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)

Langerhans cell histiocytosis (LCH) of the female genital tract is very rare. A review of the literature revealed that only 12 cases of primary vulvar LCH have previously been published. We describe an additional case of LCH in which the disease was confined to the vulva. A 64-year-old woman was admitted to our hospital with a nodular mass on her left labium minus, and complete surgical excision was performed. On histological and immunohistochemical examination, the tumor fulfilled the criteria of LCH. A metastatic workup did not reveal any evidence of disease beyond the vulva. The patient received local radiotherapy, and 22 months later she is in excellent condition without local recurrence or metastatic disease.
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PMID:Primary langerhans cell histiocytosis of the vulva. 1630 84

Central diabetes insipidus is the end result of a number of different diseases that affect the hypothalamic-neurohypophyseal system. In many patients, especially children and young adults, it is caused by the destruction or degeneration of neurons that originate in the supraoptic and paraventricular nuclei of the hypothalamus. The known causes of these lesions include germinoma or craniopharyngioma; Langerhans cell histiocytosis; local inflammatory, autoimmune or vascular diseases; trauma resulting from surgery or an accident; sarcoidosis; metastases; and midline cerebral and cranial malformations. In rare cases, genetic defects in AVP synthesis that are inherited as autosomal dominant, autosomal recessive or X-linked recessive traits are the underlying cause. Accurate diagnostic differentiation is essential for both safe and effective disease management. Proper etiological diagnosis can be achieved via a series of steps that start with clinical observations and then progress, as needed, to more sophisticated methods. Indeed, magnetic resonance imaging (MRI) represents the examination method of choice for evaluating hypothalamic-pituitary-related endocrine diseases due to its ability to provide strongly-contrasted high-resolution multi-planar and spatial images. Specifically, MRI allows a detailed and precise anatomical study of the pituitary gland by differentiating between the anterior and posterior pituitary lobes. MRI identification of pituitary hyperintensity in the posterior part of the sella, now considered to be a clear marker of neurohypophyseal functional integrity, together with careful analysis of pituitary stalk shape and size, have provided the most striking recent findings contributing to the diagnosis and understanding of some forms of 'idiopathic' central diabetes insipidus.
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PMID:The diagnosis of children with central diabetes insipidus. 1745 Oct 74

Bone marrow oedema is associated with a wide variety of pathological processes including both benign and malignant bone tumours. This imaging finding in relation to intraosseous tumours can aid in providing a more focused differential diagnosis. In this review, we will discuss the MR imaging of bone marrow oedema surrounding intraosseous neoplasms. The different pulse sequences used in differentiating underlying tumour from surrounding oedema are discussed along with the role of dynamic contrast enhanced MRI. Benign lesions commonly associated with bone marrow oedema include osteoid osteoma, osteoblastoma, chondroblastoma and Langerhan's cell histiocytosis. Metastases and malignant primary bone tumours such as osteosarcoma, Ewing's sarcoma and chondrosarcoma may also be surrounded by bone marrow oedema. The imaging findings of these conditions are reviewed and illustrated. Finally, the importance of bone marrow oedema in assessment of post chemotherapeutic response is addressed.
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PMID:Bone marrow oedema associated with benign and malignant bone tumours. 1835 60

We report a case of primary multifocal osseous lymphoma in a 6-year-old girl presenting with multifocal osteolytic lesions without systemic symptoms or identifiable non-osseous primary tumor. The differential diagnoses for such a presentation include histiocytosis X, chronic recurrent multifocal osteomyelitis, acute lymphoblastic leukemia, metastatic disease, and primary bone lymphoma. Although non-Hodgkin lymphoma is common in the pediatric population, its presentation as a primary bone tumor, especially with multifocal disease, is extremely rare and is frequently misdiagnosed. We hope that awareness of this entity will help radiologists achieve timely diagnosis and intervention.
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PMID:Primary multifocal osseous lymphoma in a child. 1877 59

Tumor-related activities that seem to be operationally induced by the division of function, such as inflammation, neoangiogenesis, Warburg effect, immune response, extracellular matrix remodeling, cell proliferation rate, apoptosis, coagulation effects, present itself from a systems perspective as an enhancement of complexity. We hypothesized, that tumor systems-directed therapies might have the capability to use aggregated action effects, as adjustable sizes to therapeutically modulate the tumor systems' stability, homeostasis, and robustness. We performed a retrospective analysis of recently published data on 224 patients with advanced and heavily pre-treated (10% to 63%) vascular sarcoma, melanoma, renal clear cell, cholangiocellular, carcinoma, hormone-refractory prostate cancer, and multivisceral Langerhans' cell histiocytosis enrolled in nine multi-center phase II trials (11 centers). Each patient received a multi-targeted systems-directed therapy that consisted of metronomic low-dose chemotherapy, a COX-2 inhibitor, combined with one or two transcription modulators, pioglitazone +/- dexamethasone or IFN-alpha. These treatment schedules may attenuate the metastatic potential, tumor-associated inflammation, may exert site-specific activities, and induce long-term disease stabilization followed by prolonged objective response (3% to 48%) despite poor monoactivity of the respective drugs. Progression-free survival data are comparable with those of reductionist-designed standard first-line therapies. The differential response patterns indicate the therapies' systems biological activity. Understanding systems biology as adjustable size may break through the barrier of complex tumor-stroma-interactions in a therapeutically relevant way: Comparatively high efficacy at moderate toxicity. Structured systems-directed therapies in metastatic cancer may get a source for detecting the topology of tumor-associated complex aggregated action effects as adjustable sizes available for targeted biomodulatory therapies.
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PMID:Systems biology: a therapeutic target for tumor therapy. 1930 94

For bone marrow screening, multimodality algorithms including conventional radiographs, bone scintigraphy, multislice computed tomography CT (MS-CT) scan, and dedicated magnetic resonance imaging (MRI) are widely established in clinical routine. Although radiographs are used as a basic imaging procedure for clarification of suspected focal bone pathologies, low sensitivity has been reported for the detection of limited osteolytic bone marrow destruction. Therefore, skeletal scintigraphy often is used as a more sensitive and integrated method in patients with suspected malignant bone marrow disease. MS-CT scan is the method of choice in the assessment of bone stability and allows for evaluation of fracture risk. Hybrid imaging concepts, such as positron emission tomography-computed tomography (PET-CT) scan, have been established as an effective tool for the detection of skeletal metastases, using the additional metabolic information of a PET scan for the assessment of tumor viability and therapy response. MRI is an imaging technique that allows direct visualization of bone marrow components with high spatial resolution. The unique soft-tissue contrast of MRI enables precise assessment of bone marrow infiltration before osteolytic changes become visible in MS-CT or metabolic changes occur in bone scintigraphy or a PET scan. Furthermore it can depict tumor expansion into adjacent paraosseous structures, such as the spinal canal. The development of multichannel whole-body MRI (WB-MRI) systems has enabled bone marrow screening without use of ionizing radiation at high diagnostic accuracy. Parallel imaging techniques in combination with global matrix coil concepts, as well as the introduction of high-field whole-body scanners, have substantially reduced acquisition times without compromises in spatial resolution. WB-MRI has successfully been applied for screening of bone metastases and hematologic bone marrow diseases, like multiple myeloma, lymphoma, and histiocytosis X. Furthermore, it has recently been proposed for the assessment of primarily benign bone diseases predisposing for malignancy (e.g., multiple cartilaginous exostoses). This article provides an overview of state-of-art whole-body imaging of the bone marrow and highlights present and potential future applications, especially in the field of WB-MRI.
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PMID:Whole-body imaging of bone marrow. 1945 75

Malignant extraconal orbital tumors are very rare during childhood and must be referred as soon as possible to a highly specialized center to be managed by a multidisciplinary team. They are often referred on an emergency basis. Both diagnosis and treatment must be undertaken as soon as possible. The course of these malignant tumors can be acute and can jeopardize the function of the eye or be life-threatening, especially in the event of metastatic locations. Extra-axial proptosis is by far the most frequent revealing symptom. Local and general examinations are of utmost importance. Sometimes diagnosis should be clear with the association of an orbital tumor and deterioration of the general health status favoring metastatic disease. Most metastatic neuroblastomas present such clinical symptoms in young children. Today both CT and MRI are highly valuable in assessing the diagnosis and starting the management of these tumors. Biopsy is mandatory to confirm the diagnosis. It can be reinforced by molecular biology. Among the primitive tumors, soft tissue sarcomas, especially rhabdomyosarcomas, are the most frequent. The diagnosis is suggested when the onset of the disease is acute and the course is rapid. Most respond to neoadjuvant chemotherapy. In the event of a residual tumor, local treatment is indicated so that surgery and/or radiotherapy are used as second-line treatment. Prognosis is closely related to histology. It can be satisfactory (Langerhans' cell histiocytosis, lymphoma, meningioma, infantile fibrosarcoma) or poor (metastatic tumor, rhabdoid tumor).
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PMID:[Malignant extraconal tumors of the orbit in childhood]. 2030 50

Gas containing lesions of the lung can be differentiated into cystic and cavitary lesions based on their imaging characteristics, which is helpful for differential diagnosis. The imaging features include number, size, thickness and smoothness of the walls, content, lesion distribution and occasional associated lesions. Some imaging features have characteristic diagnostic implications: mural nodule and meniscus sign. Cysts are characterized by thin and regular walls, less than 2 mm in thickness. The differential diagnosis includes: histiocytosis X, lymphangioleiomyomatosis, cystic metastases, septic emboli, pulmonary sequestration. Emphysema, honeycombing and cystic bronchiectases should also be considered. Cavitary lesion have thicker walls. The most frequent etiologies include abscess, cavitary tumors, mycobacterial infections and fungal infections.
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PMID:[Cystic and cavitary lesions of the lung: imaging characteristics and differential diagnosis]. 2051 2

Two hundred eight neonates with malignant tumors and cutaneous metastases were reviewed. Malignancies most often associated with cutaneous metastases, in order of rank, were leukemia, multisystem Langerhans cell histiocytosis, neuroblastoma, rhabdoid tumor, rhabdomyosarcoma, primitive neuroectodermal tumor, choriocarcinoma, and adrenocortical carcinoma. Bluish skin nodules producing the "blueberry muffin baby"-like appearance were the most common dermatologic finding in 171, or 82% of 208 neonates. The tendency of newborns to present with skin nodules is one of the significant differences between malignancies in younger and older children. Patients with rhabdoid tumor and rhabdomyosarcoma had the lowest survival rates, 4% and 15%, respectively, compared with leukemia, 37.5%, and neuroblastoma, 58%. Overall survival was 39%.
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PMID:Cutaneous metastases in neonates: a review. 2150 42

Objective is to identify epidemiologic and radiologic criteria allowing specific diagnoses of tumors and tumor-like lesions in the hip region and pelvis, and to optimize pre-operative staging. Patients with pelvic tumors are usually older, and their tumors are larger relative to patients with tumors in extremities. The majority of tumors in the pelvis are malignant (metastases, myeloma, chondrosarcoma, Ewing-, osteo-, and MFH/fibrosarcoma), while those in the proximal femur are in majority benign (fibrous dysplasia, solitary bone cyst, and osteoid osteoma). Soft tissue masses in the thigh in the elderly are typically sarcomas without tumor specific signs. Common tumor-like lesions occurring in the hip and pelvis that can mimic neoplasm are: infections (including tuberculosis), insufficiency/avulsion fractures, cysts, fibrous dysplasia, aneurysmal bone cyst, Langerhans cell histiocytosis, and Paget's disease. Local MR staging is based on the compartmental anatomy. The psoas and gluteal muscles are easily invaded by sarcoma originating in the ileum. The pectineus muscle protects the neurovascular bundle at the level of the hip. The thigh is separated into three compartments, some structures (Sartorius muscle) cross borders between compartments. Immobile joints (SI-joints, osteoarthritic hip) are relatively easily crossed by sarcoma and giant cell tumor.
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PMID:Bone and soft tissue tumors of hip and pelvis. 2152 68


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