Multiple concurrent atypical parathyroid adenomas: a rare occurrence

2020 ◽  
Vol 13 (5) ◽  
pp. e234421
Author(s):  
Jack Faulkner ◽  
Kiran Varadharajan ◽  
Natasha Choudhury
Keyword(s):  
Parathyroid Adenoma ◽  
Vascular Invasion ◽  
Parathyroid Carcinoma ◽  
Postoperative Recovery ◽  
Rare Entity ◽  
Rare Occurrence ◽  
First Case ◽  
Parathyroid Adenomas ◽  
Atypical Parathyroid Adenoma ◽  
Histological Characteristics

We present a case of an 80-year-old patient with three concurrent multiple atypical parathyroid adenomas. The patient was managed with bilateral parathyroid exploration and concurrent parathyroidectomy. Postoperative recovery was uneventful. Atypical parathyroid adenomas are a rare entity that are clinically difficult to distinguish from parathyroid carcinoma. Histological characteristics are similar however atypical adenomas lack complete capsular or vascular invasion and cannot be considered malignant. All reported cases of atypical parathyroid adenoma are solitary lesions. We present the first case of multiple concurrent atypical parathyroid adenomas.

scholarly journals An Interesting Case of Life-Threatening Hypercalcemia Secondary to Atypical Parathyroid Adenoma versus Parathyroid Carcinoma

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Ankur Mishra ◽  
David Newman
Keyword(s):  
Parathyroid Adenoma ◽  
Serum Calcium ◽  
Parathyroid Carcinoma ◽  
Interesting Case ◽  
High Serum ◽  
En Bloc ◽  
Severe Hypercalcemia ◽  
Parathyroid Adenomas ◽  
Life Threatening ◽  
Atypical Parathyroid Adenoma

Context.Severe hypercalcemia is a life-threatening condition. Atypical parathyroid adenoma and parathyroid carcinomas are uncommon causes which can be difficult to differentiate.Objective.We report a case of a 36-year-old male with very high serum calcium due to a possible atypical parathyroid adenoma versus parathyroid carcinoma.Case Illustration.A serum calcium level of 23.2 mg/dl was noted on admission. He was initially treated with IV hydration, pamidronate, and salmon calcitonin to lower his calcium levels. He also underwent a surgical en bloc resection of parathyroid mass. Pathology showed a mixed picture consistent with possible atypical adenoma versus parathyroid carcinoma. However, due to the possible involvement of the recurrent laryngeal nerve, parathyroid carcinoma was more likely. Also after operation the patient developed hungry bones syndrome and his calcium was replaced vigorously. He continues to be on calcium, vitamin D, and calcitriol supplementation.Results.A review of the literature was conducted to identify previous studies pertaining to parathyroid adenomas and parathyroid cancer.Conclusion.We thereby conclude that hypercalcemia requires very careful monitoring especially after operation. Also it can be very difficult to distinguish between atypical parathyroid adenomas and parathyroid carcinomas as in our case and no clear cut guidelines yet exist to differentiate the two based on histology.

scholarly journals Atypical Parathyroid Adenoma in a Young Man

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A182-A182
Author(s):  
Elizabeth R Bowen ◽  
Joseph Stephen Dillon
Keyword(s):  
Parathyroid Hormone ◽  
Parathyroid Adenoma ◽  
Mitotic Activity ◽  
Parathyroid Carcinoma ◽  
Sudden Onset ◽  
Lytic Lesion ◽  
Brown Tumor ◽  
Adjacent Structures ◽  
Parathyroid Adenomas ◽  
Atypical Parathyroid Adenoma

Abstract Patient is a 38-year-old man who developed sudden onset of pain in the upper left arm while carrying a case of water. The pain recurred periodically over the next few months. He presented to the emergency department where imaging showed a pathologic fracture in the proximal left humerus through a lytic lesion. Laboratory testing showed: calcium 13.8 mg/dL (8.5–10.1 mg/dL), 25-OH vitamin D 8 ng/mL (31–100 ng/mL), parathyroid hormone 1583.1 pg/mL (18.5–88 pg/mL). Neck ultrasound showed a complex nodule in the left neck measuring 4.2 x 1.7 x 2.5 cm. Subsequent biopsy of the left arm lesion showed a Brown tumor. The patient was referred to endocrinology. There was concern for parathyroid carcinoma given the elevated parathyroid hormone and large neck lesion. Technetium-99m sestamibi scan showed a 4.1 x 2.6 x 2.5 cm mass posterior to the left thyroid lobe. He subsequently underwent left hemithyroidectomy and parathyroidectomy. Pathology revealed a 3.1 cm parathyroid mass with scattered fibrous bands, foci of prominent nucleoli and foci of sheet-like trabecular and spindled architecture. It was without necrosis, lymphovascular invasion, perineural invasion, increased mitotic activity, atypical mitoses or invasion into adjacent structures. Thus, it was classified as an atypical parathyroid adenoma. Primary hyperparathyroidism is the third most common endocrine disorder, but fewer than 2% occur as a result of an atypical parathyroid adenoma. Patients with an atypical parathyroid adenoma tend to have a more dramatic clinical presentation compared to those with classic adenoma. We see higher calcium and parathyroid hormone levels. Atypical parathyroid adenomas are classified as such due to other features which place the lesion at a higher risk for malignant behavior including higher mitotic activity, adherence to adjacent structures, banding fibrosis and a growth pattern that is either solid or trabecular. In contrast to parathyroid carcinoma, atypical parathyroid adenomas do not show invasion into the surrounding tissues or lymphatic/vascular vessels, and there should be no evidence of metastatic disease. The exact definition of an atypical parathyroid adenoma varies among institutions, and recognition of the defining features (or lack thereof) necessitates an experienced endocrine pathologist. Due to their uncertain malignant potential, patients with an atypical parathyroid adenoma should undergo routine surveillance for recurrence. Routine follow-up of calcium, parathyroid hormone and imaging is indicated to detect for recurrence. A CDC73 germline mutation is associated with recurrence compared to CDC73-negative patients. Parafibromin expression is also thought to play a role although studies so far have had discrepant results.

scholarly journals Benign, Malignant or Something in Between: A Rapidly Developing Atypical Parathyroid Adenoma

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A183-A184
Author(s):  
Albana Sykja ◽  
Ye Lynn Ko ◽  
Rajeev Raghavan ◽  
Harit Buch
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Adenoma ◽  
Parathyroid Carcinoma ◽  
Malignant Potential ◽  
Thyroid Lobe ◽  
Uncertain Malignant Potential ◽  
Parathyroid Adenomas ◽  
Atypical Parathyroid Adenoma ◽  
Left Thyroid Lobe

Abstract Introduction: Although Primary Hyperparathyroidism (PHPT) is the third most common endocrine disorder, parathyroid carcinoma and atypical parathyroid adenoma are the rarest of endocrine tumours. The true incidence of atypical parathyroid adenomas has been elusive to endocrinologists since it is not possible to differentiate clinically between parathyroid carcinoma and atypical parathyroid adenomas before histological analysis. Atypical parathyroid adenoma represents a group of an intermediate form of parathyroid neoplasms with uncertain malignant potential. The majority of patients present with hypercalcaemia, however the development of atypical adenoma in patients with known PHPT is extremely rare. Clinical Case: A 78-year-old gentleman presented at the emergency department with lethargy and slurred speech which had started 1 week ago. Blood tests revealed severe hypercalcaemia. (Ca 4.98 mmol/L, PTH 114.2 pmol/L). The patient had a background of primary hyperparathyroidism which was diagnosed due to incidental mild hypercalcaemia (Ca 2.71 mmol/L, PTH 17.57 pmol/L, 25OH-vitamin D3 55 nmol/L). 2 weeks prior to presentation to the Emergency Department calcium and PTH levels were stable. On clinical examination, he was found mildly confused with no other clinical findings. No precipitating factors were identified. Hydration with IV crystalloids commenced and bisphosphonate IV was given. In view of PHPT Cinacalcet was added to treatment (30 mg BD). While the calcium levels seemed to improve initially, (lowest level achieved Ca 3.05 mmol/L) a week later they started to rise gradually. Hypercalcaemia proved refractory to medical treatment despite concomitant use of aggressive hydration, increased cinacalcet dose, second intravenous bisphosphonate, and intravenous calcitonin. Neck U/S revealed a probable parathyroid adenoma measuring 2cm axially at the inferior pole of the left thyroid lobe. In view of the severity and refractory nature of hypercalcaemia, a PET CT was requested which identified an 18 mm soft tissue mass in the left lower neck posterior to the left thyroid lobe with moderate to intense FDG uptake. There was no evidence of increased uptake elsewhere. The patient required 2 sessions of haemodialysis to maintain calcium levels around 3.5 preoperatively. He underwent parathyroidectomy with histological findings in keeping with atypical parathyroid adenoma. Gradual reduction of calcium levels was noted post-operatively with the lowest on day 10 (1.99) when he was started on oral calcium supplementation. The patient remains under follow-up with normal calcium levels 6 months postoperatively while remains on calcium and vitamin D3 supplements. Conclusion: To our knowledge, this is the only case of a patient with known primary hyperparathyroidism and mild hypercalcaemia, to develop severe parathyroid crisis with refractory to medical management hypercalcaemia within 2 weeks. Prompt surgical intervention remains of paramount importance in the management of these patients. They should have lifelong follow up in the view of uncertain malignant potential of the atypical parathyroid adenoma.

scholarly journals Atypical parathyroid adenomas as a rare cause of primary hyperparathyroidism - in an academic institution experience

2021 ◽  
Author(s):  
Grzegorz Kowalski ◽  
Grzegorz Buła ◽  
Adam Bednarczyk ◽  
Agata Gawrychowska ◽  
Jacek Gawrychowski
Keyword(s):  
Retrospective Study ◽  
Primary Hyperparathyroidism ◽  
Parathyroid Adenoma ◽  
Parathyroid Carcinoma ◽  
Clinical Presentation ◽  
Parathyroid Hyperplasia ◽  
Endocrine Surgery ◽  
Parathyroid Adenomas ◽  
Multiple Gland Disease ◽  
Atypical Parathyroid Adenoma

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

Brown tumour of hyperparathyroidism in the mandible associated with atypical parathyroid adenoma

2000 ◽  
Vol 114 (4) ◽  
pp. 302-304 ◽  
Author(s):  
Oran Goshen ◽  
Sarit Aviel-Ronen ◽  
Shay Dori ◽  
Yoav P. Talmi
Keyword(s):  
Parathyroid Adenoma ◽  
Parathyroid Carcinoma ◽  
Hormone Level ◽  
Parathyroid Tissue ◽  
Bone Tumour ◽  
Blood Analysis ◽  
Solid Mass ◽  
Brown Tumour ◽  
First Case ◽  
Atypical Parathyroid Adenoma

The brown tumour of hyperparathyroidism is a localized bone tumour and an uncommon manifestation of hyperparathyroidism. A 27-year-old woman presented with a mandibular 8 × 10 cm solid mass diagnosed as central giant cell granuloma. Chemical blood analysis revealed increased serum calcium levels of 12.46 mg/dL and the parathyroid hormone level was 124 pg/dL. The patient underwent surgery with removal of a parathyroid mass. Histologically, this parathyroid tissue was seen to be limited by a fibrous capsule with morphological features consistent with atypical parathyroid adenoma. The mandibular tumour has receded and the patient declined further procedures. This is the first case reported of brown tumour as the primary manifestation of an atypical parathyroid adenoma, a lesion that shares some features with parathyroid carcinoma without the unequivocal properties of malignancy.

Do patients with atypical parathyroid adenoma need a close follow-up?

2021 ◽  
Author(s):  
Federica Saponaro ◽  
Elena Pardi ◽  
Laura Mazoni ◽  
Simona Borsari ◽  
Liborio Torregrossa ◽  
...  
Keyword(s):  
Parathyroid Adenoma ◽  
Serum Calcium ◽  
Good Prognosis ◽  
Histopathological Diagnosis ◽  
Symptomatic Disease ◽  
Asymptomatic Patients ◽  
Parathyroid Adenomas ◽  
Relevant Role ◽  
Atypical Parathyroid Adenoma

Abstract Context Atypical parathyroid adenomas (APAs) are neoplasms with uncertain malignant potential but lack unequivocal histological signs of malignancy. Objective To retrospectively evaluate the clinical and biochemical profiles of patients with APA, the outcome after parathyroidectomy (PTX), and the presence of CDC73 germline and somatic mutations. Design Monocentric study on consecutive patients undergoing PTX for primary hyperparathyroidism (PHPT) between June 2000 and December 2020. Patients Fifty-eight patients with a confirmed histopathological diagnosis of APA. Age and sex-matched controls with parathyroid adenoma (PA) were also included. Results Fifty-four patients had sporadic PHPT and four familial isolated hyperparathyroidism (FIHP). Thirty-four patients (59%) had a symptomatic disease. Serum calcium and PTH levels were significantly higher in symptomatic compared to asymptomatic patients (P=0.048 and 0.008, respectively). FIHP patients were younger than the sporadic counterpart (30±17yr vs. 55±13 yrs). APA patients had significantly higher serum calcium and PTH levels and lower 25(OH)D concentration, BMD and T-score at 1/3 distal radius compared to those with PA. Four of 56 APA patients displayed a CDC73 germline mutation. No somatic CDC73 mutation was identified in 24 tumor specimens. The mean follow-up after surgery was of 60±56.4 months. All but six patients (90%), five with apparently sporadic PHPT and one with FIHP, were cured after surgery. Conclusions The large majority of patients with APA, despite a moderate/severe phenotype, have a good prognosis. Germline CDC73 mutation-positive patients had a higher rate of persistent/recurrent disease. CDC73 gene alterations do not seem to have a relevant role in the tumorigenesis of sporadic APA.

scholarly journals MGMT Promoter Methylation and Parathyroid Carcinoma

2019 ◽  
Vol 3 (11) ◽  
pp. 2114-2122
Author(s):  
Sara Storvall ◽  
Eeva Ryhänen ◽  
Ilkka Heiskanen ◽  
Tiina Vesterinen ◽  
Frank V Bensch ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Adenoma ◽  
Promoter Methylation ◽  
Parathyroid Carcinoma ◽  
Methylation Status ◽  
Mgmt Promoter Methylation ◽  
Promoter Methylation Status ◽  
Mgmt Promoter ◽  
Atypical Parathyroid Adenoma ◽  
Mgmt Promoter Methylation Status

Abstract Context Parathyroid carcinoma (PC) is extremely rare. Prognosis is poor, with no known evidence-based systemic therapies. We previously reported complete remission in a patient with metastasized parathyroid carcinoma and high tumor MGMT promoter methylation status who was treated with temozolomide. Objective To study MGMT promoter methylation status in an additional set of aggressive parathyroid tumors. Design/Setting The study included 12 patients: 7 with sporadic and 5 with familial primary hyperparathyroidism (two of the latter carried a CDC73 gross deletion). Patient 9 is the previously described patient with PC and high MGMT methylation status. Her daughter (patient 12) had surgery for severe primary hyperparathyroidism due to atypical parathyroid adenoma during pregnancy. Eleven patients thus had PC and one had atypical parathyroid adenoma. MGMT promoter methylation status was determined from DNA extracted from primary (n = 10) or metastatic (n = 2) tumors. A mean methylation level >20% was considered high. Patient 11 had metastatic PC and received temozolomide cycles. Results Only the previously published patient (patient 9) had high tumor MGMT promoter methylation status. This was not a characteristic of the atypical parathyroid adenoma of the daughter (patient 12). Patient 11 (CDC73 intragenic deletion) has disseminated PC, low MGMT promoter methylation, and stable disease on follow-up after temozolomide treatment. Conclusion High MGMT promoter methylation status seems rare in PC. However, as demonstrated in other neuroendocrine tumors, some patients with disseminated PC might benefit from temozolomide. Demonstration of high methylation status could be a predictor of positive response to temozolomide treatment.

scholarly journals Atypical parathyroid adenoma with multiple brown tumors as initial presentation: A rare entity

2017 ◽  
Vol 32 (2) ◽  
pp. 133 ◽  
Author(s):  
ManishiL Narayan ◽  
VS Krishna Mohan ◽  
Arun Mukka ◽  
Bharath Bachimanchi ◽  
AmitKumar Chowhan ◽  
...  
Keyword(s):  
Parathyroid Adenoma ◽  
Initial Presentation ◽  
Rare Entity ◽  
Atypical Parathyroid Adenoma ◽  
Brown Tumors

scholarly journals Genetic Alteration Profiles and Clinicopathological Associations in Atypical Parathyroid Adenoma

2021 ◽  
Vol 2021 ◽  
pp. 1-9
Author(s):  
Xinxin Mao ◽  
Yan Wu ◽  
Shuangni Yu ◽  
Jie Chen
Keyword(s):  
Parathyroid Adenoma ◽  
De Novo ◽  
Sequencing Analysis ◽  
Sequencing Data ◽  
Genomic Alterations ◽  
Parathyroid Tumors ◽  
Formalin Fixed Paraffin ◽  
Parathyroid Adenomas ◽  
Formalin Fixed Paraffin Embedded ◽  
Atypical Parathyroid Adenoma

Genomic aberrations associated with atypical parathyroid adenoma (AA) are poorly understood. Thus, herein, we sought to expand our current understanding of the molecular basis of atypical parathyroid adenomas. We analyzed 134 samples that had been surgically obtained from parathyroid tumors, including parathyroid carcinomas, atypical parathyroid adenomas, and parathyroid adenomas. The tumors were harvested from formalin-fixed, paraffin-embedded tissues. Fifteen tumor-related genes from recently published genome sequencing data were subjected to targeted sequencing analysis, and an average sequencing depth of 500x was achieved. Sixteen (16/50, 32%) AA tumors harbored at least one of the following genomic alterations: CDC73 (12, 24%), EZH2 (4, 8%), HIC1 (1, 2%), and CDKN2A (1, 2%). Our study identified, for the first time, a relatively high frequency of genomic alterations in patients with AA in a Chinese population. This suggests that AA arises de novo, rather than developing from a parathyroid adenoma. Altogether, these findings will improve our understanding of the malignant potential of parathyroid tumors at the molecular level.

scholarly journals SAT-379 Giant Parathyroid Adenoma

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Sima Saberi ◽  
Matthew J Wasco ◽  
Ramin Behjatnia ◽  
Beth Kimball
Keyword(s):  
Vitamin D ◽  
Parathyroid Adenoma ◽  
Serum Calcium ◽  
Parathyroid Carcinoma ◽  
Width Ratio ◽  
Normal Range ◽  
Urine Calcium ◽  
Spot Urine ◽  
Parathyroid Adenomas ◽  
25 Oh Vitamin D

Abstract Giant parathyroid adenoma Background: Primary hyperparathyroidism is the most common cause of hypercalcemia. On ultrasound PTH adenomas are typically homogenous, hypoechoic, oval or bean-shaped with peripheral vascularity. Clinical Case: A 60 year old woman with a history of calcium oxalate nephrolithiasis presented with fatigue, worsening depression, body aches of 3 months duration. Labs showed a serum calcium 11.1 mg/dl (normal range 8.5–10.1 mg/dl), PTH 114.3 pg/ml (normal range 12–88 pg/ml), 25 OH Vitamin D 11 ng/ml (normal range above 29 ng/ml), alkaline phosphatase 137IU/L (normal range 27–120 IU/L), spot urine calcium 34.8 mg/dl, spot urine creatinine 92.1 mg/dl (estimated 24 hour urine calcium 415 mg/dl). She was started on Vitamin D 1000 IU daily. A PTH scan with SPECT/CT showed a right parathyroid adenoma and possible thyroid nodules. A neck ultrasound demonstrated a left 1.5 cm thyroid nodule and a right 3cm lesion. She underwent FNA of the left thyroid nodule and pathology was suggestive of a benign follicular nodule. She underwent parathyroid gland exploration with resection of the right lesion which was a 3.5 x 2.5 x 1.4 cm right superior 5.68 gm PTH adenoma. Postoperatively her serum calcium normalized to 10.1 mg/dl, PTH was 8.4 pg/ml, 25 OH vitamin D was 15 ng/ml. Her Vitamin D dose was increased. Clinical Lessons: A normal parathyroid gland typically weighs 30–60 mg and is 3–4 mm in size. The differential diagnosis for large parathyroid lesions is parathyroid carcinoma vs giant parathyroid adenoma. Although there is not a definitive size cutoff to define giant parathyroid adenomas, a size greater than 3.5 gm has been used (1). On ultrasound giant parathyroid adenomas are homogenous with smooth borders whereas parathyroid carcinomas are large lobulated heterogeneous hypoechoic lesions (2). A depth/width ratio on ultrasound may be the ultrasound parameter with greatest discriminatory capacity as a depth/width ratio greater than or equal to 1 had 94% sensitivity and 95% specificity for parathyroid carcinoma (2). Whether vitamin D deficiency is a risk factor for the development of large parathyroid glands is controversial as there has been conflicting data on this (1,3). Because there is no serum calcium level that distinguishes parathyroid carcinoma from a parathyroid adenoma neck ultrasound may be a helpful tool in evaluating these patients. References: 1. Spanhemier PM, Stoltze AJ, Howe JR, et al. Do giant PTH adenomas represent a distinct clinical entity? Surgery. 2013 Oct; 154(4):714–719. 2. Hara H, Igarashi A, Yano Y, et al. Ultrasonagraphic features of PTH carcinoma. Endocr J. 2001 April 48(2):213–217. 3. Rao DS, Honasoge M, Divine GW, et al. Effect of vitamin D nutrition on PTH adenoma weight: pathogenetic and clinical implications. J Clin Endocrinol Metab. 2000 Mar 85(3): 1054–1058.

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