitant secondary hyperparathyroidism may cause mild hypercalcemia compared to parathyroid carcinoma alone due to the added modification of dialysis. Although our patient also presented with mild hypercalcemia, a D/W ratio > 1 on preoperative echocardiography and presence of recurrent nerve palsy on laryngoscopy led to the suspicion and treatment of parathyroid carcinoma preoperatively.
Topics: Humans; Female; Adult; Middle Aged; Parathyroid Neoplasms; Hypercalcemia; Calcium; Hyperparathyroidism, Secondary; Paralysis; Parathyroid Hormone
PubMed: 37193977
DOI: 10.1186/s12902-023-01370-x -
BMC Endocrine Disorders Dec 2022The hallmark of hyperparathyroidism is hypersecretion of parathyroid hormone (PTH) which results in hypercalcemia and hypophosphatemia. While hypercalcemia due to...
BACKGROUND
The hallmark of hyperparathyroidism is hypersecretion of parathyroid hormone (PTH) which results in hypercalcemia and hypophosphatemia. While hypercalcemia due to malignancy is often brought about by PTH-related protein in adults, PTH-producing tumors are quite rare in clinical practice. Additionally, from the point of embryology, it is very difficult to examine ectopic PTH-producing tissue such as ectopic parathyroid glands. Furthermore, clear histopathological criteria are not present.
CASE PRESENTATION
A 57-year-old woman was referred to our hospital for hypercalcemia. Her parathyroid hormone (PTH) level was elevated, but there were no enlarged parathyroid glands. Although 99mTc-MIBI confirmed a localized and slightly hyperfunctioning parathyroid tissue in the anterior mediastinum, it was not typical as hyperfunctioning parathyroid. We finally diagnosed her as ectopic PTH-producing cyst-like tumor with venous sampling of PTH. She underwent anterosuperior mediastinal ectopic PTH-producing cyst-like tumor resection. It is noted that intact-PTH concentration of the fluid in the cyst was very high (19,960,000 pg/mL). Based on histopathological findings, we finally diagnosed her as ectopic PTH-producing parathyroid cyst inside the thymus. After resection of anterosuperior mediastinal thymus including ectopic PTH-producing parathyroid cyst, calcium and intact-PTH levels were decreased, and this patient was discharged without any sequelae.
CONCLUSIONS
We should know the possibility of superior mediastinal ectopic PTH-producing parathyroid cyst inside the thymus among subjects with ectopic PTH-producing parathyroid glands. Particularly when the cyst is present in the superior mediastinum, it is necessary to do careful diagnosis based on not only positive but also negative findings in 99mTc-MIBI. It is noted that the patient's bloody fluid in the cyst contained 19,960,000 pg/mL of intact-PTH, and its overflow into blood stream resulted in hyperparathyroidism and hypercalcemia. Moreover, in such cases, the diagnosis is usually confirmed after through histological examination of ectopic PTH-producing parathyroid glands. We think that it is very meaningful to let clinicians know this case.
Topics: Humans; Adult; Female; Middle Aged; Parathyroid Glands; Parathyroid Hormone; Hypercalcemia; Hormones, Ectopic; Parathyroid Neoplasms; Hyperparathyroidism, Primary; Cysts
PubMed: 36544116
DOI: 10.1186/s12902-022-01256-4 -
Medicine Nov 2022Secondary parathyroid hyperplasia canceration is very rare and thus easily be overlooked during parathyroid ultrasound examination. However, secondary parathyroid...
INTRODUCTION
Secondary parathyroid hyperplasia canceration is very rare and thus easily be overlooked during parathyroid ultrasound examination. However, secondary parathyroid hyperplasia still has the possibility of canceration, and it is still important to be alert to its occurrence when performing ultrasound examinations and clinical treatment.
PATIENT CONCERNS
A 49-years-old man visited our outpatient department with generalized weakness and pain in both lower extremities a month ago.
DIAGNOSIS
Hyperparathyroidism secondary to chronic renal failure.
INTERVENTIONS
The patient underwent ultrasound and other preoperative examinations. The preoperative ultrasound showed 3 parathyroid enlargements, 2 on the left and 1 on the right. The patient then underwent surgical treatment.
OUTCOMES
Ultrasonography suggested the presence of 3 parathyroid hyperplasias, and ectopic right inferior parathyroid gland was visible during intraoperative examination. 10 days after surgery, the patient's Parathyroid Hormone returned to the normal range.
CONCLUSION
Secondary parathyroid hyperplasia has the potential to become cancerous, so doctors should be alert to its occurrence when conducting ultrasound examinations. Ultrasound examination is the key to its diagnosis and subsequent treatment.
Topics: Humans; Male; Middle Aged; Hyperplasia; Parathyroid Neoplasms; Parathyroid Glands; Hyperparathyroidism, Primary; Carcinoma
PubMed: 36401397
DOI: 10.1097/MD.0000000000031362