Renal Medullary Carcinoma

Abstract

Renal medullary carcinoma is a recently recognized epithelial malignant tumor arising in the renal parenchyma. The tumor is almost exclusive to young black patients with the sickle cell trait. Most patients present with metastatic disease and have a poor prognosis. An Hispanic woman with renal medullary carcinoma who initially responded to chemotherapy with methotrexate, vinblastine, doxorubicin, and cisplatin and survived for 12 months is presented. The clinical, histologic, and radiologic features of this tumor are described, and chemotherapeutic regimens used in this disease are detailed. Treatment modalities have proved largely unsuccessful in the setting of advanced disease. Given the shared demographic, clinical, and radiographic features of these patients, awareness and early diagnosis may prove essential in improving survival.

Renal Medullary Carcinoma in an Adolescent With Unknown Sickle Cell Trait

“…Homozygous Hb SS disease is not associated with RMC [4]. In patients younger than 25 years, RMC has a higher prevalence in male sex (2-3:1, M:F); no sex differences have been observed in patients older than 25 years [5][6][7].…”

“…Satellite lesions are usually found in the renal cortex and soft perirenal tissues. Right kidney affection is more common [1,[5][6][7][8][9]. Pathology shows a solid tumor, with cells in a desmoplastic stroma, acute and chronic inflammatory infiltrate, necrosis, hemorrhagic foci, and drepanocytes (sickle cells).…”

Novel Patient Metastatic Pleural Effusion-Derived Xenograft Model of Renal Medullary Carcinoma Demonstrates Therapeutic Efficacy of Sunitinib

“…The patient was treated by chemotherapy, including bortezomib, paclitaxel/carboplatin/ bevacizumab, doxorubicin/bevacizumab, and sunitinib A B (Supplemental Table 1). Treatment regimens were chosen based on the patient's tumor pathology, previously reported regimens of RMC (6,(11)(12)(13)(14)(15)(16)(17), and internal tumor board discussions. Further details and rationale for each regimen are summarized in Supplemental Table 1.…”