1/80. Severe rhabdomyolysis as a complication of high-dose chemotherapy in a patient with advanced testicular cancer.BACKGROUND: A 38-year-old man was diagnosed with choriocarcinoma in the left testis, a 9 cm retroperitoneal tumor, left hydronephrosis, multiple lung metastasis and gynecomastia. His serum hCG level decreased but was still above the normal range after four courses of standard chemotherapy. methods: High-dose chemotherapy was then given with ice (ifosfamide, carboplatin, etoposide) from days -7 to-3. RESULTS/DISCUSSION: On day -2 the patient became confused and hallucinated, so the sedatives haloperidol and flunitrazepam were administrated. His creatinine phosphokinase levels increased to 6150 IU/L (reference range <197 IU/L) on day 0. myoglobinuria and myoglobinemia were noted and the renal dysfunction progressed. A peripheral blood stem cell transplantation was performed after hemodialysis on day 0. Although his bone marrow recovered, the patient died of respiratory failure on day 18. An autopsy revealed massive pulmonary bleeding. Pretreatment renal dysfunction, renal damage by high-dose chemotherapy, ifosfamide-induced hallucinations and the administration of sedatives, such as haloperidol and flunitrazepam, may have been causative factors for the rhabdomyolysis observed in this patient.- - - - - - - - - - ranking = 1keywords = peripheral (Clic here for more details about this article) |
2/80. Ossifying fibromyxoid tumor of soft parts in a child: a case report.Ossifying fibromyxoid tumor (OFMT) is a relatively rare tumor, most of which occur in adults with preferential localization in subcutis or muscles of the extremeties. Although Schwannian or cartilage origin has been suggested, histogenesis of this tumor still is unclear. The authors present a 6-year-old girl with retroperitoneal OFMT showing paraspinal extension, who is alive and tumor free 9 years after excision. The current case is the youngest reported patient showing unusual deep trunk site with surgically identified association with the spinal nerve. Both the clinical and histopathologic features strongly suggested Schwannian origin of this tumor.- - - - - - - - - - ranking = 0.77935791015625keywords = nerve (Clic here for more details about this article) |
3/80. leptin and leptin receptor expression in a lipoblastoma in an 8-year-old girl.leptin is a hormone that is produced by adipocytes. leptin acts on specific receptors in the hypothalamus. rna was isolated from a lipoblastoma of an 8-year-old girl and the expression of leptin and leptin receptor mRNA was analyzed by RT-PCR. The lipoblastoma tumor, a rare form of childhood tumors, expressed leptin and leptin receptors in a fashion similar to normal adipose tissue. We hypothesize that the peripheral action of leptin via its receptors could play a role in the development and/or progression of lipoblastoma. Whether or not leptin and leptin receptor expression play a role in the development and/or progression of lipoblastoma and other tumors is not clear to date. Copyrightz1999S.KargerAG, Basel- - - - - - - - - - ranking = 1keywords = peripheral (Clic here for more details about this article) |
4/80. A case of klinefelter syndrome with retroperitoneal teratoma.klinefelter syndrome (KS) is often associated with various neoplasms, especially germ cell tumors. mediastinum is the most favored site of extragonadal germ cell tumors with KS, which is somewhat different from those without KS. The retroperitoneal germ cell tumor in KS is very rare. A five-month-old boy with an abdominal mass was found to have a retroperitoneal tumor. After surgical removal, he was diagnosed to have mature cystic teratoma. Cytogenetic study of his peripheral lymphocytes revealed that his karyotype was consistent with KS. This case suggests that patients with KS might be at risk of having germ cell tumors in sites other than mediastinum. It also suggests that all cases with these tumors should be screened for the presence of karyotypic abnormalities, and it might help to assess the exact correlation between germ cell tumors and KS, and to treat them accordingly.- - - - - - - - - - ranking = 1keywords = peripheral (Clic here for more details about this article) |
5/80. 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.- - - - - - - - - - ranking = 129.3060034235keywords = neurofibromatosis (Clic here for more details about this article) |
6/80. Evidence of nerve sheath differentiation and high grade morphology in sclerosing epithelioid fibrosarcoma.Sclerosing epithelioid fibrosarcoma is a recently described sarcoma in which ultrastructural evidence of fibroblastic differentiation forms part of the diagnostic criteria. This report describes a further case of this tumour, which showed evidence of both fibroblastic and perineurial differentiation by immunohistochemistry and electron microscopy, and which had areas of high grade morphology. The tumour metastasised and the patient died of disease 12 months after presentation. The relevance of these findings to diagnosis and differentiation in these tumours is discussed.- - - - - - - - - - ranking = 3.117431640625keywords = nerve (Clic here for more details about this article) |
7/80. 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.- - - - - - - - - - ranking = 267.50879639779keywords = neurofibromatosis, peripheral, nerve (Clic here for more details about this article) |
8/80. Atypical pleomorphic extraosseous ewing tumor/peripheral primitive neuroectodermal tumor with unusual phenotypic/genotypic profile.A pleomorphic undifferentiated tumor primarily located in the retroperitoneum with a phenotype compatible with an extraosseous Ewing tumor/peripheral primitive neuroectodermal tumor (ET/pPNET) pattern and unusual molecular features is described. Immunohistochemically, HBA-71 (CD99/mic2) and several neural markers were intensively expressed together with scattered cells expressing carcinoembryonic antigen (CEA). Short-term culture showed biphasic neuroblastic and epithelioid cell populations, with the latter expressing germ cell markers (CEA, alpha-fetoprotein, and the beta-subunit of chorionic gonadotrophin). Conventional cytogenetics displayed several chromosomic rearrangements, especially a complex translocation t(17,2,22,13) (q21::q11-->q33::q12-->q13::q14). These structural abnormalities were confirmed using fluorescence in situ hybridization analysis. Molecular studies revealed EWS-FEV fusion transcripts (exon 7 of the EWS gene and exon 2 of the FEV gene). In addition, a new p53 mutation not previously reported in ET/pPNET involving exon 5 codon 138: GCC to GAC (Ala/Asp) was detected.In our case, we emphasize the presence of atypical features not only from the phenotypic point of view but also at the genetic level as well as the value of detecting such markers in the differential diagnosis with other abdominal pleomorphic tumors.- - - - - - - - - - ranking = 5keywords = peripheral (Clic here for more details about this article) |
9/80. Massive retroperitoneal pseudotumour in a patient with type 3 von Willebrand disease.Formation of destructive haemorrhagic pseudocysts or pseudotumours thought to arise from unresolved, encapsulated haematomas is a well-recognized, rare complication of severe haemophilia A or B, and has been reported in a single patient with von Willebrand disease (vWD). We report a 41-year-old patient with type 3 vWD who underwent incomplete resection of a large retroperitoneal pseudocyst in 1995 and presented with a recurrent, extensive right abdominal and flank mass and signs and symptoms of large bowel obstruction. He required emergency partial colectomy for bowel ischaemia and removal of his right kidney, which was hydronephrotic due to prolonged ureteral obstruction by the pseudocyst. Following repeat partial resection of the pseudotumour, he developed persistent bleeding into the operative site despite aggressive administration of von willebrand factor (vWF)-rich factor viii concentrates, resulting in retroperitoneal haematomas and abscesses, which resolved after 13 months of percutaneous drainage, extended supplementation of vWF and antibiotic therapy.- - - - - - - - - - ranking = 5.9509933367878keywords = von (Clic here for more details about this article) |
10/80. Retroperitoneal schwannoma extending into the intravertebral foramen.A 35-year-old Japanese man first visited the hospital for episodes of numbness in the right medial femoral region and loss of muscular strength in the right thigh. A CT scan of the abdomen showed a 10 x 9-cm well-circumscribed in homogeneous multilayered mass, in contact with the lumbar vertebrae. MRI demonstrated a hypodense and hyperdense mixed mass. Tumor extension into the L(2)/L(3) intravertebral foramen was shown. To avoid spinal cord damage, we chose enucleation of the tumor in the intravertebral foramen. A follow-up CT after 1 year showed no evidence of a recurrent mass. In conclusion, we advocate the nerve-sparing operation in cases like that of our patient.- - - - - - - - - - ranking = 0.77935791015625keywords = nerve (Clic here for more details about this article) |
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