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The Journal of Clinical Endocrinology... Dec 2023Parathyroid carcinoma (PC) is usually associated with severe symptomatic primary hyperparathyroidism (PHPT) and accounts for less than 1% of all cases of PHPT and...
Parathyroid carcinoma (PC) is usually associated with severe symptomatic primary hyperparathyroidism (PHPT) and accounts for less than 1% of all cases of PHPT and approximately 0.005% of all cancers. PC most commonly occurs as a sporadic disease and somatic CDC73 mutations can be detected in up to 80% of cases. Approximately 30% of patients harbor a germline mutation of the CDC73 gene. Preoperative diagnosis of PC is difficult because no disease-specific markers are available, and PC should be suspected in patients with severe hypercalcemia and end-organ complications. The diagnosis is based on the evidence of invasive tumor growth at histology and/or metastases. En bloc resection of the tumor, together with the ipsilateral thyroid lobe and adjacent structures, should be performed by an experienced surgeon when PC is suspected. This surgical approach reduces the risk of recurrence and metastasis and offers the highest chance of cure. Nonetheless, PC has a recurrence rate of 40% to 60% and, if feasible, multiple surgical procedures should be performed. When surgery is no longer an option, medical treatment is aimed to reduce hypercalcemia and target organ complications. Targeted agents have been effectively used in a few cases. We describe herein a patient with severe PHPT due to PC and provide a systematic diagnostic and treatment approach. A thorough review of the medical history, a typical clinical and biochemical phenotype and, in some cases, the revision of the histological examination provide the clues for the diagnosis of PC.
Topics: Humans; Parathyroid Neoplasms; Hypercalcemia; Germ-Line Mutation; Thyroid Gland
PubMed: 37531615
DOI: 10.1210/clinem/dgad455 -
Frontiers in Endocrinology 2023
Topics: Humans; Thyroid Neoplasms; Endocrinology
PubMed: 37484953
DOI: 10.3389/fendo.2023.1236887 -
Romanian Journal of Internal Medicine =... Dec 2023The cortical bone is the most severely affected site in patients with primary hyperparathyroidism (PHPT) and thus, a low bone mineral density (BMD) is predominantly...
INTRODUCTION
The cortical bone is the most severely affected site in patients with primary hyperparathyroidism (PHPT) and thus, a low bone mineral density (BMD) is predominantly observed in distal forearm. Several studies have investigated potential associations between the weight of the gland and bone mineral loss. In this study, we wanted to investigate the relationship between parathyroid adenoma (PTA) volume and bone mineral loss.
METHODS
All patients with a diagnosis of PHPT who were operated at our hospital, and with a histologically proven single PTA were retrospectively analyzed. Z-scores were used as the main variable in our analysis to eliminate the effects of age, sex and gonadal status on BMD.
RESULTS
Total of 153 patients who met the inclusion criteria were eligible for the study. A significant negative correlation between the PTA volume and z-score for distal third of the radius (DR) ( = 0.006, r = -0.297) was shown. The cut-off value of gland volume for predicting cortical bone mineral loss was 9043.2 mm. There was also a significant negative correlation between the 24-hour urine calcium and z-scores for lumbar vertebrae and total hip. A significant negative correlation was found between preoperative 25-hydroxy vitamin D levels and the PTA weight.
CONCLUSIONS
As the first study that evaluated any possible association between the volume of a parathyroid adenoma and bone mineral loss in patients with PHPT, we found a significant negative correlation between DR z-scores and resected gland volume. Since the volume of a PTA can also be determined by a preoperative US, our findings may be helpful during the preoperative evaluation of a patient with a preliminary diagnosis of PHPT.
Topics: Humans; Bone Density; Forearm; Parathyroid Neoplasms; Retrospective Studies; Bone Diseases, Metabolic; Minerals; Absorptiometry, Photon
PubMed: 37493634
DOI: 10.2478/rjim-2023-0018 -
Annals of Surgical Treatment and... Aug 2023Primary hyperparathyroidism (PHPT) is caused by typical adenoma (TA), multiglandular disease (MD), or parathyroid carcinoma (PC), and in a smaller percentage of cases by...
PURPOSE
Primary hyperparathyroidism (PHPT) is caused by typical adenoma (TA), multiglandular disease (MD), or parathyroid carcinoma (PC), and in a smaller percentage of cases by atypical parathyroid tumor (APT). The objective of this study is the retrospective analysis of clinical features and parathyroid hormone (PTH)/calcium response to surgery in patients who underwent parathyroidectomy for symptomatic PHPT with histological evidence of APT.
METHODS
We retrospectively reviewed our institutional experience in the management of PHPT from January 2016 to December 2021 focusing on those patients presenting APTs. We analyzed the clinical features of this disease and PTH/calcium response to surgical treatment in APTs compared to the other pathological conditions causing PHPT.
RESULTS
In a cohort of 125 patients with PHPT we found 112 TAs (89.6%), 6 APTs (4.8%), 6 PCs (4.8%), and only 1 MD (0.8%). APTs in comparison to other parathyroid diseases showed peculiar features such as adhesion to the surrounding structures and a frequent intrathyroidal location, which may justify thyroid loboistmectomy adopted in most of the observed cases. APTs showed significantly higher preoperative PTH values compared to TA + MD and were relevant to PC.
CONCLUSION
Due to its rarity, there is a lack of specific indications in the management of APTs. Biochemical features observed in APT and PC can be related to similar biological behavior. However, some specific features observed preoperatively in some cases of PHPT might suggest presence of an APT, which could be helpful mostly in surgical and postoperative management. Further studies are required to confirm the results of the present preliminary report.
PubMed: 37564944
DOI: 10.4174/astr.2023.105.2.76 -
Medicine Dec 2023To retrospectively analyze the diagnosis and treatment of secondary hyperparathyroidism (SHPT) combined with thyroid disease, and to investigate the correlation between...
To retrospectively analyze the diagnosis and treatment of secondary hyperparathyroidism (SHPT) combined with thyroid disease, and to investigate the correlation between SHPT and papillary thyroid carcinoma (PTC), SHPT and thyroid disease, and the importance of preoperative localization diagnosis in patients with SHPT. Clinical data of 101 patients who underwent surgical treatment for SHPT at the Third Hospital of Hebei Medical University were collected from August 2014 to May 2023, and patients were divided into SHPT without PTC group (n = 94) and SHPT with PTC group (n = 7) according to their postoperative pathology. Patients were divided into SHPT without thyroid disease group (n = 32) and SHPT with thyroid disease group (n = 69) according to their preoperative ultrasound diagnosis and postoperative pathology. The differences between the 2 groups were compared to explore the association between SHPT and PTC and between SHPT and thyroid disease. Of the 101 patients with SHPT, 65 were male and 36 were female with a mean age of (44.26 ± 11.16) years. There were 69 patients (68.32%) with concomitant thyroid disease and 32 patients (31.68%) without concomitant thyroid disease, including 7 patients (6.93%) with PTC. The results of univariate analysis showed that the differences in age and preoperative PTH levels between the SHPT without PTC group and the SHPT with PTC group were statistically significant (P < 0. 05),There were no significant differences in age, gender, preoperative PTH, preoperative alkaline phosphatase, preoperative serum calcium, preoperative serum phosphorus, preoperative serum creatinine, duration of dialysis disease, and whether they were accompanied by hypertension or not between the SHPT without thyroid disease group and the SHPT with thyroid disease group (P > 0. 05), logistic regression analysis showed that there was a correlation between the age of patients with SHPT and the level of preoperative PTH with PTC. In patients with SHPT, concomitant thyroid disease is more common, so patients with SHPT should be screened for thyroid disease at the same time as routine preoperative ultrasonography combined with nuclear scan for localized diagnosis, and surgical resection is preferred if concomitant PTC is present.
Topics: Humans; Male; Female; Adult; Middle Aged; Retrospective Studies; Renal Dialysis; Hyperparathyroidism, Secondary; Thyroid Cancer, Papillary; Thyroid Neoplasms; Parathyroid Hormone
PubMed: 38115353
DOI: 10.1097/MD.0000000000036514 -
World Journal of Clinical Cases Sep 2023Parathyroid carcinoma (PC) is a rare, slow-growing malignant tumor and a rare cause of primary hyperfunctioning of the parathyroid, with a highly variable clinical...
BACKGROUND
Parathyroid carcinoma (PC) is a rare, slow-growing malignant tumor and a rare cause of primary hyperfunctioning of the parathyroid, with a highly variable clinical course, depending on the aggressiveness of the individual tumor and the degree of hypercalcemia.
CASE SUMMARY
The aim of this report is to summarize the diagnosis and treatment of three cases of PC and to review and conclude aspects regarding the three collected cases with reference to other relevant cases to explore the value of ultrasound in the diagnosis of PC. All three patients had hypercalcemia, consisting of a high serum calcium level and a high level of parathyroid hormone that was > 2-fold (even > 30-fold) of the normal upper limit. The ultrasonographic findings of the parathyroid gland showed that the glands were all > 30 mm, and the internal echo was uneven. All patients underwent surgery. PC in three cases was confirmed by routine histopathology and immunohistochemistry.
CONCLUSION
As clinical signs and laboratory results are nonspecific, it is difficult to diagnose PC preoperatively, so imaging examinations are often needed.
PubMed: 37727485
DOI: 10.12998/wjcc.v11.i25.5934 -
Endocrine Practice : Official Journal... Jun 2024In contrast to adults, primary hyperparathyroidism (PHPT) in children and adolescents is a rare endocrine disorder.
OBJECTIVE
In contrast to adults, primary hyperparathyroidism (PHPT) in children and adolescents is a rare endocrine disorder.
METHODS
A retrospective review of PHPT cases between 2005 and 2022 from a single tertiary university medical center, including clinical signs and symptoms, laboratory findings, radiological evaluation, treatment, and postoperative complications.
RESULTS
Ten children (mean age at diagnosis 16.3 ± 1.3 years) were diagnosed with PHPT. All patients were in late pubertal stages without sex predominance and 8 were symptomatic. Mean calcium level was 13.6 ± 2.5 mg/dL, and mean parathyroid hormone levels were 204.8 ± 163.1 pg/mL. Parathyroid adenoma was confirmed by the postsurgical pathology results.
CONCLUSIONS
PHPT in children and adolescents is often symptomatic and more severe than adults. The main cause is single parathyroid adenoma. Associated hypercalcemic syndromes were not found. Patients were cured after surgical removal of the adenoma without significant postoperative complications and no recurrence during 10.4 ± 5.9 years follow-up.
Topics: Humans; Adolescent; Parathyroid Neoplasms; Female; Male; Hyperparathyroidism, Primary; Retrospective Studies; Adenoma; Child; Parathyroid Hormone
PubMed: 38556080
DOI: 10.1016/j.eprac.2024.03.390 -
Journal of Experimental & Clinical... Nov 2023Liver cancer stem cells (LCSCs) play an important role in hepatocellular carcinoma (HCC), but the mechanisms that link LCSCs to HCC metastasis remain largely unknown....
BACKGROUND
Liver cancer stem cells (LCSCs) play an important role in hepatocellular carcinoma (HCC), but the mechanisms that link LCSCs to HCC metastasis remain largely unknown. This study aims to reveal the contributions of NRCAM to LCSC function and HCC metastasis, and further explore its mechanism in detail.
METHODS
117 HCC and 29 non-HCC patients with focal liver lesions were collected and analyzed to assess the association between NRCAM and HCC metastasis. Single-cell RNA sequencing (scRNA-seq) was used to explore the biological characteristics of cells with high NRCAM expression in metastatic HCC. The role and mechanism of NRCAM in LCSC dissemination and metastasis was explored in vitro and in vivo using MYC-driven LCSC organoids from murine liver cells.
RESULTS
Serum NRCAM is associated with HCC metastasis and poor prognosis. A scRNA-seq analysis identified that NRCAM was highly expressed in LCSCs with MYC activation in metastatic HCC. Moreover, NRCAM facilitated LCSC migration and invasion, which was confirmed in MYC-driven LCSC organoids. The in vivo tumor allografts demonstrated that NRCAM mediated intra-hepatic/lung HCC metastasis by enhancing the ability of LCSCs to escape from tumors into the bloodstream. Nrcam expression inhibition in LCSCs blocked HCC metastasis. Mechanistically, NRCAM activated epithelial-mesenchymal transition (EMT) and metastasis-related matrix metalloproteinases (MMPs) through the MACF1 mediated β-catenin signaling pathway in LCSCs.
CONCLUSIONS
LCSCs typified by high NRCAM expression have a strong ability to invade and migrate, which is an important factor leading to HCC metastasis.
Topics: Humans; Animals; Mice; Carcinoma, Hepatocellular; Liver Neoplasms; Neoplastic Stem Cells; Signal Transduction; Lung Neoplasms; Cell Line, Tumor; Epithelial-Mesenchymal Transition; Cell Movement; Cell Adhesion Molecules
PubMed: 37993901
DOI: 10.1186/s13046-023-02893-w -
Otolaryngologic Clinics of North America Feb 2024
Topics: Humans; Thyroid Gland; Parathyroid Glands; Thyroidectomy; Thyroid Neoplasms
PubMed: 37640561
DOI: 10.1016/j.otc.2023.08.003