Left Inferior Parathyroid Research Articles

Atypical parathyroid adenoma: Series of two consecutive cases from a tertiary care hospital in Qatar.

IntroductionAtypical parathyroid adenomas (APA) are an uncommon cause of hypercalcemia and comprise a minority of parathyroid adenomas.Presentation of casesCase 1 - Egyptian male, 48 years old with history of type 2 diabetes mellitus, incidentally discovered increased serum of calcium level on routine investigation, was diagnosed as PHPT, US and MIBI scan showed large left inferior parathyroid adenoma, focused exploration and excision of the APA was undertaken, histopathology confirmed APA. Case 2 - Egyptian male, 60 years old, cardiac patient with history of diabetes, hypertension and multiple cardiac interventions, had nausea, vomiting, constipation abdominal pain, polyuria, polydipsia, and history of passing renal stones, hypercalcemia workup showed primary hyperparathyroidism (PHPT), MIBI was negative and SPECT scan suggested right inferior parathyroid adenoma, focused exploration and excision of the APA was undertaken, histopathology confirmed APA.DiscussionAPA are an uncommon cause of hypercalcemia and are responsible for a minority of parathyroid adenomas. Combined US and MIBI and SPECT scans can detect APA. Focused exploration and excision of the APA under general anaesthesia can completely remove the APA.ConclusionAwareness of the physician and a high index of suspicion to symptoms or signs that could reflect an underlying PHPT is essential. Yearly biochemical and neck US follow up are required to detect any risk of recurrence or malignancy in the long term.

Intrathyroidal Parathyroid Carcinoma: Case Report and Literature Review.

Intrathyroidal parathyroid carcinoma (PC) is a rare malignancy that is usually difficult to diagnose. We present a case of a 31-year-old male with a history of hyperparathyroidism who was found to have intrathyroidal PC upon review of immunostains along with a review of the current literature. A systematic review of the literature utilizing the PubMed database identified 24 relevant, full-text articles. 25 cases were analyzed, including our own report. The case of a 31-year-old man with a history of hyperparathyroidism managed with subtotal thyroidectomy and subtotal parathyroidectomy who had persistent hypercalcemia and elevated parathyroid hormone. Abnormal radiotracer uptake was noted in the left thyroid gland. Neck exploration with left parathyroidectomy and revision thyroidectomy was performed. A candidate left inferior parathyroid was found within the left thyroid lobe remnant and identified as parathyroid carcinoma. Immunostains determined an intrathyroidal parathyroid carcinoma. The literature review shows the average presenting age was 50.9years. 54.17% (CI, 43-82%) of affected patients are female. Right-sided thyroid involvement is seen in 54.17% (CI, 34-74%) of cases. The inferior aspect of the thyroid is involved in 66.67% of cases (CI, 53-89%). Intrathyroidal parathyroid carcinoma is a rare and challenging diagnosis due to similarities with other more common endocrine abnormalities. This review found that the inferior parathyroid is more likely to be located within the thyroid gland. Surgeons may consider aberrant anatomical locations, including intrathyroidal locations, for the inferior parathyroid glands.

Double water-clear cell parathyroid adenoma: a case report and literature review

Water-clear cell parathyroid adenoma is an uncommon cause of primary hyperparathyroidism. Herein, we report an interesting case of a 56-year-old man who presented with weight loss, bone pain, fatigue, and a palpable right neck mass. Laboratory tests indicated hypercalcemia, elevated parathyroid hormone (PTH) levels, and normal thyroid function. Further examinations detected osteoporosis and kidney stones. The ultrasound of neck revealed bilateral extrathyroidal tumors, which were sestamibi-avid. The patient underwent resection of the large right inferior and left inferior parathyroid tumors. Histopathology revealed a double water-clear cell parathyroid adenoma. His serum calcium and PTH levels normalized after surgery. The literature review identified 37 cases of water-clear cell parathyroid adenoma between 1985 and 2021. The median age at diagnosis was 56 years. Classic complications were common, including nephrolithiasis in nine and skeletal presentations in 10 patients. Before surgery, the median calcium and PTH levels were 12.0 mg/dL and 290 pg/mL, respectively. Overall, 89% were localized on ultrasonography, and 60% were positive on scintigraphy. Four patients had double adenomas. The median maximum diameter was 3.8 cm, and the median weight of the resected adenoma was 5.27 g. In summary, water-clear cell parathyroid adenoma has certain unique features. These include larger tumor size, relatively indolent biochemical profile, high prevalence of complications and nonspecific symptoms, an isoechoic appearance on ultrasonography, and reduced scintigraphic sensitivity.

Osteitis fibrosa cystica mimicking bone tumor, a case report

BackgroundWe report a case of osteitis fibrosa cystica, a rare benign resorptive bone lesion caused by hyperparathyroidism, that presented on imaging as an aggressive bone tumor.Case presentationThe patient is a 51-year-old male complaining of severe sustained pain of the right hip region. Imaging studies were suspicious for a malignant tumor of the right iliac bone. Biopsy under CT guidance was performed and showed remodeled bone trabeculae with numerous osteoclasts, excluding bone tumor and raising the possibility of osteitis fibrosa cystica. Complementary tests disclosed elevated blood level of parathyroid hormone and a partially cystic enlarged left inferior parathyroid gland consistent with adenoma. After parathyroidectomy, the clinical symptoms were relieved and the radiological findings were significantly improved, which confirmed the diagnosis.ConclusionsMetabolic diseases-associated bone lesions should always be considered in the differential diagnosis of bone tumors, to avoid unnecessary surgeries and treatments.

Ectopic Intact PTH Secretion Causing Humoral Hypercalcemia of Malignancy

Background: Tumor-generated ectopic intact PTH is difficult to diagnose and should be suspected in patients with apparent primary hyperparathyroidism but with normal parathyroid glands.Clinical Case: A 72-year-old man presented with symptoms of hypercalcemia including generalized weakness, polyuria, and polydipsia. Initial labs were consistent with primary hyperparathyroidism: calcium 12.1 mg/dL (n 8.6–10.3 mg/dL, albumin-corrected 12.5 mg/dL), intact PTH (iPTH) 115.6 pg/mL (n 10–65 pg/mL), low normal 25-OH vitamin D (25 ng/mL, n 25–100 ng/mL), and relatively high normal 1,25 dihydroxyvitamin D (52 pg/mL, n 18–78 pg/mL). 24-hour urine calcium was 381 mg/day (n 100–300 mg/day) and PTHrP was 1.6 pmol/L (n <4.2 pmol/L). Neck ultrasound demonstrated a 0.5 x 1 cm hypoechoic mass near right thyroid inferior pole, though sestamibi SPECT/CT scan did not reveal scintographic evidence of a parathyroid adenoma. He underwent subtotal parathyroidectomy with largest excised gland weighing 0.262 grams. The left inferior parathyroid gland appeared normal intraoperatively, thus was clipped and left in place. PTH decreased from 194 pg/mL to 98 pg/mL postoperatively. Pathological examination revealed three normocellular parathyroid glands with enlargement of only the right superior gland. Venous sampling of the parathyroid vasculature failed to identify the source of autonomous iPTH post operatively. Due to refractory hypercalcemia, cinacalcet was initiated. However, hypercalcemia as high as 12.6 mg/dl and hyperparathyroidism to 672 pg/mL persisted despite dose escalation. He eventually received pamidronate with subsequent transition to denosumab due to declining renal function. A 68Ga DOTATATE scan was performed to locate occult ectopic parathyroid, which reported multiple foci of presumed somatostatin receptor expression involving the liver and intra-abdominal lymph nodes without significant uptake in the neck concerning for metastatic disease. Liver lesion biopsy was consistent with pancreato-biliary adenocarcinoma. Surprisingly, the biopsy was negative for iPTH and neuroendocrine tumor markers on staining/immunohistochemistry. Given his poor prognosis and multiple comorbidities, the patient opted not to pursue any further workup or therapy for his malignancy.Conclusion: Occult malignancy should be suspected for a patient with persistent hyperparathyroidism after parathyroidectomy. Treatment of the malignancy may lead to an improvement in hypercalcemia and iPTH levels. Employment of iPTH mRNA testing or intra-abdominal venous sampling to prove ectopic iPTH secretion would be ideal, as iPTH staining could be falsely negative. Further testing was not completed as the patient declined further evaluation.

Night Sweats as the Presenting Symptom of Primary Hyperparathyroidism

Background: Approximately 25–40% of patients report night sweats in the previous month during appointments with their primary care clinicians. The differential diagnosis for night sweats is broad, with hyperthyroidism, carcinoid syndrome, pheochromocytoma, medullary thyroid carcinoma, insulinoma, and acromegaly as established endocrine causes. We present a case of primary hyperparathyroidism (PHPT) in which the patient’s chief complaint was night sweats and resolution occurred after parathyroidectomy. Case. A 39-year-old female reported one-year of daily night sweats that required changes of clothes and bedding. She denied excessive daytime sweating, frequent palpitations, tremors, nightmares, rashes, fevers, chills, cough, headaches, dizziness, abdominal pain, diarrhea, disrupted menses, or unintentional weight loss. Vital signs and examination were unremarkable. Hypercalcemia (11.0 mg/dL, 8.6–10.3) was noted and confirmed by additional serum calcium measurements. Intact PTH ranged from 27–33 pg/mL (12–88), and 24 h urine calcium (258 mg) excluded familial hypocalciuric hypercalcemia (FHH). Parathyroid scintigraphy and neck ultrasound identified a left neck mass, and the patient underwent successful resection of a left inferior parathyroid adenoma. Hypercalcemia and night sweats initially resolved after surgery, but the patient returned six weeks later with recurrence of night sweats. Reevaluation was notable for serum calcium 10.4 mg/dL, phosphorus 2.4 mg/dL (2.5–5.0), and intact PTH 104 pg/mL. A right superior parathyroid adenoma was identified on repeat parathyroidectomy, and the patient experienced durable resolution of night sweats and hypercalcemia following her second parathyroid surgery. She was screened for multiple endocrine neoplasia type 1 (MEN1) due to multiple parathyroid tumors, though no known pathogenic menin gene variants were identified. Conclusions: A title/abstract search in PubMed linking “hyperparathyroidism” and “hypercalcemia” to “night sweats,” “sleep hyperhidrosis,” “sweating,” “hot flashes,” “hot flushes,” “diaphoresis” and “vasomotor symptoms” yielded only one relevant case of a postmenopausal woman with hot flushes unresponsive to hormone replacement that resolved after parathyroidectomy for PHPT. Hypercalcemia is known to affect central nervous system function. It is possible that in rare cases hypercalcemia alters function of the medial preoptic area, lowering the temperature threshold above which peripheral vasodilatation and perspiration occur to dissipate heat. The patient’s predisposition to only night sweats is unclear, though unlike the first patient reported with PHPT and sweating, our patient is premenopausal. This case indicates that vasomotor symptoms may occur with PHPT and resolve after successful parathyroid surgery.

Hypercalcemia in Pheochromocytoma: From MEN to VHL

Background: Co-occurrence of phaeochromocytoma and primary hyperparathyroidism is usually seen in patients of Multiple Endocrine neoplasia 2A(MEN2A) and is rare in Von Hippel Lindau disease (VHL). Parathyroid adenoma with pheochromocytoma in a genetically confirmed VHL has been reported only once till date (1). Clinical Case: A 30-year male was admitted for evaluation of hypertension and incidentally diagnosed adrenal mass on ultrasound. 9-years back, he was diagnosed as a case of VHL (right renal clear cell carcinoma, pancreatic cystadenoma, spinal hemangioblastoma and epididymal cysts). Right nephrectomy and pancreatic cyst excision had been done and past work-up for pheochromocytoma was normal. He also had been operated twice for recurrent renal calculi. Family history revealed surgery in mother for pancreatic mass. Current imaging revealed right adrenal mass (4.7*4.6 cm) with left renal cysts and calculi, pancreatic cysts and spinal- medullary hemangioblastoma and epididymal cysts. Fundus examination was normal. 24-hours urinary fractionated normetanephrines were elevated (2062 mcg/24 hours) and I131MIBG scan showed 4.7*4.6cm concentrating lesion in right renal fossa suggestive of right adrenal pheochromocytoma. However his biochemical evaluation revealed hypercalcemia (12.1 mg/dl), low phosphorus (3.2 mg/dl), low 25(OH) D (24.84 nmol/l), and raised PTH (121pg/ml). Ultrasound neck and Tc99m-Sestamibi localized left inferior parathyroid adenoma. DEXA scan showed severe osteoporosis. Genetic analysis confirmed VHL mutation in exon-1. Calcitonin and RET mutation were normal (ruled out MEN2A). Therapeutic approach was surgical excision of adrenal pheochromocytoma followed by parathyroidectomy. We report a case of pheochromocytoma with primary hyperparathyroidism (cause: left inferior parathyroid adenoma) in a patient of VHL (Renal clear cell carcinoma, pancreatic cystadenoma, epidydymal cysts and medullary and spinal hemangioblastoma). Hypercalcemia seen in patients of VHL is either due to bone metastasis/PTHrP/IL-6 secretion from RCC or due to PTHrP/PTH/calcitonin secretion from pheochromocytoma and rarely due to associated parathyroid adenoma. Literature search revealed four case reports of parathyroid adenoma with VHL. In only one of these, VHL had pheochromocytoma associated with parathyroid adenoma (1). Conclusion: Ours is the 2nd such case reported in literature of primary hyperparathyroidism in a genetically confirmed case of VHL with pheochromocytoma. This case highlights the overlap of tumorigenesis in two rare genetically divergent syndromes and importance of long-term follow-up for sequential development of new tumors. Reference: Arao T, Okada Yet al. A case of VHL disease with bilateral pheochromocytoma, renal cell carcinoma, pelvic tumor, spinal hemangioblastoma and primary hyperparathyroidism. Endocr J. 2002 Apr;49(2):181–8.

A rare case of multiple endocrine neoplasia type 1 initially presenting as an asymptomatic, huge mediastinal mass: case report

BackgroundMultiple endocrine neoplasia type 1 (MEN1) is a rare inherited syndrome that concurrently involves various endocrine glands. We report a rare case of MEN1 in a 43-year-old man whose first manifestation was an asymptomatic mediastinal mass.Case presentationA 13-cm-sized mediastinal mass was diagnosed as an atypical thymic carcinoid by computed tomography and percutaneous needle biopsy. In addition, hypercalcemia from a left inferior parathyroid hyperplasia, and a non-functioning gastric neuroendocrine tumor seen on esophagogastroduodenoscopy were found. Therefore, the patient was clinically diagnosed with MEN1 syndrome, and underwent surgical resection of thymic carcinoid tumor after pre-operative concurrent chemoradiation therapy to decrease tumor size and volume. Parathyroid lesion and gastric neuroendocrine tumor were also removed. Finally, a MEN1 gene mutation was observed in the patient and his 7-year-old son.ConclusionDespite its rare occurrence, thymic carcinoid tumor should be considered as a MEN1-associated tumor and necessitates screening of other endocrine glands. Thymic carcinoid tumor carries a poor prognosis in patients with MEN1, and thus it needs to be carefully monitored even after radical excision.

Osteitis Fibrosis Cystica: Classical Case Report of an Uncommon Presentation in a Young Female.

Osteitis fibrosa cystica (OFC) is a skeletal disease related to long standing, end-stage hyperparathyroidism. However, nowadays hypercalcemia due to primary or secondary hyperparathyroidism can often be detected early by laboratory screening and imaging modalities; consequentially the frequency of osteitis fibrosa cystica has drastically declined. OFC, also termed as Brown tumor, can mimic primary bone tumors clinically, which often leads to misdiagnosis and inappropriate management. Moreover due to its rarity, it is usually not considered initially in the differential diagnosis of bony tumors. Case presentation: This is the case of a 19-year-old female, who presented with pain over the left ankle region for 3months. The biochemical screening revealed a picture consistent with primary hyperparathyroidism and the radiologically proven to be left inferior parathyroid adenoma. She underwent excision of the primary parathyroid adenoma, and gradual regression of symptoms was noted postoperatively. Osteitis fibrosa cystica is a rare manifestation of hyperparathtyroidism, which has become a forgotten entity lately due to early identification of hypercalcaemia by modern techniques. A delay in the diagnosis of parathyroid adenoma results in manifestations that can be avoided. The diagnosis of OFC requires a high degree of clinical suspicion. When hypersecretion of PTH is corrected, spontaneous regression of the lesion is expected.

Severe Hypercalcaemia as a Consequence of Parathyroid Crisis - A Case Report

36-years old man presenting with the complaint of sudden severe upper abdominal pain, vomiting and polyuria with raised serum creatinine (2.74 mg/dl), serum calcium (19.68 mg/dl) and intact parathyroid hormone (iPTH) (2014.70 pg/ml) level. His ultrasound image of neck region demonstrated hypoechoic mass (12×10 mm) at left inferior parathyroid gland. Parathyroid schintography reported positive for parathyroid adenoma (left lower). He underwent medical management followed by parathyroidectomy. Optimum serum calcium concentration is essential for homeostasis and function of multiple organ systems. Severe hypercalcaemia is rare but life threatening complication of primary hyperparathyroidism (PHPT). In order to prevent mortality, it is necessary to provide immediate appropriate treatment by correcting hypercalcaemia and dehydration.

A rare extensive clinical presentation of calciphylaxis due to primary hyperparathyroidism

Calciphylaxis is a dreadful condition predominantly seen in chronic kidney disease patients on haemodialysis or following renal transplant. Calciphylaxis occurring in patients with normal kidney function is extremely rare. Here we present 53-year-old women presented with painful, extensive eschar-like skin lesions involving bilateral lower limb of 3 months of duration. She had hypercalcemia, high serum parathyroid hormone (PTH) levels and elevated alkaline phosphatase. Ultrasound neck and sestamibi scan demonstrated left inferior parathyroid adenoma. Lower limb radiographic studies showed subcutaneous calcification. Cutaneous biopsy confirmed calciphylaxis. Cinacalcet to control hypercalcemia, antibiotics and pain control medications were started. Left inferior parathyroidectomy and debridement of gangrenous lesions of lower limbs were performed. Histopathology was consistent with parathyroid adenoma. Lower limb wound started granulating with Vacuum-assisted closure (VAC) dressing and skin graft was planned. Unfortunately, she succumbed 11 weeks from surgery due to proximal myopathy, aspiration pneumonitis, sepsis and multiorgan failure.

Pregnancy, Pancreatitis, and Hypoglycemia: Multi-modal Management in a Case with Multiple Endocrine Neoplasia-1 Syndrome

Abstract A 32-year-old lady presented to us at 6 months of gestation with acute pancreatitis due to parathyroid hormone-dependent hypercalcemia and with insulin-dependent hypoglycemia, owing to parathyroid adenoma and possibly insulinoma, respectively. The parathyroid adenoma was localized on the magnetic resonance imaging of the neck; however, imaging for the insulinoma could not be done due to the gravid state. Due to the simultaneous occurrence of tumours in two endocrine glands, namely, parathyroid gland and pancreatic islet cells, a diagnosis of MEN-1 (multiple endocrine neoplasia) was considered, which is very rarely seen in pregnancy. Her hypercalcemia was effectively managed with cinacalcet and alcohol ablation of the parathyroid adenoma while her hypoglycemic episodes were managed with short and long-acting octreotide injections during the antenatal period. She had a full-term cesarean delivery, with no maternal or neonatal complications, except for transient neonatal hypoglycemia. The patient was followed up for 1 year after her delivery with no further episodes of hypercalcemia and hypoglycemia, on medical management. Tc99m sestamibi scan was done after delivery which confirmed the presence of a left inferior parathyroid adenoma. MEN-1 with pregnancy thus poses a diagnostic and therapeutic challenge and our case highlights the role of multimodal medical therapy for successful management.

Single Center Experience in the Surgical Management of Primary Hyperparathyroidism.

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

SAT-386 Inconsistent Bilateral Arm Serum PTH Testing in Recurrent Hyperparathyroidism in a Patient Status-Post Parathyroid Auto-Transplantation

Background: More than 95% of patients with primary hyperparathyroidism will be cured with the initial operation by an experienced surgeon. However, localization of hyperparathyroid recurrences, especially after extensive surgery becomes challenging. For patients with transplanted parathyroid glands into the forearm, there may be utility in bilateral arm serum PTH testing to help with localization.Clinical Case:A 65-year-old woman presented to the clinic with primary hyperparathyroidism in 2008. After a localization study, she had a partial parathyroidectomy but continued to have persistent biochemical hyperparathyroidism despite negative localization studies. She was then referred to another institution for further studies. Follow up Sestamibi scans were negative but 4D-CT scans assisted in localizing the presence of a superior parathyroid gland adenoma which was later removed in 2011. During this time, the left inferior parathyroid gland was auto-transplanted into the left forearm. Again, her calcium and PTH levels rose despite negative Sestamibi scans showing no abnormalities in post-operative beds or in the forearm. Review of previous labs revealed elevated PTH levels in the ranges of 80-110 pg/mL since 2012-2019. The patient’s most recent PTH was 2408 pg/mL. At that point, the decision was made to repeat the labs on the left and right forearms simultaneously and labs showed PTH levels of 1283 pg/mL and 118 pg/mL, respectively. Repeat Sestamibi scan following these labs demonstrated evidence of increased radiotracer uptake in the region of the prior transplanted parathyroid tissue with no neck uptake concerning for hyperparathyroidism due to auto-transplanted hyperplastic tissue.Conclusion:This case demonstrates the utility of bilateral arm serum PTH testing in the evaluation of recurrent hyperparathyroidism in patient’s status-post parathyroid auto-transplantation.

MON-LB66 Altered Mental Status and Hypercalcemia: A Rare Presentation of Pure Osteolytic Metastatic Prostate Cancer

Background: We present a rare case of hypercalcemia with the concomitant presence of parathyroid adenoma, secondary hyperparathyroidism due to kidney disease and hypercalcemia of malignancy. Mild hypercalcemia due to primary hyperparathyroidism often precedes the acute, more severe hypercalcemia of malignancy. Prostate cancers are usually known to cause osteoblastic lesions. We present a rare case of prostate cancer with pure osteolytic metastasis.Case: 73 year old male with past history of ESRD on hemodialysis was brought to the ER with change in mental status. Labs showed elevated serum calcium 13.3 mg/dl (8.6-10.2 mg/dl) and creatinine 7.0 mg/dl (0.60-1.30mg/dl). Patient underwent emergent hemodialysis. Additional lab work revealed, elevated phosphorus level of 5.8mg/dl (2.5-5 mg/dl), low vitamin D 25-hydroxy of 22 ng/ml (30-100 ng/ml) and vitamin 1-25 dihydroxy level of 7 ng/ml (20-79 ng/ml). Both PTH 172.6 pg/ml (12-88 pg/ml) and PTHrP 64 pg/ml (14-27 pg/dl) levels were elevated. Parathyroid scan showed increased uptake in left inferior parathyroid gland indicating the presence of a parathyroid adenoma. Serum calcium levels remained persistently elevated despite being continued on dialysis with a low calcium bath and receiving calcium lowering therapy with calcium binding agent- cinacalcet, calcitonin, bisphosphonate. Further work up for refractory hypercalcemia revealed an elevated prostate-specific antigen (PSA) level of 1420 ng/ml (0-3.999 ng/ml). Bone scan showed no evidence of osseous metastasis. CT abdomen & pelvis showed extensive lytic bony metastases, with metastasis to lung and lymph nodes in mesenteric root and in the pelvis. Prostate gland showed asymmetric contour along the left posterolateral zone suspicious for malignancy with extracapsular spread.Biopsy from the left iliac lytic bone lesion was done that showed poorly differentiated metastatic adenocarcinoma, consistent with a prostatic primary. The patient was started on treatment with anti-androgen medication- Bicalutamide and prednisone and was planned to be started on Leuprolide as outpatient.Discussion: Hypercalcemia is uncommon in advanced prostate cancer compared to other malignancies where osteolytic metastasis is more common than osteoblastic metastasis. Incidence of malignancy in patients with primary hyperparathyroidism and vice-versa is high, hence serum PTH and PTHrP should be measured in hypercalcemic patients with malignancy. If PTHrP and PTH are both elevated, it indicates co-existent primary hyperparathyroidism. Prostate cancers are usually known to cause osteoblastic lesions and pure osteolytic metastasis from prostate carcinoma is extremely rare. Radio-nucleotide bone scan preferentially detects osteoblastic metastasis. CT or MRI is indicated to look for osteolytic lesions if suspicion for bone metastasis is high.

SAT-388 Dry Cough as Only Sign of a Parathyroid Adenoma Producer of PTH

BACKGROUND: The parathyroid adenoma producing an excess of PTH is characterized by hypercalcemia, asthenia, physical weakness and renal lithiasis. This clinical case is presented only with a dry (non-productive) cough sign of long duration.CASE: 51-year-old female born in Padre Las Casas, D.R. presenting with chief complain of dry cough for about four years. Clinical findings: (03/13/2019) Height 62”, Weight 142 lbs, Temperature 36.2 Celsius, BP 90/60 mmHg, RR 16 rpm, HR 60 bpm, on her neck no adenopathies or thyroid changes. Occasional coughing. A sonographic evaluation of the neck (04/09/2019) reveals a solid, heterogeneous nodular image of 0.7 cm x 0.5 cm in the left lobe of the Thyroid (Fig. 1) which by FNAB (04/10/2019) showed a benign adenomatoid node with cystic changes (Bethesda II) (Fig. 2). TEST: (03/20/2019) anti-TG 0.10 IU/mL (NV -115), anti-TPO 9.00 IU/mL (NV -34), TG 9.41 (NV -78 ng/mL), TSH 0.34 μIU/mL (VN 0.27-4.20), free T3 2.05 pg/mL (NV 2.04-4.40), total T3 0.74 ng/mL (NV 0.83-2.00), total T4 8.46 μg/dL (NV 5.1-14.1), free T4 1.61 ng/dL (NV 0.93-1.71) Calcium 10.4 mg/dL (NV 8.1-10.4), Phosphorus 2.6 mg/dL (NV 2.5-4.5), PTH-Intact 157 pg/mL (NV 14.5-87.1) Thyroid-Parathyroid scintigraphy (Sestamibi-Technetium 99mTc04: 15 mCi) (04/23/2019) shows lower left Parathyroid Adenoma (Fig. 3). She undergoes surgery (05/23/2019) removing the left thyroid lobe and left inferior parathyroid gland whose pathology shows chronic nodular colloid goiter, with areas of hemorrhage. Parathyroid adenoma of main cells (Fig. 4-5). Post-surgical TEST (06/24/2019) PTH-intact 69.0 pg/mL (NV 14.5-87.1), Calcium 8.6 mg/dL (NV 8.1-10.4), Phosphorus 2.7 mg/dL (NV 2.5-4.5), anti-TG 10.0 IU/mL (NV <115), anti-TPO 9.00 IU/mL (NV <34), TG 8.92 ng/mL (NV <78), total T3 0.68 ng/mL (NV 0.83-2.00), free T3 1.95 pg/mL (NV 2.04-4.40), total T4 6.40 μg/dL (NV 5.1-14.1), free T4 1.02 ng/dL (NV 0.93-1.71). Post-surgical clinical evaluation (06/21/2019) Weight 142 lbs, Temperature 36.5 Celsius, BP 110/70 mmHg, RR 16 rpm, HR 60 bpm. Patient has not shown signs of coughing. Last TESTS (10/20/2019) Calcium 9.40 mg/dL, Phosphorus 3.10 mg/dL, PTH-intact 24.40 pg/mL, TG 11.90 ng/mL, total T4 6.80 μg/dL, free T4 1.23 ng/dL, total T3 0.88 ng/mL, free T3 2.66 pg/mL, anti-TPO 11.14 IU/mL, anti-TG 10 IU/mL. CONCLUSIONS: Lower left (benign) parathyroid adenoma whose clinical manifestations are not common. Dry (non-productive) cough is not known as a manifestation of elevated PTH-intact. Calcium and Phosphorus levels in normal values. In addition, histological alterations of the left thyroid lobe of benign character with few manifestations of hormonal alterations and normal antibodies. It is of crucial clinical importance to observe and document more cases with similar presentation in order to identify the possible causes of cough with an elevated PTH manifestation.

SAT-349 Cinacalcet Resistant Tertiary Hyperparathyroidism with One Large Parathyroid Gland

Tertiary hyperparathyroidism is thought to develop after long term secondary hyperparathyroidism, such as CKD on dialysis. In This case, all parathyroid glands are significantly enlarged.We report a case of an 84-year-old female with a past medical history of ESRD on HD and recurrent nephrolithiasis who was found to have an enlarged multinodular goiter with the dominant mass in the lower pole of the right gland measuring about 4.7 cm on thyroid ultrasound. Blood work was done which showed elevated intact PTH levels at 2720 pg/mL (12–88). Her calcium level was normal at that time at 10.5 mg/dL (8.6–10.8) with an albumin of 4.2 g/dL (3.5–5.7), and a phosphorus of 5.7 mg/dL (2.5–4.5). Patient had a DEXA scan which showed severe osteoporosis in the lumbar spine, left hip, and right forearm. Patient had increased PTH levels despite being on Cinacalcet. She had a nuclear medicine parathyroid scan with SPECT CT which showed increased uptake along the right inferior thyroid concerning for a large right lower parathyroid adenoma or functional thyroid nodule. FNA of the nodule was done and showed colloid nodule but PTH wash showed elevated PTH at 7634 pg/mL. She was referred for right lower parathyroidectomy and Cinacalcet was discontinued prior to surgery. She had right and left inferior parathyroidectomy and 4 gland exploration. Pathology showed the right inferior parathyroid gland to be markedly hyper-cellular, weighing 36 grams consistent with hyperplasia and the left inferior parathyroid gland to be slightly hyper-cellular parathyroid gland consistent with hyperplasia. No evidence of malignancy was noted on pathology. Prior to surgery her calcium level of 10.6 mg/dL which went down to 9.6 mg/dL post-operatively. Patient then developed severe hypocalcemia and hungry bone syndrome following the surgery requiring a calcium drip for 3 days post-operatively. Was changed to oral calcium but required large amounts of calcium gluconate and calcitriol supplementation and an extended hospital stay of 13 days. However, throughout the hospital stay, PTH levels continued trending back up to 239 which may represent either increased activity from remaining parathyroid glands or residual parathyroid adenoma with incomplete resection. Post-operative US neck showed Post-surgical collection in the lower pole of the right gland measuring 4.8 cm compatible with recent resection.In conclusion, tertiary hyperparathyroidism can develop due to one enlarged parathyroid gland or an adenoma, which can be resistant to cinacalcet.

SAT-364 Brown Tumor of the Mandible in Severe Uncontrolled Primary Hyperparathyroidism

Background: Brown tumors are a part of the complex “osteitis fibrosa cystica” which is a diffuse resorptive process of the bone resulting from uncontrolled hyperparathyroidism. Although these brown tumors were fairly common in the past1, the incidence of brown tumors is now extremely rare in the United States due to early diagnosis and treatment of hyperparathyroidism. Here we describe an unusual presentation for osteitis fibrosa cystica. Clinical Case: A 38 year old woman was admitted to the hospital with rapidly growing facial swelling in June 2019. The swelling initially appeared in January 2019 and had rapidly increased in size since April 2019. The mass was malodorous, painful and interfered with oral intake. The patient smoked 1 pack per day for 13 years. A CT scan of the head and neck with contrast showed a 3.6 x 4.5 x 3.3 cm mass destroying the right mandibular body, involving the right platysma muscle and displacing the central and lateral incisor teeth and was concerning for malignancy. The initial impression was that this was likely a squamous cell carcinoma with local and perhaps distant metastases, complicated by humoral hypercalcemia of malignancy and she was admitted for management.Upon admission, her calcium level was 14 mg/dL, albumin 4.1 g/dL, phosphorus 1.4 mg/dL PTH level was 890 pg/mL and vitamin D level was 22 ng/mL. Since the high PTH did not fit with hypercalcemia of malignancy, further evaluation was undertaken. A thyroid ultrasound showed a 4.6 x 1.8 x 1.5 cm isoechoic, heterogeneous lesion in the left lower pole, and Tc-99 sestamibi scan suggested parathyroid origin. The biopsy of the mouth lesion showed fibro histiocytic proliferation with multiple giant cells, negative for malignancy and consistent with a brown tumor. CT scan of the chest, abdomen and pelvis did not reveal any other masses or lytic lesions. . A skeletal survey showed another 2 cm lytic lesion in the proximal left humeral metaphysis which was not biopsied.On parathyroidectomy, the patient was found to have an enlarged left inferior parathyroid gland measuring 3.3 x 3.0 x 0.8 cm weighing 7.093 grams. Intraoperatively PTH level decreased from 890 pg/mL to 69 pg/mL. Her calcium levels returned to normal post-operatively and she was discharged home. Pathologic examination report revealed the mass to be a parathyroid adenoma. The patient reports a reduction in the size of her mandible mass since the surgery.Conclusion: Although uncommon, brown tumors can be seen in uncontrolled primary hyperparathyroidism. The mandible is a common site, though, as in this case, it is less common for it to be the only site affectedReference:1. Rosenberg, E. H. (1962). Hyperparathyroidism. A review of 220 proved cases with special emphasis on findings in the jaws. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 15(2), 84–94.

Management of polycythemia vera by endoscopic parathyroidectomy

Polycythemia vera (PV) is a hemotologic disease. Majority of persons with PV, essential thrombocythemia and primary myelofibrosis show the Janus kinase (JAK) 2 gene– which is needed for the normal development of blood cells. PV is generally controlled with medication: hydroxyurea or pipobroman. Phlebotomy is the mainstay of therapy for PV. Association between hyperparathyroidism and PV has been very sparingly considered. Primary hyperparathyroidism may produce a growth factor, which induces pancytosis, especially in the presence of high levels of ionized calcium. It is found that parathyroid hormone levels (PTH) could influence hemopoiesis through a direct action on hemopoietic precursors. A 52-year-old female presented with complaints of fever associated with headache and generalized weakness. On evaluation, her hemoglobin value was found to be high (Hb– 18.8 g/dl) with leukocytosis. She was evaluated for polycythemia, which showed JAK-2 mutation positivity. She was started on Enteric Coated Aspirin. She was managed initially with twice-weekly venesection to keep packed cell volume <45. It was observed that she was having very high PTH– 569.9 pg/ml and low Vitamin D levels– 8.41 mg/ml. She underwent a Technetium 99M Sestamibi Scan (TcMIBI), which showed a large adenoma involving the left inferior parathyroid gland. She was taken up for endoscopic parathyroid removal through the left axillo-breast approach. Intraoperatively, PTH value had dropped from 569.9 pg/ml to 62 pg/ml. Hemoglobin level decreased to normalcy by the 2nd week of surgery without any medication or venesection. Patients with polycythemia must be screened for hyperparathyroidism. Early treatment of hyperparathyroidism by parathyroid removal will take care of polycythemia as well. Endoscopic parathyroidectomy is very feasible, safe, and well accepted by patients.

SAT-232 Is There a Role for Genetic Testing in Guiding the Management of Primary Hyperparathyroidism in Pregnancy?

Introduction Primary hyperparathyroidism (PHPT) in pregnancy is rare, with a reported incidence of <1%. It is associated with serious maternal and foetal complications, and is genetically determined in ~10% of cases. Confirming a genetic diagnosis can guide management of inherited PHPT, and enables screening for syndrome-related diseases. This case series highlights the role of genetic testing in the tailored management of patients with PHPT in pregnancy. Clinical cases Case 1 is a 20 year old female who was diagnosed with PHPT at 5 weeks gestation after presentation with nausea. She was hypercalcaemic (2.78 mmol/L at diagnosis; 2.98 mmol/L pre-parathyroidectomy), had negative neck ultrasonography and a MEN1 mutation (exon 2, c.236_237del, p.(Pro79fs)) on genetic testing in pregnancy. After initial management with oral and IV rehydration, her severe symptomatic hypercalcaemia required treatment with open neck exploration and a 3 gland parathyroidectomy in the 2nd trimester. The standard surgical approach of a 3.5 gland parathyroidectomy was avoided due to the risk of inducing hypoparathyroidism. Intra-operative PTH dropped 53.8% post 3 gland parathyroidectomy. Parathyroid hyperplasia on histology was consistent with the diagnosis of MEN1 syndrome. The patient’s symptoms resolved post-operatively and foetal investigations are normal at 28 weeks gestation. She is receiving close obstetric follow-up including 3-weekly foetal growth scans due to the increased risk of IUGR. She was counseled on her increased risk of pancreatic and pituitary tumours and her 1st degree relatives are undergoing genetic screening. Case 2 is a 40 year old female with a known diagnosis of PHPT who was awaiting parathyroidectomy prior to pregnancy. She was hypercalcaemic (2.78 mmol/L at 7 weeks; 2.99 mmol/L pre-parathyroidectomy), and had a lower left parathyroid adenoma on ultrasound and pre-pregnancy SPECT-CT. Genetic testing was not performed as she was 40 at diagnosis and had no contributory family history. After failure of conservative management, parathyroidectomy of the left superior parathyroid gland was performed in the 2nd trimester. Histology results confirmed a left inferior parathyroid adenoma, concordant with the radiological findings. Her symptoms improved post-operatively; she is receiving close obstetric follow-up. Clinical lessons Genetic testing enables the management of PHPT to be tailored appropriately. It can better inform the surgical approach; including the need for subtotal parathyroidectomy and bilateral neck exploration in those cases at risk of multiple parathyroid gland disease, as demonstrated in our case with MEN1. Finally this case series demonstrates that surgical management of PHPT should be considered for select cases in pregnancy and thus far no maternal or foetal complications have arisen in these featured cases following surgical intervention.