Concurrent Parathyroid Adenomas and Carcinoma in the Setting of Multiple Endocrine Neoplasia Type 1: Presentation as Hypercalcemic Crisis

• Published in: Mayo Clinic proceedings Vol. 77; no. 8; pp. 866 - 869
• Main Authors: Dionisi, Simona, Minisola, Salvatore, Pepe, Jessica, De Geronimo, Simona, Paglia, Federica, Memeo, Lorenzo, Fitzpatrick, Lorraine A.
• Format: Journal Article
• Language: English
• Published: Rochester, MN Elsevier Inc 01.08.2002; Mayo Medical Ventures; Elsevier Limited
• Subjects: Acute Disease; Adenoma - diagnosis; Adenoma - pathology; Adult; Biological and medical sciences; Carcinoma - diagnosis; Carcinoma - pathology; Endocrinopathies; Fatal Outcome; Gastrinoma - diagnosis; Gastrinoma - pathology; Gastrins - blood; Humans; Hypercalcemia - etiology; Hyperparathyroidism - etiology; Male; Malignant tumors; Medical sciences; Multiple Endocrine Neoplasia Type 1 - diagnosis; Multiple Endocrine Neoplasia Type 1 - pathology; Pancreatic Neoplasms - diagnosis; Pancreatic Neoplasms - pathology; Parathyroid Hormone - blood; Parathyroid Neoplasms - diagnosis; Parathyroid Neoplasms - pathology; Parathyroids. Parafollicular cells. Cholecalciferol. Phosphocalcic homeostasis (diseases); ZES; MEN 1; PTHrP; PTH; HPT; Endocrinopathy; Human; Hydroelectrolytic balance disorder; Adenoma; Malignant tumor; Inorganic element; Case study; Hypercalcemia; Metabolic disorder; Treatment; Parathyroid diseases; Parathyroid glands; Diagnosis
• ISSN: 0025-6196; 1942-5546
• DOI: 10.4065/77.8.866

We describe a patient with multiple endocrine neoplasia type 1 characterized by the simultaneous occurrence of parathyroid cancer, parathyroid adenomas, and pancreatic gastrinoma, who presented with an episode of acute hypercalcemia. The rapid parathyroid hormone assay provided a basis for the diagnosis of parathyroid hyperfunction. Mediastinal metastasis of the parathyroid carcinoma was found at autopsy. However, the staining of pancreatic and gastric tissue for parathyroid hormone-related protein does not make it possible to exclude completely the contri bution of this peptide in mediating the hypercalcemia. To our knowledge, this is the first reported case of parathyroid carcinoma as part of the multiple endocrine neoplasia type 1 syndrome.