Clinical characteristics of the patients with asymptomatic primary hyperparathyroidism / 中南大学学报(医学版)

OBJECTIVES@#The prevalence of asymptomatic primary hyperparathyroidism (PHPT) in China is lower than that in European and American countries and the study about the characteristics of asymptomatic PHPT was rare in China. This study aims to explore the characteristics of asymptomatic PHPT.@*METHODS@#Clinical data of 150 patients with PHPT confirmed by operation and pathological examination were retrospectively analyzed. The patients were assigned into a symptomatic PHPT group (@*RESULTS@#The proportion of adenomas was higher than that of adenocarcinoma in the asymptomatic PHPT group. The proportion of the first diagnosis due to hypercalcemia found via biochemical examination in the asymptomatic PHPT group was higher than that in the symptomatic PHPT group (76.92% vs 25.81%, @*CONCLUSIONS@#Only a minority of PHPT patients are asymptomatic. Compared with the symptomatic PHPT patients, the primary cause of diagnosis is hypercalcemia, the duration of diagnosis and the diameter of parathyroid gland are shorter, the levels of serum calcium, and PTH are lower, the proportion of adenomas, vitamin D, and the BMD of L

Ultrasound diagnosis of type 2 multiple endocrine neoplasm: A report of 2 cases / 第二军医大学学报

ObjectiveTo explore the pathogeneic characteristics and ideas of ultrasonic diagnosis of type 2 multiple endocrine neoplasm (MEN-2) , so as to improve the clinical diagnosis rate. Methods The clinical data of 2 patients with pathologically confirmed MEN-2 in Changzheng Hospital of Naval Medical University (Second Military Medical University) were retrospectively analyzed. The pathogenic characteristics of 2 patients were summarized. The process of ultrasound diagnosis was discussed, and the ideas of ultrasound diagnosis was summarized. ResultsPatient 1 presented with hypertension. Abdominal ultrasonography showed multiple pheochromocytomas in the bilateral adrenal glands, which attracted attention because of bilateral involvement. Further examination of other endocrine glands revealed that the right upper and middle thyroid junction had an occupation, and there was no abnormality in the pancreas. Ultrasound finally diagnosed medullary thyroid carcinoma. Patient 2 had recurrent urinary calculi for many years. Considering its symptoms might be caused by hyperparathyroidism, we performed ultrasonography and found hyperplasia of parathyroid gland and medullary thyroid cancer. Therefore, we further examined several endocrine organs such as pancreas and adrenal gland, and finally found pheochromocytoma of adrenal gland. Based on the different clinical symptoms of the 2 patients, we made accurate diagnoses through ultrasound examination of endocrine glands associated with multiple lesions combined with pathological results. ConclusionMEN-2 has a variety of clinical manifestations and is difficult to diagnose. It is helpful for early diagnosis of MEN-2, a multi-organ involvement disease, by mastering its pathogenic characteristics, clinical characteristics and ultrasound diagnosis ideas.

Hiperplasia paratiroidea en un adolescente / Parathyroid hyperplasia in an adolescente

El hiperparatiroidismo primario esporádico es una de las causas de hipercalcemia en la edad pediátrica. Constituye una entidad muy poco frecuente, más aún si es debido a hiperplasia paratiroidea. Se presenta el caso de un adolescente con historia de litiasis renal, hipercalcemia asociada a cifras elevadas de la hormona paratiroidea, e imágenes sugestivas de adenoma en paratiroide inferior izquierda por gammagrafía con sestamibi marcado con tecnecio-99m. Se realizó exéresis quirúrgica de la glándula afectada. El examen anatomo-patológico de la pieza arrojó hiperplasia paratiroidea. La presentación de este caso contribuye al reconocimiento del hiperparatiroidismo primario, entre las posibilidades diagnósticas al evaluar un paciente pediátrico con hipercalcemia(AU)

Sporadic primary hyperparathyroidism is one of the causes of hypercalcemia in pediatric ages. A very uncommon condition, it is even less frequent when caused by parathyroid hyperplasia. A presentation is provided of the case of an adolescent with a history of renal lithiasis, hypercalcemia associated to high parathyroid hormone values, and imaging suggestive of lower left parathyroid adenoma by gammagraphy with sestamibi marked with technetium-99m. Surgical exeresis of the affected gland was performed. Anatomopathological examination of the piece revealed the presence of parathyroid hyperplasia. This case presentation contributes to the recognition of primary hyperparathyroidism as one of the diagnostic possibilities when evaluating a pediatric patient with hypercalcemia(AU)

Hiperplasia paratiroidea ectópica de doble localización, intratiroidea e intratorácica / Double ectopic parathyroid hyperplasia, intrathyroidal and intrathoracic

Se presenta el caso de una paciente sometida satisfactoriamente a hemitiroidectomía izquierda por diagnóstico de bocio nodular. El estudio anátomo-patológico de la pieza operatoria informó que el aumento de tamaño del lóbulo tiroideo izquierdo se debía a glándula paratiroides hiperplásica intratiroidea. Con este resultado se solicitó gammagrafía paratiroidea, en la que se encontró un área de captación en la región torácica derecha. Se concluyó que se trató de hiperplasia paratiroidea ectópica de doble localización, intratiroidea e intratorácica.

The case of a patient who satisfactorily underwent left hemithyroidectomy for nodular goiter is reported. Pathology study of the specimen stated enlarged left thyroid gland lobe due to hyperplasic intrathyroidal parathyroid. With this result parathyroid gammagraphy was obtained and a right thoracic capture area was reported. Final diagnosis was ectopic parathyroid hyperplasia of double localization, intrathoracic and intrathyroidal.

Ultrasound-guided percutaneous thermal ablation for benign parathyroid nodules / 第二军医大学学报

Objective To establish treatment strategies using percutaneous radiofrequency ablation (RFA) and microwave ablation (MWA) for benign parathyroid nodules and to investigate the related techniques, procedures, safety and efficacy. Methods Percutaneous RFA and MWA were conducted on 317 benign parathyroid nodules in 96 patients using an auto-controlled bipolar electrode system (Celon ProBreath, Germany) and thyroid-specified microwave antenna (Thy-ablation™, China). The ablation strategy, optimal puncture route, protection of vital neck vessels and recurrent laryngeal nerve, reduction of bleeding from core-needle biopsy, indicator for complete therapy, ablation time (AT) for single nodule, and total operation time (TOT) for each case were investigated and analyzed. The focal changes of ablated region on multimode ultrasound, serum parathyroid hormone (PTH) level, serum calcium values, clinical manifestations and pathological alterations were assessed during follow-up. Results Cross section simultaneously demonstrating the ablation target, ablation needles and vital anatomic structures was essential for a safe ablation procedure of parathyroid lesions. Liquid isolating zone maneuver was beneficial for creating safe puncture route and for protecting laryngeal nerves, esophagus and trachea from heat damage. Thermal blocking of blood flow prior to core-needle biopsy effectively reduced bleeding and successfully treated one patient with intensive subcutaneous hemorrhage. Contrast-enhanced ultrasound was an important and essential indicator for a complete therapy. In primary hyperparathyroidism (PHPT) group the TOT for each case was about (572. 47 ± 75. 79) s and AT for single nodule was about (194. 82 ± 46. 39) s, and the numbers in secondary hyperparathyroidism (SHPT) group were (1 548. 47 ± 323.83) s and (217. 55 ± 52. 42) s, respectively. Two months after ablation, the ablated region began to shrink, and RFA was superior to MWA regarding the speed and extent of shrinkage. At the end of twelfth month, ultrasound scanning revealed that the ablated region was completely dissolved in 73. 1%(19/26) of nodules in PHPT group and 53. 6% (156/291) of nodules in SHPT. Pre-ablative 99mTc-MIBI disclosed 93. 1% (295/317) of the parathyroid lesions and none of them were visible after ablation. Ultrasound elastography disclosed hardening of the ablated region, and it gradually became soft, paralleling with shrinkage of the ablated region. Serum PTH level decreased rapidly after ablation, particularly in PHPT patients. In the later course PTH levels had relapse in some patients, but the relapses were quite different in timing and extent between PHPT and SHPT groups. Two cases with hyperparathyroidism crisis regained consciousness two hours after ablation therapy. Conclusion Ultrasound-guided percutaneous thermal therapy of parathyroid benign nodules is a new modality of surgical intervention for hyperparathyroidism. The ablative procedure is precise, accurate, safe, and time-saving. The lesion can be completely inactivated and the absorbed. Rapid dropping of PTH level makes it valuable for emergency treatment of crisis. The relapse of PTH may indicate new parathyroid lesion.

A Case of Asymptomatic Multiple Endocrine Neoplasia Type 1 Detected Incidentally on Health Screening / 대한이비인후과학회지

Multiple endocrine neoplasia type 1 (MEN 1) is characterized by the coexistence of primary hyperparathyroidism, enteropancreatic tumors, and anterior pituitary adenoma. Also adrenal adenoma, lipoma, carcinoid tumors could exist simultaneously on the atypical clinical course of MEN 1. Among these diseases, primary hyperparathyroidism is the most common manisfestation of MEN 1 syndrome. However, it sometimes presents no clinical symptoms and is incidentally detected on medical checkup. A 48-year-old woman, while undergoing a regular medical check-up, was diagnosed with primary hyperparathyroidism. Further studies showed concurrence of pancreatic tumor and adrenal tumor, but there was no pituitary lesion. The patient underwent parathyroidectomy with auto-implantation of parathyroid tissue, pylorus preserving pancreatico-duodenectomy and partial adrenalectomy. The pathological test confirmed it to be parathyroid hyperplasia, well-differentiated pancreatic endocrine carcinoma and adrenal cortical adenoma. We report this atypical and asymptomatic case of MEN 1 with a review of the relevant literature.

Imaging Finding of Multiple Endocrine Neoplasia Type 1: Case Report

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant inherited syndrome with characteristic clinical and radiological manifestations. Many reports on MEN1 have been published; however, no cases of radiologically diagnosed MEN1 have been reported. Therefore, we report on a radiologically diagnosed case of MEN1 with clinical symptoms of gastroduodenal ulcer.

Hiperparatiroidismo primario de origen multiglandular con glándula paratiroides intratiroídea hiperfuncionante / Multiglandular primary hyperparathyroidism with a hyperfunctional intrathyroid gland: case report

We report a 56 years old female, admitted to the hospital for the study of general weakness, constipation, a recent radial fracture, hypercalcemia of 18 mg/dl, hypophosphatemia of 1.2 meq/dl and a serum parathormone (PTH) of 995 pg/ml. A thyroid ultrasound disclosed a nodule of 6 cm diameter. Following emergency management and normalization of the hypercalcemia, that patient was subjected to a total thyroidectomy and parathyroidectomy. The pathological study of the surgical piece showed a multiglandular hyperplasia of the parathyroid glands. One of these was located inside the left thyroid lobe.

Se presenta el caso de una paciente de 56 años que ingresó al hospital por una historia caracterizada por decaimiento, constipación, fractura reciente de radio, hipercalcemia de 18 mg/dl, hipofosfemia de 1,2 meq/ dl y elevación de paratohormona de 995 pg/ml. Además, presentaba un nódulo tiroídeo de 6 cm. Posterior al tratamiento médico de la hipercalcemia, se realizó el tratamiento quirúrgico con el diagnóstico definitivo de hiperplasia paratiroídea multiglandular con una de ellas en ubicación intratiroídea.

A Case of Multiple Endocrine Neoplasia Type 1 Combined with Papillary Thyroid Carcinoma

This is the first report of papillary thyroid carcinoma combined with multiple endocrine neoplasia type 1 (MEN1) in Korea. MEN1 is a hereditary disease comprising neoplastic disorders such as pituitary, parathyroid and pancreatic neuroendocrine tumor, such as gastrinoma. But papillary thyroid cancer was never regarded as its component before in Korea. Herein we present a 39-year-old woman who manifested typical features of MEN1 with a coincidental papillary thyroid carcinoma. Although the family history of MEN1 was definite, her genetic analysis of DNA had revealed no germline mutation in MEN1 gene locus. Unidentified culprit gene unable us further genetic study to find LOH (loss of heterogeneity) in 11q13, the possible explanation of papillary thyroid carcinoma as a new component of MEN1. As we have first experienced a case of MEN1 combined with papillary thyroid carcinoma in Korea, we report it with the review of literature.

A Case of Multiple Endocrine Neoplasia Type 1 with Papillary Thyroid Carcinoma / 대한내분비학회지

This is the first report of papillary thyroid carcinoma combined with multiple endocrine neoplasia type 1 (MEN 1). It is an hereditary syndrome characterized by neoplastic disorders such as pituitary adenoma, parathyroid adenoma or hyperplasia and pancreatic neuroendocrine tumor, such as gastrinoma just like in our case. But sometimes pheochromocytoma, mucosal ganglioneuromas, lipoma, forgut carcinoid and thyroid disease could be accompany the disease, but coincidental papillary thyroid carcinoma was never reported before in Korea. Herein we represent a 39-year-old woman who manifested typical features of MEN 1 with coincidental papillary thyroid carcinoma. Despite with definite family history of MEN 1, her genetic analysis of DNA had not found any germline mutation in MEN 1 gene. Unidentified culprit gene unable further genetic study of finding LOH (loss of heterogeneity) in 11q13, the possible explanation of papillary thyroid carcinoma as a new component of MEN 1. As we have experienced a case of MEN 1 combined with papillary thyroid carcinoma, we report it with the review of literature.

False-Positive Parathyroid Sestamibi in Minimally Invasive Radioguided Parathyroidectomy

A (99m)Tc-sestamibi scan has become the most widely used localizing test for identifying a parathyroid adenoma. Despite its popularity, the effectiveness of (99m)Tc-sestamibi scan for parathyroid localization is still controversial due to the large number of false-positive results. The false positive (99m)Tc-sestamibi scan can be attributed to a thyroid adenoma, nodular hyperplasia, metastatic thyroid cancer and other proliferating thyroid diseases because (99m)Tc-sestamibi is specific to the mitochondrial membrane of cells with high-level metabolic status, and not specific to the parathyroid itself. Minimally invasive radio-guided parathyroidectomy (MIRGP) was performed on a 61 year-old woman. The (99m)Tc-sestamibi focus was completely excised with gamma-probe guidance. However, the frozen pathology showed the excised tissue to be a thyroid papillary carcinoma. We present the unexpected false-positive (99m)Tc-sestamibi in MIRGP, and discuss the considerations in order to reduce the number of false-positive parathyroid (99m)Tc-sestamibi scans.

The Experience of Surgical Management for Parathyroid Hyperplasia / 대한내분비외과학회지

Parathyroid hyperplasia is a pathologic finding that can be found in hyperparathyroidism. Unlike parathyroid adenoma, treatment of parathyroid hyperplasia is still quite controversial. In addition, the relative merits of two alternative surgical approaches-subtotal parathyroidectomy versus total parathyroidectomy with autotransplantation have not been clearyly elucidated. The records of 10 patients who had parathyroid hyperplaisa and who underwent parathyroid surgery at the Department of Surgery, Hanyang University Hospital, between April 1992 and April 2003 were retrospectively reviewed. The 10 patients were comprised of 3 males and 7 females. The age distribution was between 29 and 73 years. The presenting clinical manifestations were associated with bone pain in 8 patients, muscle weakness in 4, headache in 4, gastrointestinal symptoms in 3, renal symptoms in 3 and psychologic symptoms in 2. The serum parathyroid hormone level was elevated in all patients. The serum alkaline phosphatase level was elevated in seven among the ten patients. Histopathologic findings revealed chief cell hyperplasia in all patients. Postoperative transient hypocalcemia occurred in 5 patients and they were supplied with oral calcium and calcitriol for several months. There were no major complications. The results indicate that a subtotal parathyroidectomy can be performed without mortality or morbidity and provides good control of primary parathyroid hyperplasia, A total prathyroidectomy with autotransplantation can be performed without mortality or morbidity and provides good contril of secondary and tertiary parathyroid hyperplaisa.

A case of concomitant hyperthyroidism and hyperparathyroidism due to parathyroid hyperplasia / 대한내과학회지

Concurrent hyperthyroidism and primary hyperparathyroidism in the same patient is rare. In most cases of concomitant hyperthyroidism and primary hyperparathyroidism, serum calcium levels are quite elevated. However, recently we diagnosed a patient who had hyperthyroidism [Triiodothyroxine 6.81 nmol/L (1.23-3.39), Free thyroxine 92.88 pmol/L (10.32-25.80), TSH 0.05 mU/L (0.2-7.0)] and primary hyperparathyroidism [parathyroid hormone 163 pg/mL (12-72)] with mild hypercalcemia 2.78 mmol/L (2.03-2.60)]. We treated this patient with propylthiouracil and pamidronate and normalized her thyroid function and serum calcium levels. We then performed subtotal thyroidectomy and removed two parathyroid glands and clipped one parathyroid gland using a hemoclip. The pathologic diagnosis was confirmed to be Graves' disease and parathyroid hyperplasia. The patient was healthy, and serum levels of PTH, calcium and phosphorus were normal after the operation.

A case of concomitant hyperthyroidism and hyperparathyroidism due to parathyroid hyperplasia / 대한내과학회지

Concurrent hyperthyroidism and primary hyperparathyroidism in the same patient is rare. In most cases of concomitant hyperthyroidism and primary hyperparathyroidism, serum calcium levels are quite elevated. However, recently we diagnosed a patient who had hyperthyroidism [Triiodothyroxine 6.81 nmol/L (1.23-3.39), Free thyroxine 92.88 pmol/L (10.32-25.80), TSH 0.05 mU/L (0.2-7.0)] and primary hyperparathyroidism [parathyroid hormone 163 pg/mL (12-72)] with mild hypercalcemia 2.78 mmol/L (2.03-2.60)]. We treated this patient with propylthiouracil and pamidronate and normalized her thyroid function and serum calcium levels. We then performed subtotal thyroidectomy and removed two parathyroid glands and clipped one parathyroid gland using a hemoclip. The pathologic diagnosis was confirmed to be Graves' disease and parathyroid hyperplasia. The patient was healthy, and serum levels of PTH, calcium and phosphorus were normal after the operation.

A Case of Multiple Endocrine Neoplasia Type I Presenting with a Watery Diarrhea / 대한내분비학회지

MEN1 is characterized by the combined occurrence of tumors of the parathyroids, pancreatic islet cells and the anterior pituitary. Pancreatic islet cell tumors occur in 40% of MEN1 patients. Pancreatic polypeptidomas occur most commonly but they are asymptomatic. Gastrinomas are the second most common type. VIPomas are rare and there has been no report of a case in Korea so far. We encountered a case of pancreatic VIPoma in MEN Type I. A 49 year old man was referred from his local hospital presenting with a sudden onset of an explosive watery diarrhea of 3 months duration. Abnormal findings in his blood chemistry were hypercalcemia and hypokalemia. The 99mTc-sestamibi sintigraphy showed an increased uptake in right lower parathyroid gland. Abdominal CT demonstrated a mass of 6x4 cm in tail of the pancreas and multiple lesions in both hepatic lobes. Serum levels of VIP hormones were elevated. Subtotal parathyroidectomy and subtotal pancreatectomy were done. Postoperatively his symptoms were improved transiently, however the patient showed repetitive attacks of watery diarrhea. So in order to palliate his symptoms, an RF ablation of the metastatic liver masses was performed. After that therapy his clinical symptoms were reduced dramatically. Unfortunately, the patients condition worsened again. Despite of continuous octreotide therapy, interferon and two courses of combination chemotherapy, the hepatic metastases failed to regress and the patient died 10 months after the diagnosis of a metastatic VIPoma. This is the first report of pancreatic VIPoma in MEN type I in Korea.

Ultrasonography of secondary hyperparathyroidism / 第二军医大学学报

Objective: To investigate the ultrasonographic features of hyperplastic parathyroid glands in patients with secondary hyperparathyroidism. Methods: Fifty-nine uremic patients accompanied with hyperparathyroidism underwent high resolution parathyroid ultrasonography. On 3 of them ultrasound-guided parathyroid biopsies were performed. The ultrasonographic manifestations of the hyperplastic parathyroids were compared with the optical histology from biopsy. Results :(1) The overall detection rate of parathyroid glands was 83.1% by high resolution ultrasonography, and among those 93. 9% were found to have 1 to 2 glands detectable. (2) Five kinds of manifestations were found, among which the homogeneous internal hypoechoes and multiple hyperechoic rings of calcification in the parathyroid parenchyma were characteristic. (3) One patient showed coexistence of adenoma hyperechoes and hyperplasia hypoechoes in a single parathyroid gland. (4) Lithonephria occured in the transplanted kidney in a patient received renal treated by a successful transplantation, which was indicative of tertiary hyperparathyroidism. Conclusion: High resolution ultrasonography is sensitive and specific to detect the abnormalities of parathyroid glands in the patients with secondary hyperparathyroidism. It is complementary to the laboratory tests of serum parathyroid hormone and serum calcium, and it is valuable for the diagnosis and followup evaluation of hyperparathyroidism. It may be used for the screening of presence or future development of secondary hyperparathyroidism in uremic candidates.

A Case of Multiple Endocrine Neoplasia Type I Presented with Secondary Amenorrhea and Osteoporosis / 대한내분비학회지

MEN type 1 is characterized primarily by the presence of functioning and nonfunctioning tumors or hyperplasia of the pituitary gland, parathyroid glands, and pancreatic islet cells. Pancreatic islet tumors in MEN type 1 produce different kinds of hormone which were pancreatic polypeptide, gastrin, glucagon, insulin and so on. To date, ten cases had been reported in Korea. We report another case with MEN type 1 having prolatin-secreating pituitary adenoma, parathyroid hyperplasia and insulinoma. A 36-year-old woman was admitted because of long-standing amenorrhea and recently diagnosed osteoporosis. Otherwise, she had been in good health except experiencing one episode of loss of consciousness after skipped meal. The blood chemistries were normal except hypercalcemia and hypophosphatemia. Hormonal studies revealed elevated levels of intact PTH and prolactin and decreased value of estradiol with low LH and FSH. The neck CT revealed 1 cm-sized nodule at posterior portion of right thyroid gland and 99mTc-sestamibi sintigraphy showed a increased uptake in left lower and right lower parathyroid glands. The sella MRI showed 0.7 cm-sized enhanced lesion in the left pituitary gland. The ratio of immunoreactive insulin to glucose was elevated and 3-4 pancreatic masses of variable size were identified by endoscopic ultrasonography and angiography. Subtotal parathyroidectomy and pyrolus-preserving pancreaticoduodenectomy with spleen-preserving distal pancreatectomy was done. Postoperative she had been doing well with normocalcemia and normoglycemia. Transsphenoidal adenonectomy was done 5 months later. Histologic examination of removed tissues revealed a single insulinoma, prathyroid hyperplasia and prolactin-secreating pituitary adenoma.

A Case of Multiple endocrine neoplasia type 2a / 대한내분비학회지

Multiple endocrine neoplasia type2a (MEN type2a) is a dominantly inherited cancer syndrome which is characterized by medullary thyroid carcinoma, pheochromocytoma and parathyroid hyperplasia or adenoma. Recent reports show that DNA analysis will be introduced into screening of MEN type2a families. Regular prospective screening and appropriate surgical intervention can reduce the morbidity and mortality due to MEN type2a. We experienced a case of MEN type 2a in a 46-year-old female patient. She had undergone bilateral adrenalectomy due to pheochromocytoma, followed by a total radical thyroidectomy, which revealed medullary thyroid carcinoma of the both thyroid gland and parathyroid hyperplasia.

A case of primary hyperparathyroidism with hypercalcemic nephropathy in children

Primary hyperparathyroidism is a rare disease in children and is characterized by conspicuous skeletal and renal changes. A 12 year old male patient presented with symptoms of polydipsia, polyuria, general weakness, nausea, and vomiting which had begun 3 months earlier, and showed typical laboratory findings of primary hyperparathyroidism. Confirmatory diagnosis was made by elevated parathyroid hormone concentration in serum, technetium-thallium subtraction scan imaging method and histopathologic finding of chief cell hyperplasia. The laboratory findings revealed elevated levels of BUN, creatinine and decreased GFR. Kidney biopsy showed typical calcium deposits in tubules with marked tubulointerstitial infiltration. After subtotal parathyroidectomy, clinical findings improved remarkably.