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)

Adrenal cortical carcinosarcoma is a rare variant of adrenal cortical carcinoma. Sarcomatous change in adrenal cortical carcinomas is exceptionally rare, with only 9 cases previously described. Adrenal cortical carcinosarcomas tend to be aggressive tumors, with locoregional recurrence and rapid metastases from the sarcomatoid component. We describe what seems to be the first case of sarcoma arising in oncocytic adrenal cortical carcinoma. The sarcomatous component here was pleomorphic rhabdomyosarcoma. This occurred in a 45-year-old man who had nodal and pulmonary metastases of the rhabdomyosarcomatous component at presentation and who died of progressive disease 11 months later. Here, we discuss the clinical, radiologic, and pathologic findings and review the literature on adrenal cortical carcinosarcomas.
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PMID:Oncocytic adrenal cortical carcinosarcoma with pleomorphic rhabdomyosarcomatous metastases. 2234 39

A 14-year-old boy presented with chronic myelogenous leukemia (CML) in blast phase with segregated extramedullary (nodal) myeloid sarcoma and T-lymphoblastic lymphoma. Immunohistochemical stains performed on the lymphadenectomy sample demonstrated T lymphoblasts in the lymph nodes and myeloblasts in the adjacent soft tissue. Fluorescence in situ hybridization performed on paraffin sections confirmed that both T-lymphoblast and myeloblast populations were positive for the t(9;22) BCR/ABL1 translocation. Subsequent flow cytometry analysis of the bone marrow showed expanded populations of abnormal myeloblasts and T lymphoblasts diagnostic of blast phase CML. To the best of our knowledge, bilineal blast phase of CML with segregated extramedullary T lymphoblasts and myeloblasts has not been reported.
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PMID:Chronic myelogenous leukemia presenting in blast phase with nodal, bilineal myeloid sarcoma and T-lymphoblastic lymphoma in a child. 2317 Dec 93

Annexin A5 (Anxa5) promotes pancreatic adenocarcinoma, sarcoma, tumorigenesis and progression of breast cancer and prostate cancer stem cells. It is involved with metastasis, invasion and development of squamous cell carcinoma, and facilitates nodal progression of bladder cancer and angiogenesis and progression of glioma. Anxa5 de-regulation is associated with drug resistance in nasopharyngeal carcinoma and gastric cancer. Although Anxa5 protein up-regulation promotes cervical cancer progression, it is markedly suppressed in cervical carcinoma cells. Anxa5 is negatively correlated with thyroid cancer malignancy. In this review, we explore the mechanisms of Anxa5 action in tumors. Anxa5 could be a predictive biomarker for tumor development, metastasis and invasion, and be of diagnostic, prognostic and therapeutic significance in cancer.
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PMID:Annexin A5 as a potential marker in tumors. 2412 Oct 31

Histiocytic sarcoma is a rare hematologic malignant neoplasia originating from histiocytic or dendritic cell clones. The lesions may be in nodal or extranodal sites, most commonly in the gastrointestinal tract. A small number of cases presents as unique cutaneous lesions. The definitive diagnosis is made by positivity for the immunohistochemical markers CD163, CD68, CD4 and lysozyme. The treatment is controversial, often with combined systemic chemotherapy. This is a case of cutaneous histiocytic sarcoma in an 82-year-old patient presenting two nodular lesions in the breast and right arm which were treated with simple excision and multidisciplinary follow-up, avoiding aggressive management and exhaustive investigations. Although most studies report aggressive evolution, the patient had good and stable clinical status during the twelve-month follow-up period.
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PMID:Case report of cutaneous histiocytic sarcoma: diagnostic and therapeutic dilemmas. 2417 90

Myeloid sarcoma (MS), previously known as granulocytic sarcoma, is a rare, localized, tumor mass composed of myeloid precursor cells, with or without maturation, and occurring at an anatomical site other than the bone marrow (BM). Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), in contrast, is a B-cell hematological malignancy. We describe the first reported case of concurrent presentation of nodal MS and of BM CLL/SLL in the same patient. Fatal leukemic central nervous system infiltration was the final outcome. We provide possible explanations and investigate the pathophysiology of this unique, previously unreported co-morbidity.
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PMID:Concurrent presentation of nodal myeloid sarcoma and bone marrow chronic lymphocytic leukemia/small lymphocytic lymphoma: a unique association. 2450 38

Cancer robustness is generated by the positive feedback loops. The positive loops hyperactivate AKT locus forming a cancer phenotype in leukemia, lymphoma, myeloma, plasmocytoma, sarcoma and carcinoma. The positive loops inducing AKT hyperphosphorylation increase activity of the AKT locus and the nodal associated and interconnected signaling genes. Only genes expressed above the threshold in the AKT signaling interactome networks, participate in the formation of the complex cancer phenotype. AKT is the switching locus for the cancer phenotype. The phenotype formation and maintenance is regulated by the AKT locus through an entropy/enthalpy processes. Targeting the AKT by locus chemotherapy, changing redox balance (antioxidant/oxidant), affects phosphorylation and activity of the AKT, inducing conversion of the positive feedback loops and disappearance of the malignant phenotype.
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PMID:AKT as locus of cancer phenotype. 2516 73

Sarcomatoid variant of anaplastic large cell lymphoma (ALCL) is one of the rarest histologic variants of ALCL that consists of large, bizarre, often spindle-shaped, neoplastic cells resembling a soft tissue sarcoma. We report here such a case of ALCL with both pulmonary and multiple nodal involvement in a 47-year-old woman who initially presented with fever, cough, sputum, itching skin, and weight loss. The initial transbronchial lung biopsy showed discohesive pleomorphic malignant cells in a strong inflammatory milieu reminiscent of inflammatory malignant fibrous histiocytoma (MFH). Subsequent cervical lymph node biopsy revealed a spindle cell sarcoma predominantly composed of plump spindle and oval neoplastic cells in interweaving fascicles, with sparse inflammatory infiltrates, resembling pleomorphic-storiform type of MFH. However, these tumor cells in the lung and node lesions revealed essentially similar immunohistochemical features that were positive for CD30, EMA, TIA-1, granzyme B, and fascin, but negative for anaplastic lymphoma kinase (ALK), and T- or B-lineage-specific marker. The spindled cells stains diffuse strong positive for smooth muscle actin (SMA), along with vimentin. Further studies showed that the tumor produced large quantities of the proinflammatory cytokines interleukin-2 (IL-2), IL-6, and IL-8, which we believe may contribute to the pathogenesis of sarcomatoid transformation of this tumor, and was associated with the patient's inflammatory symptoms. To the best of our knowledge, this is the first reported case of sarcomatoid variant of ALK-negative ALCL with null cell phenotype and in situ production of proinflammatory cytokines presenting as multiple nodes and pulmonary involvement.
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PMID:Sarcomatoid variant of ALK- anaplastic large cell lymphoma involving multiple lymph nodes and both lungs with production of proinflammatory cytokines: report of a case and review of literature. 2519 51

Our understanding of the association between synovial sarcoma and peripheral neurolymphomatosis is limited to a few case reports in literature. Delay in diagnosis or misdiagnosis is possible due to its insidious onset and varied presentation compounded by non-specific imaging findings. Needle biopsy also may not be confirmatory especially, in cases of biphasic sarcoma as in our case, and it may be necessary to proceed to open biopsy. Here, is a case of a non-tender right calf muscle mass, which was reported as biphasic synovial sarcoma by FNAC. Positron emission tomography computed tomography-computed tomography (PETCT) showed right sciatic nerve involvement and multiple infra diaphragmatic lymph nodal lesions. Intensity of (18)F FDG ((18)Flourine labeled fluro de oxy glucose) uptake and the infra diaphragmatic lymph nodal lesions distribution, was more in favour of a lymphoma diagnosis rather than a sarcoma, (which are usually low metabolically active tumors). Thus, this case highlights the usefulness of FDG PETCT in arriving at a diagnosis in the background of indeterminate clinicopathological and radiologic findings.
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PMID:Primary peripheral neurolymphomatosis mimicking synovial sarcoma: FDG PETCT to the rescue. 2531 68

Sentinel lymph node biopsy (SLNB) is an established staging technique in many malignancies, but reports describing this procedure for the evaluation of regional lymph nodes in childhood and adolescents are still scarce. SLNB has progressively gained a role in the staging of pediatric melanoma and some histotypes among soft tissue sarcomas (rhabdomyosarcoma, synovial sarcoma, epithelioid sarcoma, and clear cell sarcoma). We report our experience with 29 patients who were evaluated with SLNB during the past 12 years: 9 alveolar rhabdomyosarcoma, 1 embryonal rhabdomyosarcoma, 7 Ewing Sarcoma/pPNET, 1 clear cells sarcoma, 2 epithelioid sarcoma, 1 epithelioid sclerosing fibrosarcoma, 3 myxoid liposarcoma, 1 adult fibrosarcoma, 1 malignant peripheral nerve sheath tumor, 1 undifferentiated sarcoma, 1 sarcoma not otherwise specified, and 1 melanoma. All, but one, patients underwent a lymphoscintigraphy, and in more than half of the patients, an intraoperative lymphatic mapping with a blue dye injection was performed. In our experience, SLNB was a feasible and reliable technique that allowed a targeted sampling of regional lymph nodes. This technique avoids aggressive operations or random biopsies, leading to an accurate staging without complications. Recently new imaging techniques, such as positron emission tomography-computed tomography scan, have obtained good results in identifying distant and lymphatic metastases, but they have been found to be less reliable than SLNB, especially for small volume metastatic nodal disease in sarcoma.
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PMID:Sentinel node biopsy in pediatric patients: the experience in a single institution. 2547 65

Loss of expression of the SMARCB1 (INI1/BAF47/SNF5) tumor-suppressor protein, originally identified in pediatric malignant rhabdoid tumors, has been noted in significant percentages of epithelioid sarcomas of classical and proximal-type and in myoepithelial carcinomas. Epithelioid sarcoma and myoepithelial carcinoma are very rare in the vulvar region, and few of these cases have been evaluated for SMARCB1 protein loss by immunohistochemistry (IHC) or for SMARCB1 gene alterations by molecular genetic techniques. We studied the clinicopathologic, IHC, and molecular genetic features of 14 SMARCB1-deficient vulvar neoplasms. All available routinely stained sections were reexamined, and IHC analysis for wide-spectrum cytokeratins, high-molecular weight cytokeratins, epithelial membrane antigen, S100 protein, CD34, smooth muscle actin, desmin, and SMARCB1 was performed. Multiplex ligation-dependent probe amplification and DNA sequencing of the SMARCB1 gene was performed on 12 cases with sufficient available tissue. The 14 vulvar tumors occurred in adult women (mean age 46 y, range 22 to 62 y) and measured 1.1 to 8.8 cm in size (mean 4.7 cm). Tumors were classified as classical-type epithelioid sarcoma (N=1), proximal-type epithelioid sarcoma (N=6), myoepithelial carcinoma (N=4), and "SMARCB1-deficient vulvar sarcoma, not otherwise specified" (N=3) on the basis of combined histopathologic and IHC findings. One myoepithelial carcinoma showed divergent rhabdomyoblastic differentiation. All tested cases showed partial or complete SMARCB1 deletions (homozygous: 9 cases; heterozygous: 3 cases). One case with a heterozygous deletion also showed a c.528delC mutation in exon 5. Fluorescence in situ hybridization for EWSR1 rearrangement was performed for 3 cases classified as myoepithelial carcinoma and was negative. Follow-up (13 patients, range 5 to 72 mo, mean 31 mo) data showed 3 patients dead of disease, 1 alive with unresectable metastatic disease, 1 alive with radiographic evidence of extensive lymph nodal disease, and 8 alive without disease. We conclude that SMARCB1-deficient vulvar neoplasms chiefly comprise epithelioid sarcoma and myoepithelial carcinoma, although some defy easy classification. No association was seen between clinical behavior and the type of SMARCB1 alteration.
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PMID:SMARCB1-deficient Vulvar Neoplasms: A Clinicopathologic, Immunohistochemical, and Molecular Genetic Study of 14 Cases. 2565 69


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