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

Goenaga-Vázquez, Yamila; Colón, Gloria; Barrios, Nilka J.; Corrêa, Maria Salete Nahás Pires

doi:10.1007/s13730-018-0308-3

Cited by 6 publications

(3 citation statements)

References 15 publications

(19 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…To date, platinum-based chemotherapy is the recommended initial standard of care for patients diagnosed with RMC, given the rapid progression that can be seen in these patients [42]. This includes high-dose intensity MVAC (a combination of methotrexate, vinblastine, doxorubicin, and cisplatin) [28,43] and PCG (paclitaxel, cisplatin or carboplatin, and gemcitabine) [44][45][46], which are regimens used in urothelial carcinoma [47]. Following upfront chemotherapy, surgery is considered-particularly for those who have responded to chemotherapy.…”

Section: Therapeutic Considerations From Biologymentioning

confidence: 99%

Recent Advances in Renal Medullary Carcinoma

Hong

2022

IJMS

View full text Add to dashboard Cite

Renal medullary carcinoma (RMC) is a rare renal malignancy that has been associated with sickle hemoglobinopathies. RMC is aggressive, difficult to treat, and occurs primarily in adolescents and young adults of African ancestry. This cancer is driven by the loss of SMARCB1, a tumor suppressor seen in a number of primarily rare childhood cancers (e.g., rhabdoid tumor of the kidney and atypical teratoid rhabdoid tumor). Treatment options remain limited due in part to the limited knowledge of RMC biology. However, significant advances have been made in unraveling the biology of RMC, from genomics to therapeutic targets, over the past 5 years. In this review, we will present these advances and discuss what new questions exist in the field.

show abstract

Section: Therapeutic Considerations From Biologymentioning

confidence: 99%

Recent Advances in Renal Medullary Carcinoma

Hong

2022

IJMS

View full text Add to dashboard Cite

show abstract

“…Most people with sickle cell trait live asymptomatically. Despite SCT being perceived as an asymptomatic condition several case reports and reviews reported an increased incidence of renal medullary carcinoma among young patients with SCT with the age wide from 9 to 69 years (12)(13)(14). Other forms of SCT include HbAC, and HbAE although these are rare forms (9).…”

Section: Introductionmentioning

confidence: 99%

Determination of birth prevalence of sickle cell disease using point of care test HemoTypeSC TM at Rundu hospital, Namibia

Mano,

Kuona,

Misihairabgwi

2023

Preprint

View full text Add to dashboard Cite

Background: Sickle cell disease (SCD), a non-communicable disease has its highest burden in Sub Saharan Africa. The majority of children (50-90%), with SCD die before their 5th birthday with approximately 150,000–300,000 annual SCD child deaths in Africa. In developed countries, newborn screening (NBS) has been shown to improve survival of children with sickle cell disease with under 5 childhood mortality reduced 10 fold due to interventions done before development of complications. Point of care tests have been developed for resource limited settings to expand newborn screening. The aim of the study was to determine the birth prevalence of sickle cell disease using the point of care test HemoTypeSC in Namibia. Methods: A cross sectional descriptive study was carried out at Rundu Intermediate hospital in Kavango East Region. Two hundred and two (202) well newborns within 72 hours of birth were recruited in the study from 22 of February to the 28th of March 2023. Descriptive statistics was used to compute the hemoglobin types of the study participants. Results: The majority of the participants (n=105) (52%) were females and (n= 97) ,(48%) males. The median age of the participants was 23 hours interquartile range (IQR), (11-33 hours) with the age range of 2-98 hours. One hundred and eight three (183) ( 90.6%) had normal haemoglobin HbAA; 19 (9.4%) sickle cell trait (HbAS) and no participant was found to have sickle cell disease (HbSS). Conclusions: The study is the first to carry out birth prevalence for SCD and sickle cell trait as well as the first application of HemotypeSC as screening method in Namibia. There was a high prevalence of sickle cell trait but no SCD. This is a baseline study that can inform policy on the possible adoption of sickle cell disease newborn screening in Namibia.

show abstract

“…Most people with sickle cell traits live asymptomatically. Despite SCT being perceived as an asymptomatic condition, several case reports and reviews have reported an increased incidence of renal medullary carcinoma among young patients with SCT aged 9 to 69 years [12][13][14]. Other forms of traits include HbAC and HbAE, although these are rare forms [9].…”

mentioning

confidence: 99%

Determination of birth prevalence of sickle cell disease using point of care test HemotypeSC™ at Rundu Hospital, Namibia

Mano,

Kuona,

Misihairabgwi

2024

BMC Pediatr

View full text Add to dashboard Cite

Background Sickle cell disease (SCD), a noncommunicable disease, has the greatest burden in sub-Saharan Africa. The majority of children (50–90%) with SCD die before their 5th birthday, with approximately 150,000–300,000 annual SCD child deaths in Africa. In developed countries, newborn screening (NBS) has been shown to improve the survival of children with sickle cell disease, with under5 childhood mortality reduced tenfold due to interventions performed before the development of complications. Point –of-care tests have been developed for resource limited settings to expand NBS. The aim of this study was to determine the birth prevalence of sickle cell disease in Namibia using the HemoTypeSC™ point-of-care test. Methods A cross-sectional descriptive study was carried out at Rundu Intermediate Hospital in the Kavango East Region. Two hundred and two (202) well newborns within 72 h of birth were recruited for the study from 22 February to the 23th March 2023. Descriptive statistics were used to compute the haemoglobin types of the study participants. Results The majority of the participants (n = 105, 52%) were females, and (n = 97,48%) were males. The median age of the participants was 23 h (Q1, Q3; 11; 33),) with an age range of 2–98 h. Sickle cell trait was present in 9.4% of the screened newborns, no homozygous disease was detected, and 90.6% had Hb AA. Conclusions This study is the first to measure HbS gene carriage at birth using HemotypeSC point-of-care testing in Namibia. There was a moderate prevalence of sickle cell traits but no SCD. This baseline study may provide the foundation for larger epidemiological surveys to map HbS gene carriage in Namibia to provide evidence for policy makers to fashion appropriate SCD newborn screening services.

show abstract

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

Cited by 6 publications

References 15 publications

Recent Advances in Renal Medullary Carcinoma

Recent Advances in Renal Medullary Carcinoma

Determination of birth prevalence of sickle cell disease using point of care test HemoTypeSC TM at Rundu hospital, Namibia

Determination of birth prevalence of sickle cell disease using point of care test HemotypeSC™ at Rundu Hospital, Namibia

Contact Info

Product

Resources

About