Recent Advances in Renal Medullary Carcinoma

Su, Yongdong; Hong, Andrew L.

doi:10.3390/ijms23137097

Cited by 7 publications

(13 citation statements)

References 89 publications

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“…Despite advancements in the understanding of this disease, no significant improvement in disease-specific survival has been observed over the past decade, and treatment is currently based on chemotherapy and nephrectomy, despite a lack of solid scientific evidence ( 18 – 20 ). In 2016, Beckermann et al.…”

Section: Discussionmentioning

confidence: 99%

Surgical and oncological management of renal medullary carcinoma in a young patient: a case report

et al. 2023

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Renal medullary carcinoma (RMC) is a rare form of renal cell carcinoma that has a poor prognosis. It is known to be associated with sickle cell trait or disease, although the exact underlying mechanisms are still unclear. The diagnosis is made through immunochemical staining for SMARCB1 (INI1). In this report, we present a case of a 31-year-old male patient with sickle cell trait who was diagnosed with stage III right RMC. Despite the poor prognosis, the patient survived for a remarkable duration of 37 months. Radiological assessment and follow-up were primarily performed using 18F-FDG PET/MRI. The patient underwent upfront cisplatin-based cytotoxic chemotherapy before surgical removal of the right kidney and retroperitoneal lymph node dissection. Identical adjuvant chemotherapy was administered post-surgery. Disease relapses were detected in the retroperitoneal lymph nodes; these were managed with chemotherapy and surgical rechallenges. We also discuss the oncological and surgical management of RMC, which currently relies on perioperative cytotoxic chemotherapy strategies, as there are no known alternative therapies that have been shown to be superior to date.

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Section: Discussionmentioning

confidence: 99%

Surgical and oncological management of renal medullary carcinoma in a young patient: a case report

et al. 2023

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“…It is often found at an advanced stage or with distant metastases, and recent studies have shown that SMARCB1 -deficient RMC is also associated with the sickle-cell trait [ 82 ]. Specific symptoms are usually abdominal pain, hematuria, and weight loss [ 80 ], while distant metastases can be found in the renal lymph nodes, adrenal glands, lungs, and liver [ 83 ]. Due to the prevalence of SMARCB1 -deficient RMC in children and adolescents and the aggressive nature of the tumor, early recognition and diagnosis are a priority for physicians.…”

Section: Smarcb1 -Deficient Renal Medullary Carcinomamentioning

confidence: 99%

“…With the advancement of detection technology in recent years, some characteristic manifestations of SMARCB1 -deficient RMC have been gradually proposed. First, in addition to the previously mentioned clinical symptoms and prodromal nature during adolescence, SMARCB1 -deficient RMC usually develops with SCT and SCD [ 83 ]. Secondly, the tumor is often already at a high grade at the time of detection, showing infiltrative growth and exhibiting a sieve or reticular appearance [ 85 , 86 ].…”

Section: Smarcb1 -Deficient Renal Medullary Carcinomamentioning

confidence: 99%

“…Secondly, the tumor is often already at a high grade at the time of detection, showing infiltrative growth and exhibiting a sieve or reticular appearance [ 85 , 86 ]. In addition to this, and most importantly, all SMARCB1 -deficient RMC showed negative staining for SMARCB1 when IHC for the detection of the SMARCB1 protein was performed [ 83 ]. Therefore, when adolescent RCC patients with hematologic disorders such as SCT are identified in the clinic, physicians should perform IHC testing as early as possible to determine whether SMARCB1 -deficient RMC is present.…”

Section: Smarcb1 -Deficient Renal Medullary Carcinomamentioning

confidence: 99%

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Clinical Characteristics of Molecularly Defined Renal Cell Carcinomas

Tan

Zhu

2023

CIMB

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Kidney tumors comprise a broad spectrum of different histopathological entities, with more than 0.4 million newly diagnosed cases each year, mostly in middle-aged and older men. Based on the description of the 2022 World Health Organization (WHO) classification of renal cell carcinoma (RCC), some new categories of tumor types have been added according to their specific molecular typing. However, studies on these types of RCC are still superficial, many types of these RCC currently lack accurate diagnostic standards in the clinic, and treatment protocols are largely consistent with the treatment guidelines for clear cell RCC (ccRCC), which might result in worse treatment outcomes for patients with these types of molecularly defined RCC. In this article, we conduct a narrative review of the literature published in the last 15 years on molecularly defined RCC. The purpose of this review is to summarize the clinical features and the current status of research on the detection and treatment of molecularly defined RCC.

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“…Studies indicated that RMC tumors often harbor translocations and deletions of tumor suppressor SWI/SNF-related matrixassociated actin-dependent regulator of chromatin subfamily B member 1 (SMARCB1) or rearrangement in anaplastic lymphoma kinase (ALK) [67]. The absence of SMARCB1 protein in RMC leads to activation of the c-MYC pathway and subsequently induction of high levels of DNA replication stress, which results in the upregulation of cell-cycle checkpoint pathways [68]. The presence of a BCR/ABL rearrangement was reported in one study.…”

Section: Renal Medullary Cell Carcinoma (Rmc)mentioning

confidence: 99%

Non-Clear Cell Renal Cell Carcinoma: Molecular Pathogenesis, Innovative Modeling, and Targeted Therapeutic Approaches

Rad

Vahidyeganeh

Mohammadi

et al. 2022

IJTM

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Non-clear cell renal cell carcinomas (nccRCC) are a diverse group of kidney cancers with histopathologically and genetically heterogeneous features. About 25% of renal cell carcinomas (RCCs) are nccRCC types. The management and treatment of nccRCCs are rather limited, and the data are often estimated from studies in the more common clear cell renal cell carcinoma (ccRCC). Each subtype has its own distinctive biological and therapeutic profile. Our knowledge of the underlying biological features of nccRCC has directed and continues to shape the use of novel therapy targeting the main signaling pathways and leading to improved overall survival (OS) of the patients. This review discusses the characteristic molecular features of the major types of nccRCC and current cell-based and animal models for studying them. In the following, we highlighted major signaling pathways and therapeutic approaches for nccRCC patients.

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Recent Advances in Renal Medullary Carcinoma

Cited by 7 publications

References 89 publications

Surgical and oncological management of renal medullary carcinoma in a young patient: a case report

Surgical and oncological management of renal medullary carcinoma in a young patient: a case report

Clinical Characteristics of Molecularly Defined Renal Cell Carcinomas

Non-Clear Cell Renal Cell Carcinoma: Molecular Pathogenesis, Innovative Modeling, and Targeted Therapeutic Approaches

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