Table 3.

Immunohistochemistry.

Patient No.	Prior Diagnosis	Renal Neoplasm	Immunophenotype
1	Wilms Tumor	Clear Cell RCC	Diffuse "box-like" CAIX, Melan A−
2	Wilms Tumor	Clear Cell RCC	Diffuse "box-like" CAIX, Melan A−
3	Wilms Tumor	Tumor1: Clear Cell RCC	Diffuse "box-like" CAIX, Melan A−
		Tumor2: Oncocytoma	CD117+, CK7−
4	Wilms Tumor	Clear Cell RCC	Diffuse "box-like" CAIX, Melan A−
5	Wilms Tumor	Metanephric Adenoma	WT1+, BRAF V600E− (NGS: negative for BRAF alterations)
6	Wilms Tumor	Tumor1 (left): Metanephric Adenoma	WT1+, BRAF V600E− (NGS: negative for BRAF alterations), Parafibromin loss
		Tumor2 (left): Oncocytoma	CD117+, CK7−
		Tumor3 (left): Metanephric Adenoma	WT1+, BRAF V600E− (NGS: insufficient amplifiable DNA), Parafibromin loss
		Tumor4 (left): Unknown	Unknown
7	Neuroblastoma	t(6; 11) RCC*	Cathepsin K+, Melan A+, HMB45+
8	Neuroblastoma	Clear Cell RCC	Diffuse "box-like" CAIX, Melan A−
9	Neuroblastoma	Clear Cell RCC	Diffuse "box-like" CAIX, Melan A−
10	Neuroblastoma	Clear Cell RCC	-
11	Neuroblastoma	Clear Cell RCC	Diffuse "box-like" CAIX, Melan A−
12	Acute Lymphoblastic Leukemia	FH-Deficient RCC	CK7−, FH-deficient, increased 2SC
13	Acute Lymphoblastic Leukemia	Clear Cell RCC	Diffuse "box-like" CAIX, Melan A−
14	SLE	Tumor1 (right): ASPL-TFE3** rearranged RCC2	Diffuse: TFE3, CD10; Focal: CK AE/AE3, Cam5.2
		Tumor2 (left): PRCC-TFE3** rearranged RCC	Diffuse: TFE3, CD10; Focal: CK AE/AE3, Cam5.2

RCC: renal cell carcinoma; FH: fumarate hydratase; NGS: next generation sequencing; 2SC: S-(2-succino)-cysteine.

^*Patient 7 was reported previously by Gupta et al (2019) and

^**Patient 14 was reported previously by Argani et al (2001 and 2006).