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1/16. Microvascular endothelial abnormality in skeletal muscle from a patient with gastric cancer without dermatomyositis.

    We found a microvascular endothelial abnormality in a biopsy specimen from the gastrocnemius muscle of a patient with gastric cancer, who had severe myalgia and angialgia in the calf region with the symptoms of thrombophlebitis. There were no definite findings of inflammatory myopathy in histochemical and immunohistochemical studies. Electron microscopic examination revealed the accumulation of abnormal mitochondria in the subsarcolemmal area, and a fair number of degenerating capillaries. Immunohistochemical analysis of procoagulant or anticoagulant factors revealed marked reduction of thrombomodulin (TM) expression on small vessels and capillaries. Although a reduction of TM on small vessels has been observed around perifascicular atrophic fibers in patients with dermatomyositis, histochemical findings of the present patient showed no perifascicular atrophy or severely degenerating fibers. These pathological findings in the patient may be related to a malignant neoplasm and may be one of the causes of disseminated intravascular coagulation (DIC), which is the main complication of malignant neoplasms. Further studies are necessary to determine whether the reduction of TM on the small vessels and capillaries in skeletal muscle is a predictor of some severe condition such as DIC or a rare pathological finding in some special condition such as scirrhous carcinoma with thrombophlebitis.
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ranking = 1
keywords = neoplasm
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2/16. Epithelioid hemangioendothelioma of the liver associated with thrombocytopenia and coagulopathy.

    Epithelioid hemangioendothelioma of the liver is a rare vascular neoplasm with intermediate malignant potential. The prognosis is highly unpredictable. We report the case of a 59-year-old woman who had the tumor radically resected, but multiple metastases of the liver developed associated with thrombocytopenia and consumption coagulopathy, as observed in kasabach-merritt syndrome. The patient did not respond to any treatment and the behavior of the tumor was very aggressive. The patient died 15 months after radical resection of the tumor.
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ranking = 0.5
keywords = neoplasm
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3/16. disseminated intravascular coagulation during total hip replacement.

    disseminated intravascular coagulation (DIC) is an extremely serious hemorrhagic disorder and one that is potentially lethal. DIC is more frequently observed as a complication of obstetric pathologies, diffused neoplasms, bacterial sepsis, blood transfusions, traumas and adipose embolias. The literature reports very few cases occurring after total hip replacement, two of which the cause of death. In all of the cases described one or boh prosthetic components are cemented. The authors report a case observed after total hip replacement in a patient submitted one year previously to contralateral hip replacement. Severe hypotension, bradychardia and profuse bleeding of the wound and from the drainages one hour after suturing constituted the initial symptom. Recognition of the clinical findings, after exclusion of a iatrogenic lesion, and subsequent treatment in intensive therapy allowed us to save the patient. The syndrome must be suspected and immediately diagnosed when profuse bleeding that cannot be related to vascular lesion is observed immediately postsurgery after cemented or hybrid hip replacement. Confirmation through hematological tests and timely treatment in a specialized center are indispensable requirements to save the life of the patient.
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ranking = 0.5
keywords = neoplasm
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4/16. Acute myeloid leukemia mimicking primary testicular neoplasm. Presentation of a case with review of literature.

    We describe a new unique case of acute myeloid leukemia (AML) in a 21-yr-old male presenting with abdominal pain, bilateral testicular masses and gynecomastia. Further work-up with computed tomography of the chest, abdomen and pelvis revealed massive retroperitoneal, peripancreatic and mediastinal lymphadenopathy, suggesting primary testicular neoplasm. The patient was subjected to right orchiectomy that showed infiltration of testicular tissue with malignant cells, originally misinterpreted as undifferentiated carcinoma. immunohistochemistry studies, however, showed these cells to be strongly positive for myeloperoxidase and CD45, indicating a myeloid cell origin. bone marrow (BM) aspirate and biopsy demonstrated replacement of marrow with immature myeloid cells. Both the morphology and immunophenotype of the blast cells were consistent with AML type M4 (acute myelo-monocytic leukemia), using French-American-British (FAB) classification. The patient received standard induction chemotherapy with cytosine arabinoside (ARA-C) and daunorubicin followed with two cycles of consolidation therapy with high dose ARA-C, which resulted in remission of BM disease and resolution of lymphadenopathy and left testicular masses. After the second cycle of consolidation therapy, the patient developed sepsis that was complicated by refractory disseminated intravascular coagulopathy. He expired with a clinical picture of multiple organ failure. The unique features of this case are presented and the related literature is reviewed.
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ranking = 2.5
keywords = neoplasm
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5/16. Infantile fibrosarcoma of the hand associated with coagulopathy.

    BACKGROUND: Large congenital neoplasms of the extremities may be associated with coagulopathies and significant hemorrhage in the neonatal period. At times, the differences between coagulation derangements can be very subtle, leading to errors in diagnosis. Infants with vascular lesions and coagulopathies are often found to have the Kasabach-Merritt phenomenon, which is a platelet-trapping coagulopathy. However, other neoplasms or vascular malformations can be accompanied by disseminated intravascular coagulation. It is important to obtain accurate diagnoses of the neoplasm and the coagulopathy because the treatments of similar-appearing tumors and coagulopathies can be markedly different. methods: The authors report the case of a newborn with a congenital tumor of the left hand that was accompanied by a coagulopathy that caused significant bleeding. RESULTS: A presumption was made by the neonatal critical care physicians and hematologists that the infant had a kaposiform hemangioendothelioma along with the Kasabach-Merritt phenomenon. However, steroid treatment did not reduce the size of the mass or correct the coagulopathy. Only after obtaining consultation with a hand surgeon and a tissue diagnosis was it learned that the patient had an infantile fibrosarcoma that was accompanied by disseminated intravascular coagulation. Limb-sparing resection of the lesion along with chemotherapy markedly improved the patient's condition. CONCLUSIONS: Large congenital neoplasms presenting with attendant bleeding diatheses must be rapidly and accurately diagnosed with both a biopsy-proven tissue diagnosis and a hematologic characterization of the nature of the coagulopathy. The differential diagnosis of a vascular-appearing mass in the extremity can be subtle, and presumptive diagnosis, as occurred in this case, can lead to incorrect or delayed treatment. Specifically, kaposiform hemangioendothelioma must be differentiated from infantile fibrosarcoma. The principles of infantile fibrosarcoma treatment are limb-sparing resection and chemotherapy.
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ranking = 2
keywords = neoplasm
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6/16. Congenital mesoblastic nephroma, hemorrhagic shock, and disseminated intravascular coagulation in a newborn infant.

    Congenital mesoblastic nephroma is a rare tumor of infancy that usually presents as an asymptomatic abdominal mass. A full-term newborn infant with an atypical variant of this neoplasm developed hemorrhagic shock and disseminated intravascular coagulation. The stormy course was complicated by persistent fetal circulation and then the inability to withdraw ventilatory support due to the mass effect of the tumor. After the removal of the tumor at 10 days of age, transient conjugated hyperbilirubinemia developed. At 15 months of age, the infant was thriving without evidence of recurrence of the tumor.
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ranking = 0.5
keywords = neoplasm
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7/16. Metastatic angiosarcoma of the ovary.

    A case of metastatic angiosarcoma of the ovary, complicated by disseminated intravascular coagulation, is described. The tumour was diagnosed by histopathologic examination and immunohistochemical studies. Angiosarcoma of the ovary, although extremely rare, appears to be a highly malignant neoplasm with an aggressive course and effective therapy is unknown.
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ranking = 0.5
keywords = neoplasm
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8/16. Non-immune fetal hydrops with hepatic hemangioendothelioma and kasabach-merritt syndrome: a case report.

    A premature infant presented with non-immune hydrops fetalis, a liver mass, thrombocytopenia, and hypofibrinogenemia. Histologic examination of the liver tumor showed an infantile hemangioendothelioma. The clinical features of this case can be explained by anemia, hypoalbuminemia, and coagulopathy. The association with kasabach-merritt syndrome, the pathophysiology of non-immune hydrops fetalis, and primary hepatic neoplasms of the neonate are discussed.
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ranking = 0.5
keywords = neoplasm
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9/16. Subdural hematoma secondary to metastatic neoplasm: report of two cases and a review of the literature.

    Subdural hematoma secondary to dural metastases is a rare event. Vascular obstruction due to invasion of dural vessels is an important etiological factor. When this is associated with a clotting defect, the subdural hematoma can cause significant clinical symptoms and death. Two cases are presented with a review of the literature.
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ranking = 2
keywords = neoplasm
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10/16. adenocarcinoma of Skene's duct associated with a systemic coagulopathy.

    adenocarcinoma of the urethra is a rare neoplasm which is believed to arise from the periurethral ducts. Four such cases have been diagnosed at our institution during the past 25 years and their clinical courses are summarized in this report. In one patient, a disseminated coagulopathy with a predominantly fibrinolytic component developed. The pathologic mechanism of fibrinolysis is addressed and a successful approach to the medical management of this confusing coagulopathy is outlined.
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ranking = 0.5
keywords = neoplasm
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