Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 55-year-old woman first underwent total thyroidectomy in 1973, and the histopathology was medullary thyroid carcinoma. There was no familial history of endocrine neoplasm. She remained asymptomatic for the next 19 years. Neck and tracheal recurrence was treated with tracheal stent and radiation, in 1997. On July 1998, she presented with headache. Magnetic resonance imaging revealed a gadolinium enhanced mass in the left inferior temporal gyrus. Angiogram revealed a marked tumor stain feeding from the middle and inferior temporal artery. The tumor was successfully excised via a temporal craniotomy. Histopathological sections of the resected specimen confirmed the diagnosis, it demonstrated the medullary pattern that was composed of vascular stroma and clumps of cells. Immunocytochemistry, using carcino-embryonic antigen and calcitonin showed a strongly positive reaction, and MIB-1 was 5.4%. The patient subsequently underwent a course of whole brain radiotherapy, a total of 30 Gy/10 fr. She remains asymptomatic 14 months after treatment. Brain metastasis from medullary carcinoma of the thyroid is extremely rare and it does not usually occur distant spread. Thyroid carcinoma with brain metastasis tends to be identified in patients with older age, larger primary tumor, more frequent evidence of extrathyroidal invasion, and more aggressive histologies. The number of medullary thyroid carcinoma with brain metastasis is small and there has been little evidence from benefit of therapy. Neither is it clear wether radiation therapy is beneficial also is not clear. In this case, surgical resection and radiation therapy was effective against recurrence of other lesion.
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PMID:[Solitary brain metastasis from medullary carcinoma of the thyroid]. 1296 94

Medullary thyroid cancer (MTC) is a C cell neoplasm-secreting calcitonin. Surgery remains the only treatment as the primary tumor and metastases resist radio- and chemotherapies. MTC produces high amounts of prostaglandins (PGs). Nonsteroidal antiinflammatory drugs have an antitumoral effect, generally related to the decrease of PG levels. We assessed the therapeutic potential of indomethacin in a model of human (TT cells) tumors in nude mice. Indomethacin (1.5 or 2.0 mg/kg body weight.d for 7 wk) inhibited tumor volume by 49 or 77%, respectively, and decreased the plasma level of CT. Although the terminal deoxynucleotidyltransferase-mediated deoxyuridine triphosphate nick end labeling method revealed few apoptotic nuclei, the number of proliferating cells was significantly decreased (Ki-67 antigen study). Immunological effector recruitment and vascular network was not modified by treatment. The inducible synthesis enzyme, cyclooxygenase-2 (COX-2), was revealed only in infiltrating cells, both in treated and control tumors. The expression of the constitutive synthesis enzyme COX-1 was diminished, and the expression of 15-prostaglandin dehydrogenase, the key enzyme catabolizing PGs, was increased in treated tumors. Thus, our results demonstrated the potential of indomethacin, inhibitor of COX-1 and COX-2, to prevent MTC growth. The synthesis enzyme, COX-1, and the catabolism enzyme 15-prostaglandin dehydrogenase, could be involved in MTC development.
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PMID:Tumor growth inhibition by indomethacin in a mouse model of human medullary thyroid cancer: implication of cyclooxygenases and 15-hydroxyprostaglandin dehydrogenase. 1473 30

Most positron emission tomography (PET) imaging studies in head and neck cancer are performed using the radiotracer 18-fluorodeoxyglucose ((18)FDG). PET with FDG has become a standard clinical imaging modality in patients with head and neck cancer. It contributes valuable information in localizing a primary tumor in patients with neck nodal metastases from an unknown primary, in the staging of primary head and neck cancer, and in the detection of recurrent disease. In addition, FDG-PET provides independent prognostic information in patients with newly diagnosed and recurrent head and neck cancer. PET/CT improves lesion localization and accuracy of FDG-PET and is strongly recommended in patients with head and neck cancer. After thyroidectomy, FDG-PET has proven useful in patients with clinical or serological evidence of recurrent or metastatic thyroid carcinoma but negative whole body iodine scan. PET shows metastatic disease in up to 90% of these patients, thereby providing a rational basis for further studies and therapy. In patients with medullary thyroid cancer with elevated calcitonin levels following thyroidectomy, FDG-PET has a sensitivity of 70-75% for localizing metastatic disease. Occasionally incidental intense FDG uptake is observed in the thyroid gland on whole body PET studies performed for other indications. Although diffuse FDG uptake usually indicates thyroiditis, focal uptake has been related to thyroid cancer in 25-50% of cases and should therefore be evaluated further if a proven malignancy would cause a change in patient management.
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PMID:Positron emission imaging of head and neck cancer, including thyroid carcinoma. 1520

A characteristic feature of neuroendocrine tumors is production and release of peptide hormone. Ghrelin is a 28-amino acid hormone that stimulates GH release. In this paper, we describe a patient with a metastasizing gastric neuroendocrine tumor displaying intense immunoreactivity for ghrelin and extremely high circulating levels of ghrelin. Tumor tissue biopsies from the primary tumor and one liver metastasis were examined by immunohistochemistry. Ghrelin and several other hormones and tumor markers were measured in blood. The clinical course of the patient was followed. Tumor tissue biopsies showed immunoreactivity for cytokeratin, chromogranin A, human synaptic vesicle protein 2, synaptophysin, and ghrelin. Grossly elevated circulating levels of total ghrelin, 2100 microg/liter (reference interval < 5 microg/liter) and active ghrelin, 28 microg/liter (reference interval < 0.1 microg/liter) were found at presentation. Chromogranin A, chromogranin B, and calcitonin levels were also increased. Both total and active ghrelin increased, despite treatment, during follow-up of the patient. We have identified and characterized a patient with a malignant gastric neuroendocrine tumor secreting ghrelin as the main hormone. This might be a new tumor entity of the stomach, and it is suggested that patients with malignant gastric neuroendocrine tumors should be investigated for ghrelin production.
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PMID:Malignant gastric ghrelinoma with hyperghrelinemia. 1529 99

Breast cancer is the malignant neoplasm most commonly associated with hypercalcemia. In breast cancer the majority of the hypercalcemia cases result from osteolytic metastatic bone disease of the primary tumor. In a few patients hypercalcemia results from other conditions like primary hyperparathyroidism. Here, we present two female patients who were treated for breast cancer. Hypercalcemia in these two patients was diagnosed as being due to primary hyperparathyroidism. One of them was submitted to surgery and the calcium level dropped to the normal level thereafter. The other one refused surgery and was treated with biphosphonate and calcitonin. We suggest that when hypercalcemia occurs in breast cancer, primary hyperparathyroidism should be considered as possible cause.
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PMID:Breast cancer and concomitant primary hyperparathyroidism: description of two patients. 1551 83

Medullary thyroid carcinoma (MTC) is a rare disease, and most studies are either based on small numbers or multicenter studies with their inherent difficulties. Since 1995, a total of 440 patients with MTC underwent surgery in our clinic. A primary operation was performed in 188 patients (43% of 440). In 60 patients, the primary operation was performed because of a germline RET mutation ("prophylactic surgery"). Most (84%, 158/188) of the patients had pathologic calcitonin levels. Notably, MTC was found in almost 10% (3/30) of patients with normal calcitonin levels. However, all patients with lymph node metastases (LNMs) had elevated calcitonin levels. Total thyroidectomy (TTx) was performed in all patients. Lymph node dissection (LND) was performed at various extensions: one-compartment LND in 35% (66/188), three-compartment LND in 31% (58/188), and four-compartment LND in 29% (22/188). In general, lymph node dissection increased the likelihood of complications. LNM and distant metastases (DM) correlated with the extent of the primary tumor (pT category). The presence of LNM ranged from 17% (pT1 tumor) to 100% (pT4 tumor), whereas the presence of DM ranged from 0% (pT1 tumor) to 81% (pT4 tumor). Biochemical cure (normal calcitonin levels) was obtained in 72% (137/188) of patients. All 60 patients undergoing prophylactic surgery (tumor stage pT0/pT1) were biochemically cured. In contrast, only 60% (77/128) of the remaining patients were cured. The data suggest that primary surgery should be scheduled as soon as possible to treat patients at a node-negative stage. In the case of normal basal and elevated stimulated calcitonin levels, TTx and cervicocentral LND is recommended. If the basal calcitonin level is elevated, LND should include the cervicolateral compartment.
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PMID:Single center experience in primary surgery for medullary thyroid carcinoma. 1551 88

FDG-PET has become a standard clinical imaging modality in patients with head and neck cancer. It contributes valuable information in localizing a primary tumor in patients with neck nodal metastases from an unknown primary, in the staging of primary head and neck cancer, and in the detection of recurrent disease. In addition, FDG-PET provides independent prognostic information in patients with newly diagnosed and recurrent head and neck cancer. PET-CT improves lesion localization and accuracy of FDG-PET and is strongly recommended in patients with head and neck cancer. After thyroidectomy, FDG-PET has proven useful in patients with clinical or serological evidence of recurrent or metastatic thyroid carcinoma but negative whole body iodine scan. PET shows metastatic disease in up to 90% of these patients, thereby providing a rational basis for further studies and therapy. In patients with medullary thyroid cancer with elevated calcitonin levels following thyroidectomy, FDG-PET has a sensitivity of 70-75% for localizing metastatic disease. Occasionally incidental intense FDG uptake is observed in the thyroid gland on whole-body PET studies performed for other indications. Although diffuse FDG uptake usually indicates thyroiditis, focal uptake has been related to thyroid cancer in 25-50% of cases and should therefore be evaluated further if a proven malignancy would cause a change in patient management.
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PMID:FDG-PET in head and neck, and thyroid cancer. 1608 43

Spindle epithelial tumor with thymus-like differentiation (SETTLE) is an extremely rare type of thyroid tumor. It has been reported only 20 times in the English literature. This tumor occurs predominantly in young patients and has a protracted clinical course despite the occurrence of metastases. In the recent literature SETTLE has been considered to be a tumor of low malignant potential with distant metastases developing some years after diagnosis. Herein we report a case of SETTLE in a 22-yr-old man in which a lymph node metastasis developed soon after the primary tumor manifestation. Histological examination of the tumor showed the predominantly monophasic variant of SETTLE. The primary and metastatic lesions were highly cellular tumors composed of sheets of spindle cells that were positive for pan-cytokeratin and vimentin and negative for thyroglobulin, calcitonin, and S-100 protein.
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PMID:Spindle epithelial tumor with thymus-like differentiation (SETTLE) of the thyroid with neck lymph node metastasis: a case report. 1619 99

Medullary thyroid carcinoma (MTC) is a rare malignancy of the thyroid C cells. It occurs in hereditary (25% of cases) and sporadic (75%) forms. Sporadic MTCs frequently metastasize to cervical lymph nodes. Thorough surgical extirpation of the primary tumor and nodal metastases by compartment-oriented resection has been the mainstay of treatment (level IV evidence). Surgical resection of residual and recurrent disease is effective in reducing calcitonin levels and controlling complications of central neck disease (level IV evidence). Radioactive iodine, external beam radiation therapy, and conventional chemotherapy have not been effective. Newer systemic treatments, with agents that target abnormal RET proteins hold promise and are being tested in clinical trials for patients with metastatic disease.
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PMID:Evidence-based approach to the management of sporadic medullary thyroid carcinoma. 1742 1

Medullary thyroid carcinoma (MTC) is an uncommon malignancy of the parafollicular C cells of the thyroid, with a propensity for early lymph node spread and distant metastasis. It is hereditary in approximately 25% of cases, involving specific point mutations of the RET proto-oncogene inherited in an autosomal dominant fashion. While European professional organizations have put forth calcitonin screening guidelines for earlier detection of MTC, the American Thyroid Association, which has published recent guidelines for MTC treatment, have not had a position on routine screening in the USA. Surgical extirpation of the primary tumor and involved lymph node metastases is the mainstay of treatment and the only chance for cure. Conventional systemic chemotherapies for metastatic MTC have been disappointing; however, newer agents which affect specific RET proteins and tyrosine kinase growth factor receptors show promise in phase 1 and 2 clinical trials.
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PMID:Current management of medullary thyroid cancer. 2021 15


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