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)

In summary, ZES is a syndrome caused by gastrinoma, usually located within the gastrinoma triangle and associated with symptoms of peptic ulcer disease, GERD, and diarrhea. The diagnosis of ZES is made by measuring fasting levels of serum gastrin, BAO, and the secretin stimulation test. Because of the high association of ZES and MEN1, HPT must be excluded by obtaining a serum calcium and parathyroid hormone level. Treatment of ZES consists of medical control of symptoms with PPIs and evaluation for potentially curative surgical intervention. Noninvasive imaging studies including SRS, CT, and MRI should be performed initially to evaluate for metastases and identify resectable disease. Invasive imaging modalities such as EUS may be performed to further evaluate primary tumors. IOUS, palpation, and duodenotomy are used for intraoperative localization of gastrinomas. In patients with MEN1, surgical resection should be pursued only if there is an identifiable tumor larger than 2 cm and after surgery for the primary hyperparathyroidism (3 1/2-gland parathyroidectomy). All patients with resectable localized sporadic gastrinoma should undergo surgical exploration, even those with biochemical evidence but negative imaging studies. Tumor is most commonly found in the duodenum, and the cure rate is high. In patients with liver metastases, surgery should be considered if all identifiable tumor can be safely removed. A multidisciplinary approach including surgical and nonsurgical therapies should be taken in patients with advanced disease.
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PMID:Current management of the Zollinger-Ellison syndrome. 2429 44

The most common cause of hypercalcemia in hospitalized patients is malignancy. Primary hyperparathyroidism most commonly causes hypercalcemia in the outpatient setting. These two account for over 90% of all cases of hypercalcemia. Hypercalcemia can be divided into PTH-mediated and PTH-independent variants. Primary hyperparathyroidism, familial hypocalciuric hypercalcemia, familial hyperparathyroidism, and secondary hyperparathyroidism are PTH mediated. The most common PTH-independent type of hypercalcemia is malignancy related. Several mechanisms lead to hypercalcemia in malignancy-direct osteolysis by metastatic disease or, more commonly, production of humoral factors by the primary tumor also known as humoral hypercalcemia of malignancy that accounts for about 80% of malignancy-related hypercalcemia. The majority of HHM is caused by tumor-produced parathyroid hormone-related protein and less frequently production of 1,25-dihydroxyvitamin D or parathyroid hormone by the tumor. We report the rare case of a patient with hypercalcemia and diagnosed primary hyperparathyroidism. The patient had persistent hypercalcemia after surgical removal of parathyroid adenoma with recorded significant decrease in PTH level. After continued investigation it was found that the patient also had elevated 1,25-dihydroxyvitamin D and further studies confirmed a large spleen mass that was later confirmed to be a lymphoma. This is a rare example of two concomitant causes of hypercalcemia requiring therapy.
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PMID:Multifactorial hypercalcemia and literature review on primary hyperparathyroidism associated with lymphoma. 2471 7

A 45 year-old woman who presented with non-specific neck and shoulder pain, was found to have mild hypercalcaemia, markedly elevated parathyroid hormone levels, and an irregular parathyroid gland on imaging. The patient underwent a parathyroidectomy and the pathology report came back positive for parathyroid carcinoma with muscular invasion. Parathyroid carcinoma is an exceptionally rare cause of primary hyperparathyroidism and can have a poor prognosis due to metastases and a high propensity to recur after resection. Reports of non-functioning parathyroid carcinomas tend to behave even more aggressively. Repeat imaging on this patient showed residual cancer present, so the patient underwent a second surgery with radical neck dissection and has since been doing very well postoperatively. Diagnosis and treatment is challenging and it is critical to continuously follow-up for recurrent disease.
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PMID:Parathyroid carcinoma, a rare cause of primary hyperparathyroidism. 2513 18

Parathyroid carcinoma (PCA), accounting for less than one per cent of all endocrine malignancies, is a rare cause of primary hyperparathyroidism. A diagnosis of parathyroid carcinoma may be challenging in the presence of localised disease and involves a histological diagnosis based on capsular, vascular, or perineural invasion or the presence of metastasis. Distant metastasis remains a rare presentation, with the lung being the most common site. Surgery remains the treatment of choice as radiotherapy and chemotherapy have proved to be of limited benefit in metastatic disease. This case reports suggests that radiofrequency ablation has the potential to be a novel and effective treatment option in these patients.
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PMID:Metastatic parathyroid carcinoma treated with radiofrequency ablation: A novel therapeutic modality. 2532 2

Parathyroid carcinoma is an uncommon malignancy and a rare cause of primary hyperparathyroidism. Although this tumor is capable of metastasis, metastatic disease is very uncommon intracranially, with only seven cases reported in the literature. When intracranial metastases occur, they typically present months to years following the diagnosis of the primary tumor with hypercalcemia refractory to medical conservative treatment. Aggressive surgical resection of all metastases is necessary for control of the disease. We report a case of metastatic parathyroid carcinoma with two intracranial metastatic foci (in the left frontal lobe and left cerebellar hemisphere) identified at the time of the primary tumor diagnosis in a patient who presented with symptomatic hypercalcemia.
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PMID:Parathyroid carcinoma with intracranial metastasis at diagnosis in a patient with uncontrolled hypercalcemia. 2536 37

Parathyroid carcinoma is a malignant neoplasm affecting 0.5 to 5.0% of all patients suffering from primary hyperparathyroidism. This cancer continues to cause challenges for diagnosis and treatment because of its rarity, overlapping features with benign parathyroid disease, and lack of distinct characteristics. The third/second generation PTH assay ratio provides valuable information to distinguish between benign parathyroid disease and parathyroid carcinoma. An abnormal ratio (>1) could indicate a high suspicion regarding carcinoma and metastatic disease. Early en bloc surgical resection of the primary tumour with clear margins remains the best curative treatment. Although prolonged survival is possible with recurrent or metastatic disease, cure is rarely achievable. The efficacy of classical adjuvant therapies, such as radiotherapy and chemotherapy, in management of persistent, recurrent, or metastatic disease has been disappointing. In metastatic disease the goal of therapeutic support is to control the PTH-driven hypercalcemia that represents the primary cause of mortality. Calcimimetics, which are allosteric modulators of the calcium sensing receptor, have a sustained effect in lowering serum calcium levels. Bone anti-resorptive therapy, like intravenous bisphosphonates (pamidronate and zolendronate), or more recently denosumab (fully human monoclonal antibody with high affinity to bind RANK ligand) might be temporarily useful. In a small number of cases treated with anti-PTH immunotherapy, inducing anti-PTH antibodies, promising results have been seen with clinical improvements and decrease of calcemia. In one case metastasis shrinkage has been observed.
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PMID:Parathyroid carcinoma: Challenges in diagnosis and treatment. 2591 Sep 97

Parathyroid carcinoma (PC) is a rare endocrine malignancy and the cause of primary hyperparathyroidism. It is usually associated with a high rate of local and distant recurrence. Laboratory findings and clinical symptoms may be similar to those in parathyroid adenoma. The histological features of PC may also be non-specific and the affected gland is often indistinguishable from a benign lesion. The proper diagnosis is commonly made months to years later when the disease recurs or metastases are present. Therefore, parathyroid carcinoma still remains a diagnostic and management challenge for many physicians. However, there are some features that, in combination, may help in diagnosis. Surgery still remains the only curative treatment, even in metastatic disease. In advanced, non-operable subjects, managing hypercalcaemia and controlling a tumour are the main goals. Morbidity is caused by hypercalcaemia rather than metastases. A multidisciplinary approach with experienced endocrinologists, pathologists, radiologists, nuclear medicine doctors, oncologists, and surgeons is needed to optimize patient outcome.
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PMID:[Primary hyperparathyroidism due to parathyroid cancer - a diagnostic and management challenge]. 2593 Oct 46

Parathyroid carcinoma (PC) is a rare endocrine malignancy. The tumor is mostly functioning, causing severe primary hyperparathyroidism, with high serum calcium and parathyroid hormone (PTH) levels. Nonfunctioning PC is extremely rare. We report a 50-year-old male patient who was referred to our Department for a right thyroid nodule, incidentally detected on carotid Doppler ultrasound scan, with a fine-needle aspiration cytology showing a follicular lesion. At the time of our evaluation, neck ultrasound showed a 1.3 cm right hypoechoic thyroid nodule with irregular margins and the absence of enlarged bilateral cervical lymph nodes. Thyroid function tests were normal. Serum calcium was normal and plasma PTH slightly above the upper limit of the normal range. The patients underwent right lobectomy. The intraoperative frozen-section pathological examination raised the suspicion of a PC. Definitive histology showed a markedly irregular infiltrative growth of the tumor with invasion of the thyroid tissue and cervical soft tissues. Immunostaining for thyroglobulin was negative, whereas staining for chromogranin A and PTH showed a strong reactivity. Based on the microscopic findings and the immunohistochemical profile, the tumor was diagnosed as a PC. Postoperative serum calcium and phosphate levels were in the normal range. One month after surgery, serum calcium and PTH were normal. Neck ultrasound and total body computed tomography scan were negative for local and metastatic disease. Eight months later, serum calcium was normal and plasma PTH level remained around the upper limit of normal range. Neck ultrasound did not show any pathological lesions. This is the first case of a nonfunctioning sporadic PC misdiagnosed prior of surgery as a follicular thyroid nodule. The parathyroid nature of the neck lesion could not be suspected before surgery. Fine-needle aspiration cytology (FNAC) may fail to distinguish a parathyroid tumor from a benign thyroid nodule because at FNAC, parathyroid and thyroid lesions have some morphological similarities. Histological criteria are not always sufficient for the differential diagnosis, which can definitely be established using immunohistochemistry.
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PMID:A nonfunctioning parathyroid carcinoma misdiagnosed as a follicular thyroid nodule. 2635 Apr 18

Parathyroid carcinoma only represents <1% of all cases of primary hyperparathyroidism (PHPT). Even rare, chronic PHPT may lead to excessive osteoclast activity, and the increased resorption leads to destruction of cortical bone and formation of fibrous cysts with deposits of hemosiderin-so-called brown tumors. These benign, osteolytic lesions may demonstrate FDG-avidity on (18)F-FDG PET/CT, and as such are misinterpreted as skeletal metastases. Regression of the lesions may occur following successful treatment. We present a case demonstrating the diagnostic work-up and follow-up of a patient with PHPT due to parathyroid carcinoma and with presence of brown tumors on (18)F-FDG PET/CT, visualizing the possible role of this imaging modality in the evaluation of treatment response in these patients.
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PMID:Brown Tumors Due to Primary Hyperparathyroidism in a Patient with Parathyroid Carcinoma Mimicking Skeletal Metastases on (18)F-FDG PET/CT. 2685 55

Ultrasonography (US) represents the most sensitive and efficient method for the evaluation of the thyroid and parathyroid glands. Infectious and autoimmune thyroiditis are common diseases, usually diagnosed and followed up by clinical examination and laboratory analyses. Nevertheless, US plays an important role in confirming diagnoses, predicting outcomes and, in autoimmune hyperthyroidism, in titrating therapy. Conversely, in nodular thyroid disease US is the imaging method of choice for the characterization and surveillance of lesions. It provides consistent clues in predicting the risk of malignancy, thus directing patient referral for fine-needle aspiration (FNA) biopsy. Suspicious US features generally include marked hypoechogenicity, a shape taller than it is wide, ill-defined or irregular borders, microcalcifications and hardness at elastographic evaluation. Finally, the role of US in thyroid cancer is to evaluate extension beyond the thyroid capsule and to assess nodal metastases or tumor recurrence. The main application of US in parathyroid diseases is represented by primary hyperparathyroidism. In this condition, US plays a role after biochemical diagnosis, and it should always be strictly performed for localization purposes. In both thyroidal and parathyroid diseases, US is recommended as a guide in FNA biopsies.
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PMID:Sonography of Normal and Abnormal Thyroid and Parathyroid Glands. 2700 29


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