Cases reported "Abdominal Neoplasms"

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11/161. Myxoid liposarcoma with transition to round-cell lesion-cell cycle regulator genes and telomerase activity characterizing tumor progression: a case report.

    A mixed myxoid/round cell liposarcoma was macrodissected in its 2 histologic components and investigated for genetic differences between its low-grade myxoid and the high-grade round-cell region. For both, we failed to detect p53 gene mutations, loss of heterozygosity at the p53 or Rb genes, and p53 protein expression. The round-cell component showed a high telomerase activity, and an elevated c-myc mRNA and protein expression. The myxoid component was characterized by a lack of telomerase activity and low c-myc mRNA expression, and immunohistochemistry failed to detect the c-myc protein. There was a higher Mib-1 proliferation index in the round-cell portion. The same specific translocation t(12;16) and the fusion transcript type II in both components confirmed the close relationship between myxoid and round-cell liposarcomas. telomerase activity and increased c-myc expression seem to be helpful molecular markers for characterizing tumor progression in myxoid liposarcoma.
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ranking = 1
keywords = sarcoma
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12/161. Granulocytic sarcoma as the cause of giant abdominal mass: diagnosis by fine needle aspiration and review of the literature.

    Granulocytic sarcoma (GS) or chloroma is a neoplasia consisting of myeloid precursors in an extramedullary site. It is generally associated with myeloproliferative disorders especially with myeloid neoplasias. A young woman with huge abdominal mass due to GS associated with chronic myelocytic leukemia (CML) has been reported and literature is reviewed.
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ranking = 0.71428571428571
keywords = sarcoma
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13/161. Fine needle aspiration cytology of well-differentiated liposarcoma. A report of two cases.

    BACKGROUND: Well-differentiated liposarcoma is difficult to diagnose on fine needle aspiration cytology (FNAC) smears and may create considerable diagnostic problems. CASES: Males aged 60 and 45 years presented with a swelling in the groin and retroperitoneal region, respectively. FNAC showed large cells with multilobulated nuclei and mature-looking fat tissue. A soft tissue tumor with bizarre cells was diagnosed cytologically in case 1 and liposarcoma in case 2. Histologically, both cases were diagnosed as well-differentiated sclerosing liposarcoma. CONCLUSION: The cytologic diagnosis of well-differentiated liposarcoma should be done with caution, and the sites should be taken into consideration. Deep-seated tumors with large, bizarre, giant cells should have wide excision as they recur more frequently.
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ranking = 1.1428571428571
keywords = sarcoma
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14/161. Renal angiomyolipoma with epithelioid sarcomatous transformation and metastases: demonstration of the same genetic defects in the primary and metastatic lesions.

    angiomyolipoma (AML) is a benign neoplasm that occurs either sporadically or in patients with tuberous sclerosis complex (TSC) and shows frequent allelic losses at chromosome arm 16p. It has been suggested recently that the melanogenesis marker-positive perivascular epithelioid cell (PEC) has been found consistently in AML. The authors report a 50-year-old woman without evidence of TSC affected by classic renal AML containing an area composed of atypical epithelioid cells with the same morphoimmunophenotypic characters of PEC. After 7 years from surgical removal of the lesion, the patient developed a local recurrence and successive lung and abdominal metastases that showed morphologic and immunohistochemical features overlapping those of the epithelioid area of the previously removed AML. Genetic analysis showed that the classic AML and its epithelioid area as well as the pulmonary and abdominal metastases shared the same allelic loss on chromosome arm 16p. Based on these findings, the authors view this case as evidence of a malignant transformation of a classic AML with morphologic, immunophenotypic, and genetic demonstration of its clonal origin.
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ranking = 0.57142857142857
keywords = sarcoma
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15/161. Pathologic, cytologic and immunohistochemical findings of an intra-abdominal desmoplastic small round cell tumor in a 15-year-old male.

    The intra-abdominal desmoplastic small round cell tumor is a rare neoplasm. It usually occurs in young males and diffusely involves the peritoneum and pursues an aggressive clinical course. The present patient was a 15-year-old male who experienced abdominal pain and abdominal swelling. The patient was diagnosed with an intestinal myogenic sarcoma, and surgery for tumor resection was performed in June 1999. The tumor was a 20 x 15 x 15 cm well-defined mass in the peritoneum involving the transverse colon and stomach with peritoneal disseminations and splenic metastasis. Microscopic findings were well-defined nests composed of small round cells and separated by abundant desmoplastic stroma. Cytologically, the tumor cells consisted of small, round to oval cells with a scant amount of light blue cytoplasm. Immunohistochemically, the tumor cells were positive for anti-epithelial membrane antigen, vimentin, desmin, neuron-specific enolase and WT1 protein antibodies. Similar pathologic features with other small round cell tumors may lead to differential diagnostic difficulties that require the application of ancillary diagnostic methods, such as immunohistochemistry and cytogenetic techniques.
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ranking = 0.14285714285714
keywords = sarcoma
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16/161. Paraneoplastic pemphigus caused by an epithelioid leiomyosarcoma and associated with fatal respiratory failure.

    A patient is described who initially presented with pemphigus vulgaris, limited to the oral cavity, and weight loss. Although the various laboratory studies pointed to the diagnosis of paraneoplastic pemphigus (PNP), the underlying neoplasm was not detected until 6 months later, when the patient developed shortness of breath and routine physical examination on admission revealed an abdominal mass, which eventually was proven to be an epithelioid leiomyosarcoma. In spite of radical excision of the tumour and intensive treatment of the dyspnoea, the patient died of respiratory failure 19 months after the PNP had been diagnosed. early diagnosis of PNP is stressed to possibly prevent fatal pulmonary involvement.
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ranking = 0.71428571428571
keywords = sarcoma
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17/161. Emergency stent-graft repair of a ruptured hepatic artery secondary to local postoperative peritonitis.

    PURPOSE: To describe the use of a stent-graft for emergent repair of life-threatening hepatic artery hemorrhage. methods AND RESULTS: A 57-year-old man with a 17-year history of myxoid liposarcoma underwent surgery for a recurrent abdominal mass. Multivisceral resection including a Kausch-Whipple procedure with an extended right hemicolectomy was performed. Three weeks later, an episode of gastrointestinal bleeding prompted surgical repair of the hepatic artery, which had been eroded by infection due to a leaking bilioenteric anastomosis. After 3 weeks of programmed abdominal lavage, bleeding recurred. angiography documented another rupture of the proximal hepatic artery. After an unsuccessful attempt at coil embolization, a Hemobahn stent-graft was implanted percutaneously during simultaneous cardiopulmonary resuscitation. hemostasis was secured, and the patient recovered. Over the 10-month follow-up, no bleeding or infection has been observed at the site of the repair, and flow through the hepatic artery endograft remains satisfactory. CONCLUSIONS: Percutaneous stent-graft placement can be employed for emergent treatment of visceral artery rupture in patients at high risk for conventional surgical repair.
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ranking = 0.14285714285714
keywords = sarcoma
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18/161. Fine needle aspiration cytology of follicular dendritic cell sarcoma. Report of a case with cytologic detection in an extranodal site.

    BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is a rare malignancy of accessory immune cells that can present in both nodal and extranodal sites. Previous cytologic case reports of FDCS have focused on fine needle aspiration (FNA) findings in nodal sites with low grade morphology and indolent clinical courses. CASE: A 33-year-old female presented with a three-month history of abdominal distention, early satiety and nausea. Initial imaging studies showed a large abdominal mass, with subsequent studies showing lung, liver and lymph node metastases. Examination of primary and metastatic tumors by a combination of conventional histology, immunohistochemistry and FNA demonstrated an extranodal intraabdominal follicular dendritic cell sarcoma. CONCLUSION: FDCS demonstrates a characteristic cytologic picture on FNA, with important cytologic features, including both syncytial and discohesive large epithelioid to spindled malignant cells with intranuclear inclusions, nuclear grooves and a prominent, mature, lymphocytic inflammatory component. No evidence of morphologic tumor progression was noted in comparison of primary and metastatic tumors. To aid in the cytologic distinction of FDCS from other similar-appearing neoplasms, we recommend acquisition of material for immunohistochemical studies, recognition of diverse clinical presentations (including extranodal and aggressive) and acknowledgment of the range of tumor morphologic grades.
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ranking = 0.85714285714286
keywords = sarcoma
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19/161. Epithelioid leiomyosarcoma in a non-immunocompromised infant: additional differential diagnosis of pediatric "round cell tumors".

    We report an 18-month-old Japanese girl with purely epithelioid leiomyosarcoma presenting as a huge intraabdominal mass. The patient had been well from birth and had shown no signs of immunodeficiency. She was negative for human immunodeficiency virus. blood examination revealed elevated serum neuron specific enolase (NSE). Histologically, the tumor was comprised of solid growths of round or polygonal cells with vesicular nuclei and often vacuolated cytoplasm rich in glycogen. The tumor cells were positive for vimentin, NSE, and MIC2, and were negative for desmin and neurofilament. The age, clinical presentation, and histologic findings mostly favored Ewing's sarcoma/primitive neuroectodermal tumor. silver stain, however, demonstrated well-developed reticulin fibers often outlining individual tumor cells. An expanded panel of immunostains showed that the tumor cells were intensely positive for smooth muscle actin, and ultrastructural study revealed abundant fine cytoplasmic filaments with focal subsarcolemmal densities, various amounts of glycogen, and irregularly arranged, thick basal lamina. The diagnosis of epithelioid leiomyosarcoma was made. Following reduction in tumor size by chemotherapy, the serum NSE level was normalized. From the surgical finding, the primary site was presumed to be the urachus or the urinary bladder dome. Although extremely rare, epithelioid leiomyosarcoma should be added in the list of differential diagnoses of pediatric "round cell tumors."
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ranking = 1.1428571428571
keywords = sarcoma
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20/161. Sarcomatoid carcinoma of the pyriform sinus with brain and subcutaneous metastases: an unusual metastatic spread pattern.

    A 63-year-old man presented a polypoid tumor in the laryngeal space involving the right pyriform fossa. The patient underwent a total laryngectomy with bilateral functional neck dissection, and the diagnosis of sarcomatoid carcinoma with malignant fibrous histiocytoma-like stroma was established. The tumor showed an uncommon behavior, with distant metastases to the brain and to the subcutaneous tissue of the abdominal wall. The patient died 1 year after the development of the metastases. Despite its polypoid pattern of growth, sarcomatoid carcinoma of the larynx may behave very aggressively. It is important for clinicians to be aware of the possibility of distant subcutaneous and brain metastases in sarcomatoid tumors of the laryngeal space. If such metastases develop, the prognosis is ominous, with an average life expectancy of 3 months.
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ranking = 0.42857142857143
keywords = sarcoma
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