Treatment of adult rhabdoid renal cell carcinoma with sorafenib

Abstract

631operating room notes, pathological records and other relevant documents were used as a resource to compile information on this case. We also included recent assessments of the patient in this report. A literature review was conducted using a search of EMBASE, MEDLINE/PubMed and the Cochrane Database. We used the search terms "rhabdoid," "renal cell carcinoma," "adult" and "tumor." References of articles found were also searched for relevant articles. CaseA 47-year-old woman presented to her family physician with a 3-month history of leftsided flank pain and nausea. On physical examination a hard palpable mass was found in her abdomen. Her past medical history included hypertension, migraine headaches, anemia and a previous right inguinal hernia. She had no family history of urological malignancies, but had a paternal cousin with colon cancer and paternal uncle with lung cancer. The patient was a lifelong nonsmoker.Ultrasonography of her abdomen revealed a complex hypervascular mass in the upper pole of her left kidney measuring 11.1 × 11.8 × 7.5 cm. The mass enlarged the left kidney to 16.0 cm in length. The right kidney appeared to be healthy. A contrast-enhanced computed tomograpy scan showed a hypervascular tumour in her left kidney infiltrating the left psoas and spleen, with evidence of retroperitoneal lymphadenopathy and tumour thrombus extending to the infrahepatic vena cava. Lesions highly suspected as metastastic deposits were found in the liver. A chest radiograph showed multiple nodules in both lung fields, also suggestive of metastatic disease. A bone scan revealed an ill-defined area in the left sixth rib and abnormal uptake in both distal femurs, consistent with metastasis to these sites. Treatment of adult rhabdoid renal cell carcinoma with sorafenib CASE REPORT AbstractRenal cell carcinoma (RCC) with rhabdoid features is an uncommon and highly aggressive malignancy. We report a case of adult clear-cell RCC with extensive rhabdoid features treated with the tyrosine kinase inhibitor sorafenib. A review of the literature summarizes important aspects of this malignancy. We discuss clinical and histological findings as well as the patient's response to sorafenib therapy.

Review of renal cell carcinoma with rhabdoid features with focus on clinical and pathobiological aspects

“…Reported imaging features are non-specific and consist of a complex hypervascular mass on ultrasonographic examination [9]. On computed tomography (CT) examinations, a heterogeneous, irregular mass which may invade the surrounding tissue is encountered [9,11].…”

“…Some cases responding to tyrosine kinase inhibitors (TKI) (sorafenib and sunitinib) have been reported [9,52]. Stronger expression of VEGF-A in the rhabdoid component than the clear cell RCC component may suggest a better response to TKI [52].…”

“…Contrast enhancement on CT is frequently observed [4]. In some tumors, lymphadenopathy may be radiologically detected [9,10,13].…”

“…Patients with RCC with rhabdoid features frequently present with an abdominal mass, hematuria, flank or abdominal pain and nausea [2,4,5,[9][10][11][12]. However, some primary as well as metastatic lesions may be incidentally discovered on radiological examinations [4,5,13,14].…”

“…On computed tomography (CT) examinations, a heterogeneous, irregular mass which may invade the surrounding tissue is encountered [9,11]. Contrast enhancement on CT is frequently observed [4].…”