Medical and Pediatric Oncology 16:381-383 (1988)

PROCEEDINGS OF THE TUMOR BOARD OF THE CHILDREN’S HOSPITAL OF PHILADELPHIA Giulio J. D’Angio, MD, Series Editor, and Audrey E. Evans, MD, Associate Editor

lntrascrotal Metastasis From Wilms’ Tumor

Beatriz de Camargo, MD, Jose Pinus, MD, Henrique Lederman, MD, and Leda Saba, MD

1 Key words: renal embryoma,

Beatriz de Camargo, MD (Pediatric Oncologist)

Our patient today is a child with Wilms’ tumor who had an unusual clinical evolution.

The patient was a 5-year-old boy who was found to have a right-sided abdominal mass on routine physical examination. The tumor was noted by the mother while dressing the child. He was normal in other respects except for the presence of a hare lip. The past history was noteworthy in that the mother was said to have had a kidney tumor at birth. It was removed, and there are no further details available regarding the nature of the tu- mor. Except for the operation, however, no additional treatment was given.

Imaging studies were performed at another institution, where the child was first seen. Dr. Lederman, will you please review those findings for us.

Henrique Lederman, MD (Pediatric Radiologist)

Excretory urography revealed a huge right-sided, in- trinsic kidney mass that crossed the midline. The distor- tion of the collecting system was typical of an intrarenal mass. Computerized tomographic (CT) scan showed it to be solid and intrarenal. The diagnosis of Wilms’ tumor was most likely, and this impression was strengthened by the chest X-ray films and CT of the chest, which dem- onstrated bilateral pulmonary nodules, consistent with metastatic foci.

Jose Pinus, MD (Pediatric Surgeon)

The X-ray films and physical examination indicated that this tumor was extremely large, and that there were real risks associated with attempted removal. In view of the almost certain diagnosis of Wilms’ tumor, we there- fore elected to defer surgery until chemotherapy had an opportunity to reduce the size of the mass. Chemother- apy was then administered by Dr. de Camargo at the A.C. Camargo Hospital.

nephroblastoma, epididymis 1

Dr. de Camargo. Chemotherapy was initiated using actinomycin D and vincristine. The first drug was given in standard fashion (15 pg/kg/day) on days 0 through 4, and three doses of vincristine (1.5 mg/m2) on days 0, 4, and 11. The tumor promptly shrank in size, and the patient was referred back to Dr. Pinus.

Dr. Pinus. The much-reduced tumor was excised without event. There was no infiltration into surrounding structures, the lymph nodes appeared normal, and the opposite kidney, which was thoroughly explored, showed none of the stigmata of nephroblastomatosis or other abnormality. The lymph nodes were routinely biopsied, and the abdomen was closed without any prob- lems being encountered during the operation.

Leda Saba, MD (Pediatric Pathologist)

The specimen showed a predominant blastematous Wilms’ tumor with scarce tubules and with areas of necrosis, apparently the result of the prior chemotherapy. The capsule was infiltrated, and there was a neoplastic thrombus in the extrarenal vessels. The lymph nodes that accompanied the specimen were free of tumor, which was considered to be of the favorable histologic type according to the criteria of Beckwith and Palmer [ 11.

Dr. Lederman. Postoperative films showed that the pulmonary nodules had responded to chemotherapy, and the lung fields were entirely clear.

Dr. de Camargo. The child was treated with acti-

From the Department of Pediatrics, Hospital A.C. Camargo (B.d.C., L.S.) and the Escola Paulista de Medicina (J.P., H.L.), Sao Paulo, Brazil.

Address reprint requests to Giulio J . D’Angio, MD, Children’s Cancer Research Center, Children’s Hospital of Philadelphia, 34th Street & Civic Center Boulevard, Philadelphia, PA 19104.

0 1988 Alan R. Liss, Inc.

382 de Carmargo et al.

Fig. 1. A: tubules (bottom). B: Wilms’ tumor.

Intrascrotal metastasis. Low-power view shows a nest of tumor (top) adjacent to epididymal High-power view shows mixed epithelial and blastemal elements typical of

nomycin D, vincristine, and Adriamycin according to Regimen DD of the third National Wilms’ Tumor Study (NWTS) [2]. Whole-lung irradiation was added accord- ing to that protocol with the delivery of 150 cGy on 8 treatment days for a total of 1,200 cGy. The child tolerated the chemotherapy very well, and 7 months after the initiation of treatment, the mother noticed a swelling in the left side of the scrotum. The child was examined by us at that time, and we elected to observe the nodule because of the extreme rarity of Wilms’ tumor metastatic to the testicle. The mass grew, however, during that interval and became extremely firm. The patient was then referred to Dr. Pinus.

Dr. Pinus. We concurred that there was a solid, firm nodule occupying the left scrotum, and the mass was removed using the traditional radical orchiectomy ap- proach, when it became apparent that the epididymis was engulfed by tumor. There were no lymph nodes encoun- tered in the inguinal region.

Dr. Saba. Histologic examination of the specimen confirmed that there was a metastatic focus of Wilms’ tumor invading the epididymis wall. (Fig. 1). The his- tologic type was similar to the original tumor but showed more tubules and glomeruloid structures. Our diagnosis, therefore, was metastatic Wilms’ tumor to the epi- didymis, favorable histology type.

Dr. de Camargo. Chemotherapy was changed to VP- 16 and cisplatin. Three cycles of this combined-agent chemotherapy was given, when the parents elected to discontinue all therapy. A relapse then followed, with a large mass growing in the left inguinal canal. This was treated first with chemotherapy for 1 month, again using actinomycin D, vincristine, and Adriamycin. It was then completely excised and given 2,000 cGy. He was well when last seen 4 months after the second relapse, when he again was lost to follow-up.

The evolution of this case points up that Wilms’ tumor can metastasize to strange sites. Most of the metastases

Intrascrotal Metastasis From Wilms’ Tumor 383

tageous in the control of lung deposits. It is interesting to note in closing that we have not devoted much discussion to the fact this child had stage IV disease at diagnosis, with bilateral lung lesions. We have become very confi- dent that combined modality care such as this child received will cure most such patients. The 2-year sur- vival for stage IV/FH patients is almost 90% according to the NWTS-3 results [2].

occur in the lung (80%). Liver, bone, brain, and intraab- dominal regions account for almost all the remainder [3,4]. All other foci of involvement are termed “unusual” by the NWTS. Forty of 1,467 NWTS-3 patients with unilateral favorable histology tumors (2.7%) developed metastases in unusual regions, and at least 29 of these were in the chest wall, pleura, mediasti- num, or other intrathoracic structure [5] . Thus, me- tastases to extrathoracic organs and structures are ex- ceedingly rare: 11 of 1,467 or 0.7%. Of those 11, only three were found in the female or male genital areas: two in the ovary, one in the vagina, and one in the scrotum; therefore, like our patient. There were none recorded in the testicle itself. Of the 24 metastases to unusual sites from nearly 400 NWTS-3 patients with tumors of unfa- vorable histologic type, there was one recorded in the “testicle” (not verified). Two cases of intrascrotal and cord deposits from Wilms’ tumor have been recorded by others; interestingly, both were left-sided, too [6,7]. Yadav and Pathak make the point that the spermatic vein was not affected at the time of initial surgery in their patient, as it was not in ours. These metastases can be found in children with various other neoplastic intraab- dominal or retroperitoneal conditions: neuroblastoma, for example. Some authorities believe that the spread is via occult hernial sacs and that the metastases do not involve the testicle except secondarily.

What the relapse sites imply for our patient in terms of eventual long-term survival is not known; certainly, not too much can be made of this particular case because of the parental refusal of therapy. Also, one cannot infer that chemotherapy penetration into the paratesticular tis- sues was inadequate, reaching that conclusion because the intrascrotal tumor developed while the pulmonary lesions remained under control. Irradiation of the lungs had been given, and radiation therapy clearly is advan-

A C K N O W L E D G M E N T S

Dr. de Camargo and her coauthors, along with the Editors, are grateful to Dr. Jane Chatten, Department of Pathology, Children’s Hospital of Philadelphia for her review of the pathology and for providing the photomi- crographs.

REFERENCES

1. Beckwith JB, Palmer NF: Histopathology and prognosis of Wilms’ tumor. Results from the National Wilms’ Tumor Study. Cancer

2. D’Angio GJ, Breslow N, Beckwith JB, Evans AE, Baum E, deLorimier A, Fernbach D, Hrabovsky E, Jones B, Kelalis P, Othersen HB, Tefft M, Thomas PRM: The treatment of Wilms’ tumor: Results of the Third National Wilms’ Tumor Study. Cancer (in press).

3 . Breslow N, Churchill G, Beckwith JB, Fernbach DJ, Othersen HB, Tefft M, D’Angio GJ: Prognosis for Wilms’ tumor patients with non-metastatic disease at diagnosis-Results of the second National Wilms’ Tumor Study. J Clin Oncol 3:521-531, 1985.

4. Breslow NE, Churchill G, Nesmith B, Thomas P, Beckwith JB, Othersen HB, D’ Angio GJ: Clinicopathologic features and prog- nosis for Wilms’ tumor patients with metastases at diagnosis. Cancer 58:2501-2511, 1986.

5. Unpublished observations kindly provided by the NWTS Group. 6. Yadav K , Pathak IC: Wilms’ tumour with left cord metastasis. Br

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