Renal medullary carcinoma and sickle cell trait: A systematic review

Abstract

Sickle cell trait (SCT) carries a small risk of renal medullary carcinoma (RMC). We conducted a systematic literature review and reported new four RMC cases (total N = 217). Eighty eight percent had SCT and 8% had sickle cell disease; 50% were children. Males had 2.4× risk than females. Isolated hematuria or in combination with abdominal or flank pain was the presenting sign in 66% cases. Tumor-related mortality was 95%. Four non-metastatic patients were long-term disease-free survivors. Although risk appears to be very low, individuals with SCT should be informed about the low risk of RMC with the hope of early diagnosis. Hematuria should prompt immediate investigation.

Lung tumor in a young African American patient with sickle trait: Pieces of a puzzle

“…The most common metastatic sites include lymph nodes, lungs, liver, adrenal glands, and bone. [1] Some patients first present with signs of metastatic disease as was seen in our patient who presented with cough and shortness of breath.…”

“…Eighty-eight percent of the patients have sickle cell trait. [1] It has a poor prognosis with an overall survival of 4 months in patients with metastases and 17 months without. [2] Widely metastatic disease at the time of diagnosis is typical.…”

Renal medullary carcinoma: a nearly fatal malignancy specifically affecting patients with a so-called benign condition

“…The mean duration of life after surgery was 15 weeks. In 2015, Alvarez et al [3] published the first systematic review of the 217 RMC cases reported to date, 88·6% of which were associated with SCT. Median overall survival was 10 months.…”

“…Several studies have been published describing the role of surgery, chemotherapy, immunotherapy, stem cell transplant, or radiation in the treatment of RMC but have found unfavorable outcomes with an overall mortality of 95% [3,4]. The two most commonly used chemotherapy regimens have been the high-dose intensity methotrexate, vinblastine, doxorubicin, and cisplatin (MVAC) and paclitaxel, cisplatin or carboplatin, and gemcitabine (PCG).…”

“…Importantly, 94% of the cases presented as metastatic disease, with most common sites being the regional lymph nodes, adrenal glands, and lung. Most case reports to date have had prolonged duration of symptoms before diagnosis, with an average of 5 months [3].…”