Case ReportUnexpectedFavorableOutcomeof CongenitalStage IllRhabdomyosarcoma

Atiya Khan, M.D.Gregory Hoy, M.D.Lucius F. Sinks, M.D.

The patient is a two-year-old black maleinfant born to a 23-year-old female (03P2 AbO) after a normal, full-term pregnancy, labor, and delivery. On the baby'ssecond day, the mother observed a smallmass on his left posterior thigh, whichshe reported to the doctors. Physical examination found the baby healthy, except for a round, 3 x 3 cm swelling on theleft posterior midthigh; this was hard,nonmobile, and nontender. There was noregional lymphadenopathy, and x-rays ofthe legs revealed no bony abnormality. Asurgical consult was obtained, and thediagnostic impression was hematoma.The baby was sent home, having beenscheduled to return in one week. He wasbrought back in three weeks, and according to the mother, did well at home, except that the swelling had become muchlarger. On examination, the mass nowmeasured 8 x 10 cm, and was hard, superficial, and nontender. Regional lymphnodes were palpable. The baby was ad

Dr. Khan is an Instructor, Division of Pediatric and Adolescent Oncology, VincentT. Lombardi Cancer Research Center,Georgetown University Hospital, Washington, D.C.Dr. Hoy is Assistant Professor, Departmentof Surgery, Georgetown University Hospital, Washington, D.C.Dr. Sinks is Chief, Division of Pediatric andAdolescent Oncology, Vincent T. LombardiCancer Research Center, Georgetown University Hospital, Washington, D.C.

mitted to the hospital with a presumptivediagnosis of rhabdomyosarcoma. CBC,uninanalysis, chest x-ray, skeletal survey,bone marrow, and serum uric acid wereall within normal limits.

The baby was taken to surgery forpossible resection of the tumor, but as itwas found to be unresectable, only a biopsy was performed. Pathological examination revealed a poorly differentiated soft tissue sarcoma. According toIntergroup Rhabdomyosarcoma Study(IRS) guidelines, the disease was diagnosed as stage III. Postoperative chemotherapy was begun with Cytoxan (10mg/kg/day i.v. x 5), vincristine (2 mg/m2/week), and actinomycin-D (0.5 mg/kg/day i.v. x 5). The patient had numerous electrolyte problems (hyponatremiaas a part of inappropriate ADH and hyperkalemia), as well as severe pancytopenia (WBC as low as 100/mm3, platelets 19,000/mm3, and hematocnit 26),secondary to chemotherapy. He later developed bradycardic episodes, and required supported ventilation for severaldays. The child was thought to be septic,and was treated with ampicillin and gentamicin i.v. for 10 days; he also sufferedseizures and became comatose. A CTscan of the head at this time was negative, and a diagnosis of vincristine encephalopathy was entertained. In addition, the patient developed a systemicfunga! infection; this was treated with5-azocytadine.

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In spite of all these problems, thetumor regressed considerably, and bythe end of nine weeks, it was hardlypalpable. The baby gradually improvedwith signs of bone marrow regeneration,clearing of fungal infection, and selfbreathing. At the age of two-and-onehalf months, he was discharged.

A second-look operation was carriedout at three months of age, and the residual tumor was resected. On gross examination, the tumor appeared necroticand soft, without evidence of infiltrationof surrounding tissue. Pathology revealed tumor at the resection margins,and radiation therapy was administered.The patient received 4940 rads to hisleft thigh in 50 days, the field size beingS x 6 cm, and this was tolerated well,with no side effects.

Chemotherapy was reinstituted.After one dose of vincristine, the babydeveloped diarrhea and vomiting, andthe mother, believing this to be relatedto the drug, refused further chemotherapy. Despite constant persuasion fromthe medical staff, she would not returnfor continued treatment.

At the age of 18 months, the motherbrought the child in for a well babycheckup and routine immunizations.Physical examination revealed normalgrowth and development. The only abnormality was in his left thigh, which

was thinner and ½ cm shorter than theright, but gait and mobility were notcompromised.

Two years have elapsed since theinitial diagnosis, and as of September,1979, the child is free of disease according to all available diagnostic methods.

Comment

It is extraordinary for this tumor to occur at such an early age.'.2 In the seniorauthor's experience, only two such caseshave been seen. However, both have responded dramatically to multimodalitytherapy and appear to be cured (the second case is now four years from diagnosis). It is suggestive that the diagnosisof rhabdomyosarcoma in the neonate maycarry with it a good prognosis, but extreme caution must be exercised in administering chemotherapy to the newborn. This may mean reducing the dosesof certain chemotherapeutic agents.@

References

1. Grosfeld IL, Clatworthy HW In, NewtonWA Ir: Combined therapy in childhoodrhabdomyosarcoma: an analysis of 42 cases.I Pediatr Sung 4:637-645, 1969.2. Green DM, Jaffe N: Progress and controversy in the treatment of childhoodrhabdomyosarcoma. Cancer Treat Rev 5:7-27, 1978.

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