1/63. Case of retroperitoneal dedifferentiated mixed-type liposarcoma: comparison of proliferative activity in specimens from four operations.In a case of retroperitoneal dedifferentiated mixed-type liposarcoma, a dedifferentiated component was observed in the so-called mixed-type liposarcoma consisting of well-differentiated and myxoid components. The proliferative activity was compared among the different components of the tumor by immunohistochemical study using the proliferating cell nuclear antigen (PCNA) and MIB-1 monoclonal antibodies. The dedifferentiated component showed higher positivity than the well-differentiated and myxoid components, and tumor progression was most advanced in the dedifferentiated component. In the chronological examination of each component, the labeling indices of PCNA and MIB-1 were significantly higher at the third recurrence than in the primary lesion in all types, indicating that the proliferative activity of the tumor cells increased gradually. Considering the surgical treatment of liposarcoma, an extended resection encompassing normal adjacent tissues is required in cases containing the dedifferentiated component in comparison to the cases containing only well-differentiated or myxoid components.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/63. Fine needle aspiration diagnosis of lymphangiomyomatosis. A case report.BACKGROUND: Lymphangiomyomatosis is a rare disease of females, usually of reproductive age. There is a proliferation of lymphatic smooth muscle in mediastinal, retroperitoneal and often pulmonary lymphatics and lymph nodes. CASE: A 45-year-old female presented with a right pleural effusion and increasing retroperitoneal adenopathy with palpable left inguinal adenopathy. Three months previously she had undergone a right salpingo-oophrectomy for an ovarian fibroma with concomitant left ovarian wedge biopsy, myomectomy for leiomyomas and partial omentectomy. Three years previously, at age 42, she had experienced two transient episodes of chylous pleural effusion with no sequelae. She underwent computed tomography-guided fine needle aspiration of a 4-cm inguinal lymph node to rule out lymphoma. CONCLUSION: Fine needle aspiration of lymphangiomyomatosis yields distinctive cytologic morphology. This characteristic morphology, in combination with the appropriate history, permits a minimally invasive, timely and in this particular case, entirely unexpected diagnosis.- - - - - - - - - - ranking = 0.14285714285714keywords = life (Clic here for more details about this article) |
3/63. 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.- - - - - - - - - - ranking = 0.14285714285714keywords = life (Clic here for more details about this article) |
4/63. Medical management of unresectable, recurrent low-grade retroperitoneal liposarcoma: integration of cytotoxic and non-cytotoxic therapies into multimodality care.Liposarcomas of the retroperitoneum are rare tumors, and best managed by an expert multidisciplinary team consisting of a surgical oncologist with appropriate medical oncology and radiation oncology collaboration. For large tumors, surgical excision with wide margins is difficult to achieve, and even wide margins cannot ensure that microscopic remnants of residual disease will not grow back in the future. For these reasons, even following expert resection, local recurrence is common. For patients with recurrent and unresectable liposarcoma, treating the sarcoma while maintaining quality of life becomes the major therapeutic goal. Importantly, patients with advanced recurrent disease demonstrate the need for multidisciplinary team involvement, with timely consideration of palliative surgical, radiation therapy, and chemotherapy options. Such patients also represent ideal candidates for investigational approaches aimed at identifying new agents with which to treat this disease. In addition to the development of new cytotoxic agents, patients may be candidates for novel strategies such as differentiation therapies or anti-angiogenic approaches. The recent explosion of knowledge regarding the cytogenetics, molecular, and cellular biology of liposarcomas allows us to remain positive that new translational therapies will be developed to improve the clinical outcomes of patients with these diseases. Current strategies, such as the use of PPARgamma ligands to differentiate liposarcomas, will soon be tested in major national collaborative trials, and the cooperation of surgeons and medical oncologists at all levels of community and academic practice will be crucial to obtain answers in this field. This review will summarize an illustrative case in the process of describing the natural history and potential interventions which should be considered for patients with this disease.- - - - - - - - - - ranking = 0.14285714285714keywords = life (Clic here for more details about this article) |
5/63. Cytologic finding of chyloascites in lymphangioleiomyomatosis. A case report.BACKGROUND: lymphangioleiomyomatosis is a rare disease, histologically characterized by an abnormal proliferation of smooth muscle around the lymphatics. lung is the most common site of involvement, and patients usually present with dyspnea, chest pain, and cough. Chylous pleural effusion and ascites occasionally appear during the course of the disease. There are only a few reports on the cytologic findings in this disease. To our knowledge, the cytologic findings of chylous pleural effusion and chyloascites have not been reported before. CASE: A 23-year-old female presented with chylothorax, chyloascites and a retroperitoneal mass. Cytologic examination of chylous pleural effusion and chyloascites revealed numerous cohesive and thick clusters of cells with a high nuclear/cytoplasmic ratio, oval nuclei and slightly increased chromatin content. mitosis and necrosis were not observed. Exploratory laparotomy and transbronchial lung biopsy were performed, and the histologic diagnosis was lymphangioleiomyomatosis involving the retroperitoneal lymph nodes, uterine fundus and lungs. immunohistochemistry showed that the characteristic clusters in chylous fluids were positive for alpha-smooth muscle actin. CONCLUSION: A diagnosis of lymphangioleiomyomatosis is possible from cytologic findings of effusions with the aid of clinical findings.- - - - - - - - - - ranking = 0.14285714285714keywords = life (Clic here for more details about this article) |
6/63. Idiopathic myelofibrosis with nodal, serosal and parenchymatous infiltration. Case report and review of the literature.Idiopathic myelofibrosis (IMF) is a breakpoint cluster region rearrangement-negative chronic myeloproliferative disease with progressive bone marrow fibrosis. We report a female patient (65 years old) who was admitted to our hospital in 1996. Trephine bone marrow biopsy revealed diffuse fibrosis with atypical multilobulated megakaryocytes. A cellular phase of IMF was diagnosed. Three years later, despite being at intermediate risk, the patient developed generalized lymphadenopathy and multiple sclerosing tumors throughout the peritoneum and retroperitoneum. biopsy specimens from these tumors revealed sclerosing hematopoietic infiltrates. The present case demonstrates that IMF not only can progress to acute leukemia but can also spread uncontrollably, or 'metastasize' with extensive sclerosing hematopoietic tumors throughout the body.- - - - - - - - - - ranking = 0.14285714285714keywords = life (Clic here for more details about this article) |
7/63. Retroperitoneal schwannoma misdiagnosed as a psoas abscess: report of a case.A retroperitoneal cystic mass compressing the right psoas muscle was found incidentally by ultrasonography in a 67-year-old woman. The radiological findings and a history of costal caries led us to suspect a psoas cold abscess. Ultrasound-guided needle aspiration was done to establish the diagnosis and to drain the content, but only a small amount of sterile fluid was obtained. The patient complained of neuralgia in her right leg at the time of puncture. Under the preoperative diagnosis of a neurogenic tumor, the mass was surgically resected, and found to be filled with old blood. The solid region consisted of a proliferation of fusiform cells, leading to a diagnosis of benign schwannoma. Retroperitoneal schwannoma is often misdiagnosed as an adjacent anatomical structure. Thus, we conclude that both microbiological and cytological examination of an aspiration specimen is important when psoas abscess is considered in a differential diagnosis.- - - - - - - - - - ranking = 0.14285714285714keywords = life (Clic here for more details about this article) |
8/63. Solitary fibrous tumor in the retroperitoneal space: report of a case.solitary fibrous tumors (SFTs) are spindle-cell neoplasms originally described in the pleura. It is now known that these tumors can develop in many sites. This report describes the case of a well-circumscribed tumor located around the superior mesenteric artery (SMA), which was initially thought to be either a superior SMA aneurysm, a lymphoma, or a neurogenic tumor. Histological examination demonstrated the tumor to be composed of a cellular proliferation of ovoid to spindle cells with a fine collagenous matrix in the short fascicles. Immunohistochemical staining was strongly positive for CD34 and negative for factor viii, cytokeratin, desmin, and muscle-specific actin (HHF-35). These findings suggested a diagnosis of SFT in the retroperitoneal space. To our knowledge, this is the first report of an SFT located around the SMA. Based on the above findings, it is important to include SFT in the differential diagnosis of retroperitoneal tumors located around the SMA.- - - - - - - - - - ranking = 0.14285714285714keywords = life (Clic here for more details about this article) |
9/63. Retroperitoneal kaposiform hemangioendothelioma with tufted angioma-like features in an infant with kasabach-merritt syndrome.kasabach-merritt syndrome denotes profound thrombocytopenia and coagulopathy in an infant with a vascular tumor. A retroperitoneal vascular tumor with an unusual combination of histopathological features is reported, and compared with vascular lesions described in the reported cases of kasabach-merritt syndrome in the literature. A large retroperitoneal tumor that had expanded through the sigmoid mesocolon into the sigmoid colon wall was resected from an 8-month-old infant with fully developed kasabach-merritt syndrome. Histological examination revealed a combination of venous (cavernous) malformation, kaposiform hemangioendothelioma and tufted angioma-like areas. Cellular tumor components (especially tufted angioma-like parts) infiltrated the wall of the sigmoid colon to the submucosal level. Immunohistochemical staining with antibodies to the ki-67 antigen and proliferating cell nuclear antigen showed a low proliferative activity, whereas the antiapoptotic bcl-2 protein was expressed diffusely in tumor cells. This is the first reported case of a vascular tumor with tufted angioma-like elements found in the retroperitoneum, and the first reported in combination with kaposiform hemangioendothelioma and venous malformation in the same lesion. Considering the immunohistochemical results and overlapping histological features, it may be considered that tufted angioma and kaposiform hemangioendothelioma represent different growth patterns or stages in the development of a single type of hemangioma.- - - - - - - - - - ranking = 0.28571428571429keywords = life (Clic here for more details about this article) |
10/63. Treatment of lymphangioleiomyomatosis by ovariectomy, interferon alpha 2b and tamoxifen--a case report.The gender-specific prevalence of lymphangioleiomyomatosis (LAM) in premenopausal women suggests a hormonal etiology. Despite the antiestrogenic treatment (ovariectomy, tamoxifen) this disease is often refractory to therapy and almost inevitably leads to the patient's death. We describe a case where the antiproliferative effect of systemically applied interferon alpha 2b was successfully employed in addition to ovariectomy and the patient reached complete remission.- - - - - - - - - - ranking = 0.14285714285714keywords = life (Clic here for more details about this article) |
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