Renal medullary carcinoma masquerading as renal infection: a case report

Huang, Zhongming; Hai, Wang; Ji, Zhigang

doi:10.1186/s12882-020-01730-y

Cited by 4 publications

(4 citation statements)

References 8 publications

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“…RMC occurs in medulla where there is a hypoxic microenvironment created by hyperosmolarity, low pH and stasis which in turn enhances sickling and causes ischemic damage. [2,7,9] Renal artery being longer promotes stasis and hypoxia and hence 70% of the tumors are right sided like our case.…”

Section: Figure 4: Hplc Chromatogram Showing Increased Hbs Of 369 % (...supporting

confidence: 54%

“…Common metastatic sites are lymph nodes, lung, liver, adrenal glands, and bone. Brain was an uncommon site [7,9]. Ultrasonography, CT imaging, and magnetic resonance imaging do not provide findings specific for RMC [8] The exact pathogenesis of RMC is unclear, but new data and concepts are emerging.…”

Section: Figure 4: Hplc Chromatogram Showing Increased Hbs Of 369 % (...mentioning

confidence: 99%

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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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Section: Figure 4: Hplc Chromatogram Showing Increased Hbs Of 369 % (...supporting

confidence: 54%

Section: Figure 4: Hplc Chromatogram Showing Increased Hbs Of 369 % (...mentioning

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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“…"Unclassified RCC with medullary features" or "RCC unclassified, with medullary phenotype" (RCCU-MP) [70] mostly tends to happen in non-African ethnicities; as reported in the literature, within six Chinese patients with RMC, only one was found to have SCT [71]. It is noticeable that these Chinese patients were older than those reported African-American cases, ranging from 22 to 72 years old [71,72]. These findings are suggestive that this entity, "Unclassified renal cell carcinoma with medullary phenotype" is a variant of malignant rhabdoid tumor seen in young children.…”

Section: Unclassified Renal Cell Carcinoma With Medullary Phenotypementioning

confidence: 90%

Recent Advances in Renal Medullary Carcinoma

Hong

2022

IJMS

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Renal medullary carcinoma (RMC) is a rare renal malignancy that has been associated with sickle hemoglobinopathies. RMC is aggressive, difficult to treat, and occurs primarily in adolescents and young adults of African ancestry. This cancer is driven by the loss of SMARCB1, a tumor suppressor seen in a number of primarily rare childhood cancers (e.g., rhabdoid tumor of the kidney and atypical teratoid rhabdoid tumor). Treatment options remain limited due in part to the limited knowledge of RMC biology. However, significant advances have been made in unraveling the biology of RMC, from genomics to therapeutic targets, over the past 5 years. In this review, we will present these advances and discuss what new questions exist in the field.

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“…RMC was initially observed in a population of 34 patients, nearly all African-American, and predominantly young males, with known hemoglobinopathies (either sickle cell trait or hemoglobin SC disease) [ 2 ]. The initial clinical presentation typically includes hematuria and/or flank pain with concern for urinary tract infections or abscesses, with suspicion for malignancy only increased after the failure of antibiotic therapy [ 3 , 4 ]. Additionally, patients may describe fatigue and weight loss, and symptoms are present on average about five months prior to diagnosis [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

Unusual Presentation of Renal Medullary Carcinoma With Undiagnosed Sickle Cell Trait

Basher

Dutcher

England

et al. 2020

Cureus

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Renal medullary carcinoma (RMC) is an extremely rare malignancy that has been described in younger male patients of African descent with a history of sickle cell disease or trait. We describe a rather unique case of RMC in an older male patient who initially presented with acute on chronic urinary retention and concern for infection. Further investigation revealed a history of hematuria and long-standing microcytic anemia, and the patient was found to have sickle cell trait (SCT) as part of a workup for malignancy of unknown primary. Imaging findings initially interpreted as hydronephrosis later characterized a mass in the renal pelvis concerning for a genitourinary malignancy, later biopsy-proven RMC. RMC typically presents in its advanced stages, with associated poor prognosis, and treatment options are limited and have been extrapolated from standard regimens for other genitourinary malignancies. Therefore, early clinical suspicion in patients with microcytic anemia, flank pain, hematuria, and urinary symptoms, can aid in the diagnosis of RMC and allow for prompt intervention.

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Renal medullary carcinoma masquerading as renal infection: a case report

Cited by 4 publications

References 8 publications

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

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

Recent Advances in Renal Medullary Carcinoma

Unusual Presentation of Renal Medullary Carcinoma With Undiagnosed Sickle Cell Trait

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