Cases reported "Abdominal Neoplasms"

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1/114. Disseminated cystic lymphangiomatosis presenting with acute abdomen: report of a case and review of the literature.

    lymphangioma is an uncommon tumor. Lymphangiomatosis, a benign tumor consisting of a cluster of dilated lymphatic channels, is very unusual. Most lymphangiomatoses are found in the neck and head area. Less than 5% are diagnosed intraabdominally and they are very infrequently encountered in the retroperitoneal area. Herein, we report a rare case of a 32 year-old woman who had disseminated intra-abdominal and retroperitoneal cystic lymphangiomatosis, which presented as acute abdomen. She received exploratory laparotomy due to the suspicion of malignancy, which was finally confirmed as cystic lymphangiomatosis. The clinical manifestations, imaging features, and management of this patient are discussed and compared with previous literature.
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keywords = intra-abdominal
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2/114. A case of intra-abdominal multiple lymphangiomas in an adult in whom the immunological evaluation supported the diagnosis.

    A 60-year-old patient with intra-abdominal lymphangiomatosis is described. He presented with anaemia due to enteric haemorrhage, hypoproteinaemia with heavy hypogammaglobulinaemia and T-cell lymphopenia. Duodenal biopsy showed lymphangiectasia while a small bowel study revealed several filling defects in the terminal ileum. On exploratory laparotomy, numerous inoperable lymphangio-haemangiomata were found, involving the small and large intestine, appendix, mesenterium, gallbladder and main biliary tract. The importance of T-cell lymphopenia and hypogammaglobulinaemia in the diagnosis of intra-abdominal lymphangiomatosis with lymphangiectasia is stressed.
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ranking = 6
keywords = intra-abdominal
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3/114. Pseudo sister mary joseph's nodule.

    The sister mary joseph's nodule is a significant finding in the physical examination. It is sometimes the only indication of an intra-abdominal metastatic malignancy. We report a patient who presented with an umbilical nodule that was discovered to be an omphalith. A review of the literature discusses the sister mary joseph's nodule and this unusual finding.
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ranking = 1
keywords = intra-abdominal
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4/114. Intra-abdominal embryonal rhabdomyosarcoma in an adult.

    rhabdomyosarcoma is an uncommon neoplasm in the adult population. Sporadic cases of primary rhabdomyosarcoma arising in the abdomen have been reported, but these cases are limited almost exclusively to the pediatric population. We report a well-documented case of primary intra-abdominal rhabdomyosarcoma in a 57-year-old woman. The patient presented with a pelvic mass and an elevated serum CA 125 and was referred to gynecologic oncologists at our institution for a presumed primary gynecologic malignancy. Intraoperatively, amorphous gelatinous tumor comprised a large portion of the peritoneal cavity. Surgical exploration of the abdomen failed to implicate any specific organ as the site of origin of the tumor. The overall histologic pattern of the resected tumor was most consistent with embryonal type rhabdomyosarcoma. To our knowledge this is the first well-documented case report of non-hepatobiliary, adult, intra-abdominal embryonal rhabdomyosarcoma in the English language literature. The presentation of a rare adult sarcoma mimicking a gynecologic malignancy was an unusual feature that complicated the diagnosis in this case.
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ranking = 2
keywords = intra-abdominal
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5/114. Squamous cell carcinoma of the cervix simulating an advanced malignancy of the ovaries.

    A 40-year-old woman was diagnosed as having stage II squamous cell cervical carcinoma and managed with radiotherapy. Three months after treatment, she presented with features suggestive of an advanced ovarian tumour including gross abdominal swelling, bilateral ovarian tumours, multiple tumour seedlings in the abdominal cavity and ascites. There was also pleural effusion. Operative findings revealed widespread intra-abdominal metastases whose histology, contrary to expectations, showed squamous cell carcinoma of cervical origin. Distant metastases from squamous cell carcinoma of the cervix are rare. A high index of suspicion is necessary to detect this unusual mode of presentation.
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ranking = 1
keywords = intra-abdominal
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6/114. Cytokeratin-negative desmoplastic small round cell tumor: a report of two cases emphasizing the utility of reverse transcriptase-polymerase chain reaction.

    desmoplastic small round cell tumor (DSRCT) is a unique, highly aggressive neoplasm that chiefly affects male adolescents and young adults. This tumor is characterized by nests of small undifferentiated cells that show immunohistochemical evidence of epithelial, mesenchymal, and neural differentiation. We report two cases of DSRCT that lacked immunohistochemical evidence of epithelial differentiation, but were found to have the fusion transcripts characteristic of this tumor. Both patients (a 41-year-old male and a 31-year-old female) presented with large intra-abdominal masses. After diagnostic biopsy, both were treated with multi-agent chemotherapy. One patient expired 18 days after diagnosis, and the other is currently alive 28 months later. Histologically, both tumors had the characteristic features of DSRCT and were composed of small round cells with hyperchromatic nuclei and scanty cytoplasm. In one of the cases, perinuclear intracytoplasmic hyaline inclusions were seen. Immunohistochemically, neither case expressed any of the epithelial markers tested, including AE1/AE3, CAM 5.2 and EMA. Both tumors were diffusely immunoreactive for desmin with a prominent globoid "dot-like" pattern of staining in one case. Both tumors stained for vimentin, neuron specific enolase, and synaptophysin, but were negative for CD99, muscle-specific actin, and myogenin. Reverse transcriptase-polymerase chain reaction revealed EWS-WT1 fusion transcripts characteristic of this neoplasm. In conclusion, we describe two cases of DSRCT that lacked immunohistochemical evidence of epithelial differentiation but had histologic and other immunohistochemical features which suggested this diagnosis. The ability to confirm the diagnosis of this rare tumor using molecular genetic techniques is particularly useful in those cases with unusual histologic or immunophenotypic features.
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ranking = 1
keywords = intra-abdominal
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7/114. Intra-abdominal pulmonary sequestration: a benign suprarenal mass.

    A 34-year-old white woman presented with an 8.5-cm left suprarenal mass. Evaluation revealed normal adrenal hormone function. Pathologic examination after surgical removal revealed a rare benign condition, intra-abdominal extralobar pulmonary sequestration (accessory lung).
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ranking = 1
keywords = intra-abdominal
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8/114. Intra-abdominal desmoplastic small round-cell tumor: a rare cause of peritoneal malignancy in young people.

    We report the CT and pathological findings of an intra-abdominal desmoplastic small round-cell tumor in a young man. Computed tomography showed an extensive peritoneal and mesenteric disease with gross bulky masses, direct liver invasion, and lymph node involvement. This entity should be considered in the differential diagnosis of a young male patient presenting with features of widespread peritoneal malignant disease.
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ranking = 1
keywords = intra-abdominal
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9/114. 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 = 5
keywords = intra-abdominal
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10/114. Intra-abdominal desmoplastic small round cell tumor.

    BACKGROUND: Intra-abdominal desmoplastic small round cell tumor is a rare malignancy with a predilection for young males. Unique histological and immunocytochemical features distinguish the tumor from other members of the family of small round cell tumors of infancy and childhood. The aggressive nature of tumor spread, relative insensitivity to chemotherapy, and generally incomplete resectability result in a very poor prognosis. The authors report a case of a 39-year-old man with diffuse abdominal and pelvic involvement of intra-abdominal desmoplastic small round cell tumor treated with aggressive chemotherapy and surgery. methods: Computed-tomography (CT)-guided biopsy of an omental mass was performed. Histologically, discrete nests of uniform closely packed malignant cells were distributed in a background of focally desmoplastic stroma. Immunocchemistry demonstrated positivity for epithelial, mesenchymal, and neural markers. On the basis of these unique histological and immunohistochemical characteristics, the diagnosis of desmoplastic small round cell tumor was made. The patient was treated with aggressive neoadjuvant chemotherapy consisting of a high-dose alkylator -based combination regimen, followed by surgery. RESULTS: The patient had a 10 to 15 percent regression in tumor mass in response to chemotherapy. laparotomy revealed two large omental masses, another large mass adherent to the left colon and pelvic sidewall, and diaphragmatic, peritoneal and mesenteric studding with small nodules. Complete surgical resection was not possible. CONCLUSIONS: Intra-abdominal desmoplastic small round cell tumor remains an aggressive malignancy with an extremely poor prognosis. Although some response to chemotherapy may be possible, complete resection is rare, and surgical efforts are general palliative.
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ranking = 1
keywords = intra-abdominal
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