Diagnosis and management of paratesticular rhabdomyosarcoma in childhood
HUANG Cheng-ru, SUN Ning, ZHANG Wei-ping, BAI Ji-wu, TIAN Jun, XIE Xiang-hui, LI Ming-lei, SONG Hong-cheng
Published 2009-09-15
Cite as Chin J Pediatr Surg, 2009,30(09): 597-599. DOI: 10.3760/cma.j.issn.0253-3006.2009.09.006
Abstract
Objective To improve the diagnostic accuracy and therapeutic effect of paratesticu-lar rhabdomyosarcoma (PARA RMS). Methods Fourteen patients diagnosed with PARA RMS from 1977 to June 2008 were retrospectively analyzed. The patients aged from 1 to 15 years, with the mean age of 6 years. Of all the cases, 6 were younger than 5 years. Nine sinistral and 5 dextral PARA RMSs were diagnosed. All patients manifested as scrotum swollen and diagnosed with PARA RMS at stage Ⅰ by roentgenographic findings. Of all the patients, 10 underwent radical inguinal orchiectomy, and 4 patients who had undergone orchiectomy ( 1 case) or tumor enucleation (3 cases) through a trans-scrotal incision in other hospitals underwent inguinal exploration with removal of the testis or the remaining sperrnatic cord. Excision of the scar of scrotal incision were also performed on the 4 above-mentioned patients. Thirteen cases were diagnosed as the spindle cell type of embryonic RMS and one the as the gland alveolar type. Chemotherapy (VAC, vincristine + actinomycin + cyclophosphamide) was performed on all patients. Results Thirteen of 14 patients were followed up for 1 to 24 years (mean, 6 years). Eleven patients were followed up for at least 2 years, including 10 (91%) survivors without tumor recurrence. For the other one patient with the age of 8 years, who underwent only 6 periods of chemotherapy and whose parents gave up the following chemotherapy, had the recurrence of a retroperitoneal metastatic tumor with the diameter more than 8 cm at 2 years postoperatively. A 9 year old patient who was admitted in 1981 had no sperm in his semen at follow-up. Conclusions PARA RMS often shows as a unilateral scrotal swelling or mass, thus it could be diagnosed earlier than other genitourinary tumors. Most cases of early diagnosed with PARA RMS are at stage Ⅰ, with the pathologic diagnosis of spindle cell type which indicated a good prognosis. The curative rate for pa-tients in stage 1 can be up to 90% after radical inguinal orchiectomy combined with VAC chemothera-py. In recent years, only VCR+ ACTD is suggested for type 1 PARA RMS for 1 year, to avoid infer-tility.
Key words:
Rhabdomyosarcoma; Paratesticular; Diagnosis and management
Contributor Information
HUANG Cheng-ru
Department of Surgery, Beijing Children's Hospital, Beijing, 100045, China
SUN Ning
Department of Surgery, Beijing Children's Hospital, Beijing, 100045, China
ZHANG Wei-ping
Department of Surgery, Beijing Children's Hospital, Beijing, 100045, China
BAI Ji-wu
Department of Surgery, Beijing Children's Hospital, Beijing, 100045, China
TIAN Jun
Department of Surgery, Beijing Children's Hospital, Beijing, 100045, China
XIE Xiang-hui
Department of Surgery, Beijing Children's Hospital, Beijing, 100045, China
LI Ming-lei
Department of Surgery, Beijing Children's Hospital, Beijing, 100045, China
SONG Hong-cheng
Department of Surgery, Beijing Children's Hospital, Beijing, 100045, China