PLEOMORPHIC RHABDOMYOSARCOMA IN THE HEAD AND NECK REGION: A RARE CASE REPORT

Rhabdomyosarcoma (RMS) is a rare, aggressive, malignant mesenchymal tumor of skeletal muscle cells. The pleomorphic histological variant of RMS occurs in adults beyond 45years of age and represents the most aggressive subtype with an incidence of approximately 0.44/100,000. The diagnosis of RMS is difcult with the 5year overall survival rate less than 50%. It presents varied clinical and biological behavior and requires individualized management. The common region of metastasis includes lymph nodes, lungs and bone marrow. Here we report a case of pleomorphic rhabdomyosarcoma in 58year old female. The patient reported with a swelling in the neck region and had a history of surgical treatment for ovarian tumor. Histopathology ndings revealed metastatic undifferentiated carcinoma. The case was positive for immunohistochemistry markers and their ndings are diagnosed as pleomorphic rhabdomyosarcoma. This is a rare case of RMS which had metastasized to head and neck and this article emphasizes the importance of IHC in accurate and clear diagnosis of RMS.

Tran's Arterial Embolization of an Unusual Pleomorphic Rhabdomyosarcoma in Adulthood for Better Preoperative Conditioning

Rhabdomyosarcomas, being the most common sarcomas of childhood, have an extremely poor prognosis, and pleomorphic rhabdomyosarcomas occur very rarely in adults. Despite advanced diagnostics, it is not uncommon for such tumours to be misinterpreted. The 73-year-old patient was diagnosed with a swelling on the inner side of the left thigh 3 years previously after a minor trauma, which was interpreted as a small well-revascularised haemangioma in original CT angiography. While this mass showed little change over the last 2.5 years, a sudden progression of growth occurred 6 months ago. With the working diagnosis of a haemangioma, differential diagnosis of a schwanoma, the vessels supplying the tumour from profunda femoral artery were visualised preoperatively in a selective angiography and embolised. Shortly before the operation, dupex sonography revealed a renewed hypervasculariasis of the tumour. The supplied vessels from the superficial femoral artery and from the main left profunda trunc were embolised with Embosphere Microspheres 500-700 (Merit). Complete devascularisation of the tumour was achieved, so that the tumour could be resected very effectively in accordance with the compartment. The histological work-up of the tumour resulted in the diagnosis of a pleomorphic rhabdomyosarcoma grade II with tumor-free margins. Preoperative embolisation could be an effective method for preoperative conditioning of such rhabdomyosarcoma. Using this example, this study discusses the current diagnostic and therapeutic options for adult rhabdomyosarcomas.

UNUSUAL PRESENTATION OF PRIMARY PLEOMORPHIC RHABDOMYOSARCOMA OF HEART: COMPLETE ATRIOVENTRICULAR BLOCK AND SEVERE MITRAL REGURGITATION

Primary cardiac tumours are rare and difcult to diagnose because most are asymptomatic or have varied non-specic presentations. This report describes a 29-year-old man presenting with complete heart block, primary cardiac tumour in the left atrium, and severe mitral regurgitation. In view of the primary severe mitral regurgitation and complete heart block, mitral valve repair and pacemaker insertion were planned. Mitral valve repair was done with 29mm St Jude tailor annuloplasty ring, and the biopsy was taken from the nodules noted in the left atrium; temporary right ventricular epicardial pacemaker implantation and CABG with SVG to PDA graft were done to look for the recovery of complete heart block. Histopathological examination revealed pleomorphic rhabdomyosarcoma. The patient developed renal failure and liver failure during the postoperative period and expired after 10 days

CASE REPORT: TREATMENT OF PLEOMORPHIC RHABDOMYOSARCOMA IN ADULT

Rhabdomyosarcoma, a malignant tumor of the striated muscle, is highly variable in terms of frequency and type according to age, the presence of this tumor in adults being even rarer. The present case is about oncological management of pleomorphic rhabdomyosarcoma in adults with ICE scheme, obtaining a good oncological response and can be considered a good scheme for tumor relapse.