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)

An extremely rare case of metachronous primary hyperparathyroidism (PHP) due to a parathyroid adenoma and subsequent carcinoma with local lymphatic spread is presented herein. A 55-year-old woman was operated on for a parathyroid adenoma in the right inferior gland. Thirteen years after the first operation, she was again hospitalized for hypercalcemia and the presence of a hard mass in the right anterior neck region. Exploratory surgery and a histological examination of the resected tumor provided evidence of a parathyroid neoplasm in the right superior gland but the malignancy was equivocal. Postoperatively, her serum parathyroid hormone (PTH) level remained at 1.5-fold the upper limit of the normal range, and hypercalcemia again gradually developed. The results of higher positive rates by Ki-67 immunohistochemical staining and an aneuploid pattern by a flow cytometric analysis of the second neoplasm were consistent with a histological diagnosis of carcinoma, and she therefore underwent further surgery. A radical neck dissection revealed two lymph node metastases which were both successfully removed. The postoperative serum PTH and calcium levels then returned to within the normal ranges. These findings indicate the usefulness of Ki-67 immunohistochemical staining and a flow cytometric analysis for differentiating malignant lesions from benign parathyroid lesions, and the importance of surgically treating cases limited to local regions without distant metastasis.
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PMID:Metachronous primary hyperparathyroidism due to a parathyroid adenoma and a subsequent carcinoma: report of a case. 1175 85

A case of multiple endocrine neoplasia syndrome type-2A (MEN-2A) with primary hyperparathyroidism and medullary carcinoma of thyroid initially treated by surgery is reported. The presence of pulmonary nodules six years after the initial treatment was thought to be related to metastasis of medullary carcinoma, and the increase in serum calcium concentrations was assumed to be caused by persistence of parathyroid adenomatous or hyperplastic tissue. The patient underwent surgery again and the pulmonary nodules were confirmed to be metastases of a parathyroid carcinoma, a very rare entity in MEN-2A syndrome.
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PMID:MEN-2A syndrome and pulmonary metastasis. 1179 76

Parathyroid carcinoma accounts for 0.5 to 4.0% of cases of primary hyperparathyroidism. The prognosis depends largely on the extent of successful resection at the time of initial operation. Therefore, early diagnosis before surgery is important. We report 3 cases of primary hyperparathyroidism. The first patient, a 20-year-old uremic female, had refractory hypercalcemia after 5 years of hemodialysis treatment. Hypercalcemia persisted despite repeated parathyroidectomy. Pathology revealed diffuse hyperplasia of the parathyroid glands with focal adenomatous changes. Multiple pulmonary metastases were found later. The second patient, a 45-year-old female with history of nephrolithiasis, presented with severe osteoporosis. She underwent repeated parathyroidectomy for local recurrence. Pathology disclosed typical features of parathyroid carcinoma with adjacent lymph node metastasis. The third patient, a 34-year-old male, had recurrent episodes of extremity fracture and hypercalcemia with palpable neck mass. He underwent resection of the parathyroid tumor. Vascular and capsular invasions were noted microscopically. All three patients were relatively young and had extremely high intact parathyroid hormone (iPTH) level (15 to 31 times the upper limit of normal). The first patient died of hypercalcemia and respiratory failure and the other 2 were treated successfully with surgical excision and, in case 2, combined chemotherapy and radiotherapy. The latter 2 patients had no recurrence during 18 months and 14 months of follow-up, respectively. Our experience with these cases suggests that the combination of the following characteristics are highly suggestive of parathyroid carcinoma: young age, palpable neck mass, concomitant renal and skeletal disease, and extremely high iPTH level in patients with PTH-dependent hypercalcemia.
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PMID:Variability of clinical presentations in three cases of parathyroid carcinoma. 1283 92

About 25% of patients with ZES have MEN 1. Except 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 levels are also similar whether in the basal state or after secretin stimulation. Primary hyperparathyroidism (PHPT) exists in the majority of ZES-MEN 1 patients, 30% have pituitary adenoma (prolactinomas for half), 30% adrenal involvement, 25 to 30% have EC-Lomas; 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 tumors. The spread of the disease: metastases to the liver (LM), mediastinum, bones, is evaluated at best by Octreoscan. 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 case of associated life-threatening condition such as insulinoma and has been advocated to prevent LM development in patients with large pancreatic tumor(s). However although, indeed, the size of the tumor, when located in the pancreas > 3 cm, favours metachronous LM occurrence, surgery, in our experience, has not been able to prevent LM development. Hepatic malignancies remain however the most pejorative prognostic determinant for survival and raise the most difficult therapeutic challenge. Surgery is the best option whenever feasible; specific chemotherapy and chemo-embolisation have not conclusively achieved definite successes. Long-term octreotide treatment, however, has been shown recently to obtain tumour stabilisation. Internal irradiation with 90 Ytrium-labelled octreotide is a new promising option, presently under evaluation (Novartis European trial). Preliminary results are promising.
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PMID:[Diagnostic and therapeutic strategies in Zollinger-Ellison syndrome associated with multiple endocrine neoplasia type I (MEN-I): experience of the Zollinger-Ellison Syndrome Research Group: Bichat 1958-1999]. 1514 2

A 32-year-old woman sustained a minor sliding accident with moderate to severe pain about the left distal thigh and hip and the right shoulder. Radiographs showed marked osteopenia, multiple osteolytic bone lesions, and a pathologic fracture of the left distal femur. Surgical intervention was conducted for the pathologic fracture, and the pathological findings confirmed the diagnosis of primary hyperparathyroidism with osteitis fibrosa cystica. We report this rare case here because it may be mistaken for neoplastic metastatic disease on radiographs and bone scan. The pathologic fracture usually heals and the bone mineral density improves significantly within 6 months after parathyroidectomy.
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PMID:Pathologic fracture of the distal femur in osteitis fibrosa cystica simulating metastatic disease. 1516 33

Parathyroid carcinoma is a rare cause of primary hyperparathyroidism, and the efficacy of medical therapy and chemo- and radiotherapy is poor in recurrent or metastatic disease. We report the first case of PTH immunization in which tumor shrinkage accompanied hormonal, biochemical, and clinical improvements in a patient with metastatic parathyroid carcinoma.A 50-yr-old woman with refractory parathyroid carcinoma and pulmonary metastases was immunized eight times between February 2001 and December 2003 with bovine and modified human PTH fragments and intact human PTH, mixed with Freund's adjuvant. Total and ionized calcium and PTH levels were assayed weekly for 6 months and regularly thereafter. Thoracic computed tomography scans were performed regularly. Antibodies to all PTH fragments were detected after two immunizations. Baseline PTH and total calcium were 213.0 ng/liter and 13.96 mg/dl, respectively, and remained elevated during the first three immunizations. From the fourth immunization onward, PTH and calcium decreased, and the patient's clinical condition improved markedly. PTH and calcium levels have remained controlled for more than 24 months, and the sizes (surface area) of pulmonary metastases decreased from baseline by 39-71%. This is the first evidence that PTH immunization not only can improve clinical, hormonal, and biochemical measures in parathyroid carcinoma but also has an antitumor effect.
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PMID:Hormonal and biochemical normalization and tumor shrinkage induced by anti-parathyroid hormone immunotherapy in a patient with metastatic parathyroid carcinoma. 1524 Jun 24

Parathyroid carcinoma constitutes less than 1% of primary hyperparathyroidism. The exact etiology is not known. Prior radiation to neck, chronic renal failure and genetic factors are thought to play a role. The male to female ratio is one. Parathyroid carcinomas are slow growing, have a tendency to recur locally and metastasize late. 95% of parathyroid carcinomas are functioning. The major distinguishing features of malignant hyperparathyroidism are presence of a palpable mass in the neck and features of severe hypercalcemia. By far the most important test to diagnose primary hyperparathyroidism is serum level of Immunoreactive PTH. The diagnosis of primary hyperparathyroidism is essentially clinical and biochemical. Biopsy is not necessary before definitive surgery. CT scan appears to be the best investigation for detecting the primary tumor, its local extent and metastases. Most of the symptoms are attributable to hypercalcemia, which needs to be treated aggressively. Early surgery with 'en bloc' resection of the tumor is the only potentially curative treatment. Parathyroid carcinoma is traditionally said to be resistant to radiotherapy. Various chemotherapeutic agents have been used with partial anecdotal responses. The 5-year survival is about 50% and 10-year survival varies from 13-49%.
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PMID:The carcinoma of parathyroid gland. 1531 9

Parathyroid carcinoma (PC) is an uncommon finding, accounting for only 1-2% of patients with primary hyperparathyroidism (HPT), but a relatively higher incidence has been reported in Italy and Japan. The etiology of the tumour remains unclear, but molecular analysis studies have hypothesised the involvement of mutations of several genes in the pathogenesis of PC, including the oncogene cyclin Dl or PRADI located at the chromosome 13, the retinoblastoma and the p53 tumour suppressor gene. The clinical presentation of patients with PC is mainly related to the increased secretion of PTH rather than to the tumour burden. The pre-operative diagnosis of malignancy is very difficult to obtain, and, thus, intra-operative recognition of PC is mandatory. However, reliable signs of malignancy are rarely detectable. Probably, only vascular invasion, that correlates with tumour recurrence and metastases, should be considered useful in confirming malignancy, although both Ki-67 and Cyclin D1 have been recently used to aid in the definitive diagnosis. The en bloc resection of the tumour, together with ipsilateral thyroid lobe and adjacent structures, only if involved, avoiding any capsular rupture of the mass, represents the gold standard of surgical treatment of patients. Although the PC has traditionally been considered as a radioresistant tumour, there are some retrospective data holding a possible benefit from post-operative irradiation. No cytotoxic regimen with proven efficacy is currently available for patients with PC, but since hypercalcemia is ultimately the most frequent cause of death, several studies have suggested the usefulness of bisphosphonates (i.e., clodronate, pamidronate and zoledronate), calcitonin, and calcimimetic agents (i.e., cinacalcet) in patients with PC and severe hypercalcemia. In conclusion, PC is a rare malignancy and the NCDB survey reports an overall five- and ten-year survival rate of 85% and 49%, respectively. However, it is very difficult to predict the clinical behaviour of patients with PC and probably the ultimate prognosis depends on successful resection of the tumour at the initial surgery.
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PMID:Parathyroid cancer: etiology, clinical presentation and treatment. 1721 44

In parathyroidectomy, it has been recognized that a shift to a minimally invasive procedure may be accompanied by a possibility of missing thyroid pathology. However, only a few findings concerning preoperative thyroid evaluation have been reported. We investigated the prevalence of concomitant thyroid pathology by preoperative neck ultrasonography (US) in patients with primary hyperparathyroidism. There were 85 patients (66 women, 19 men; mean age 57 years) in the study group. The mean preoperative calcium level was 11.2mg/dL, and the mean intact parathyroid hormone level was 206 pg/mL. All patients underwent neck US following fine-needle aspiration biopsy (FNAB). Of the 85 patients, 21 (24.7%) had thyroid nodules. Among 21 patients with thyroid nodules, 9 (10.6%) had malignant thyroid tumors, while 12 (14.1%) patients had benign thyroid nodules including multinodular goiter. Of the 9 patients with malignant thyroid nodules, 4 had papillary carcinomas with lymph node metastases. The prevalence of thyroid disease associated with hyperparathyroidism is high, and evaluation of the thyroid pathology by US enables the shift from bilateral neck exploration to the minimally invasive parathyroid surgery.
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PMID:Preoperative evaluation of thyroid pathology in patients with primary hyperparathyroidism. 1727 51

With over 2 000 articles published on thyroid cancer between January 1, 2006 and September 10, 2007 it is difficult to offer an updated and complete review on this malignancy. Thus, I elected to summarize papers published in 2007 on topics frequently overlooked in other reviews or books, and papers that are likely to be followed by interesting developments. Papers include: 1) the accuracy and currency of websites on thyroid cancer; 2) the detection of the V600E BRAF mutation in very small papillary thyroid cancers that are detected histologically; 3) the relationship between thyroid cancer and Hashimoto's thyroiditis or hepatitis C virus, an association that appears to be nonrandom; 4) the not negligible frequency of coexistence of thyroid cancer with primary hyperparathyroidism; 5) the value of ultrasound elastography of thyroid nodules in distinguishing malignant form benign lesions; 6) the value of percutaneous ethanol injection in the treatment of thyroid or nodal recurrences of thyroid cancer; 7) the relatively benign course of intrathyroid metastases from renal cell carcinoma; 8) the exceedingly rare thyroid paraganglioma, though the rate of reports has increased recently; and 9) the increase in serum calcitonin caused by chronic alcoholism, an increase that cannot be reversed by three weeks of alcohol weaning.
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PMID:Update on thyroid cancer. 1849 Dec 51


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