Clear cell sarcoma of the kidney: a review of 351 cases from the National
Wilms Tumor Study Group Pathology Center.
Argani P, Perlman EJ, Breslow NE, Browning NG, Green DM, D'Angio GJ, Beckwith JB
Department of Pathology, The Johns Hopkins Hospital, Baltimore, Maryland 21287, USA.
We reviewed 351 cases of clear cell sarcoma of the kidney (CCSK), including 182 cases
entered on National Wilms Tumor Study Group (NWTSG) trials 1-4 for which
clinical follow-up information was available. Tumors were restaged using NWTS 5 criteria. Mean
age at diagnosis in the NWTS group was 36 months with a range of 2 months to 14 years. The
male to female ratio was 2:1. Typical gross features included large size (mean diameter 11.3
cm), a mucoid texture, foci of necrosis, and prominent cyst formation. Nine major histologic
patterns were identified (classic, myxoid, sclerosing, cellular, epithelioid, palisading, spindle,
storiform, and anaplastic); virtually all tumors contained multiple patterns that blended with one
another. Immunohistochemical stains were performed on 45 cases; only vimentin was
consistently immunoreactive. Consistently negative results with other antibodies helped
exclude other tumors in the differential diagnosis; all CCSKs were cytokeratin-negative, including
epithelioid tumors that mimicked Wilms tumor, and MIC2-negative, including cellular tumors that
mimicked primitive neuroectodermal tumor. The p53 gene product was rarely overexpressed in
non-anaplastic CCSKs, but strikingly overexpressed in two of three anaplastic CCSKs. Overall
survival was 69%. Multivariate analysis revealed four independent prognostic factors for
survival: treatment with doxorubicin, stage, age at diagnosis, and tumor necrosis. Of note, stage 1
patients had a remarkable 98% survival rate. No other histologic or clinical variable independently
correlated with survival.
PMID: 10632483, UI: 20096344
1: Bone Marrow Transplant 1998 Aug;22(4):381-3
Successful double autografts for patients with relapsed clear cell sarcoma of the kidney.
Yumura-Yagi K, Inoue M, Wakabayashi R, Mabuchi S, Nakayama M,
Yoneda A, Imura K, Kawa-Ha K
Department of Pediatrics, Osaka Medical Center and Research Institute for Maternal and Child
Health, Izumi, Japan.
Although the prognosis of clear cell sarcoma of the kidney (CCSK) has improved, when
metastases occur the probability of cure is very low. We have treated two pediatric patients
with relapsed CCSK, one with multiple bone metastases and another with brain
metastases. After on or two courses of re-induction chemotherapy and radiation therapy to the sites
of metastasis, they received double high-dose chemotherapy with autologous bone marrow
rescue. Conditioning regimens were ifosphamide plus melphalan for the first autograft and busulfan
plus thiotepa for the second. Hematological recovery was prompt, and no severe complications
were observed. They are doing well without evidence of recurrence at 19 and 49 months after the
second autograft, respectively.
PMID: 9722074, UI: 98387529