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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.

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

Summary A 55-year-old female was referred with abnormal thyroid function tests (TFTs); the free thyroxine level (FT4) was undetectable 100 mIU/L respectively at 24 h, The FT4 however remained undetectable throughout. Being on thyroxine 100 µg/day for one month, her FT4 level increased to 15 pmol/L and TSH fell to 0.08 mIU/L. Four years earlier at another hospital, her FT4 level had been low (6.8 pmol/L) with a normal TSH and a raised Tc-99 uptake of 20% (normal Learning points: This is a previously unreported form of dyshormonogenetic goitre. This goitre progressively loses its ability to synthesize T4 but not T3. The inability to synthesize T4 was demonstrated by giving rhTSH.

Introduction: Preoperative localization modalities for patients with primary hyperparathyroidism today play an important role in clinical decision making, surgical procedure, and the prognosis of patients. One of the most common preoperative imaging modalities is the sestamibi scan labeled with Technetium-99m, which is capable of determining the location of parathyroid lesions in primary hyperparathyroidism patients with high sensitivity. Methods: In this cross-sectional study, 25 patients with primary hyperparathyroidism, referred to endocrine clinic of Tabriz University of Medical Siences during 2016-2018 were enrolled. All patients underwent a preoperative Sestamibi scan. Comparing the results of the scan with the surgical findings as a standard gold method for diagnosis, the diagnostic value of the scan was evaluated for the localization of parathyroid lesions. Results: According to the items observed in surgery and pathologic findings, the sensitivity of Sestamibi scan for the localization of parathyroid lesions was 84.6%. Specificity and positive predictive value and negative predictive value were 95.6%, 89.6%, and 94.8%, respectively. A significant relationship was observed between the type of lesion and the sensitivity of Sestamibi scan (p=0.002). Conclusion: Sestamibi scan has high sensitivity and diagnostic and therapeutic value for patients with primary hyperparathyroidism. However, the existence of other pre- and intraoperative localization modalities, at the request of surgeons, is useful and warranted to reduce the rate of recurrent surgery as well as to minimize false negatives.

Persistent primary hyperparathyroidism is commonly caused by an ectopic parathyroid adenoma. The incidence of ectopic parathyroid tissue at carotid bifurcation is rare. Knowledge of embryogenesis is essential in localising and resecting these lesions. A 31-year-old gentleman with persistent primary hyperparathyroidism following previous negative bilateral neck exploration (BNE) and sestamibi scan was referred to a tertiary specialist head and neck unit. He was found to have an ectopic parathyroid gland adjacent to the right internal carotid artery after specialist localisation studies with four-dimensional computed tomography (CT), and head and neck multidisciplinary discussion. Surgical exploration of the neck revealed a fusiform swelling adherent to the vagus nerve juxtaposing the right internal carotid artery. The lesion was excised and histology confirmed a 9-mm parathyroid adenoma. The patient recovered uneventfully with normalisation of serum calcium and parathyroid hormone (PTH), with no complications. This case highlights the importance of understanding the embryogenesis and thereby potential ectopic locations of parathyroid glands. It also reflects the clinical importance of dedicated specialist head and neck unit comprising of radiologists, surgeons and pathologists in managing rare ectopic parathyroid adenomata following previous surgical exploration alongside negative sestamibi scintigraphy.

Background Parathyroidectomy for patients with secondary hyperparathyroidism (sHPT) generally requires a four-gland exploration. Some groups have strongly recommended routine preoperative Tc-99m-sestamibi scans; others practice scanning for only selected patients. To determine the utility of sestamibi scans in this patient population, we reviewed our experience. Methods We performed a retrospective review of patients who underwent parathyroidectomy for sHPT by one surgeon between 2000 and 2018. Data reviewed included patient demographics, laboratory results, pathology and radiology reports, and clinical and operative notes. Results Of the 72 patients in the cohort, mean age was 47.2 ± 15.6, and 50% were female. The preoperative mean calcium and parathyroid hormone levels were 9.6 ± 1.1 mg/dL and 1192.1 ± 914.1 pg/mL, respectively. Sestamibi scans were performed in 21 patients (29%). Of these, 17 were reoperative cases. Of all sHPT patients, 27.8% had ectopic glands. In the sestamibi cohort, only four patients had ectopic glands identified on the scan. Among the 51 patients without preoperative imaging, 16 had ectopic glands (26.2% of nonimaged patients). All these 16 ectopic glands were found by the surgeon at the time of operation without the need for preoperative imaging. All patients in the series were cured with a minimum follow-up of 6 mo. Conclusions Ectopic parathyroid glands are commonly seen in patients undergoing parathyroidectomy for sHPT. The majority of ectopic glands were successfully identified during the operation without preoperative sestamibi scan. Therefore, routine preoperative Tc-99m-sestamibi scans are not needed for successful parathyroidectomy for sHPT.

Background: Ectopic parathyroid glands can present in a challenging location in the superior posterior mediastinum. Methods: Two patients with primary hyperparathyroidism were operated on for ectopic paraesophageal parathyroid glands in the superior posterior mediastinum. Sestamibi scan, computed tomography (CT) scan, and photon emission CT were used to identify the exact location of these glands. We describe a minimally invasive resection using a three-arm robotic-assisted thoracoscopic technique. Results: Both lesions were completely resected with using the port-based robotic approach with expedited recovery. There was no perioperative morbidity. Patient had low postoperative pain scores and improved symptomatically. Conclusion: Robotic approach for resection of superior posterior mediastinal parathyroids is safe and effective in this challenging operative location.

Background: The association of thyroid disease and primary hyperparathyroidism is well described, with thyroid carcinoma being reported in 2-15% of cases. The most commonly associated carcinoma is non-medullary thyroid cancer. While the association of PHPT and medullary thyroid cancer (MCT) is well known, that of NMTC, despite its increasing incidence, is still not established. Our study is a review of incidence and underlying mechanisms of non-medullary thyroid cancer associated with PHPT. Also, best imaging tools for concomitant diagnosis is reviewed to ensure an adequate plan of care. Methods & findings: A search was done using two databases: Medline & Embase. The search conducted from the period of 2008 until April 2018 yielded a total of 142 studies. After an adequate screening, 26 studies were reviewed. Incidence of DTC in association with PHPT in the literature ranged between 0.91% and 17.6%. The main histological thyroid malignancy found is micropapillary carcinoma. Despite its less aggressive presentation, these microcarcinomas may grow or develop nodal metastases on follow up. Although bilateral neck exploration with hemi/total thyroidectomy carries the risk of temporary recurrent laryngeal nerve injury or hypoparathyroidism, permanent complications are rare especially when compared to re-do neck surgery. Recently, parathyroid surgeries are going towards minimal invasive procedures, requiring an adequate imaging tool to ensure diagnosis of both diseases. Multiple risk factors for concomitant diseases were hypothesized, the more robust are the common embryologic origin and activation of angiogenic growth factors. Conclusion: NMTC is frequently associated with PHPT especially in endemic goiter areas. With the high prevalence of micropapillary carcinoma and its risks, a partial/total thyroidectomy in addition to parathyroidectomy may be warranted. With the recent need of adequate diagnostic tools, combining both Technetium Sestamibi scintigraphy and thyroid ultrasound improved sensitivities and accuracy of diagnosis, but dual-isotope scintigraphy (I-123 sodium iodide/ 99mTc-sestamibi) seems an attractive modality in hyperparathyroid patients with concomitant suspicious thyroid nodules. However, further studies for validation may be needed.

Objectives The aim of this observational clinical study was to evaluate the success of angiographic selective venous sampling (ASVS) in locating parathyroid adenoma in patients with primary hyperparathyroidism (PHPT), in whom the other imaging modalities have failed, and and to evaluate its possible contribution to the applicability of minimal invasive surgery. Material and methods Fifty-five patients who were admitted to our hospital's General Surgery department between January 2012 and January 2015 for PHPT in whom ultrasound and sestamibi scintigraphy have failed to localize the diseased gland were included to the study. Patients were divided into two groups: those who underwent ASVS and those who did not. The outcomes of patients were reviewed retrospectively. Results Among 55 patients, 20 underwent ASVS. ASVS successfully lateralized the diseased gland in 17 (85%) patients, and minimally invasive parathyroidectomy could be performed in 14 (70%) patients. The cut-off value of parathormon gradient was considered 10% for lateralization and the accuracy of ASVS in lateralization was 94.1%. In 11 (59%) patients, the superior-inferior discrimination could be achieved in addition to lateralization. Conclusion ASVS has a high sensitivity in locating the diseased gland in patients with PHPT in whom ultrasound and sestamibi scan have failed, and thereby, rendering the performance of minimally invasive surgery possible. Further studies may reveal the role of ASVS in providing useful information about not only lateralization but also the superior-inferior discrimination.

Background Parathyroid cancer is a rare cause of primary hyperparathyroidism. It presents a diagnostic and therapeutic challenge that may not be recognized preoperatively, and is often not conclusively identified during the operation. We present the case of a lady with backache and hypercalcemia, but with inadequate work-up for her condition for several years. Case presentation A middle aged lady of Asian descent presented with backache. Initial work up revealed mild hypercalcemia, negative work up for multiple myeloma, negative sestamibi scan for parathyroid pathology. A phenomenally elevated parathormone (PTH) level—2105 pg/mL (16–87 pg/mL), and rising serum calcium, 15.1 mg/dL, (8.6–10.5 mg/dL), ordered years later prompted a repeat sestamibi scan and ultrasonography of neck. Based on these investigations, a diagnosis of primary hyperparathyroidism, with high suspicion of parathyroid cancer was made. The patient underwent surgical tumour resection, with subsequent histopathological confirmation of diagnosis. Conclusion In the setting of hypercalcemia, PTH level assessment is a must. This helps to differentiate between the parathyroid dependant and independent causes of high serum calcium, thereby encouraging a comprehensive pathway to the work up of the cause of hypercalcemia. The parathyroid cancer is a very rare cause of hypercalcemia, which needs to be considered in the differentials of primary hyperparathyroidism, particularly in the setting of high PTH levels. Electronic supplementary material The online version of this article (10.1186/s13104-018-3711-0) contains supplementary material, which is available to authorized users.

Summary We report a case of elderly Chinese lady with neurofibromatosis type-1 presenting with longstanding palpitation, paroxysmal hypertension and osteoporosis. Biochemical testing showed mild hypercalcaemia with non-suppressed parathyroid hormone level suggestive of primary hyperparathyroidism, and mildly elevated urinary fractionated normetanephrine and plasma-free normetanephrine pointing to a catecholamine-secreting pheochromocytoma/paraganglioma. Further scintigraphic investigation revealed evidence of a solitary parathyroid adenoma causing primary hyperparathyroidism and a left pheochromocytoma. Resection of the parathyroid adenoma and pheochromocytoma resulted in normalization of biochemical abnormalities and hypertension. The rare concurrence of primary hyperparathyroidism and pheochromocytoma in neurofibromatosis type-1 is discussed. Learning points: All NF-1 patients who have symptoms suggestive of a pheochromocytoma/paraganglioma (PPGL), even remotely, should undergo biochemical testing. The initial biochemical tests of choice for PPGL in NF-1 are either plasma-free metanephrines or urinary fractionated metanephrines. Any elevations of metanephrines should be carefully evaluated for the presence of PPGLs in NF-1 patients. Primary hyperparathyroidism (PHPT) is described in subjects with NF-1. Due to the lack of epidemiological and functional studies, their association is yet to be substantiated. Meanwhile, PHPT may further exacerbate the metabolic bone defect in these patients and should be treated when present according to published guidelines. Coexistence of PPGL and PHPT can occur in subjects with NF-1, mimicking multiple endocrine neoplasia type 2 (MEN2).

Background We investigated the rate of persistent and recurrent hyperparathyroidism after focused unilateral exploration (UE) with intraoperative monitoring of intact parathyroid hormone (IOPTH). Study Design A prospective cohort of 915 patients with primary hyperparathyroidism (PHP) underwent parathyroid surgery by a single surgeon from January 2003 to September 2013. A total of 556 patients with at least a single positive preoperative localization by ultrasound (US) and/or sestamibi scan (STS) underwent UE with IOPTH. The criterion for completion of surgery was an IOPTH fall of 50% from the highest intraoperative level and into the normal range 5 to 10 minutes after resection of the localized gland. Results Fifteen patients had either persistent or recurrent PHP, yielding a 2.7% (95% CI 1.6% to 4.4%) overall recurrence rate based on the refined Wilson method with continuity correction. Four patients had persistent PHP. Three of these patients were cured with reoperation, and the fourth patient was followed nonoperatively. Eleven patients had recurrent PHP, with 5 corrected by surgery and 6 patients followed nonoperatively. The mean postoperative serum calcium (Ca) level was 9.4 mg/dL over a mean follow-up interval of 44.0 months. Preoperative localization rates by each localization study were: US 74.3% (n = 413), STS 86.9% (n = 483), and US and STS 71.4% (n = 397). There was no difference in the preoperative study that localized the hyperfunctional parathyroid gland in recurrent vs nonrecurrent patients by the Fisher's exact test (US, p =1.00; STS, p =0.65; US and STS, p =1.00). Conclusions The low rate of recurrent PHP after focused unilateral exploration with IOPTH suggests that this procedure should not be abandoned.

Purpose The improvement of intraoperative parathyroid hormone (IOPTH) assay and localization studies has enabled a minimally invasive parathyroidectomy (MIP) in primary hyperparathyroidism (pHPT). The aim of this study is to analyze the demographics, clinical presentations, and surgical outcomes of the pHPT patients who received surgical management with versus without IOPTH. Methods Analysis of a database was performed on 53 patients who underwent parathyroidectomy for pHPT from 2004 to 2013. Preoperative localization was done by both sestamibi scan and ultrasonography. We divided the patients into two groups (without IOPTH versus with IOPTH) and analyzed the surgical outcomes statistically between two groups. Results The concordance rate of Technetium 99m sestamibi scan and ultrasonography was 73.6% and 90.6%, respectively. The overall cure rate of group 1 (without IOPTH) was 94.9% and that of group 2 (with IOPTH) was 100%. The decline of PTH at postoperative 5 minutes and 10 minutes was 75.2% ± 14.9% and 84.9% ± 8.6% in cured patients. On the other hand, that of noncured patients at 5 minutes and 10 minutes was 17.2% ± 9.7% and 8.2% ± 2.2%. There was a significant difference in the drop rate of IOPTH between cured and persistent patients (P < 0.01). Pathological examination showed adenoma in 41 of 53 patients (77.4%) and hyperplasia in 10 of 53 patients (18.9%). Conclusion Even though the localization studies were successful, IOPTH monitoring is essential to avoid a surgical failure in MIP.

Background Intraneural parathyroid adenomas are rare, with only 9 cases of intravagal adenomas reported. All but one of the reported cases was found after multiple neck explorations. To the best of our knowledge, we report the first case of nonsupernumerary ectopic intravagal parathyroid identified at primary exploration. Methods and Results A 17-year-old girl with primary hyperparathyroidism and nephrolithiasis was referred with a sestamibi scan reporting a left lower parathyroid adenoma. No eutopic parathyroid tissue was identified during full exploration of the left side of the neck. Exploration of the carotid sheath revealed a fusiform swelling of the vagus nerve at the level of the carotid bifurcation. Longitudinal incision of the vagal perineurium revealed a 7-mm parathyroid adenoma, which was enucleated. The patient recovered uneventfully, with normalization of serum calcium, parathyroid hormone (PTH), and normal vocal cord function. Conclusion We believe that this is the first reported case of nonsupernumerary intravagal parathyroid adenoma resected at initial exploration. The vagus nerve is a rare location for a parathyroid adenoma, but one that should be considered, even during primary exploration. © 2015 Wiley Periodicals, Inc. Head Neck 37: E200–E204, 2015

SummaryObjective Pre-operative imaging techniques have enabled minimally invasive parathyroid surgery to supersede the traditional approach to hyperparathyroidism (HPT) surgery, which included cervical exploration. Cervical ultrasound (US) and sestamibi scan (MIBI) are commonly performed, but the results of these localization tests do not always match. This study correlated surgical outcomes with pre-operative localization findings, including matched positive US and MIBI studies, one positive study (US or MIBI), conflicting studies or negative results. Design Retrospective medical record review. Patients A hundred and sixty nine consecutive patients who underwent parathyroidectomy from January 2005 to December 2012. Measurements Correlation between surgical outcomes and pre-operative localization tests. Results All patients (134F/35M, 59·6 ± 13·5 years of age) had primary HPT. US and MIBI localization studies matched in 76%, whereas 10·7% had positive MIBI only and 8·3% US only. Studies were negative in 3·6% and contradictory in 1·8%. Minimally invasive parathyroidectomy was performed in 87% of the matched group and 89% of the MIBI-only group. Surgical success rate, defined as postoperative normalization of calcium and PTH levels, was similar in patients with a single positive study (MIBI or US) vs double-matched studies (MIBI and US). Patients were followed up for 6 weeks. Overall, pathology was consistent with adenoma in 95%. Discussion Parathyroidectomy success rate was similar in patients with primary HPT and MIBI-only or US-only positive localization studies compared to those with matched US/MIBI studies. The results support a clinical algorithm in which positive results from one imaging technique, either MIBI or US, are sufficient to refer a patient for parathyroid surgery.

Primary hyperparathyroidism (HPT) is one of the most common endocrine disorders. The standard treatment is surgical removal via a cervical incision. In rare cases, abnormal parathyroid tissue may be present in the mediastinum and therefore inaccessible through the standard incision. A 57-year-old female presented in our clinic with high ionized calcium and high parathyroid hormone levels. A technetium-99m sestamibi scan showed a focal mass in the superior mediastinum. A single-stage cervical exploration and video-assisted mediastinoscopic (VAM) removal of the intrathoracic ectopic parathyroid adenoma was performed. Our approach proved to be successful. There were no surgical complications and the patient was discharged on postoperative day 2. We suggest that in the case of occult mediastinal hyperfunctioning parathyroid tissue, a VAM is a valuable extension of the standard explorative neck surgery.

Successful localization is mandatory for focused parathyroidectomy. If ultrasound and sestamibi scan are negative, bilateral neck exploration is necessary. We examined the contribution of complementary computed tomography (CT) scan to identify the affected parathyroid gland. Between November 1999 and April 2014, 25 patients (20 females and 5 males; mean age 67 ± 11 years) with negative or dubious standard imaging (ultrasound and sestamibi scan) underwent CT scan prior to parathyroidectomy and were included in this study. Fifteen patients had had previous neck surgery for parathyroidectomy (n = 11) or thyroidectomy (n = 4). Thin-slice CT (n = 9) or four-dimensional (4D) CT imaging (n = 16) was used. Cure was defined as >50 % post-excision fall of intraoperatively measured parathyroid hormone or fall into the normal range, confirmed by normocalcaemia at least 6 months after surgery. Preoperative CT scan provided correct localization in 13 out of 25 patients (52 %) and was false positive once. Parathyroidectomy was performed by a focused approach in 11 of these 13 patients as well as in 1 patient guided by intraoperatively measured parathyroid hormone (ioPTH). Thirteen patients required bilateral neck exploration. The cure rate was 96 % (24/25 patients). One patient has persistent primary hyperparathyroidism (pHPT) and one a recurrent disease. Six patients presented a multiglandular disease. A CT scan identifies about half of abnormal parathyroid glands missed by conventional imaging and allows focused surgery in selected cases.

Osteitis fibrosa cystica, a manifestation of severe hyperparathyroidism presenting with crippling bone deformities is a rare presentation these days. We report a case of 40-year-old male patient who presented with generalized aches and pains and bony deformities. Radiographs showed diffuse osteopenia, brown tumors and pathological fractures of phalanges but common manifestations associated with hyperparathyroidism like pancreatic calcifications and nephrolithiasis were absent. Serum calcium and parathyroid hormone levels were elevated while ultrasound imaging of neck showed the presence of a left lower parathyroid adenoma and was confirmed by Tc99- sestamibi scan. Large parathyroid lesion along with high calcium levels and severely elevated PTH puts this patient in high-risk category for postoperative hungry bone syndrome leading to severe hypocalcemia postoperatively. Hypocalcemia usually results in neuromuscular irritability manifesting as paresthesia, Chvostek and Trousseau sign, carpopedal spasm and even convulsions in severe cases. However, our patient had none of the common manifestations, but developed hypocalcemic cardiac failure postoperatively. Case history and management of case is presented. How to cite this article Maturi R, Makineni H, Marri SSK. A Unique Case of Osteitis Fibrosa Cystica with Postoperative Hungry Bone Syndrome and Hypocalcemic Cardiac Failure. World J Endoc Surg 2015;7(1):10-13.

Accurate preoperative localization is critical to the success of minimally invasive parathyroidectomy. This investigation aimed to assess the correlation among preoperative imaging results, intraoperative findings, and postoperative cure rates in patients undergoing operation for primary hyperparathyroidism.A retrospective review of all patients who underwent operation for primary hyperparathyroidism between June 2010 and March 2016 was performed.During the study period, 398 patients underwent parathyroidectomy. The overall cure rate was 97.5%. The ultrasonography performed by the surgeon was superior to the ultrasonography performed by the radiologist and to the sestamibi scan in lateralizing the adenoma correctly (80% vs 62% vs 70%, P.001, respectively), and had the greatest sensitivity (93%) and accuracy (80%) among all tests (P.001). Age ≥65 was found to be associated with lesser cure rates (94% vs 99.2%, P = .003). The number of positive preoperative studies correlated with cure rate, ranging from 80% for patients with 0 positive studies, to 100% in those with 4 positive studies (P = .0004). In patients with a negative sestamibi and an ultrasonography performed by the radiologist, there was no significant difference in the cure rates among those with no preoperative computed tomography, a positive preoperative computed tomography, or a negative preoperative computed tomography.An ultrasonography performed by an experienced surgeon is an extremely valuable preoperative localization modality. The cure rate obtained is proportional to the number of positive imaging studies. In patients with negative ultrasonography performed by a nonexperienced radiologist and a negative sestamibi scan, the performance of computed tomography does not seem to increase cure rate. Patients with no positive preoperative scans represent a challenging subgroup, with cure rates of approximately 80%.