Dr. med. Dirk Manski

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Rhabdomyosarcoma of the Bladder

Definition and Epidemiology

Sarcoma of the bladder is a rare malignant tumor that derives from connective tissue cells of the bladder.


Rhabdomyosarcoma has a share of 1–3% of all pediatric cancer cases, and the incidence is 3–7 cases per 1 million children, depending on age. 20% of rhabdomyosarcoma arise from the urogenital tract, with the most frequent localization in the prostate, bladder, and paratesticular, rarely uterus and vagina. Peak incidence in the first two years.

Etiology and Genetics

Genetic risk factors:

Pathology of Bladder Sarcoma

Histological typing:

The most common histological types are rhabdomyosarcoma in children and leiomyosarcoma in adulthood:

TMN Staging (UICC 2017):

Signs and Symptoms of Bladder Sarcoma

Subvesical obstruction with lower urinary tract symptoms, hematuria, urinary retention, disturbed defecation, abdominal pain, and palpable lower abdominal tumor.

Diagnostic Workup of Bladder Sarcoma

Treatment of Bladder Sarcoma

Treatment of Rhabdomyosarcoma of the Bladder:

Treatment should be carried out within clinical trials to improve the treatment of this rare disease. After diagnosis, the patients are stratified to an appropriate study protocol in relation to the tumor stage and risk group. The following factors are considered: tumor location, tumor size, histology, grading, lymph node involvement, metastases, and results of surgical treatment. Study protocols are, e.g., Children's Oncology Group (COG), which is the successor of the Intergroup Rhabdomyosarcoma Study Group (IRSG) or European Cooperative Groups (SIOP).

IRS-Grouping is used both by COG and European study groups.

The goal of rhabdomyosarcoma treatment is healing and preserving a functional lower urinary tract. A tumor resection is performed when the bladder can be spared (partial cystectomy). Since the most common tumor sites are the trigone and the prostate, primary bladder-sparing surgery is not possible. This group of patients will receive a neoadjuvant treatment with chemotherapy and possibly radiation. The subsequent operation can be performed with a bladder-preserving technique in over half of the cases. Effective chemotherapeutic agents are vincristine, actinomycin D, cyclophosphamide, adriamycin, etoposide, and ifosfamide, which are used in various combinations. The role of neoadjuvant radiation is controversial, because the tumor resection and reconstruction of the urinary tract becomes more difficult (Filipas et al., 2004). Also controversial is the technique of urinary diversion after cystectomy. Some authors advocate a transient incontinent urinary diversion until the oncological risk can be finally assessed, and re-operations are unlikely. Others advocate continent urinary diversion with the primary tumor resection; this should be limited to situations with a good prognosis.

Treatment of Leiomyosarcoma of the Bladder:

Bladder leiomyosarcoma in adults is treated with radical surgery (cystoprostatectomy), followed by adjuvant irradiation and/or chemotherapy.

Related diseases

Index: 1–9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z


National Cancer Institute: Childhood Rhabdomyosarcoma Treatment (PDQ)–Health Professional Version https://www.cancer.gov/types/soft-tissue-sarcoma/hp/rhabdomyosarcoma-treatment-pdq

HAYS, D. M.: Bladder/prostate rhabdomyosarcoma: results of the multi-institutional trials of the Intergroup Rhabdomyosarcoma Study.
In: Semin Surg Oncol
9 (1993), Nr. 6, S. 520–3

Filipas, D.; Fisch, M.; Stein, R.; Gutjahr, P.; Hohenfellner, R. & Thüroff, J. W. Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery.
BJU Int, 2004, 93, 125-129.

Linardic CM. PAX3-FOXO1 fusion gene in rhabdomyosarcoma. Cancer Lett. 2008 Oct 18;270(1):10-8. doi: 10.1016/j.canlet.2008.03.035. Epub 2008 May 23. PMID: 18457914; PMCID: PMC2575376.

NIGRO, K. G. ; MACLENNAN, G. T.: Rhabdomyosarcoma of the bladder and prostate.
In: J Urol
173 (2005), Nr. 4, S. 1365

  Deutsche Version: Sarkom der Harnblase