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Pleomorphic Rhabdomyosarcoma


Presentation

  • We present a case of a 73-year-old patient diagnosed with pleomorphic rhabdomyosarcoma of the uterus. Together with the case report, a systematic review of the literature is presented focusing on different treatment strategies and their outcome.[ncbi.nlm.nih.gov]
Soft Tissue Mass
  • The patient was a 65-year-old man with an inguinal soft tissue mass. Following surgical excision, the patient was given radiotherapy and was well without disease after 6 years.[ncbi.nlm.nih.gov]
  • (a) T1-weighted coronal section showing iso- to hypo-intense soft tissue mass lesion. (b) T2-weighted axial section showing iso- to hyper-intense soft tissue mass lesion.[ccij-online.org]
  • In the present study, the patient was misdiagnosed with schwannoma, which led to the progression of the disease for half a year; therefore, fine-needle aspiration is crucial to the diagnosis of soft tissue masses.[spandidos-publications.com]
Recurrent Urinary Tract Infection
  • Abstract An unusual case of pleomorphic rhabdomyosarcoma is presented which arose in the urinary bladder of an adult male in association with recurrent urinary-tract infection and bladder outflow obstruction, secondary to a chronic post-traumatic urethral[ncbi.nlm.nih.gov]
Pleural Effusion
Diastolic Murmur
  • Auscultatory findings revealed mid-diastolic murmur in the mitral area with Grade 3 parasystolic murmur in the tricuspid area. S1 and S2 were normal with loud P2. Complete hemogram, biochemical, and urine examination findings were normal.[ijpmonline.org]
Heart Failure
  • Symptoms of cardiac sarcomas depend on the degree and exact site of involvement and may include valvular obstruction, arrhythmias, heart failure, pericardium effusion, systemic embolization, and systemic symptoms.[ijpmonline.org]
Mid-Diastolic Murmur
  • Auscultatory findings revealed mid-diastolic murmur in the mitral area with Grade 3 parasystolic murmur in the tricuspid area. S1 and S2 were normal with loud P2. Complete hemogram, biochemical, and urine examination findings were normal.[ijpmonline.org]
Impulsivity
  • On palpation, apical impulse was felt in the fifth intercostal space. Epigastric pulsation was present with Grade 3 left parasternal heave. The left second intercostal area was dull on percussion.[ijpmonline.org]
Enlarged Uterus
  • Vaginal ultrasonography showed enlarged uterus, 82 mm x 64 mm in size. The patient underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy with postoperative chemotherapy due to pleomorphic rhabdomyosarcoma of the uterus.[ncbi.nlm.nih.gov]

Workup

  • Metastatic workup with contrast enhanced computed tomography scan of thorax and abdomen were negative.[ccij-online.org]
  • Immunohistochemical workup was done for further characterization of the tumour and the antibodies included vimentin, desmin, alpha smooth muscle actin (ASMA), S100, CD34, CD68 and cytokeratins.[jpma.org.pk]
  • The initial diagnostic workup must address 2 key issues. First, the nature and extent of the primary disease must be determined. To accomplish this, surgical biopsy is performed early in the diagnostic process.[emedicine.medscape.com]
Mediastinal Mass
  • A large mediastinal mass was detected on chest CT scan. The chest surgeons advised against open biopsy.[ncbi.nlm.nih.gov]
Normal Chest X-Ray
  • Chest X-ray showed centrally placed trachea, enlarged left atrium and right atrium with double atrial shadow, prominent pulmonary artery with plethoric lung fields, and prominent vascular markings.[ijpmonline.org]
Pleural Effusion

Treatment

  • Secondary neoplasms following radiation and surgical treatment of rectal adenocarcinomas have not been reported in the English literature.[ncbi.nlm.nih.gov]

Prognosis

  • This is a very aggressive tumor with dismal prognosis. We report a new case of pleomorphic rhabdomyosarcoma of the kidney in an adult patient.[ncbi.nlm.nih.gov]

Etiology

  • (Etiology) The cause and mechanism of Pleomorphic Rhabdomyosarcoma formation is unknown The rapidly forming and acutely infiltrative tumor is said to arise from embryonic connective tissues responsible for skeletal muscle development It is suspected that[dovemed.com]

Epidemiology

  • Also included is a discussion on the different types of rhabdomyosarcoma with reviews of their histological features, epidemiology and common sites of origin.[ncbi.nlm.nih.gov]
  • […] occurring almost exclusively in adults and displaying evidence of skeletal muscle differentiation Diagnosis requires identification of pleomorphic rhabdomyoblasts and expression of skeletal muscle specific immunohistochemical markers CLINICAL ISSUES Epidemiology[basicmedicalkey.com]
  • Essential features High grade sarcoma composed of atypical cells that display skeletal muscle differentiation No embryonal or alveolar component of rhabdomyosarcoma Epidemiology Usually older than 50 70% male Clinical features Rapidly growing, painful[pathologyoutlines.com]
  • Epidemiology Clinical features True adult rhabdomyosarcomas occur predominantly in the lower limb, trunk wall or upper limb. Radiology Macroscopic appearances Tumours most often arise in deep soft tissues, often striated muscle.[e-immunohistochemistry.info]
Sex distribution
Age distribution

Prevention

  • NCT ID Phase Drugs 1 Surgery Followed by Chemotherapy in Treating Young Patients With Soft Tissue Sarcoma Completed NCT00002898 Phase 3 carboplatin;cyclophosphamide;epirubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate 2 Cyproheptadine in Preventing[malacards.org]
  • US20120135415A1 ( en ) * 2002-11-15 2012-05-31 Morehouse School Of Medicine Detecting cancer with anti-cxcl13 and anti-cxcr5 antibodies US8512701B2 ( en ) 2002-11-15 2013-08-20 Morehouse School Of Medicine Anti-CXCL13 and anti-CXCR5 antibodies for the prevention[google.sr]
  • A recent study by Bharathy et al. found that deacetylase inhibitor, entinostat works in aggressive subtype, alveolar rhabdomyosarcoma (aRMS) by specifically blocking the activity of HDAC3, thereby preventing epigenetic suppression of a microRNA that inhibits[en.wikipedia.org]

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