Renal Medullary Carcinoma Response to Chemotherapy: a Referral Center Experience in Brazil
Marina Cavalcanti Maroja Silvino, Camila Motta Venchiarutti Moniz, Gustavo Henrique Munhoz Piotto,Sheila Siqueira,Ariel Galapo Kann, and Carlos Dzik1
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.
Key words: kidney neoplasms, renal medullary carcinoma, carcinoma, renal cell, sickle cell trait.
Renal Medullary Carcinoma: CT and MRI Features
Netta M. Blitman1 Robert G. Berkenblit Alla M. Rozenblit2 Terry L. Levin1
Blitman NM, Berk
Abstract
Reviewing the cross-sectional imaging findings of six cases of pathologically proven renal medullary carcinoma in patients with sickle cell trait. MRI findings were available in three of the patients. To our knowledge, only one previous report has addressed MRI features of this rare disease.
Renal Medullary Carcinoma; A Rare Entity
Selahattin Çalışkan, MD, Ali Murat Gökçe, MD, Gülistan Gümrükçü, MD, and Mine Önenerk, MD
Abstract
Renal medullary carcinoma (RMC) is an uncommon aggressive neoplasm of the kidney. RMC is biologically aggressive with a very poor prognosis, and metastasis is seen in up to 95% of the patients at diagnosis or shortly thereafter. The common sites of metastasis are respectively lymph nodes, lungs, livers, and adrenal glands in order of frequency. The presence of poorly differentiated eosinophilic cells in a characteristic fibro-inflammatory stroma is seen in histological examination. The origin and pathogenesis of RMC are unclear. The radiographical and pathological findings suggest that RMC probably originates in the calyceal epithelium in or near the renal papillae, which could be the result of chronic ischemic damage in the renal papillae epithelium by sickled erythrocytes. Positivity of VEGF and HIF-1α supports the chronic hypoxia that may be caused in the pathogenesis of RMC. Other factors such as genetic or environmental factors are important. Although hemoglobinopathy is very common, RMC is very rare. An understanding of the molecular and genetic factors of this rare disease is important for its prevention and treatment. We herein describe an adult Turkish patient, who presented with hematuria. The diagnosis was RMC after pathological examination.
Clinical and computed tomography imaging features of renal medullary carcinoma: A report of six cases
Zhenshan Shi, Qian Zhuang, Ruixiong You, Yueming Li, Jian Li, Dairong Cao
Abstract
Patients with renal medullary carcinoma (RMC) have a poor prognosis, usually due to late diagnosis. Computed tomography (CT) analysis may aid the differentiation between RMC and other types of renal cell carcinoma, in order to establish an accurate early diagnosis. There is a limited number of reports in the literature focusing on clinical and multi‑slice CT (MSCT) imaging findings of RMC. Consequently, the present study aimed to characterize the clinical and MSCT imaging features of RMC. For this purpose, the MSCT imaging findings of 6 patients with RMC were retrospectively studied. The patients were subjected to MSCT in order to investigate the characteristics of the tumors, including location, size, density, calcification, cystic or solid appearance, capsule sign, enhancement pattern and presence of retroperitoneal lymph node metastasis. The tumors in the current study presented a mean diameter of 7.48±3.25 cm, and were observed to be solitary and heterogeneous with necrotic components. The majority of the tumors did not contain calcifications (5/6); displayed an ill‑defined margin (4/6); were centered in the medulla; extended into the renal pelvis or peripelvic tissues (6/6); and did not exhibit a fibrous capsule. Localized caliectasis was observed in 3 of the 6 cases. The attenuation of the solid region of the RMC on unenhanced CT was equal to that of the renal cortex or medulla (42.3±2.7 vs. 40.7±3.6 and 41.2±3.9 Hounsfield units, respectively; P>0.05) while, on enhanced CT, the enhancement of the tumor was lower than that of the normal renal cortex and medulla during all phases (cortical phase, 52.6±4.8 vs. l99.5±9.7 and 72.7±6.4; medullary phase, 58.6±5.7 vs. 184.6±10.8 and 93.5±7.8; delayed phase, 56.8±6.1 vs. 175.7±8.5 and 96.5±7.9, respectively; P<0.05). In conclusion, RMC tends to be an infiltrative, ill‑defined heterogeneous mass with intratumoral necrosis, which arises from the renal medulla, and displays lower enhancement than the renal cortex and medulla during all phases on enhanced CT. Despite its rarity in adults, RMC should be included in a differential diagnosis when CT imaging reveals these features.
Renal Medullary Carcinoma: A Case Report and Brief Review of the Literature
Aditya Shetty, MD and Marc R. Matrana, MD, MS
Abstract
We present a case illustrative of many of the classic findings of RMC. A 25-year-old female with a past medical history of sickle cell trait presented to the emergency room with right-sided flank pain. Initially, a computed tomography (CT) scan showed only a benign-appearing cystic lesion 4.5 cm in diameter in the right kidney. Six months later, the patient re-presented with hematuria, lightheadedness, and dizziness. A repeat CT scan showed that the renal cyst had enlarged to 5.6 cm. The patient underwent a decortication and fulguration procedure that revealed malignancy in the wall of the cyst. She was given a series of treatments but experienced progressive disease and passed away 2 years and 8 months after metastatic diagnosis.
Renal Medullary Carcinoma: A Case Report and Brief Review of the Literature
Aditya Shetty, MD and Marc R. Matrana, MD, MS
Abstract
Renal medullary carcinoma (RMC) is uncommon yet an aggressive form of renal cancer. Most patients present with metastatic disease. Systemic therapies recommended for clear cell carcinomas have not been well investigated for RMC. This report illustrates a case of a young female diagnosed with metastatic RMC and treated with 4 lines of systemic therapy. She remains alive and maintaining an excellent quality of life more than 3 years after the start of systemic therapy.
Question of the week?
What is gene therapy and how can it help patients with Sickle Cell Disease?
Email your answers to office@rmcresearchfoundation.com
