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Rhabdomyosarcoma of the Prostate

Rhabdomyosarcoma of Prostate


Presentation

  • Embryonal rhabdomyosarcoma of the prostate is typically a rare mesenchymal tumor of young boys, presenting with obstructive urinary symptoms or an abdominal mass.[ncbi.nlm.nih.gov]
  • The patient received chemotherapy and radiation therapy but died 10 months after initial presentation.[ncbi.nlm.nih.gov]
  • He presented with gross hematuria and high fever. The level of prostate-specific antigen (PSA) was 27.9 ng/ml. Computed tomography revealed a suspected infectious cyst in the prostate. After antibiotic treatment was begun, the PSA level normalized.[ncbi.nlm.nih.gov]
  • Of the 50% who had metastatic disease at presentation, two were dead within 12 months despite aggressive chemotherapy and irradiation. The third is currently on treatment.[ncbi.nlm.nih.gov]
  • AR, Shanggar K, Muhilan P, Dublin N, George Lee EG, Azad Hassan (0021) AN UNUSUAL PRESENTATION OF A RARE RHABDOMYOSARCOMA OF URINARY BLADDER AND PROSTATE - CASE REPORT. Journal of Health and Translational Medicine.[repository.um.edu.my]
High Fever
  • He presented with gross hematuria and high fever. The level of prostate-specific antigen (PSA) was 27.9 ng/ml. Computed tomography revealed a suspected infectious cyst in the prostate. After antibiotic treatment was begun, the PSA level normalized.[ncbi.nlm.nih.gov]
Sudden Infant Death Syndrome
  • An incidental rhabdomyosarcoma of the prostate gland in a sudden infant death syndrome (SIDS) case, which highlights the value of histological examination of all organs in autopsy cases, is reported.[ncbi.nlm.nih.gov]
  • The American Journal of Forensic Medicine and Pathology: March 1994 Original Article: PDF Only Abstract An incidental rhabdomyosarcoma of the prostate gland in a sudden infant death syndrome ( SIDS ) case, which highlights the value of histological examination[journals.lww.com]
Soft Tissue Mass
  • CT scan and MRI study reveal a large soft tissue mass with areas of necrosis replacing the whole prostate, but the radiological differential diagnosis with prostate adenocarcinoma can be very difficult.[ncbi.nlm.nih.gov]
  • Computed tomography of abdomen unveiled an incidental, 6 5 5 cm homogenous soft tissue mass rising from prostrate. The prostate specific antigen (PSA) and prostatic acid phosphatase (PAP) were within normal limits.[casesjournal.biomedcentral.com]
Pelvic Mass
  • Embryonal rhabdomyosarcoma of the prostate in a 19 year old Posted by: Philip Alex Posted on: May 27th, 2006 0 Comments A 19 year old boy presented to us with edema, difficulty in passing urine and a pelvic mass extending till the umbilicus.[manalihospital.com]
  • It is characterized by a high degree of malignancy, both local rapid growth with formation of large pelvic masses, often leading to renal failure due to urethral obstruction, and systemic spread, commonly to the lungs, liver and bone.[ncbi.nlm.nih.gov]
Pelvic Mass
  • Embryonal rhabdomyosarcoma of the prostate in a 19 year old Posted by: Philip Alex Posted on: May 27th, 2006 0 Comments A 19 year old boy presented to us with edema, difficulty in passing urine and a pelvic mass extending till the umbilicus.[manalihospital.com]
  • It is characterized by a high degree of malignancy, both local rapid growth with formation of large pelvic masses, often leading to renal failure due to urethral obstruction, and systemic spread, commonly to the lungs, liver and bone.[ncbi.nlm.nih.gov]
Urinary Retention
  • Transurethral resection (TUR) of the prostate was performed to diagnosis and treat for complete urinary retention.[ncbi.nlm.nih.gov]
  • A 32-year-old man visited our hospital due to urinary retention. Prostate-specific antigen was 13.7 ng/mL. Imaging examinations showed a large prostatic tumor and lung and internal iliac lymph node metastases.[ncbi.nlm.nih.gov]
  • The presenting symptoms are often related to urethral obstruction, with frequency, hesitancy, and dysuria as predominant symptoms or, less often, with hematuria and acute urinary retention.[ncbi.nlm.nih.gov]
Prostatic Mass
  • After chemotherapy completion, CT and CT/PET showed a complete remission of pulmonary disease and partial response of the prostate mass.[ncbi.nlm.nih.gov]
  • The CT findings in the prostatic tumors included an enlarged, inhomogeneous prostatic mass with invasion of the perirectal fat.[journals.lww.com]
Prostatic Disease
  • Conclusion While it remains questionable which therapeutic approach for prostate ERMS in adults is the most appropriate, our report demonstrates that a chemo-radiation combined treatment can control the prostate disease, reducing the symptoms and improving[ncbi.nlm.nih.gov]
Enlargement of the Prostate
  • prostate nodular prostate polyp of prostate Type 1 Excludes benign neoplasms of prostate (adenoma, benign) (fibroadenoma) (fibroma) (myoma) ( D29.1 ) Type 2 Excludes malignant neoplasm of prostate ( C61 ) Adenocarcinoma - see also Neoplasm, malignant[icd10data.com]

Workup

  • The workup of these tumors typically includes the following studies: CT and MRI scans of affected region(s). Chest x-ray or CT scan of chest. Bone scan. Bone marrow biopsy. For genitourinary location: Cystoscopy (scope to look inside the bladder).[oncolink.org]
  • […] resonance imaging (MRI) Bone scanning Ultrasonography Echocardiography Biopsy Open or core needle biopsy: To obtain tissue sampling for diagnosis and molecular studies Bone marrow aspiration and biopsy: To assess for metastatic spread to bone marrow See Workup[emedicine.medscape.com]

Treatment

  • During treatment for acute lymphatic leukemia at the age of 6 years, the patient had been exposed to a cumulative radiation dose of 10 Gy across his entire body.[ncbi.nlm.nih.gov]
  • Treatment is combined radical surgery and chemotherapy, and usually radiotherapy as well. The prognosis is extremely poor in all patients. Our case is added to the few in the literature in which this disease occurred in a man more than 50 years old.[ncbi.nlm.nih.gov]
  • Three patients had an unsuccessful treatment attempt with sildenafil. 8 patients, including the three failures of oral PDE-5-inhibitors, were successfully treated with intracavernous injection of Alprostadil (mean post treatment SIEF-NS 38.25).[spuonline.org]
  • The third is currently on treatment. Although chemotherapy has markedly improved the prognosis, surgery is still necessary in most cases for cure.[ncbi.nlm.nih.gov]

Prognosis

  • The prognosis is extremely poor in all patients. Our case is added to the few in the literature in which this disease occurred in a man more than 50 years old.[ncbi.nlm.nih.gov]
  • The prognosis for rhabdomyosarcoma of the prostate has improved significantly in the last few decades. The management involves a combination of chemotherapy, radiotherapy and surgery.[ncbi.nlm.nih.gov]
  • In adults prognosis is very poor, therefore early diagnosis is crucial. To date, only three cases of spindle cell subtype of embryonal rhabdomyosarcoma of the prostate in an adult have been published.[ncbi.nlm.nih.gov]
  • Although chemotherapy has markedly improved the prognosis, surgery is still necessary in most cases for cure.[ncbi.nlm.nih.gov]
  • Recently, Nabi et al. 16 reported two cases of adults prostate ERMS with poor prognosis for both.[ncbi.nlm.nih.gov]

Etiology

  • Use Additional Use Additional Help Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10data.com]
  • Etiology of Prostate Sarcoma Li-Fraumeni syndrome: Li-Fraumeni syndrome is associated with mutation of the p53 suppressor gene: this leads to children with sarcoma whose mothers have a high risk of breast cancer and siblings with an increased cancer risk[urology-textbook.com]
  • […] ejaculatory duct Cowper gland Benign prostatic hyperplasia Acute inflammation of the prostate Chronic inflammation of the prostate Nonspecific granulomatous prostatitis Post-biopsy granuloma Prostatic xanthoma Granulomatous inflammation of infectious etiology[elsevier.com]

Epidemiology

  • Karger AG, Basel References Sultan I, Qaddoumi I, Yaser S, et al: Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: an analysis of 2,600 patients. J Clin Oncol 2009;27:3391-3397.[karger.com]
  • Back to 2016 Fall Congress Pediatric Bladder and Prostatic Sarcomas: A Surveillance, Epidemiology, and End Results (SEER) Analysis Casey A. Seideman, MD , Andrew Jacobs, MD, Lane S. Palmer, MD, Richard Glick, MD.[fallcongress.spuonline.org]
  • Essential features Small round cell to spindle cell tumor, variable cellularity Occurs in young patients and requires aggressive treatment Epidemiology Most common prostatic malignancy in children / infants Clinical features Firm, smooth enlargement of[pathologyoutlines.com]
  • Rhabdomyosarcoma in childhood and adolescence: Epidemiology, pathology, and molecular pathogenesis. . Accessed Nov. 30, 2017. Okcu MF, et al. Rhabdomyosarcoma in childhood and adolescence: Clinical presentation, diagnostic evaluation, and staging. .[mayoclinic.org]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology Although the tumor is believed to arise from primitive muscle cells, tumors can occur anywhere in the body except bone. The most common sites are the head and neck (28%), extremities (24%), and genitourinary (GU) tract (18%).[indiacancersurgerysite.com]
  • […] ear ( J Clin Oncol 1995;13:610 ) Also common in the paratesticular soft tissues and the genitourinary tract Extremity involvement of the somatic soft tissues is less common ( 9%) Metastasizes to soft tissue, serosa, lung, lymph nodes and bone marrow Pathophysiology[pathologyoutlines.com]
  • Pathophysiology The tumor is believed to arise from primitive muscle cells, but tumors can occur anywhere in the body; however, a primary bone rhabdomyosarcoma has not been reported.[emedicine.medscape.com]

Prevention

  • According to the American Institute for Cancer Research, 40 percent of cancers can be prevented through healthy eating, exercise and lifestyle changes.[protonpals.org]
  • Is chronic lung disease in low birth weight infants preventable? A survey of eight centers. ‎ Página 84 - Tanner JM, Whitehouse RH, Marubini E, Resele LF. The adolescent growth spurt of boys and girls of the Harpenden growth study. ‎[books.google.es]
  • Post operated cases are dealt with good results in preventing the recurrence.[cancerhealercenter.com]
  • They can be used to evaluate medications or treatments to prevent cancer, detect it earlier, or help manage side effects. Clinical trials are extremely important in furthering our knowledge of this disease.[oncolink.org]
  • Rhabdomyosarcoma is not associated with environmental factors, and it is important to keep in mind that there is nothing you could have done, or avoided doing, that would have prevented the tumor from developing.[danafarberbostonchildrens.org]

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