Renal medullary carcinoma response to chemotherapy: a referral center experience in Brazil

Abstract

Renal medullary carcinoma (RMC) is rare, accounting for less than 1% of all renal neoplasms. Case reports suggest RMC is highly aggressive, poorly responsive to chemotherapy, often metastatic at diagnosis, affects young men with sickle cell trait, and median overall survival (mOS) is less than 12 months. We report the epidemiological characteristics, treatments performed, response rate to each treatment and mOS of five patients with RMC. All patients had sickle cell trait, four were male, three had metastatic disease at diagnosis and mean age at diagnosis was 25 years. Non-metastatic patients were submitted to nephrectomy. Two patients had partial response to first line chemotherapy including cisplatin and gemcitabine. There was no response to sunitinib or second line chemo - therapy; mOS was 6 months. Due to its rarity, case series are the only evidence available to discuss the treatment for RMC. In our experience, only cisplatin and gemcitabine based regimen offered response.

Keywords: carcinoma; kidney neoplasms; renal cell; renal medullary carcinoma; sickle cell trait.

Figure 1.

A) Sheets of cells with reticular growth pattern and a more compact adenoid cystic morphology (Hematoxylin & Eosin stain, Zeiss Axioshop 40, magnification×100). B) Sickled erythrocytes (Hematoxylin & Eosin stain, Zeiss Axioshop 40, magnification×400).

Figure 2.

A) Paraaortic lymphadenopathy, patient case #2. B) Response after platin-based chemotherapy. C) Mediastinal lymphadenopathy, patient case #2. D) Response after platin-based chemotherapy.

Figure 3.

A) Lung metastasis, patient case #3. B) Response after platin-based chemotherapy. C) Mediastinal lymphadenopathy, patient case #3. D) Response after platin-based chemotherapy.