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Introduction Surgical treatment of hyperparathyroidism related to chronic kidney disease is a real challenge for Brazilian public health care. High cost medications and long waiting lines to perform preoperative exams, especially technetium Tc 99m Sestamibi (MIBI) are some of the reasons. Despite the reality that the aid of localization exams are questionable in this scenario, doctors are too apprehensive in performing surgery without it. Objective The study aimed at evaluating the efficacy of surgery for renal hyperparathyroidism without preoperative MIBI. Methods A total of 114 patients were surgically treated. Total parathyroidectomy with autotransplantation and subtotal parathyroidectomy were carried out without preoperative MIBI. Results and conclusion Among the 114 patients undergoing surgery, 37 had secondary hyperparathyroidism in dialysis replacement, and 77 patients had post-renal transplant persistent disease. We were successful in 107 cases with only 7 failures (93.8% of success rate). Among these failures, only one parathyroid gland was not found in 4 cases, 2 parathyroid glands were not found in 2 cases and in 1 patient the 4 glands were found but this patient remained hypercalcemic and a postoperative diagnosis of supernumerary parathyroid gland was made. Surgery for treatment of renal hyperparathyroidism proved to be an effective (93.8%) and reproductible procedure, even without MIBI.

Neck ultrasound (US) and Technetium-99 m Sestamibi (MIBI) scan are the most commonly used imaging studies for preoperative localization of parathyroid adenomas. The aim of this study was to determine the added value of MIBI scan and its effect on the operative plan via a hypothetical model where a stepwise approach is conducted and MIBI is considered only after the ultrasound is evaluated. Patients who underwent parathyroidectomy for primary hyperparathyroidism (PHPT) between 2012 and 2019 at two tertiary centers were included. Data collected included demographic data, preoperative workup, operative findings and follow-up. The added value of MIBI scans was determined for patients with positive ultrasound. A total of 513 patients with positive US result and a MIBI scan were included. If a stepwise approach was conducted then MIBI scan would not change the operative plan in 492 (95.9%). Among the remaining 21 patients, MIBI scan would correctly change the ultrasound-based operative plan in only 12 (2.3%) patients, while incorrectly change the plan in 9 (1.8%), resulting in unnecessary exploration of the contralateral side. In patients with sonographic appearance of a parathyroid gland larger than 1.2 cm, MIBI scan would correctly change the operative plan in only 1 of the 287 (0.35%) patients. Our study suggests that the routine use of MIBI scans may have limited added value in patients with PHPT and a positive neck ultrasound, especially in those with adenoma size larger than 1.2 cm. Positive ultrasound alone may be sufficient for the preoperative localization of parathyroid disease.

Summary Brown tumors are osteoclastic, benign lesions characterized by fibrotic stroma, intense vascularization and multinucleated giant cells. They are the terminal expression of the bone remodelling process occurring in advanced hyperparathyroidism. Nowadays, due to earlier diagnosis, primary hyperparathyroidism keeps few of the classical manifestations and brown tumors are definitely unexpected. Thus, it may happen that they are misdiagnosed as primary or metastatic bone cancer. Besides bone imaging, endocrine evaluation including measurement of serum parathyroid hormone and calcium (Ca) levels supports the pathologist to address the diagnosis. Herein, a case of multiple large brown tumors misdiagnosed as a non-treatable osteosarcoma is described, with special regards to diagnostic work-up. After selective parathyroidectomy, treatment with denosumab was initiated and a regular follow-up was established. The central role of multidisciplinary approach involving pathologist, endocrinologist and oncologist in the diagnostic and therapeutic work-up is reported. In our opinion, the discussion of this case would be functional especially for clinicians and pathologists not used to the differential diagnosis in uncommon bone disorders. Learning points: Brown tumors develop during the remodelling process of bone in advanced and long-lasting primary or secondary hyperparathyroidism. Although rare, they should be considered during the challenging diagnostic work-up of giant cell lesions. Coexistence of high parathyroid hormone levels and hypercalcemia in primary hyperparathyroidism is crucial for the diagnosis. A detailed imaging study includes bone X-ray, bone scintiscan and total body CT; to rule out bone malignancy, evaluation of bone lesion biopsy should include immunostaining for neoplastic markers as H3G34W and Ki67 index. If primary hyperparathyroidism is confirmed, selective parathyroidectomy is the first-line treatment. In advanced bone disease, treatment with denosumab should be considered, ensuring a strict control of Ca levels.

Summary Brown tumors (BTs) are expansile osteolytic lesions complicating severe primary hyperparathyroidism (PHPT). Clinical, radiological and histological features of BTs share many similarities with other giant cell-containing lesions of the bone, which can make their diagnosis challenging. We report the case of a 32-year-old man in whom an aggressive osteolytic lesion of the iliac crest was initially diagnosed as a giant cell tumor by biopsy. The patient was scheduled for surgical curettage, with a course of neoadjuvant denosumab. Routine biochemical workup prior to denosumab administration incidentally revealed high serum calcium levels. The patient was diagnosed with PHPT and a parathyroid adenoma was identified. In light of these findings, histological slices of the iliac lesion were reviewed and diagnosis of a BT was confirmed. Follow-up CT-scans performed 2 and 7 months after parathyroidectomy showed regression and re-ossification of the bone lesion. The aim of this case report is to underline the importance of distinguishing BTs from other giant cell-containing lesions of the bone and to highlight the relevance of measuring serum calcium as part of the initial evaluation of osteolytic bone lesions. This can have a major impact on patients’ management and can prevent unnecessary invasive surgical interventions. Learning points: Although rare, brown tumors should always be considered in the differential diagnosis of osteolytic giant cell-containing bone lesions. Among giant cell-containing lesions of the bone, the main differential diagnoses of brown tumors are giant cell tumors and aneurysmal bone cysts. Clinical, radiological and histological characteristics can be non-discriminating between brown tumors and giant cell tumors. One of the best ways to distinguish these two diagnoses appears to be through biochemical workup. Differentiating brown tumors from giant cell tumors and aneurysmal bone cysts is crucial in order to ensure better patient care and prevent unnecessary morbid surgical interventions.

A 77-year-old man with a history of hypertension, prostate hyperplasia, and urolithiasis was admitted for acute kidney injury caused by hypercalcemia. Neck ultrasonography showed a large cyst adjacent to the right lower thyroid lobe. Although a 99mtechnetium sestamibi scan was negative, an extremely high intracystic intact parathyroid hormone level suggested that the cyst had a parathyroid origin and that a functional parathyroid cyst was present. Immunohistochemical staining for the calcium-sensing receptor (CaSR) after right lower parathyroidectomy revealed CaSR-positive cells lining the cyst, indicating that the functional parathyroid cyst had originated from the hemorrhagic degeneration of a parathyroid adenoma.

Primary hyperparathyroidism (PHPT) is the most common cause of hypercalcemia; patients are usually asymptomatic and the cause in 80-85% of cases is a single parathyroid adenoma (PA). Parathyroid adenomas arise from clonal expansion of tumor cells and may be located either posteriorly to the thyroid lobes or in ectopic sites. The incidence of intrathyroidal PAs varies from 1% to 6% and although uncommon, they pose certain diagnostic difficulties which may complicate treatment. The identification of the adenoma requires a combination of clinical evidence, imaging information and cytological findings due to the challenging distinction between thyroid and parathyroid lesions. We present the case of a patient with a large, partially cystic intrathyroidal parathyroid adenoma which was initially identified as a malignant thyroid nodule. We discuss the caveats that present in these rare cases and the important clinical and histological features that aid in the final diagnosis. In the case of our patient the cytological similarities between thyroid malignant cells and parathyroid cells, in combination with the negative sestamibi scan, resulted in a more invasive surgery than that a single parathyroid adenoma would require. Clinicians need to be vigilant in terms of the similarities between parathyroid and thyroid cytology in order to provide optimal patient care in these rare cases.

Highlights • Neonatal severe primary hyperparathyroidism is an exceedingly rare condition that has high mortality and morbidity if left untreated. • Medical therapy must be initiated as soon as the condition is diagnosed, and early surgery must be performed in patients who are refractory to the medical therapy. • Scintigraphic studies might sometimes fail to detect ectopic parathyroid glands. Intraoperative parathormone monitoring is particularly important to ensure complete removal of the parathyroid glands., Introduction Neonatal severe primary hyperthyroidism is an extremely rare disorder that occurs in the first six months of life. Early recognition and prompt surgical intervention are of vital importance for survival and to avoid neurological sequel. Hypotonia, lethargy, respiratory distress, and growth and developmental delay occur in association with elevated serum parathormone levels and hypercalcemia (Gannon et al., 2014). Definitive therapy involves total parathyroidectomy. Case presentation We are presenting a patient with Neonatal severe primary hyperparathyroidism, who successfully underwent total parathyroidectomy. The patient had been followed up with medical therapy until he was seven months old, with no adequate clinical response to medical therapy. Parathormone levels rapidly declined following total parathyroidectomy, and the parathormone level fell to zero after removal of the ectopic tissue with a second surgery, and the patient was discharged with full recovery. Discussion Sestamibi scintigraphy might not always show an ectopic parathyroid gland. In such conditions, confirmation of parathyroid glands excised with total parathyroidectomy by frozen biopsy is not sufficient to terminate surgery. Intraoperative parathormone monitoring is particularly important at this point. Persistently elevated parathormone levels should suggest a remnant parathyroid tissue at the surgical site or an ectopic parathyroid gland that needs to be excised. Conclusion Neonatal severe primary hyperparathyroidism is a life-threatening condition. Early surgery is life-saving in cases in whom medical therapy fails to control the disease.

Background Intra-operative identification of parathyroid glands is often a challenge for surgeons performing parathyroid or thyroid surgery. Parathyroid glands stimulated by near-infrared light emit autofluorescence, which allows their discrimination from all other tissues in the region, and this may be of value during thyroid and parathyroid surgery. In this study, we present the results of the utilization of a low-cost device developed for the identification of parathyroid glands in surgery for primary hyperparathyroidism. Case presentation In 5 patients operated in our hospital with the diagnosis of primary hyperparathyroidism and non-concordant ultrasonography and Sestamibi scan, we used a 780 nm Light Emitting Diode (LED) to stimulate the cervical area. The resulting autofluorescence was visualized with night vision goggles with a 832 nm filter assembled. In all the five patients, an easily distinguishable nodule was identified and excised, and confirmed as parathyroid adenoma by histological exam. Intra-operative PTH assay showed significant decrease compared with basal values, fulfilling the Miami Criteria for surgical success in use in our institution. Conclusion The utilization of autofluorescence for intra-operative identification of parathyroid glands may have a clinical application in surgery for primary hyperparathyroidism, being of special utility when ultrasonography and Sestamibi Scan are non concordant.

International audience; BACKGROUND:Mild primary hyperparathyroidism (serum calcium ≤ 2.85 mmol/L) is the most representative form of pHPT nowadays. The aim of this study was to evaluate its subtypes and the multiglandular disease (MGD) rate as it may lower the sensitivity of preoperative parathyroid scintigraphy and the surgical cure rate.METHODS:We retrospectively included patients with mild pHPT who underwent parathyroid dual-tracer scintigraphy with 99mTc-MIBI SPECT/CT and surgery between January 2013 and December 2015. Cure was defined as normalization of serum calcium (or PTH in the normocalcemic form) at 6 months. MGD was defined by either two abnormal resected glands or persistent disease after resection of at least one abnormal gland.RESULTS:We included 121 patients. Median preoperative serum calcium was 2.68 mmol/L and median PTH was 83.4 pg/mL. A total of 141 glands were resected (95 adenomas, 33 hyperplasias). The subtypes were 57% classic, 32.2% normohormonal, and 10.7% normocalcemic. MGD occurred in 23.5% of patients divided as 13%, 30%, and 64% respectively (p = 0.0011). The surgical cure rate was 85.2%. The normocalcemic form had lower cure rate than the normohormonal (45% vs 84%, p = 0.018) and classic forms (45% vs 93%, p = 0.0006). MIBI scintigraphy identified at least one abnormal lesion, later confirmed by the pathologist in 90/98 patients, making the sensitivity per patient 91.8% (95% CI 84.1-96.2%).CONCLUSIONS:MGD is strongly associated with mild pHPT, especially the normocalcemic form where it accounts for 64% of cases. Bilateral neck exploration should be performed in this population to improve the cure rate, even if the scintigraphy shows a single focus.

The most common cause of primary hyperparathyroidism (PHPT) is a solid parathyroid adenoma. Less than 2% of cases of PHPT are caused by cystic parathyroid adenomas formed from degeneration of pre-existing solid parathyroid adenomas. Cystic parathyroid adenomas are non-functional in over 90% of cases. In this case we describe management of a 56-year-old man with acute-onset polydipsia, polyuria, xerostomia, nausea, and constipation. Serum chemistry upon admission revealed hypercalcemia, hyperparathyroidism, and reduced serum phosphorus. Neck sonogram revealed a predominantly anechoic lesion later confirmed by pathology to be a cystic parathyroid adenoma in the right thyroid lobe. Sestamibi scan did not show uptake in parathyroid gland, and parathyroid hormone (PTH) was elevated in fine-needle aspiration sample. Otolaryngology removed the cystic lesion via surgical excision, which led to normalization of PTH level. This case demonstrates the importance of evaluation of cystic components for PTH levels and if confirmed should be treated as a parathyroid adenoma.

Introduction: Primary hyperparathyroidism (PHPT) is a rare condition in the pediatric population. Parathyroid carcinoma (PC) is a very uncommon cause of PHPT, accounting for < 1% of pediatric PHPT cases. It is challenging to distinguish between parathyroid adenoma (PA), the most common cause of PHPT, and PC. In this report, we described a young female who presented with a history of progressive limping and was finally diagnosed with PC. Case Presentation: A 15-year-old girl presented with progressive limping and bone pain for 8 years. She was referred by an orthopedic surgeon because of elevated intact parathyroid hormone (iPTH) for further evaluation. Physical examination revealed a large, firm, and non-tender neck mass, left hip tenderness, and limited range of motion. The initial biochemistry tests showed a borderline high calcium level of 10.8 mg/dl, an elevated iPTH level of 2876 pg/mL, and a decreased phosphorus level of 2.4 mg/dL. The 99mTechnetium (Tc) sestamibi scan displayed early intense activity in the right thyroid lobe persisting in the three-hour repeat scan, compatible with a parathyroid lesion. The patient underwent right-sided neck exploration and parathyroidectomy. Intraoperative and pathology findings confirmed the diagnosis of PC. Immunohistochemistry (IHC) staining revealed creatine kinase (CK) and CD31 in endothelial cells of the tumor. Ki67 staining was also positive in 2% - 3% of tumor cells. The whole exome sequencing (WES) study was negative for cell division cycle 73 (CDC73) and multiple endocrine neoplasia 1 (MEN1) genes. Conclusions: PC should be considered as a differential diagnosis of PHPT in the pediatric population, even in the presence of mild hypercalcemia.

Over the last three decades, preoperative imaging for hyperparathyroidism (HPT) has drastically improved. It is crucial to recognize that preoperative localization studies should only be utilized for surgical planning in patients with the confirmed diagnosis of HPT. The success of the primary operation still relies on the experience of the surgeon and his or her ability to interpret the information obtained from preoperative imaging studies within the context of the operative findings. As such, preoperative parathyroid imaging studies serve as adjuncts for the operating surgeon, who must recognize and understand the limitations of each of these modalities when planning and performing a parathyroid operation.

Multiple endocrine neoplasia 1 (MEN1) is a rare genetic syndrome characterized by the manifestation of tumors in endocrine glands most often in the parathyroid gland (PG). Treatment may involve several parathyroidectomies (PTX), especially in young patients, which increases the risk of postoperative complications. We present a 16-year-old patient with a family history of MEN1 syndrome. The patient started to show biochemical signs of hyperparathyroidism (HPT) and hypercalcemia at the age of 10. One and a half years later a PTX was successfully performed with removal of the two left PGs. However, a rise in plasma parathyroid hormone and ionized calcium was observed 4 years later. Preoperative noninvasive imaging with 99mTc-sestamibi scintigraphy showed no definitive parathyroid adenoma. A 11C-methionine position emission tomography combined with MRI (MET-PET/MRI) was then performed and detected a focus posterior to the lower part of the right thyroid lobe. Intraoperative angiography with fluorescence and indocyanine green dye was used to assess the vascularization of the remaining PGs. The lower right PG was removed. The patient was discharged with normalized biochemical values and without postoperative complications. Recurrence of primary HPT is frequent in MEN1 patients which often necessitates repeated operations. Our case report showed that the use of advanced noninvasive preoperative imaging techniques and intraoperative fluorescent imaging are valuable tools and should be taken into consideration in selected cases to avoid postoperative complications. To our knowledge, this is the first case where MET-PET/MRI has been used to detect parathyroid pathology. Learning points: MEN1 patients will develop parathyroid disease, which eventually will lead to surgical treatment with removal of the pathological glands. Preoperatively usage of MRI combined with PET tracers such as 11C-methionine and 18F-Fluorocholine are able to detect parathyroid pathology with a higher sensitivity than conventional imaging. Techniques using intraoperatively angiography with fluorescence and florescent dyes allow surgeons to verify the vascularization of each parathyroid gland. Optimization of noninvasive preoperative imaging techniques and intraoperative fluorescent imaging are valuable tools and should be taken into consideration when performing PTX consecutively in the same patient to avoid postoperative complications.

Summary Isolated, adult-onset central hypothyroidism is very rare, and its diagnosis can be challenging. A 42-year-old patient was referred for evaluation of a 2.8 cm thyroid nodule. She referred symptoms that could be attributed to hypothyroidism and thyroid tests showed low TSH and normal-low levels of free T4. However, evaluation of the remaining pituitary hormones and pituitary MRI were normal, yet a radionuclide scanning revealed that the thyroid nodule was ‘hot’ and the tracer uptake in the remaining thyroid tissue was suppressed. Interpretation of these studies led to a misdiagnosis of subclinical hyperthyroidism and the patient was treated with radioiodine. Soon after treatment, she developed a frank hypothyroidism without appropriate elevation of TSH and the diagnosis of central hypothyroidism was made a posteriori. Long term follow-up revealed a progressive pituitary failure, with subsequent deficiency of ACTH and GH. This case should alert to the possibility of overlooking central hypothyroidism in patients simultaneously bearing primary thyroid diseases able to cause subclinical hyperthyroidism. Learning points: Although rarely, acquired central hypothyroidism can occur in the absence of other pituitary hormone deficiencies. In these cases, diagnosis is challenging, as symptoms are unspecific and usually mild, and laboratory findings are variable, including low, normal or even slightly elevated TSH levels, along with low or low-normal concentrations of free T4. In cases with low TSH levels, the coexistence of otherwise common disorders able to cause primary thyroid hyperfunction, such as autonomous nodular disease, may lead to a misdiagnosis of subclinical hyperthyroidism.

Summary Distinguishing primary hyperparathyroidism (PHPT) from familial hypocalciuric hypercalcaemia (FHH) can be challenging. Currently, 24-h urinary calcium is used to differentiate between the two conditions in vitamin D replete patients, with urinary calcium creatinine clearance ratio (UCCR) 0.02 supportive of PHPT. A 26-year-old Caucasian gentleman presented with recurrent mild hypercalcaemia and inappropriately normal parathyroid hormone (PTH) following previous parathyroidectomy 3 years prior. He had symptoms of fatigue and light-headedness. He did not have any other symptoms of hypercalcaemia. His previous evaluation appeared to be consistent with PHPT as evidenced by hypercalcaemia with inappropriately normal PTH and UCCR of 0.0118 (borderline low using guidelines of >0.01 consistent with PHPT). He underwent parathyroidectomy and three parathyroid glands were removed. His calcium briefly normalised after surgery, but rose again to pre-surgery levels within 3 months. Subsequently, he presented to our centre and repeated investigations showed 24-h urinary calcium of 4.6 mmol/day and UCCR of 0.0081 which prompted assessment for FHH. His calcium-sensing receptor (CASR) gene was sequenced and a rare inactivating variant was detected. This variant was described once previously in the literature. His mother was also confirmed to have mild hypercalcaemia with hypocalciuria and, on further enquiry, had the same CASR variant. The CASR variant was classified as likely pathogenic and is consistent with the diagnosis of FHH. This case highlights the challenges in differentiating FHH from PHPT. Accurate diagnosis is vital to prevent unnecessary surgical intervention in the FHH population and is not always straightforward. Learning points: Distinguishing FHH from PHPT with co-existing vitamin D deficiency is difficult as this can mimic FHH. Therefore, ensure patients are vitamin D replete prior to performing 24-h urinary calcium collection. Individuals with borderline UCCR could have either FHH or PHPT. Consider performing CASR gene sequencing for UCCR between 0.01 and 0.02. Parathyroid imaging is not required for making the diagnosis of PHPT. It is performed when surgery is considered after confirming the diagnosis of PHPT.

Currently, neck ultrasound is the preferred preoperative imaging in patients with secondary/tertiary hyperparathyroidism, and the use of Tc-99m sestamibi scan is limited in these patients. We conducted this study to compare the diagnostic utilities of F-18 fluorocholine PET/CT, Tc-99m sestamibi scintigraphy, and neck ultrasound for localizing hyperfunctioning parathyroid glands in secondary/tertiary hyperparathyroidism. We prospectively enrolled 30 dialysis patients with a diagnosis of secondary/tertiary hyperparathyroidism; of these, 27 participants underwent all three imaging modalities, including dual-phase F-18 fluorocholine PET/CT (PET acquired 5 and 60 min after tracer injection), dual-phase Tc-99 m sestamibi SPECT/CT, and neck ultrasound. All patients underwent parathyroidectomy after imaging. We compared the lesion-based sensitivity, specificity, and accuracy of the three image tools using histopathology as the reference. A total of 27 patients (107 lesions) underwent all three imaging modalities and entered the final analysis. The lesion-based sensitivities of F-18 fluorocholine PET/CT, Tc-99m sestamibi, and ultrasound were 86%, 55%, and 62%, respectively (both p

The aim of this study is to evaluate the effectiveness of preoperative imaging localization of hyper functioning parathyroid glands and comparing it with operative neck exploration in secondary hyperparathyroidism. This study was performed in Minia University Hospital between January 2017 and May 2018. Twenty patients with secondary hyperparathyroidism were included in this study. Ultrasonography was done for all patients, and then we classify our patients into two groups: in group 1 patients, Technetium-99 m sestamibi scan and computed tomography were done preoperatively while in group 2, patients underwent operation with neck exploration directly without other preoperative localization procedures. Mean age in our cases was 45.2 ± 9.46, while the most affected age group was (40–49) years by (35%), then (30–39) years by (30%), then (50–59) years by (25%), and then (≥ 60) years by (10%), while females were more affected than male by (75%) of cases. CT sensitivity in detection of parathyroid disease was 70%, and sestamibi detects 80% while neck exploration detection sensitivity was 100%. Neck exploration is the only reliable localization procedure in the cases of secondary hyperparathyroidism.

Brown tumor is a bone lesion that arises in the setting of excess osteoclast activity in hyperparathyroidism. It consists of fibrous tissue, woven bone, and supporting vasculature, while contains no matrix. The characteristic of brown-colored lesion is a result of hemosiderin deposition into the osteolytic cysts. Two cases of young women aged 26 and 29 years old, respectively, are known with a history of end-stage renal disease (ESRD). Dialysis is performed two times/week over the last 7 years. Our patients presented with an intraoral mass of the hard palate since 12 months ago and decreased body height of 10 cm. The lesion causes difficulties in swallowing and talking. Laboratory workup showed elevated parathormone or PTH (3.391 pg/mL and >5.000 pg/mL). Neck ultrasound showed enlargement of the parathyroid glands. Supporting examination to diagnose brown tumor are neck ultrasound, CT of the neck, and parathyroid sestamibi scan. We performed parathyroidectomy. Pathology revealed hyperplasia of the parathyroid. The tumor regressed significantly within 2 weeks following the surgery, and we still observe tumor regression as well as reduction in PTH level. As clinicians, we should be alert to other possible causes of bony lesions. Clinical examination, laboratory finding, and imaging present important information to diagnose brown tumor.

Background Single parathyroid adenoma is the commonest cause of primary hyperparathyroidism. Localization of the affected gland preoperatively is a critical step in management. Surgery is considered as the main line of treatment for single parathyroid adenoma. Focused technique for parathyroid excision was found by many researchers to have a good success rate even without intraoperative parathyroid hormone measurement. Objective The aim of this study was to assess the feasibility, safety, and adequacy of focused parathyroidectomy via an open lateral approach using preoperative positive Sestamibi (MIBI) scan and/or ultrasound without utilizing any intraoperative aiding tools. Methods A case-series prospective analysis of focused open parathyroidectomy for 20 patients presented to the department of surgery or referred from the orthopedic department between October 2012 and January 2015 at the Faculty of Medicine, Al-Azhar University, Cairo, Egypt, diagnosed as sporadic hyperparathyroidism with either positive MIBI scan or ultrasound were done. Normalization of the postoperative levels of serum calcium and PTH was considered as a marker of success. Data entry and analysis were done using the IBM-SPSS version 22. Results In this study, focused minimal access parathyroidectomy was successful in 95% with no major surgical complications, i.e., recurrent laryngeal nerve palsy or permanent hypoparathyroidism. The patient's mean age was 46.5±12 years. The preoperative serum calcium level ranged from 8.8 to 15.2 mg/dL. The parathyroid hormone level was elevated ranging from 123-2000 pg/mL. In 90% of the cases, serum levels of alkaline phosphatase were elevated, while serum phosphorus was low with range 1.5-4.7 mg/dL. The left inferior parathyroid gland was the most commonly involved gland (40%). Using ultrasound and Sestamibi scan for preoperative localization was accurate in 75% and 90%, respectively. When both techniques were combined localization accuracy increased to 95%. Conclusion Our study has shown that focused parathyroidectomy is considered as a good option for single gland disease even if there are no available intraoperative helping aids, provided that good patient selection is undertaken.

Introduction Highly specific preoperative localizing test is required to select patients for minimally invasive parathyroidectomy (MIP) in lieu of traditional four-gland exploration. We hypothesized that Tc-99m sestamibi scan interpretation incorporating numerical measurements on the degree of asymmetrical activity from bilateral thyroid beds can be useful in localizing single adenoma for MIP. Methods We devised a quantitative interpretation method for Tc-99m sestamibi scan based on the numerically graded asymmetrical activity on early phase. The numerical ratio value of each scan was obtained by dividing the number of counts from symmetrically drawn regions of interest (ROI) over bilateral thyroid beds. The final pathology and clinical outcome of 109 patients were used to perform receiver operating curve (ROC) analysis. Results Receiver operating curve analysis revealed the area under the curve (AUC) was calculated to be 0.71 (P = 0.0032), validating this method as a diagnostic tool. The optimal cut-off point for the ratio value with maximal combined sensitivity and specificity was found with corresponding sensitivity of 67.9% (56.5-77.2%, 95% CI) and specificity of 75.0% (52.8-91.8%, 95% CI). An additional higher cut-off with higher specificity with minimal possible sacrifice on sensitivity was also selected, yielding sensitivity of 28.6% (18.8-38.6%, 95% CI) and specificity of 90.0% (69.6-98.8%, 95% CI). Conclusions Our results demonstrated that the more asymmetrical activity on the initial phase, the more successful it is to localize a single parathyroid adenoma on sestamibi scans. Using early-phase Tc-99m sestamibi scan only, we were able to select patients for minimally invasive parathyroidectomy with 90% specificity.

Background The objective of this study was to evaluate the performance of dual-energy computed tomography (DECT) for preoperative parathyroid tumor (PT) localization in primary hyperparathyroid (PHP) patients. Methods A retrospective review was carried out of the medical records of all PHP patients who underwent ultrasound (US), Tc-99m sestamibi noncontrast single photon emission computed tomography (CT-MIBI), DECT and parathyroidectomy at a single center. Results The sensitivities and accuracies for preoperative PT localization in the 97 patient study population were: US 40% and 93%, CT-MIBI 64.0% and 97%, and DECT 84% and 96%, respectively. In the one third of the study population that did not localize preoperatively with CT-MIBI and US, DECT correctly localized a PT in 21 cases (66%). DE-CT and US correctly localized a PT in 86% of cases, and only 5 (8%) of the cases that were accurately localized by a combination of CT-MIBI and US were not identified by DE-CT. Conclusions DECT should be utilized as a first line preoperative PT localization study in PHP patients, and is also a sensitive salvage localization test.

PURPOSE Patients with asymptomatic primary hyperparathyroidism might have a positive Tc-sestamibi parathyroid but do not meet criteria for surgical resection when the disease is diagnosed. However, many of these asymptomatic patients will become symptomatic or meet criteria for surgery later on. The aim of this study was to determine whether a positive sestamibi scan can precede future surgical eligibility in these patients. METHODS This retrospective study reviewed the records of 94 asymptomatic patients who had hyperparathyroidism and underwent Tc-sestamibi study with SPECT/CT imaging. Among them, 35 patients, including 12 with positive and 23 with negative sestamibi parathyroid scan, did not meet the criteria for surgery at the time of imaging, and follow-up record for at least 1 year was assessed on these patients. The imaging findings and the eligibility for surgery at the end of the follow-up were compared. RESULTS With a median follow-up of 2.4 years (range, 1-4 years), among all 35 patients who were initially not eligible for surgery, 6 of 12 patients with a positive sestamibi scan became eligible for surgery, whereas 20 of 23 patients with negative scans remained ineligible for surgery. CONCLUSIONS Positive parathyroid SPECT/CT predicted a higher possibility of meeting surgical criteria over time in asymptomatic hyperparathyroidism patients who are not surgery eligible at the time of presentation.

Objective We tested the hypothesis that a single-phase neck computed tomography (CT) is not inferior to multiphase neck CT (MPNCT), ultrasound, or nuclear medicine sestamibi scan in identifying parathyroid adenomas (PAs). Methods A total of 29 patients who had an MPNCT for the evaluation of a PA were identified; 11 patients met the inclusion criteria. During the 30-second arterial phase CT (APNCT), a normalized Hounsfield unit of suspected PA was compared to Hounsfield unit of a normal-appearing level I and a level II lymph node. A PA was defined as a lesion with a ratio of greater than 1.4 when compared to the level I and level II lymph node. This cutoff was determined based on the normalized ratios between level II and level I lymph nodes. Results of intraoperative parathyroid hormone assays and surgical pathology were used to validate the accuracy of this technique. Results The sensitivity of this method in APNCT is 90.9% whereas positive predictive value is 100%. The sensitivity or positive predictive value of ultrasound and nuclear medicine are 60% or 100% and 90% or 100%, respectively. Conclusion The sensitivity of APNCT is equivalent to that of MPNCT in identifying PA.

Introduction: MIBI scintigraphy is commonly being used for the preoperative localization of parathyroid adenomas. Multiple studies showed MIBI uptake in pituitary adenomas are likely due to higher metabolic activity. When hyperfunctioning pituitary adenomas were reported, both had CD [1,2]. We present the third case of increased pituitary uptake on a MIBI scan later confirmed as CD. CaseA 64-year-old Caucasian female s/p renal transplantation for RPGN who presented for evaluation of hypercalcemia. Evaluation confirmed primary hyperparathyroidism with persistently elevated PTH levels 74-108 pg/ml (11-68), serum calcium levels 10.0-10.4 mg/dl (8.4-10.3), albumin 4.1-4.3 g/dl (3.6-5.1), phosphorus 3.0-3.2 mg/dl (2.5-4.5), creatinine 0.94-1.07 mg/dl. 24-hour urine calcium 60 mg/day (35-250). Vitamin D-25 OH level was 37 ng/dL (30-100). A sestamibi scan showed uptake in the right lower parathyroid, the midsternal chest region and the pituitary gland. MRI of the pituitary revealed a 7mm cystic pituitary microadenoma in the right posterior pituitary. CD was confirmed by the findings of persistently elevated 8 AM serum cortisol levels of 28.4 and 24.2 mcg/dl (4-22), ACTH levels of 59 and 39 pg/ml (10-48), and an elevated plasma free cortisol of 1.43 mcg/dl (0.07-0.93). CT of the abdomen showed L adrenal thickening suggesting adrenal hyperplasia from CD. Plasma cortisol suppressed to 1.2 mg/dl following 1 mg of dexamethasone. 24 urine for free cortisol 26.7 mcg/day (4-50). The patient had no proximal muscle weakness, striae or Cushingoid facial features. She had no hyperglycemia or hypertension. Patient was diagnosed with an ACTH secreting pituitary microadenoma with mild CD and adrenal hyperplasia. Her DXA scan showed osteoporosis. Genetic testing for MEN1 mutation was negative. Patient did not wish surgery for either her hyperparathyroidism or her CD and is being evaluated for medical treatment of hypercortisolism. Conclusion: There are two prior case reports of an incidentally discovered pituitary adenoma on sestamibi scan later diagnosed as CD [1,2]. Corticotrophs may have a strong affinity for sestamibi. Our case is the first, to our knowledge, of pituitary MRI confirmation of the ACTH secreting pituitary incidentaloma initially suspected by pituitary uptake on a sestamibi scan in a patient with hyperparathyroidism. Reference1. Kuhadiya ND et al. Incidentally Discovered ACTH-Secreting Pituitary Adenoma on a Sestamibi Scan in a Patient With Hyperparathyroidism. AACE Clinical Case Reports. 2015;1(3):e152-5. 2. Gierach M et al. The case of Cushing’s disease imaging by SPECT examination without manifestation of pituitary adenoma in MRI examination. Nuclear Medicine Review. 2005;8(2):137-9.

Introduction: Presentation of hyperparathyroidism varies and is highly non-specific. The automated calcium analyzer has made the diagnosis easy. Similarly, the advent of Sestamibi scan has paved the way to minimally invasive parathyroidectomy indicated for parathyroid adenoma. There is no uniformity in the extent of minimally invasive parathyroidectomy that is done through limited incision under radio or sonar guidance and endoscopically. In this study, we are presenting the focused parathyroidectomy performed under sonar guidance and superficial cervical block (SCB). The prerequisite is concordant preoperative Sestamibi and ultrasound imaging. Objective: To reflect on the result of sonar-guided focused parathyroidectomy under SCB. Method: A two-year review of parathyroidectomies performed between January 2013 and December 2014. Result: There was good correlation between the pre-operative imaging, the intra-operative findings and the postoperative histology result of the 15 cases analysed. Conclusion: The focused parathyroidectomy under SCB yielded a good result with concordant preoperative Sestamibi and ultrasound findings.

Background The role of preoperative localization studies in patients with hyperparathyroidism and expected multigland disease remains poorly defined. Our study investigates the usefulness of obtaining preoperative sestamibi scans and ultrasonography of the neck in identifying ectopic glands in this group of patients. Methods Under Institutional Review Board approval, we performed a retrospective review of patients who underwent operation for secondary hyperparathyroidism, tertiary hyperparathyroidism, lithium-induced hyperparathyroidism, and multiple endocrine neoplasia syndrome at a tertiary institution between 2004 and 2015. We reviewed patient demographics, laboratory, radiology, pathology, and operative reports. Results Of 2,975 parathyroidectomies performed during this period, 154 operations were performed in 149 patients who met the criteria. Of the 149 patients, 82 (55.0%) had secondary, 31 (20.8%) had tertiary, 23 (15.4%) had lithium-induced HPT, and 13 (10.1%) had multiple endocrine neoplasia syndrome; 86 ectopic glands were identified in 64 patients (43.0%). Sensitivity for identification of ectopic glands was 29% for sestamibi scan and 7% for ultrasonography, while 89% of mediastinal glands were localized by sestamibi scans and thoracotomy, thoracoscopy, or sternotomy occurred in 4.7% of patients. Conclusion We found a greater rate of preoperative localization of ectopic glands than reported previously. Because the sensitivity of sestamibi for identification of ectopic glands is 23.0%, the implication of missing mediastinal glands warrants preoperative imaging.

Summary Parathyroid carcinoma is an extremely rare endocrine malignancy that accounts for less than 1% of cases of primary hyperparathyroidism. We report a 44-year-old woman who presented with fatigue and diffuse bone pain. Laboratory findings revealed highly elevated serum calcium and parathyroid hormone (PTH) levels and a 4.5 × 3 × 2.5 cm cystic lesion in the lower pole of the right thyroid lobe that was shown histologically to be a parathyroid carcinoma. Ten years later, the patient developed brain and pulmonary metastases and recurrence of PTH-related hypercalcemia. Treatment of hypercalcemia along with localized radiotherapy and various chemotherapy regimens failed to induce a biochemical or radiological response. In conclusion, parathyroid carcinoma is a rare neoplasia that may develop metastases even after prolonged follow-up, for which there is no evidence-based treatment besides surgery. Different chemotherapeutic schemes did not prove to be of any benefit in our case highlighting the need for registering such patients to better understand tumor biology and develop specific treatment. Learning points: Metastases can develop many years after parathyroid cancer diagnosis. Surgery is the only curative treatment for parathyroid carcinoma. Chemotherapy and radiotherapy prove to be ineffective in parathyroid cancer treatment. Patient registering is required in order to delineate underlining pathology and offer specific treatment.

Accurate localization of parathyroid adenomas allows for minimally invasive parathyroidectomy. This results in a shorter length of stay and increased patient satisfaction. Preoperative Technetium (99mTc) sestamibi scans accurately localize parathyroid adenomas in 70 to 85 per cent of cases. If a patient has a negative scan, it is logical to believe that with a preoperative sestamibi injection, the gamma probe may fail to help find an adenoma. We hypothesized that the gamma probe would not be useful intraoperatively for patients with primary hyperparathyroidism (PHPTH) and a negative sestamibi scan. We retrospectively reviewed the cases of parathyroidectomy at our institution from 2010 to 2016. We selected patients with PHPTH and negative sestamibi scan. In all cases, an attempt was made to find adenomas intraoperatively with the gamma probe. A frozen section was obtained as well as intraoperative parathyroid hormone levels to confirm removal of hyperfunctioning parathyroid tissue. There were 132 parathyroidectomies of which 22 had PHPTH and a negative sestamibi scan. One case was excluded because of insufficient documentation of the intraoperative use of the gamma probe. In 19 of the 21 patients analyzed, the gamma probe successfully identified the adenoma in the operating room (sensitivity, 90.5%). In two patients, the gamma probe did not aid in localization. There were no false positives. In all cases, the parathyroid resected was confirmed by frozen section. The intraoperative parathyroid hormone levels dropped >50 per cent in all but three cases, two of which corresponded to those cases where the gamma probe did not help. Even in patients with negative sestamibi scans, intraoperative use of the gamma probe after preoperative sestamibi injection is effective in localizing parathyroid adenomas.

Objective We review our experience with focused neck exploration (FNE) based on ultrasound (USN) alone, in patients with primary hyperparathyroidism (PHP) and negative sestamibi scans (SES). Methods 124 patients with PHP were evaluated for FNE and studied with SES and USN. 53 patients (43%) had a negative SES. 49 of those patients (92%) were selected for FNE based on USN criteria of a single abnormal gland, .5 cm or greater, as determined by an experienced surgeon-sonographer. Results 40 of 49 patients (82%) selected on USN criteria alone underwent successful FNE. Of the 9 patients who required neck exploration 6 (12%) were incorrectly localized, 2 (4%) were found to have hyperplasia, and 1 (2%) was correctly localized but not found on the initial exploration. US had a 97.5% sensitivity and a 25% specificity as a diagnostic test for single adenomas, both within the 95% confidence interval. Of those glands successfully imaged by USN, image size correlated well with the measured size of the adenoma at path, on average within 0.40 cm (Standard deviation 0.30 cm). Conclusion We feel our data show that USN provides reliable information in patient selection for FNE. The literature supports SES as a more reliable study when positive for parathyroid adenoma, but false negative rates of 30–40% have been reported. Routine use of preoperative US by experienced surgeon-sonographers would result in more patients selected for FNE vs. routine neck exploration with expected benefits.

The two types of parathyroid cysts are functional and non-functional cysts. Cystic parathyroid lesions are a rare cause of hypercalcemia and often pose a diagnostic challenge due to the reduced detection on preoperative imaging studies. We, herein, present a rare case of an elderly female presenting to the emergency department with altered mental status associated with hypercalcemic crisis and a negative sestamibi scan. Following surgical resection, pathology revealed the diagnosis of cystic parathyroid adenoma and normalization of serum calcium levels.

Successful identification of an enlarged parathyroid gland (or at least a dominant, normal-size gland)—the only gland of interest 80% of the time—directs the initial approach for minimally invasive surgery. Identification of additional enlarged parathyroid glands (double adenomas or hyperplasia) prior to surgery allows the surgeon to plan a four-gland exploration but is a tougher radiologic task. Identification of a possible ectopic mediastinal gland (sometimes a supernumerary gland) is essential knowledge for the surgeon no matter what the planned surgery. Contrast CT scan supplemented by ultrasound can provide an excellent set of initial screening studies though there are difficulties in separating the enlarged parathyroid glands from abnormal thyroid tissue. Sestamibi scan is helpful in post surgical cases.

Parathyroid carcinoma is a rare malignant neoplasm of the parathyroid gland accounting for approximately 0.5–1% of patients with primary hyperparathyroidism. Because of the rarity of this cancer, the diagnosis is difficult to establish. The diagnosis could be suspected from findings of a very high serum calcium level usually higher than 14–16 mg/dL with corresponding elevation of serum parathyroid hormone level 10–15-fold higher than the normal range. The 5- and 10-year survival rates for patients with parathyroid carcinoma are 78–85% and 49–70%, respectively. There are no good imaging studies that can clearly establish the diagnosis of parathyroid carcinoma, but some imaging modalities such as ultrasound and CT scan can be helpful in the initial evaluation of patients with a suspected diagnosis of parathyroid carcinoma. Sestamibi scans can also help in localization.

The four cases seen in Figs. 5.1, 5.2, 5.3, and 5.4 illustrate images showing right inferior parathyroid adenomas—masses that ultrasound shows to be located posteriorly and inferiorly to the inferior pole of the right thyroid lobe. When Tc99m sestamibi SPECT/CT scans are used, the scintigraphic findings are compatible with a parathyroid adenoma at the level of the lower pole of the right lobe of the thyroid gland. On immediate images a focus of increased tracer activity can be seen at the level of the lower pole of the right lobe. The 3-hour delayed images show a persistent focus of increased tracer activity at the lower pole of the right lobe, which is compatible with a parathyroid adenoma.

Derived from glands with rich capillary networks, hyperplastic parathyroid glands and parathyroid adenomas are hypervascular and have a vascular stain on arteriography and prominent enhancement on CTscan. Multiple studies have shown excellant contrast CT scan detection of enlarged parathyroid glands and adenomas as initial screening study and in cases of failed sestamibi studies. The hypervascularity is also identifiable on color Doppler ultrasound and adds specificity to the ultrasound diagnosis of enlarged parathyroid glands.

Figures 6.1, 6.2, 6.3, 6.4, and 6.5 show images from five cases of left superior parathyroid adenomas—masses that ultrasound shows to be located posteriorly to the superior pole of the left thyroid lobe. When sestamibi scans are used, in some cases persistent tracer activity within each lobe of the thyroid gland severely limits evaluation for a parathyroid adenoma, even on 3-hour delayed images. When viewed in light of the ultrasound findings, however, a very mild persistent focus of increased tracer activity may be identified. More accurate testing to detect a parathyroid adenoma would consist of a SPECT/CT sestamibi scan (rather than a regular sestamibi scan) in combination with a dedicated parathyroid ultrasound study or 4D CT scan.

Minimally invasive parathyroidectomy guided by noninvasive localization studies has become the standard surgical approach to primary hyperparathyroidism. About 80% of the time, primary hyperparathyroidism is due to a solitary parathyroid adenoma, so accurate identification on screening studies will usually result in the desired limited surgery, verified by a drop of intraoperative parathormone level. The limited surgery may be converted to a four-gland exploration if the intraoperative parathormone does not drop. Four-gland exploration will be planned if multigland disease is diagnosed (or highly suspect) or if all studies are negative. If a mediastinal adenoma is diagnosed and no enlarged parathyroid gland is identified in the neck, mediastinal surgery is performed before a neck exploration. Ultrasound, sestamibi scan, contrast CT scan, and MRI are the noninvasive modalities available to find the starting point in evaluating patients with clinically diagnosed primary hyperparathyroidism.

A left inferior parathyroid adenoma—a mass identified as adjoining the inferior pole of the left thyroid lobe—can be identified through imaging with ultrasound and Tc 99 m sestamibi SPECT/CT scans, as illustrated in the four cases seen in Figs. 7.1, 7.2, 7.3, and 7.4.

A right superior parathyroid adenoma—a mass identified as adjoining the superior pole of the right thyroid lobe—can be identified through imaging with ultrasound and Tc 99 m sestamibi SPECT/CT scans, as illustrated in the four cases seen in Figs. 4.1, 4.2, 4.3, and 4.4.

This chapter presents eight cases of intrathyroidal parathyroid adenoma (Figs. 8.1, 8.2, 8.3, 8.4, 8.5, and 8.6) or cystic parathyroid adenoma (Figs. 8.7 and 8.8), which are all identified using ultrasound and SPECT/CT imaging.

Rare parathyroid lesions: (1) Cystic parathyroid lesions are uncommon. The cyst may be functional (with the presence of clinical primary hyperparathyroidism) or nonfunctional. Most functional parathyroid cysts are cystic degeneration or hemorrhage into a parathyroid adenoma derived from superior or inferior glands. Nonfunctional cysts, which are quite rare, are derived from inferior parathyroid glands and may be found in the mediastinum. (2). Parathyroid carcinoma is very rare but may be diagnosed if there is clear invasion of adjacent structures. Carcinoma should be considered if the adenoma is quite large, has calcification and has irregular, poorly defined margins. (3) Parathyroid lipoadenoma is a rare lesion consisting of nests of parathyroid parenchyma (chief cells and some oncocytic cells) in mature adipose tissue. These have predominantly fat character on CT and ultrasound and require a positive sestamibi scan for identification. (4) Parathyromatosis may result from seeding of cells during surgery or overgrowth from embryologic parathyroid rests. In postsurgical setting sestamibi scan is essential to distinguish small nodules of parathyroid tissue from scar. (5) Water-clear cell hyperplasia and adenomas is a very rare entity of proliferated vacuolated water-clear cells, which have abundant cytoplasmic glycogen. The glycogen content may give rise to unusual CT and ultrasound features.

Background The purpose of this study was to determine the expression profile of several biomarkers in sestamibi-positive (n = 23) and sestamibi-negative (n = 6) parathyroid adenomas. Methods A tissue microarray of parathyroid adenomas from 29 patients was constructed and slides were stained for several proteins involved in angiogenesis, inflammation, cell adhesion, cell cycle, apoptosis, and with markers of the sonic hedgehog, mTOR, Forkhead box O and WNT signal transduction pathways. Protein expression was determined using an image-analysis software (Spectrum Plus©, 38 Aperio). Results Protein expression analysis revealed that the vascular endothelial growth factor receptor 2 (VEGFR2) score was significantly higher in the sestamibi-positive cohort compared to sestamibi-negative adenomas (P = .038). Other proteins were not differentially expressed between sestamibi-positive and sestamibi-negative adenomas. Conclusion It is hypothesized that VEGFR-2 overexpression in parathyroid adenomas increases vascular permeability resulting in a higher uptake of sestamibi.

Objectives The use of intraoperative parathyroid hormone (ioPTH) monitoring has been recommended as an adjunct to improve likelihood of successful parathyroid surgery. We sought to compare the surgical outcomes as well as operative times and associated costs in patients with and without ioPTH in the setting of co-localizing studies for primary hyperparathyroidism. Methods A retrospective review was conducted of patients undergoing surgery for nonfamilial primary hyperparathyroidism at a single institution from January 2013 to April 2019. Patients with a suspected single adenoma that co-localized on sestamibi and ultrasound were included resulting in 141 patients, of which 110 (78%) had ioPTH testing (group 1) while 31 (22%) did not (group 2). Time driven activity-based cost (TDABC) analysis was performed incorporating physician and staff compensation and anesthetic usage to determine the cost differential between groups. Study Design Retrospective chart review. Results In group 1, the use of ioPTH prompted re-exploration in 2 patients (1.8%). In both cases, the initial gland identified was normal parathyroid and persistent elevation of ioPTH resulted in additional dissection and removal of the adenoma. All but two group 1 patients had normal post-operative calcium levels at last follow up for an overall success rate of 97.3%. One group 2 patient (3.2%) was found to have recurrent hyperparathyroidism 33 months later with an adenoma identified in the same location as the initial excision. Otherwise all group 2 patients were eucalcemic at last follow-up for an overall success rate of 96.8%. The average time for the procedure in group 1 was 91.7 ± 28.1 minutes compared to 52.3 ± 16.9 minutes in group 2 (p Conclusions In patients with co-localizing sestamibi scan and ultrasound and concordant operative findings, the use of ioPTH did not improve the success rate of minimally invasive parathyroid surgery, while increasing operative times and cost.

Introduction: Parathyromatosis is a rare cause of recurrent hyperparathyroidism in which multiple nodules of hyperplastic parathyroid tissue are scattered throughout the neck and superior mediastinum. It is proposed to be a result of spillage and seeding of parathyroid tissue during parathyroid surgery. It presents as recurrent or persistent PTH mediated hypercalcemia. Management of Parathyromatosis is challenging and frequently refractory to surgical intervention; hence there is a need for an effective medical treatment. Case: 34 year old female was diagnosed with hyperparathyroidism in 2003 at the age of 16 years. She presented with fatigue, muscle weakness, bone pain and markedly elevated calcium and PTH levels. Sestamibi scan showed parathyroid adenoma with a cystic lesion. She underwent surgical removal, which was complicated by parathyroid cyst rupture. However, the patient remained asymptomatic for 4 years of follow-up. Then in 2007, she had a recurrence of the same initial presentation with laboratory values confirming relapse of hyperparathyroidism. A nuclear parathyroid scan showed remnant parathyroid tissue inferior and posterior to the right thyroid lobe along with multiple subcutaneous nodules. Neck exploration was performed, and histopathological examination showed multiple nodules of hyperplastic parathyroid tissue and cystic changes consistent with the diagnosis of Parathyromatosis. Attempts at satisfactory surgical resection failed, and the patient had recurrent replaces with multiple hospitalizations for nephrolithiasis and had undergone 4 more surgical interventions for remnant parathyroid tissue in a span of 10 years (2008–2018). We started the patient on cinacalcet in August 2018 with an initial dose of 60 mg/day that was gradually increased to 180 mg/day, besides vitamin D analog. This helped in achieving a partial but not full control of calcium levels, so we decided to add Denosumab in April 2019 at the dose of 60 mg SC every 6 months. Dexa scan was done before starting treatment and showed a normal Z score. This regimen has successfully maintained a stable calcium level with normal corrected calcium, and our patient has been in remission with no recurrence of renal stones or other symptoms for the past 18 months, with the last follow-up showing a calcium level of 2.64 mmol/l (2.2–2.65), PTH of 23 pmol/l (1.1–8.43), vitamin D of 24 ng/ml (20–32), albumin of 38 g/L (35–52) and creatinine of 58 umol/l (49 - 90), compared to calcium level of 2.93 mmol/l, PTH of 50 pmol/l and Vitamin D 13 ng/ml before starting treatment. Conclusion: Although it is a rare condition, Parathyromatosis should be kept as a differential diagnosis in the event of recurrent hyperparathyroidism. The Addition of Denosumab to Cinacalcet can make a big difference in achieving a successful medical treatment of the condition, therefore avoiding the detrimental effects of long-standing hypercalcemia.

Background: It has been debated that "remote access endoscopic parathyroidectomy" requires extensive dissection, thereby, increasing the risk of complications. Hence, the aim of this study is to assess the feasibility and safety of remote access endoscopic parathyroidectomy via the unilateral axilla-breast approach (ABA) and transoral vestibular approach (TOEPVA). Method: The study includes 17 patients with primary hyperparathyroidism undergoing endoscopic parathyroidectomy from January 2016 to December 2020. Of these, 11 patients underwent parathyroidectomy via unilateral ABA and 6 had TOEPVA. Preoperatively, ultrasonography of neck, sestamibi scan and CT scan neck were done to localise the diseased gland. Post operatively, Serum calcium and parathyroid hormone levels were monitored.Results: The mean age of patients was 35.6±10.5 years. Except one, all the patients were females. The mean operative time of the unilateral ABA and TOEPVA group was 93.67±28.64 minutes and 138.6±31 minutes respectively. Except for three patients, in all the patients, the parathormone levels normalised. One patient had hungry bone syndrome postoperatively. The mean hospital stay of the unilateral ABA and TOEPVA group was 3±1.5 days and 4±3.09 days respectively. One patient had post-operative transient recurrent laryngeal nerve paresis. Seroma and surgical emphysema were seen in two patients each. Conclusions: Remote Access Endoscopic parathyroidectomy may be considered safe in treatment of parathyroid tumours. Precise preoperative localisation is mandatory. In addition to a magnified view and a better illumination, the potential advantages of endoscopic techniques are better cosmetic results, decreased hospital stay and better patient comfort.

Background The routine use of intraoperative parathyroid hormone assay (IOPTH) in minimally invasive parathyroidectomy (MIP) for primary hyperparathyroidism has been questioned. In this study, we selectively employed IOPTH only to cases which did not show concordant results in Sestamibi Scan, ultrasound, and operative findings. Method 100 patients received MIP with selective use of IOPTH for primary hyperparathyroidism from March 2006 to June 2015 (“Selective IOPTH” group), were compared to a historical cohort of 57 patients who received MIP with mandatory IOPTH between Mar 2002 and Feb 2006 (“Mandatory IOPTH” group). For the Selective IOPTH group, all cases had preoperative Sestamibi Scan and ultrasound performed; where there were concordant results in Sestamibi Scan, ultrasound, and operative findings, IOPTH would not be performed. The surgical cure rate and operative time of the 2 groups were compared. Results We found no significant difference in the surgical cure rate between the “Selective IOPTH” group and the “Mandatory IOPTH” group. (Short term 6 months postoperative cure rate: 99vs.100%, respectively, p = 1.0; long term cure rate: 99.0 vs. 98.2%; p = 1.0 ) However, operative time was significantly shorter in the “Selective IOPTH” group when compared with the “Mandatory IOPTH” group. (64vs.92 min, respectively; p

Background: Primary hyperparathyroidism remains one of the leading causes of hypercalcemia in the world today. To plan the appropriate surgery with the least morbidity for the patient, preoperative localization of the abnormal parathyroid gland is necessary. Tc99m sestamibi scintigraphy and ultrasonography are the two main modalities for imaging the parathyroid glands. Patients and methods: This prospective interventional non-randomized non-controlled study was conducted in the Department of General Surgery at Ain Shams University hospitals in the period from April 2013 to October 2016 on thirty patients (7 males and 23 females) with primary hyperparathyroidism. Informed consent was obtained from all patients included in the study. Results: Out of thirty patients, the combined preoperative ultrasonography and Tc99m sestamibi scintigraphy was accurate in twenty eight patients (93.33%) while they could not identify the pathological gland in two patients (6.66%). Accuracy of ultrasonography alone was 76.66% (23 patients), while accuracy of T99m sestamibi scan alone was 86.66% (26 patients). Conclusion: The preoperative combination of ultrasonography and Tc99m sestamibi scintigraphy is effective with high accuracy in predicting the location of abnormal parathyroid gland(s) in patients with primary hyperparathyroidism.

Aim To evaluate the use of intraoperative gamma probe survey (IGPS) and frozen section (FS) in the surgical treatment of parathyroid adenoma (PA) in children. Patients and methods Children operated for PA between 2000 and 2015 were evaluated for age, sex, clinical features, presenting symptoms, diagnostic methods and results of surgical treatment retrospectively. Results Seven patients operated for PA were included in the study. The mean age of the patients was 14.58years (11–17years) and male to female ratio was 4:3. Presenting symptoms were non-specific such as fatigue, weakness and abdominal pain ( n =4). Six patients (85.8%) had adenoma of single parathyroid gland whereas remaining patient has involvement of two glands (14.2%). Localizations of adenomas were left inferior ( n =3, 42.8%), right inferior ( n =2, 28.3%), right superior ( n =1, 14.2%), left superior ( n =1, 14.2%) and intrathymic ( n =1, 14.2%). In case with involvement of more than one gland, adenomas were localized at right superior/left inferior glands. The mean longest diameter of the adenomas were 14.8mm (4–23mm) and all of the cases were underwent USG and Tc-99m sestamibi scan. During surgical excision, FS were used in 5 cases, and 4 cases had IGPS after preoperative radioactive substance injection. In one patient, injection was performed directly into the adenoma and 3 patients had systemic injection. Patient who had injection directly into the involved gland had positive gamma counts in the adjacent tissues because of local spread of radioactive substance and diagnosis was also confirmed with FS. In two of the cases, adenoma excision was performed without FS with only IGPS. None of the patients had preoperative and postoperative complications. Conclusion PA is rare in children and appropriate gland excision can be obtained also by the help of IGPS after injection of radioactive substance, and frozen section examination as well as intraoperative parathormone (PTH) screening. IGPS is a helpful tool to define the target tissue during surgery in order to avoid extensive dissection. Since direct injection of involved gland may cause accumulation of radioactivity in the adjacent tissues, surgeons must be aware of advantages and disadvantages of different radiolabeling techniques.

Objectives/Hypothesis The prevalence of multiglandular disease (MGD) of the parathyroid has been reported to be higher in patients with primary hyperparathyroidism and low baseline intact parathyroid hormone (PTH) levels (

Objectives: In primary hyperthyroidism Tc-99m Sestamibi (MIBI) scanning is commonly used for localization of abnormal parathyroid gland and the reported sensitivity is very high. However, false negative scan remain a problem. We examined whether serum calcium and parathormone (PTH) level have any impact in the sensitivity of MIBI scan.Study design: A retrospective review of 55 patients with primary hyperparathyroidism who underwent MIBI scan from January 2012 to December 2014 were included in this study. All patients underwent parathyroid surgery followed by histopathological confirmation.Results: In total, 55 patients were studied. Mean age was 41.3 ± 19.8 years (range 21-63 years). MIBI scan was true positive in 37 cases and false negative in 18 cases. The sensitivity of MIBI scan was 67.2 %. Mean serum calcium level was 12.3 mg/ dL. More than 62.2 % of patients with calcium level greater than 12.3 mg/dL had a positive scan as compared with 37.8% of those with lesser value (P