Extra‐renal clear cell renal cell carcinoma probably arising from mesodermal embryonic remnants

Terada, Tadashi

doi:10.1111/j.1440-1827.2011.02780.x

Cited by 19 publications

(25 citation statements)

References 5 publications

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“…Seven patients had surgery to treat a solitary metastatic lesion, with no additional systemic treatment provided. 8,9,11,[15][16][17] Eleven patients described in 8 case reports received targeted therapies with sunitinib (n ¼ 6), 6,7,10,18,19 pazopanib (n ¼ 2), 14,19 temsirolimus (n ¼ 2), 12 and everolimus (n ¼ 1). 13 Efficacy data showed that 8 (72%) of 11 patients responded to treatment, and 9 (82%) of 11 were alive when the cases were reported, surviving between 6 weeks and 36 months.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, some CUP-mRCC might have metastasized early; the primary tumors were too small for initial detection but became visible on repeated scans during follow-up, as was the case in one of the patients in this study. Another explanation is that the metastasis we found was actually a primary cancer in rudimentary embryonic mesonephric remnants 15 or was ectopic kidney tissue. 16 This embryonic explanation is well described in testicular cancer, where it is known as extragonadal germ-cell tumors.…”

Section: Anders Overby Et Almentioning

confidence: 91%

“…This entity has only rarely been described in the literature. [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Renal-cell carcinoma (RCC) is a group of cancers arising from the nephron, where 70% of RCC are clear-cell RCC. Because of mutation in the Von Hippel-Lindau gene, a high amount of vascular endothelial growth factor (VEGF) is produced, resulting in increased angiogenesis, cell growth, metabolism, and immunesuppression.…”

Section: Introductionmentioning

confidence: 99%

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Carcinoma of Unknown Primary Site (CUP) With Metastatic Renal-Cell Carcinoma (mRCC) Histologic and Immunohistochemical Characteristics (CUP-mRCC): Results From Consecutive Patients Treated With Targeted Therapy and Review of Literature

Øverby

Duval

Ladekarl

et al. 2019

Clinical Genitourinary Cancer

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Diagnostic assessment occasionally presents carcinoma of unknown primary site (CUP) with metastatic renalcell carcinoma (mRCC) histologic and immunohistochemical characteristics (CUP-mRCC). We reviewed our single-institution experience and searched the PubMed database, and identified 10 patients treated at our institution; 60% were poor risk patients. Objective response rate was 40%, progression-free survival was 2.5 months, and overall survival was 5.7 months; and the literature search identified 42 CUP-mRCC cases, for a total of 52 cases of CUP-mRCC. Vascular endothelial growth factoretargeted therapy is valid, feasible, and safe in CUP-mRCC, and these patients should thus be identified among CUP patients for specific renal-cell carcinoma therapy. Background: Carcinoma of unknown primary site (CUP) is a heterogenous group of metastatic cancer with no detectable primary tumor site. Diagnostic assessment occasionally presents CUP with metastatic renal-cell carcinoma (mRCC) histologic and immunohistochemical characteristics (CUP-mRCC). Efficacy and toxicity data for vascular endothelial growth factor inhibitor therapies in CUP-mRCC patients are few. Patients and Methods: We retrospectively reviewed consecutive patients with CUP-mRCC at a single institution between 2007 and 2018. Treatment outcomes were assessed from initiation of renal-cell carcinomaespecific therapy, including response rate, progression-free survival, and overall survival. Results: Ten patients with CUP-mRCC were identified. Median age was 64 years. Histologies were clear-cell (30%), papillary type II (20%), and unclassified renal-cell (50%) carcinoma. International Metastatic Renal Cell Carcinoma Database Consortium risk group were favorable, intermediate, and poor in 0, 40%, and 60%, respectively. One patient received empiric first-line chemotherapy. Targeted treatments were pazopanib (n ¼ 7), sunitinib (n ¼ 2), and sorafenib (n ¼ 1). Objective response rate was 40%, progression-free survival was 2.5 months (95% confidence interval, 1.2-3.8), and overall survival was 5.7 months (95% confidence interval, 0-24.0). Stratified for International Metastatic Renal Cell Carcinoma Database Consortium risk, overall survival in intermediate versus poor risk group were 18.6 months and 2.3 months, respectively. Second-line therapy did not result in disease control. No new or unexpected toxicities were observed. Conclusion: CUP-mRCC treated with vascular endothelial growth factoretargeted therapy is valid, feasible, and safe even though these patients had several negative prognostic factors. CUP-mRCC patients should be identified among CUP patients for specific renal-cell carcinoma therapy.

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Section: Discussionmentioning

confidence: 99%

Section: Anders Overby Et Almentioning

confidence: 91%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Carcinoma of Unknown Primary Site (CUP) With Metastatic Renal-Cell Carcinoma (mRCC) Histologic and Immunohistochemical Characteristics (CUP-mRCC): Results From Consecutive Patients Treated With Targeted Therapy and Review of Literature

Øverby

Duval

Ladekarl

et al. 2019

Clinical Genitourinary Cancer

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“…Renal cell carcinomas have been sporadically described in the literature to occur in ectopic,[ 3 ] and supernumerary kidneys,[ 4 ] however only a single published report describes this entity in the presence of normal bilateral kidneys. [ 2 ] Extra-renal RCC refers to the occurrence of RCC in locations other than the normal native kidneys, likely arising from mesonephric remnants. [ 2 ] We report this first case of RCC masquerading as an adrenal mass, in the presence of normal bilateral kidneys.…”

Section: Discussionmentioning

confidence: 99%

“…[ 1 ] Extra-renal localization of RCC, with intact bilateral kidneys, is an exceptionally rare occurrence and has been reported only once before in English literature. [ 2 ] Herein, we describe an interesting and rare case of extra-renal RCC, camouflaging as an adrenal mass.…”

Section: Introductionmentioning

confidence: 99%

Primary extra-renal clear cell renal cell carcinoma masquerading as an adrenal mass: A diagnostic challenge

et al. 2015

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We present the first case of a nonmetastasizing renal cell carcinoma (RCC) masquerading as an adrenal mass, in the presence of normal bilateral native kidneys, in a young adult. The possibility of this mass developing in a supernumerary kidney was ruled out, since no identifiable renal tissue, pelvis or ureters was seen within the mass, nor was any separate systemic arterial supply to the mass seen. The diagnosis of extra-renal clear cell RCC was based on cyto-morphological features, further confirmed by immunohistochemistry findings. The origin of this extra-renal clear cell renal cell is proposed to be from the mesodermal embryonic rests.

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Metastatic renal cell carcinoma without evidence of a renal primary

et al. 2015

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Purpose Metastatic renal cell carcinoma (RCC), without an identified kidney primary, has been reported rarely. We report a patient with RCC metastatic to bilateral adrenal glands and liver, without an apparent renal primary. We detail the immunohistochemical and molecular studies employed to substantiate the diagnosis of RCC and direct therapy. Methods Histopathologic findings were correlated with imaging data and supplemented by a panel of immunohistochemical stains, as well as tumor sequence analysis. Results Despite the presence of bilateral adrenal masses and lack of tumor within kidney parenchyma, the diagnosis of RCC was substantiated by immunohistochemistry (RCC+/PAX2+/PAX8+/Melan-A−/SF-1− among others) and molecular genetic analysis, harboring mutations in VHL, TP53, KDM5C, and PBRM1. After debulking surgery, based on the diagnosis of RCC and the molecular profile, the patient was treated with a tyrosine kinase inhibitor (sunitinib), resulting in stablilization of disease. Conclusions This case illustrates the role of mutational analysis in carcinomas with rare or unusual presentations, such as metastatic RCC without a renal primary.

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Extra‐renal clear cell renal cell carcinoma probably arising from mesodermal embryonic remnants

Cited by 19 publications

References 5 publications

Carcinoma of Unknown Primary Site (CUP) With Metastatic Renal-Cell Carcinoma (mRCC) Histologic and Immunohistochemical Characteristics (CUP-mRCC): Results From Consecutive Patients Treated With Targeted Therapy and Review of Literature

Carcinoma of Unknown Primary Site (CUP) With Metastatic Renal-Cell Carcinoma (mRCC) Histologic and Immunohistochemical Characteristics (CUP-mRCC): Results From Consecutive Patients Treated With Targeted Therapy and Review of Literature

Primary extra-renal clear cell renal cell carcinoma masquerading as an adrenal mass: A diagnostic challenge

Metastatic renal cell carcinoma without evidence of a renal primary

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