For patients with suspected Wilms tumors, preoperative staging studies should include a computed tomography (CT) scan of the abdomen/pelvis and chest. Preoperative assessment of intravascular extension of Wilms tumor is essential to guide management. The presence of intravenous tumor thrombus, which has been reported in up to 11.3% of Wilms tumor cases, can lead to differences in management. In North America, local staging of Wilms tumor is performed with CT or magnetic resonance imaging of the abdomen and pelvis. Contrast-enhanced CT has high sensitivity and specificity for detection of cavoatrial tumor thrombus in Wilms tumor cases that may impact surgical approach. Routine Doppler evaluation after CT has been performed is not required.
There are several types of plasma cell neoplasms. These diseases are all associated with a monoclonal (or myeloma) protein (M protein). They include monoclonal gammopathy of undetermined significance (MGUS), isolated plasmacytoma of the bone, extramedullary plasmacytoma, and multiple myeloma.
(Refer to the Lymphoplasmacytic Lymphoma (Waldenström Macroglobulinemia) section in the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information.)
Incidence and Mortality
The stage is determined by the results of the imaging studies and both the surgical and pathologic findings at nephrectomy and is the same for tumors with favorable or anaplastic histology. Thus, patients should be characterized by a statement of both criteria (for example, stage II, favorable histology or stage II, anaplastic histology).[2,3]
The staging system was originally developed by the National Wilms Tumor Study Group and still used by the Children's Oncology group. The staging system and incidence by stage are outlined below.
In stage I Wilms tumor (43% of patients), all of the following criteria must be met:
Tumor is limited to the kidney and is completely resected.
The renal capsule is intact.
The tumor is not ruptured or biopsied prior to removal.
No evidence of the tumor at or beyond the margins of resection.
For a tumor to qualify for certain therapeutic protocols as stage I, regional lymph nodes must be examined microscopically.
In stage II Wilms tumor (20% of patients), the tumor is completely resected, and there is no evidence of tumor at or beyond the margins of resection. The tumor extends beyond the kidney as evidenced by any one of the following criteria:
There is regional extension of the tumor (i.e., penetration of the renal sinus capsule, or extensive invasion of the soft tissue of the renal sinus, as discussed below).
Blood vessels within the nephrectomy specimen outside the renal parenchyma, including those of the renal sinus, contain tumor.