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Presented by Dr. Jonathan Epstein and prepared by Dr. Sintawat Wangsiricharoen.
A 55 year old man had a 3 cm cystic mass resected.
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Answer: A. Eosinophilic Solid and Cystic (ESC) RCC
Histology: The tumor was grossly and microscopically cystic with only small solid foci. The cysts were lined by cuboidal epithelium or hobnail cells with eosinophilic cytoplasm. The solid foci have similar cells with abundant eosinophilic cytoplasm. A minority of cells had prominent basophilic to purple cytoplasmic irregular granular stippling. Nuclei were round to oval, with often irregular nuclei. Focallly, cells had prominent nucleoli.
Discussion: ESC is a relatively newly described variant of RCC. It occurs most commonly in females with a broad age range, including pediatric patients. It is usually unifocal. The great majority are sporadic, but rare tumors with identical morphology have been reported in patients with tuberous sclerosis complex (TSC). There are somatic and bi-allelic mutations in the TSC genes, TSC1 and TSC2. Most cases are predominantly solid with focal cyst formation. However, at the other ends of the spectrums, some ESCs are very cystic as the case presented herein and some are purely solid. This case has the typical morphology with the exception of a more prominent cystic component. CK20 is present in almost all cases, either diffusely or focally, which is almost pathognomonic as other RCCs lack CK20. CK20 was positive in this case. Most cases are indolent with rare metastases. A grade is not assigned as they would in most cases be assigned a nucleolar grade 3, which would not reflect their biology.
Reference: Trpkov K, Williamson SR, Gill AJ, et al. Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia. Modern Pathology (in press).
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Answer: A. Eosinophilic Solid and Cystic (ESC) RCC
Histology: The tumor was grossly and microscopically cystic with only small solid foci. The cysts were lined by cuboidal epithelium or hobnail cells with eosinophilic cytoplasm. The solid foci have similar cells with abundant eosinophilic cytoplasm. A minority of cells had prominent basophilic to purple cytoplasmic irregular granular stippling. Nuclei were round to oval, with often irregular nuclei. Focallly, cells had prominent nucleoli.
Discussion: ESC is a relatively newly described variant of RCC. It occurs most commonly in females with a broad age range, including pediatric patients. It is usually unifocal. The great majority are sporadic, but rare tumors with identical morphology have been reported in patients with tuberous sclerosis complex (TSC). There are somatic and bi-allelic mutations in the TSC genes, TSC1 and TSC2. Most cases are predominantly solid with focal cyst formation. However, at the other ends of the spectrums, some ESCs are very cystic as the case presented herein and some are purely solid. This case has the typical morphology with the exception of a more prominent cystic component. CK20 is present in almost all cases, either diffusely or focally, which is almost pathognomonic as other RCCs lack CK20. CK20 was positive in this case. Most cases are indolent with rare metastases. A grade is not assigned as they would in most cases be assigned a nucleolar grade 3, which would not reflect their biology.
Reference: Trpkov K, Williamson SR, Gill AJ, et al. Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia. Modern Pathology (in press).