scholarly journals Unusual Suspect: A Case Report of Tubulocystic Renal Cell Carcinoma with Features of Cystic Renal Oncocytoma

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Jacob D. McFadden ◽  
Isabell A. Sesterhenn ◽  
Sean Q. Kern
Keyword(s):  
Renal Cell Carcinoma ◽  
Case Report ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clinical Course ◽  
World Health ◽  
Renal Oncocytoma ◽  
Renal Masses ◽  
Small Collection ◽  
Health Organization

Tubulocystic renal cell carcinoma is an uncommon subtype of renal cell carcinoma that was only recently acknowledged by the World Health Organization. There is a relatively small collection of literature dedicated to the features and clinical course of this lesion. Despite its rarity, this diagnosis should remain in the differential for all cystic renal masses. We present a case report of tubulocystic renal cell carcinoma (TC-RCC) with remarkable similarity to cystic renal oncocytoma, highlighting the diagnostic challenges associated with this unusual renal malignancy.

scholarly journals MiT Family Translocation Renal Cell Carcinoma: from the Early Descriptions to the Current Knowledge

Cancers ◽  
2019 ◽  
Vol 11 (8) ◽  
pp. 1110 ◽  
Author(s):  
Anna Caliò ◽  
Diego Segala ◽  
Enrico Munari ◽  
Matteo Brunelli ◽  
Guido Martignoni
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clinical Course ◽  
Current Knowledge ◽  
Wide Spectrum ◽  
Cathepsin K ◽  
World Health ◽  
Epithelioid Angiomyolipoma ◽  
Immunohistochemical Markers

The new category of MiT family translocation renal cell carcinoma has been included into the World Health Organization (WHO) classification in 2016. The MiT family translocation renal cell carcinoma comprises Xp11 translocation renal cell carcinoma harboring TFE3 gene fusions and t(6;11) renal cell carcinoma harboring TFEB gene fusion. At the beginning, they were recognized in childhood; nevertheless, it has been demonstrated that these neoplasms can occur in adults as well. In the nineties, among Xp11 renal cell carcinoma, ASPL, PRCC, and SFPQ (PSF) were the first genes recognized as partners in TFE3 rearrangement. Recently, many other genes have been identified, and a wide spectrum of morphologies has been described. For this reason, the diagnosis may be challenging based on the histology, and the differential diagnosis includes the most common renal cell neoplasms and pure epithelioid PEComa/epithelioid angiomyolipoma of the kidney. During the last decades, many efforts have been made to identify immunohistochemical markers to reach the right diagnosis. To date, staining for PAX8, cathepsin K, and melanogenesis markers are the most useful identifiers. However, the diagnosis requires the demonstration of the chromosomal rearrangement, and fluorescent in situ hybridization (FISH) is considered the gold standard. The outcome of Xp11 translocation renal cell carcinoma is highly variable, with some patients surviving decades with indolent disease and others dying rapidly of progressive disease. Despite most instances of t(6;11) renal cell carcinoma having an indolent clinical course, a few published cases demonstrate aggressive behavior. Recently, renal cell carcinomas with TFEB amplification have been described in connection with t(6;11) renal cell carcinoma. Those tumors appear to be associated with a more aggressive clinical course. For the aggressive cases of MiT family translocation carcinoma, the optimal therapy remains to be determined; however, new target therapies seem to be promising, and the search for predictive markers is mandatory.

scholarly journals Succinate Dehydrogenase–Deficient Renal Cell Carcinoma

2018 ◽  
Vol 143 (5) ◽  
pp. 643-647 ◽  
Author(s):  
Tsung-Heng Tsai ◽  
Wen-Ying Lee
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Succinate Dehydrogenase ◽  
Renal Cell ◽  
World Health ◽  
Low Grade ◽  
Long Term Follow Up ◽  
Health Organization

Succinate dehydrogenase (SDH)–deficient renal cell carcinoma is a recently recognized distinct subtype of renal cell carcinoma in the 2016 World Health Organization classification. It is associated with SDH gene germline mutations, which also cause paraganglioma/pheochromocytoma, gastrointestinal stromal tumor, and pituitary adenoma. The tumor most commonly presents in young adulthood. The tumors are arranged in solid nests or in tubules and frequently show cystic change. The tumors are composed of cuboidal to oval cells with round nuclei, dispersed chromatin, and inconspicuous nucleoli. The cytoplasm is eosinophilic or flocculent but not truly oncocytic. The most distinctive histologic feature is the presence of cytoplasmic vacuoles or inclusions. Loss of SDH subunit B immunostaining is needed for a definite diagnosis. The prognosis is good for low-grade tumors but worse for tumors with high-grade nuclei, sarcomatoid change, or coagulative necrosis. Long-term follow-up is indicated.

scholarly journals Papillary renal cell carcinoma within a renal oncocytoma: Case report of very rare coexistence

2014 ◽  
Vol 8 (11-12) ◽  
pp. 928 ◽  
Author(s):  
Cevahir Özer ◽  
Mehmet Resit Gören ◽  
Tulga Egilmez ◽  
Nebil Bal
Keyword(s):  
Renal Cell Carcinoma ◽  
Case Report ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Radical Nephrectomy ◽  
Papillary Renal Cell Carcinoma ◽  
Renal Oncocytoma ◽  
Renal Neoplasms ◽  
Renal Oncocytomas ◽  
Papillary Rcc

Renal oncocytomas accounts for 3% to 9% of primary renal neoplasms. The coexistence of renal cell carcinoma (RCC) within the oncocytoma is extremely rare. We report the case of an asymptomatic 74-year-old man with papillary RCC within oncocytoma managed with left radical nephrectomy.

scholarly journals Morphologic, Molecular, and Taxonomic Evolution of Renal Cell Carcinoma: A Conceptual Perspective With Emphasis on Updates to the 2016 World Health Organization Classification

2016 ◽  
Vol 140 (10) ◽  
pp. 1026-1037 ◽  
Author(s):  
Aaron M. Udager ◽  
Rohit Mehra
Keyword(s):  
Renal Cell Carcinoma ◽  
World Health Organization ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Elongation Factor ◽  
World Health ◽  
World Health Organization Classification ◽  
Anaplastic Lymphoma ◽  
Diagnosis And Classification ◽  
Health Organization

Molecular and morphologic interrogation has driven a much-needed reexamination of renal cell carcinoma (RCC). Indeed, the recently released 2016 World Health Organization classification now recognizes 12 distinct RCC subtypes, as well as several other emerging/provisional RCC entities. From a clinical perspective, accurate RCC classification may have important implications for patients and their families, including prognostic risk stratification, targeted therapeutics selection, and identification for genetic testing. In this review, we provide a conceptual framework for approaching RCC diagnosis and classification by categorizing RCCs as tumors with clear cytoplasm, papillary architecture, and eosinophilic (oncocytic) cytoplasm. The currently recognized 2016 World Health Organization classification for RCC subtypes is briefly discussed, including new diagnostic entities (clear cell papillary RCC, hereditary leiomyomatosis and RCC-associated RCC, succinate dehydrogenase–deficient RCC, tubulocystic RCC, and acquired cystic disease–associated RCC) and areas of evolving RCC classification, such as transcription elongation factor B subunit 1 (TCEB1)–mutated RCC/RCC with angioleiomyoma-like stroma/RCC with leiomyomatous stroma, RCC associated with anaplastic lymphoma receptor tyrosine kinase (ALK) gene rearrangement, thyroidlike follicular RCC, and RCC in neuroblastoma survivors. For each RCC subtype, relevant clinical, molecular, gross, and microscopic findings are reviewed, and ancillary studies helpful for its differential diagnosis are presented, providing a practical approach to modern RCC classification.

scholarly journals Synchronous Malignant Pheochromocytoma With Renal Cell Carcinoma: A Case Report

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A159-A159
Author(s):  
Charles Ma ◽  
Noah Bloomgarden ◽  
Simona Stefan
Keyword(s):  
Renal Cell Carcinoma ◽  
Case Report ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Malignant Pheochromocytoma ◽  
Laboratory Evaluation ◽  
High Morbidity ◽  
Genetic Syndromes ◽  
Renal Masses ◽  
Familial Pheochromocytoma

Abstract Introduction: In the US, Pheochromocytoma/paraganglioma incidence is estimated to be 2–8 per 1 million people each year with around 100–200 of these cases being malignant. Malignant pheochromocytoma is defined by documented presence of metastases or evidence of extensive local invasion. There are certain genetic syndromes which are also associated with renal cell carcinoma, including SDHB mutations type 4, VHL disease and familial pheochromocytoma. These syndromes are important to recognize as they may signify a worse prognosis. Here, we describe a case of co-occurrence of malignant PC with renal cell carcinoma. Case Report: A 53-year-old Hispanic male with history of HTN and recently diagnosed metastatic pheochromocytoma was admitted for surgical debulking of the left retroperitoneal/adrenal and renal masses. Symptoms began five months prior after he presented with an ischemic stroke in the setting of labile hypertension. He was diagnosed with a 6.3 x 4.6 x 6.8 cm incidental left retroperitoneal mass and suspicious left renal mass on CT imaging but also noted several lytic bony lesions concerning for bone metastasis. A spinal biopsy was obtained which was consistent with a well-differentiated metastatic neuroendocrine tumor. Laboratory evaluation was notable for Chromogranin A level of 6959ng/mL (25–140). He was started on Lanreotide. Given persistently difficult to control HTN he underwent work up for secondary hypertension. Hormonal evaluation was notable for plasma free metanephrine of 534pg/mL (<57pg/mL), normetanephrine 6155pg/mL(<148pg/mL), and total metanephrine of 6689pg/mL (205pg/mL) consistent with metastatic Pheochromocytoma. After appropriate alpha blockade he underwent left adrenalectomy, nephrectomy and liver tumor microwave ablation. Pathology was consistent with an 8.7cm pheochromocytoma with extensive retroperitoneal soft tissue invasion and PASS score of 9 as well as a 3.6 cm renal cell (clear cell-papillary type) carcinoma. On follow up, Plasma metanephrine decreased significantly postoperatively to a free metanephrine of 28pg/ml, normetanephrine 1153pg/ml, and total metanephrine of 1181pg/mL. He was referred for genetic testing but unfortunately, he was readmitted one month later with cerebral hemorrhage and expired. Conclusion: Advancements in genetics have led to improved understanding of the molecular etiologies of pheochromocytomas. A number of genetic defects are associated with PC and RCC, including SDHB mutations type 4, VHL and familial pheochromocytoma. Our case underscores the high morbidity and mortality in patients with metastatic PC with RCC and perhaps the catastrophic outcomes in such patients. Assessing patient’s genetics in these cases is now the standard of care, however further research studies are warranted to better understand the significance of tumor genetics on prognosis and management.

A simple cyst is not always simply a cyst: A case of cystic recurrence after nephrectomy for tubulocystic renal cell carcinoma and literature review

2019 ◽  
Vol 87 (3) ◽  
pp. 119-124
Author(s):  
Fabio Salvatori ◽  
Marco Macchini ◽  
Marco Misericordia ◽  
Enrico Paci ◽  
Andrea Giovagnoni ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clinical Characteristics ◽  
Renal Tumor ◽  
World Health ◽  
Imaging Features ◽  
Simple Cyst ◽  
Left Hypochondrium ◽  
Health Organization

Tubulocystic renal cell carcinoma is a rare subtype of renal tumor according to the 2016 World Health Organization, and less than 100 cases have been documented up to date in literature. The imaging features are not well known and to the best of our knowledge, there is not a radiology description of recurrence from tubulocystic renal cell carcinoma in the literature. We describe the case of a 70-year-old man with unusual cystic lesions in the left hypochondrium 11 years after a nephrectomy for tubulocystic renal cell carcinoma on the same side, and we report a review of the clinical characteristics of metastatic tubulocystic renal cell carcinoma.

scholarly journals Palpable Abdominal Mass is a Renal Oncocytoma: Not All Large Renal Masses are Malignant

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Sumi Dey ◽  
Sabrina L. Noyes ◽  
Ghayas Uddin ◽  
Brian R. Lane
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Renal Mass ◽  
Abdominal Mass ◽  
Renal Oncocytoma ◽  
Cell Renal Cell Carcinoma ◽  
Renal Masses ◽  
Initial Imaging ◽  
Palpable Abdominal Mass

A 59-year-old woman presented with abdominal pain and a palpable abdominal mass. Initial imaging revealed a 14cm solid, enhancing renal mass and suspicion for liver and bone metastases. Family history included a brother with clear cell renal cell carcinoma and mother with glioblastoma multiforme. After liver biopsy was inconclusive, she underwent radical nephrectomy with final pathologic diagnosis of oncocytoma. Renal oncocytoma is the most common benign renal tumor but remains difficult to distinguish clinically and radiographically from renal cell carcinoma. Should urologists use renal mass biopsy even more frequently prior to surgical intervention?

scholarly journals Comparing World Health Organization/International Society of Urological Pathology Grading and Fuhrman Grading with the Prognostic Value of Nuclear Area in Patients with Renal Cell Carcinoma

Uro ◽  
2021 ◽  
Vol 1 (1) ◽  
pp. 2-13
Author(s):  
Maj Rabjerg ◽  
Oke Gerke ◽  
Birte Engvad ◽  
Niels Marcussen
Keyword(s):  
Renal Cell Carcinoma ◽  
World Health Organization ◽  
Cell Carcinoma ◽  
International Society ◽  
Renal Cell ◽  
World Health ◽  
Nuclear Area ◽  
Prognostic Information ◽  
Cancer Specific Survival ◽  
Health Organization

This study was undertaken to compare Fuhrman grading with World Health Organization/International Society of Urological Pathology (WHO/ISUP) grading and stereologically measured nuclear area in patients with Clear Cell Renal Cell Carcinoma (ccRCC) or Papillary Renal Cell Carcinoma (PRCC) and to evaluate the independent predictive value of Fuhrman, WHO/ISUP and stereologically measured nuclear area combined with necrosis in a series of patients with ccRCC in relation to cancer-specific survival. In all, 124 cases of ccRCC and PRCC were included. All slides were blindly scored by two trained pathologists according to the Fuhrman and WHO/ISUP grading systems. Nuclear measurements were performed on digitally scanned slides in Visiopharm® and correlated to survival. Analysis of ccRCC and PRCC cases showed that application of WHO/ISUP grading resulted in a significant downgrading of cases from G2 to G1, when comparing with Fuhrman grading. Neither of these patients experienced progression. Cancer specific survival estimates in 101 ccRCC patients showed that WHO/ISUP grading was slightly superior in predicting cancer-specific survival. Novel models included WHO/ISUP grading and mean nuclear area (MNA) each of which combined with necrosis. Both demonstrated an increased ability to predict cancer-specific survival. The study demonstrates that WHO/ISUP grading provides superior prognostic information compared to Fuhrman grading and stereologically measured nuclear area. Necrosis in combination with either WHO/ISUP grading or MNA adds additional prognostic information.

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