Urology Textbook
Clinical Essentials
By Dirk Manski, MD

 You are here: Urology Textbook > Prostate > Prostate sarcoma

Prostate Sarcoma

Review literature: (Merks et al., 2025) (National Cancer Institute, 2023).

Definition

Sarcoma of the prostate is a malignant mesenchymal tumor of the prostate.

Epidemiology

Epidemiology:

Rhabdomyosarcomas account for 1–3 % of all pediatric cancers, with an age-dependent incidence of 3–7 cases per 1 million children. 20 % of rhabdomyosarcomas originate in the genitourinary tract, most commonly in the prostate, urinary bladder, and paratesticular region, and less commonly in the uterus and vagina. Prostatic leiomyosarcoma is a rare tumor of adulthood.

Etiology and Genetics

Genetic Risk Factors:

Sarcomatous dedifferentiation:

Prostatic leiomyosarcomas may arise de novo or, rarely, after radiation therapy or androgen deprivation therapy in patients with pre-existing prostate cancer.

Pathology

Histological Typing:

Rhabdomyosarcoma predominates in children, whereas leiomyosarcoma predominates in adults.

TNM Staging for Adult Soft Tissue Sarcomas:

Risk stratification of rhabdomyosarcoma is not based on the TNM classification presented here, but on a modified TNM classification used by pediatric study groups; see Therapy.

T1:

Confined to the organ of origin.

T2:

Infiltration of adjacent structures.

T3:

Tumor size 10–15 cm or macroscopic tumor growth beyond the serosa.

T4:

Multifocal tumor or tumor > 15 cm.

N0:

No regional lymph node metastases.

N1:

Regional lymph node metastases.

M0:

No distant metastases.

M1:

Distant metastases.

G:

G1: well differentiated, G2: moderately differentiated, G3: poorly differentiated.

Signs and Symptoms

Hematuria, obstructive voiding symptoms, defecatory symptoms, lower abdominal pain, and a palpable lower abdominal mass.

Diagnosis

Imaging:

MRI of the pelvis and abdomen for local staging and CT of the chest to detect pulmonary metastases; CT of the abdomen, bone scintigraphy, or PET/CT may be added depending on the clinical question.

Biopsy:

Before definitive treatment planning, biopsy should be performed at a center with molecular diagnostic facilities. Either image-guided core needle biopsy or endoscopic biopsy should provide sufficient material for histology, immunohistochemistry, and molecular testing, including FOXO1 fusion analysis.

Treatment

Rhabdomyosarcoma:

Because of the rarity of the disease, treatment should be provided at a pediatric oncology sarcoma center within a study protocol. After diagnostic evaluation and biopsy, patients are treated according to their risk group, for example according to protocols of the North American Children's Oncology Group (COG), the European paediatric Soft tissue sarcoma Study Group (EpSSG), or the German Cooperative Soft Tissue Sarcoma Study Group (CWS). Tumor site, tumor size, histology, FOXO1 fusion status, lymph node involvement, metastases, and resection status are considered.

IRS Groups:

This grouping is used by the COG and European study groups.

In addition to cure, treatment aims to preserve a functional lower urinary tract as well as sexual function and fertility. If the tumor location is favorable, tumor resection is performed when the urinary bladder can be preserved (partial cystectomy). However, the most common sites are the trigone and prostate, so primary bladder-sparing surgery is not possible. These patients initially receive multimodal neoadjuvant treatment with chemotherapy and, if indicated, radiation therapy; subsequent bladder-preserving surgery is then possible in more than half of cases. Particularly effective agents are vincristine, actinomycin D, and cyclophosphamide; ifosfamide, doxorubicin, etoposide, irinotecan, and maintenance therapy with vinorelbine and cyclophosphamide are used according to risk. Urinary diversion after cystoprostatectomy should be determined individually, taking prognosis, recurrence risk, planned radiation therapy, and reconstructive options into account; continent diversions are possible with a favorable prognosis and controlled local disease.

Leiomyosarcoma:

Adult prostatic leiomyosarcoma should be treated at a sarcoma center. Localized disease requires complete resection with negative margins, either by cystoprostatectomy or pelvic exenteration. Perioperative radiation therapy or systemic therapy should be determined by an interdisciplinary team according to operability, tumor size, resection status, grade, and metastatic risk.

Prognosis

Rhabdomyosarcoma:

Prognostic factors include local extension, tumor size, lymph node involvement, distant metastases, histological subtype, FOXO1 fusion status, response to systemic therapy, and completeness of local control. Long-term survival is frequently possible in localized disease; in metastatic disease, the 5-year survival rate is below 50 % in many series. Late sequelae include recurrence, functional impairment of the lower urinary tract, complications of urinary diversion, and impaired sexual function and fertility.

Leiomyosarcoma:

Advanced prostatic leiomyosarcoma has an unfavorable prognosis. Resectability, tumor size, grade, and metastatic status are prognostic factors. Long-term survival is possible in localized disease after complete resection.

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

References

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.

J. H. M. Merks et al., “European standard clinical practice recommendations for children and adolescents with Rhabdomyosarcoma a joint EpSSG, CWS and ERN PaedCan project,” EJC Paediatric Oncology, vol. 5, p. 100229, 2025, doi: https://doi.org/10.1016/j.ejcped.2025.100229.

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



  Deutsche Version: Prostatasarkom

Urology-Textbook.com – Choose the Ad-Free, Professional Resource

This website is designed for physicians and medical professionals. It presents diseases of the genital organs through detailed text and images. Some content may not be suitable for children or sensitive readers. Many illustrations are available exclusively to Steady members. Are you a physician and interested in supporting this project? Join Steady to unlock full access to all images and enjoy an ad-free experience. Try it free for 7 days—no obligation.

New release: The first edition of the Urology Textbook as an e-book—ideal for offline reading and quick reference. With over 1300 pages and hundreds of illustrations, it’s the perfect companion for residents and medical students. After your 7-day trial has ended, you will receive a download link for your exclusive e-book.