Introduction: Renal cell carcinoma (RCC) represents less than 2-6% of pediatric renal tumors. Few reports of long-term outcomes exist. We sought to determine the presentation and outcome of patients at our institution.
Methods: We retrospectively reviewed the age, mode of presentation, histological subtype, tumor grade, stage and survival of all RCC patients from 1980-2005 at our pediatric institution.
Results: 15 patients were identified. Mean age at presentation was 7.9 years (range 2.5 -18 years). Symptomatic presentations in nearly 75% included gross hematuria (6/15), abdominal pain (4/15) or polycythemia (1/15). The remaining 25% were asymptomatic, identified with a renal mass either on physical examination or incidentally on imaging. Surgical resection consisted of radical nephrectomy in 10 and partial nephrectomy in 5. Pathology revealed papillary RCC in 8 and clear cell RCC in 7. Six patients were found to have higher stage disease (> Robson stage II). One patient with stage IV disease died 8 months following surgery. The remaining 14 patients are all alive with a mean 4.9 years (range 1 - 11 years) of follow-up. All but one of the patients remain recurrence free including all of those who underwent nephron-sparing surgery.
Conclusions: Our series represents one of the largest reported in the literature from a single institution. In general, pediatric patients with RCC tend to be older and more likely to present symptomatically compared to the typical Wilms’ tumor patient. Hematuria and abdominal pain were the most common presentations in our series, and papillary RCC is proportionally more common in pediatric versus adult patients. Equivalent oncologic cure rates can be expected from a nephron sparing approach in appropriately selected patients.

Patient Number	Age (yrs.mo)	Gender	Presentation	Surgical Procedure	Tumor Histology	Grade	Robson Stage	Previous or Adjuvant Therapy	Outcome (F/U)
1	3.8	M	Polycythemia	Left PN	Clear cell	I	I	None	NED (127 mo)
2	3.1	F	Asymptomatic abdominal mass	Left RN	Clear cell	I	III	None	NED (124 mo)
3	6.2	F	Painless gross hematuria	Right RN	Papillary	II	III	Abdominal radiotherapy (45 Gy)	NED (122 mo)
4	13.8	M	Painless gross hematuria	Left RN	Papillary	III	IV	Resection of RCC left lower lobe lung metastasis	NED (60 mo)
5	10.9	F	Fever and abdominal pain	Left PN	Papillary	I	II	None	NED (98 mo)
6	8.7	F	Painless gross hematuria	Right PN	Clear cell	I	I	None	NED (55 mo)
7	2.6	F	Painless gross hematuria	Right RN	Papillary	II	I	None	NED (13 mo)
8	2.8	M	Fever and abdominal pain	Right RN	Papillary	II	I	None	NED (39 mo)
9	8.2	F	Incidental MRI finding	Right RN	Clear cell	III	II	Previous surgery and chemotherapy for GNB	NED (36 mo)
10	4.11	M	Abdominal pain	Right RN	Clear cell	I	I	None	NED (51 mo)
11	11.2	M	Incidental MRI finding	Left PN	Clear cell	I	I	Previous surgery and chemotherapy for prostatic embryonal rhabdomyosarcoma	NED (12 mo)
12	11.7	M	Abdominal pain and anemia	Left RN	Papillary	III	IV	Trial of immunotherapy, resection of left lung and adrenal RCC metastases	AWD (14 mo)
13	7.8	M	Painless gross hematuria	Left RN	Clear cell	III	IV	Radiotherapy, chemotherapy, immunotherapy	DOD (8 mo)
14	12.5	F	Painless gross hematuria	Right RN	Papillary	II	II	None	NED (63 mo)
15	18.3	F	Incidental U/S finding	Right PN	Papillary	I	II	None	NED (15 mo)

Surgery	Number of Patients	Mean Tumor Size (range)	Robson Stage	DFS	OS	Mean Follow-up (range)
Radical nephrectomy	10	7.3 cm (2-11.5 cm)	I-3 II-2 III-2 IV-3	80%	90%	53 months (8-124 months)
Partial nephrectomy	5	2.8 cm (1.3-4 cm)	I-3 II-2 III-0 IV-0	100%	100%	61 months (12-127 months)

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