Costello syndrome is caused by heterozygous de novo missense mutations in the protooncogene HRAS with tumor predisposition, especially rhabdomyosarcoma. We here report two pediatric patients with Costello syndrome and umbilical ligament rhabdomyosarcoma. A review of the literature published in English in MEDLINE from January 1971 to June 2016 using the search terms "Costello syndrome" and "rhab...

The dose-limiting toxicity caused by standard chemotherapy has become a major roadblock to successful rhabdomyosarcoma chemotherapy. By screening a thiazolidinone library including 372 compounds, a novel synthetic compound, 2-((4-hydroxyphenyl)imino)-5-(3-methoxybenzylidene)thiazolidin-4-one (MHPT), was identified as a potent and selective anti-rhabdomyosarcoma agent. MHPT inhibited 50% of the ...

Rhabdomyosarcoma of the middle ear is a rare tumor, even rarer in adults and has a very poor prognosis. We report here an unusual case of rhabdomyosarcoma in middle ear of an adult, mimicking chronic suppurative otitis media and facial nerve palsy.

To address the poor outcomes in rhabdomyosarcoma, particularly the alveolar subtype, new therapies are needed. Potential cancer-specific alterations that may be molecular targets include gene fusions or copy number changes. Following the latter strategy, an attractive antigene approach was developed to inhibit MYCN oncogene expression in rhabdomyosarcoma.

Adult renal rhabdomyosarcoma is a rare subtype of renal sarcoma. We present a case of a renal mass treated with radical nephrectomy that subsequently was shown to be renal rhabdomyosarcoma. We discuss the clinical presentation, imaging findings, and histology for this case and review the available literature.

Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin typically affects children and adolescents, with an annual incidence of 4.3 cases per 1 million population aged <20 years. Para testicular RMS is rare, constituting 4-7% of all RMS in children and young adults. Here we report a case of 16-year old boy with right paratesticular solid mass. Ultrasound revealed vascular paratesticul...

A patient with rhabdomyosarcoma is described in whom the presenting clinical and laboratory features were those of disseminated intravascular coagulation. The patient's rapid downhill course was primarily expressed by haemorrhagic tendency. An alveolar rhabdomyosarcoma, affecting many organs, including vascular and cardiac lumina, was found at necropsy and was considered to be the cause of the ...

A 7-month-old Holstein heifer presented with posterior paresis and megaesophagus. At post mortem examination, a nodular tumor was found attached to the thoracic wall and the eighth to eleventh thoracic vertebrae, adjacent to the left posterior pulmonary lobe. The tumor was diagnosed as rhabdomyosarcoma by histology. This is a very rare case of spinal infiltration of rhabdomyosarcoma in cattle.

The PI3K/AKT/mTOR and RAS/RAF/MAPK pathways play essential roles in rhabdomyosarcoma. Singular targeting of each pathway is ineffective due to extensive cross-talk and compensatory feedback between these two pathways. Dual blockade with inhibitors of PI3K and MAPK in combination synergistically inhibits growth of rhabdomyosarcoma both in vitro and in vivo.

Rhabdomyosarcoma is the most frequent soft tissue sarcoma in children and adolescents, with a high rate of relapse that dramatically affects the clinical outcome. Multiagent chemotherapy, in combination with surgery and/or radiation therapy, is the treatment of choice. However, the relapse rate is disappointingly high and identification of new therapeutic tools is urgently needed. Under this re...