Persistent Hypercalcemia Crisis and Recurrent Acute Pancreatitis Due to Multiple Ectopic Parathyroid Carcinomas: Case Report and Literature Review of Mediastinal Parathyroid Carcinoma

Abstract

Mediastinal parathyroid carcinoma (PC) is a rare entity in primary hyperparathyroidism. The aim of this report is to demonstrate a case of mediastinal PC, and to provide a systemic literature review of this rare condition. A 34-year-old woman who had already undergone two cervical operations for hyperparathyroidism suffered from another recurrence, presenting with recurrent acute pancreatitis and persistent hypercalcemic crisis. Technetium-99 methoxyisobutylisonitrile imaging (MIBI) and computed tomography scanning (CT) identified three possible parathyroid tumors, one of which was the recurrence of residual tumor locating in the thyroid region, while the other two were ectopic tumors locating in the suprasternal fossa and thymus region, respectively. Pathological examination confirmed the diagnosis of PC. We conducted a systemic literature review by searching the PubMed MEDLINE from 1951 to 2019 for studies of all types in the English language only, using terms "mediastinal, mediastinum, parathyroid, carcinoma." Including our reported case, a total of 21 cases with ectopic mediastinal PCs were assessed for demographic data, tumor location and size, biochemical findings, and symptomatology, etc. Two thirds of the patients were men, with a mean age of 44 years old, a mean serum calcium of 14.2 mg/dl, and a mean serum intact parathyroid hormone of 1,216 pg/ml. We identified 89.5% of carcinomas in the anterosuperior mediastinum, and 10.5% in the middle mediastinum, with a mean diameter of 54 mm, and a mean weight of 216 g. MIBI and CT were the most commonly used methods to localize these mediastinal tumors, with 69.2 and 100% sensitivity, respectively. Half of the patients underwent more than one operation. Diagnosis and treatment of mediastinal PCs represent a challenge. Early suspicion, appropriate preoperative localization studies, and the cooperation of endocrinologists and surgeons are crucial in the effective management.

A case report of mediastinal parathyroid carcinoma in a chronic kidney disease patient: Addressing management conundrum

“…Clinical features that suggest a diagnosis of PC include age less than 50 years; markedly elevated PTH and calcium levels (usually >14mgdL), severe bone pain and lesions, and recurrent laryngeal nerve palsy from tumor invasion. 9 Most complications involve the skeletal and renal systems, although pancreatitis and hypercalcemic crisis may occur. 9 The Schulte 3 + 3 rule was initially developed for cervical PC, but it has been applied to mediastinal PC with 100% specificity and positive predictive value in ruling out malignancy.…”

Chirurgische Therapie kritischer Hyperkalzämien und hyperkalzämischer Krisen

“…Anamnese Aufgrund der o. g. pathophysiologischen Bedeutung kritischer Hyperkalzämien ist es besonders wichtig, neben einer symptomatischen Therapie zur Senkung des Serum-Kalziumspiegels [33] so schnell wie möglich die zugrunde liegende Ursache der Hyperkalzämie zu erkennen [7,11,14,24,32,34,35,36,37,38]. Hierzu sollte neben der genauen Eigenanamnese auch die Familienanamnese erfragt werden, um bei Malignomen oder PHPT genetisch bedingte Ursachen auszuschließen.…”

“…Bei der Suche nach einer oder mehreren vergrößerten Nebenschilddrüsen im Rahmen eines PHPT ist bei kritischer Hyperkalzämie häufig mit einer ektopen, auch intrathorakalen Lage beteiligter NSD zu rechnen [5]. Umgekehrt werden ektop gelegene NSD-Adenome und Nebenschilddrüsenkarzinome oft erst diagnostiziert, wenn sie zu einer kritischen Hyperkalzämie oder hyperkalzämischen Krise geführt haben [37,41].…”

Mediastinal parathyroid carcinoma: a case report and review of the literature

“…The first case of mediastinal parathyroid carcinoma was reported by Weissman in 1957 [ 8 ] and we identified that there were only scattered reports published before 1982, which were not included in this review due to their inherent differences with current radiological examination and treatment methods [ 9 , 10 ]. Table 1 summarized 20 cases included in our final sample [ 11 – 30 ].…”

“…Table 1 summarized 20 cases included in our final sample [11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30].…”