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Table 1.

Clinical and general characteristics of the 12 patients with Xp11.2/TFE RCCs.

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Table 2.

Imaging characteristics of the 12 patients with Xp11.2/TFE RCCs.

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Figure 1.

Translocation RCC in a 29-year-old woman (patient 5 in Table 12) with a left palpable abdominal mass.

A–C, Axial T2WI and plain (non-contrast) T1WI (B, IP in phase; C, OP out of phase) showing a large, well-defined, irregular mass (T2, high-low heterogeneous signal intensity; T1, iso-signal intensity) with a large patchy hemorrhage and necrosis in the center of the mass. D–F, CMP and NP gradient-echo MR images showing heterogeneous and prolonged enhancement of the mass. G, The tumor cells are polygonal, with voluminous eosinophilic cytoplasm containing a few hyaline nodules and forming a papillary structure (HE 10 & 10).

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Figure 2.

Translocation RCC in a 13-year-old boy (patient 6 in Tables 12) with internal gross hematuria and a left cervical mass (biopsy proved metastases).

A–C, Axial T2WI and plain (non-contrast) T1WI (B, IP; C, OP) showing a large, ill-defined, irregular mass (T2, high-low mixed signal intensity; T1, iso-high mixed signal intensity; liquid-gas surface) with a large, patchy hemorrhage and necrosis in the center of the left renal mass, without an integrated capsule. Multiple retroperitoneal metastases are detected. D–E, CMP and NP gradient-echo MR images showing marked enhancement, predominantly in the periphery of the mass. F, Coronal delay phase image showing marked enhancement in the periphery of the mass, invading the left renal pelvic space and vessels. G, The tumor cells contain hyaline cytoplasm and show aciniform, papillary and micro-papillary structures (HE 10 & 20).

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Figure 3.

Translocation RCC in a 25-year-old woman (patient 8 in Tables 1, 2, 3) with an incidentally detected left renal mass on US.

A, Axial unenhanced CT image showing an ill-defined, slightly high attenuation of the mass within the left kidney contour. B–C, Axial CMP and NP contrast-enhanced CT images demonstrating heterogeneous and prolonged enhancement of the mass in the left kidney. D–E, Axial T2WI and plain (non-contrast) T1WI showing an irregular ill-defined left renal mass with a heterogeneous, slightly high signal intensity, which seemed to break the capsule. Axial CMP (F) and NP (G) gradient-echo MR images showing heterogeneous moderate enhancement of the left renal mass. H, Coronal delay phase image showing delay enhancement of the mass. I, The tumor cells contain hyaline cytoplasm, eosinophilic cells with papillary structure and psammoma bodies (HE 10 & 40).

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Figure 4.

Translocation RCC in a 36-year-old woman (patient 9 in Table 1, 2, 3) with gross hematuria.

A–C, Axial T2WI and plain (non-contrast) T1WI (B, IP in phase; C, OP) showing an irregular well-defined mass (T2, iso-high mixed signal intensity; T1, slightly high signal intensity) with a patchy area of cystic necrosis in the left renal pelvis. Axial CMP (D) and NP (E) gradient-echo MR images showing moderate heterogeneous enhancement in the left renal mass. F, Coronal delay phase image showing a delayed-enhancement mass within the contour of the kidney. G–H, Unenhanced CT images showing a well-defined, slightly high-density renal mass with mottling calcification. I, The tumor cells contain hyaline cytoplasm with a papillary structure (HE 10 & 40).

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Figure 5.

Translocation RCC in a 40-year-old woman (patient 10 in Tables 1, 2, 3) complaining of left flank pain for approximately half a year that became more serious 1 month prior to imaging.

A, Axial unenhanced CT image showing an ill-defined, slightly higher attenuation soft tissue mass in the left kidney. Retroperitoneal adenopathy is present. B–D, Axial CMP, EP and NP contrast-enhanced CT images demonstrating moderate and prolonged enhancement of the mass without an integrated capsule. E, Liver metastasis with hemorrhage on EP is observed. F, The tumor cells consist of poorly differentiated clear cells (HE 10 & 40).

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Figure 6.

A 16-year-old boy (patient 12 in Tables 1, 2, 3) with XP11.2/TFE 3 confirmed by biopsy.

A, Axial unenhanced CT image showing an ill-defined, irregular, slightly higher attenuation mass with a bulk of plaque-like calcifications in the right kidney. Retroperitoneal adenopathy is observed. B–D, Axial CMP and NP contrast-enhanced CT showing a heterogeneously enhanced mass. E, The liver and porta hepatis areas and right retroperitoneal multiple lymphoma metastases are indicated. F, Immunohistochemical analysis demonstrated TFE3 nuclear staining (HE 20 & 10).

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Table 3.

HU value of CT attenuation in the 6 patients with Xp11.2/TFE RCCs.

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