Successful Salvage and Long-Term Survival after Recurrent Malignant Rhabdoid Tumor

Purpose. The objective of this study is to report a case of a rare, highly lethal tumor, extrarenal malignant rhabdoid tumor (EMRT) in a 43-year-old man who initially presented with a local recurrence and is now continuously disease free 14 years after aggressive surgical treatment. The case and literature are discussed

Identification of heterogeneity among soft tissue sarcomas by gene expression profiles from different tumors

The heterogeneity that soft tissue sarcomas (STS) exhibit in their clinical behavior, even within histological subtypes, complicates patient care. Histological appearance is determined by gene expression. Morphologic features are generally good predictors of biologic behavior, however, metastatic propensity, tumor growth, and response to chemotherapy may be determined by gene expression patterns that do not correlate well with morphology. One approach to identify heterogeneity is to search for genetic markers that correlate with differences in tumor behavior. Alternatively, subsets may be identified based on gene expression patterns alone, independent of knowledge of clinical outcome. We have reported gene expression patterns that distinguish two subgroups of clear cell renal carcinoma (ccRCC), and other gene expression patterns that distinguish heterogeneity of serous ovarian carcinoma (OVCA) and aggressive fibromatosis (AF). In this study, gene expression in 53 samples of STS and AF [including 16 malignant fibrous histiocytoma (MFH), 9 leiomyosarcoma, 12 liposarcoma, 4 synovial sarcoma, and 12 samples of AF] was determined at Gene Logic Inc. (Gaithersburg, MD) using Affymetrix GeneChip® U_133 arrays containing approximately 40,000 genes/ESTs. Gene expression analysis was performed with the Gene Logic Genesis Enterprise System® Software and Expressionist software. Hierarchical clustering of the STS using our three previously reported gene sets, each generated subgroups within the STS that for some subtypes correlated with histology, and also suggested the existence of subsets of MFH. All three gene sets also recognized the same two subsets of the fibromatosis samples that we had found in our earlier study of AF. These results suggest that these subgroups may have biological significance, and that these gene sets may be useful for sub-classification of STS. In addition, several genes that are targets of some anti-tumor drugs were found to be differentially expressed in particular subsets of STS

Giant cell tumor of the uterus: case report and response to chemotherapy

BACKGROUND: Giant cell tumor (GCT) is usually a benign but locally aggressive primary bone neoplasm in which monocytic macrophage/osteoclast precursor cells and multinucleated osteoclast-like giant cells infiltrate the tumor. The etiology of GCT is unknown, however the tumor cells of GCT have been reported to produce chemoattractants that can attract osteoclasts and osteoclast precursors. Rarely, GCT can originate at extraosseous sites. More rarely, GCT may exhibit a much more aggressive phenotype. The role of chemotherapy in metastatic GCT is not well defined. CASE PRESENTATION: We report a case of an aggressive GCT of the uterus with rapidly growing lung metastases, and its response to chemotherapy with pegylated-liposomal doxorubicin, ifosfamide, and bevacizumab, along with a review of the literature. CONCLUSION: Aggressive metastasizing GCT may arise in the uterus, and may respond to combination chemotherapy

Surgery and Actinomycin Improve Survival in Malignant Rhabdoid Tumor

Purpose. Malignant rhabdoid tumor (MRT) is an uncommon tumor that rarely occurs outside of renal and central nervous system (CNS) sites. Data from the literature were compiled to determine prognostic factors, including both demographic and treatment variables of malignant rhabdoid tumor, focusing on those tumors arising in extra-renal, extra-CNS (ER/EC MRT) sites. Patients and Methods. A systematic review and meta-analysis was performed by extracting demographic, treatment, and survival follow up on 167 cases of primary ER/EC MRT identified in the literature. Results. No survival differences were observed between those treated with or without radiation, or with or without chemotherapy. A Cox regression of overall survival revealed several independent prognostic factors. Surgical excision had a 74% (P = 0.0003) improvement in survival. Actinomycin had a 73% (P = 0.093) improvement in survival. Older age was associated with improved survival. The four-year survival, by Kaplan-Meier estimates, comparing patients less than two years old versus older than two at diagnosis was 11% versus 35%, respectively (P = 0.0001, Log-Rank). Conclusion. ER/EC MRT is a rare, soft-tissue tumor with a poor prognosis most commonly occurring in children. Surgical resection, treatment with actinomycin, and older age at diagnosis are all associated with improved survival