Renal Medullary Carcinoma: Case Report of an Aggressive Malignancy with Near-Complete Response to Dose-Dense Methotrexate, Vinblastine, Doxorubicin, and Cisplatin Chemotherapy

Amjad, Ali Imran; Ali, Hira; Appleman, Leonard Joseph; Maranchie, Jodi K.; Jackman, Stephen V.; Parwani, Anil V.; Dhir, Rajiv; Parikh, Rahul Atul

doi:10.1155/2014/615895

Cited by 7 publications

(4 citation statements)

References 23 publications

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Section: Discussionmentioning

confidence: 99%

“…Recently, Amjad et al [21] reported a case where a 23-year-old African American patient with sickle cell anemia had a near complete clinical and pathological response to ddMVAC. This particular patient had six cycles of neoadjuvant ddMVAC with great tolerance and no need of hospitalization, and then proceeded with a total nephrectomy with retroperitoneal lymphadenectomy, showing a near complete pathological response, developing recurrence only 16 months later [21]. Another case report from Alabama University described an 11-year-old African American patient who was submitted to three cycles of neoadjuvant carboplatin, gemcitabine and paclitaxel, followed by a right nephrectomy with an aortocaval lymphadenectomy and multiple nodule resections of the lungs, with no evidence of diseases on the surgical specimens of the lymph nodes and pulmonary nodules.…”

Section: Discussionmentioning

confidence: 99%

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RMC (Renal Medullary Cancer): A Case Report and Literature Review

Taranto¹,

Mariano²,

Eiger³

et al. 2016

JPP

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RMC (renal medullary carcinoma) is considered a rare entity, corresponding to approximately 2% of primary kidney tumors, usually occurring in the second decade of the patient's life and it is closely related with previous history of sickle cell disease. Studies have reported RMC as highly aggressive, poorly responsive to chemotherapy, surgery, radiotherapy or targeted therapy. This disease's median overall survival is less than 12 months. We are reporting a case of a 21-year-old woman with sickle cell trait, whose initial clinical presentation was RMC already metastatic to the liver. She underwent total right nephrectomy, and her clinical picture worsened during hospitalization with pneumonia, bowel obstruction, oliguria and death in less than two months after diagnosis, with no performance status to attempt any treatment options to her cancer at any time. Although some cases in the literature had reported good response to neoadjuvant therapy, this disease has early recurrence and little response to treatment. Our objective is to call the attention for this aggressive desease to estimulate new perspectives and further studies with chemotherapy and other different kinds of treatment, mainly because only surgical intervention is an incomplete aproach.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

RMC (Renal Medullary Cancer): A Case Report and Literature Review

Taranto¹,

Mariano²,

Eiger³

et al. 2016

JPP

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“…34 RMC was first identified by the Genitourinary Pathology Department, Armed Forces Institute of Pathology, Washington, DC, USA in 1995, where it was recognized as "the seventh sickle cell nephropathy", adding to a list that includes gross hematuria, papillary necrosis, nephrotic syndrome, renal infarction, the inability to concentrate urine, and pyelonephritis. 35 The original study reported a mean patient age of 22 years, with a male predominance of 2:1.…”

Section: Renal Medullary Carcinoma Backgroundmentioning

confidence: 99%

Treating renal cell carcinoma in young adults: challenges and solutions

Matrana¹,

Dreisin²

2016

COAYA

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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.

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“…Its codes for protein INI1 (hSNF5/SMARCB1/BAF47) which is present in all normal tissues from yeasts to humans. It is involved in transcriptional activation and repression by chromatin remodeling and also interacts with other cancer associated pathways like WNT signaling pathway and hedgehog signaling pathway [11,12,13]. Mutation of this gene resulting in loss of expression is seen in RMC which is hence also called SMARCB 1 deficient renal medullary carcinoma.…”

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confidence: 99%

Renal Medullary Carcinoma, A Rare But Aggressive Sickle Cell Nephropathy: A Case Report

Kumar,

Nair,

Aravindan

et al. 2023

Ann of Pathol and Lab Med

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Renal Medullary Carcinoma (RMC) is a highly aggressive cancer that typically affects adults in second to third decades of life with male preponderance and dismal prognosis. As the name indicates, it is medulla centric and is almost exclusively associated with sickle cell hemoglobinopathy. Tumor shows SMARCB1 /INI-1 deficiency and hence called SMARCB1 deficient renal medullary carcinoma. As there are only around 600 cases been reported worldwide till date, we report this case as an eye opener for having high degree of clinical and pathological suspicion in renal cancer patients of younger age, especially if there is no known history of hemoglobinopathy. Prompt diagnosis and treatment is warranted to prolong survival. Our case is that of a 36yr old male who presented with hematuria and loin pain of six months without any significant past history. Radiological evaluation showed right renal mass with extensive metastatic disease. Palliative right radical nephrectomy was done which on thorough histomorphological and immunohistochemical examination was diagnosed as renal medullary carcinoma. Tumor cells showed loss of INI 1 expression which is a surrogate marker for SMARCB1 deficient status. High performance liquid chromatography was done which revealed sickle cell trait. He was started on immunotherapy and chemotherapy, following which he had a favorable treatment response.

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Renal Medullary Carcinoma: Case Report of an Aggressive Malignancy with Near-Complete Response to Dose-Dense Methotrexate, Vinblastine, Doxorubicin, and Cisplatin Chemotherapy

Cited by 7 publications

References 23 publications

RMC (Renal Medullary Cancer): A Case Report and Literature Review

RMC (Renal Medullary Cancer): A Case Report and Literature Review

Treating renal cell carcinoma in young adults: challenges and solutions

Renal Medullary Carcinoma, A Rare But Aggressive Sickle Cell Nephropathy: A Case Report

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