Shetty Aditya, Matrana Marc R
Department of Genitourinary Medical Oncology, University of Texas MD Anderson Cancer Center, Houston, TX.
Department of Hematology and Oncology, Ochsner Clinic Foundation, New Orleans, LA.
Ochsner J. 2014 Summer;14(2):270-5.
Renal medullary carcinoma (RMC) is an aggressive form of non-clear cell kidney cancer that typically affects young adults and is almost exclusively associated with sickle cell trait. Typical RMC patients tend to be young black males (2:1 male to female predominance) with sickle cell trait who present with pain and hematuria and are found to have metastatic disease at diagnosis. Prognosis is extremely poor, with a mean survival of less than a year in most cases.
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.
Neither systemic therapy nor radiation therapy has been found to be particularly efficacious in the treatment of RMC. As the underlying biologic mechanisms of RMC are further elucidated on a basic science level, we expect new translational research opportunities will emerge and novel treatment options will be developed for this rare and dismal disease.
肾髓质癌（RMC）是一种侵袭性非透明细胞肾癌，通常影响年轻成年人，几乎仅与镰状细胞性状相关。典型的RMC患者往往是具有镰状细胞性状的年轻黑人男性（男女比例为2:1），表现为疼痛和血尿，诊断时已发现有转移性疾病。预后极差，大多数情况下平均生存期不到一年。
我们报告一例具有许多RMC典型表现的病例。一名有镰状细胞性状病史的25岁女性因右侧胁腹疼痛就诊于急诊室。最初，计算机断层扫描（CT）显示右肾仅有一个直径4.5厘米、外观良性的囊性病变。六个月后，患者再次出现血尿、头晕和眩晕。复查CT扫描显示肾囊肿已增大至5.6厘米。患者接受了囊肿剥除和电灼术，结果显示囊肿壁有恶性病变。她接受了一系列治疗，但病情仍进展，在转移性诊断后2年零8个月去世。
在RMC的治疗中，尚未发现全身治疗或放射治疗特别有效。随着RMC潜在生物学机制在基础科学层面得到进一步阐明，我们预计将出现新的转化研究机会，并为这种罕见且预后不良的疾病开发新的治疗选择。
