Renal Medullary Carcinoma: The Bronx Experience

Hakimi, A. Ari; Koi, Phillip T.; Milhoua, Paul M.; Blitman, Netta M.; Li, Maomi; Hugec, Vladimir; Dutcher, Janice P.; Ghavamian, Reza

doi:10.1016/j.urology.2007.06.1124

Cited by 83 publications

(75 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Unclassified

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%

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

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%

“…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%

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

“…On this basis the tumor, which was termed medullary carcinoma on morphologic grounds, was designated the seventh sickle cell nephropathy (the others being gross hematuria, papillary necrosis, nephrotic syndrome, renal infarction, inability to concentrate urine and pyelonephritis); 23 since the original study over 160 cases have been reported. [24][25][26][27][28][29][30][31][32][33] Patient age at diagnosis ranges from 5 to 69 years with a mean age of 19 years. There is a male predominance with a male/female ratio of 2:1, although for patients o10 years the male/female ratio is 5:1.…”

Section: Renal Medullary Carcinomamentioning

confidence: 99%

Uncommon and recently described renal carcinomas

2009

View full text Add to dashboard Cite

Major consensus conferences held over a decade ago laid the foundations for the current (2004) WHO classification of renal carcinoma. Clear cell, papillary and chromophobe carcinomas account for 85-90% carcinomas seen in routine practice. The remaining 10-15% of carcinomas consist of rare sporadic and hereditary tumors, some of which had been long recognized, but many of which only emerged as distinct entities in the decade leading up to the WHO publication. Collecting-duct carcinoma is a rare, often lethal form of carcinoma. Medullary carcinoma associated with sickle cell trait, has emerged as a distinctive tumor showing some overlapping features with upper tract urothelial carcinoma. Mucinous tubular and spindle-cell carcinoma and tubulocystic carcinoma were earlier considered as patterns of low-grade collecting-duct carcinoma, but are now recognized as separate tumor entities. Carcinomas associated with somatic translocations of TFE3 and TFEB comprise a significant proportion of pediatric renal carcinomas. Oncocytoid renal carcinomas in neuroblastoma survivors was recognized as a unique tumor category in the WHO classification. Renal carcinoma associated with end-stage renal disease is now recognized as having distinct morphological patterns and behavior. In addition there is a group of rare recently described carcinomas, including clear cell papillary carcinoma, oncocytic papillary renal cell carcinoma, follicular renal carcinoma and leiomyomatous renal cell carcinoma. It behooves the surgical pathologist to not only be capable of diagnosing the common forms of renal cancer, but also to be aware of the rare types of renal carcinoma, many of which have emerged in recent years.

show abstract

“…Most cases of RMC have proved resistant to standard treatment modalities, including various combinations of surgery, chemotherapy, immunotherapy, and radiation. [37][38][39][40] The average life expectancy from the time of diagnosis is 4 months. 41 …”

Section: Renal Medullary Carcinoma Backgroundmentioning

confidence: 99%

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: The Bronx Experience

Cited by 83 publications

References 17 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

Uncommon and recently described renal carcinomas

Treating renal cell carcinoma in young adults: challenges and solutions

Contact Info

Product

Resources

About