Scand J Urol Nephrol25: 329-330, 1991
A N ADULT MAN WITH A RHABDOMYOSARCOMA OF T H E PROSTATE A Case Report Siebe D. Bos and Ed Te Slaa From the Department of Surgery and Urology, Delft. The Netherlands
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Newcastle on 01/04/15 For personal use only.
(Submitted October 16, 1990. Accepted for publication November 27, 1990)
Sarcomas of the prostate are rare. There is an enormous variety in the treatment of rhabdomyosarcomas of the prostate including surgery, radiotherapy and multi drug chemotherapy or a combination of these options with a 5-year survival rate below 40%. CASE REPORT A 34-year-old patient with a negative urological histor y presented at our hospital with complaints of obstipation, obstructive voiding problems and perineal discomfort. Digital rectal examination revealed an enormous irregular prostate growing into the rectum with an intact rectal mucosa. Fine needle biopsy was taken. Immunopathological investigation showed a strong positive staining with vimentine and electronmicroscopy showed a mesenchymal tumor with some act in filaments suggestive of a rhabdomyosarcoma (Fig. I). Physical examination showed no further abnormalities. Standard blood tests were negative. Carcinoembryonic antigen, a-l -fetoprotein and p-human chorionic gonadotropin were normal. Chest X-ray: normal. Bone scan: hotspot left hip and right clavicular. CT-scan showed an enormous prostate with compression of the rectum (Fig. 2). The right seminal vesicle was enlarged. The patient was treated with adriamycin 60 mglm' and cisplatinum 100 mg/m' during 3 courses. Digital examination showed regression of the tumor. On CT-scan however, no regression was seen while multiple lung metastases had developed. Because the patient was unresponsive to the treatment the chemotherapy was changed in ifosfamide 6000 mg, vincristine 2 mg and actinomycin-D 1 mg. After 3 courses there was progression of tumor growth. After the treatment was stopped the patient succumbed one month later.
DISCUSSION Sarcomas of the Prostate are rare, constituting only 0.1 O/o to 0.2 O/o of all primary prostatic tu-
mors. They may be subclassified as rhabdomyosarcomas, leiom yosarcomas and fibrosarcomas. Rhabdomyosarcomas of the prostate frequently occur in children and young adults while leiom yosarcomas occur in older persons. The lung is the most common site of metastases of soft tissue sarcomas, as in the present case. Ahlering et at. (1) described two patients 17 and 27 years old with a rhabdomyosarcoma of the prostate. The first patient had a stage 111
Fig. 1. Electron microscopy of a mesenchymal tumor. The nucleus is in the lower right corner. The white stripes in the left corner abovethe nucleus are actine filaments suggestive for a rhabdomyosarcoma of the prostate. Scand J Urol Nephrol25
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Newcastle on 01/04/15 For personal use only.
330
S. D.Bos and E. Te Slaa
Fig. 2. CT-scan of the tumor in the prostate and the compression of the rectum. The dark arrow indicates the prostate and the open arrow the compressed rectum.
tumor and was treated with 4 courses of cisplatinum, etoposide, vincristine, doxorubicin and cyclophosphamide to which he was unresponsive and died 4 months later. The second patient who was treated with 4 courses of cisplatinum and doxorubicin followed by prostatectomy and external beam radiotherapy is free of
Scand J Urol Nephrol25
disease after 2 years. Ghavimi et al. (2) described 14 patients with a mean age of 9.5 years. All patients received multi drug chemotherapy (Dactinomycin, doxorubicin, vincristine and cyclophosphamide or combined with bleomycin and methotrexate), 1 1 radical cystoprostatectomy and 12 patients received external beam radiotherapy. Nine patients are still alive with a mean duration of survival of 74 months. Therapy is changing into the direction of multi drug chemotherapy without extensive operation. After reducing the tumor surgery and possible adjuvant radiotherapy is at this time the therapy of choice. During treatment the reaction of the tumor must be carefully monitored to obtain the best result for this very aggressive tumor. REFERENCES I . Ahlering TE, Weintraub P, Skinner DG. Management of adult sarcomas of the bladder and prostate. J Urol 1988; 140: 1397-1399. 2. Ghavimi F, Herr H, Jereb B, Exelby PR. Treatment of genito-urinary rhabdomyosarcomas in children. J Urol 1984; 132: 313-319.
A N ADULT MAN WITH A RHABDOMYOSARCOMA OF T H E PROSTATE A Case Report Siebe D. Bos and Ed Te Slaa From the Department of Surgery and Urology, Delft. The Netherlands
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Newcastle on 01/04/15 For personal use only.
(Submitted October 16, 1990. Accepted for publication November 27, 1990)
Sarcomas of the prostate are rare. There is an enormous variety in the treatment of rhabdomyosarcomas of the prostate including surgery, radiotherapy and multi drug chemotherapy or a combination of these options with a 5-year survival rate below 40%. CASE REPORT A 34-year-old patient with a negative urological histor y presented at our hospital with complaints of obstipation, obstructive voiding problems and perineal discomfort. Digital rectal examination revealed an enormous irregular prostate growing into the rectum with an intact rectal mucosa. Fine needle biopsy was taken. Immunopathological investigation showed a strong positive staining with vimentine and electronmicroscopy showed a mesenchymal tumor with some act in filaments suggestive of a rhabdomyosarcoma (Fig. I). Physical examination showed no further abnormalities. Standard blood tests were negative. Carcinoembryonic antigen, a-l -fetoprotein and p-human chorionic gonadotropin were normal. Chest X-ray: normal. Bone scan: hotspot left hip and right clavicular. CT-scan showed an enormous prostate with compression of the rectum (Fig. 2). The right seminal vesicle was enlarged. The patient was treated with adriamycin 60 mglm' and cisplatinum 100 mg/m' during 3 courses. Digital examination showed regression of the tumor. On CT-scan however, no regression was seen while multiple lung metastases had developed. Because the patient was unresponsive to the treatment the chemotherapy was changed in ifosfamide 6000 mg, vincristine 2 mg and actinomycin-D 1 mg. After 3 courses there was progression of tumor growth. After the treatment was stopped the patient succumbed one month later.
DISCUSSION Sarcomas of the Prostate are rare, constituting only 0.1 O/o to 0.2 O/o of all primary prostatic tu-
mors. They may be subclassified as rhabdomyosarcomas, leiom yosarcomas and fibrosarcomas. Rhabdomyosarcomas of the prostate frequently occur in children and young adults while leiom yosarcomas occur in older persons. The lung is the most common site of metastases of soft tissue sarcomas, as in the present case. Ahlering et at. (1) described two patients 17 and 27 years old with a rhabdomyosarcoma of the prostate. The first patient had a stage 111
Fig. 1. Electron microscopy of a mesenchymal tumor. The nucleus is in the lower right corner. The white stripes in the left corner abovethe nucleus are actine filaments suggestive for a rhabdomyosarcoma of the prostate. Scand J Urol Nephrol25
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Newcastle on 01/04/15 For personal use only.
330
S. D.Bos and E. Te Slaa
Fig. 2. CT-scan of the tumor in the prostate and the compression of the rectum. The dark arrow indicates the prostate and the open arrow the compressed rectum.
tumor and was treated with 4 courses of cisplatinum, etoposide, vincristine, doxorubicin and cyclophosphamide to which he was unresponsive and died 4 months later. The second patient who was treated with 4 courses of cisplatinum and doxorubicin followed by prostatectomy and external beam radiotherapy is free of
Scand J Urol Nephrol25
disease after 2 years. Ghavimi et al. (2) described 14 patients with a mean age of 9.5 years. All patients received multi drug chemotherapy (Dactinomycin, doxorubicin, vincristine and cyclophosphamide or combined with bleomycin and methotrexate), 1 1 radical cystoprostatectomy and 12 patients received external beam radiotherapy. Nine patients are still alive with a mean duration of survival of 74 months. Therapy is changing into the direction of multi drug chemotherapy without extensive operation. After reducing the tumor surgery and possible adjuvant radiotherapy is at this time the therapy of choice. During treatment the reaction of the tumor must be carefully monitored to obtain the best result for this very aggressive tumor. REFERENCES I . Ahlering TE, Weintraub P, Skinner DG. Management of adult sarcomas of the bladder and prostate. J Urol 1988; 140: 1397-1399. 2. Ghavimi F, Herr H, Jereb B, Exelby PR. Treatment of genito-urinary rhabdomyosarcomas in children. J Urol 1984; 132: 313-319.
