scholarly journals SAT-LB65 A Rare Existence

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Jiselle Aimee Yaplito Bedia ◽  
Maria Honolina S Gomez

Abstract Primary hyperparathyroidism is a common endocrine disorder of metabolism usually due to a parathyroid adenoma. Although, the clinical presentation of primary hyperparathyroidism has changed from Albright’s description of a disease of bones and stones, the central target organs affected by this disorder continue to be the skeleton and kidneys. With the advent of routine biochemical screening, the typical diagnosis of primary hyperparathyroidism is no longer accompanied by overt skeletal and renal involvement. Majority of the cases of primary hyperparathyroidism are due to parathyroid adenomas. Giant glands were defined as greater than the 95th percentile, characterized as glands weighing > 3.5 grams. This present case in a 54-year old female is a rare case of primary hyperparathyroidism secondary to a giant parathyroid adenoma measuring 10.7 x 8.0 x 40.0 cm and weighing 145 grams, the largest giant parathyroid adenoma reported to date, with co-existent silent thymoma, multinodular goiter and osteosclerosis of the vertebral spine, metaphorically known as the “rugger-jersey spine”. The association between thymoma and parathyroid adenoma is rare, and only 3 cases have been reported in the literature. We characterized the correlation of preoperative imaging, intraoperative location, and postoperative course, including significant postoperative hypoparathyroidism, as compared to other patients with PHPT to determine whether giant adenomas represent a clinical entity with distinct clinical characteristics. Keywords: primary hyperparathyroidism, giant parathyroid adenoma, rugger-jersey spine, thymoma Abbreviation PHPT Primary hyperparathyroidism

2021 ◽  
Author(s):  
Grzegorz Kowalski ◽  
Grzegorz Buła ◽  
Adam Bednarczyk ◽  
Agata Gawrychowska ◽  
Jacek Gawrychowski

Abstract BACKGROUND Primary hyperparathyroidism (PHPT) is caused by benign and malignant conditions. Most commonly by typical adenoma/single gland disease (PA) - this is 80-85% cases of PHPT. Parathyroid hyperplasia or multiple gland disease accounts for 10-15% of cases of PHPT. Atypical parathyroid adenoma (APA) and carcinoma (PC) - very rare conditions - are both responsible for PHPT in approximately 0,5 - 1,5% of cases. OBJECTIVES To estimate occurrence of atypical parathyroid adenoma, parathyroid carcinoma and parathyroid hyperplasia along with characterize them based on their etiology, clinical presentation, diagnosis and treatment METHODS We performed a retrospective study and enrolled 1,019 patients with primary hyperparathyroidism undergoing parathyroidectomy at academic Department of General and Endocrine Surgery between 1983 and 2018. RESULTS Out of 1,019 cases of primary hyperparathyroidism, 850 (83.4%) cases were due to typical parathyroid adenoma (PA), 135 (13.2%) cases were due to parathyroid hyperplasia, 29 (2.8%) cases were due to parathyroid carcinoma (PC), and 5 (0.5%) cases were due to atypical parathyroid adenoma (APA).


2021 ◽  
pp. 014556132110209
Author(s):  
Christian Caceres ◽  
Kourosh Parham

Parathyroid adenomas are responsible for 80% to 85% of cases of primary hyperparathyroidism. Giant parathyroid adenomas are a rare type of parathyroid adenoma defined as weighing >3.5 grams. Although giant parathyroid adenomas are rare entities whose clinical presentation may not be atypical, their surgical management can be challenging, especially in the setting of anatomical variants. We present here a case of a 29-year-old female with a 37-gram giant adenoma which was diagnosed after presentation with recurrent severe headaches. The presentation was also unique in that it was associated with a right aberrant subclavian artery and nonrecurrent laryngeal nerve.


Author(s):  
Brent D. Bauman ◽  
Maria Evasovich ◽  
Amanda Louiselle ◽  
Eugene Zheng ◽  
Kevin Goodwin ◽  
...  

AbstractBackground:Hyperparathyroidism (HPT) is a rare disease in the pediatric population, and optimal management may be unclear if it is due to an occult parathyroid adenoma. We present a case report of a pediatric patient with an occult, ectopic, supernumerary, parathyroid adenoma.Case presentation:A 13-year-old female who initially presented with anxiety was diagnosed with HPT. Preoperative imaging and bilateral neck exploration with four-gland biopsy were negative for any parathyroid adenoma. Postoperative MRI identified a thymic mass. She subsequently underwent video-assisted thoracoscopic thymectomy with resection of an intrathymic parathyroid adenoma.Conclusions:The diagnosis of pediatric HPT is increasing. Supernumerary or occult parathyroid adenomas are rare and add complexity to presurgical planning and management. Our case represents the rare occurrence of a pediatric ectopic supernumerary occult parathyroid adenoma treated with a two-stage approach utilizing multiple imaging studies. We provide a review of the pathology and propose an algorithmic approach to manage these complex patients.


2019 ◽  
Vol 3 (12) ◽  
pp. 2305-2312 ◽  
Author(s):  
Elena Castellano ◽  
Roberto Attanasio ◽  
Alberto Boriano ◽  
Giorgio Borretta

Abstract Background The clinical presentation of primary hyperparathyroidism (PHPT) has changed greatly during the past few decades. Our aim was to evaluate whether the clinical presentation at diagnosis differed according to age. Methods We evaluated retrospectively a monocentric series of 462 consecutive patients with PHPT, dividing them according to a cutoff of 65 years of age. Results No differences were found in the mean serum PTH, calcium, or vitamin D levels. In older patients (n = 212; 45.9%), the urinary calcium levels were significantly lower (median, 205 mg/24 hour; interquartile range, 220 mg/24 hour) compared with those in younger patients (median, 308 mg/24 hour; interquartile range, 233 mg/24 hour). In addition, renal involvement was significantly less frequent (25% vs 49.2%), and bone involvement significantly more frequent (58% vs 44%) in older patients compared with younger patients. The clinical presentation was significantly different between the two age groups, with a lower frequency of symptomatic forms and a greater frequency of asymptomatic forms not meeting surgical criteria in the older patients (44.4% vs 57.2% and 18.4% vs 5.6%, respectively). Osteoporosis was significantly more frequent in the older adults than in their younger counterparts. The most affected bone site was the forearm in older adults and the lumbar spine in younger ones (50.3% and 50.5%, respectively). Conclusion The clinical presentation of PHPT differs according to age, and this difference can affect the selection of management modalities.


2010 ◽  
Vol 2 (3) ◽  
pp. 131-133
Author(s):  
Geoffrey B Thompson ◽  
Benzon M Dy ◽  
Bianca Vazquez ◽  
Peter J Tebben ◽  
Seema Kumar

ABSTRACT Introduction Severe hypercalcemia due to primary hyperparathyroidism (PHPT) is rare in the setting of MEN 2A. Materials and methods Two patients with MEN 2A and severe hypercalcemia were identified recently. Their clinical presentation, evaluation, surgical management and outcomes are reviewed. Results Two patients with MEN 2A were identified with severe hypercalcemia secondary to a parathyroid adenoma. Calcium levels were elevated to 12.7 mg/dL and 15.1 mg/dL, respectively (normal range = 8.9-10.1 mg/dL). In each case, a single parathyroid adenoma was identified and surgically excised with normalization of parathyroid and calcium levels postoperatively. Clinical manifestations at the time of diagnosis included constipation, polyuria, hypercalciuria, and decreased bone mineral density. Conclusion Severe elevation of serum calcium is a rare presentation of PHPT in MEN2A. The differential diagnosis should include parathyroid adenoma, hyperplasia and parathyroid carcinoma. Early surgical management is essential in the treatment of hyperparathyroidism with severe hypercalcemia to prevent further complications.


1976 ◽  
Vol 81 (2) ◽  
pp. 298-309 ◽  
Author(s):  
P. Burckhardt ◽  
A. Bischof-Delaloye ◽  
B. Ruedi ◽  
B. Delaloye

ABSTRACT In 22 patients who underwent surgery suspected of primary hyperparathyroidism, the surgical findings were compared with the results obtained by pre-operative parathyroid scanning and biochemical screening. Thirteen of 15 parathyroid adenomas were localized by pre-operative scanning, but in five of them a false positive focus was also described. The technique was less useful in primary hyperplasia. Comparable results were reported by other investigators. In both instances the best results were obtained in patients with high parathyroid activity as measured by plasma calcium, plasma alkaline phosphatase and tubular reabsorption of phosphorus (TRP). Parathyroid scintigraphy was especially helpful in the presence of ectopic adenomas and in patients who had undergone previous parathyroid surgery. Unfortunately, the possibility of false positive results makes it unreliable for the diagnosis of primary hyperparathyroidism.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A266-A267
Author(s):  
Timur Gusov ◽  
John Chen Liu ◽  
Sowjanya Naha ◽  
F N U Marium ◽  
Joseph Theressa Nehu Parimi ◽  
...  

Abstract Primary hyperparathyroidism (PHPT) is defined as excessive secretion of parathyroid hormone (PTH) originating from the parathyroid gland. The most common cause is a single parathyroid adenoma which is typically solid. Cystic parathyroid adenomas (CPA) are the cause of about 1–2% of cases of primary hyperparathyroidism. It is known that cystic parathyroid adenomas are a result of degeneration of an existing parathyroid adenoma. SestaMIBI is an imaging study based on uptake of radioactive technetium99 and used to localize parathyroid adenomas. We describe an unusual case of severe hypercalcemia secondary to 99mTc sestaMIBI negative atypical parathyroid cystic adenoma. A 56-year-old male presented to our facility with nausea and vomiting. His past medical history included hypertension and hepatitis C with no history of fractures or kidney disease. Physical examination was normal. Upon admission the patient was afebrile with blood pressure of 170/120 mmHg and heart rate of 62 bpm. Chemistry showed Calcium of 14.5 mg/dL (8.6–10.2mg/dL), phosphorus 2.2 (2.7–4.5) mh/dL, magnesium 1.8 (1.7–2.6)mg/dL, intact PTH of 375 (15–65) pg/mL, PTH-related peptide <2.0 pmol/L(<2 pmol/L), 25-OH vitamin D of 19 ng/ml (30–80), Creatinine 1.22 (0.7–1.2)mg/dL, alkaline phosphatase 95 (40–129) units/L. He was started on aggressive hydration, calcitonin 4 units/kg, 4 mg of IV Zolendroninc acid. Neck sonogram revealed a large, complex, predominantly anechoic lesion with solid vascular components and thick internal septations in the inferior and medial aspect of the right thyroid lobe measuring 3 x 2 x 5.5 cm. Findings were confirmed with CT of the neck. Since Sestamibi scan (planar and SPECT/CT) did not show uptake in parathyroid glands, the cyst was thought to be of thyroid origin. Fine needle aspiration was not able to detect cellular material, but PTH was >100 pg/ml on the FNA sample. Otolaryngology service was consulted for parathyroidectomy. During the surgical treatment, the right upper parathyroid gland was removed with no changes in serum PTH. Next, the cystic lesion was removed with normalization of serum PTH (from 218 pg/ml to 35.2 pg/ml respectively). Intraoperative frozen section analysis was read as a cystic parathyroid adenoma. The final pathology report revealed cystic parathyroid tissue favoring parathyroid adenoma with focal atypia. Hypercalcemia resolved. Conclusions: Atypical cystic parathyroid adenomas are a rare cause of PHPT. 90% of parathyroid cysts are nonfunctional. Above mention is a case of a patient presenting with hypercalcemic crisis secondary to cystic parathyroid adenoma, which posed a diagnostic challenge as both neck ultrasound and 99mTc sestaMIBI scan were inconclusive. These findings should trigger suspicion for functional parathyroid lesions. Cystic components should be evaluated for PTH levels and if significantly elevated should be treated as a parathyroid adenoma.


2020 ◽  
Vol 10 (2) ◽  
pp. 94-101
Author(s):  
L. M. Farkhutdinova

The article is devoted to one of the actual medical and social problems — primary hyperparathyroidism, the late diagnosis of which leads to the development of severe complications and an increased risk of premature death. Unlike developed countries, where 80% of cases are represented by mild forms of the disease, in the Russian Federation this indicator does not exceed 30%, while 70% are manifest forms. Widespread awareness of doctors of various specialties in the diagnosis of parathyroid adenoma is necessary for the timely detection of the disease. The article reflects the main stages of the study of the disease, the pathogenesis of the clinical manifestations of primary hyperparathyroidism, the classic symptoms of which are changes in the target organs of the parathyroid hormone — bone tissue, urinary system and gastrointestinal tract, is considered. Bone disorders are the most common manifestation of hyperparathyroidism and are characterized by increased bone metabolism with a progressive decrease in bone mineral density. Typical changes in the kidneys include nephrolithiasis and nephrocalcinosis, causing the formation of renal failure. Gastrointestinal signs of hyperparathyroidism are erosion and ulcers of the stomach and duodenum, prone to bleeding, recurrent pancreatitis. Diagnosis of the disease is based on laboratory results, characterized by elevated levels of calcium and parathyroid hormone in the blood. Visualization of the paradenomas in most cases is provided by ultrasound and scintigraphy. Removal of parathyroid adenoma is the most effective treatment. A clinical case of a severe form of the disease is presented, indicating an urgent need to take measures to solve the problem of primary hyperparathyroidism.


2020 ◽  
Vol 13 (3) ◽  
pp. 285-290
Author(s):  
Berat Demir ◽  
Adem Binnetoglu ◽  
Akın Sahin ◽  
Dilek Gogas Yavuz

Objectives. As calcium included as a part of routine laboratory screening early diagnosis of primary hyperparathyroidism (PHPT) has been increased. Surgical resection of parathyroid adenoma or hyperplasia still is the mainstay of the treatment for most PHPT patients. The aim of this study was to evaluate of the surgical outcomes of patients with PHPT that referred to our ENT department of our University Hospital for the last 6 years.Methods. One hundred thirty-seven patients with PHPT who underwent parathyroid surgery in our clinic between October 2011 and January 2018 included in this retrospective study. Data on demographics, clinical findings, past medical history, preoperative laboratory values in 3 months, preoperative localizing imaging studies including ultrasonography (USG) and 99mTc-sestamibi (methoxyisobutyl isonitrile, [MIBI]) scan, operative findings, postoperative laboratory values, and pathology reports were recorded. MIBI scan and USG are used as the first-line modalities in our center. Single-photon emission computed tomography was used for challenging situations of re-exploration and ectopic parathyroid pathology. Four-dimensional computed tomography scanning is was preferred as the last imaging modality. Focused unilateral neck exploration (FUNE) was performed with intraoperative frozen section analysis as a routine procedure. Bilateral neck exploration (BNE) was used only in re-exploration, ectopic parathyroid, and with high suspicion of multigland disease.Results. Totally 137 patients (female:male, 3:3; mean age, 54.6±13.2 years) included in the study. Single parathyroid adenoma was found in 108 patients (78.8%). Most common adenoma localization was left inferior parathyroid gland (46.7%). FUNE was performed in 89.8% of the patients and BNE for 10.2% of the patients. Postoperative normocalcemia was reached in 132 patients and permanent hypocalcemia was observed in two patients. Persistence hypercalcemia observed in three patients. Postoperative pathology reports revealed three patients have parathyroid carcinoma.Conclusion. Preoperative imaging modalities is very important in parathyroidectomy surgery. Routine use of preoperative imaging modalities reduced the risk of complications in our clinic.


2021 ◽  
Vol 67 (6) ◽  
pp. 31-38
Author(s):  
E. E. Bibik ◽  
A. K. Eremkina ◽  
O. A. Knyazeva ◽  
N. G. Mokrysheva

Multiple lesions of the parathyroid glands (PTG) in primary hyperparathyroidism (PHPT) can be sporadic or develop as part of hereditary syndromes, manifesting at young age. There the description of a severe sporadic PHPT with big parathyroid neoplasms in the young patient is presented. Clinical data made it possible to suspect MEN-1 syndrome or parathyroid carcinomas; however, mutations CDKN, CDC73, MEN1 were excluded. The patient underwent removal of three identified tumors: benign adenomas of the left PTG and hyperplasia of the right one. Postoperative hypocalcemia and severe hungry bone syndrome required the administration of vitamin D and calcium carbonate preparations. However, a year after the operation, a «mild» recurrent disease was confirmed. Taking into account the patient’s refusal to reoperation and a significant improvement of the target organs state, active observation was continued. The patient needs further careful dynamic monitoring by specialists in order to timely identify indications for repeated surgical treatment to improve the life quality and span.


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