Paratesticular rhabdomyosarcoma in a neonate

CASE REPORTS

Paratesticular Rhabdomyosarcoma in a Neonate ¨ zden C¸akmak, Ays¸e Karaman, Yusuf Hakan C¸avus¸og˘lu, and Ays¸egu¨l Oksal By O Ankara, Turkey

A 13-day-old boy presented with left scrotal tumor and coronary hypospadias. Left radical orchiectomy was performed. Histological diagnosis was embryonal-type paratesticular rhabdomyosarcoma. To the authors’ knowledge, this is the first reported case of paratesticular rhabdomyosarcoma in a neonate in Englishlanguage literature. Also, theassociation of a testicular tumor with hypospadias has not been noted.

J Pediatr Surg 35:605-606. Copyright 娀 2000 by W.B. Saunders Company.

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Histologically it was confirmed to be an embryonal RMS with interlacing bundles of spindle-shaped cells, differentiating rhabdomyoblasts with cells with dark nuclei and budlike accumulations of intensely eosinophilic cytoplasm. There also were bizarre cells with multiple nuclei and eosinophilic cytoplasm (Fig 2). A 1-year combined chemotherapy regimen was started with vincristine and actinomycin D.

UMORS OF THE TESTES are relatively uncommon lesions, representing approximately 1% to 2% of all malignancies in the pediatric age group.1 Seventyfive percent of testicular tumors observed in children are of germ cell origin and majority are malignant.2 However, paratesticular rhabdomyosarcoma (RMS) accounts for only 12% of childhood scrotal tumors.2,3 Also, most of the paratesticular RMS usually present between 41⁄2 and 5 years of age.4 To our knowledge, this is the first report of a case of paratesticular RMS in a neonate. CASE REPORT A 13-day-old boy presented with a 10-day history of left scrotal swelling. He was a full-term birth from the third gestation of a 33-year-old mother. On physical examination, a painless, solid left scrotal mass, measuring 4 ⫻ 3 cm, clearly discrete from the ipsilateral testis, was noted. The contralateral testis was normal. Also, a coronal hypospadias without chordee was noted. The findings of the remainder of physical examination was unremarkable. Plain abdominal and chest x-rays and abdominal ultrasonographic examination were normal. Scrotal ultrasonographic examination showed a 32- ⫻ 20- ⫻ 25-mm heterogenic solid mass, clearly defined from the surrounding tissues in the left scrotum. Hematologic parameters, liver function findings, serum ␤-human chorionic gonadotropin, ␣-fetoprotein, carcinoembryonic antigen, and total alkaline phosphatase were in normal range. Serum placental alkaline phosphatase was 1.5 U/mL (normal range, 0.0 U/mL). He was operated on through a left inguinal incision. Before handling the testicular tumor, the external inguinal ring was identified and incised in the long axis. The structures of the spermatic cord were occluded with a noncrushing vascular clamp at the level of the internal inguinal ring. When the testis was explored, a firm, encapsulated, white-colored mass was found just superior to testis, measuring 4 ⫻ 3 ⫻ 3 cm. The mass had invaded the cord structures (Fig 1). High ligation proximal to the site of occlusion and en bloc resection were performed. On macroscopic examination, the specimen was a yellowish-white, well-capsulated, paratesticular mass measuring 4 ⫻ 3 ⫻ 3 cm on the spermatic cord, attached to the testis, which measured 1.2 ⫻ 0.8 ⫻ 0.6 cm. Journal of Pediatric Surgery, Vol 35, No 4 (April), 2000: pp 605-606

INDEX WORDS: Rhabdomyosarcoma, paratesticular, neonate, coronary hypospadias.

DISCUSSION

RMS is the most common soft tissue sarcoma occurring in infancy and childhood; only 0.4% of them occur in neonates.5 Paratesticular RMS that arises in the distal area of the spermatic cord and may invade the testis and surrounding tissues accounts for 7% of RMS and 12% of childhood scrotal tumors.3,6 Presentation often is a unilateral painless scrotal swelling or mass above the testis. Most paratesticular RMS have embryonal histology findings, whereas some are mixed, and others may show an undifferentiated pattern.2 Some studies suggest that the median age of presentation is 17 months.7 Although 60% of childhood testicular tumors present before age 2, paratesticular RMS usually present between 41⁄2 and 5 years of age.1,4,8 Initial therapy consisted of radical orchiectomy. Retroperitoneal lymph node dissection (RPLND) is controversial. Considering the high relapse-free and 100% overall survival rates, neither RPLND nor node biopsy is recommended in the current IRS IV trial in children with

From the Departments of Pediatric Surgery and Pathology, Dr Sami Ulus Children’s Hospital, Ankara, Turkey. ¨ zden C¸akmak, MD, C¸ocuk Cerrahisi Address reprint requests to O Klinig˘i, Dr Sami Ulus C¸ocuk Hastanesi, Babu¨r Cad. 44, Altındag˘, 06080 Ankara, Turkey. Copyright 娀 2000 by W.B. Saunders Company 0022-3468/00/3504-0016$03.00/0 605

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Fig 1. A firm, encapsulated mass measuring 4 ⴛ 3 ⴛ 3 cm just superior to left testis. Coronary hypospadias without chordee also is noted.

Fig 2. Embryonal rhabdomyosarcoma with interlacing bundles of spindle shaped cells, and bizarre cells with multiple nuclei and eosinophilic cytoplasm. (H&E, original magnification ⴛ400.)

localized, completely resected tumors whose imaging results are negative.6 After the initial surgical procedure, adjuvant chemotherapy is controversial. Some investigators believe that, regardless of stage, all patients should receive at least vincristine and actinomycin D therapy for 1 to 2 years.8 Paratesticular RMS is associated with a good prognosis, and there is no relationship between histological grouping and outcome.9 Inguinal hernia and undescended testes are the most common anomalies associated with hypospadias. Several

reports have noted a high incidence of upper urinary tract anomalies associated with hypospadias. The occasional association with myelomeningocele and imperforated anus also has been reported. However, the finding of a testicular tumor with hypospadias has not been noted previously.10 Because paratesticular RMS now has been encountered in the newborn, a pediatric surgeon also should consider this possibility in the differential diagnosis of neonatal scrotal masses.

REFERENCES 1. Frey P, Fliegel CH, Herzog B: Testicular tumors in infancy and childhood—A review of 10 germ cell tumors and 10 non germ cell tumors. Z Kinderchir 45:229-234, 1990 2. Rowe MI, O’Neill JA, Grosfeld JL, et al: Gonadal tumors, in Essentials of Pediatric Surgery. St Louis, MO, Mosby, 1995, pp 319-323 (chap 33) 3. Andrassy RJ: Rhabdomyosarcoma. Semin Pediatr Surg 6:17-23, 1997 4. Brosman SA: Testicular tumors in prepubertal children. Urology 13:581-588, 1979 5. Lobe TE, Weiner ES, Hays DM, et al: Neonatal rhabdomyosarcoma: The IRS experience. J Pediatr Surg 29:1167-1170, 1994

6. Wiener ES, Lawrence W, Hays D, et al: Retroperitoneal node biopsy in paratesticular rhabdomyosarcoma. J Pediatr Surg 29:171-178, 1994 7. Kaplan GW, Cromie WC, Kelalis PP, et al: Prepubertal yolk sac testicular tumors—Report of the testicular Tumor Registry. J Urol 140:1109-1112, 1988 8. Cromie WJ, Raney RB, Duckett JW: Paratesticular rhabdomyosarcoma in children. J Urol 122:80-82, 1979 9. Stewart LH, Lioe TF, Johnston SR: Thirty-year review of intra scrotal rhabdomyosarcoma. Br J Urol 68:418-420, 1991 10. Khuri FJ, Hardy BE, Churchill BM: Urologic anomalies associated with hypospadias. Urol Clin North Am 8:565-571, 1981