Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1261473 (sarcoma)
 25,952 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 43-year-old woman with a past medical history of breast cancer and an acute myeloid leukemia (AML) presented with headache over a 3-week period. The clinical examination was completely unremarkable. CT and MRI scans showed a contrast enhancing lesion in the left temporal lobe. Histopathologic examination revealed a malignant, hematopoietic tumor with high mitotic activity, areas of necrosis and diffuse infiltration of the brain parenchyma. Positive staining for Chloroacetateesterase and lysozyme of tumor cells identified its myeloid lineage. The diagnosis was granulocytic sarcoma (GS)/chloroma, a metastatic manifestation of AML. Granulocytic sarcoma (GS) most often occurs in patients with AML, myelodysplastic syndromes and myeloproliferative disorders, and can involve any organ. However intracerebral manifestation of GS is a rare event. In this case histopathological features and differential diagnoses of intracerebral GS are discussed.
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PMID:A 43-year-old woman with a temporal mass. 1661 89

A 31-year-old man was referred to our department for investigation and treatment of a rapidly-growing mass in the perineal region. MRI showed a periurethral tumor in the perineal region measuring 3.5cm in maximum diameter and bilateral inguinal lymph nodes enlargement. Therefore, the tumor was extirpated immediately with bilateral inguinal lymphadenectomy. The pathological examination with immunohistochemistry revealed that the tumor was proximal-type epithelioid sarcoma. Multidisciplinary therapy with irradiation, chemotherapy, and tumor ablation with a microwave tissue coagulator, was not effective for local recurrence and metastasis, and finally he died of progressive disease 14 months after the operation. Epithelioid sarcoma originating from the thigh or trunk is classified into the proximal-type, which shows significantly poorer prognosis than the distal-type that develops in the distal extremities. The proximal-type epithelioid sarcoma of the perineum is rare and, to our knowledge, the present report is the fourth case in the Japanese literatures.
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PMID:[Case of proximal-type epithelioid sarcoma of the perineum]. 1661 63

The purpose was to evaluate the safety and efficacy of high-dose-rate (HDR) CT-guided interstitial brachytherapy in the treatment of extrahepatic, extrapulmonary, secondary malignancies. Nineteen patients were included in this prospective study. The median age was 66 years (49-77). Underlying primaries comprised colorectal carcinomas in six, renal cell carcinoma in three, pancreatic carcinoma in three, cervical cancer in two, endometrial cancer in two and NSCLC, breast cancer and sarcoma in one patient each. All patients had undergone extensive pretreatments. CT-guided HDR brachytherapy employed a 192Iridium source. Dose planning for brachytherapy was performed using 3D CT data acquired after CT-guided percutaneous applicator positioning. MRI follow-up was performed 6 weeks and every 3 months post intervention. Primary endpoints were complications, local tumor control and progression-free survival. The median tumor diameter was 6 cm (2-15 cm). Tumor locations included the hepatoduodenal ligament, mesentery, adrenal gland, mesogastrium and local recurrences after rectal or pancreatic cancer. The minimal median dose in the target volume was 11 Gy (4-18 Gy). Minor complications comprised pain and fever (n=6, 32%). Major complications included one hospital death of unknown causes (n=1; 5%). Median follow-up was 7 months (1-16). Four patients (21%) died during the follow-up period. Local tumor control was 76.5% after 6 months and progression-free survival 47% after 6 months. Minimally invasive CT-guided HDR brachytherapy is safe and effective in the palliative treatment of extrahepatic, extrapulmonary secondary malignancies.
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PMID:CT-guided interstitial brachytherapy in the local treatment of extrahepatic, extrapulmonary secondary malignancies. 1662 47

Primary malignant lesions of the heart are rare. Although myxomas have been extensively described, there is a paucity of large studies on non-myxomatous cardiac tumours. On the other hand, there are several case reports on specific histopathological variants, in small numbers. Consequently there exists no consensus on therapeutic modalities for cardiac sarcomas. The prognosis for these lesions remains dismal, despite the enhanced diagnostic ability of newer technology. The reasons for the dismal prognosis are (1) the advanced tumour stage at presentation, (2) non-specific symptomatolgy, (3) insufficient awareness of these lesions, due to their rarity, (4) delayed diagnosis and/or misdiagnosis, which leads to (5) advanced tumour stage at presentation. Thus a vicious cycle is created. This article addresses these issues, deals with the surgically relevant modes of presentation, rather than the histopathology, and reviews the diagnosis and management options for the various sarcomas, categorized by the site and extent of cardiac involvement. Clinicians should be familiar with the presentation of these tumours and have a high index of suspicion, since the potential for long-term survival following resection does exist. Wide surgical resection remains the cornerstone of sarcoma therapy. Complete characterization of tumour extent using echocardiography and CT/MRI is mandatory to achieve this goal. Radical resections such as 'bench surgery' and transplantation may reduce local recurrence, but the risk of metastatic disease remains. The clinical experience with such approaches is limited. The role of adjuvant therapy is not yet established. In no other field of cardiac surgery would a multidisciplinary approach be more useful, in achieving cure or long-term palliation.
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PMID:Primary cardiac sarcoma. 1704 73

Retinoblastoma is the most frequent eye tumor in children, with an incidence of 1/15 000 births. Sixty per cent are unilateral: the median age at diagnosis is 2 years and most of these forms are not hereditary. Retinoblastoma is bilateral in 40%: the median age at diagnosis is 1 year. All bilateral and multifocal unilateral forms are hereditary. Hereditary retinoblastoma constitutes a cancer predisposition syndrome: a subject carrying a constitutional RB1 gene mutation has a greater than 90% risk of developing retinoblastoma, but is also at increased risk of developing secondary cancers. The 2 most frequent revealing symptoms are leucocoria and strabismus. Diagnosis is made by fundoscopy. US, MRI, CT scans may contribute to diagnosis. Management of patients with retinoblastoma must take into account the various aspects of the disease: the visual risk, the possibly hereditary nature of the disease and the life-threatening risk. Enucleation is still often necessary in unilateral disease; adjuvant treatment is decided according to the histological risk factors. Conservative treatment of at least 1 eye is possible in most of the bilateral cases: laser alone or combined with chemotherapy, cryotherapy and brachytherapy. The indication for external beam radiotherapy should be restricted to large ocular tumors and diffuse vitreous seeding because of the risk of late effects, including secondary sarcoma. Long-term follow-up and early information to retinoblastoma patients regarding the risk of second primary tumors and transmission is actually important.
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PMID:[Retinoblatoma: a review]. 1693 Sep 63

Alveolar soft-part sarcoma (ASPS) is rare. We present a case of a 30-year-old woman with a 6-month history of a palpable mass in her left thigh. MRI showed an 8-cm mass and a satellite nodule at the left gluteus muscle. The main tumor exhibited an isointense signal on T1-weighted images and high-signal-intensity areas with low-signal-intensity scanty solid components on T2-weighted images. MR angiography showed dilated and tortuous veins around the tumor. Histologic findings were compatible with ASPS.
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PMID:MRI of alveolar soft-part sarcoma. 1705 31

The diagnostic and therapeutic management of patients with soft-tissue tumors would be similar to the approach used for bone tumors if it were not for one crucial factor: the absolute necessity to recognize a sarcoma. The predominant features are the size of the tumor and its superficial or deep localization. If the tumor is small and superficial, biopsy can be associated with immediate resection without risk of dissemination to the deep tissues: this is the biopsy-resection approach. If the tumor is deep or superficial but large sized, search for locoregional spread with MRI is necessary before undertaking any surgical procedure. MRI can help guide the biopsy and plan resection if the tumor is a sarcoma. A first biopsy is necessary to establish the histological diagnosis and elaborate the therapeutic strategy. Samples should be sent immediately to the pathology lab which should examine sterile fresh tissue. Experience has demonstrated that proper rules for diagnosis and treatment are not necessarily applied initially in approximately one-fourth of all subjects with a malignant soft-tissue tumor. Besides the medical problems caused by this situation, the patient loses a chance for cure. When the tumor is a sarcoma, surgery is the basis of treatment. Complementary radiation therapy may be necessary, particularly for high-grade tumors or if the surgical margin was insufficient. Systemic or locoregional chemotherapy can also be used for high-grade or non-resectable tumors.
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PMID:[Diagnosis and treatment of soft-tissue tumors]. 1712 47

This is a case of a 36-year-old gentleman with haemophilia A who was presented with an acute atraumatic soft tissue swelling in the right thigh. Open biopsy was performed with the resultant diagnosis of a synovial cell sarcoma. Although the clinical findings were nonspecific they could easily have been found in a bleeding haemophilic pseudotumour. The findings reported on MRI scan initially were highly consistent with those present in patients with mild haemophilia. An important part of orthopaedic management in haemophilia is concerned with intraarticular and intramuscular bleeding. Haematomas are common and sarcomas are rare. However the absence of trauma should alert the clinician to the possibility that the abnormality may represent haemorrhage into a tumour and not just haematoma, even in a haemophilic patient.
Sarcoma 2006
PMID:Synovial sarcoma mimicking haemophilic pseudotumour. 1725 56

Novel strategies for cancer treatment involving macromolecular therapeutic agents have been recently developed and show promising results. Inadequate and heterogeneous uptake in tumor tissue has been shown to be a major obstacle for these compounds in clinical cancer therapy. Such distributions have been difficult to account for in predictive models. A three-dimensional computational model was developed to investigate the role of heterogeneous vasculature on interstitial transport within a murine sarcoma. The model accounts for extravasation and extracellular transport in a porous media. Spatial variation of fluid filtration rate per unit volume of tissue and vascular permeability were estimated from a dynamic contrast-enhanced (DCE)-MRI data set. Fluid filtration (L(p)S/V) and permeability (PS/V) maps were embedded in a model of tumor tissue and used to predict interstitial fluid pressure (IFP) and fluid flow. As in previous studies, pressure profiles were predicted to be elevated within the tumor. The model predicted boundary-dependent variation in outwardly directed interstitial velocity with lower velocities predicted near the skin boundary. Simulated tissue distribution of a macromolecular albumin tracer (MW approximately 60 kDa) was found to be heterogeneous with lower concentrations predicted in certain central regions. Simulated distributions of Gd-DTPA tracer (MW approximately 0.57 kDa) were less heterogeneous than albumin tracer. In sensitivity analysis, predicted tracer uptake was enhanced by increasing vascular leakiness. Increasing the interstitial hydraulic conductivity relative to the surrounding tissue reduced the overall drug uptake.
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PMID:Effect of heterogeneous vasculature on interstitial transport within a solid tumor. 1730 3

Cat-scratch disease is an infection by Bartonella henselea and resolves spontaneously. We present a case of untypical Bartonella infection which was misinterpreted by MRI as a soft-tissue sarcoma of the upper arm. The histological findings of an incision biopsy were unspecific, without signs of malignancy. The tumor was excised completely and the final histology, however, showed no evidence of malignant changes. The Bartonella henselae infection could be confirmed by polymerase chain reaction. The untypical presentation of cat-scratch disease should be considered in the differential diagnosis of clinically suspected soft tissue sarcoma.
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PMID:[Painful soft-tissue swelling of the upper arm]. 1744 4


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