Abstract
BackgroundParathyroid carcinoma is a rare neoplasm that may present sporadically or in the context of a genetic syndrome. Diagnosis and management are challenging due to the lack of clinical and pathological features that may reliably distinguish malignant from benign disease.MethodsFrom January 2013 to December 2017, from 358 consecutive patients affected by parathyroid diseases, 3 patients with parathyroid carcinoma were treated at our academic Department of General Surgery. We present our experience as illustrative of the different features of clinical presentation of parathyroid carcinoma and review its management considering the recent relevant literature.ResultsCase 1: A 62-year-old man was hospitalized for left-sided palpable neck mass, hypercalcemia and elevated PTH. US-guided FNA was suspect for parathyroid carcinoma. A large cystic mass was excised in bloc with total thyroidectomy and central neck dissection. Genetic studies framed a pathologically confirmed parathyroid carcinoma within MEN1 syndrome. Case 2: A 48-year-old woman with hypothyroidism had total thyroidectomy performed for a suspect for right follicular thyroid lesion. Pathology revealed parathyroid carcinoma. Case 3: A 47 year-old man was admitted for hypercalcaemic crisis and renal failure in the context of PHPT. A lesion suggestive on US and MIBI scan for parathyroid adenoma in the right lower position was removed by mini-invasive approach. Pathology revealed parathyroid cancer and patient had completion hemythyroidectomy and central neck dissection.ConclusionParathyroid cancer is a particularly rare endocrine malignancy, however it should be suspected in patients with primary hyperparathyroidism when severe hypercalcemia is associated to cervical mass, renal and skeletal disease. Parathyroid surgery remains the mainstay of treatment. Radical tumour resection and expedited treatment in a dedicated endocrine Center represent crucial prognostic factors.
Highlights
Parathyroid carcinoma is a rare neoplasm that may present sporadically or in the context of a genetic syndrome
In a previous study we addressed parathyroid carcinoma arising in the context of Ferraro et al BMC Endocrine Disorders 2019, 19(Suppl 1)
Case 1: a 62-year-old gentleman with a background of nephrolithiasis, constipation, thalassaemia minor and non-insulin-dependent diabetes was admitted with an asymptomatic, left-sided large neck mass, raised serum calcium (2,92 mmol/L), elevated Parathyroid Hormone (PTH) (391.7 pg/ml) and polyuria
Summary
Parathyroid carcinoma is a rare neoplasm that may present sporadically or in the context of a genetic syndrome. Diagnosis and management are challenging due to the lack of clinical and pathological features that may reliably distinguish malignant from benign disease. Parathyroid carcinoma (PC) is an uncommon malignancy, less than 1000 recorded cases reported in literature since described by de Quervain in 1904 [1] as a non-functioning metastatic carcinoma [2,3,4,5,6]. The aim of this study is to appraise the recent literature on management of PC with respect to three illustrative cases of PCs treated consecutively at our Institution over the last 5 years
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