A Challenging Diagnosis of Primary Hyperparathyroidism in an Adolescent Female

Abstract Background: Hypercalcemia secondary to primary hyperparathyroidism (PHPT) is less common in children than adults. Single parathyroid adenoma is commonly the cause of primary hyperparathyroidism in children. Clinical Case: We present a 15-year-old female with one-week history of abdominal pain despite taking over the counter antacids. Her initial work up by her primary care provider revealed serum calcium of 11.8 mg/dL (9.0–11.5) and creatinine of 0.8 mg/dL (0.4–1.2). A week later, she presented to the emergency department with same complaint. In ED, she was found to have hypercalcemia (12.8 mg/dl) with elevated parathyroid hormone (PTH) at 78.5 pg/mL (15–65). Her random urine calcium creatinine ratio was high at 2.1. Her 25OHD was 25 ng/mL (30–100). She had negative urine pregnancy test but had trace ketones, leukocyte esterase, blood and bacteria. CBC and CMP were otherwise unremarkable. She continued to complain abdominal pain with nausea, decrease appetite, fatigue, and general muscle weakness. There was no known family history of calcium or metabolic bone disorders. Her vital signs and physical exam were normal. Subsequent labs showed mild improvement of calcium between (11–12.3 mg/dL), PTH between 54.5 and 77 pg/mL, normal thyroid function. Ionized calcium was mildly elevated 6.0 mg/dL (4.5–5.3) but her repeat 25OHD was low at18 ng/mL. Serum phosphorus levels were relatively normal with lowest level of 2.5 mg/dL (2.7–4.5). Gliadin Deamidated IgA was detectable 15 U/mL (< 15.0 U/). Ultrasound of abdomen was significant for nonspecific mild hepatomegaly; kidneys were normal in size and appearance. Ultrasound of thyroid was significant for probably intrathyroid parathyroid, measuring 6 x 8 x 8 mm. Tc-Sestamibi scan did not confirm a parathyroid adenoma. Genetic testing for MEN-1 was negative. FHH- related genes (i.e. CASR) was positive for p.R990G variant resulting in a mild gain of function of the calcium-sensing receptor. Although previous Tc-Sestamibi scan was unremarkable, an over read of it raised a concern for questionable uptake in the left superior lobe. SPEC-CT demonstrated possible abnormal parathyroid tissue in the upper pole of the left thyroid. FNA of the left thyroid nodule confirmed likely intrathyroidal parathyroid adenoma. Subsequent follow up and treatment, including parathyroidectomy, was done by another institution. She underwent a left parathyroidectomy with normalization of serum calcium and PTH levels post operatively (10.1 mg/dl and 8 pg/mL, respectively) and has complete resolution of her previous abdominal and gastrointestinal symptoms. Conclusion: PHPT is uncommon in children and adolescents and is typically associated with a single parathyroid adenoma. High index of suspicion is key for early diagnosis of PHPT despite a negative Tc-Sestamibi initially.

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SAT-353 Familial Hyperparathyroidism - Due to a Rare Genetic Mutation

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.470 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Adeyinka Taiwo ◽

Joseph Stephen Dillon ◽

Jennifer Stinson

Keyword(s):

Family History ◽

Primary Hyperparathyroidism ◽

Parathyroid Adenoma ◽

Gene Mutation ◽

Parathyroid Tissue ◽

Clinical Manifestations ◽

Young Age ◽

Parathyroid Cancer ◽

History Of ◽

Familial Hyperparathyroidism

Abstract Introduction: Hyperparathyroidism occurs most commonly in middle age patients, predominantly in women. It can be caused by parathyroid adenoma, hyperplasia or parathyroid carcinoma. Genetic predisposition can be found in about 10% of primary hyperparathyroidism due to certain gene mutations. This case emphasizes the importance of taking a detailed family history when patients present with hyperparathyroidism at a young age, so that familial hyperparathyroidism, if present, can be detected and relatives screened. Clinical case: A 26 y.o. male presented with symptoms of fatigue and polydipsia for several years. He was noted to have a serum calcium of 12.4 mg/dL(8.5–10.5), with parathyroid hormone of 213 pg/ml (15–65). He denied any history of kidney stones, fractures and no palpable neck masses. The patient’s family history was significant for his paternal half-sister who had parathyroidectomy for hyperparathyroidism at 20yrs old and paternal grandmother died of parathyroid cancer in her 50s. The patient’s father died of pancreatic cancer at 41yrs old. A neck ultrasound revealed a mass posterior to the left inferior thyroid. A Sestamibi parathyroid scan revealed a parathyroid adenoma at the posteroinferior aspect of the left hemithyroid. Labs for free metanephrines and normetanephrine, prolactin and gastrin levels were all normal. Due to his young age and the possibility of having familial hyperparathyroidism, he underwent bilateral neck exploration and parathyroidectomy, with removal of his left inferior, right superior, left superior parathyroid glands and left upper thymus. Surgical pathology revealed, hypercellular parathyroid tissue. Post operatively, his calcium and vitamin D remained within the normal range. Genetic studies revealed a mutation in the parafibromin gene - CDC73 (also called HRPT2), a tumor suppressor gene, which is on chromosome 1q25. The patient currently has 6 children ranging from age 5 months to 6 years. He was advised to have his children tested any time from age 7 years for the gene mutation. The patient has remained stable 4yrs post operatively, with normal calcium and PTH levels. He does not have any history of jaw tumor. He never had an ultrasound kidney done. He is being monitored with yearly lab tests. Conclusion: CDC73 gene mutation-associated disorders are inherited as an autosomal dominant fashion, with variable penetrance. This gene mutation can be found in conditions such as hyperparathyroidism jaw tumor, familial hyperparathyroidism and parathyroid cancer. Reference: 1. CDC73-Related Disorders: Clinical Manifestations and Case Detection in Primary Hyperparathyroidism. J Clin Endocrinol Metab. 2017 Dec 1;102(12):4534–4540.

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Spontaneous Resolution of Primary Hyperparathyroidism Following COVID-19 Infection

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.454 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A223-A224

Author(s):

Mahwash F Siddiqui ◽

Naim Maalouf

Keyword(s):

Primary Hyperparathyroidism ◽

Parathyroid Adenoma ◽

Receptor Gene ◽

Elevated Serum ◽

Parathyroid Tissue ◽

Symptomatic Treatment ◽

Underlying Mechanism ◽

Normal Serum ◽

Spontaneous Resolution ◽

History Of

Abstract Background: Spontaneous resolution of primary hyperparathyroidism (PHPT) is extremely rare, and has previously been reported exclusively in the setting of apoplexy (auto-infarction) of a large parathyroid adenoma outgrowing its blood supply. We report an unusual case of spontaneous PHPT resolution after Covid-19 infection. Clinical Case: A 38-year-old Hispanic woman was seen in clinic for evaluation of hypercalcemia first noted on routine bloodwork in 2018. She had a history of nephrolithiasis (1 episode) but no other symptom associated with hypercalcemia. She was not on medications associated with hypercalcemia, and had no family history of Ca disorder. Serum biochemistry was notable for hypercalcemia (Ca: 10.9, 10.5, and 10.8 mg/dL, nl 8.4–10.2), normal albumin and Cr, elevated PTH (106 and 70 pg/mL, nl 15–65), with low phosphate (2.3 mg/dL, nl 2.4–4.5) and 25-OH-Vit. D (14 ng/mL, nl 20–80). 24-hr urinary studies showed elevated urine Ca (285 and 375 mg/day, nl 100–250), with urine Cr of 1.3 and 1.5 g/day, respectively. Findings of hypercalcemia, hypercalciuria, and elevated serum PTH, led to the diagnosis of PHPT. Neck ultrasound and sestamibi scan of the parathyroids with SPECT-CT did not localize any abnormal parathyroid gland. A month after PHPT diagnosis, and prior to surgical evaluation, patient presented with a 9-day history of fever, cough, and shortness of breath. She tested positive for Covid-19 infection by SARS-CoV-2 PCR on nasal swab, and was quarantined at home with symptomatic treatment. No glucocorticoids were given. Serum Ca was normal (9.5 mg/dL) at the time of Covid-19 diagnosis. All Covid-19 symptoms subsided 14 days after onset. Repeat labs 1 and 3 months after Covid-19 infection showed persistently normal serum Ca (10.0 and 9.8 mg/dL), with low then normal PTH (13 and 43 pg/mL), compatible with spontaneous resolution of PHPT. Patient denied any neck discomfort before, during, or after Covid-19 infection. Spontaneous resolution of PHPT is rare and follows apoplexy of a large parathyroid adenoma. In our patient, imaging failed to localize a large parathyroid adenoma, making it less likely that resolution of her PHPT was caused by apoplexy. Resolution of PHPT temporally coincided with Covid-19 infection, although the link between the two conditions is unclear at this time. Hypothesized mechanisms include an imbalance in the normal PTH-Ca axis caused by SARS-CoV-2 mediated release of inflammatory cytokines (e.g. interferon, previously reported to lower serum calcium), or development of antibodies against the parathyroid or CaSR. RNA and protein expression of ACE2, the SARS-CoV-2 cell receptor gene, is not detected in normal parathyroid tissue. Conclusion: To our knowledge, this is the first reported case of spontaneous resolution of PHPT after Covid-19 infection. Further studies are needed to understand the frequency of this occurrence, and the underlying mechanism.

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Normocalcemic primary hyperparathyroidism

Arquivos Brasileiros de Endocrinologia & Metabologia ◽

10.1590/s0004-27302010000200004 ◽

2010 ◽

Vol 54 (2) ◽

pp. 106-109 ◽

Cited By ~ 61

Author(s):

John P. Bilezikian ◽

Shonni J. Silverberg

Keyword(s):

Parathyroid Hormone ◽

Primary Hyperparathyroidism ◽

Serum Calcium ◽

Kidney Stones ◽

Bone Disease ◽

Clinical Phenotype ◽

Mineral Metabolism ◽

Historical View ◽

History Of ◽

Excessive Secretion

Primary hyperparathyroidism is a common disorder of mineral metabolism characterized by incompletely regulated, excessive secretion of parathyroid hormone from one or more of the parathyroid glands. The historical view of this disease describes two distinct entities marked by two eras. When primary hyperparathyroidism was first discovered about 80 years ago, it was always symptomatic with kidney stones, bone disease and marked hypercalcemia. With the advent of the multichannel autoanalyzer about 40 years ago, the clinical phenotype changed to a disorder characterized by mild hypercalcemia and the absence of classical other features of the disease. We may now be entering a 3rd era in the history of this disease in which patients are being discovered with normal total and ionized serum calcium concentrations but with parathyroid hormone levels that are consistently elevated. In this article, we describe this new entity, normocalcemic primary hyperparathyroidism, a forme fruste of the disease.

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SAT-380 A Case of Primary Hyperparathyroidism on the Third Trimester of Pregnancy

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.1884 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Maria Alexandra Carranceja Villapol ◽

Maria Princess L Kanapi

Keyword(s):

Blood Pressure ◽

Abdominal Pain ◽

Primary Hyperparathyroidism ◽

Parathyroid Adenoma ◽

Elevated Serum ◽

Stable Condition ◽

Abdominal Ultrasound ◽

Pressure Control ◽

Ibandronic Acid ◽

The Right

Abstract Introduction: This is the case of a pregnant woman on her 3rd trimester who was diagnosed with primary hyperparathyroidism. Since there are two patients involved, the potential complications that can be brought about by the diagnostic tests and the treatment had to be weighed against the benefits. Case: The patient is SA, a 34-year old female on her 29th week of pregnancy, admitted due to a month history of abdominal pain described as intermittent, crampy, generalized, non-radiating, and mild-to-moderately severe in intensity. She was advised to do tests but did not comply. In the interim, there was recurrence of symptoms but with resolution. However, the crampy abdominal pain recurred, now localized at the right upper quadrant and epigastric areas, radiating to the right upper back, moderate in intensity, and with associated nausea and vomiting, leading to admission. She was first managed under OB-Gynecology, given hydration, pain management and Betamethasone. She was also referred to Cardiology for blood pressure control, and Surgery for evaluation of the abdominal pain. Due to an increasing trend of her blood glucose, she was referred to Endocrinology and started on insulin. Mild bilateral nephrocalcinoses seen in an abdominal ultrasound prompted work-up showing an elevated serum ionized calcium at 1.88 meq/L (n 1.12-1.32 meq/L), elevated intact PTH at 451.13 pg/ml (n <67.9 pg/ml), and low Vitamin D at 10.96 ng/ml (n >30ng/ml). Parathyroid ultrasound showed nonthyroidal tissue measuring 0.4 x 0.6 cm at the right inferior area. Saline hydration and diuresis with Furosemide were started to manage the hypercalcemia. A multi-disciplinary meeting was held to discuss the options for management and risks involved. The goal was to deliver the baby in stable condition possibly to term, while keeping maternal calcium levels and blood pressure normal. However on her 30th week of gestation, she had persistent elevated blood pressure and underwent emergency caesarian section. After delivery, the patient was advised against breastfeeding for adequate management of her hypercalcemia. She was started on Cinacalcet, Calcitonin, and Ibandronic Acid. A Parathyroid Sestamibi Scan done showed a parathyroid adenoma on the right inferior lobe, and she underwent right inferior parathyroidectomy, with left thyroidectomy and isthmusectomy. Findings showed a right inferior parathyroid adenoma and a benign follicular nodule on the left thyroid. She was started on Calcium Carbonate and Calcitriol, and discharged stable. Conclusions: This case shows that when two lives are at stake every step of the management, whether diagnostic or therapeutic, must be communicated well to the patient and to the other members of the team. It is ultimately a choice made by the expectant mother but through the proper guidance and updated knowledge of the team, combined with a good clinical eye especially in the treatment of pregnant women.

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SAT-058 A Rare Case of Primary Hyperparathyroidism in a Pediatric Patient

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.702 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Natalia Salazar ◽

Jeff M Merz ◽

Liliana Burdea ◽

Carla Minutti

Keyword(s):

Abdominal Pain ◽

Primary Hyperparathyroidism ◽

Clinical Significance ◽

Parathyroid Tissue ◽

High Serum ◽

Genetic Evaluation ◽

Atypical Presentation ◽

Pediatric Endocrinology ◽

Endocrine Disorder ◽

Ret Gene

Abstract Introduction: Primary hyperparathyroidism (PHPT) is the third most common endocrine disorder in adult patients, but it is rare in pediatric patients. It is usually diagnosed when patients present with symptomatic hypercalcemia or known complications. In children, atypical presentation often results in delayed diagnosis and increased morbidity. We report a 10-year-old boy presenting with abdominal pain and emesis, found to have hypercalcemia and, ultimately, PHPT. His genetic evaluation was notable for a mutation in the RET gene (3.2C>A) of unknown clinical significance. Case presentation: A 10-year-old male with a history of constipation presented to the emergency department with five days of abdominal pain and emesis. Initial workup revealed high serum calcium (Ca) of 17.3 mg/dL (8.7-10.7) and ionized Ca (ICal) of 2.01 mmol/L (0.95-1.32). Further evaluations revealed low phosphorus level 3.5 mg/dL (4.5-6.5) and high parathyroid hormone level (iPTH) of 329.2 pg/mL (8.0-85.0). These findings were consistent with PHPT. Neck US demonstrated a cervical mass in the mid-right thyroid measuring 0.5 x0.3 x0.5cm, questionable for parathyroid adenoma, which was confirmed with 99mTc-MIBI scintigraphy and neck CT. His Ca level initially responded to fluid resuscitation and Lasix, with Ca level decreasing to 13.6. However, on hospital day two, his Ca level became refractory to all interventions, rising to 16 and prompting the use of bisphosphonates. The patient underwent neck exploration with partial parathyroidectomy and lymph node excision. Pathology revealed hypercellular parathyroid tissue consistent with parathyroid hyperplasia. Intraoperatively iPTH was reduced from 3,134.7 to 79.8 and remained within normal limits since. Postoperatively course was uncomplicated, and the patient was discharged home on oral Ca carbonate and vitamin D. A genetic evaluation was remarkable for a change in the RET gene (3.2C>A), a finding of unknown clinical significance. This change has not been seen in association with an individual who fulfills the clinical diagnosis of MEN2A. To further determine if the variant identified in the RET gene is a benign variant, the mother was tested for MEN2A and was negative. Father could not be tested, but family history was significant for thyroid malignancies. The patient is currently doing well four months postoperatively. His Ca level remains normal. Due to the genetic finding and the concern of MEN2A syndrome, the patient is followed closely by pediatric endocrinology and genetics. Conclusion: PHPT is a common endocrine disorder in adults but rare in children. The diagnosis of pediatric PHPT is almost always delayed due to atypical presentation and rarity of the disease. As secondary organ damage is common, a multi-organ assessment is mandatory. Due to the association with other syndromes, a genetic evaluation should be performed.

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Night Sweats as the Presenting Symptom of Primary Hyperparathyroidism

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.421 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A207-A207

Author(s):

Vanessa Williams ◽

Hadoun Jabri ◽

Michael G Jakoby

Keyword(s):

Primary Hyperparathyroidism ◽

Parathyroid Adenoma ◽

Serum Calcium ◽

Vasomotor Symptoms ◽

Temperature Threshold ◽

Hot Flushes ◽

Parathyroid Surgery ◽

Intact Pth ◽

Patient Reported ◽

Night Sweats

Abstract 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.

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Thoracoscopic surgical case of an ectopic mediastinal parathyroid adenoma detected by chance: a case report

BMC Surgery ◽

10.1186/s12893-019-0641-2 ◽

2019 ◽

Vol 19 (1) ◽

Cited By ~ 1

Author(s):

Shota Mitsuboshi ◽

Hideyuki Maeda ◽

Hiroe Aoshima ◽

Tamami Isaka ◽

Takako Matsumoto ◽

...

Keyword(s):

Thyroid Cancer ◽

Parathyroid Adenoma ◽

Serum Calcium ◽

Tumor Resection ◽

Anterior Mediastinum ◽

Pathological Examination ◽

Cervical Approach ◽

History Of ◽

Mediastinal Parathyroid Adenoma ◽

Mediastinal Parathyroid

Abstract Background Ectopic mediastinal parathyroid tumor (EMPT) is a rare cause of primary hyperparathyroidism (PHPT); it is difficult to resect using the cervical approach. We describe a case of using video-assisted thoracic surgery (VATS) for EMPT resection. Case presentation A 67-year-old woman with a history of postoperative thyroid cancer had no symptoms. She was diagnosed with PHPT and underwent thyroid cancer surgery. She had serum calcium and intact parathyroid hormone (PTH) levels of 11.1 mg/dL and 206 pg/mL, respectively. Chest computed tomography showed a 10-mm nodule in the anterior mediastinum. Technetium-99 m methoxyisobutyl isonitrile scintigraphy showed an abnormal uptake lesion in the anterior mediastinum. She was diagnosed with PHPT caused by EMPT and underwent VATS. The pathological examination confirmed parathyroid adenoma. Her serum calcium and intact PTH levels were normal from 15 min after tumor resection. She has had no recurrence of EMPT. Conclusions The VATS approach was effective for the resection of EMPT.

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Incidental Parathyroid Disease during Thyroid Surgery: Should We Remove Them?

ISRN Surgery ◽

10.5402/2011/962186 ◽

2011 ◽

Vol 2011 ◽

pp. 1-5 ◽

Cited By ~ 2

Author(s):

S. Helme ◽

A. Lulsegged ◽

P. Sinha

Keyword(s):

Primary Hyperparathyroidism ◽

Parathyroid Gland ◽

Thyroid Surgery ◽

Parathyroid Tissue ◽

Neck Surgery ◽

Safe Procedure ◽

Preclinical Phase ◽

Abnormal Parathyroid Gland ◽

Parathyroid Disease ◽

History Of

Aim. Despite an incidence of parathyroid “incidentalomas” of 0.2%–4.5%, only approximately 135 cases have been reported in the literature. We present eight patients in whom an incidental abnormal parathyroid gland was found during routine thyroid surgery. We have reviewed the literature and postulate whether these glands could represent further evidence of a preclinical stage of primary hyperparathyroidism. Methods. A retrospective analysis of all 236 thyroid operations performed by a single surgeon was performed to identify patients in whom abnormal parathyroid tissue was removed at surgery. Results. 8/236 patients (3.39%) had a single macroscopically abnormal parathyroid gland removed and sent for analysis. Seven patients were found to have histological evidence of a parathyroid adenoma or hyperplasia. None of the patients had abnormal serum calcium detected preoperatively. Postoperatively, four patients had normal calcium, three had temporary hypocalcaemia and one refused followup. No patients had recurrent laryngeal nerve impairment. Conclusions. Despite the risk of removing a histologically normal gland, we believe that when parathyroid “incidentalomas” are found during surgery they should be excised and sent for histological analysis. We have found this to be a safe procedure with minimal morbidity to the patient. As the natural history of primary hyperparathyroidism is better understood, these glands found in normocalcaemic patients may in fact represent the early or preclinical phase of the disease. By removing them at the original operation, the patient is saved redo neck surgery with its high complication rate as or when clinically apparent primary hyperparthryoidism develops in the future.

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Rare Case of Severe Hypercalcemia Secondary to Atypical Sestamibi Negative Parathyroid Cystic Adenoma

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.540 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A266-A267

Author(s):

Timur Gusov ◽

John Chen Liu ◽

Sowjanya Naha ◽

F N U Marium ◽

Joseph Theressa Nehu Parimi ◽

...

Keyword(s):

Primary Hyperparathyroidism ◽

Parathyroid Adenoma ◽

Parathyroid Gland ◽

Severe Hypercalcemia ◽

Parathyroid Adenomas ◽

Sestamibi Scan ◽

Serum Pth ◽

The Right ◽

99Mtc Sestamibi ◽

Cystic Adenoma

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

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

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.2251 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Ajinkya Kulkarni ◽

Mrunal Kulkarni ◽

Rithikaa Ellangovan ◽

Rajesh Thirumaran

Keyword(s):

Prostate Cancer ◽

Primary Hyperparathyroidism ◽

Parathyroid Adenoma ◽

Mental Status ◽

Serum Calcium ◽

Bone Scan ◽

Rare Case ◽

Osteolytic Metastasis ◽

Prostate Cancers ◽

Osteoblastic Lesions

Abstract 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.

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