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)

The Carney triad is the clinical association of gastric stromal sarcomas, pulmonary cartilaginous tumors, and extra-adrenal paragangliomas. The pulmonary tumors are its second commonest component and have been misinterpreted clinically and pathologically as metastases from the gastric tumors and pulmonary cartilaginous hamartomas, respectively. They have not been previously described in detail in the pathology literature or compared with pulmonary cartilaginous hamartomas. Forty-two patients with pulmonary cartilaginous tumors as a component of Carney triad were identified. Clinical, radiographic, and pathologic findings in the cases were tabulated. Hematoxylin and eosin-stained sections of the neoplasms were evaluated for a series of histologic features. A subgroup of 41 tumors from the latter was compared with those in a group of pulmonary cartilaginous hamartomas. Patients with Carney triad group were predominantly young women. Their pulmonary neoplasm(s) were usually asymptomatic, often multiple, well circumscribed, medium-sized (mean diameter=2.8 cm), and composed almost exclusively of cartilage and bone surrounded by a fibrous pseudocapsule. The cartilage was usually myxoid, less frequently hyaline, and commonly calcified, ossified, or both. They showed no fat, smooth muscle or entrapped respiratory epithelium, tissues that were common in pulmonary hamartoma (P<0.0001). None of the tumors metastasized or was fatal. The pulmonary neoplasms in the Carney triad are well-differentiated benign cartilaginous tumors that are best designated as chondromas. They differ pathologically from pulmonary cartilaginous hamartomas on the basis of the presence of a thin fibrous pseudocapsule, frequent bone metaplasia, and calcification, and also the absence of entrapped epithelium and fat.
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PMID:Pulmonary chondroma: a tumor associated with Carney triad and different from pulmonary hamartoma. 1804 38

Mucoid impaction is a relatively common finding at chest radiography and computed tomography (CT). Both congenital and acquired abnormalities may cause mucoid impaction of the large airways that often manifests as tubular opacities known as the finger-in-glove sign. The congenital conditions in which this sign most often appears are segmental bronchial atresia and cystic fibrosis. The sign also may be observed in many acquired conditions, include inflammatory and infectious diseases (allergic bronchopulmonary aspergillosis, broncholithiasis, and foreign body aspiration), benign neoplastic processes (bronchial hamartoma, lipoma, and papillomatosis), and malignancies (bronchogenic carcinoma, carcinoid tumor, and metastases). To point to the correct diagnosis, the radiologist must be familiar with the key radiographic and CT features that enable differentiation among the various likely causes. CT is more useful than chest radiography for differentiating between mucoid impaction and other disease processes, such as arteriovenous malformation, and for directing further diagnostic evaluation. In addition, knowledge of the patient's medical history, clinical symptoms and signs, and predisposing factors is important.
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PMID:Mucoid impactions: finger-in-glove sign and other CT and radiographic features. 1879 13

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

Small intestinal neoplasms are uncommon cancers. Benign small intestinal tumors (e.g., leiomyoma, lipoma, hamartoma, or desmoid tumor) usually are asymptomatic but may present with complications. Primary malignancies of the small intestine, including adenocarcinoma, leiomyosarcoma, carcinoid, and lymphoma, are often symptomatic and may present with intestinal obstruction, jaundice, bleeding, or pain. Metastatic neoplasms may involve the small intestine via contiguous spread, peritoneal metastases or hematogenous metastases. Because the small intestine is relatively inaccessible to routine endoscopy, diagnosis of small intestinal neoplasms is often delayed for months after onset of symptoms. During last years the increase of small bowel endoscopy and other diagnostic tools allow earlier non-operative diagnosis. Even though radical resection of small bowel cancer plays an important role, the 5 yr overall survival remains low.
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PMID:Surgical treatment of small bowel neoplasms. 2049 43

The skin plays a critical role in the detection of internal malignances. Cutaneous signs of these disorders afford clinicians opportunities for early diagnosis and treatment. We aim to succinctly review the recognition, diagnosis, and treatment of selected cutaneous paraneoplastic diseases. Skin disorders that may be associated with paraneoplastic syndromes include: cutaneous metastases, tripe palms, Sweet's syndrome, glucagonoma, Paget's disease and extramammary Paget's disease, acanthosis nigricans, Birt-Hogg-Dube syndrome, basal cell nevus syndrome, Bazex syndrome (acrokeratosis paraneoplastica), carcinoid syndrome, Cowden's disease(multiple hamartoma syndrome), dermatomyositis, erythema gyratum repens, ichthyosis aquisita, von Recklinghausen's disease, pityriasis rotunda, pyoderma gangrenosum, Quincke's edema (angioedema and paraneoplastic uricaria), paraneoplastic pemphigus, Degos' disease, superior vena cava syndrome, Werner's syndrome, diffuse normolipemic plane xanthomas, and yellow nail syndrome. Treatment for these disorders depends on the nature and anatomic distribution of the primary neoplastic process.
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PMID:Diagnosis and treatment of cutaneous paraneoplastic disorders. 2105 10

Gangliocytic paraganglioma (GP) is generally considered to be a benign periampullary lesion, although it is unclear whether it should be classified as a hamartoma or as a neoplasm. Here, we present a GP case with lymph node metastasis. A 16-year-old boy complained of exertional dyspnea. Upper endoscopy and imaging studies revealed a polypoid ampullary tumor. Pancreaticoduodenectomy with lymph node dissection was performed due to swelling of peripancreatic lymph nodes. Histologically, the tumor consisted of three cell types: epithelioid; spindle; and ganglion cells. In addition to these typical components of GP, a distinct glandular component was also present. There was substantial invasion of tumor cells into the lymphovascular vessels, associated with lymph node metastases. These lymph node metastases were histologically similar to the primary tumor. To judge from these findings GP may be a true neoplasm with metastatic capacity. Pre- and intraoperative investigations for lymph node or distant metastases are required for adequate resection of this kind of tumor.
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PMID:Duodenal gangliocytic paraganglioma with regional lymph node metastasis and a glandular component. 2125 88

Neurocristic hamartomas are rare pigmented lesions comprised of melanocytes, Schwann cells, and pigmented dendritic spindle cells that involve the skin and soft tissue. Malignant transformation can rarely arise within neurocristic hamartomas. Up to date, there has been only 1 series of 7 cases of malignant neurocristic hamartomas (MNHs), with 3 cases that developed metastases. We present the histology and clinical course of 3 additional cases of MNH, 2 of which were metastatic. CD117 was strongly positive in all cases with available archival materials--the tumors and background neurocristic hamartoma of 3 cases, and 1 lymph node metastasis; however, KIT sequencing for exons 11, 13, 17, and 18 was negative. Mutational analyses of recurrent mutations of 17 cancer genes, including BRAF and KIT, were also negative. Although our series is small, KIT overexpression in MNH does not seem to correlate with gene mutation. The lack of BRAF, NRAS, GNAQ, and KIT mutations seems to support the notion that MNH may be distinct from conventional melanoma and from other dermal melanomas, such as malignant blue nevi and melanoma arising in congenital nevi.
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PMID:Malignant neurocristic hamartoma: a tumor distinct from conventional melanoma and malignant blue nevus. 2193 81

Intracranial invasion of cellular blue nevus is extremely rare, and its malignant transformation is even less common. The differential diagnosis includes neurocutaneous melanosis and neurocristic cutaneous hamartoma. A 50-year-old female presented with intracranial melanoma in contiguity with a congenital blue nevus on the scalp. The patient showed a wide pigmented lesion on the scalp that had grown in the last few years over the congenital blue nevus. Magnetic resonance imaging revealed an intracranial tumor lying contiguous to the nevus. Despite aggressive surgery, the tumor relapsed and the patient developed systemic metastases. We report a rare case of cellular blue nevus showing an unexpected aggressive behavior with extensive extra- and intracranial expansion and distant metastases.
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PMID:Dermal melanocytosis of the scalp associated to intracranial melanoma: malignant blue nevus, neurocutaneous melanosis, or neurocristic cutaneous hamartoma? 2212 66

We report a case of pulmonary adenofibroma in a 29-year-old female found by CT scan during work-up for midline chest pain. To our knowledge, the cytological features of this entity have not been previously reported. Cytology demonstrated bland epithelial and stromal cells of varying size without karyorrhexis, pyknosis, or necrosis and with very rare mitoses. Stromal cells were present as either naked bipolar nuclei, as spindle cells with fragile eosinophilic cytoplasm, or as rare larger carrot shaped nuclei. Epithelial cells were present as small loosely cohesive groups with smooth round nuclei and moderate amounts of cytoplasm. Histologically, this lesion consisted of a leaf-like fibroepithelial pattern in which the clefts were lined by a single layer of cuboidal epithelium reminiscent of adenofibroma occurring in the female genital tract. Immunohistochemical analysis demonstrated epithelium that stained positively for pan-cytokeratin and TTF-1. The stroma stained positively for vimentin and desmin, and was weakly positive for SMA-1. The lesion was confirmed to be pulmonary adenofibroma with a smooth muscle component. The differential diagnosis for this lesion includes, but is not limited to, pulmonary hamartoma, pulmonary blastoma, adenomyofibroma, synovial sarcoma, and visceral metastases. It is important for cytopathologists to be aware of this benign entity because it can be encountered on lung FNA specimens. Considering this benign lesion in the differential diagnosis may help plan for minimal lung resection. Confirmatory intraoperative frozen section is a reasonable option.
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PMID:Pulmonary adenofibroma: cytologic and clinicopathologic features of a rare benign primary lung lesion. 2264 35

Approximately one out of 500 chest radiographs shows the incidental finding of a solitary pulmonary nodule and almost one half of these pulmonary lesions are caused by a tumor. Unfortunately, only 2% to 5% of all lung tumors are of benign origin, e. g. lipoma, fibroma, hamartoma, and chondroma, and the majority are malignant neoplasms, most commonly primary lung cancer followed by metastases of extrapulmonary primary carcinomas. Thus, a careful diagnostic work up of solitary pulmonary nodules, including histological diagnosis, is mandatory for an adequate management and treatment of patients with pulmonary lesions. Despite all recent improvements of treatment modalities, lung cancer continues to be a major cause of morbidity and mortality among malignant diseases worldwide. The prognosis of affected patients is still very poor and a 5-years survival rate of only 14% makes lung cancer the number one cause of death due to cancer in Switzerland. Active and passive tobacco smoking are by far the best known risk factor for the development of lung cancer, but there are severe other probably less known factors that may increase the individual risk for malignant neoplasms of the lung. These risk factors include e. g. exposure to natural ionic radiation, consisting of terrestrial radiation and indoor radiation caused by radon gas, exposure to respirable dust and Diesel engine emissions, asbestos, and polycyclic aromatic hydrocarbons. In the majority of cases, the latency between exposure and development of cancer is years to decades and the person concerned was occupationally exposed. Therefore, a detailed evaluation of a patient's medical and occupational history is needed. Due to its poor prognosis, prevention and early diagnosis of lung cancer is crucial to improve our patients' outcome. Good knowledge of epidemiology and aetiology of pulmonary tumors is the key to preventive measures and identification of individuals at increased risk for lung cancer. An overview will be provided on the epidemiology of lung tumors and predominantly preventable risk factors for lung cancer.
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PMID:[Epidemiology of lung tumors]. 2275 85


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