Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the case of a 46-year-old male patient who, after disease-free intervals of five, four and one and a half years following resection of an 'atypical' parathyroid adenoma in 1982, relapsed with clinical and laboratory recurrence of primary hyperparathyroidism (PHP). Noninvasive, traditional and modern imaging methods localized small distinct metastatic foci in both lungs without evidence of primary thyroid, neck or mediastinal tumor. Three successive bilateral lung nodule excisions resulted in a long PHP remission, while a three month treatment with normal saline infusions, diuretics, calcitonine and pamidronate infusions, following the last recurrence, resulted in moderate improvement of hypercalcemia and hypercalciuria with no effect on both PTH secretion or on the size of new metastatic lung foci. Recurrent mPCa with or without secretion of biologically active PTH is optimally treatable with successive surgical resections of the metastases and intermittent medical treatment to achieve PTH secreting tumor mass reduction and a beneficial metabolic effect.
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PMID:Metastatic parathyroid carcinoma (mPCa): natural history and treatment of a case. 950 19

About 25% of patients with ZES have MEN-1. Except for diarrhoea, less frequent in patients with ZES MEN-1 than in sporadic ZES, and specific MEN-1-related signs, clinical characteristics are similar in both ZES types. Acid output and gastrin level are also similar whether in the basal state or after secretin. Primary hyperparathyroidism (pHPT) exists in the majority of ZES MEN-1 patients, 30% have pituitary adenoma (prolactinomas for half), 30% adrenal involvement, 25-30% have ECLomas: bronchial and thymic carcinoids have probably been underevaluated. Gastrinomas are multiple predominantly located in the duodenal wall, but also in the pancreas in association with clinically silent endocrine tumours. The spread of the disease metastases to the liver (LM), mediastinum, bones, is evaluated best by Octreoscan. Associated endoscopic ultrasonography evaluates the number, size and anatomical characteristics of gastrinomas. Patients without LM have an excellent prognosis. Surgery never cures ZES, but is necessary in cases of associated life-threatening conditions such as insulinoma. Although the size of the tumour, when located in the pancreas >3 cm, favours metachronous LM occurrence, surgery in our experience has not been able to prevent LM development.
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PMID:Diagnostic and therapeutic criteria in patients with Zollinger-Ellison syndrome and multiple endocrine neoplasia type 1. 968 47

The two most common causes of hypercalcemia are malignancy and primary hyperparathyroidism (1 degree HPT). The radiographic presentations and the histological findings on bone biopsy are important for differential diagnosis of underlying diseases. We report a patient with hypercalcemia who presented unusual bone manifestations. A 43 y/o woman was admitted due to right femoral fracture. X-ray on the right tibia revealed several osteolytic cystic lesions with sclerotic rims. Blood biochemistry showed anemia, impaired renal function and hypercalcemia. Multiple osteolytic lesions on the skull and bilateral forearms were also noted. Malignancy, such as multiple myeloma or metastatic cancer was suspected. However, this was excluded because of the absence of M-component on serum protein electrophoresis and the negative finding of plasma cells or other malignant cell on bone biopsy examination. Abdominal sonography demonstrated bilateral medullary nephrocalcinosis. The final diagnosis of 1 degree HPT was made, based on the findings of classic pathological pictures (brown tumor) and the markedly elevated intact parathyroid hormone (1267.4 pg/ml) level. Sonography on the neck and 201Tl/99mTc parathyroid subtraction scan localized a left lower parathyroid tumor and fine needle aspiration confirmed the parathyroid origin. Diagnosis of 1 degree HPT could only be made from recurrent urolithiasis and X-ray picture of osteitis fibrosa cystica in the past. This patient presented the full-blown skeletal changes which are uncommonly seen nowadays. The characteristic sclerotic rims suggesting increased bone formation provides a further important clue for differential diagnosis of 1 degree HPT from other malignancies with osteolytic bone lesions.
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PMID:A patient of primary hyperparathyroidism with full-blown bone changes simulating malignancy. 979 3

The purpose of this study was to estimate clinical validity of a new available immunoradiometric assay for circulating intact human BGP (N-tact Osteo SP) by measuring this protein in a large number of normal subjects and patients with the most common metabolic bone diseases. One hundred normal subjects were studied in order to obtain our normal ranges (4.9 +/- 1.7 ng/ml). The mean values found in 28 patients with primary hyperparathyroidism (17.5 +/- 22.8 ng/ml, P < 0.001), 15 glucocorticoid-treated patients (1.9 +/- 1.5, P < 0. 001), 10 patients with hypoparathyroidism (1.5 +/- 0.7, P < 0.001), 9 with hyperthyroidism (8.3 +/- 3.8, P < 0.001), 8 with skeletal metastases (7.2 +/- 2.3, P < 0.001), and 4 with humoral hypercalcemia of malignancy (2.42 +/- 1.91, P < 0.005) were significantly different from mean values found in normal subjects. Mean decrease of serum osteocalcin T-score values was significantly greater when evaluated by N-tact Osteo SP assay in 15 steroid-treated patients (-1.4 +/- 1.0) and 19 primary hyperparathyroid (PHPT) patients (3.6 +/- 1.9), compared with the mean values obtained with the Elsa-Osteo assay (-0.67 +/- 1.2, P < 0. 002 and 4.3 +/- 2.8, P < 0.04, respectively). We found significant correlations between the global skeletal uptake of 99mTc-methylendiphosphonate and serum BGP levels assayed by both N-tact Osteo SP (P < 0.01) and Elsa-Ost-Nat assay (P < 0.05). Our results indicate that this new immunoradiometric assay for the intact human osteocalcin has the potential for good discrimination between normal subjects and patients with both low and high bone turnover. Furthermore, our findings emphasize the fact that, in the absence of available standardized commercial assays, one should rely on only one assay because different results are obtained by different assays under different clinical conditions.
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PMID:Clinical validation of a new immunoradiometric assay for intact human osteocalcin. 1020 10

Parathyroid carcinoma is a rare cause of primary hyperparathyroidism (1-2%). In this paper a case of parathyroid cancer of the right inferior parathyroid gland cyst (5 cm in diameter) localised mostly retrosternally is presented in a patient suffering from severe hypercalcaemia (Ca--3.7 mmol/l, Ca(++)--1.8 mmol/l), severe bone pains and weakness, huge osteoporosis with following L5 compression fracture and recurrent nephrolithiasis. PTH blood level before surgical treatment was 1243 pg/ml (Norm: 10-60 pg/ml). In the preoperative ultrasonography of the neck, a lesion was found, but it was considered to be a cyst in the inferior pole of the right thyroid lobe. In a fine-needle biopsy from the lesion-colloid mass without any cells was found. In a 99mTc-MIBI scintigraphy of the neck a suspicion of focus lesion in the right superior parathyroid gland was made, which was not confirmed intra-operatively. Parathyroid carcinoma was diagnosed intraoperatively in histological examination. 'En block' resection of the retrosternal parathyroid tumor and right thyroid lobe was performed from the jugular incision. Any enlarged jugular lymph nodes were not found. In a postoperative period a transient hypocalcaemia was present, which disappeared after pharmacological treatment. During 32 months of follow-up neither no features of local recurrence nor distant metastases were found. Calcium level in blood was normal, PTH 216.0 pg/ml, severity of bone pains and weakness lessened, osteoporosis in a course of pharmacological treatment--with no further progress.
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PMID:[Retrosternal parathyroid gland cystic neoplasm as a cause of primary hyperparathyroidism]. 1069 86

This report describes 3 cases of primary hyperparathyroidism (PHP) in which presurgical localization of the culprit lesions was essential. In 2 cases, the initial parathyroid surgery had failed to restore normal levels of either parathyroid hormone (PTH) or calcium (Ca(++)). In each of these cases experienced surgeons performed the initial parathyroid explorations. In one of these 2 cases, a positive 99mTc sestamibi (MIBI) scan was obtained prior to the initial surgery. 11C-methionine-positron emission tomography (PET) scans and magnetic resonance imaging (MRI) studies were performed prior to repeat surgical exploration with these data coregistered (the digital data sets from each modality were spatially aligned). In each case, the 11C-methionine-PET studies identified metabolically active tumors whose anatomic locations were accurately determined on the PET coregistration. The third case had previously undergone a thyroidectomy and modified right radical neck dissection for removal of a thyroid cancer and regional lymph node metastases. Following the diagnosis of PHP, MIBI, thallium, and ultrasound studies were performed and were all negative. 11C-methionine-PET and MRI were performed identifying a parathyroid adenoma in the prior surgical field. In these 3 cases culprit lesions were readily located at surgery, excised and confirmed as parathyroid neoplasms. This 11C-methionine PET-MRI coregistration technique appears to be effective at localizing parathyroid neoplasms in high risk patients with PHP in the setting of initially failed surgery or when PHP occurs following previous regional neck surgery.
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PMID:Three Cases of Primary Hyperparathyroidism (PHP) with Prior Failed Surgery Where Culprit Lesions Were Identified by 11C-Methionine Positron Emission Tomography (PET) and Accurately Localized with PET-MRI Coregistration. 1074 79

The paper presents current diagnostic and therapeutic approach in parathyroid cancer. The suspicion of parathyroid cancer should be taken into account in patients with primary hyperparathyroidism symptoms (and elevated total and ionised calcium blood level and PTH blood level), present palpable cervical mass and enlarged cervical lymph nodes. Further diagnosis should be based on ultrasound of the neck (including power-Doppler ultrasound), subtraction 99mTc-MIBI and 123J scintigraphy, ultrasound-guided fine needle aspiration, and CT or MRI if necessary to assess the stage of the process. The elective surgical procedure should be primary 'en block' excision of the tumour with the unilateral thyroid lobe and the following loco-regional adjuvant radiotherapy. Patients who underwent surgical treatment due to parathyroid cancer require long-term follow-up as the risk of local recurrence or distant metastases (mostly into lungs or bones) remains high even many years after initial operation. Patients with dissemination usually suffer from severe hypercalcemia leading to death if untreated correctly. Thus, so crucial is effective treatment of hypercalcemia (diuresis, biphosphonates, calcitonine, oktreotide).
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PMID:[Parathyroid neoplasm--diagnostic challenge and therapeutic difficulty]. 1090 88

Two cases of women with primary hyperparathyroidism caused by parathyroid cancer were presented. The authors noticed the following characteristic features of primary hyperparathyroidism in the course of the cancer: rich clinical symptomatology usually in form of considerable bone destruction, renal stones and nephrocalcinosis, biochemically very high level of calcium, above 14-16 mg%, threatening with hypercalcemic crisis and considerably higher parathormone serum concentration even up to twenty times above the norm. Parathyroid cancer, more often than adenoma, is a stiff and large neck tumour accessible for palpation. There are no specific biochemical and imagining examination techniques to recognise beyond any doubt the cancer character of primary hyperparathyroidism before operation. The histopathological diagnosis of this cancer is difficult and is not usually done intraoperatively. The recurrences of the malignancy are typical and they require reoperations after stating places of relapse or metastases.
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PMID:[Characteristic features of primary hyperparathyroidism caused by parathyroid cancer--based on 2 cases]. 1110 73

The major phenotypes of multiple endocrine neoplasia type 1 (MEN 1) consist of three lesions characterized by hyperparathyroidism, pituitary tumors, and endocrine pancreatic tumors. The endocrine pancreatic tumors are a significant cause of disease-related mortality in MEN 1. Although symptomatic pancreatic tumors such as insulinoma and gastrinoma should be resected, the management of asymptomatic pancreatic tumors is not established. In asymptomatic pancreatic tumors, the most important factor is the propensity for malignant transformation of the tumors. Although there are no means to foresee it, the size of the pancreatic tumors might be predictive of malignant development in MEN 1. We report here a patient with MEN 1 who had a large asymptomatic pancreatic tumor. The patient (72-yr-old man) was diagnosed with primary hyperparathyroidism and underwent a total parathyroidectomy. Genetic examination showed a germline mutation of the MEN1 gene (E45G). Abdominal magnetic resonance imaging revealed a large (>6 cm) tumor with a heterogeneous pattern in the tail of the pancreas. No metastases of the tumor were evident. Serum levels of insulin, gastrin, and glucagon were normal, and the patient had no symptoms. Operative resection was performed, and microscopic examination revealed that the tumor was an islet cell tumor stained with multiple hormones. This is a case indicating that asymptomatic pancreatic tumors associated with MEN 1 might be indolent independent of their size.
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PMID:Large and asymptomatic pancreatic islet cell tumor in a patient with multiple endocrine neoplasia type 1. 1121 36

The association between primary hyperparathyroidism and nonmedullary thyroid malignancies is well known. There is also, however, some evidence for an association between secondary hyperparathyroidism (SHPT) and thyroid cancer. We report three patients in whom invasive papillary thyroid carcinoma (PTC) was diagnosed before (one case) or at the time of (two cases) parathyroidectomy for SHPT. Three women (ages 23, 54, and 64 years) presented with bone pain and pruritus typical of SHPT. All three patients had biopsy-proven parathyroid bone disease and elevated parathormone levels (664, 1674, and 2051 pg/mL). All underwent subtotal parathyroidectomy and total thyroidectomy without complications. Pathology revealed diffuse parathyroid hyperplasia with multifocal invasive papillary thyroid carcinoma (two cases) and follicular variant of papillary thyroid carcinoma (one case). Two cases were associated with metastatic disease to local lymph nodes. The patients received adjuvant radioactive 131I, and remained tumor free 24 to 36 months after surgery with complete resolution of SHPT. We conclude: 1) PTC may accompany SHPT, 2) PTCs associated with SHPT may be locally aggressive although usually they are early tumors, 3) surgeons need to have an index of suspicion for thyroid tumor when operating on patients with SHPT, and 4) routine removal of the thymus as part of the operation for SHPT may have a secondary benefit in diagnosing PTC in the occasional patient.
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PMID:Secondary hyperparathyroidism: evidence for an association with papillary thyroid cancer. 1137 34


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