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      Functional parathyroid cyst in a patient with systemic lupus erythematosus: a case report

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          Summary

          Functional parathyroid cysts are a rare cause of primary hyperparathyroidism and are often mistaken for thyroid cysts. Systemic lupus erythematosus (SLE) is also a very rare cause of hypercalcemia. We report the case of a 62-year-old woman, who was diagnosed with SLE 30 years ago, presenting with clinical and biochemical features of primary hyperparathyroidism. Laboratory investigation revealed increased serum calcium and parathyroid hormone (PTH) levels; neck ultrasonography (USG) revealed 40×34×26 mm cystic mass in the left lobe of thyroid gland. PTH level in the cysts was >2500 pg/ml, determined by USG-guided fine-needle aspiration (FNA). In this case, no evidence for potential pathogenic association between parathyroid cyst and SLE was uncovered. However, the recognition of this association is very important because the therapeutical strategy is completely different. Operative management is usually straightforward and alleviates symptoms and any biochemical abnormalities caused by the cyst.

          Learning points

          • Functional parathyroid cysts are the rare cause of primary hyperparathyroidism and are often mistaken for thyroid cysts.

          • SLE is also a very rare cause of hypercalcemia.

          • Ultrasound-guided FNA of cystic fluid with assay for PTH level is an accurate method of differentiating parathyroid cyst from thyroid cyst.

          • Appropriate management of functional parathyroid cysts is surgical excision.

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          Most cited references15

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          A single-institution 25-year review of true parathyroid cysts.

          Parathyroid cysts (PCs) are rare, and their origin is a subject of debate. They have been described as either functional, causing hyperparathyroidism, or non-functional in eucalcaemic patients. We have performed a 25-year departmental review of PCs. Features studied included the clinical presentation and intra-operative findings, and a histological review was performed. Cases of cystic degeneration of parathyroid adenomas and pseudocystic change were excluded. Over 25 years, 22,009 thyroidectomies and 2,505 parathyroidectomies were performed in our department. Amongst these, 38 non-functional PCs were documented in 37 patients. The mode of presentation included incidental findings on routine chest x-ray, compressive symptoms or an asymptomatic palpable neck mass. Aspiration was the initial treatment in 14 patients and was curative in 10 of these. Four out of 14 patients underwent surgical procedures for recurrence of the cyst that occurred 6 to 48 months after aspiration. In 27 patients, surgery was performed and all identified PCs were localized in the inferior parathyroid glands. Histologically, the cyst wall consisted in associations of lymphoid, muscular, thymic, salivary, adipose and mesenchymal tissues. PCs are rare but should be included within the differential diagnosis of a neck lump. True PCs are non-functional. Pathological and immunohistochemical findings are suggestive of a branchial origin. Fine-needle aspiration may be curative and is diagnostic due to the characteristic appearance of the fluid and high PTH levels on assay.
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            Parathyroid cyst: often mistaken for a thyroid cyst.

            Parathyroid cysts are rare but clinically significant lesions. They can be functional, mistaken for a thyroid cyst, and/or managed nonoperatively on occasion. We identified seven patients (1 male, 6 females) with the diagnosis of parathyroid cyst from 1998 to 2003. Altogether, 33% of the patients had functional cysts. Sestamibi scans were performed in three of the seven patients, including two with functional cysts; none showed focal uptake. In toto, six of the seven cysts (86%) were found in an inferior parathyroid gland. All of the cysts had crystal-clear aspirate. C-terminal parathormone (PTH) levels were obtained from the aspirate from five of the seven (71%) patients. The mean level was 269,736 pg/ml (1970-1,268,074 pg/ml). Surgery was performed in three of the seven (43%) patients. All patients who underwent surgery improved postoperatively based on symptoms and serum levels of calcium and PTH. The four patients (57%) who were treated nonoperatively were subjected to aspiration alone; three (75%) of those patients required multiple aspirations. Most parathyroid cysts are nonfunctional and are rarely symptomatic. They are usually located in an inferior gland. If aspirated, most of the cysts reaccumulate fluid. Operative management is usually straightforward and alleviates symptoms and any biochemical abnormalities caused by the cyst.
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              Hypercalcemia and systemic lupus erythematosus.

              Hypercalcemia is commonly caused by the increased production of parathyroid hormone-related protein (PTHrP) by a malignancy. In fact, the demonstration of increased PTHrP production in a patient with hypercalcemia is virtually pathognomonic of malignancy. We studied a patient with systemic lupus erythematosus (SLE), generalized lymphadenopathy, and hypercalcemia. Immunohistology of 2 biopsied lymph nodes revealed the abundant expression of PTHrP and the absence of malignant transformation. Although apparently rare, PTHrP production by non-malignant lymphoid tissue may occur in SLE and should be considered in the differential diagnosis of hypercalcemia.
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                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                edm
                EDM Case Reports
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                1 February 2015
                2015
                : 2015
                : 140100
                Affiliations
                [1]Department of Endocrinology, The First Affiliated Hospital of Nanjing Medical University , 300 Guangzhou Road, Nanjing, 210029, People's Republic of China
                [1 ]Department of Endocrinology and Metabolism, Zhongshan Hospital, Fudan University , Shanghai, 200032, People's Republic of China
                [2 ]Department of General Surgery, The First Affiliated Hospital of Nanjing Medical University , Nanjing, 210029, People's Republic of China
                [3 ]Department of Nuclear Medicine, The First Affiliated Hospital of Nanjing Medical University , Nanjing, 210029, People's Republic of China
                Author notes
                Correspondence should be addressed to M Zhang Email: zhangmei@ 123456njmu.edu.cn
                Article
                EDM140100
                10.1530/EDM-14-0100
                4315945
                25755881
                1e01e512-4dbd-451d-affb-9c792d641962
                © 2015 The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

                History
                : 24 December 2014
                : 14 January 2015
                Categories
                Error in Diagnosis/Pitfalls and Caveats

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