Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Myxofibrosarcoma is rarely found in the orbit, let alone as a primary orbital tumor. With this having only been previously reported 5 times in the literature, there is a very limited understanding of its presentation and course. Typical management involves surgical excision with or without postoperative radiotherapy. Low-grade malignancy, although rare to metastasize, still has a high rate of local recurrence and with each recurrence increases the risk of metastatic potential. With this in mind, analysis of each case is critical in better understanding the disease, and this case adds to the current literature.
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PMID:Orbital Myxofibrosarcoma: Case Report and Review of Literature. 3020 36

Myxofibrosarcomas (MFSs) are sarcomas most commonly seen in older patients. These are tumors of deep soft tissue seen in subcutaneous tissue and deep fascia, with frequent muscle involvement. These sarcomas are notorious for recurrences and progression to a higher grade with notable metastatic potential. They are very often under-diagnosed owing to their inherent morphological variability. A case of MFS is presented as a cutaneous, exophytic, polypoidal mass because of its rarity and importance of timely diagnosis, as under-diagnosis may lead to inadequate clearance of tumor, recurrences, metastases and increased mortality.
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PMID:Rare, risky, recurrent: An enigmatic cutaneous polyp. 3068 40

Myxofibrosarcoma (MFS) and undifferentiated pleomorphic sarcoma (UPS) are highly genetically complex soft tissue sarcomas. Up to 50% of patients develop distant metastases, but current systemic therapies have limited efficacy. MFS and UPS have recently been shown to commonly harbor copy number alterations or mutations in the tumor suppressor genes RB1 and TP53. As these alterations have been shown to engender dependence on the oncogenic protein Skp2 for survival of transformed cells in mouse models, we sought to examine its function and potential as a therapeutic target in MFS/UPS. Comparative genomic hybridization and next-generation sequencing confirmed that a significant fraction of MFS and UPS patient samples (n = 94) harbor chromosomal deletions and/or loss-of-function mutations in RB1 and TP53 (88% carry alterations in at least one gene; 60% carry alterations in both). Tissue microarray analysis identified a correlation between absent Rb and p53 expression and positive expression of Skp2. Downregulation of Skp2 or treatment with the Skp2-specific inhibitor C1 revealed that Skp2 drives proliferation of patient-derived MFS/UPS cell lines deficient in both Rb and p53 by degrading p21 and p27. Inhibition of Skp2 using the neddylation-activating enzyme inhibitor pevonedistat decreased growth of Rb/p53-negative patient-derived cell lines and mouse xenografts. These results demonstrate that loss of both Rb and p53 renders MFS and UPS dependent on Skp2, which can be therapeutically exploited and could provide the basis for promising novel systemic therapies for MFS and UPS. SIGNIFICANCE: Loss of both Rb and p53 renders myxofibrosarcoma and undifferentiated pleomorphic sarcoma dependent on Skp2, which could provide the basis for promising novel systemic therapies.See related commentary by Lambert and Jones, p. 2437.
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PMID:Rb and p53-Deficient Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma Require Skp2 for Survival. 3254 Aug 53

Myxofibrosarcoma is one of the most common soft tissue sarcomas in the elderly. It is characterized by an extremely high rate of local recurrence, higher than other soft tissue tumors, and a relatively low risk of distant metastases.Magnetic resonance imaging (MRI) is the imaging modality of choice for the assessment of myxofibrosarcoma and plays a key role in the preoperative setting of these patients.MRI features associated with high risk of local recurrence are: high myxoid matrix content (water-like appearance of the lesions), high grade of contrast enhancement, presence of an infiltrative pattern ("tail sign"). On the other hand, MRI features associated with worse sarcoma specific survival are: large size of the lesion, deep location, high grade of contrast enhancement. Recognizing the above-mentioned imaging features of myxofibrosarcoma may be helpful to stratify the risk for local recurrence and disease-specific survival. Moreover, the surgical planning should be adjusted according to the MRI features.
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PMID:Myxofibrosarcoma: clinical and prognostic value of MRI features. 3272 25

The main therapeutic strategy for metastatic Myxofibrosarcoma (MFS) is palliative chemotherapy. A number of studies have demonstrated that anti-angiogenic therapy and immunotherapy could improve the survival rate of patients with metastases. However, the effectiveness of the combination of anti-angiogenic therapy and immunotherapy for the therapy of MFS is undetermined. The current study reports a case of metastatic myxofibrosarcoma that was treated with combination Nivolumab (monoclonal antibody, PD-1 inhibitor) and Bevacizumab (monoclonal antibody, anti-VEGF) after progression from the single use of Nivolumab. The aim of the current study is to assess the efficacy and safety of Nivolumab and Bevacizumab for metastatic myxofibrosarcoma and to review the literature. Up to the termination of the follow-up, the patient achieved a partial response for 16 months, had an overall survival for over 29 months since the metastasis and demonstrated a sustained benefit from treatment. The most frequent adverse events were fatigue, abnormality of Alanine aminotransferase (ALT), hypertension and proteinuria. Nivolumab and Bevacizumab treatment indicate beneficial clinical effects and are indicated to be safe to use in patients with metastatic myxofibrosarcoma.
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PMID:Combination nivolumab and bevacizumab for metastatic myxofibrosarcoma: A case report and review of the literature. 3290 14

Myxofibrosarcoma is a soft tissue neoplasm that usually affects the extremities of the elderly. It usually presents as a slow-growing painless mass that can metastasize to the lung and bone. However, the reported incidence of primary osseous myxofibrosarcoma is very low. Moreover, the metastatic pattern of these bone tumors is largely unknown. We describe a unique case of a young Caucasian male with symptomatic low-grade myxofibrosarcoma arising in the left clavicle, who presented with multiple bone metastases.
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PMID:Primary Osseous Low-Grade Myxofibrosarcoma of Clavicle Presenting With Multiple Skeletal Metastases. 3302 50


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