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Polypoid Fibromyxosarcoma of the Urinary Bladder in a Child
THE JOURNAL OF UROLOGY
Vol. 73, No. 2, February 1955 Printed in U.S.A.
POLYPOID FIBROMYXOSARCOMA OF THE URINARY BLADDER IN A CHILD R. H. FLOCKS
AND
D. A. CULP
From the Department of Urology, University Hospital, Iowa City, Iowa
Polypoid fibromyxosarcoma of the urinary bladder is a rare disease. The material to be presented has already been reported by Mostofi and Morse* in a group of 10 cases collected by the Army Institute of Pathology. However, the complete details were not presented, and since the total number of cases is extremely small, all the facts from each observed case should be recorded so that the life history and course of this disease can be understood. In addition, this patient has survived for 8 years and, as far as we can determine, is the only one to survive more than 24 months. There has been considerable confusion in the classification and terminology of this disease which stems from the fact that several types of cells may be present. Histologically, these tumors may be composed of loosely arranged, round cells; elongated cells with centrally placed nuclei, longitudinal fibrillae, and cross striations; and vacuolated, multinucleated giant cells. While all three of these cell types have been observed, they need not be present in all tumors. The neoplasm is thought to arise from embryonic tissue which is capable of differentiation into myxomatous, fibrous, or muscular cells and may account for the varied histological picture. Some of the tumors contain a much greater component of myxomatous elements than others, producing a polypoid gross appearance. Because of this histological confusion, the term "sarcoma botryoides" has been used to describe these tumors. Actually, this is a poor term since it only denotes a neoplasm arising from mesenchyme which has a grape-like appearance. Therefore, it may be best to simply classify these tumors by the type of cells present until further light has been spread upon their embryogenesis. CASE REPORT
J. S., No. 46-7796. On July 16, 1946, a 5-year-old white boy entered the Urology Department of University Hospitals with a 7-week history of straining on urination, hematuria, and passage of small pieces of tissue and clots. Four weeks prior to admission he was unable to void and was catheterized by the family physician. After that incident he had marked frequency and nocturia. Physical examination revealed a well-developed, well-nourished, white male child in no acute distress. Aside from a slight enlargement of a few cervical lymph nodes, the head and neck were normal. The lungs were clear to percussion and auscultation. There was a systolic murmur over the mitral and pulmonic areas. No masses or organs were palpable in the abdomen. Rectal examination revealed no abnormalities. The extremities were normal. At the time of admission the hemoglobin was 11.7 gm., a red blood count 4,100,000 and a white blood count 30,900. A urine analysis revealed a pH of
* Journal
of Urology 67: 681-687, 1952. 299
300
R. H. FLOCKS AND D. A. CULP
Fm. 1. Air cystogram in oblique position demonstrates filling defect arising from posterior inferior wall of bladder and protruding intravesically.
Fm. 2. Polypoid mass of intravesical neoplasm removed suprapubically
7.0, specific gravity of 1.015, negative albumin and sugar reaction with 40-50 red blood cells, and 4-5 white blood cells per high power microscopic field. The blood urea nitrogen was 14 mg. per cent and the creatinine 1.2 mg. per cent. Opaque and air cystograms revealed the bladder to be of normal size with a polypoid filling defect located inferiorly and posteriorly just to the left of the midline (fig. 1). A chest x-ray was normal. The day follo~g admission a cystoscopic examination under general anesthesia was performed, and the filling defect noted on the cystogram was identified
POLYPOID FIBROMYXOSARCOMA OF BLADDER
301
LF Fm. 3. A, photomicrograph of polypoid intravesical neoplasm. B, high power view exhibits cellular structure of polypoid intravesical neoplasm. ·
as a mass of polypoid neoplasm arising from the bladder neck. The remainder of the bladder appeared normal. On July 19, 1946, the bladder was exposed suprapubically; and, when it was opened, the neoplasm looked like a bunch of grapes lying in the bladder. The neoplasm was resected flush with the bladder; and the
302
R. H. FLOCKS AND D. A. CULP
base, which was located within the internal urethral orifice, was thoroughly coagulated with the high frequency current. The gross pathological specimen consisted of twenty grams of polypoid masses of soft, pale, transluscent tissue with many globular masses measuring 0.5 to 2 cc in diameter, covered with a smooth, glistening epithelium and filled with a pale, transluscent, jelly-like substance (fig. 2). The microscopic sections showed a highly vascular, loose connective tissue resembling embryonic mesenchyme with an investing layer of normal appearing transitional cell epithelium (fig. 3, A). Cellular elements consisted of thinly spaced cells with large, richly chromatic ovoid, spindle-shaped, or stellate nuclei. The cytoplasm was abundant, stained faintly basophilic, and formed a network of fibrillar intercellular processes (fig. 3, B). Mitotic figures were fairly numerous. In several areas there was hemorrhage, necrosis, and infiltration by chronic inflammatory cells. Clusters of very large, monocellular cells with vacuolated cytoplasm were seen in several areas. The pathological diagnosis was fibromyxosarcoma of the bladder. The child made an uneventful recovery and was discharged on August 17, 1946, with the suprapubic incision completely healed. Following discharge, the urethral stream gradually became smaller and difficult to start. Three weeks prior to re-admission he began complaining of severe suprapubic burning each time he voided. The family physician felt that bladder neck obstruction had recurred, and the incision in the bladder had re-opened. He made a small suprapubic skin incision, re-establishing the suprapubic sinus. On October 25, 1946, the patient was re-admitted to the hospital voiding partially
Fm. 4. A, sodium iodide cystogram showing negative filling defect in inferior portion of bladder representing regrowth of polypoid neoplasm. B, excretory urogram reveals mild bilateral hydro-ureters.
POLYPOID FIBROMYXOSARCOMA OF BLADDER
303
through the urethra, but leaking the major portion of urine through the suprapubic wound. A cystoscopic examination was performed through the suprapubic sinus, and recurrent neoplasm was found arising from the bladder neck, obstructing the internal urethral orifice. A repeat opaque cystogram revealed a filling defect in the inferior half of the urinary bladder which was irregular and ragged in outline (fig. 4, A). An intravenous urogram revealed a mild bilateral hydroureter (fig. 4, B). Because of the rapid growth of the neoplasm and recurrence of bladder neck obstruction, additional therapy was recommended. Since this type of neoplasm is radio-resistant, cystectomy and uretero-intestinal anastomosis were advised. On November 30, 1946, a bilateral uretero-intestinal anastomosis was performed. ·when the abdomen was opened, the bladder was noted to be three times normal size and full of tumor. A small extravesical nodule was noted and removed. The pathological report was chronic hyperplastic lymphadenitis with granulomatous changes. Both ureters were slightly dilated but otherwise appeared normaL A side-to-side anastomosis with an intact ureter was performed. Ureteral catheters were used as splints and allo-wed to exit through the anus. The child responded well postoperatively, and 10 days later the lower segment of the intact ureter was ligated. The patient continued to improve, and on December 27, 1946, a total cystectomy was performed. There was no evidence of local spread or metastases to the regional lymph nodes. The gross pathological specimen consisted of a bladder in which a large, polypoid, myxomatous tumor mass was growing out of the posterior wall and filling
Frn. 5. Gross specimen
304
R. H. FLOCKS AND D. A. CULP
the bladder lumen (fig. 5). The specimen weighed 120 gm. The tumor tissue was soft, friable, myxomatous, and marked by interstitial hemorrhage. On cut section the neoplastic tissue appeared to be invading the bladder wall, but the serosal surface was not involved by the neoplasm.
Fm. 6. A, photomicrograph of base of neoplasm shows involvement of tunica propria. B, prostatic urethra with extension of neoplasm into connective tissue of prostatic stroma.
POLYPOID FIBROMYXOSARCOMA OF BLADDER
305
The microscopic sections showed the neoplastic tissue to consist of a vascular, loose, connective tissue. The neoplastic cells were thinly spaced with large, hyperchromatic stellate or spindle-shaped nuclei. There was a moderate amount of cellular basophilic cytoplasm which formed a very light network of fibrillar intercellular processes. The polypoid processes ,vere covered by a layer of essentially normal-appearing transitional epithelium. The anaplastic cells were seen to be growing freely from the connective tissue of the bladder tunica propria (fig. 6, A). No invasion of the muscularis was noted. Sections of the prostatic urethra and prostate showed the neoplastic, myxomatous connective tissue1 to be continuous with the connective tissue of the prostatic stroma (fig. 6, B). The pathological diagnosis was fibromyxosarcoma of the bladder, prostatic urethra, and prostate. The child made an uneventful recovery and was discharged from the hospital on January 16, 1947. He was passing his urine freely through the rectum, and a postoperative intravenous urogram revealed a normal right pyelogram and mild hydronephrosis on the left (fig. 7). Throughout 1947 repeated examinations were performed in the out-patient department. The child gained weight, played vigorously, and had no complaints. Several chest x-rays were normal, and repeated intravenous urograms revealed mild left hydronephrosis. The last contact with the patient was on October 16, 1953, at which time he was 13 years of age, attending school regularly, and joining in normal activities. Sections of the tumor were examined by the Army Institute of Pathology, and
FIG. 7. Postoperative excretory urogram revealing mild left hydro-ureter and hydronephrosis.
306
R. H. FLOCKS AND D. A. CULP
their impression was: "Accession No. 235129: Your case has aroused considerable interest. The staff believes that the tumor is analogous to sarcoma botryoides in the vagina of children." CONCLUSION
The case history of a very interesting and rare bladder neoplasm found in a 5-year-old boy has been presented. The neoplasm arose from the region of the internal urethral orifice and was classified as a fibromyxosarcoma. Treatment consisted of suprapubic excisions and fulguration of the base of the neoplasm, but was rapidly followed by recurrence of the growth and bladder neck obstruction. The final definitive therapy consisted of uretero-intestinal anastomosis and total cystectomy. The child has now survived 8 years. REFERENCES CAPPELL, D. F. AND MONTGOMERY, G. L.: On rhabdomyoma and myoblastoma. J. Path. & Bact., 44: 517, 1937. DEMING, C. L.: Primary bladder tumors in the first decade of life. Surg., Gynec. & Obst., 39: 432-442, 1924. KHOURY, E. N. AND SPEER, F. D.: Rhabdomyosarcoma of the urinary bladder. J. Urol., 51: 505--516, 1944. MosTOFI, F. K. AND MORSE, W. H.: Polypoid rhabdomyosarcoma (sarcoma botyroides) of bladder in children. J. Urol., 67: 681-687, 1952.
Vol. 73, No. 2, February 1955 Printed in U.S.A.
POLYPOID FIBROMYXOSARCOMA OF THE URINARY BLADDER IN A CHILD R. H. FLOCKS
AND
D. A. CULP
From the Department of Urology, University Hospital, Iowa City, Iowa
Polypoid fibromyxosarcoma of the urinary bladder is a rare disease. The material to be presented has already been reported by Mostofi and Morse* in a group of 10 cases collected by the Army Institute of Pathology. However, the complete details were not presented, and since the total number of cases is extremely small, all the facts from each observed case should be recorded so that the life history and course of this disease can be understood. In addition, this patient has survived for 8 years and, as far as we can determine, is the only one to survive more than 24 months. There has been considerable confusion in the classification and terminology of this disease which stems from the fact that several types of cells may be present. Histologically, these tumors may be composed of loosely arranged, round cells; elongated cells with centrally placed nuclei, longitudinal fibrillae, and cross striations; and vacuolated, multinucleated giant cells. While all three of these cell types have been observed, they need not be present in all tumors. The neoplasm is thought to arise from embryonic tissue which is capable of differentiation into myxomatous, fibrous, or muscular cells and may account for the varied histological picture. Some of the tumors contain a much greater component of myxomatous elements than others, producing a polypoid gross appearance. Because of this histological confusion, the term "sarcoma botryoides" has been used to describe these tumors. Actually, this is a poor term since it only denotes a neoplasm arising from mesenchyme which has a grape-like appearance. Therefore, it may be best to simply classify these tumors by the type of cells present until further light has been spread upon their embryogenesis. CASE REPORT
J. S., No. 46-7796. On July 16, 1946, a 5-year-old white boy entered the Urology Department of University Hospitals with a 7-week history of straining on urination, hematuria, and passage of small pieces of tissue and clots. Four weeks prior to admission he was unable to void and was catheterized by the family physician. After that incident he had marked frequency and nocturia. Physical examination revealed a well-developed, well-nourished, white male child in no acute distress. Aside from a slight enlargement of a few cervical lymph nodes, the head and neck were normal. The lungs were clear to percussion and auscultation. There was a systolic murmur over the mitral and pulmonic areas. No masses or organs were palpable in the abdomen. Rectal examination revealed no abnormalities. The extremities were normal. At the time of admission the hemoglobin was 11.7 gm., a red blood count 4,100,000 and a white blood count 30,900. A urine analysis revealed a pH of
* Journal
of Urology 67: 681-687, 1952. 299
300
R. H. FLOCKS AND D. A. CULP
Fm. 1. Air cystogram in oblique position demonstrates filling defect arising from posterior inferior wall of bladder and protruding intravesically.
Fm. 2. Polypoid mass of intravesical neoplasm removed suprapubically
7.0, specific gravity of 1.015, negative albumin and sugar reaction with 40-50 red blood cells, and 4-5 white blood cells per high power microscopic field. The blood urea nitrogen was 14 mg. per cent and the creatinine 1.2 mg. per cent. Opaque and air cystograms revealed the bladder to be of normal size with a polypoid filling defect located inferiorly and posteriorly just to the left of the midline (fig. 1). A chest x-ray was normal. The day follo~g admission a cystoscopic examination under general anesthesia was performed, and the filling defect noted on the cystogram was identified
POLYPOID FIBROMYXOSARCOMA OF BLADDER
301
LF Fm. 3. A, photomicrograph of polypoid intravesical neoplasm. B, high power view exhibits cellular structure of polypoid intravesical neoplasm. ·
as a mass of polypoid neoplasm arising from the bladder neck. The remainder of the bladder appeared normal. On July 19, 1946, the bladder was exposed suprapubically; and, when it was opened, the neoplasm looked like a bunch of grapes lying in the bladder. The neoplasm was resected flush with the bladder; and the
302
R. H. FLOCKS AND D. A. CULP
base, which was located within the internal urethral orifice, was thoroughly coagulated with the high frequency current. The gross pathological specimen consisted of twenty grams of polypoid masses of soft, pale, transluscent tissue with many globular masses measuring 0.5 to 2 cc in diameter, covered with a smooth, glistening epithelium and filled with a pale, transluscent, jelly-like substance (fig. 2). The microscopic sections showed a highly vascular, loose connective tissue resembling embryonic mesenchyme with an investing layer of normal appearing transitional cell epithelium (fig. 3, A). Cellular elements consisted of thinly spaced cells with large, richly chromatic ovoid, spindle-shaped, or stellate nuclei. The cytoplasm was abundant, stained faintly basophilic, and formed a network of fibrillar intercellular processes (fig. 3, B). Mitotic figures were fairly numerous. In several areas there was hemorrhage, necrosis, and infiltration by chronic inflammatory cells. Clusters of very large, monocellular cells with vacuolated cytoplasm were seen in several areas. The pathological diagnosis was fibromyxosarcoma of the bladder. The child made an uneventful recovery and was discharged on August 17, 1946, with the suprapubic incision completely healed. Following discharge, the urethral stream gradually became smaller and difficult to start. Three weeks prior to re-admission he began complaining of severe suprapubic burning each time he voided. The family physician felt that bladder neck obstruction had recurred, and the incision in the bladder had re-opened. He made a small suprapubic skin incision, re-establishing the suprapubic sinus. On October 25, 1946, the patient was re-admitted to the hospital voiding partially
Fm. 4. A, sodium iodide cystogram showing negative filling defect in inferior portion of bladder representing regrowth of polypoid neoplasm. B, excretory urogram reveals mild bilateral hydro-ureters.
POLYPOID FIBROMYXOSARCOMA OF BLADDER
303
through the urethra, but leaking the major portion of urine through the suprapubic wound. A cystoscopic examination was performed through the suprapubic sinus, and recurrent neoplasm was found arising from the bladder neck, obstructing the internal urethral orifice. A repeat opaque cystogram revealed a filling defect in the inferior half of the urinary bladder which was irregular and ragged in outline (fig. 4, A). An intravenous urogram revealed a mild bilateral hydroureter (fig. 4, B). Because of the rapid growth of the neoplasm and recurrence of bladder neck obstruction, additional therapy was recommended. Since this type of neoplasm is radio-resistant, cystectomy and uretero-intestinal anastomosis were advised. On November 30, 1946, a bilateral uretero-intestinal anastomosis was performed. ·when the abdomen was opened, the bladder was noted to be three times normal size and full of tumor. A small extravesical nodule was noted and removed. The pathological report was chronic hyperplastic lymphadenitis with granulomatous changes. Both ureters were slightly dilated but otherwise appeared normaL A side-to-side anastomosis with an intact ureter was performed. Ureteral catheters were used as splints and allo-wed to exit through the anus. The child responded well postoperatively, and 10 days later the lower segment of the intact ureter was ligated. The patient continued to improve, and on December 27, 1946, a total cystectomy was performed. There was no evidence of local spread or metastases to the regional lymph nodes. The gross pathological specimen consisted of a bladder in which a large, polypoid, myxomatous tumor mass was growing out of the posterior wall and filling
Frn. 5. Gross specimen
304
R. H. FLOCKS AND D. A. CULP
the bladder lumen (fig. 5). The specimen weighed 120 gm. The tumor tissue was soft, friable, myxomatous, and marked by interstitial hemorrhage. On cut section the neoplastic tissue appeared to be invading the bladder wall, but the serosal surface was not involved by the neoplasm.
Fm. 6. A, photomicrograph of base of neoplasm shows involvement of tunica propria. B, prostatic urethra with extension of neoplasm into connective tissue of prostatic stroma.
POLYPOID FIBROMYXOSARCOMA OF BLADDER
305
The microscopic sections showed the neoplastic tissue to consist of a vascular, loose, connective tissue. The neoplastic cells were thinly spaced with large, hyperchromatic stellate or spindle-shaped nuclei. There was a moderate amount of cellular basophilic cytoplasm which formed a very light network of fibrillar intercellular processes. The polypoid processes ,vere covered by a layer of essentially normal-appearing transitional epithelium. The anaplastic cells were seen to be growing freely from the connective tissue of the bladder tunica propria (fig. 6, A). No invasion of the muscularis was noted. Sections of the prostatic urethra and prostate showed the neoplastic, myxomatous connective tissue1 to be continuous with the connective tissue of the prostatic stroma (fig. 6, B). The pathological diagnosis was fibromyxosarcoma of the bladder, prostatic urethra, and prostate. The child made an uneventful recovery and was discharged from the hospital on January 16, 1947. He was passing his urine freely through the rectum, and a postoperative intravenous urogram revealed a normal right pyelogram and mild hydronephrosis on the left (fig. 7). Throughout 1947 repeated examinations were performed in the out-patient department. The child gained weight, played vigorously, and had no complaints. Several chest x-rays were normal, and repeated intravenous urograms revealed mild left hydronephrosis. The last contact with the patient was on October 16, 1953, at which time he was 13 years of age, attending school regularly, and joining in normal activities. Sections of the tumor were examined by the Army Institute of Pathology, and
FIG. 7. Postoperative excretory urogram revealing mild left hydro-ureter and hydronephrosis.
306
R. H. FLOCKS AND D. A. CULP
their impression was: "Accession No. 235129: Your case has aroused considerable interest. The staff believes that the tumor is analogous to sarcoma botryoides in the vagina of children." CONCLUSION
The case history of a very interesting and rare bladder neoplasm found in a 5-year-old boy has been presented. The neoplasm arose from the region of the internal urethral orifice and was classified as a fibromyxosarcoma. Treatment consisted of suprapubic excisions and fulguration of the base of the neoplasm, but was rapidly followed by recurrence of the growth and bladder neck obstruction. The final definitive therapy consisted of uretero-intestinal anastomosis and total cystectomy. The child has now survived 8 years. REFERENCES CAPPELL, D. F. AND MONTGOMERY, G. L.: On rhabdomyoma and myoblastoma. J. Path. & Bact., 44: 517, 1937. DEMING, C. L.: Primary bladder tumors in the first decade of life. Surg., Gynec. & Obst., 39: 432-442, 1924. KHOURY, E. N. AND SPEER, F. D.: Rhabdomyosarcoma of the urinary bladder. J. Urol., 51: 505--516, 1944. MosTOFI, F. K. AND MORSE, W. H.: Polypoid rhabdomyosarcoma (sarcoma botyroides) of bladder in children. J. Urol., 67: 681-687, 1952.