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Review literature: (Nigro and MacLennan, 2005) (Hays, 1993).
Definition of Prostate Sarcoma
Sarcoma of the prostate is a rare malignant tumor, which derives from connective tissue cells of the prostate.
Epidemiology of Prostate Sarcoma
Rhabdomyosarcoma has a share of 15% of all pediatric solid tumors. Of these, 20% arise from the urogenital tract, with most frequent localization in the prostate, bladder and paratesticular; rarely uterus and vagina. Peak incidence in the first two years. Leiomyosarcoma is very rare and has its peak incidence in adulthood.
Etiology of Prostate Sarcoma
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.
Half of prostatic leiomyosarcomas arise from pre-existing prostate cancer, especially after irradiation or hormone therapy.
Pathology of Prostate Sarcoma
Most common histological types are rhabdomyosarcoma in children and leiomyosarcoma in adulthood:
- Embryonic rhabdomyosarcoma: the most common type of the genitourinary system. Some show a very good prognosis, especially with spindle cell or botryoid histology.
- Alveolar rhabdomyosarcoma: is more common in the extremities as in the urogenital system, it has a poor prognosis.
- Leiomyosarcoma: the prevailing type in adulthood
- Rarities: angiosarcoma, fibrosarcoma, lymphosarcoma ...
Signs and Symptoms of Prostate Sarcoma
Subvesical obstruction with lower urinary tract symptoms, hematuria, urinary retention, disturbed defecation, abdominal pain and palpable lower abdominal tumor.
Diagnostic Work-Up of Prostatic Sarcoma
- Cystoscopy, in some cases TURB
- TRUS with prostate biopsy
- Computed tomography or better MRI from the abdomen and pelvis
- Chest x-ray
- Bone scan
Treatment of Prostate Sarcoma
Treatment of Prostatic Rhabdomyosarcoma:
Therapy should be carried out within clinical trials to improve treatment of this rare disease. After diagnosis, the patients are stratified to an appropriate study protocol in relation the 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 protocolls are available at e.g. Children's Oncology Group (COG), which is the successor of the Intergroup Rhabdomyosarcoma Study Group (IRSG).
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 in 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 resection of the tumor 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 Prostatic Leiomyosarcoma:
The prostatic leiomyosarcoma of adults is treated with radical surgery (cystoprostatectomy), followed by adjuvant irradiation and/or chemotherapy.
Prognosis of Prostate Sarcoma
The following clinical factors determine the prognosis: local tumor extension, tumor size, lymph node involvement, distant metastasis and the histological subtype. A goog prognosis (survival) depends critically on the response of neoadjuvant chemotherapy and complete surgical resection. Long-term survival is possible in 50–60% of the cases. In the further course after successful treatment, there is a risk for tumor recurrences, development of contracted bladders after irradiation and complications of urinary diversion.
Long-term survival is possible in 50% of cases.
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- 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.
- NIGRO, K. G. ; MACLENNAN, G. T.:
- Rhabdomyosarcoma of the bladder and prostate.
In: J Urol
173 (2005), Nr. 4, S. 1365
Deutsche Version: Prostatasarkom