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

Local control is vital for long-term survival for patients with stage III neuroblastoma, and although cure is difficult, ultimate success in stage IV neuroblastoma will necessitate control of the primary tumor as well as effective therapy of the metastases. The proper timing of surgical resection of the primary tumor is uncertain. Patients with stage III and IV neuroblastoma treated from 1977 to 1988 were retrospectively reviewed as to whether the resection was performed before or after chemotherapy. Complications assessed include significant blood loss, damage to adjacent organs, and delays before postsurgical chemotherapy could be given. Sixty patients were treated primarily at the authors' institution: 18 with stage III and 42 with stage IV disease. Chemotherapy consisted of combinations of nitrogen mustard, adriamycin, dacarbazine (DTIC), cisplatin, vincristine, and cyclophosphamide (MADDOC). Nine patients with stage III neuroblastoma underwent initial resection of the primary tumor before receiving chemotherapy. Three had complications, all with excessive blood loss (0.57, 2.0, and 3.0 times the estimated total blood volume [TBV]). One patient had renal infarction, and another had regrowth of the tumor before chemotherapy could be administered 35 days after surgery. There were no complications in the eight secondary explorations, four of which were complete resections. All had viable tumor in the resected specimen. Eleven of the 42 stage IV patients had primary resections, 5 of whom had complications: colocutaneous fistula, unilateral renal necrosis, chylothorax, and excessive blood loss (1.3 and 2 TBV). None of the 18 patients with delayed resection after 3 to 12 courses of chemotherapy had surgical complications with complete (14 patients), near complete (2 patients), or subtotal resections (2 patients).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Surgical management of stage III and IV neuroblastoma: resection before or after chemotherapy? 194 91

Recurrent pleural effusion is often a problem in metastases from breast cancer, and may be treated by instillation of irritating drugs combined with external drainage for some days. Less frequently chylothorax is a complication to malignant disease. Drainage of the pleural effusion to the peritoneum is possible, and this article describes how four patients with metastases from breast carcinoma and one patient with chylothorax caused by malignant lymphoma were all treated successfully from 40 days to ten months by means of a simple siliconated T-shaped drain from the pleural cavity to the peritoneum. In one patient the drain occluded after three months. It does not seem to be necessary to use drains with valves for this purpose.
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PMID:[Pleuroperitoneal shunt in recurrent pleural effusion]. 225 51

A 63-year-old woman, who had undergone radical hysterectomy and radiation therapy for cervical cancer of the uterus three years previously, was found to have pleural effusion and ascites. A diagnosis of chylothorax and chylous ascites was made on the basis of these fluids' characteristics. She received medium-chain triglyceride (MCT) in her diet and intra-venous hyperalimentation to decrease the leakages of chyle into the pleural and peritoneal cavities, but she died of respiratory and renal failures after six months. At autopsy, metastases from the cervical cancer of the uterus to the lymph nodes in the mediastinum and around the abdominal aorta were proved histologically. Lymph node swelling due to metastasis had caused a rupture of the thoracic duct, leading to chylothorax and chylous ascites. The diagnosis, evaluation and therapeutic modalities of the condition are outlined and the literature reviewed.
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PMID:Chylothorax and chylous ascites in a patient with uterine cancer. 328 82

Pleural disease provided the first and, for many years, the only indication for thoracoscopy. It remains the most efficient way of obtaining a diagnosis in cases of pleural effusions not diagnosed by thoracentesis and closed-needle biopsy, especially when malignancy is suspected. Thoracoscopy also can provide enough tissue to define cell type. In malignant mesothelioma, it can help assess the resectability of the tumor. In cases of metastatic disease or inoperable malignant mesothelioma, treatment of the effusion by talc poudrage can be combined with a diagnostic procedure. Any case of empyema in which a chest tube does not result in defervescence or complete evacuation of the pleural fluid within 2 to 3 days should be considered for thoracoscopy. In early empyemas, adhesions and loculatons can be addressed, the infected material removed, and the cavity irrigated. If the lung then fully expands, the tubes may be removed when the drainage ceases, precluding the prolonged retention of empyema tubes. Thoracoscopy also has proved useful in the management of benign pleural tumors, hemothorax, and chylothorax.
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PMID:Thoracoscopy for the evaluation and treatment of pleural space disease. 795 79

Clinical, macroscopic, and histologic features of 12 lymphangiosarcomas in cats are described. Nine tumors were located in the subcutaneous tissue at the caudoventral abdominal wall (eight cats) or in the neck (one cat). The remaining three cats had lymphangiosarcomas around the cranial mesenteric artery (two cats) or precardial in the mediastinum (one cat). Macroscopically, the tumors were noncircumscribed, white, edematous, and intermixed with fat tissue. Histologic features varied from cleft-forming and cavernous growth to papilliform and solid patterns. Follow-up data were available for seven cats with subcutaneous lymphangiosarcomas. All these cats died or were euthanatized within 6 months after surgery because of poor wound healing, local recurrence, or distant metastases. The cats with abdominal or thoracic masses were either euthanatized at surgery or within 6 months after the first surgery because of recurrent chylothorax, chyloperitoneum, or distant metastases.
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PMID:Lymphangiosarcomas in cats: a retrospective study of 12 cases. 1009 47

In its anatomy and physiology the pig is comparable with humans and its organs can be considered for xenotransplantation. We have studied the lymphatic drainage of the heart and lungs in 15 pigs. A coloured mass was injected into the myocardium and/or beneath the visceral pleura. The first nodes coloured were directly injected again. No lymph node was observed inside the heart and lungs. The first lymph nodes coloured were the peritracheobronchial nodes. There was no node in front of the thoracic trachea (Barety's compartment in man). Left suprabronchial nodes were connected with the thoracic duct in the mediastinum. The lymphatics of the heart and lungs in the pig are similar to those of human. Phylogenesis explains "skipping" metastases and the significance of N1 disease in lung cancer, as well as chylothorax occurring after heart and lung surgery.
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PMID:Lymphatic drainage of heart and lungs: comparison between pig and man. 1086 47

Lymphangioleiomyomatosis (LAM) is a rare disease of women that is characterized by a proliferation of abnormal smooth muscle-like cells (LAM cells), which leads to cystic lung lesions, lymphatic abnormalities, and abdominal tumors (e.g., angiomyolipomas). LAM occurs sporadically or in association with tuberous sclerosis complex, an autosomal dominant syndrome characterized by hamartoma-like tumor growths. The tumor suppressor genes TSC1 and TSC2 have been implicated in the etiology of LAM, as mutations and loss of heterozygosity (LOH) in TSC2 have been detected in LAM cells. TSC1 encodes hamartin, with a postulated role in actin cytoskeleton reorganization. TSC2 encodes tuberin, a protein with roles in cell growth and proliferation, transcriptional activation, and endocytosis. LAM cells, as defined by TSC2 LOH, have been detected in blood and body fluids, and can metastasize. LAM presents insidiously with progressive breathlessness, or dramatically with recurrent pneumothorax, chylothorax, or sudden abdominal hemorrhage. CT scans show numerous thin-walled cysts throughout the lungs, abdominal angiomyolipomas, and lymphangioleiomyomas. Pulmonary function tests show reduced flow rates (FEV1) and diffusion capacity (DL(CO)). Twenty per cent of patients have positive bronchodilator responses. Exercise testing shows gas-exchange abnormalities, ventilatory limitation, and hypoxemia that may occur with near-normal lung function. Progression of disease is best assessed by measurements of DL(CO) and FEV1. In the proper clinical setting, LAM may be diagnosed by a thoraco-abdominal CT scan. Tissue biopsy with special stains (HMB-45) should be reserved for cases with atypical presentations. There is no effective treatment for LAM, but on-going therapeutic trials with rapamycin appear promising.
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PMID:Clinical and molecular insights into lymphangioleiomyomatosis. 1645 17

Lymphangioleiomyomatosis (LAM) is a multisystem disease of women, characterized by proliferation of abnormal smooth muscle-like cells (LAM cells) that can metastasize, leading to the formation of lung cysts, fluid-filled cystic structures in the axial lymphatics (e.g., lymphangioleiomyomas), and angiomyolipomas, benign tumors usually involving the kidneys, comprising LAM cells and adipocytes, intermixed with incompletely developed vascular structures. LAM occurs sporadically or in association with tuberous sclerosis complex, an autosomal dominant syndrome characterized by hamartoma-like tumor growths. LAM may present with progressive dyspnea, recurrent pneumothorax, chylothorax, or abdominal hemorrhage. Computed tomography scans show thin-walled cysts scattered throughout the lungs, abdominal angiomyolipomas, and lymphangioleiomyomas. Pulmonary function tests show reduced flow rates (FEV(1)) and diffusion capacity (DL(CO)). Exercise testing may reveal gas exchange abnormalities, ventilatory limitation, and hypoxemia, which can occur with near-normal lung function. Methods used to grade the severity of disease are the LAM histology score, semiquantitative and quantitative computer tomography, pulmonary function testing, and cardiopulmonary exercise testing. Currently, progression of disease is best assessed by serial measurements of FEV(1), DL(CO), and exercise performance. New quantitative radiographic techniques that may offer advantages over physiologic testing are now available. Several potential biomarkers, such as LAM cells in peripheral blood, urine, and chyle and chemokines, vascular endothelial growth factors, and matrix metalloproteinases, may be useful as diagnostic tools or markers of organ involvement, disease severity, and progression.
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PMID:The natural history of lymphangioleiomyomatosis: markers of severity, rate of progression and prognosis. 2023 83

We report the case of a 61-year-old female with advanced gastric cancer and mediastinal lymph node metastasis who developed chylothorax. The patient presented with cough, dyspnea and pain in the left lower limb, back, and hips. Her lower limb symptoms were attributed to cellulitis. Computed tomography revealed right-sided pleural effusion, multiple lymph node swelling, and thickening of the gastric wall. Following pleural aspiration, the effusion was identified to be chyle. Cytopathologically, numerous adenocarcinoma cells were detected in clumps, and subsequent esophagogastroduodenoscopy revealed type 3 gastric cancer. We diagnosed multiple lymph node metastases with pleural dissemination. Chemotherapy was administered; however, she eventually succumbed to disease progression. We suspected that the chylothorax resulted from the mediastinal lymph node metastasis that caused thoracic duct obstruction.
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PMID:[Chylothorax in a patient with advanced gastric cancer and mediastinal lymph node metastasis causing thoracic duct obstruction]. 2418 23

Lymphangioleiomyomatosis (LAM) is a rare neoplastic disease affecting predominantly young women. Clinical symptoms of this progressive disease include dyspnoea, cough, recurrent pneumothorax, hemoptysis and chylothorax. LAM is generally aggressive in nature and ultimately results in respiratory failure. Important hallmark features of this metastatic disease include the formation of lesions of abnormal smooth muscle cells, cystic destruction of the lung tissue and lymphangiogenesis affecting the lungs, abdomen and lymphatics. Research over the last 10-15 years has significantly enhanced our understanding of the molecular and cellular processes associated with LAM. These processes include mutational inactivation of the tuberous sclerosis complex genes, TSC1 and TSC2, activation of the mammalian target of rapamycin (mTOR) pathway, enhanced cell proliferation and migration, lymphangiogenesis, metastatic spread through the blood and lymphatic circulations, sex steroid sensitivity and dysregulated autophagy. Despite this increased knowledge there is currently no cure for LAM and treatment options remain limited. Whilst the mTOR inhibitor rapamycin has shown some benefit in patients with LAM, with stabilisation of lung function and improved quality of life, cessation of treatment results in recurrence of the disease progression. This highlights the urgent need to identify novel targets and new treatment regimens. The focus of this review is to summarise our current understanding of the cellular and molecular processes associated with LAM and highlight emerging treatments.
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PMID:Lymphangioleiomyomatosis: Current understanding and potential treatments. 2671 79


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