Renal medullary carcinoma: molecular, pathological and clinical evidence for treatment with topoisomerase‐inhibiting therapy

Schaeffer, Edward M.; Guzzo, Thomas J.; Furge, Kyle A.; Netto, George J.; Westphal, Michael; Dykema, Karl; Yang, Ximing J.; Zhou, Ming; Teh, Bin Tean; Pavlovich, Christian P.

doi:10.1111/j.1464-410x.2009.09139.x

Cited by 47 publications

(37 citation statements)

References 16 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The most significant clinical challenge presented by RMC is that chemo-, immuno-and radiotherapy have all been unsuccessful for the treatment of RMC, based on previous studies (4,5,(17)(18)(19)(20)(21)(22). Therefore, an early diagnosis may improve the survival rates of patients with RMC.…”

Section: Discussionmentioning

confidence: 99%

“…The histopathological features of RMC include epithelial cells with reticular, adenoid cystic plasia, and prominent inflammation (4).…”

Section: Introductionmentioning

confidence: 99%

“…At present, the prognosis for patients with RMC remains very poor, since~95% of cases present at an advanced stage at the time of diagnosis and the tumor is resistant to chemotherapy in addition to biological therapy (4)(5)(6). A mean survival of 19 weeks from the time of initial diagnosis of RMC was reported by Simpson et al (7).…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Clinical and computed tomography imaging features of renal medullary carcinoma: A report of six cases

et al. 2015

View full text Add to dashboard Cite

Abstract. Patients with renal medullary carcinoma (RMC) have a poor prognosis, usually due to late diagnosis. Computed tomography (CT) analysis may aid the differentiation between RMC and other types of renal cell carcinoma, in order to establish an accurate early diagnosis. There is a limited number of reports in the literature focusing on clinical and multi-slice CT (MSCT) imaging findings of RMC. Consequently, the present study aimed to characterize the clinical and MSCT imaging features of RMC. For this purpose, the MSCT imaging findings of 6 patients with RMC were retrospectively studied. The patients were subjected to MSCT in order to investigate the characteristics of the tumors, including location, size, density, calcification, cystic or solid appearance, capsule sign, enhancement pattern and presence of retroperitoneal lymph node metastasis. The tumors in the current study presented a mean diameter of 7.48±3.25 cm, and were observed to be solitary and heterogeneous with necrotic components. The majority of the tumors did not contain calcifications (5/6); displayed an ill-defined margin (4/6); were centered in the medulla; extended into the renal pelvis or peripelvic tissues (6/6); and did not exhibit a fibrous capsule. Localized caliectasis was observed in 3 of the 6 cases. The attenuation of the solid region of the RMC on unenhanced CT was equal to that of the renal cortex or medulla (42.3±2.7 vs. 40.7±3.6 and 41.2±3.9 Hounsfield units, respectively; P>0.05) while, on enhanced CT, the enhancement of the tumor was lower than that of the normal renal cortex and medulla during all phases (cortical phase, 52.6±4.8 vs. l99.5±9.7 and 72.7±6.4; medullary phase, 58.6±5.7 vs. 184.6±10.8 and 93.5±7.8; delayed phase, 56.8±6.1 vs. 175.7±8.5 and 96.5±7.9, respectively; P<0.05).In conclusion, RMC tends to be an infiltrative, ill-defined heterogeneous mass with intratumoral necrosis, which arises from the renal medulla, and displays lower enhancement than the renal cortex and medulla during all phases on enhanced CT. Despite its rarity in adults, RMC should be included in a differential diagnosis when CT imaging reveals these features.

show abstract

Section: Discussionmentioning

confidence: 99%

“…The histopathological features of RMC include epithelial cells with reticular, adenoid cystic plasia, and prominent inflammation (4).…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Clinical and computed tomography imaging features of renal medullary carcinoma: A report of six cases

et al. 2015

View full text Add to dashboard Cite

show abstract

“…58 The therapeutic experience with other subtypes of RCC, such as papillary RCC, is limited. 27,54,59 In the future, the stratification of subtypes of RCC and evaluation of responses of patients with different subtypes of RCC to targeted therapies will be critical for success of the targeted therapy.…”

Section: Resultsmentioning

confidence: 99%

Predictors of Response to Targeted Therapy in Renal Cell Carcinoma

Eisengart

MacVicar²,

Yang³

2012

Archives of Pathology &Amp; Laboratory Medicine

View full text Add to dashboard Cite

Context.—The prognosis for patients with metastatic renal cell carcinoma is poor, with an average 5-year survival of approximately 10%. Use of traditional cytokine therapy, specifically high-dose interleukin 2, is limited by significant toxicity. Better understanding of the molecular pathogenesis of renal cell carcinoma has led to the development of targeted therapies to inhibit specific cellular pathways leading to tumorigenesis. These drugs provide improved survival with a more favorable toxicity profile. There is ongoing investigation of markers that predict response of an individual patient to different targeted therapies. Objective.—To explain the molecular basis for vascular endothelial growth factor inhibitor (antiangiogenic) and mammalian target of rapamycin inhibitor therapies for renal cell carcinoma, summarize the clinical trials demonstrating the effectiveness of these drugs, and describe the biomarkers shown to correlate with outcome in patients treated with targeted therapy. Data Sources.—All included sources are from peer-reviewed journals in PubMed (US National Library of Medicine). Conclusion.—Emerging evidence shows promise that biomarkers will be useful for predicting an individual patient's response to targeted therapy, leading to a more personalized approach to treating renal cell carcinoma.

show abstract

“…A separate study identified the absence of SMARCB1/INI1 with loss of heterozygosity at the SMARCB1/INI1 gene locus on chromosome 22. 44 SMARCB1 is a tumor suppressor gene involved in chromatin remodeling, cell cycle control, and regulation of cytoskeletal dynamics. Finally, a study by Schaeffer et al 45 revealed high expression of topoisomerase II in a patient who achieved 9 months of complete remission with doxorubicin.…”

Section: Geneticsmentioning

confidence: 97%

Treating renal cell carcinoma in young adults: challenges and solutions

Matrana¹,

Dreisin²

2016

COAYA

View full text Add to dashboard Cite

Renal cell carcinoma (RCC) is uncommon in adolescents and young adults, although rare variants of the disease, including Xp11.2 translocation carcinoma, collecting duct carcinoma, and renal medullary carcinoma, occur with a higher preponderance in young patients. Likewise, non-RCC kidney tumors such as those of the Ewing sarcoma family of tumors are also seen in younger patients. The mainstay therapy for most forms of localized RCC and other kidney cancer is surgical removal of the tumors. Advances in treatments for metastatic clear cell carcinoma have been made in the last decade with the development of several new targeted agents. These therapies have revolutionized the treatment of metastatic clear cell RCC but are not targeted for other types of kidney cancer that are more often found in young patients. The management of RCC in adolescents and young adults remains a challenge for clinicians, but further advances are anticipated as less selective targeted immunotherapies become more widely available.

show abstract

Renal medullary carcinoma: molecular, pathological and clinical evidence for treatment with topoisomerase‐inhibiting therapy

Cited by 47 publications

References 16 publications

Clinical and computed tomography imaging features of renal medullary carcinoma: A report of six cases

Clinical and computed tomography imaging features of renal medullary carcinoma: A report of six cases

Predictors of Response to Targeted Therapy in Renal Cell Carcinoma

Treating renal cell carcinoma in young adults: challenges and solutions

Contact Info

Product

Resources

About