J o u r n a l of Surgical O n c o l o g y 9:603-606 (1977)

Nonsurgical Treatment of Pelvic Rhabdomyosarcoma: A Case Report

..................................................................................... ..................................................................................... MARTIN L. BRECHER, M.D.,ARNOLD I. FREEMAN, M.D., P.R.M. THOMAS, M.B.,M.R.C.P., F.R.C.R., and LUCIUS F. SINKS. M.D.

A 2%ycdr-old male child with rhabdomyosarconia of the prostate was treated with radiation to the pelvis, consisting ot' 5,600 rads in 7?h weeks, and combination chemotherapy of Vincristine and Actinomycin D. Surgery had been refused. The child is alive and disease-free inore than 5 years after diagnosis, and over 2 years following the cessation of all therapy. Recent advances in radiotherapy and adjuvant chemotherapy have significantly improved the outlook in this aggressive childhood neoplasm and have made a less aggressive and less mutilating surgical approach often desirable. This, in turn, holds out the prospect of improved quality of survival in childrcn so afflicted.

..... Key words: rhabdomyosarcoma, combination chemotherapy, multimodality, exenteration

INTRODUCTION Rhabdomyosarconia is the most common soft tissue sarcoma seen in the pediatric age group, representing 10-1 5% of childhood malignant solid tumors (Maurer, 1975). It is a very aggressive tumor that tends to infiltrate local structures and metastasize distantly, particularly to the lungs. In children, this tumor often has its origin in pelvic structures, especially the bladder, prostate, or vagina. Until recently, survival rates of children with pelvic rhabdomyosarcoma have been disappointing, despite attempts at radical surgical removal of apparently localized lesions. Over the last decade, however, dramatic strides have been made through a multimodality approach combining aggressive surgical excision followed by radiotherapy and adjuvant chemotherapy (Maurer, 1975). The following is a case report of a child with biopsy-proven rhabdomyosarcoma o f the prostate who is alive and disease-free more than 5 years after diagnosis, having undergone therapy consisting only of radiotherapy and combination chemotherapy. From the Departments of Pediatrics and Radiation Therapy, Roswell Park Memorial Institute, Buffalo, New York Dr. Sinks is now at the Vince Lombardi Cancer Research Center, Georgetown University Medical Center, Washington, D.C. Address reprint requests to Dr. Martin Brecher, Roswell Park Memorial Institute, 666 Elm Street, Buffalo. NY 14263.

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CASE REPORT A 2%-year-old white male presented t o Buffalo Children’s Hospital in December 1970 with a 2-month history o f tenderness in the perineal area, a 2-week history of dysuria, and a 3-day history o f decreased urinary frequency. On physical examination, the child was well developed for his age and in no distress. A tender, stoney hard induration was noted along the bulbar urethra. On rectal examination, the left lobe of the prostate was markedly enlarged and stoney hard in consistency. The mass extended about 5 cm suprapubically, and the rectal ampulla was decreased in caliber. An IVP showed the base o f the bladder t o be elevated by a large mass lying deep in the pelvis. A voiding cystourethrogram demonstrated an enlarged and elongated prostatic urethra that stretched over a mass situated inferiorly t o it. Chest x-ray and skeletal survey were negative for metastatic disease. A needle biopsy was performed and a diagnosis of embryonal rhabdoniyosarcoma, likely arising from the prostate, was made. Radical surgery was discussed with the parents, but they refused all surgical intervention. The patient was then transferred t o Roswell Park Memorial Institute, where he was treated with radiotherapy and combination chemotherapy. From January through March 1971 the radiation was delivered to the entire pelvis and perineum b y photons from a 6 MeV linear accelerator using anterior and posterior parallel opposing fields measuring 13 cni longitudinally by 9 cm transversely. The total midplane dose was 5,600 rads given in 28 sessions in 7.5 weeks. A split course was used: 3,000 rads in 3 weeks were followed after a 2-week rest period b y 2,600 rads in 2.5 weeks. On completion of the radiotherapy, the patient’s rectal examination revealed considerable tumor regression. Chemotherapy consisted o f cycles of Actinomycin D at a dose of 15 pg/kg/day for 5 days and Vincristine at 0.075 mg/kg/week for 6 weeks. Vincristine therapy was begun on Day 21 of the cycle, and this cycle was repeated every 6 3 days for 2 years. Chernotherapy was begun concomittently with radiotherapy (Fig. 1). A residual mass continued t o be palpable on rectal examination for several months with a fullness detectable in the suprapubic area. This fullness disappeared conipletely approximately 1 year after the start of therapy. The child is alive and disease-free more than 5 years after diagnosis and over 2 years after the cessation of all therapy.

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DISCUSSION Until recently, rhabdomyosarconia of the prostate has been an almost universally lethal neoplasm in childhood. Unlike similar tumors of the bladder, which usually cause symptoms at a very early stage, prostatic sarcomas are often quite advanced when symptomatology first appears. Radical surgery has been the mainstay of treatment of embryonal rhabdomyosarcoma. In 1950, Slobbe and Dargeon noted that some of these tumors are radiosensitive. In 1961, Pinkel and Pickren recommended a regimen of surgery, radiation therapy, and chemotherapy for the treatment of rhabdomyosarcoma, and suggested that “prophylactic” chemotherapy following complete surgical excision of gross tumor might decrease the incidence of metastatic spread. Initial studies, employing either Vincristine or Actinomycin D (Sutow et al., 1966; James et al., 1966; James, 1965), demonstrated effect from single agents. More encouraging results have been obtained with approaches combining surgery, radiotherapy, and combination chemotherapy consisting of Vincristine, Actinoniycin D, and, in some cases, cyclophosphamide (James et al., 1966; Pratt, 1969; Piver et al., 1973;Ghavimi et al., 1975;Donaldsonet al., 1973;and Timmons et al., 1975). At the same time, radiotherapy for rhabdomyosarcoma has been refined. Nelson (1968) recommended radical radiotherapy in cases of incomplete excision of rhabdomyosarcoma. Edland (1967) suggested that insufficient radiotherapy dosage had been employed for control of local disease and recommended using a tumor dose of 6,000 rads over a 6-week period. Programs utilizing a combination of surgical excision, adequate radiotherapy, and combination chemotherapy have produced overall 2-year survival rates ranging from 4 3 t o 79% for rhabdomyosarcoma arising in all locations (Maurer, 1975). As adjuncts to surgery have shown increasing success, attempts have been made t o limit the scope of the surgery so that the quality of survival can be improved. Kilman et al. (1973) have advanced the concept of “reasonable surgery” to initiate treatment of rhabdomyosarcoma, by which they mean removal of as much tumor as possible with maximum conservation of normal anatomy. Ortega et al. (1975) note that the morbidity and secondary problems related to treatment of pelvic rhabdomyosarconia in particular are primarily related t o surgical exenteration, and they suggest that the extent of the surgical procedure could be reduced. In the particular case presented here, no surgery was performed because of parental objection to this form of therapy. The child has survived more than 5 years and is very likely cured of his disease. This in no way implies that limited surgery to remove the bulk of tumor tissue should not be performed when possible, but it does add to the growing body of evidence that suggests that the presence of residual disease need not significantly decrease the prospects for cure if optimum use is made of radiotherapy and combination chemotherapy, and it suggests that a less radical procedure than exenteration may eventually become the procedure of choice in pelvic rhabdomyosarcoma. This nonextirpative approach in turn would hold out the prospect of greatly improved quality of survival in the growing number of children who survive this previously lethal disease.

ACKNOWLEDGMENTS This work was supported in part by USPHS Grant CA 07918 and the Association for the Research of Childhood Cancer (AROCC).

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