Cases reported "Splenic Neoplasms"

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1/73. Ruptured primary splenic angiosarcoma into the colon. Presentation as anal bleeding.

    A 71-year-old woman presented with a six month history of constipation and abdominal discomfort, with anal bleeding during the last days. ultrasonography and CT-scan of the abdomen showed a large heterogeneous mass that was located in the splenic region, but the nature and origin of the tumour could not be clearly established preoperatively. The clinical diagnosis was of abdominal tumour with colonic and splenic involvement, and a left hemicolectomy and splenectomy were performed. Pathologic examination revealed a primary angiosarcoma of the spleen with penetration and fistulization of the tumour into the large bowel. The patient received adjuvant radiation therapy, but she died of extensive metastastic disease from her primary angiosarcoma of the spleen nine months after surgery. In summary, splenic angiosarcoma is very difficult to diagnose preoperatively. This highly aggressive neoplasm has an overall poor prognosis, specially if it is associated with rupture and haemoperitoneum. As this case highlights, unusual forms of rupture may lead to atypical clinical presentations, increasing even more the difficulty in the diagnosis.
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2/73. Pleomorphic large cell sarcoma of the spleen with rhabdomyosarcomatous differentiation.

    An unusual case is reported of pleomorphic large cell sarcoma of the spleen with rhabdomyosarcomatous differentiation in a 34-year old male. According to our knowledge, such a neoplasm has never been reported in the literature.
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3/73. Benign vascular neoplasms of the spleen with myoid and angioendotheliomatous features.

    AIMS: To present the clinical light microscopic and immunophenotypic features of a distinctive vascular neoplasm of the spleen. methods AND RESULTS: Two of the splenic lesions arose in children, and one was found in an adult. They ranged from 19 to 40 mm diameter and histologically were quite similar. Sheets of large epithelioid cells with a spectrum of nuclear configurations ranging from oval and vesicular to twisted and hyperchromatic were noted in each case. Distinct or prominent nucleoli were present in many cells, and occasional cells had nuclear pseudoinclusions. In two cases, bands of basophilic, fibroblast-rich stroma with scattered chronic inflammatory cells were present. The mitotic rate ranged from 0/10 high-power fields (HPF) to 0.5/10 HPF in these epithelioid cells. The vascular nature of these tumours was manifested as a sieve-like array of round, erythrocyte-filled spaces, most with attenuated and cytologically bland lining cells. The polygonal, epithelioid cells exhibited the following phenotype: smooth muscle actin (SMA) , muscle specific actin (MSA) , vimentin , CD31-, CD34-, CD21-, CD8-, CD68- (2/3 cases), S100-, while the lining cells were CD34 , vimentin and SMA-, with variable CD31 and factor viii related antigen expression. Elongated SMA , MSA cell processes were evident in one case, reminiscent of previously characterized myoid elements of the normal spleen. An uneventful follow-up was noted for all three patients. CONCLUSIONS: The histology and immunophenotype set these neoplasms apart from classic hamartomas, haemangiomas and previously characterized (haem)angioendotheliomas of the spleen, and may represent proliferations of myoid elements native to the spleen.
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4/73. Primary splenic angiosarcoma: case report and literature review.

    Angiosarcoma of the spleen is a rare neoplasm. We describe the clinical findings of an index case at our institution and the results of a literature review of the topic. Generally, this cancer has a poor prognosis and prompt splenectomy offers the only cure, as the cancer is poorly responsive to adjuvant chemotherapy.
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5/73. Splenic smooth-muscle tumors in children with acquired immunodeficiency syndrome: report of two cases of this unusual location with evidence of an association with Epstein-Barr virus.

    Smooth-muscle neoplasms are rarely located in the spleen. They have been previously reported in five cases of children with human immunodeficiency virus (hiv) infection/acquired immunodeficiency syndrome (AIDS). Two cases of children with hiv infection/AIDS with autopsy and surgical pathology evidence of multiple smooth-muscle neoplasms with splenic involvement are presented. dna was extracted from histology slides in both cases for analysis for Epstein Barr (EB) virus. In both cases, the presence of EB virus was confirmed. This paper documents two additional cases of the unusual phenomenon of splenic involvement by smooth-muscle neoplasms in the setting of AIDS in childhood and further supports the role of EB virus in the development of these neoplasms.
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6/73. Intravascular occlusive therapy: use of interventional radiology in cancer patients.

    Selective transcatheter intravascular occlusion in the treatment of cancer patients is a valuable extension of interventional diagnostic radiology. Intra-arterial embolization may be performed with various substances, including autologous clot, autologous subcutaneous tissue, Gelfoam, and stainless steel coil. Clinical applications in cancer patients include control of gastrointestinal and genitourinary hemorrhage, preoperative reduction of tumor vascularity, control of local symptoms, and therapeutic reduction of tumor bulk. The technique has been used for preoperative and palliative treatment of neoplasms of the head and neck, kidney, liver, spleen, and soft tissue and bone. Transcatheter intravascular occlusion should be performed only by radiologists experienced in angiographic techniques. Inadvertent occlusion of a normal vessel and thromboembolism are possible complications.
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7/73. Primary pancreatic lymphoma: a case report, literature review, and proposal for nomenclature.

    Primary neoplasms of the pancreas are most often adenocarcinoma. Non-Hodgkin's lymphoma (NHL) involving the pancreas is less common but well documented; the pancreas as the primary site of NHL is rare. The majority of patients with pancreatic cancer, whether it is adenocarcinoma or lymphoma, present with a mass in the head of the gland. Pancreatic lymphoma is often described as a large homogeneous mass with extra-pancreatic extension, with or without associated lymphadenopathy. Less common presentations are masses in the body or tail, or more rarely diffuse involvement of the pancreas. We present a case of diffuse pancreatic lymphoma with extra-pancreatic dissemination to the spleen and lymph nodes, and review the literature on pancreatic lymphoma. Because the definition of pancreatic lymphoma and primary pancreatic lymphoma varies, we also propose a nomenclature system to make future studies of pancreatic lymphoma more comparable.
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8/73. Inflammatory pseudotumor of the spleen: report of a case.

    A case of a 45-year-old Japanese man with a splenic inflammatory pseudotumor is described. This benign lesion is rarely reported in the world literature. We preoperatively could not rule out the possibility of a malignant neoplasm, due to the fact that the tumor had grown in size after a 2-year observation. However, after performing a splenectomy, a histological examination of the mass revealed an inflammatory process. Inflammatory pseudotumors often pose diagnostic difficulties because the clinical and radiological findings tend to suggest a malignancy. The clinical and pathological features of such previously reported cases are also reviewed.
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9/73. Management of recurrent juvenile granulosa cell tumor of the ovary.

    BACKGROUND: Juvenile granulosa cell tumors of the ovary are a rare form of neoplasm that makes up less than 5% of ovarian tumors in childhood and adolescence. About 90% are diagnosed in stage I with a favorable prognosis. More advanced stages (FIGO stages II--IV) have a poor prognosis. CASE: A patient was initially diagnosed at age 17 with FIGO stage IIIC disease and treated with a right salpingo-oophorectomy, debulking, and staging followed by six cycles of carboplatin and etoposide chemotherapy. Tumor recurrence in the liver and adjacent to the spleen occurred 13 months after completion of primary therapy. Aggressive surgical removal of tumor followed by six cycles of bleomycin and taxol as salvage chemotherapy resulted in 44 months of disease-free survival. On November 27, 2000, she had a cesarean delivery of a 2335-g normal male due to a breech presentation. Exploration revealed no evidence of tumor. CONCLUSION: This is the second case report of a patient with advanced juvenile granulosa cell tumor to become pregnant after apparently successful chemotherapy. These results are encouraging, but the best treatment for extensive and recurrent disease has yet to be determined.
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10/73. Epithelioid angiosarcoma of the splenic capsule. Report of a case reiterating the concept of inert foreign body tumorigenesis.

    We report an epithelioid angiosarcoma involving the splenic capsule. This neoplasm developed because of a gauze sponge, retained for 38 years following left-sided nephrectomy. Clinical, radiological, and histological features of this angiosarcoma add to the validity of the concept of inert foreign body tumorigenesis in humans.
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