Medical and Pediatric Oncology 28:201–204 (1997)

Metastatic Renal Cell Carcinoma in a Child:

11-Year Disease-Free Survival Following Surgery
Ron Grant, MD, FRCPC,1,2 Cynthia Trevenen, MD, FRCPC,3
William C. Hyndman, MD, FRCSC,4 Steven Z. Rubin, MD, FRCS(E), FRCSC,4 and
Max J. Coppes, MD, PhD1,2

A child with metastatic renal cell carcinoma agement of metastatic RCC is reviewed and
(RCC) is presented. This case is unusual in that the genetic mechanisms leading to its develop-
the patient has remained disease free for 11 ment briefly discussed. Med. Pediatr. Oncol.
years following surgery and only one course of 28:201–204 Q 1997 Wiley-Liss, Inc.
chemotherapy prior to thoracotomy. The man-

Key words: renal cell carcinoma; childhood; survival; genetics

INTRODUCTION in the periphery of the lower left lobe, consistent with a

metastatic lesion. Subsequently, a computed axial tomog-
Renal cell carcinoma (RCC) represents approximately
raphy (CAT) scan of the chest confirmed the presence of
2% of all new adult cancers [1]. By contrast, RCC is
the lesion in the lower left lung. In addition, a second,
very rare in children and accounts for less than 0.05%
0.6 cm, well-defined nodular density was shown in the
of childhood cancers [2], the most common malignant
right lower lobe posteromedially. In view of the age of
renal neoplasm of childhood being Wilms’ tumor [3,4].
the patient, these findings were considered to be consis-
The rarity of childhood RCC has precluded the devel-
tent with metastatic Wilms’ tumor and a diagnostic lapa-
opment of optimal management strategies for its treat-
rotomy was performed.
ment. Nevertheless, the overall survival for children with
At surgery, a large tumor was found, uniformly dis-
RCC is estimated to be approximately 65% [5]. Those
tending the upper half of the kidney. The left kidney,
having localized disease have an excellent outcome with
colon, and liver were inspected and appeared grossly
nephrectomy only or, alternatively, with nephrectomy fol-
normal. A right-sided radical nephrectomy was per-
lowed by additional radiation therapy and/or chemother-
formed. Histopathologic examination showed a renal cell
apy. Metastatic childhood RCC, on the other hand, has a
carcinoma with infiltration of the capsule. The tumor
poor outcome [5–9]. Here, we report a long-term survivor
extensively invaded the renal vasculature. Histopatholog-
of metastatic childhood RCC who, except for one course
ically, the tumor consisted predominantly of tubules lined
of chemotherapy, was successfully treated with surgery
by cells with abundant clear cytoplasm, hyperchromatic
only.
nuclei with single distinct nucleoli, and rare mitoses.
CASE REPORT Based on the criteria proposed by Fuhrman et al. [10],
this tumor was assigned a grade 3 (Fig. 1). None of the
A previously well 7-year-old white boy was admitted removed lymph nodes showed evidence of metastatic
to the Alberta Children’s Hospital with a 2-week history involvement.
of macroscopic hematuria. His history was unremarkable. To assess response to chemotherapy, the patient under-
In particular, he had not experienced pain nor had the
parents noticed an abdominal mass. There had been no
weight loss, fever, or night sweats. On physical examina- Ron Grant’s present address is: Hospital for Sick Children, Toronto,
tion, a large, firm, nontender mass was palpated in the Ontario, Canada.
right upper quadrant of the abdomen. No evidence of Steven Z. Rubin’s present address is: Children’s Hospital of Eastern
genitourinary malformations (including varicocele), hemi- Ontario, Ottawa, Ontario, Canada.
hypertrophy, or aniridia was found. Urinalysis confirmed From the Departments of 1Oncology, 2Pediatrics, 3Pathology and 4Sur-
the hematuria; urinary vanillylmandelic acid and homova- gery, University of Calgary, Calgary, Alberta, Canada.
nillic acid levels were within normal range. An intrave- *Correspondence to: Dr. Max Coppes, Pediatric Oncology Program,
nous pyelogram showed a normal left kidney and a large Alberta Children’s Hospital, 1820 Richmond Road SW, Calgary, Al-
mass arising from the mid and superior pole of the right berta, Canada T2T 5C7.
kidney. Radiograph of the chest showed a 1.3 cm nodule Received 28 July 1995; accepted 22 March 1996.
q 1997 Wiley-Liss, Inc.

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202 Grant et al.

Fig. 1. Renal cell carcinoma with clear cells arranged in cords. Original magnification 3325.

went one course of chemotherapy consisting of doxorubi- at presentation correlates with poor survival. In adult
cin (total dose, 45 mg) and vinblastine (4.5 mg). Three patients, metastatic disease at presentation has been asso-
weeks later, there were no changes in tumor sizes as ciated with a mortality rate of greater than 90% at 3 years,
determined by a CAT scan of the chest. The patient then with a median survival time of approximately 10 months.
underwent a left thoracotomy and wedge resection of the However, if a patient presents with only one pulmonary
left lower lobe. Histopathologic examination confirmed nodule, resection of the solitary metastatic lesion results in
the presence of metastatic RCC. Two weeks later, a right an estimated 5-year survival rate of greater than 45% [13].
thoracotomy and right lower lobe medial basilar segmen- Morphologic parameters also have prognostic signifi-
tectomy were performed. Histopathologic examination cance. The grading system proposed by Fuhrman et al.
again confirmed the presence of metastatic RCC. Both [10] has been demonstrated to be prognostically more
metastatic lesions were histologically identical to the pri- effective than other morphologic parameters. In addition,
mary tumor and showed no evidence of chemotherapy- it can be applied to everyday practice. Unlike other grad-
induced necrosis. The postoperative course following ing systems, it is based on nuclear features only distin-
both procedures was unremarkable. In view of the fact guishing four groups: grade 1 (with excellent outcome),
that the metastatic lesions had failed to respond to chemo- grades 2 and 3 (with intermediate outcome), and grade
therapy, the parents elected no further treatment. With 4 (with poor outcome). Our patient was classified as a
a follow-up of 11 years, the patient has remained in grade 3 patient, a category associated with a high likeli-
complete remission. hood of metastases.
Neoadjuvant chemotherapy and/or radiotherapy have
DISCUSSION generally been ineffective in the treatment of adult RCC.
Approximately half of adult patients with RCC have Single-agent therapy has generally yielded response rates
localized disease at diagnosis; the other half has either of less than 10% [14], with the possible exception of
extensive regional involvement or metastatic spread [11]. vinblastine therapy with a consistent response rate of
Hematogenous spread to either lungs or bones is the most approximately 15% [15]. Theoretically, it is possible that
important and most frequent way of tumor dissemination. childhood RCC has a different biologic behavior than
Based on a limited number of patients, a similar distribu- adult RCC and, as a consequence, is more sensitive to
tion has been noted in childhood RCC [7–9,12]. chemotherapy and/or radiotherapy. The very limited ex-
Because one of the most significant prognostic factors perience in childhood RCC does not seem to support this
involves the extent of disease, the presence of metastases hypothesis [8]. In our patient, nonhormonal chemotherapy

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 Metastatic RCC in a Child 203

TABLE I. Cytogenetic Characteristics of Childhood Renal Cell Carcinomas

Gender Age (yr) Cytogenetics Reference
Male 1.5 46,Y,t(X;17)(p11.2;q25) 22
Male 2 46,Y,t(X;1)(p11.2;q21.2) 27
Male 15 49,Y,t(X;1)(p11.2;q21), 1 der(X)t(X;1)(p11.2;q21), 15, 216, 117, 118 28
Female 9 46,X,t(X;1)(p11.2;q21) 29
Male 8 46,XY,t(X;17)(p11.2;q25) 30

was used briefly with no evidence of response; both pul- ior of childhood RCC, children presenting with metastatic
monary metastases remained radiographically un- disease be offered an aggressive surgical approach.
changed, and following surgical removal no histologic Whether IL-2 and/or IFN-a are to be recommended as
changes were noted compatible with response to presurgi- “best therapy” for advanced childhood RCC [21] remains
cal treatment. Therefore, the lack of response to chemo- to be determined.
therapy in our patient concurs with the experience re-
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