Eosinophilic solid and cystic renal cell carcinoma (ESC RCC) is a novel tumour with unique morphological and immunohistochemical features. It is a recently described entity after the 2016 World Health Organization Classification of Tumours of the Urinary System and Male Genital Organs and is characterised by a solid cystic tumour composed of polygonal cells with voluminous eosinophilic cytoplasm and CK20 positivity. This tumour has uncertain malignant potential and also has an association with tuberous sclerosis complex (TSC). Sarcomatoid differentiation has not been reported in ESC RCC till now. ESC RCC poses a diagnostic challenge as many eosinophilic/oncocytic renal tumours are included in the differentials. We present a case of ESC RCC with sarcomatoid differentiation in an elderly female without any clinical features of TSC and discuss the differential diagnosis of oncocytic renal tumours.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9999703PMC
http://dx.doi.org/10.5146/tjpath.2021.01531DOI Listing

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Introduction And Significance: Eosinophilic solid and cystic renal cell carcinoma (ESC RCC) is a rare renal tumor primarily associated with female patients and those with tuberous sclerosis complex (TSC). Despite lacking distinct clinical or radiological features, its unique histological characteristics allow for differentiation from other renal neoplasms. While it often exhibits indolent growth, metastatic potential remains a concern.

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  • Pathogenic mutations in genes associated with tuberous sclerosis complex (TSC) affect various types of kidney tumors like eosinophilic solid and cystic renal cell carcinoma (ESC RCC) and oncocytic tumors.
  • A study validated a TSC2 immunohistochemistry (IHC) assay on tissues from renal tumors to assess the presence of TSC2 mutations, achieving high predictive values for diagnosing these tumors.
  • The findings highlight that TSC2 IHC can help identify renal tumors linked to TSC/mTOR pathway mutations, supporting its use in diagnostic processes.
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  • - Oncocytic renal neoplasms pose significant diagnostic challenges, yet are generally nonaggressive, prompting discussion about the necessity of differentiating emerging subtypes like eosinophilic solid and cystic renal cell carcinoma and others.
  • - A survey of 63 urologic pathologists revealed that many encounter complex oncocytic tumors frequently, with 70% agreeing that eosinophilic solid and cystic renal cell carcinoma should be recognized as a distinct category, while opinions were more divided on other types.
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Background: Eosinophilic solid and cystic (ESC) renal cell carcinoma (RCC), a unique and emerging subtype of RCC, has an indolent nature; in some rare instances, it may exhibit metastatic potential. Current cases are inadequate to precisely predict the clinical outcome of ESC RCC and determine treatment choices.

Case Summary: Herein, we report two patients with ESC RCC.

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