Renal medullary carcinoma
Renal medullary carcinoma | |
---|---|
Location of renal medulla. | |
Specialty | Oncology/nephrology |
Renal medullary carcinoma is a rare type of
Signs and symptoms
In a case series of 34 patients, Davis and colleagues reported the following signs and symptoms:[citation needed]
- macroscopically visible (gross) hematuria (60%)
- abdominal or back/flank pain (50%)
- significant weight loss (25%)
Other researchers have reported a palpable renal mass
Causes
The
Mechanism
The finding that virtually all people affected by renal medullary carcinoma carry at least one copy of the HbS mutation suggests that sickle cell trait somehow predisposes to this type of cancer.
Diagnosis
The diagnosis of renal medullary carcinoma is typically made after individuals with sickle cell trait present with the typical signs and symptoms outlined above, in combination with
Classification
Renal medullary carcinoma has been termed "the seventh
Prevention
Renal medullary carcinoma is extremely rare and it is not currently possible to predict those individuals with sickle cell trait who will eventually develop this cancer. It is hoped that early detection could result in better outcomes but screening is not currently feasible. With genomics and science advancing, we can possibly find these mutated genes with proactive genetic screening so that future generations don’t differ but science can interfere with its natural course to a more directed course, avoiding such mutations from manifesting and cause death in later generations.[citation needed]
Management
This cancer is typically
Prognosis
Since the cancer most often presents at an advanced stage, prognosis is generally very poor, with median survival times of 3 months (range 1–7 months).[5] Longer survival of beyond one year was reported in one patient[6] and of up to eight years in one individual whose tumor was well circumscribed and non-metastatic at the time of diagnosis,[10] suggesting that early detection could dramatically improve survival.
Epidemiology
As of 2009, there have been approximately 120 reported cases of renal medullary carcinoma.[11] In every instance except for one, the patients were positive for cell sickling. Wilms' tumor, the most common renal tumor of childhood, is responsible for 6-7% of childhood cancer whereas all remaining primary renal tumors (among which is included renal medullary carcinoma) collectively account for less than 1% of all childhood cancer and less than 10% of primary kidney tumors in childhood.[12]
History
Renal medullary carcinoma was first described as a clinicopathologic entity in 1995.[3]