1/85. Malignant mixed Mullerian tumor with rhabdoid features: a report of two cases and a review of the literature.Rhabdoid tumors were originally described as a type of pediatric renal neoplasm that contains cells resembling rhabdomyoblasts but lacking muscle differentiation. Extrarenal rhabdoid tumors have since been reported in multiple anatomic sites in the pediatric and adult population. These tumors are characterized by an aggressive clinical course, resistance to treatment, and a rapidly fatal outcome. Eight cases of uterine neoplasms with rhabdoid differentiation have been previously reported. In the three cases where clinical follow-up was available, the patients died of disease within 3 to 17 months after the diagnosis was established. We report two cases of uterine malignant mixed Mullerian tumor (carcinosarcoma) with rhabdoid differentiation. The findings and clinical outcome confirm the aggressive nature of uterine tumors with rhabdoid differentiation. One of the patients died of disease 3 months after initial operative treatment while the other patient's tumor recurred in 1 month and she died within 10 weeks. The poor prognosis of these neoplasms makes their histopathologic recognition important.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/85. Atypical teratoid/rhabdoid tumor of the CNS: cytopathology and immunohistochemistry of insulin-like growth factor-II, insulin-like growth factor receptor type 1, cathepsin d, and Ki-67.insulin-like growth factor (IGF)-II is a potent growth factor, normally controlled by a number of other factors, including IGF binding proteins and IGF binding protein proteases. In general, the latter increase the bioavailability of IGF by cleaving IGF binding proteins. cathepsin d (an IGF binding protein protease) was also implicated in tumor invasion. Although IGF-II was implicated in the pathogeneses of various childhood neoplasms, its significance in the pathogenesis of atypical teratoid/rhabdoid tumor of the central nervous system (ATRT-CNS) was not studied to date. We present clinicopathologic features of two cases of ATRT-CNS. In addition, formalin-fixed, paraffin-embedded tissue sections were stained immunohistochemically for IGF-II, IGF receptor type 1, cathepsin d, and Ki-67. Both tumors demonstrated diffuse strong cytoplasmic positivity for IGF-II, diffuse cytoplasmic and focal membranous positivity for IGF receptor type I, and diffuse cytoplasmic positivity for cathepsin d. The Ki-67 labeling indices were 10.0% and 1.4%. We conclude that ATRT-CNS cells express both IGF-II and IGF receptor type 1, supporting the hypothesis that autocrine/paracrine stimulation of cell growth by IGF-II might be one mechanism involved in ATRT-CNS tumorigenesis. cathepsin d expressed by the tumor cells might also be involved in both tumor cell invasion and growth. The exact pathogenesis of ATRT-CNS remains to be elucidated.- - - - - - - - - - ranking = 0.33333333333333keywords = neoplasm (Clic here for more details about this article) |
3/85. Analysis of chromosome 22q as an aid to the diagnosis of rhabdoid tumor: a case report.Malignant rhabdoid tumor is a highly aggressive tumor of childhood that may present as a soft-tissue primary tumor. We report a soft-tissue neoplasm that was polyphenotypic by immunohistochemical expression of epithelial, mesenchymal, and neural markers and did not meet the criteria for any of the usual pediatric small round-cell tumors. The findings raised the diagnosis of rhabdoid tumor, leading to testing for WT1 mRNA and protein expression, which were positive, as has been reported for renal rhabdoid tumor. This tumor had the typical clinical behavior of rhabdoid tumor with therapy resistance and early tumor-related death. Multicolor spectral karyotyping of this neoplasm showed a balanced translocation between chromosomes 1 and 22 with breakpoints at 1p36 and 22q11-12. The latter region is commonly involved in rhabdoid tumor. This change was also identified by fluorescence in situ hybridization. This case suggests that studies of chromosome 22 may be required to distinguish rhabdoid tumor from other soft-tissue tumors.- - - - - - - - - - ranking = 0.66666666666667keywords = neoplasm (Clic here for more details about this article) |
4/85. Simultaneous presentation of atypical teratoid/rhabdoid tumor in siblings.Atypical rhabdoid/teratoid tumor (ATT/RHT) is a rare malignant neoplasm which appears in early childhood. The present paper describes clinical and pathological features of ATT/RHT which occurred simultaneously in 2 sisters diagnosed at a 15 day interval. Both children were treated by surgical resection, subtotal in the first case and total in the second. Postoperatively, chemotherapy followed by radiotherapy, 50.4 Gy on the posterior fossa, were administered. Despite this therapy, both sisters died at 14 months and 26 months respectively. The tumors express vimentin and EMA; cells contained intracytoplasmic inclusions. No karyotypic anomaly was detected. This is the first description of familial ATT/RHT.- - - - - - - - - - ranking = 0.33333333333333keywords = neoplasm (Clic here for more details about this article) |
5/85. Collision of uterine rhabdoid tumor and endometrioid adenocarcinoma: a case report and review of the literature.Extrarenal malignant rhabdoid tumors have been reported in a variety of anatomic sites but infrequently in the female genital tract. In the uterus, they have been described as a pure tumor, in association with endometrial stromal sarcomas, and as a component of a malignant mullerian mixed tumor. This study reports an unusual uterine neoplasm in a 49-year-old woman, in which a malignant rhabdoid tumor occurred as a collision tumor with a well-differentiated endometrioid adenocarcinoma. The tumor was a 14-cm polypoid mass that filled the endometrial cavity. The two neoplastic components were distinct on microscopic and immunohistochemical examination. Ultrastructural examination confirmed the rhabdoid phenotype of the sarcomatous component. The patient died of disease 4 months after diagnosis with progression of the malignant rhabdoid tumor. The highly aggressive behavior of the rhabdoid (i.e., nonepithelial) component in this collision tumor lends support for a distinction of this neoplasm from a malignant mullerian mixed tumor, with which it may be confused.- - - - - - - - - - ranking = 0.66666666666667keywords = neoplasm (Clic here for more details about this article) |
6/85. Malignant rhabdoid tumor beside benign skin mesenchymal neoplasm with myofibromatous features.Only a few reports of primary cutaneous rhabdoid tumors have been published. We describe the case of a 3-month-old female patient who developed a rhabdoid type cutaneous sarcomatoid neoplasm in her upper back, close to a benign myofibromatous proliferation of infancy. The lesion was studied both by light microscopy and immunohistochemically. flow cytometry was performed showing a dna diploid profile of the malignant tumor. The pathological findings suggest a mesenchymal origin (hemangiopericytic or myofibroblastic type) for both tumors. The patient was surgically treated, but she died nine months later.- - - - - - - - - - ranking = 1.6666666666667keywords = neoplasm (Clic here for more details about this article) |
7/85. Primary malignant rhabdoid tumours of brain, clinicoradiological findings of two cases.Malignant rhabdoid tumours (MRT) are extremely malignant, highly aggressive and uncommon renal neoplasms of childhood with very poor prognosis. About fifteen cases of primary intracranial MRT (with their clinical details) are reported in English literature, following the recognition of this entity in 1978. Two cases of MRT are reported here. The first case, one year male baby was admitted with a very large, infiltrative, posterior fossa mass. He required elective ventilation, following the tumour decompression but ultimately died of respiratory failure during the process of weaning from the ventilator. The second child was operated for an extremely vascular, very friable, solid and lobulated tumour of temporal lobe. Radical microsurgical decompression of mass was achieved, however the child developed massive recurrence, documented five weeks after the surgery while on radiotherapy. His recurrence showed partial response to radiotherapy and chemotherapy. The child is alive at 8 month's follow up, but probably passing the terminal days of his life. Hence the recognition of this entity is very essential for the aggressive management and prognostication of the patient, which obviously seems to be different from primitive neuroectodermal tumour.- - - - - - - - - - ranking = 0.33333333333333keywords = neoplasm (Clic here for more details about this article) |
8/85. A malignant rhabdoid tumor of the kidney occurring concurrently with a brain tumor: report of a case.Malignant rhabdoid tumor of the kidney (MRTK) is one of the most lethal neoplasms to occur in young infants. Cases of MRTK accompanying an embryonal tumor in the central nervous system have occasionally been described. We present herein an interesting case of MRTK that was clinically diagnosed preoperatively. A male infant aged 6 months with both a midline brain tumor and a renal neoplasm was transferred to our institution. Although roentgenographic evaluation suggested that the renal lesion was a Wilms' tumor, midkine (MK), a growth and differentiation factor characteristically present in the urine of patients with Wilms' tumor, was not detected. A preoperative diagnosis of MRTK was established based on the lack of urinary MK in addition to the typical clinical features of the young age and the concurrent brain tumor.- - - - - - - - - - ranking = 0.66666666666667keywords = neoplasm (Clic here for more details about this article) |
9/85. Rhabdoid tumors of the central nervous system.Rhabdoid tumors of the central nervous system are rare malignancies with a still almost uniformly fatal outcome. There is still no proven curative therapy available. We report our experience with nine patients with central nervous system rhabdoid tumors. Gross complete surgical removal of the tumor was achieved in six patients. Seven patients received intensive chemotherapy. Four of these were treated in addition with both neuroaxis radiotherapy and a local boost directed to the tumor region, while two patients received local radiotherapy only. The therapy was reasonably well tolerated in most cases. Despite the aggressive therapy, eight of the nine patients died from progressive tumor disease, and one patient died from hemorrhagic brain stem lesions of unknown etiology. The mean survival time was 10 months after diagnosis. Conventional treatment, although aggressive, cannot change the fatal prognosis of central nervous system rhabdoid tumors. As these neoplasms are so rare, a coordinated register would probably be a good idea, offering a means of learning more about the tumor's biology and possible strategies of treatment.- - - - - - - - - - ranking = 0.33333333333333keywords = neoplasm (Clic here for more details about this article) |
10/85. Combined large cell neuroendocrine, small cell and squamous carcinomas of the lung with rhabdoid cells.Two unusual cases of combined lung carcinoma are presented. Both patients, aged 50 and 53 years, had strong histories of cigarette smoking and presented with lung masses. Microscopic examination revealed an uncommon combination of primary lung cancers. Both cases had a dominant histological picture of large cell neuroendocrine carcinoma. The first case was combined with both squamous and small cell carcinomas in almost equal proportions, while the second consisted of large cell neuroendocrine and squamous carcinomas with a focal area of small cell carcinoma. In addition, both cases contained rhabdoid cells. One of the cases pursued an aggressive clinical course with death in 6 months. The other patient presented with recurrent tumor 12 months after the operation and died shortly thereafter. These cases illustrate two examples of uncommon combined lung cancers: large cell neuroendocrine carcinoma combined with squamous carcinoma and small cell carcinoma. An additional feature was the presence of rhabdoid cells in both cases. It is felt that the rhabdoid component is a reflection of de-differentiation or poor differentiation, and may contribute to the aggressive nature of both tumors.- - - - - - - - - - ranking = 0.028437571678668keywords = cancer (Clic here for more details about this article) |
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