1/6. Gastrointestinal autonomic nerve tumors: a surgical point of view.AIM: Gastrointestinal autonomic nerve tumors are uncommon stromal tumors of the intestinal tract. Their histological appearance is similar to that of other gastrointestinal stromal tumors. We report two cases and performed an analysis of the literature by comparing our findings with the available case reports in the medical literature. methods: Two patients were admitted with abdominal tumor masses. One occurred in the stomach with large multiple liver metastases and the second originated in Meckel's diverticulum. The latter site has never been reported previously. Both patients underwent surgery. In one patient gastrectomy, right liver resection and colon transversum resection were performed to achieve aggressive tumor debulking. In the other patient the tumor bearing diverticulum was removed. RESULTS: Postoperative recovery of both patients was uneventful. Histological examination, immunohistochemical analysis and electron microscopy revealed the diagnosis of a gastrointestinal autonomic nerve tumor. The patient with the tumor in Meckel's diverticulum died 6 mo after surgery because of pneumonia. The patient with liver metastases have been alive 13 years after initial tumor diagnosis and 7 years after surgery with no evidence of tumor progression. In light of our results, we performed a thorough comparison with available literature reports. CONCLUSION: Radical surgical resection of gastrointestinal autonomic nerve tumors seems to be the only available curative approach to date, and long term survival is possible even in large metastasized tumors.- - - - - - - - - - ranking = 1keywords = survival (Clic here for more details about this article) |
2/6. Chemotherapy with cisplatin for AFP-secreting germ-cell tumors of the central nervous system.Therapeutic results in 17 cases of alpha-fetoprotein (AFP)-secreting tumors of the central nervous system are reported. Twelve of the patients were male and five female. The patients' ages ranged from 5 years to 25 years (mean 13.6 years). The tumors originated in the pineal region in 10 cases, in the suprasellar region in four cases, and in both regions in one case; of the other two tumors, one originated in the basal ganglia and one in the sacrococcygeal region. Extraneural metastases or subarachnoid dissemination occurred in seven cases. Increased AFP titer in the serum or cerebrospinal fluid was verified in 13 cases. The patients were divided into two groups: those treated before (Group A) and those treated after (Group B) the introduction in 1980 of cisplatin as a chemotherapeutic agent. All seven Group A patients died, even after extensive therapy consisting of surgery, radiotherapy, and chemotherapy (ACNU or adriamycin) in some of the cases. The mean survival time following diagnosis for Group A was 23.7 months. Six of the 10 Group B patients died an average of 25.8 months after diagnosis; however, the other four were still alive and well an average of 25.3 months after diagnosis. The tumors responded well to chemotherapy consisting of cisplatin either alone or combined with bleomycin and vinblastine. The initial response rate to treatment in Group B was 100% and five cases had a complete remission, verified by computerized tomography or the serum AFP level. Once the tumor recurred, the response to cisplatin was markedly decreased. It is suggested that an effective therapy must still be sought for recurrent tumors.- - - - - - - - - - ranking = 1keywords = survival (Clic here for more details about this article) |
3/6. Metastatic hemangiopericytoma with prolonged survival.A case of a hemangiopericytoma tumor metastatic to the lung from a central nervous system primary tumor in the posterior fossa is reported. The tumor was not responsive to treatment with cyclophosphamide, Adriamycin (doxorubicin), vincristine, DTIC (CYVADIC), high-dose methotrexate with citrovorum factor rescue, aziridinobenzoquinone (AZQ), or dibromodulcitol. However, the patient has had a prolonged survival, lasting for more than 26 years from original diagnosis, despite extensive recurrent primary disease and massive pulmonary metastases. Clinical and pathologic characteristics of these tumors are reviewed, with emphasis on the potential for long-term survival.- - - - - - - - - - ranking = 6keywords = survival (Clic here for more details about this article) |
4/6. Malignant pheochromocytoma (paraganglioma) of the organ of Zuckerkandl: a study of two cases.Ten percent of pheochromocytomas are found in extra-adrenal locations and are called paragangliomas. In recent years we have had the opportunity to study two patients with malignant paragangliomas of the organ of Zuckerlandl. Only 13 cases of such neoplasms have been reported previously. Our cases are of interest because of their features of recurrence, distant metastases, response to adjuvant therapy, and long survival.- - - - - - - - - - ranking = 1keywords = survival (Clic here for more details about this article) |
5/6. Intravascular malignant lymphomatosis: amelioration of neurological symptoms with plasmapheresis.Intravascular malignant lymphomatosis is a rare disease that is uniformly fatal when disseminated. Chemotherapeutic and radiation therapy regimens have provided short-term amelioration of symptoms but have not affected overall survival. We describe a patient with diffuse neurological symptoms who responded transiently but dramatically to plasmapheresis. This marked response warrants further evaluation of this therapy in intravascular malignant lymphomatosis.- - - - - - - - - - ranking = 1keywords = survival (Clic here for more details about this article) |
6/6. Amplification of N-myc gene and increase of urinary VMA and HVA in patients with neuroblastic tumors.Neuroblastic tumor cases in our departments were evaluated in terms of the stage of the tumor, N-myc amplification, urinary vanillylamndelic acid (VMA)/homovanillic acid (HVA) and survival rate. Two asymptomatic cases, diagnosed when under a year old by mass screening, had no amplifications of N-myc but showed more than one value of urinary VMA/HVA ratio. The patients are now doing well eight years after complete excision of the neuroblastoma which had originated in the sympathetic ganglion. On the other hand, two other symptomatic cases, operated on at the ages of 3 and 5 years, showed remarkable amplifications with less than one value of urinary VMA/HVA, and died from the tumor soon after partial resection of the neuroblastoma and ganglioneuroblastoma which had originated in the adrenal gland and the sympathetic ganglion, respectively. The present monograph reports our cases and discusses prognostic factors.- - - - - - - - - - ranking = 1keywords = survival (Clic here for more details about this article) |