Search for Articles:
Title / Keyword
Author / Affiliation / Email
Journal
Article Type
 
 
Advanced Search
 
Section
Special Issue
Volume
Issue
Number
Page
 
8.0
4.5
Journals
Cancers
Special Issues
Advances in Parathyroid Carcinoma: From Bench to Bedside
share announcement

Advances in Parathyroid Carcinoma: From Bench to Bedside

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Cancer Therapy".

Deadline for manuscript submissions: closed (10 November 2023) | Viewed by 9446

Special Issue Editors

Dr. Laura Giusti

E-Mail Website
Guest Editor
School of Pharmacy, University of Camerino, 62032 Camerino, Italy
Interests: proteomics and biomarkers; endocrine diseases; cancer; proteomics and nutraceuticals
Special Issues, Collections and Topics in MDPI journals
Prof. Dr. Filomena Cetani

E-Mail Website1 Website2
Guest Editor
Azienda Ospedaliero Universitaria Pisana, Pisa, Italy
Interests: pathogenesis of parathyroid tumors; primary hyperparathyroidism

Special Issue Information

Dear Colleagues,

Parathyroid carcinoma (PC) is a rare endocrine malignancy. It accounts for <1% of cases of sporadic primary hyperparathyroidism (PHPT) and up to 15% in the hereditary hyperparathyroidism–jaw tumor syndrome. Genomic alterations identified in PC are mostly represented by loss-of-function CDC73 gene mutations, leading to the loss of expression of its encoded protein termed parafibromin; nonetheless, whole exome sequencing studies have identified mutations in additional genes. On the sole basis of clinical and biochemical features, the diagnosis of PC can be difficult, and it is usually made postoperatively at histology. In some cases, the distinction between PC and atypical parathyroid adenoma (APA) can be challenging.

This Special Issue of Cancers aims to explore recent advances in PC, particularly its molecular pathogenesis, diagnosis, and management, but also expand the knowledge of the pathways involved in its pathogenesis and discover candidate biomarkers useful in pre-operative diagnosis, prognosis, and management. In this Special Issue, basic, translational, and clinical research studies and reviews are welcome. Research areas may include (but are not limited to) the following: omics approaches, translational research, surgery, medical therapy, and personalized medicine.

Dr. Laura Giusti
Prof. Dr. Filomena Cetani
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cancers is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2900 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • HPT-JT
  • primary hyperparathyroidism
  • parathyroidectomy
  • CDC73
  • HRPT2
  • parafibromin
  • en-bloc resection
  • cinacalcet
  • proteomics
  • biomarkers
  • nanostring
  • EMT
  • system biology

Published Papers (4 papers)

Download All Papers
Order results
Result details
Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:

Research

12 pages, 822 KiB  
Article
Novel PRUNE2 Germline Mutations in Aggressive and Benign Parathyroid Neoplasms
by Sara Storvall, Eeva Ryhänen, Auli Karhu and Camilla Schalin-Jäntti
Cancers 2023, 15(5), 1405; https://doi.org/10.3390/cancers15051405 - 23 Feb 2023
Cited by 1 | Viewed by 1561
Abstract
Parathyroid tumors are mostly sporadic but can also occur in familial forms, including different kinds of genetic syndromes with varying phenotypes and penetrance. Recently, somatic mutations of the tumor suppressor gene PRUNE2 were found to be frequent in parathyroid cancer (PC). The germline [...] Read more.
Parathyroid tumors are mostly sporadic but can also occur in familial forms, including different kinds of genetic syndromes with varying phenotypes and penetrance. Recently, somatic mutations of the tumor suppressor gene PRUNE2 were found to be frequent in parathyroid cancer (PC). The germline mutation status of PRUNE2 was investigated in a large cohort of patients with parathyroid tumors from the genetically homogenous Finnish population, 15 of which had PC, 16 atypical parathyroid tumors (APT), and 6 benign parathyroid adenomas (PA). Mutations in previously established hyperparathyroidism-related genes were screened with a targeted gene panel analysis. Nine PRUNE2 germline mutations with a minor allele frequency (MAF) of <0.05 were found in our cohort. Five of these were predicted to be potentially damaging and were identified in two patients with PC, two with APT, and three with PA. The mutational status was not associated with the tumor group nor related to the clinical picture or severity of the disease. Still, the frequent finding of rare germline mutations of PRUNE2 may point to the gene playing a role in the pathogenesis of parathyroid neoplasms. Full article
(This article belongs to the Special Issue Advances in Parathyroid Carcinoma: From Bench to Bedside)
►▼ Show Figures

Figure 1

18 pages, 816 KiB  
Article
Margin Free Resection Achieves Excellent Long Term Outcomes in Parathyroid Cancer
by Klaus-Martin Schulte, Nadia Talat and Gabriele Galatá
Cancers 2023, 15(1), 199; https://doi.org/10.3390/cancers15010199 - 29 Dec 2022
Cited by 3 | Viewed by 1859
Abstract
Long-term outcomes of parathyroid cancer remain poorly documented and unsatisfactory. This cohort includes 25 consecutive parathyroid cancer patients with median follow-up of 10.7 years (range 4.1–26.5 years). Pre-operative work-up in the center identified a suspicion of parathyroid cancer in 17 patients. En bloc [...] Read more.
Long-term outcomes of parathyroid cancer remain poorly documented and unsatisfactory. This cohort includes 25 consecutive parathyroid cancer patients with median follow-up of 10.7 years (range 4.1–26.5 years). Pre-operative work-up in the center identified a suspicion of parathyroid cancer in 17 patients. En bloc resection, including the recurrent laryngeal nerve in 4/17 (23.5%), achieved cancer-free resection margins (R0) in 82.4% and lasting loco-regional disease control in 94.1%. Including patients referred after initial surgery elsewhere, R0 resection was achieved in merely 17/25 (68.0%) of patients. Cancer-positive margins (R1) in 8 patients led to local recurrence in 50%. On multivariate analysis, only margin status prevailed as independent predictor of recurrence free survival (χ2 19.5, p < 0.001). Local excision alone carried a 3.5-fold higher risk of positive margins than en bloc resection (CI95: 1.1–11.3; p = 0.03), and a 6.4-fold higher risk of locoregional recurrence (CI95: 0.8–52.1; p = 0.08). R1-status was associated with an 18.0-fold higher risk of recurrence and redo surgery (CI95: 1.1–299.0; p = 0.04), and a 22.0-fold higher probability of radiation (CI95: 1.4–355.5; p = 0.03). In patients at risk, adjuvant radiation reduced the actuarial risk of locoregional recurrence (p = 0.05). When pre-operative scrutiny resulted in upfront oncological surgery achieving cancer free margins, it afforded 100% recurrence free survival at 5- and 10-year follow-up, whilst failure to achieve clear margins caused significant burden by outpatient admissions (176 vs. 4 days; χ2 980, p < 0.001) and exposure to causes for concern (1369 vs. 0 days; χ2 11.3, p = 0.003). Although limited by cohort size, our study emphasizes the paradigm of getting it right the first time as key to improve survivorship in a cancer with excellent long-term prognosis. Full article
(This article belongs to the Special Issue Advances in Parathyroid Carcinoma: From Bench to Bedside)
►▼ Show Figures

Graphical abstract

15 pages, 1243 KiB  
Article
Mediastinal Parathyroid Cancer
by Klaus-Martin Schulte, Gabriele Galatá and Nadia Talat
Cancers 2022, 14(23), 5852; https://doi.org/10.3390/cancers14235852 - 28 Nov 2022
Cited by 1 | Viewed by 1580
Abstract
Parathyroid cancer (PC) is rare, but its pre-operative recognition is important to choose appropriate access strategies and achieve oncological clearance. This study characterizes features of mediastinal parathyroid cancer (MPC) and explores criteria aiding in the pre-operative recognition of malignancy. We assembled data from [...] Read more.
Parathyroid cancer (PC) is rare, but its pre-operative recognition is important to choose appropriate access strategies and achieve oncological clearance. This study characterizes features of mediastinal parathyroid cancer (MPC) and explores criteria aiding in the pre-operative recognition of malignancy. We assembled data from 502 patients with mediastinal parathyroid neoplasms (MPNs) from a systematic review of the literature 1968–2020 (n = 467) and our own patient cohort (n = 35). Thirty-two of the 502 MPNs (6.4%) exhibited malignancy. Only 23% of MPC patients underwent oncological surgery. Local persistence and early recurrence at a median delay of 24 months were frequent (45.8%), and associated with a 21.7-fold (95%CI 1.3–351.4; p = 0.03) higher risk of death due to disease. MPCs (n = 30) were significantly larger than cervical PC (n = 330), at 54 ± 36 mm vs. 35 ± 18 mm (χ2 = 20; p < 0.0001), and larger than mediastinal parathyroid adenomas (MPA; n = 226) at 22 ± 15 mm (χ2 = 33; p < 0.01). MPC occurred more commonly in males (60%; p < 0.01), with higher calcium (p < 0.01) and parathyroid hormone (PTH) levels (p < 0.01) than MPA. Mediastinal lesions larger than 3.0 cm and associated with a corrected calcium ≥ 3.0 mM are associated with a more than 100-fold higher odds ratio of being malignant (OR 109.2; 95%CI 1.1–346; p < 0.05). The composite 3 + 3 criterion recognized 74% of all MPC with an accuracy of 83%. Inversely, no MPN presenting with a calcium < 3.0 mM and size < 3.0 cm was malignant. When faced with pHPT in mediastinal location, consideration of the 3 + 3 rule may trigger an oncological team approach based on simple, available criteria. Full article
(This article belongs to the Special Issue Advances in Parathyroid Carcinoma: From Bench to Bedside)
►▼ Show Figures

Graphical abstract

12 pages, 530 KiB  
Article
Parathyroid Carcinoma: Incidence, Survival Analysis, and Management: A Study from the SEER Database and Insights into Future Therapeutic Perspectives
by Asad Ullah, Jaffar Khan, Abdul Waheed, Nitasha Sharma, Elizabeth K. Pryor, Tanner R. Stumpe, Luis Velasquez Zarate, Frederick D. Cason, Suresh Kumar, Subhasis Misra, Sravan Kavuri, Hector Mesa, Nitin Roper, Shahin Foroutan, Nabin Raj Karki, Jaydira Del Rivero, William F. Simonds and Nagla Abdel Karim
Cancers 2022, 14(6), 1426; https://doi.org/10.3390/cancers14061426 - 10 Mar 2022
Cited by 19 | Viewed by 3679
Abstract
Introduction: Parathyroid carcinoma (PC) is an extremely rare entity, with a frequency of 0.005% of all malignancies. Most data related to this rare disease are limited to case series and a few database studies. We present a large database study that aims to [...] Read more.
Introduction: Parathyroid carcinoma (PC) is an extremely rare entity, with a frequency of 0.005% of all malignancies. Most data related to this rare disease are limited to case series and a few database studies. We present a large database study that aims to investigate the demographic, clinical, and pathological factors, prognosis, and survival of PC. Methods: Data of parathyroid carcinoma were extracted from the Surveillance, Epidemiology, and End Results (SEER) diagnosed between 1975 and 2016. Results: PC had a slightly higher incidence in men (52.2%, p < 0.005), the majority of cases affected Caucasians (75.4%, p < 0.005), and the mean age at diagnosis was 62 years. Histologically, 99.7% were adenocarcinomas not otherwise specified (p < 0.005), well-differentiated (p < 0.005), and 2–4 cm (p < 0.001) in size among the patients with available data. In cases with staging provided, most PC were organ-confined (36.8%, p < 0.001). Lymph nodes were positive in 25.2% of cases where lymph node status was reported. The main treatment modality was surgery (97.2%), followed by radiation alone (2%), and very few received chemotherapy alone (0.8%), p < 0.005. Five-year follow-up was available for 82.7% of the cases. Those who underwent surgery only or radiation alone had 5-year survivals of 83.8% and 72.2%, respectively (p < 0.037). Multivariable analysis identified tumor size >4 cm, age > 40 years, male sex, Caucasian race, distant spread, and poorly differentiated grade as independent risk factors for mortality (p < 0.001). Conclusion: PC is a very rare tumor mostly affecting Caucasian individuals in the fifth decade. Older age, poor histologic differentiation, and distant metastasis are associated with a worse prognosis. Surgical resection offers the best survival outcome. To better understand the pathogenesis and factors affecting survival, all PC patients should be enrolled in national and international registries. Full article
(This article belongs to the Special Issue Advances in Parathyroid Carcinoma: From Bench to Bedside)
►▼ Show Figures

Figure 1

Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:
 
Displaying articles 1-4
Back to TopTop