1/26. Primary pulmonary malignant meningioma.Fewer than 20 cases of primary pulmonary meningioma have been reported. Most of these cases have been histologically and clinically benign. We report an unusual case of primary pulmonary malignant meningioma with atypical histologic features and malignant behavior. A computed tomography scan of the head did not show evidence of tumor. The right upper lobe mass was resected and showed features of an atypical meningioma with loss of architectural pattern, mild nuclear pleomorphism, increased mitotic counts (up to 15 mitotic figures per 10 high power fields), and focally prominent nucleoli. Focally, cells with rhabdoid features were identified. The tumor's immunohistochemical and ultrastructural profiles were consistent with a meningioma. The tumor stained negative for estrogen and focally positive for progesterone receptors and had a MIB-1 labeling index (marker of cell proliferation) of 9.2%. Approximately 5 months after the initial resection, the patient experienced a tumor recurrence with multiple lymph node metastases, spread to the middle and lower lobes of the right lung, and metastasis to the diaphragm. Rarely, primary pulmonary meningiomas may present as high-grade malignant lesions.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
2/26. High-grade extraskeletal myxoid chondrosarcoma: a high-grade epithelioid malignancy.AIMS: Extraskeletal myxoid chondrosarcoma is typically a low-to-intermediate grade sarcoma that is associated with a prolonged clinical course. High-grade forms are rare and not well characterized. In this series we report the clinicopathological, immunohistochemical and ultrastructural findings in four cases of high-grade extraskeletal myxoid chondrosarcoma. methods AND RESULTS: The patients were three men and one woman (ages 34-73 years) with tumours located in the thigh (two cases), paraspinal soft tissue and perineum. Three patients had metastases, one at 12 weeks, one at 10 months, and one at presentation of recurrent tumour. In the latter case the original tumour was low grade and became high grade when it recurred 3.5 years later. All three patients died of disease. One patient was lost to follow-up. The most striking histological feature in all four tumours was the presence of numerous large epithelioid cells. These cells were arranged in cords within myxoid matrix and in sheets devoid of matrix. Two tumours had areas of conventional extraskeletal myxoid chondrosarcoma intermixed with the high-grade areas. One tumour showed transition to high-grade spindle cell sarcoma. One tumour had cells with rhabdoid features. Immunohistochemically, two tumours focally expressed S100 protein, and one focally expressed EMA. All were negative with cytokeratin, desmin, smooth muscle actin, HMB45, CD31 and CD34. Ultrastructural features in three cases were compatible with chondrosarcoma; one tumour had aggregates of microtubules within rough endoplasmic reticulum, a characteristic feature of this tumour. CONCLUSIONS: High-grade extraskeletal myxoid chondrosaroma is a rare and aggressive soft tissue sarcoma, and should be included in the differential diagnosis of other epithelioid malignancies.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
3/26. Malignant rhabdoid tumor of the vulva. Case report.The poor prognosis of malignant rhabdoid tumor is emphasized and histopathological criteria for distinction from epithelial sarcoma of the vulva are discussed. Immunohistochemical analyses were performed by using nine different antigens including vimentin, cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, desmin, muscle-specific actin, S-100 protein, AP-15, neuron specific enolase. This is the sixth reported case of a malignant rhabdoid tumor of the vulva. The patient died eight months after the initial diagnosis in spite of a combination of surgery, adjuvant chemotherapy and external radiotherapy.- - - - - - - - - - ranking = 6keywords = rhabdoid (Clic here for more details about this article) |
4/26. 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 = 2keywords = rhabdoid (Clic here for more details about this article) |
5/26. 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 = 7keywords = rhabdoid (Clic here for more details about this article) |
6/26. Malignant rhabdoid tumor of the vulva: case report and review of the literature with emphasis on clinical management and outcome.BACKGROUND: Malignant extrarenal rhabdoid tumors (ERTs) of the vulva are rare but aggressive neoplasms. We describe the eighth reported case and the first to have long-term survival despite initial rapid recurrence. CASE: A 40-year-old woman presented with a painless lump on the mons pubis. It was excised and revealed an ERT. One month later, the tumor had regrown. A wide local excision and superficial right groin node dissection was performed. Local recurrence was noted 11 months after the initial excision and was treated by wide excision and radiotherapy. She is without evidence of disease 61 months after initial presentation. CONCLUSION: A review of previously reported cases of ERT reveals that the median survival is 9 months. Local recurrences are common and the appearance of distant metastases is almost invariably rapidly fatal. Chemotherapy and radiotherapy do not appear to be effective in controlling disease. Initial surgical extirpation may offer the best chance of cure.- - - - - - - - - - ranking = 5keywords = rhabdoid (Clic here for more details about this article) |
7/26. meningioma with rhabdoid transformation.A 29-year-old man was operated on for an intracranial tumour with the gross features of a meningioma. histology showed features of a rhabdoid tumour. The tumour recurred in 1997, 17 months after the first operation, and was re-excised and showed identical histology. Meningiomas with rhabdoid change are very rare. The clinicopathologic features are presented and the differential diagnoses discussed.- - - - - - - - - - ranking = 8.6793352227119keywords = rhabdoid tumour, rhabdoid (Clic here for more details about this article) |
8/26. Atypical teratoid/rhabdoid tumors in adult patients: case report and review of the literature.Atypical teratoid/rhabdoid tumors (AT/RT) of the central nervous system are rare and extremely aggressive malignancies of early childhood. We report a case of AT/RT in an adult patient. A 30-year-old woman presented with headache, vomiting and ataxia during the second trimester of pregnancy. magnetic resonance imaging revealed a posterior fossa mass. A gross total resection was performed. Pathological examination revealed an AT/RT. Despite the dismal prognosis the patient decided not to undergo an abortion. For this reason postoperative accelerated hyperfractionated radiotherapy was limited to the tumor region. Six months later the woman delivered a healthy baby. One week postpartum, a central nervous system recurrence localized apart from the primary lesion was treated with radiosurgery. Two months later a diffuse progression was noted. Despite a 6 week course of oral temozolomide, the tumor progressed and the patient died 11 months after diagnosis. Although survival was short, surgery and involved field radiotherapy yielded a progression-free interval of 9 months. This allowed the patient to carry pregnancy to term. radiosurgery resulted in a complete remission of the first recurrence. Oral chemotherapy was not effective in controlling diffuse tumor spread.- - - - - - - - - - ranking = 5keywords = rhabdoid (Clic here for more details about this article) |
9/26. A case of submandibular malignant rhabdoid tumor transformed from papillary thyroid carcinoma.Malignant rhabdoid tumor (MRT) in the neck region is very rare. We report a case of MRT in a 60-year-old woman who had a history of papillary carcinoma of the thyroid gland 7 years previously. One year before admission, in 1995, thyroid carcinoma recurred, and the tumor contained a small undifferentiated region with rhabdoid features. The tumor in 1996 consisted of round to oval rhabdoid cells with abundant cytoplasm, and the growth pattern was diffuse and infiltrative, with no papillary structures. We therefore concluded that the lesion was MRT, transformed from papillary thyroid carcinoma.- - - - - - - - - - ranking = 7keywords = rhabdoid (Clic here for more details about this article) |
10/26. April 2001: a 70 year old woman with recurrent meningioma.The April Case of the Month (COM). The contributors report a case of a 70 year-old woman with recurrent meningiomas, one of which showed rhabdoid and lipomatous differentiation. Histopathological study of the first and second previous resections showed only typical meningothelial meningioma. On the third craniotomy, a new tumor specimen showed an admixture of classic meningothelial meningioma with lipomatous and rhabdoid foci. Immunohistochemical studies showed diffuse reactivity for epithelial membrane antigen and vimentin, as well as focal positivity for desmin and smooth muscle actin in the areas with rhabdoid features and S100 protein in the lipomatous foci. The presence of these three different and concomitant histological patterns only in the third surgical resection might support a metaplastic origin and, also, corroborates the concept that rhabdoid features are suggestive of an aggressive behavior.- - - - - - - - - - ranking = 4keywords = rhabdoid (Clic here for more details about this article) |
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