Cases reported "Retroperitoneal Neoplasms"

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1/53. Lymphangiectasia within angiomyolipoma in a tuberous sclerosis patient.

    This is a presentation of a case of tuberous sclerosis with multifocal hamartomas in the retroperitoneum, both kidneys, and the liver. Lymphangiectasia and numerous ectatic lymphatic channels penetrating through the retroperitoneal angiomyolipoma were demonstrated by lymphography and computed tomography. The findings in our study may document the presence of degenerative change in the lymphatic system--not just in elastic vessels--an association which has not yet been reported in the radiological literature.
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2/53. Laparoscopic resection of a retroperitoneal schwannoma.

    Although laparoscopic surgery for retroperitoneal diseases has been widely performed, there are few reports of laparoscopic resection for retroperitoneal tumors. We present the case of a 5-cm retroperitoneal tumor compressing the right common iliac vein and inferior vena cava that was successfully resected using a laparoscopic technique. dissection between the tumor and the large vessels was performed safely using a harmonic scalpel and an ultrasonic surgical aspirator. Histopathology of the resected tumor showed a benign schwannoma. Laparoscopic surgical techniques should be considered for treatment of selected retroperitoneal tumors.
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3/53. Retroperitoneal epithelioid angiomyolipoma leading to fatal outcome.

    Epithelioid angiomyolipoma (AML) is a newly established variant of AML, characterized by monomorphous epithelioid cells that show HMB-45 immunopositivity, and it often displays aggressive behavior. To date, they have mostly appeared in the kidneys; however, the present autopsy case of a 43-year-old female without the stigmata of tuberous sclerosis complex had a huge retroperitoneal mass, accompanied by involvement of the regional lymph nodes. Histopathologically, the tumor was composed of round, polygonal or short spindle-shaped monomorphous cells with abundant eosinophilic cytoplasm and large nuclei with frequent multinucleation. Mitotic figures were scattered. Mature fat cells and thick-walled abnormal blood vessels were totally absent. Immunohistochemically, the tumor cells were reactive with HMB-45 and alpha-smooth muscle actin antibodies. In spite of curative surgery and repeated radio- and chemotherapy, the tumor continued to grow and brought about the patient's death 4 years after the initial symptoms. At autopsy, the peritoneal cavity was filled with the tumor mass exceeding 5.5 kg. Histopathological features were essentially the same as those of biopsy samples, but the cellular pleomorphism and emperipolesis were more easily identified. This report calls attention to this unusual manifestation of AML in the retroperitoneum and the importance of distinguishing it from sarcomas and/or paragangliomas.
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keywords = blood vessel, vessel
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4/53. Leiomyosarcomas of great vessels.

    Sarcomas of the great vessels are rare. Altogether 400 such cases have been described in the aorta, the pulmonary artery, and inferior vena cava. The clinical symptoms are generally related to embolic phenomena, aneurysm formation, and widespread metastases, especially to bones. With improved diagnostic modalities more cases are diagnosed and treated surgically. Resection of the tumor may prolong the patient s life. In this paper authors present two cases of such rare sarcomas. In our first case a tumor has developed in the thoracic aorta with symptoms of imminent aortic dissection. The tumorous nature of the lesion was revealed only histologically, since neither the operation, nor macroscopic picture gave any clue to its tumorous nature. The second case was a male patient with a huge retroperitoneal tumor arising from the inferior vena cava, which was clinically suspected to be a carcinomaarising in the adrenal gland.
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5/53. Retroperitoneal liposarcoma presenting a indirect inguinal hernia.

    A 60-year-old man was admitted to our hospital with a right inguinal swelling that had been growing in size without any pain for 7 months. We diagnosed the growth as a right inguinal hernia and operated on him. The growth, however, was found to be a tumor it situated along the spermatic cord and testicular vessels. We diagnosed it as a lipoma. The tumor was resected near part of the internal inguinal ring. Histopathological diagnosis showed well-differentiated liposarcoma of the sclerosing type. Postoperative computed tomography (CT) revealed a large residual tumor in the retroperitoneum. We believed that the tumor was a retroperitoneal liposarcoma and that it developed in the inguinal region. The residue of the liposarcoma was resected onto the right inguinal tract. A periodic follow up has been performed and no evidence of recurrence or metastasis has been seen in the 4 years and 9 months since the second surgery. No adjuvant therapy was performed. Inguinal liposarcomas are relatively rare and in most cases these tumors are thought to originate in the spermatic cord. The origin of the tumor is believed to be the retroperitoneum.
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6/53. Diffuse, retroperitoneal mesenteric and intrahepatic periportal plexiform neurofibroma in a 5-year-old boy.

    We present a case of plexiform neurofibroma involving the retroperitoneum, mesentery, and liver in a 5-year-old boy who underwent evaluation for extent of a palpable left neck mass. The mass had intrathoracic extension with great vessel encasement and extension into the abdomen. Abdominal CT revealed a diffuse low-attenuation non-enhancing mass encasing the retroperitoneal vessels with serpiginous extension into the liver along the portal vein. This spread pattern of plexiform neurofibroma is an unusual manifestation of neurofibromatosis in a young child.
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7/53. Anterior abdominal wall malignant peripheral nerve sheath tumor in an infant.

    Malignant peripheral nerve sheath tumors (MPNST), also known as malignant schwannomas, are highly malignant lesions that tend to arise in the head and neck region or on the extremities, and only rarely in the retroperitoneum. These tumors are not common in children. Almost 50% of cases are associated with neurofibromatosis. When these neoplasms arise in the retroperitoneum, they usually are difficult to diagnose and to treat. Although various radiologic imaging methods are helpful for identifying some features of the mass, definitive diagnosis requires histologic examination and immunohistochemical staining. After treatment, the tumor recurs in 25% of patients. Five-year survival rates as high as 80% have been reported. Total excision, lack of invasion of surrounding tissues and vessels, and absence of neurofibromatosis, are features associated with better outcome. The authors present the case of a 1-month-old boy who had malignant schwannoma diagnosed in a unique location over the peritoneum of the anterior abdomen. The location and rapid growth of the tumor permitted early identification and total excision. J Pediatr Surg 36:1866-1868.
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8/53. Retroperitoneal cystic metastasis from a small clear cell renal carcinoma.

    A 39-year-old housewife was referred to our hospital for the treatment of a small renal tumor. A 25 x 35 mm cystic mass that had been detected by computerized tomography scan just caudal to the renal hilus proved to be a metastasis from the renal carcinoma of clear cell type. The pathogenesis may have been due to tumor cells obstructing a lymphatic vessel draining the kidney. Cystic metastasis from renal cell carcinoma is very rare and this appears to be the second published case in the world.
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9/53. Surgical treatment of retroperitoneal leiomyosarcoma invading the inferior vena cava: report of three cases.

    Retroperitoneal leiomyosarcoma is a rare neoplasm for which complete surgical removal provides the only effective treatment, as local recurrence adversely affects prognosis. However, invasion of major vessels may occur, making complete resection difficult. This report describes the cases of three patients who required concomitant resection of parts of the inferior vena cava because of direct tumor invasion. The major vessels should be isolated in preference to the tumor capsule during surgery to prevent sudden exsanguination or incomplete tumor resection. Resection of a recurrent sarcoma or a solitary metastasis can be effective in selected patients.
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10/53. Retroperitoneal schwannoma with features of gastrointestinal schwannoma. A case report.

    A case of retroperitoneal schwannoma with histological features of gastrointestinal (GI) schwannoma occurring in a 67-year-old woman is reported. The tumor was composed of spindle cells with focal pseudoatypism and it showed several features typical of gastrointestinal-type schwannoma such as lymphocytic infiltration, peripheral cuff of lymphocytes, lack of Antoni A pattern, and absence of thick walled vessels. Immunohistochemically, the tumor showed diffuse reactivity for S100 protein and glial fibrillary acidic protein. The authors discuss a phenotypical similarity of the lesion with GI schwannoma as well as the possible existence of GI-type schwannoma outside the tubal GI tract.
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