CHANGING
CONCEPTS IN MANAGEMENT
PELVIC RHABDOMYOSARCOMA THOMAS
H. BARTHOLOMEW,
EDMOND
T. GONZALES,
KENNETH
A. STARLING,
F. JAMES
HARBERG,
OF
IN CHILDREN
M.D.
JR., M.D. M.D.
M.D.
From the Sections of Pediatric Urology, Pediatric Hematology, and Pediatric Surgery, Texas Children’s Hospital, Houston, Texas
- Survival with embryonal rhabdomyosarcoma of all sites has improved dramatically in recent years with the increased use of long-term, cyclic, multidrug chemotherapy. Protocols have been established and are currently being evaluated by the Intergroup Rhabdomyosarcoma Study. The management of embryonal rhabdomyosarcoma of the pelvic viscera, though, remains troublesome. Limited surgical excision is rarely possible and high-dose radiotherapy to the bony pelvis may cause severe and disabling growth disorders. Yet, survival with these lesions is increasing as with rhabdomyosarcoma from all sites and is directly related to a well-planned and aggressive multidisciplinary program. We have seen 12 cases of pelvic rhabdomyosarcoma within the last seventeen years at this institution. These cases will be reviewed in regard to varying modes of therapy and survival. Our current therapeutic approach, based on national and local experience, will be presented.
ABSTRACT
the most common soft Rhabdomyosarcoma, tissue sarcoma of childhood, ranks seventh in causes of cancer deaths in children.’ Nearly 20 per cent of these tumors arise from the pelvic viscera, primarily the bladder, vagina, and prostate.’ Rhabdomyosarcomas arise from embryonic mesenchyme, probably from cells of the urogenital sinus.3 Histologic characteristics allow subdivision of rhabdomyosarcoma into three major categories. The most common among young children is the embryonal type. The alveolar type is more commonly seen in adolescents. Pleomorphic rhabdomyosarcomas are least common in children and are regarded as typical in adults. Botryoid tumors, once considered as a separate histologic category, are actually named for their gross appearance and usually have embryonal cellular appearance on microscopic examination. Growth patterns of rhabdomyosarcomas are somewhat predictable. Lesions of the bladder and vagina tend to spread beneath the mucosa and present with
UROLOGY
/ JUNE1979 / VOLUMEXIII, NUMBER6
clusters of intraluminal masses. Primary prostatic lesions spread directly along fascial planes and disseminate via lymphatic and hematogenous routes. Until 1967, results of surgery and radiotherapy for rhabdomyosarcoma were poor. In the last decade the addition of multidrug cyclic chemotherapy has prolonged disease-free intervals and increased survival rates. We have utilized this therapy in 6 patients in the past four years, with improvement in patient survival compared with that achieved during the prior twelve years at our institution. The purpose of TABLE I. Age of patients at diagnosis
Age at Diagnosis (yr.)
Male
Female
-Cl 1-3 3-5 5-8
3 2 0 3
1 2 0 1
613
Summary of clinical features in 12 patients
TABLE II. Age and Date at Sex Diagnosis
Site
Stage
Chemotherapy*
Radiotherapy (Rads)
Surgery
Status (mo.)t
Complications
1,200 postop.
83 mo.
Pelvic exenteration, ileal loop
2,000 postop.
24 mo. DOD
None
AV post
Laparotomy, resection
incomplete
3,000 postop.
149 mo. NED
None
III
AVC post
Anterior exenteration, incomplete resection, ureterosigmoiclostomy
3,000 postop.
13 mo. DOD
None
Prostate
III
AC
Laparotomy, resection
incomplete
2,000 postop.
11 mo. DOD
None
6 yr. 7172
Pelvis
III
v post
Laparotomy, resection
incomplete
600 postop.
1 mo. DOD
None
M
3 yr. 1 l/74
Prostate
IIC
AVC post
Anterior exenteration, ileal loop
5,200 postop.
39 mo. NED
R hydronephrosis; rectal fibrosis
M
7 yr. 6175
Prostate
IIC
AVC post
Anterior exenteration, sigmoicl loop
4,500 postop.
34 mo. NED
None
F
8 yr. 315176
Bladder
IIC
AVCD
Anterior exenteration, sigmoid loop
3,ooo postop.
24 mo. NED
None
M
NB l/77
Bladder
III
AVC pre
Anterior exenteration, cutaneous ureterostomies
3,000 postop.
15 mo. AWD
None
F
2% yr. 3177
Bladder
I
AVCD
Anterior exenteration, sigmoid loop
None
12 mo. NED
None
1 yr. 10177
Pelvis
Laparotomy, resection
None
5 mo. AWD
None
M
6 yr. 4.l62
Bladder
F
11 mo. lo/64
Vagina
IIC
AV post
M
2 yr. 6165
Bulbous urethra
III
M
2 mo. 12167
Prostate
M
4 mo. 3168
M
F
III
A post
pre
p re III
AVC post
Laparotomy, resection
incomplete
incomplete
None
NED
*Pre- or postoperative administration of A = actinomycin-D; V = vincristine; C = cyclophosphamide; D = doxorubicin tDOD = dead of disease; NED = no evidence of disease; AWD = alive with disease.
this report is to review management of embryonal of the pelvis. Material
and
our experience with rhabdomyosarcoma
Methods
Twelve patients ranging from newborn to eight years of age with pelvic rhabdomyosarcoma were treated at Texas Children’s Hospital since 1962: 8 were males and 4 females (Table I). Table II depicts details of therapy and sur-
involvement of contiguous structures. Those patients who manifested extensive tumor replacement of the prostate were considered to have primary prostate tumors. If a typical botryoid intravesical pattern was present, the tumor was considered to have a bladder origin. We have included 2 patients in whom specific site of origin is uncertain as the tumor arose from the retrovesical space. These tumors were of a nonspecific pelvis origin.
vival in these patients. Site of Origin Primary pelvic rhabdomyosarcoma frequently spreads into adjacent organs. In a significant number of patients, accurate assessment of actual site of origin is impossible because of this
614
Staging and Management Preoperative studies as well as surgical findings determined final staging (Table III). Only 1 child, a two and one-half-year-old female with a primary vesical lesion, had Stage I disease. The remaining 11 patients all had residual disease
UROLOGY
/
JUNE 1979
/
VOLUME XIII,
NUMBERG
TABLE III.
Staging as adopted by the Intergroup Rhabdomyosarcoma Study
tomy, and regional lymphadenectomy. Cystectomy, urethrectomy, anterior vaginectomy, and hysterectomy were done in females. In 1 case, total pelvic exenteration was necessary. Though procedures have varied, in general they have involved as near total resection as possible of gross tumor and regional lymphadenectomy. In 4 patients tumor extent was beyond the scope of complete resection, so incomplete resection only was done. All of these 4 patients were seen prior to 1972 when intensive cyclic chemotherapy was initiated. External beam radiotherapy was used if microscopic or gross residual disease was present.
Criterion for Staging
Stage I II
Localized disease, completely resected Localized disease, microscopic residual A. Grossly resected tumor with microscopic residual, no nodal involvement B. Regional nodal disease, completely resected, no microscopic residual C. Regional nodal disease, grossly resected but with evidence of microscopic residual Incomplete resection or biopsy with gross residual regional disease Distant metastases present at time of diagnosis4
III IV
Results All of the 6 patients treated after 1971 are alive. Four are alive with no evidence of disease with average follow-up of twenty-seven months, despite microscopic residual remaining after surgery in 3 of these patients. Two patients in that group are alive with disease. One child has had stable disease for fifteen months, and the residual disease has shown no evidence of progression. In the second child the huge pelvic tumor arising between the vagina and bladder was believed to be nonresectable at first exploration. Although the mass regressed initially on chemotherapy alone, this patient recently has shown rapid progression of her disease. Four of the 6 patients treated before 1972 are dead of their disease, with median survival of thirteen months. The 2 remaining in that group are alive with no evidence of disease with average follow-up of greater than ten years (Fig. 1). We noted no major toxicity from chemotherapy. In 1 patient mild rectal stenosis developed after radiotherapy. One of 5 children with urinary diversion demonstrated mild unilateral hydronephrosis. No other surgical complications occurred.
following surgery, 4 with microscopic residual (Stage II) and 7 with gross residual (Stage III). Before 1972 all patients underwent surgery and then received postoperative chemotherapy. Agents and treatment protocols varied with prevalent therapeutic programs. After 1972, an intensive chemotherapeutic program followed surgery. This consisted initially of actinomycin-D (Dactinomycin), vincristine sulfate (Oncovin), and cyclophosphamide (Cytoxan); for the past two years doxorubicin hydrochloride (Adriamycin) has also been included. The chemotherapeutic schedule now in use at this institution is shown in Table IV. Hematologic evidence of toxicity made occasional modifications of treatment necessary. Since 1976, 4 patients have had biopsies only, and then presurgical initiation of four drug cyclic chemotherapy. Surgery has been completed in 3 of this group. Tumor regression has been seen in all 4 in intervals ranging from two to five months, although in none has chemotherapy only eradicated the disease. The standard surgical procedure in males included’ cystoprostatectomy, limited urethrec-
TABLE IV.
Chemotherapy
schedule (cycled ez;ery three months) Weeks
I
Drug*
0
Actinomycin-D Vindristine Doxorubicin Cyclophosphamide
xxxx
1
2
3
xxxxxxx
xxxxxxx
/
JUNE
1979
/
VOLUME
XIII,
NUMBER
5
6
X
X
X
xxxxxxx
*Actinomycin-D: 75 pg./Kg. in four divided daily doses; vincristine: twenty-four hours after vincristine; cyclophosphamide: 150 mg.lMTday
UROLOGY
4
6
xxxxxxx
1.5 mg./M2 by mouth.
xxxxxxx
maximum
7
8
9 1011
X X xxxxxxx
1.5 mg.; doxorubicin:
60 mg./W
given
615
I
146
146
ALIVE ALIVE
J 2
WITH
NO
WITH
EVIDENCE DEAD
00
OF OF
DISEASE
m
DISEASE
0
DISEASE
m
> 2
60
FIGURE 1. Surviual time and status of children with pelvic rhabdomyosarcoma.
0” 4 I-
40
% 20
2
: 62
64
65
67
72
68 YEAR
OF
74
75
Comment Prior to 1966, use of chemotherapy in thic disease was limited to individual agents in treatment of metastases. Survival rates from 9 to 35 per cent with surgery or radiation alone were reported.5-8 Metastatic disease responded to chemotherapy with single agents in up to 83 per cent of cases, but responses were of short duration. In 1966, James et ~1.’ reported improved objective response in one third of patients treated with a combination of actinomycin-D and vincristine. In 1969, Grosfeld, Clatworthy, and NewtonlO also combined these agents, but used them in repeated courses with prolonged survival in 12 of 18 patients. In 1974, the Children’s Cancer Study Group reported responses in a prospective randomized controlled study with 86 per cent of 28 patients found diseasefree at two years when treated with surgery and combined chemotherapy. i1 Rivard et al. l2 were able to demonstrate a 4.5-fold improvement in survival among 9 patients treated with intensive preoperative chemotherapy. Our enthusiasm for this approach arose from these demonstrations of strikingly effective therapy in a disease process that previously meant extensive surgery and a dismal prognosis. This review demonstrates that 11 of 12 patients had residual tumor after surgery. The addition of cyclic multidrug chemotherapy has improved survival in this group. Both Rivard et al. l2 and Belman13 point out the effect of chemotherapy on this residual disease and suggest that extensive surgery need not be the first modality of therapy in pelvic rhabdomyosarcoma. Preoperative chemotherapy may reduce tumor bulk within the confines of the bony pelvis and thus reduce the scope of or need for
616
76
77
77
77
DIAGNOSIS
subsequent surgery. We believe that preoperative chemotherapy has caused tumor regression in 4 patients so treated and thus allowed more complete surgical removal of gross tumor. Chemotherapeutic control of microscopic residual adds further to an improved outlook for survival. Texas Children’s Hospital
6621 Fannin Street Houston, Texas 77030 (DR. GONZALES)
References 1. Miller RW: Fifty-two forms of childhood cancer: United States mortality experience, 1969-1966, J. Pediat. 75: 685 (1969). 2. Femandez CH, Sutow WW, Merino OR, and George SL: Childhood rhabdomyosarcoma, analysis of coordinated therapy and results, A. J. R. 123: 588 (1975). 3. Bat&s JG: Urogenital rhabdomyosarcoma: histogenesis and classification, J. Urol. 99: 186 (1963). 4. Heyn RM: The role of chemotherapy in the management of soft tissue sarcomas, Cancer 35: 921 (1975). 5. Steinberg J, Haddy TB, Porter FS, and Thurman WG: Clinical triab with cyclophosphamide in children with soft tissue sarcoma, Cancer Chemother. Rep. 28: 39 (1963). 6. Sutow WW: Chemotherapy in childhood cancer (except leukemia), Cancer 18: 1585 (1965). 7. Sutow WW, et al: Vincristine sulfate therapy in children with metastatic soil tissue sarcoma, Pediatrics 28: 465 (1966). 8. Tan CTC, et aE: Clinical experience with actinomycin-D, Ann. N.Y. Acad. Sci. 39: 426 (1966). 9. James DH Jr, Hustu 0, Wrenn EL, and Johnson WW: Childhood malignant tumors - concurrent chemotherapy with Dactinomycin and vincristine sulfate, J.A.M.A. 197: lO43-(1966). 10. Grosfeld IL. Clatworthv HW Ir. and Newton WA Ir: Combined therapy d childhood rhabdomybsarcoma, J. Pediatr. Surg. 4: 637 (1969). 11. Heyn RM, et al: The role of combined chemotherapy in the treatment of rhabdomyosarcoma in children, Cancer 34: 2128 (1974). 12. Rivard G, et al: Intensive chemotherapy as primary treatment for rhabdomyosarcoma of the pelvis, ibid. 35: 1593 (1975). 13. Belman AB, and Baum ES: Current trends in treatment of childhood rhabdomyosarcoma of lower genitourinary tract, Urology 8: 31 (1976).
UROLOGY
/
JUNE 1979
i
VOLUME XIII,
NUMBER 6
CONCEPTS IN MANAGEMENT
PELVIC RHABDOMYOSARCOMA THOMAS
H. BARTHOLOMEW,
EDMOND
T. GONZALES,
KENNETH
A. STARLING,
F. JAMES
HARBERG,
OF
IN CHILDREN
M.D.
JR., M.D. M.D.
M.D.
From the Sections of Pediatric Urology, Pediatric Hematology, and Pediatric Surgery, Texas Children’s Hospital, Houston, Texas
- Survival with embryonal rhabdomyosarcoma of all sites has improved dramatically in recent years with the increased use of long-term, cyclic, multidrug chemotherapy. Protocols have been established and are currently being evaluated by the Intergroup Rhabdomyosarcoma Study. The management of embryonal rhabdomyosarcoma of the pelvic viscera, though, remains troublesome. Limited surgical excision is rarely possible and high-dose radiotherapy to the bony pelvis may cause severe and disabling growth disorders. Yet, survival with these lesions is increasing as with rhabdomyosarcoma from all sites and is directly related to a well-planned and aggressive multidisciplinary program. We have seen 12 cases of pelvic rhabdomyosarcoma within the last seventeen years at this institution. These cases will be reviewed in regard to varying modes of therapy and survival. Our current therapeutic approach, based on national and local experience, will be presented.
ABSTRACT
the most common soft Rhabdomyosarcoma, tissue sarcoma of childhood, ranks seventh in causes of cancer deaths in children.’ Nearly 20 per cent of these tumors arise from the pelvic viscera, primarily the bladder, vagina, and prostate.’ Rhabdomyosarcomas arise from embryonic mesenchyme, probably from cells of the urogenital sinus.3 Histologic characteristics allow subdivision of rhabdomyosarcoma into three major categories. The most common among young children is the embryonal type. The alveolar type is more commonly seen in adolescents. Pleomorphic rhabdomyosarcomas are least common in children and are regarded as typical in adults. Botryoid tumors, once considered as a separate histologic category, are actually named for their gross appearance and usually have embryonal cellular appearance on microscopic examination. Growth patterns of rhabdomyosarcomas are somewhat predictable. Lesions of the bladder and vagina tend to spread beneath the mucosa and present with
UROLOGY
/ JUNE1979 / VOLUMEXIII, NUMBER6
clusters of intraluminal masses. Primary prostatic lesions spread directly along fascial planes and disseminate via lymphatic and hematogenous routes. Until 1967, results of surgery and radiotherapy for rhabdomyosarcoma were poor. In the last decade the addition of multidrug cyclic chemotherapy has prolonged disease-free intervals and increased survival rates. We have utilized this therapy in 6 patients in the past four years, with improvement in patient survival compared with that achieved during the prior twelve years at our institution. The purpose of TABLE I. Age of patients at diagnosis
Age at Diagnosis (yr.)
Male
Female
-Cl 1-3 3-5 5-8
3 2 0 3
1 2 0 1
613
Summary of clinical features in 12 patients
TABLE II. Age and Date at Sex Diagnosis
Site
Stage
Chemotherapy*
Radiotherapy (Rads)
Surgery
Status (mo.)t
Complications
1,200 postop.
83 mo.
Pelvic exenteration, ileal loop
2,000 postop.
24 mo. DOD
None
AV post
Laparotomy, resection
incomplete
3,000 postop.
149 mo. NED
None
III
AVC post
Anterior exenteration, incomplete resection, ureterosigmoiclostomy
3,000 postop.
13 mo. DOD
None
Prostate
III
AC
Laparotomy, resection
incomplete
2,000 postop.
11 mo. DOD
None
6 yr. 7172
Pelvis
III
v post
Laparotomy, resection
incomplete
600 postop.
1 mo. DOD
None
M
3 yr. 1 l/74
Prostate
IIC
AVC post
Anterior exenteration, ileal loop
5,200 postop.
39 mo. NED
R hydronephrosis; rectal fibrosis
M
7 yr. 6175
Prostate
IIC
AVC post
Anterior exenteration, sigmoicl loop
4,500 postop.
34 mo. NED
None
F
8 yr. 315176
Bladder
IIC
AVCD
Anterior exenteration, sigmoid loop
3,ooo postop.
24 mo. NED
None
M
NB l/77
Bladder
III
AVC pre
Anterior exenteration, cutaneous ureterostomies
3,000 postop.
15 mo. AWD
None
F
2% yr. 3177
Bladder
I
AVCD
Anterior exenteration, sigmoid loop
None
12 mo. NED
None
1 yr. 10177
Pelvis
Laparotomy, resection
None
5 mo. AWD
None
M
6 yr. 4.l62
Bladder
F
11 mo. lo/64
Vagina
IIC
AV post
M
2 yr. 6165
Bulbous urethra
III
M
2 mo. 12167
Prostate
M
4 mo. 3168
M
F
III
A post
pre
p re III
AVC post
Laparotomy, resection
incomplete
incomplete
None
NED
*Pre- or postoperative administration of A = actinomycin-D; V = vincristine; C = cyclophosphamide; D = doxorubicin tDOD = dead of disease; NED = no evidence of disease; AWD = alive with disease.
this report is to review management of embryonal of the pelvis. Material
and
our experience with rhabdomyosarcoma
Methods
Twelve patients ranging from newborn to eight years of age with pelvic rhabdomyosarcoma were treated at Texas Children’s Hospital since 1962: 8 were males and 4 females (Table I). Table II depicts details of therapy and sur-
involvement of contiguous structures. Those patients who manifested extensive tumor replacement of the prostate were considered to have primary prostate tumors. If a typical botryoid intravesical pattern was present, the tumor was considered to have a bladder origin. We have included 2 patients in whom specific site of origin is uncertain as the tumor arose from the retrovesical space. These tumors were of a nonspecific pelvis origin.
vival in these patients. Site of Origin Primary pelvic rhabdomyosarcoma frequently spreads into adjacent organs. In a significant number of patients, accurate assessment of actual site of origin is impossible because of this
614
Staging and Management Preoperative studies as well as surgical findings determined final staging (Table III). Only 1 child, a two and one-half-year-old female with a primary vesical lesion, had Stage I disease. The remaining 11 patients all had residual disease
UROLOGY
/
JUNE 1979
/
VOLUME XIII,
NUMBERG
TABLE III.
Staging as adopted by the Intergroup Rhabdomyosarcoma Study
tomy, and regional lymphadenectomy. Cystectomy, urethrectomy, anterior vaginectomy, and hysterectomy were done in females. In 1 case, total pelvic exenteration was necessary. Though procedures have varied, in general they have involved as near total resection as possible of gross tumor and regional lymphadenectomy. In 4 patients tumor extent was beyond the scope of complete resection, so incomplete resection only was done. All of these 4 patients were seen prior to 1972 when intensive cyclic chemotherapy was initiated. External beam radiotherapy was used if microscopic or gross residual disease was present.
Criterion for Staging
Stage I II
Localized disease, completely resected Localized disease, microscopic residual A. Grossly resected tumor with microscopic residual, no nodal involvement B. Regional nodal disease, completely resected, no microscopic residual C. Regional nodal disease, grossly resected but with evidence of microscopic residual Incomplete resection or biopsy with gross residual regional disease Distant metastases present at time of diagnosis4
III IV
Results All of the 6 patients treated after 1971 are alive. Four are alive with no evidence of disease with average follow-up of twenty-seven months, despite microscopic residual remaining after surgery in 3 of these patients. Two patients in that group are alive with disease. One child has had stable disease for fifteen months, and the residual disease has shown no evidence of progression. In the second child the huge pelvic tumor arising between the vagina and bladder was believed to be nonresectable at first exploration. Although the mass regressed initially on chemotherapy alone, this patient recently has shown rapid progression of her disease. Four of the 6 patients treated before 1972 are dead of their disease, with median survival of thirteen months. The 2 remaining in that group are alive with no evidence of disease with average follow-up of greater than ten years (Fig. 1). We noted no major toxicity from chemotherapy. In 1 patient mild rectal stenosis developed after radiotherapy. One of 5 children with urinary diversion demonstrated mild unilateral hydronephrosis. No other surgical complications occurred.
following surgery, 4 with microscopic residual (Stage II) and 7 with gross residual (Stage III). Before 1972 all patients underwent surgery and then received postoperative chemotherapy. Agents and treatment protocols varied with prevalent therapeutic programs. After 1972, an intensive chemotherapeutic program followed surgery. This consisted initially of actinomycin-D (Dactinomycin), vincristine sulfate (Oncovin), and cyclophosphamide (Cytoxan); for the past two years doxorubicin hydrochloride (Adriamycin) has also been included. The chemotherapeutic schedule now in use at this institution is shown in Table IV. Hematologic evidence of toxicity made occasional modifications of treatment necessary. Since 1976, 4 patients have had biopsies only, and then presurgical initiation of four drug cyclic chemotherapy. Surgery has been completed in 3 of this group. Tumor regression has been seen in all 4 in intervals ranging from two to five months, although in none has chemotherapy only eradicated the disease. The standard surgical procedure in males included’ cystoprostatectomy, limited urethrec-
TABLE IV.
Chemotherapy
schedule (cycled ez;ery three months) Weeks
I
Drug*
0
Actinomycin-D Vindristine Doxorubicin Cyclophosphamide
xxxx
1
2
3
xxxxxxx
xxxxxxx
/
JUNE
1979
/
VOLUME
XIII,
NUMBER
5
6
X
X
X
xxxxxxx
*Actinomycin-D: 75 pg./Kg. in four divided daily doses; vincristine: twenty-four hours after vincristine; cyclophosphamide: 150 mg.lMTday
UROLOGY
4
6
xxxxxxx
1.5 mg./M2 by mouth.
xxxxxxx
maximum
7
8
9 1011
X X xxxxxxx
1.5 mg.; doxorubicin:
60 mg./W
given
615
I
146
146
ALIVE ALIVE
J 2
WITH
NO
WITH
EVIDENCE DEAD
00
OF OF
DISEASE
m
DISEASE
0
DISEASE
m
> 2
60
FIGURE 1. Surviual time and status of children with pelvic rhabdomyosarcoma.
0” 4 I-
40
% 20
2
: 62
64
65
67
72
68 YEAR
OF
74
75
Comment Prior to 1966, use of chemotherapy in thic disease was limited to individual agents in treatment of metastases. Survival rates from 9 to 35 per cent with surgery or radiation alone were reported.5-8 Metastatic disease responded to chemotherapy with single agents in up to 83 per cent of cases, but responses were of short duration. In 1966, James et ~1.’ reported improved objective response in one third of patients treated with a combination of actinomycin-D and vincristine. In 1969, Grosfeld, Clatworthy, and NewtonlO also combined these agents, but used them in repeated courses with prolonged survival in 12 of 18 patients. In 1974, the Children’s Cancer Study Group reported responses in a prospective randomized controlled study with 86 per cent of 28 patients found diseasefree at two years when treated with surgery and combined chemotherapy. i1 Rivard et al. l2 were able to demonstrate a 4.5-fold improvement in survival among 9 patients treated with intensive preoperative chemotherapy. Our enthusiasm for this approach arose from these demonstrations of strikingly effective therapy in a disease process that previously meant extensive surgery and a dismal prognosis. This review demonstrates that 11 of 12 patients had residual tumor after surgery. The addition of cyclic multidrug chemotherapy has improved survival in this group. Both Rivard et al. l2 and Belman13 point out the effect of chemotherapy on this residual disease and suggest that extensive surgery need not be the first modality of therapy in pelvic rhabdomyosarcoma. Preoperative chemotherapy may reduce tumor bulk within the confines of the bony pelvis and thus reduce the scope of or need for
616
76
77
77
77
DIAGNOSIS
subsequent surgery. We believe that preoperative chemotherapy has caused tumor regression in 4 patients so treated and thus allowed more complete surgical removal of gross tumor. Chemotherapeutic control of microscopic residual adds further to an improved outlook for survival. Texas Children’s Hospital
6621 Fannin Street Houston, Texas 77030 (DR. GONZALES)
References 1. Miller RW: Fifty-two forms of childhood cancer: United States mortality experience, 1969-1966, J. Pediat. 75: 685 (1969). 2. Femandez CH, Sutow WW, Merino OR, and George SL: Childhood rhabdomyosarcoma, analysis of coordinated therapy and results, A. J. R. 123: 588 (1975). 3. Bat&s JG: Urogenital rhabdomyosarcoma: histogenesis and classification, J. Urol. 99: 186 (1963). 4. Heyn RM: The role of chemotherapy in the management of soft tissue sarcomas, Cancer 35: 921 (1975). 5. Steinberg J, Haddy TB, Porter FS, and Thurman WG: Clinical triab with cyclophosphamide in children with soft tissue sarcoma, Cancer Chemother. Rep. 28: 39 (1963). 6. Sutow WW: Chemotherapy in childhood cancer (except leukemia), Cancer 18: 1585 (1965). 7. Sutow WW, et al: Vincristine sulfate therapy in children with metastatic soil tissue sarcoma, Pediatrics 28: 465 (1966). 8. Tan CTC, et aE: Clinical experience with actinomycin-D, Ann. N.Y. Acad. Sci. 39: 426 (1966). 9. James DH Jr, Hustu 0, Wrenn EL, and Johnson WW: Childhood malignant tumors - concurrent chemotherapy with Dactinomycin and vincristine sulfate, J.A.M.A. 197: lO43-(1966). 10. Grosfeld IL. Clatworthv HW Ir. and Newton WA Ir: Combined therapy d childhood rhabdomybsarcoma, J. Pediatr. Surg. 4: 637 (1969). 11. Heyn RM, et al: The role of combined chemotherapy in the treatment of rhabdomyosarcoma in children, Cancer 34: 2128 (1974). 12. Rivard G, et al: Intensive chemotherapy as primary treatment for rhabdomyosarcoma of the pelvis, ibid. 35: 1593 (1975). 13. Belman AB, and Baum ES: Current trends in treatment of childhood rhabdomyosarcoma of lower genitourinary tract, Urology 8: 31 (1976).
UROLOGY
/
JUNE 1979
i
VOLUME XIII,
NUMBER 6
