1/11. 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 = nature (Clic here for more details about this article) |
2/11. 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 = 1keywords = nature (Clic here for more details about this article) |
3/11. A fourth ventricle atypical teratoid/rhabdoid tumor in an infant.Atypical teratoid/rhabdoid tumor (AT/RT), a recently established central nervous system tumor entity, occurs in children and is more malignant than medulloblastoma/primitive neuroectodermal tumors (PNET). We report here a case of AT/RT in a male infant who was 9 months old at the time of diagnosis. magnetic resonance imaging revealed that the tumor occupied the fourth ventricle, and at surgery it was found to adhere to the floor of the fourth ventricle. After subtotal removal of the tumor mass, chemotherapy and radiotherapy were performed, but the patient died about 8 months after the diagnosis following rapid regrowth of the residual tumor. light-microscopically, the tumor was composed mainly of nests of rhabdoid cells with fields of PNET. Occasional mesenchymal and epithelial fields were also evident. Immunohistochemically, these rhabdoid cells were positive for vimentin, epithelial membrane antigen, smooth-muscle actin, cytokeratin, and S-100 protein, and less frequently for glial fibrillary acidic protein. Electron-microscopically, the typical rhabdoid cells contained whorled bundles of intermediate filaments in their cytoplasm. Occasionally, such rhabdoid cells were covered partially by basal lamina at their stromal interface. These findings are typical of AT/RT. Although it is well known that AT/RT often arises in the posterior fossa, detailed reports of cases affecting the fourth ventricle are rare. In this case, the ultrastructural relationship between rhabdoid cells and the basal lamina, which has not so far been described in AT/RT, was of great interest when the nature of the rhabdoid cells was considered.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/11. 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 = 1keywords = nature (Clic here for more details about this article) |
5/11. Intraventricular rhabdoid tumor.Malignant rhabdoid tumor (MRT) most commonly occurs in kidney. In the central nervous system, cerebellum is the most common site of occurrence. CNS rhabdoid tumors typically occur in small children, do not respond favorably to treatment and are usually fatal within 1-year. Here is reported a 4-year-old child who presented with features of raised intracranial pressure. Apart from papillodema, there were no neurological signs. Imaging revealed a left lateral ventricular heterogeneous mass abutting the foramen of monro, with mild irregular contrast enhancement and hydrocephalus. The child underwent right ventriculo-peritoneal shunt followed by craniotomy and gross total tumor resection. He was discharged 10-days after surgery without any neurological deficits. Histopathology revealed features compatible with rhabdoid tumor. Despite radiotherapy and chemotherapy, the child died of progressive disease 10-months after surgery. The highly malignant nature of this tumor makes early diagnosis essential for aggressive management and prognostication.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/11. Papillary thyroid carcinoma with anaplastic transformation showing a rhabdoid phenotype solely in the cervical lymph node metastasis.We describe a rare case of anaplastically transformed papillary thyroid carcinoma with a rhabdoid phenotype appearing solely in a metastatic focus. A 77-year-old man presented with a rapidly enlarging, painful right lateral cervical mass. CT scan revealed a tumor in the right upper pole of the thyroid gland and a right lateral cervical mass. Examination of surgically resected specimens disclosed that the thyroid tumor was a well-differentiated papillary carcinoma (2.0 cm in diameter), and the right lateral cervical mass was an anaplastic carcinoma (2.4 cm in diameter) showing a rhabdoid phenotype with scant amounts of a papillary carcinoma component in the periphery, considered to be transformed through the metastasis of the papillary thyroid carcinoma in a cervical lymph node. The rhabdoid cells had eccentric nuclei with conspicuous nucleoli and spherical hyaline cytoplasmic inclusions, which are immunoreactive for vimentin and sarcomeric actin. Ultrastructurally, these had globular aggregation of thin and intermediate filaments. Nuclear immunoreactivity for INI1 indicated that the tumor had no INI1 abnormalities, suggesting a secondary rhabdoid tumor. recurrence developed in the right cervical and mediastinal lymph nodes, and the patient died of disease 6 months after surgery. A rhabdoid phenotype is a pathological hallmark indicating the aggressive nature not only in the neck region, but also in other organs.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/11. Extrarenal malignant rhabdoid tumour of the heel--a case report.Malignant rhabdoid tumour is a rare soft-tissue neoplasm that occurs in children and young adults. Cases have involved numerous extrarenal sites, including the thymus, liver, paravertebral region, central nervous system, heart, prostate, pelvis, chest wall, extremities, as well as soft tissues. Given their highly aggressive nature, early diagnosis of rhabdoid tumours is essential; indeed, survival times after initial presentation are typically in the range of months rather than years. The low incidence of rhabdoid tumours and confusion between this type of tumour and other tumours has precluded the collection of adequate data on its clinical presentation and there is no specific therapeutic protocol. Owing to the rarity of this condition and the difficulties encountered in its diagnosis and treatment, we report the case of a malignant tumour with rhabdoid-like features in the foot that confirms the local aggressiveness and the high metastatic potential of this type of tumour.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/11. Molecular genetic alterations and gene expression profile of a malignant rhabdoid tumor of the kidney.Malignant rhabdoid tumor of the kidney (MRTK) is a rare but highly aggressive tumor in children, and knowledge about the molecular signature of this tumor is limited. We report the molecular genetic alterations and gene expression profile of an MRTK tumor that arose in a 4-month-old Japanese girl. fluorescence in situ hybridization and Southern blot analyses revealed a homozygous deletion of an approximately 0.29-Mb genomic region bordered by the Rgr and ddt genes in these tumor cells. This deleted region encodes SMARCB1, a candidate tumor suppressor gene for MRTK. Using a high-density oligonucleotide DNA array, we found increased expression of 25 genes, including genes involved in the cell cycle (10 genes), dna replication (3 genes), cell growth (5 genes), and cell proliferation (5 genes), in this MRTK tumor sample, compared with a noncancerous kidney (NK) sample. On the other hand, 64 genes, including 4 genes regulating apoptosis, were found to show decreased expression in this MRTK tumor sample, compared with the NK sample. Among these alterations, we found alterations of expression of some genes, such as IGF2, MDK, TP53, and TNFSF10, in this MRTK tumor, as described previously. The molecular genetic alterations and altered pattern of gene expression found in this case may have contributed to the biological characteristics of the MRTK tumor that arose in our patient.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/11. Localized malignant peritoneal mesothelioma containing rhabdoid cells.A case is presented of localized malignant peritoneal mesothelioma appearing as a liver neoplasm. The patient underwent tumor resection but developed a recurrent growth and died 10 months after the initial surgery. The primary tumor showed sarcomatous features with rhabdoid cells. Examination revealed the presence of hyaluronic acid, co-immunoreactivity for cytokeratin epithelial membrane antigen and vimentin, cellular contacts with small desmosomes, and intracytoplasmic lumina. These findings supported the mesothelial nature of this tumor. The recurrent tumor was composed of predominantly tubulopapillary proliferation. It was concluded that the present tumor was a localized malignant mesothelioma of a biphasic type showing a predominantly sarcomatous component in the primary tumor.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/11. Rhabdoid cells in peritoneal fluid. A case report.BACKGROUND: rhabdoid tumor is an aggressive, malignant renal neoplasm of infants. Many extrarenal sites have been documented. CASE: A poorly differentiated carcinoma of the ovary with rhabdoid features occurred in a 36-year-old woman. The peritoneal fluid contained numerous malignant cells with rhabdoid features. Electron microscopy and immunocytochemistry corroborated the rhabdoid nature of the cells. CONCLUSION: Rhabdoid cells can be distinguished from other neoplasms with similar cytologic features by ancillary studies and clinical history.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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