Renal Medullary Carcinoma The Seventh Sickle Cell Nephropathy

Davis, Charles J.; Mostofi, F. K.; Sesterhenn, Isabell A.

doi:10.1097/00000478-199501000-00001

Cited by 524 publications

(352 citation statements)

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“…Mariñ o-Enríquez et al 2 classified their tumor as renal medullary carcinoma (RMC); we 1 did not label ours as RMC because it lacked extreme clinical aggressiveness characteristic of RMC. 8,9 Retention of INI1 by immunohistochemistry was also not in favor of RMC. 10 In a retrospect, both pediatric cases shared all clinicopathologic characteristics and the same VCL-ALK fusion.…”

mentioning

confidence: 79%

Reply to ‘Distinct ALK-rearranged and VCL-negative papillary renal cell carcinoma variants in two adults without sickle cell trait'

Debelenko

2013

Modern Pathology

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confidence: 79%

Reply to ‘Distinct ALK-rearranged and VCL-negative papillary renal cell carcinoma variants in two adults without sickle cell trait'

Debelenko

2013

Modern Pathology

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“…6 Patients with sickle cell trait have a higher incidence of gross hematuria than patients with sickle cell disease. 2 The gross hematuria can be painless and is thought to arise from bleeding immediately beneath the renal pelvic epithelium 2 or as a result of papillary necrosis. 6 It involves the left kidney in Ͼ80% of patients.…”

Section: Discussionmentioning

confidence: 99%

“…Recently, a new entity, renal medullary carcinoma, has been described in young people, including children, with sickle cell disorders. 2 Many of the reported cases have been diagnostic challenges with poor prognoses. It is unclear whether earlier diagnosis and earlier treatment will result in a better prognosis, but awareness of this newly described entity is most important.…”

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confidence: 99%

Renal Medullary Carcinoma in an Adolescent With Sickle Cell Trait

Warren

Gidvani-Diaz²,

Duval-Arnould³

1999

Pediatrics

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ABSTRACT.We describe the complex presentation of a patient with renal medullary carcinoma, a newly described entity primarily affecting young patients with sickle cell trait. Renal medullary carcinoma is an aggressive, rapidly destructive tumor associated with a delayed diagnosis and a poor outcome. The most common presenting signs and symptoms include hematuria, abdominal or flank pain, and weight loss. Sickle cell trait as the sole cause of hematuria in young black patients is a diagnosis of exclusion. Hemoglobin electrophoresis, intravenous pyelography, and computed tomography scans should be the minimal studies performed in young black patients with hematuria. Pediatrics 1999;103(2). URL: http://www.pediatrics.org/cgi/content/full/103/2/e22; sickle cell trait, hematuria, renal medullary carcinoma, renal tumors.ABBREVIATION. CT, computed tomography. R enal tumors represent 7.8% of all pediatric malignancies in the United States.1 Most of these are Wilms' tumors affecting children Ͻ5 years of age. Recently, a new entity, renal medullary carcinoma, has been described in young people, including children, with sickle cell disorders.2 Many of the reported cases have been diagnostic challenges with poor prognoses. It is unclear whether earlier diagnosis and earlier treatment will result in a better prognosis, but awareness of this newly described entity is most important. We describe an 18-year-old female with sickle cell trait who was diagnosed with renal medullary carcinoma after a complex presentation. CASE PRESENTATIONW.B. was an 18-year-old black female with known sickle trait, who was in good health until 6 months before admission when she developed swelling and stiffness in her metacarpophalangeal and knee joints. Her initial evaluation was significant for microscopic hematuria and a positive antinuclear antibody test with a titer of 1:640 and a diffuse pattern. She was treated with oral salicylates and naproxen sodium in the 2 months before this admission, but she took them infrequently.Two months after her initial presentation, she developed daily nausea and bilious vomiting, resulting in a 20-lb weight loss (15% of total body weight) over a 3-week period. She sought medical care when she developed worsening right-sided flank pain and gross hematuria. She was admitted with a presumptive diagnosis of papillary necrosis. Her review of systems at the time of admission was also significant for fever, sharp intermittent periumbilical pain, midline back pain, right scapular pain, urinary frequency with nocturia, gross hematuria with clots, loose stools, and night sweats. She denied dysuria. The patient had a past medical history of hypertension of unknown cause (untreated), epistaxis, easy bruising, and vaginal yeast infections. She had been sexually active for 4 years.At the time of physical examination, the patient was well developed and appeared well nourished despite her weight loss. She was bent over, secondary to pain. Her vital signs were significant for a blood pressure of 158/87 mm Hg. She had two café ...

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“…Renal medullary carcinoma (RMC) is a very rare form of RCC, which predominantly affects AfricanAmerican children and young adults with sickle cell trait or sickle cell disease [70,71]. Patients present clinically with high stage tumor and succumb to disease within a few weeks to months.…”

Section: Clinical Presentationmentioning

confidence: 99%