scholarly journals Management and Outcome of Parathyroid Carcinoma-Induced Primary Hyperparathyroidism: A Single-Centre Experience

2021 ◽  
Vol 2021 ◽  
pp. 1-12
Author(s):  
Loredana De Pasquale ◽  
Antonio Mario Bulfamante ◽  
Giovanni Felisati ◽  
Luca Castellani ◽  
Giorgio Ghilardi ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Carcinoma ◽  
Preoperative Evaluation ◽  
Neck Mass ◽  
En Bloc ◽  
Blood Calcium ◽  
Risk Class ◽  
Excellent Outcome ◽  
Postoperative Hypoparathyroidism

Background. Parathyroid carcinoma (PC) is the rarest endocrine cancer and an infrequent cause of primary hyperparathyroidism (PHPT), responsible for less than 1% of cases. Due to its rarity, treatment is challenging. Methods. A retrospective cohort study on 462 patients referred for parathyroidectomy to Thyroid and Parathyroid Unit at Santi Paolo e Carlo Hospital, Milan, Italy, from 2011 to 2021. We identified and individually described the patients affected with PC. Then, we split all patients treated for PHPT into four groups based on the cause: PC, adenoma, atypical adenoma, and hyperplasia. Patients’ demographics, preoperative evaluation results, intraoperative findings, and outcomes for the PC group were compared with groups of PHPT due to benign causes. Results. Eight cases of PC were identified, five males and three females. Seven cases presented with symptoms of hypercalcemia and one with a neck mass. Five underwent en bloc resections and three local excisions. Histopathological features showed capsular invasion in four patients, capsular and soft tissue invasion in three patients, and vascular invasion in one case. No patients had distant metastasis. One patient was classed as high risk based on the Schulte classification system. All patients treated for PC were alive and disease-free at a mean follow-up of 38.4 months. When compared with other PHPT patients, PC patients were more frequently male and had higher preoperative blood calcium and PTH and lower phosphate levels, larger and heavier parathyroids excised, lower postoperative calcium, and a higher rate of postoperative hypoparathyroidism. Conclusion. Our study highlights some aspects valuable to suspect PC and differentiate PHPT-PC from benign causes of PHPT preoperatively. Preoperative suspicion of malignancy is essential to guarantee the best course of treatment for patients. Although limited for size and follow-up, the excellent outcome of our series seems to support the value of both surgery extension and risk class according to the Schulte classification as possible prognostic factors for recurrence.

scholarly journals Parathyroid carcinoma

2006 ◽  
Vol 63 (8) ◽  
pp. 765-769
Author(s):  
Aleksandar Filipovic ◽  
Ivan Paunovic ◽  
Dragutin Savjak ◽  
Tamara Zivkovic
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Carcinoma ◽  
Disease Recurrence ◽  
En Bloc Resection ◽  
Bloc Resection ◽  
Endocrine Tumors ◽  
En Bloc ◽  
Histologic Diagnosis ◽  
Serum Pth

Background. Parathyroid carcinoma is the least frequent malignancy among endocrine tumors. In the most reported series of patients with primary hyperparathyroidism the incidence of carcinoma is less than 1%. Recognition by a surgeon that the parathyroid tumor is malignant, and the performance of an adequate en bloc removal of primary lesion, with histologic diagnosis offer the best treatment of a patient with this unusual malignancy. Case report. We reported a 30-year-old patient with parathyroid carcinoma, primary hyperparathyroidism, and recurrent nephrocalcinosis. Marked hypercalcemia, low serum phosphorus, and substantial elevation of serum parathyroid hormone indicated a diagnosis of primary hiperparathyroidism. General symptoms were anorexia, muscle weakness, back pain and depression. Ultrasonography done before the surgery revealed a 2 cm upper left parathyroid gland with solid and cystic areas. The neck exploration was done with en block resection of the tumor. A histopathological evaluation confirmed the diagnosis of parathyroid carcinoma. Over more than a three-year-follow-up, the patient had no evidence of the disease recurrence and his serum PTH and calcium levels remained within the normal. Conclusion. Parathyroid carcinoma is a rare cause of primary hyperparathyroidism. Preoperative diagnosis remains a challenge. Radical en bloc resection of the tumor is the treatment of choice for this malignancy.

scholarly journals Clinical suspicion and parathyroid carcinoma management

2006 ◽  
Vol 124 (1) ◽  
pp. 42-44 ◽  
Author(s):  
Fabio Luiz de Menezes Montenegro ◽  
Marcos Roberto Tavares ◽  
Marcelo Doria Durazzo ◽  
Claudio Roberto Cernea ◽  
Anói Castro Cordeiro ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Secondary Hyperparathyroidism ◽  
Parathyroid Carcinoma ◽  
Clinical Signs ◽  
Neck Surgery ◽  
Cross Sectional Study ◽  
Clinical Suspicion ◽  
En Bloc ◽  
Cross Sectional ◽  
Adjacent Structures

CONTEXT AND OBJECTIVE: Adequate management of parathyroid carcinoma apparently relates to the surgeon’s ability to identify it at the first operation. The objective of this paper was to evaluate the role of clinical suspicion in the management of parathyroid carcinoma. DESIGN AND SETTING: Retrospective analysis of parathyroid carcinoma patients treated in Department of Head and Neck Surgery, Faculdade de Medicina da Universidade de São Paulo. METHODS: Cross-sectional study of 143 patients who underwent surgery from 1995 to 2000, due to hyperparathyroidism. These cases were reviewed to ascertain whether preoperative and intraoperative suspicion of parathyroid carcinoma were helpful during the operation, and which factors demonstrated the suspicion of cancer best. RESULTS: Among 66 patients with primary hyperparathyroidism there were four cases of parathyroid carcinoma (6.1%), and one case was found in secondary hyperparathyroidism (1.3%). Palpable nodules were found in five patients with primary hyperparathyroidism, four of them with parathyroid carcinoma. Preoperative levels of calcium in primary hyperparathyroidism with cancer patients varied from 12.0 mg/dl to 18.2 mg/dl. Two patients had gross macroscopic spread of the tumor to adjacent structures. Except for one patient, with extensive disease, tumors were resected en bloc. In secondary hyperparathyroidism, parathyroid carcinoma was found in a fifth mediastinal gland. One atypical adenoma was observed. CONCLUSIONS: High levels of calcium, palpable tumors and adherence to close structures are more common in parathyroid carcinoma. These clinical signs may be helpful for decision-making during parathyroid surgery.

Parathyroid carcinoma: a report of two cases and a concise review and update of the literature

2005 ◽  
Vol 119 (7) ◽  
pp. 577-580 ◽  
Author(s):  
Humera Babar-Craig ◽  
Alberto Quaglia ◽  
Michael Stearns
Keyword(s):  
Primary Hyperparathyroidism ◽  
Dialysis Patient ◽  
Parathyroid Carcinoma ◽  
Recurrent Disease ◽  
Treatment Options ◽  
Renal Dialysis ◽  
Neck Mass ◽  
Tertiary Hyperparathyroidism ◽  
Concise Review ◽  
Recent Trends

Parathyroid carcinoma is very rare and when diagnosed can be difficult to treat as more than 50 per cent have persistent or recurrent disease. Patients generally present with profound symptoms of hypercalcaemia and a palpable neck mass. We describe a case of parathyroid carcinoma in primary hyperparathyroidism and a case in tertiary hyperparathyroidism in a renal dialysis patient. Most studies in the literature are retrospective but recent trends in presentation, treatment options and complications have been reviewed and are summarized in this paper.

scholarly journals MON-920 Pediatric Parathyroid Carcinoma Presenting With Acute Progressive Genu Valgum

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Tina Y Hu ◽  
Loren Berman ◽  
William J Parkes ◽  
Jeanne M Franzone ◽  
Tejal Mody ◽  
...  
Keyword(s):  
Parathyroid Carcinoma ◽  
Pediatric Patients ◽  
En Bloc Resection ◽  
Neck Mass ◽  
Bloc Resection ◽  
Genu Valgum ◽  
Parathyroid Tumor ◽  
En Bloc ◽  
Neck Ultrasound ◽  
Hungry Bone

Abstract BACKGROUND: Parathyroid carcinoma (PC) is exceptionally rare in children and its clinical features are poorly understood. Available reports have identified that very high serum calcium and parathyroid hormone levels would be expected in this disease. Definitive therapy for PC requires en bloc resection of the parathyroid tumor to reduce the risk of disease recurrence. Unfortunately, there are very few treatment options for metastatic PC and patients with recurrent PC have a high risk of mortality related to their disease. Because of the rarity of the condition, there are very limited data including pediatric patients. Case: A previously healthy 13-year-old girl initially presented to primary care with bilateral foot and ankle pain in the context of acute and progressive genu valgum, referred to the orthopedic clinic. Lab evaluation prompted by imaging demonstrated severe hypercalcemia (15.6 mg/dL) and hyperphosphatasia (Alkaline phosphatase 2056 units/L). She was urgently referred to our clinic where we found her to have a firm right sided neck mass within the thyroid. Lab testing at that time confirmed profound hyperparathyroidism (PTH 2970 pg/mL). Neck ultrasound confirmed the presence of a 5.8 cm hypoechoic lesion adjacent to the right lower lobe of the thyroid. The patient received IV fluid resuscitation before undergoing urgent parathyroid surgery. The parathyroid lesion was grossly adherent to the thyroid gland and she received en bloc resection of the tumor and right lobe of the thyroid. Post-operatively, PTH rapidly declined to <3.4 and she developed a prolonged hypocalcemic phase. PTH function improved, but she continued to require high doses of calcium carbonate to address hungry bone syndrome over subsequent weeks. Comprehensive evaluation included testing for genetic mutations, deletions, and duplications in CASR, CDC73, CDKN1B, MEN1 and RET, all of which were negative. Extensive genetic testing of the parathyroid tumor did not identify any specific mutations which have been previously associated with PC. Six months post-operatively, her labs have vastly improved, although she has clinical evidence of PTH resistance and her alkaline phosphatase has not yet normalized. Neck ultrasound, F-18 FDG PET/MRI imaging and Sestamibi scans show no evidence of persistent disease. Conclusions: While parathyroid carcinoma is extremely rare in pediatric patients, the diagnosis must be considered in cases of hypercalcemia with marked elevations in PTH and palpable neck mass. Although rarely described, bone changes related to PC may be dramatic and may result in lasting morbidity - including genu valgum requiring surgical intervention as well as prolonged hungry bone syndrome and metabolic bone disease. Genu valgum is a unique pediatric feature of this disease because this may only evolve in individuals whose physes are open.

scholarly journals Benign, Malignant or Something in Between: A Rapidly Developing Atypical Parathyroid Adenoma

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A183-A184
Author(s):  
Albana Sykja ◽  
Ye Lynn Ko ◽  
Rajeev Raghavan ◽  
Harit Buch
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Adenoma ◽  
Parathyroid Carcinoma ◽  
Malignant Potential ◽  
Thyroid Lobe ◽  
Uncertain Malignant Potential ◽  
Parathyroid Adenomas ◽  
Atypical Parathyroid Adenoma ◽  
Left Thyroid Lobe

Abstract Introduction: Although Primary Hyperparathyroidism (PHPT) is the third most common endocrine disorder, parathyroid carcinoma and atypical parathyroid adenoma are the rarest of endocrine tumours. The true incidence of atypical parathyroid adenomas has been elusive to endocrinologists since it is not possible to differentiate clinically between parathyroid carcinoma and atypical parathyroid adenomas before histological analysis. Atypical parathyroid adenoma represents a group of an intermediate form of parathyroid neoplasms with uncertain malignant potential. The majority of patients present with hypercalcaemia, however the development of atypical adenoma in patients with known PHPT is extremely rare. Clinical Case: A 78-year-old gentleman presented at the emergency department with lethargy and slurred speech which had started 1 week ago. Blood tests revealed severe hypercalcaemia. (Ca 4.98 mmol/L, PTH 114.2 pmol/L). The patient had a background of primary hyperparathyroidism which was diagnosed due to incidental mild hypercalcaemia (Ca 2.71 mmol/L, PTH 17.57 pmol/L, 25OH-vitamin D3 55 nmol/L). 2 weeks prior to presentation to the Emergency Department calcium and PTH levels were stable. On clinical examination, he was found mildly confused with no other clinical findings. No precipitating factors were identified. Hydration with IV crystalloids commenced and bisphosphonate IV was given. In view of PHPT Cinacalcet was added to treatment (30 mg BD). While the calcium levels seemed to improve initially, (lowest level achieved Ca 3.05 mmol/L) a week later they started to rise gradually. Hypercalcaemia proved refractory to medical treatment despite concomitant use of aggressive hydration, increased cinacalcet dose, second intravenous bisphosphonate, and intravenous calcitonin. Neck U/S revealed a probable parathyroid adenoma measuring 2cm axially at the inferior pole of the left thyroid lobe. In view of the severity and refractory nature of hypercalcaemia, a PET CT was requested which identified an 18 mm soft tissue mass in the left lower neck posterior to the left thyroid lobe with moderate to intense FDG uptake. There was no evidence of increased uptake elsewhere. The patient required 2 sessions of haemodialysis to maintain calcium levels around 3.5 preoperatively. He underwent parathyroidectomy with histological findings in keeping with atypical parathyroid adenoma. Gradual reduction of calcium levels was noted post-operatively with the lowest on day 10 (1.99) when he was started on oral calcium supplementation. The patient remains under follow-up with normal calcium levels 6 months postoperatively while remains on calcium and vitamin D3 supplements. Conclusion: To our knowledge, this is the only case of a patient with known primary hyperparathyroidism and mild hypercalcaemia, to develop severe parathyroid crisis with refractory to medical management hypercalcaemia within 2 weeks. Prompt surgical intervention remains of paramount importance in the management of these patients. They should have lifelong follow up in the view of uncertain malignant potential of the atypical parathyroid adenoma.

Parathyroid Carcinoma in Tertiary Referral Centre: A 12-Year Case Series

2021 ◽  
Vol 104 (6) ◽  
pp. 1033-1038
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Carcinoma ◽  
Case Series ◽  
En Bloc Resection ◽  
Bloc Resection ◽  
Postoperative Radiation ◽  
Tertiary Referral ◽  
En Bloc ◽  
Clinical Presentations ◽  
Laboratory Results

Background: Parathyroid carcinoma (PC) is a rare malignancy that is difficult to diagnose preoperatively, and when it is suspected from clinical features and laboratory results, en-bloc resection is performed. Objective: To retrospectively review the prevalence, clinical presentations, laboratory findings, management modalities, and treatment outcomes of PC cases in the present study tertiary referral hospital. Materials and Methods: Patients with PC that attended the present center between 2008 and 2019 were included. Demographic data, clinical presentations, preoperative laboratory results, management, and outcomes were retrospectively reviewed. Results: Ten of 102 cases (9.8%) of the primary hyperparathyroidism were diagnosed as PC. Five were male (50%), and the mean age at diagnosis was 47.90±15.50 years. All PC patients presented with renal or bony manifestations, and five out of nine (45.5%) had palpable neck mass. Mean preoperative parathyroid hormone and serum calcium were 1,688.56±858.84 pg/mL and 15.30±3.27 mg/dL, respectively. In eight of these PC cases (80%), en-bloc resection was performed, and six patients underwent postoperative radiation treatment. Two patients died due to uncontrolled hypercalcemia with distant metastasis at 5.5- and 8-years after diagnosis. Conclusion: The prevalence of PC among primary hyperparathyroidism is as high in Thailand as in other Asian countries. En-bloc resection is performed when clinical presentations and laboratory results are suggestive of PC. Postoperative radiation is considered in selected cases. Intractable hypercalcemia is the main cause of death. Keyword: Parathyroid cancer; Parathyroid carcinoma; Primary hyperparathyroidism; Hypercalcemia; Parathyroid carcinoma case series

The rare cause of primary hyperparathyroidism: Parathyroid Carcinoma

2017 ◽  
Author(s):  
Nagihan Bestepe ◽  
Didem Ozdemir ◽  
Sefika Burcak Polat ◽  
Berna Evranos ◽  
Reyhan Ersoy ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Carcinoma
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