scholarly journals Ectopic Atypical Parathyroid Adenoma Presenting With Pancreatitis

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A191-A191
Author(s):  
Paul Shiu ◽  
Caroline Tashdjian ◽  
Tarandeep Kaur
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Adenoma ◽  
Fluid Management ◽  
Anterior Mediastinum ◽  
Intermediate Form ◽  
Diagnostic Challenge ◽  
Histopathological Features ◽  
Parathyroid Neoplasm ◽  
Atypical Parathyroid Adenoma

Abstract Background: Atypical Parathyroid Adenoma (APA) and parathyroid carcinomas (PC) are rare parathyroid tumors (<1%) causing primary hyperparathyroidism. We present a case of ectopic APA in the left anterior mediastinum. Case: A 38 year old male with hyperlipidemia admitted for acute pancreatitis noted to have calcium level of 13.4 mg/dl (8.6–10.3mg/dl) in the absence of family history of hypercalcemia or kidney stones. He was treated with zoledronic acid, intravenous fluids and calcitonin for hypercalcemia. Pancreatitis resolved with fluid management. Calcium on discharge was 9.9 mg/dl. 8 week follow-up labs showed PTH of 420 pg/ml (14–64 pg/ml), calcium 10.2 mg/dl, phosphorus 1.9 mg/dl (2.5–4.5 mg/dl), vitamin D 19 ng/ml (30–100 ng/ml), 24-hour urinary calcium 115 mg/24 hour (55–300 mg/24 hour) and calcium to creatinine ratio 0.41. Hence diagnosis of primary hyperparathyroidism was made but given the degree of calcium and PTH elevation there was a concern for PC. Ultrasound of the neck demonstrated no abnormality. Sestamibi scan showed irregular focal accumulation of radiotracer in the left upper mediastinum suggestive of mediastinal ectopic parathyroid adenoma (EPA). CT chest with contrast showed a 3.9 x 2.2 x 3.4cm lobulated, heterogenous, hypodense, minimally enhancing mass in the left anterior prevascular space. Diagnosis of EPA was made and he underwent left video assisted thoracic surgery. Operative findings showed a multilobular mass at the level of the aortic arch between vagus and phrenic nerves. Intraoperative PTH went from 1124 pg/ml pre-incision to 160 at 15-minute post-excision. Postoperative calcium was 9.6 mg/dl and PTH 51 pg/ml. Final pathology showed 3.7 x 2.5 x 2cm, hypercellular parathyroid with prominent fibrous band and parafibromin retention compatible with APA. Discussion: APA is an intermediate form of parathyroid neoplasm with uncertain malignant potential, showing atypical histological features without evidence of invasive growth. This poses a diagnostic challenge of PC as the histopathological features overlap and requires an expert pathologist to make the diagnosis. Inactivating CDC73 mutations, encoding parafibromin, is the most common genetic abnormality. Loss of parafibromin has been seen in 100% cases of PC and predicts poor prognosis. Due to rarity of the disease there are no clear guidelines on follow up. Based on literature, annual follow up is suggested with biochemical testing and imaging for the first 5 years after surgery and every 2–3 years thereafter due to 3% recurrence rate and potential progression to PC. Cetani et al in 2019 reported 672 cases of APA, only 3 were ectopic in the mediastinum thus making our case rare. APA is a rare entity as biochemical profile and histopathological features overlap with PC and can be misdiagnosed. Greater awareness of APA may improve capture of cases, allowing the development of guidelines to recognize and treat it.

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.

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.

Histopathological Features of Parathyroid Adenoma and 18F-Choline Uptake in PET/MR of Primary Hyperparathyroidism

2022 ◽  
Vol 47 (2) ◽  
pp. 101-107
Author(s):  
Virginia Liberini ◽  
Gregoire B. Morand ◽  
Niels J. Rupp ◽  
Erika Orita ◽  
Désirée Deandreis ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Adenoma ◽  
Choline Uptake ◽  
Histopathological Features

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 Clinical Behaviors of Atypical Parathyroid Adenomas

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A258-A259
Author(s):  
Sarah S Pearlstein ◽  
Kim Eric ◽  
Chomsky-Higgins Kate ◽  
Elham Khanafshar ◽  
Quan-Yang Duh
Keyword(s):  
Parathyroid Adenoma ◽  
Disease Recurrence ◽  
Neck Mass ◽  
Recurrence Rates ◽  
Parathyroid Adenomas ◽  
Long Term Follow Up ◽  
Biochemical Cure ◽  
Atypical Parathyroid Adenoma

Abstract Background: Atypical parathyroid adenoma is a rare entity that share some pathology features of parathyroid carcinoma such as fibrosis. Little is known about the clinical behavior of atypical parathyroid adenomas. Pathologically, it is defined as a hypercellular parathyroid lesion with intratumoral banding fibrosis, mitotic figures, trabecular growth and presence of tumor in the surrounding capsule but lacks unequivocal evidence of invasion, such as invasion to peritumoral vessels, perineural invasion and surrounding structures. Methods: A retrospective study of 15 consecutive patients with atypical parathyroid adenoma treated at a single center between 2010 and 2020 was performed. Patient demographics, clinical characteristics, biochemical profile, indications for surgery, preoperative localization studies, intraoperative findings, histopathological characteristics, disease recurrence or persistence and survival were collected. Results: 7 of 15 were female with a median age of 62 (IQR: 52–67). Five of the 15 patients (33%) were re-operative. No patients presented with palpable neck mass. Presentations were consistent with usual primary hyperparathyroidism. Average calcium on presentation was 11.2, and average PTH was 199. One patient had known MEN1 syndrome and one patient had family history of hyperparathyroidism but had negative genetic testing. Most patients 12/15 had correctly localizing imaging pre-operatively with the other 3 having equivocal or non-localizing studies. Two patients did not have biochemical resolution of hyperparathyroidism, both were re-operative. Of the patients with biochemical cure, 6 did not have follow up beyond 6 months, and 7 patients had long term follow up with persistent biochemical resolution and no recurrence of disease for a median of 4 years (IQR: 3.75–9.25). On review of pathology, no patients had invasive features and all patients had presence of thick fibrous bands or capsule. Conclusion: Patients with atypical adenoma have good response to surgery and low recurrence rates. Reoperation with associated scarring and fibrous bands can confound pathological findings. Our experience shows that patients found to have atypical parathyroid adenoma at their primary operation with resulting biochemical cure can be followed long-term with seemingly indolent and nonaggressive behavior.

scholarly journals RE-OPERATIVE SURGERY FOR RECURRENT PRIMARY HYPERPARATHYROIDISM ASSOCIATED WITH OLIGOMENORRHEA

2015 ◽  
Vol 2 (3) ◽  
pp. 140-143
Author(s):  
Ana Valea ◽  
V. Muntean ◽  
Andra Morar ◽  
Mara Carsote ◽  
Cristina Căpățînă ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Adenoma ◽  
Female Patient ◽  
Clinical Symptoms ◽  
Major Incident ◽  
Operative Surgery ◽  
Parathyroid Adenomas ◽  
History Of ◽  
Normal Calcium

Recurrent primary hyperparathyroidism is characterized by typical symptoms and biochemical recurrence of hypercalcemia after more than 6 months of normal calcium levels after surgery. We report the case of a 39-year-old female patient presenting with menses disturbances who was diagnosed with primary hyperparathyroidism caused by a left inferior parathyroid adenoma at the age of 35. Postoperative 6-month follow-up showed normalization of biochemical and hormonal profiles, with significant improvement of clinical symptoms, dominated by muscle weakness, weight loss and oligomenorrhea. The 18-month follow-up showed elevated PTH and serum calcium levels. Imaging confirmed recurrence of primary hyperparathyroidism by highlighting a right upper parathyroid adenoma. Surgery was performed again and no major incident was seen. The particularity of this case consists in the recurrence of primary hyperparathyroidism in a young patient with no family history of the disease due to asynchronous parathyroid adenomas that were successfully removed in a female patient who in addition to classic complications such as calyceal microlithiasis and osteoporosis presented oligomenorrhea which was resolved spontaneously after the correction of hypercalcemia.

scholarly journals Primary Hyperparathyroidism with Multiple Fractures and Brown Tumors in a 14-year-old Girl: Case Report & Review of Literature

2021 ◽  
Vol 22 (2) ◽  
pp. 130-136
Author(s):  
Nasim Khan ◽  
Nazmun Nahar ◽  
Rawshan Ara ◽  
Fahima Akter Dowel ◽  
Shakila Zaman Rima ◽  
...  
Keyword(s):  
Vitamin D ◽  
Primary Hyperparathyroidism ◽  
Parathyroid Adenoma ◽  
Tumor Resection ◽  
Good Health ◽  
Vitamin D Insufficiency ◽  
Multiple Fractures ◽  
Brown Tumors ◽  
Variable Presentation

Primary hyperparathyroidism (PHPT) presenting as pathological fractures with brown tumors is very rare in children. The rarity and variable presentation of PHPT can make its diagnosis a challenge – especially in the developing countries where malnutrition may mask hypercalcemia. We report a case of a 14–year-old girl who presented with muscle pain, multiple fractures and bone pain. Laboratory tests revealed normocalcemia with vitamin D insufficiency. Subsequent biochemical, radiographic and scintigraphic findings were consistent with primary hyperparathyroidism due to a parathyroid adenoma. Tumor resection was done in a local hospital and histopathology confirmed parathyroid adenoma. Following parathyroidectomy, fractures were treated conservatively with supplementation of calcium and vitamin D. We found her quite capable of self-care and walking in good health at 1 year follow up. Though rare, PHPT-causing brown tumors related skeletal fractures is an important differential diagnosis in the evaluation of patients presenting with multiple foci of radiotracer uptake in bone scintigraphy. Bangladesh J. Nuclear Med. 22(2): 130-136, Jul 2019

scholarly journals Atypical parathyroid adenoma: clinical and anatomical pathologic features

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Alessandro Galani ◽  
Riccardo Morandi ◽  
Mira Dimko ◽  
Sarah Molfino ◽  
Carla Baronchelli ◽  
...  
Keyword(s):  
Parathyroid Hormone ◽  
Primary Hyperparathyroidism ◽  
Parathyroid Adenoma ◽  
Serum Calcium ◽  
Parathyroid Carcinoma ◽  
Ki 67 ◽  
Pathologic Features ◽  
Atypical Parathyroid Adenoma ◽  
High Concentrations ◽  
Group B

Abstract Background Primary hyperparathyroidism is an endocrine pathology that affects calcium metabolism. Patients with primary hyperparathyroidism have high concentrations of serum calcium or high concentrations of parathyroid hormone, or incorrect parathyroid hormone levels for serum calcium values. Primary hyperparathyroidism is due to the presence of an adenoma/single-gland disease in 80–85%. Multiple gland disease or hyperplasia accounts for 10–15% of cases of primary hyperparathyroidism. Atypical parathyroid adenoma and parathyroid carcinoma are both responsible for about 1.2–1.3% and 1% or less of primary hyperparathyroidism, respectively. Methods We performed a retrospective cohort study and enrolled 117 patients with primary hyperparathyroidism undergoing minimally invasive parathyroidectomy. Histological and immunohistochemical examination showed that 107 patients (91.5%) were diagnosed with typical adenoma (group A), while 10 patients (8.5%) were diagnosed with atypical parathyroid adenoma (group B). None of the patients were affected by parathyroid carcinoma. Results Significant statistical differences were found in histological and immunohistochemical parameters as pseudocapsular invasion (p <  0.001), bands of fibrosis (p <  0.001), pronounced trabecular growth (p <  0.001), mitotic rates of > 1/10 high-power fields (HPFs) (p <  0.001), nuclear pleomorphism (p = 0.036), thick capsule (p <  0.001), Ki-67+ > 4% (p <  0.001), galectin-3 + (p = 0.002), and protein gene product (PGP) 9.5 + (p = 0.038). Conclusions Atypical parathyroid adenoma is a tumor that has characteristics both of typical adenoma and parathyroid carcinoma. The diagnosis is reached by excluding with strict methods the presence of malignancy criteria. Atypical parathyroid adenoma compared to typical adenoma showed significant clinical, hematochemical, histological, and immunohistochemical differences. We did not find any disease relapse in the 10 patients with atypical parathyroid adenoma during 60 months of follow-up time.

scholarly journals Microwave ablation as an efficient therapy for primary hyperparathyroidism: Efficacy and predictors of treatment success

2021 ◽  
Author(s):  
Mehmet Sercan Ertürk ◽  
Bulent Cekic ◽  
Isilay Sarı ◽  
Baris Pamuk
Keyword(s):  
Logistic Regression ◽  
Primary Hyperparathyroidism ◽  
Parathyroid Adenoma ◽  
Microwave Ablation ◽  
Treatment Success ◽  
Significant Difference ◽  
Alkaline Phosphate ◽  
The Difference ◽  
Response Group

Background:Microwave ablation has a great potential to treat primary hyperparathyroidism; but its predictors and the therapeutic efficiency are not clear enough and so the more investigations are required. Aim:The purpose of this study was to explore safety and predictors of the MWA efficacy in patients with primary hyperparathyroidism (PHPT) induced by parathyroid adenoma. Methods: Patients with PHPT treated with MWA were included in this study. Depending on the treatment efficacy, patients divided into two groups as response and no response group. Possible predictors as age, gender, Parathyroid adenoma volume, baseline levels of calcium (Ca), phosphorous (P), alkaline phosphate (ALP), vitamin D and location of parathyroid adenoma and the instrumental parameters which are microwave ablation time and power were compared between two groups. The resulting possible predictors the MWA efficacy exhibiting statistically significant difference was investigated by using logistic regression. Results: Thirty-two patients participated into predictor analysis for MWA efficacy. Comparison of the values of response and no response groups in terms possible predictors revealed only the baseline Ca level as the potential predictor of the efficacy of MWA (P <0.05). Further logistic regression results showed the baseline Ca level as insignificant to construct a mathematical model to predict the efficacy of MWA (P = 0.071). Furthermore, a significant difference in time differences was observed after 1 day of the MWA treatments in the levels of Ca, P, and PHT except than ALP, those of which was 30 days later (P <0.001). Later on, the difference stayed steady until the end of follow up. Conclusions:MWA is an effective and safe therapy for in patients with PHPT caused by parathyroid adenoma. Baseline Ca level has been found as potential but detailed investigations revealed none of the explored factors as predictive for the assessed patients. Keywords:Primary hyperparathyroidism, microwave ablation, predictor, efficacy

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