1/22. 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 = 1keywords = rhabdoid (Clic here for more details about this article) |
2/22. 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 = 0.66666666666667keywords = rhabdoid (Clic here for more details about this article) |
3/22. 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 = 0.55555555555556keywords = rhabdoid (Clic here for more details about this article) |
4/22. 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 = 0.77777777777778keywords = rhabdoid (Clic here for more details about this article) |
5/22. Squamous cell carcinoma of skin with a rhabdoid phenotype: a case report.A 67 year old man presented with a polypoid lesion on the temple that had all the light microscopic, immunohistochemical, and ultrastructural features of a rhabdoid tumour. There was an area of intraepidermal carcinoma and invasive squamous carcinoma at the base of the polyp. The tumour progressed aggressively and the patient died five months after primary excision. Cutaneous tumours with a rhabdoid morphology have been described previously and tend to have a very poor prognosis. No previously published report describes a clear squamous histogenesis.- - - - - - - - - - ranking = 1.0799677584573keywords = rhabdoid tumour, rhabdoid (Clic here for more details about this article) |
6/22. Malignant rhabdoid tumour of the kidney occurring simultaneously with a brain tumour: a report of two cases and review of the literature.Malignant rhabdoid tumour of the kidney (MRTK), an uncommon aggressive neoplasm of children, is now recognised as a separate entity from Wilms' tumour with distinct clinical and pathological features. MRTK is unique in its significant association with primary brain tumours or brain metastases. We report two cases, aged 2 and 6 months, of MRTK occurring concurrently with a brain tumour. Radical nephrectomy and ventriculo-peritoneal shunting were performed. Both patients expired 2 and 6 months later despite receiving aggressive post-operative chemotherapy and radiotherapy. CONCLUSION: malignant rhabdoid tumour of the kidney is an uncommon neoplasm of early childhood with a poor prognosis. Due to its significant association with brain tumours or early brain metastases, concurrent brain computer tomographic examination is essential for all patients with this disease.- - - - - - - - - - ranking = 3.1464732174106keywords = rhabdoid tumour, rhabdoid (Clic here for more details about this article) |
7/22. Imaging features of multifocal primary rhabdoid tumour of the central nervous system with meningeal and spinal metastases.We present a 1-year-old boy with histologically proven multifocal rhabdoid tumour of the brain with meningeal and spinal metastasis. Although very rare and without specific radiological features, the possibility of rhabdoid tumour should be kept in mind when imaging paediatric intracranial and spinal neoplasms.- - - - - - - - - - ranking = 3.1464732174106keywords = rhabdoid tumour, rhabdoid (Clic here for more details about this article) |
8/22. Carcinoma of lung with rhabdoid features.lung tumors with rhabdoid features, included as variants of large cell carcinoma in the 1999 world health organization classification of lung tumors, are rare and have an aggressive clinical course. We report 11 patients with primary lung tumors with rhabdoid features and review the literature on this uncommon tumor. We examined samples from 7 primary (6 resections, 1 biopsy) and 4 metastatic tumor samples. All specimens were stained with immunohistochemical stains for pancytokeratin (CK), cytokeratin 7 (CK7), cytokeratin (CK20), thyroid transcription factor-1 (TTF-1), and vimentin. The patients were 7 men and 4 women whose ages ranged from 35 to 70 years. Nine patients presented with respiratory symptoms, and 9 patients had a history of heavy smoking. One patient had TNM stage I tumor, 3 had stage III tumors, and 6 had stage IV tumors at presentation; tumor stage could not be determined in 1 patient. Histological examination of these tumors showed typical rhabdoid cells: large cells with abundant cytoplasm, a large eccentric nucleus with a central macronucleolus, and a rounded eosinophilic cytoplasmic inclusion that sometimes caused nuclear indentation. These cells constituted 10% to 90% of the tumor. The "parent" neoplasm was sarcomatoid carcinoma and adenocarcinoma in 4 cases each and was large cell undifferentiated carcinoma in 3 cases. Cytoplasmic staining in the rhabdoid cells was seen in 9 of 11 cases for CK, in 4 of 10 cases for CK7, and in all 11 cases for vimentin. Nuclear staining for TTF-1 in the rhabdoid cells was absent in all 11 cases, and cytoplasmic staining for CK20 was negative in the rhabdoid cells in all 10 cases studied. Of the 9 patients with available follow-up information, 8 died of disease, and 1 is alive with no evidence of disease 20 months after the initial diagnosis. We conclude that rhabdoid features can occur in a variety of lung tumors, including sarcomatoid carcinoma. Recognizing these lesions is important because of their possibly aggressive clinical course.- - - - - - - - - - ranking = 1.2222222222222keywords = rhabdoid (Clic here for more details about this article) |
9/22. Atypical teratoid/rhabdoid tumor of the cerebellum: report of two infantile cases.Atypical teratoid/rhabdoid tumor of the CNS is an aggressive infantile neoplasm of uncertain origin. In our two infantile cases, this tumor presented as a bulky cerebellar hemispheric mass with significant mass effect to the fourth ventricle and brain stem. Although the attenuation on CT and signal intensity characteristics at MR imaging of this tumor were similar to those of vermian medulloblastoma, cerebellar hemispheric location and aggressive growth pattern could be considered as different gross morphologic characteristics of this tumor. Despite intensive chemotherapy and radiation therapy, both of our two patients died within 8 months of pathologic diagnosis.- - - - - - - - - - ranking = 0.55555555555556keywords = rhabdoid (Clic here for more details about this article) |
10/22. Multicentric atypical teratoid/rhabdoid tumors occurring in the eye and fourth ventricle of an infant: case report.Atypical teratoid/rhabdoid tumors (AT/RTs) are aggressive malignant tumors found in infants and young children. The tumor is characterized by the presence of a rhabdoid cell component in all cases, but the histological origin is still unclear. Recently, germline mutation of the hSNF5/INI1 gene has been reported in association with AT/RTs. The authors report a rare case of an intraocular AT/RT followed by a fourth ventricular tumor. The results of immunohistochemical studies of the surgical specimens revealed the presence of an AT/RT and from this finding the neural origin was inferred. A novel missense mutation of the hSNF5/INI1 gene was demonstrated by dna analysis. High-dose chemotherapy with stem cell rescue was effective in treating this patient. The immunohistochemical relationship between rhabdoid cells and the neurogenic zone, which has not been described in AT/RTs, is of great interest in view of the nature of rhabdoid cells.- - - - - - - - - - ranking = 0.88888888888889keywords = rhabdoid (Clic here for more details about this article) |
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