Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Epithelioid sarcomas are rare, morphologically distinct tumors that have a propensity to arise in the extremities. Brain metastasis from epithelioid sarcoma are a relatively rare occurrence. We report a case of brain metastasis in a 50-year-old man who was previously diagnosed with an epithelioid sarcoma arising in the elbow. Before the diagnosis of brain metastasis, he had developed an axillary lymph node metastasis. He presented with neurologic symptoms of progressively worsening headache and loss of vision on the right side. He underwent gross total resection of an occipital lobe mass. Histologically, the tumor was focally characterized by prominent perinecrotic pseudopalisading and demonstrated immunoreactivity with antibodies to cytokeratin AE1/3 and CAM5.2; the tumor did not stain with glial fibrillary acidic protein antibody. The literature is reviewed and the morphologic distinction between metastatic epithelioid sarcoma and other central nervous system neoplasms is discussed.
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PMID:Metastatic epithelioid sarcoma to the brain: palisaded necrosis mimicking glioblastoma multiforme. 1237 23

Systemic mastocytosis (SM), as opposed to cutaneous-only mastocytosis, implies the presence of neoplastic mast cell infiltration in extracutaneous tissue. Mast cell disease in adults is often systemic and often involves the bone marrow. Typical clinical and laboratory features of SM include urticaria pigmentosa, mast cell mediator symptoms (eg, headache, flushing, lightheadedness, urticaria and pruritus, nausea, diarrhea, abdominal pain, and vasodilatory shock), bone pain (eg, osteoporosis, lytic bone lesions, and fractures), hepatosplenomegaly, cytopenia, eosinophilia, elevated serum tryptase and histamine, and bone marrow fibrosis and angiogenesis. SM may be indolent (no evidence of organ dysfunction), aggressive (presence of organ dysfunction), associated with another often chronic myeloid hematologic disease (SM-AHD), or present as mast cell leukemia or sarcoma. Mast cell-mediator symptoms are treated with histamine antagonists and cromolyn sodium. Indolent SM does not require cytoreductive therapy. Aggressive SM and SM-AHD are managed based on their molecular profile. Recent information suggests that FIP1-like-1-platelet-derived growth factor receptor-alpha(+) SM responds well to imatinib mesylate, whereas interferon-alpha should be considered as a first-line treatment in all of the other cases, including patients with Asp816Val(+) SM. Cladribine has been shown to be effective in patients who develop resistance to interferon treatment.
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PMID:Systemic mastocytosis: current concepts and treatment advances. 1508 68

Apart from calvarial infiltration, intracranial involvement in multiple myeloma is uncommon. Diffuse leptomeningeal invasion with or without parenchymal involvement is most common. Dural infiltration without involvement of the parenchyma, leptomeninges or skull is rare. The differential diagnosis of a dural plasmacytoma includes meningioma, which has a similar MRI appearance, metastasis, lymphoma and sarcoma of the dura mater. We present a patient with multiple myeloma presenting with an intracerebral mass mimicking a meningioma on MRI. Multiple myeloma had been diagnosed seven years previously. The patient presented with headache and speech disturbance 12 months after autologous peripheral stem cell transplantation for recurrence of multiple myeloma. MRI revealed a left temporal extra-axial mass with a dural tail mimicking meningioma. Histopathological examination of the mass after excision showed multiple myeloma immunopositive for IgG, kappa light chain and CD38. There was no recurrence after postoperative radiotherapy. Plasmacytoma should be considered in the differential diagnosis of a solitary dural mass, particularly in a patient with multiple myeloma.
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PMID:Dural plasmacytoma mimicking meningioma in a patient with multiple myeloma. 1645 88

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

Intracranial granulocytic sarcoma (chloroma) may occur rarely in leukemia. A 27-year-old male presented with an isolated recurrence of granulocytic sarcoma manifesting as an intraaxial mass 27 months after complete remission of acute lymphoblastic leukemia. He was admitted due to a severe headache and blurred vision. Brain magnetic resonance imaging demonstrated an enhanced mass which was initially interpreted as an extraaxial tumor in the right temporal region. Because of increased intracranial pressure and the mass effect, open biopsy with surgical resection was performed. The biopsy result indicated that intraaxial lymphoblastic leukemia infiltration had caused CNS relapse. Although granulocytic sarcoma occurs primarily in patients with acute myelogenous leukemia, the authors report a rare case of intraparenchymal granulocytic sarcoma in acute lymphoblastic leukemia.
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PMID:Isolated recurrence of intracerebral granulocytic sarcoma in acute lymphoblastic leukemia: a case report. 1664 13

Sarcomas constitute 5% of all of head and neck neoplasms. Angiosarcomas comprise 2% of all soft tissue tumors, 60% appear in skin and soft tissue, 50% in the head and neck and only 4% are present in the upper aerodigestive tract. We report a case of a 52 year old healthy woman with headache, weeping, foreign body sensation in the left nostril and epixtasis. The TAC identified a tumor in the left nostril that invaded the osseous lamina of the skull. The histopathologic diagnosis was angiosarcoma. We carried out a literature review and analysis from 1976 to 2004. Agiosarcomas of the upper aerodigestive tract have a better prognosis and long term survival free of disease than their soft tissue and skin counterparts. Recurrence is strongly correlated with positive resection margins. Complete surgery and radiotherapy are considered the optimal treatment schemes.
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PMID:[Nasal cavity angiosarcoma: a case report and literature review]. 1671 50

Granulocytic sarcoma is a rare tumor composed of immature granulocytic cells. Prognosis is poor. The dura and orbits are preferentially involved. An intracranial localization is unusual. We report the case of a 31-year-old man who was admitted with a history of headache and vomiting. The neurological examination revealed hemiplegia. Computed and tomography (CT) and magnetic resonance imaging (MRI) showed a lesion which was interpreted as a meningioma. After surgical resection, pathology examination led to the diagnosis of granulocytic sarcoma. The clinical and radiological features of this case are discussed with reference to other reports in the literature.
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PMID:[Granulocytic sarcoma of the brain revealed on vascular mode]. 1856 63

Low-grade fibromyxoid sarcoma was first described in 1987 as a rare soft tissue neoplasm characterized by a bland and deceptively benign histological appearance but with aggressive behavior. A 20-year-old male patient presented with a recent history of headache and seizure. A right frontal mass was detected on MRI and he was operated upon to remove the intracranial mass. Histological examination revealed mildly atypical fibroblastic cells embedded within a myxoid matrix. Nuclear atypia and pleomorphism were minimal, and necrosis was not present. The lesion was diagnosed as a low-grade fibromyxoid sarcoma. Although primary intracranial low-grade fibromyxoid sarcoma has characteristic histological features, clinical and radiological correlation is necessary to make the correct diagnosis.
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PMID:Primary intracranial low-grade fibromyxoid sarcoma (Evans tumor). 1877 26

By analogy to gliosarcoma, the term "ependymosarcoma" has recently been coined to thematize the rare phenomenon of a malignant mesenchymal component arising within an ependymoma. We report on an example of this paradigm, involving tanycytic ependymoma as the host tumor in a 40-year-old female who underwent two tumor extirpation procedures at one-year interval. She first presented with severe headaches, and was seen by imaging to harbor a moderately enhancing mass 2.5cm in diameter at the rostral septum pellucidum accompanied by occlusive hydrocephalus. Microscopically, the tumor consisted of solid, wavy fascicles of elongated cells that were occasionally interrupted by vague perivascular pseudorosettes. Mitotic activity was absent, and less than 1% of nuclei immunoreacted for MIB-1. A histological diagnosis of tanycytic ependymoma (WHO grade II) was rendered, and no adjuvant therapy given. At recurrence, the lesion was 3.5cm in diameter, intensely enhancing, and had already seeded into the subarachnoid space. Histology showed a biphasic glial-sarcomatous architecture with remnants of the original ependymoma now displaying hypercellularity and atypical - yet not frankly anaplastic - features. The sarcomatous moiety consisted of spindle and epithelioid cells densely interwoven with reticulin fibers. While the ependymal component was GFAP and S100 protein positive, and featured punctate staining for EMA, none of these markers was expressed in the adjacent sarcoma. Instead, the latter reacted for vimentin and smooth muscle actin. To the best of our knowledge, this is the first documentation of tanycytic ependymoma undergoing malignant transformation, one driven by a highly anaplastic mesenchymal component, corresponding to "ependymosarcoma".
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PMID:Rapid spontaneous malignant progression of supratentorial tanycytic ependymoma with sarcomatous features - "Ependymosarcoma". 1985 84

Mifamurtide, also known as liposomal muramyl tripeptide phosphatidyl ethanolamine (L-MTP-PE), has been approved for the treatment of osteosarcoma in Europe. Mifamurtide's rational drug design employs MTP-PE for macrophage activation in a multilamellar liposome drug carrier, containing the synthetic phospholipids 1-palmitoyl-2-oleoyl phosphatidyl choline (POPC) and 1,2-dioleoyl phosphatidyl serine (OOPS). Although the drug is not cytotoxic towards normal or tumor cells in vitro, immune activation against osteosarcoma lung metastases in vivo accounts for mifamurtide's antiosteosarcoma effects. Phosphatidyl serine-containing lipids signal macrophage cells that have "flipped phosphatidyl serine" to the outer membrane after apoptosis (e.g., after damage of tumor cells from chemotherapy); thus, both mifamurtide's active and inactive ingredients target immune cells in the lungs. Mifamurtide administration has resulted in 8% and 13% improvement in 6- and 5-year overall survivals, when added to chemotherapy in nonmetastatic and metastatic patients with osteosarcoma, respectively. The short-term toxicities of mifamurtide (fever, headache, flu-like symptoms and rigors) are reduced or eliminated using ibuprofen (200 mg) as premedication for the first infusion; an algorithm for pre- and postmedication is presented. To date, no long-term side effects of mifamurtide have been reported. Compassionate access programs based in two major cancer centers (MD Anderson and Memorial Sloan-Kettering), have recently provided this potentially life-saving drug in North America. The experience with mifamurtide provides an outstanding example of successful cooperation among regulatory bodies and agencies, the pharmaceutical industry and pediatric oncologists to improve cancer care and outcomes for children and young people with a rare sarcoma.
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PMID:Mifamurtide in osteosarcoma--a practical review. 2051 34


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