Chromophobe Renal Cell Carcinoma with Radiologic-Pathologic Correlation

Jamie Marko; Ryan Craig; Andrew Nguyen; Aaron M Udager; Darcy J Wolfman

doi:10.1148/rg.2021200206

. 2021 Aug 13;41(5):1408–1419. doi: 10.1148/rg.2021200206

Chromophobe Renal Cell Carcinoma with Radiologic-Pathologic Correlation

Jamie Marko ¹, Ryan Craig ¹, Andrew Nguyen ¹, Aaron M Udager ¹, Darcy J Wolfman ^1,^✉

PMCID: PMC8415046 PMID: 34388049

Abstract

Renal cell carcinoma (RCC) is a heterogeneous group of neoplasms derived from the renal tubular epithelial cells. Chromophobe RCC (chRCC) is the third most common subtype of RCC, accounting for 5% of cases. chRCC may be detected as an incidental finding or less commonly may manifest with clinical symptoms. The mainstay of therapy for chRCC is surgical resection. chRCC has a better prognosis compared with the more common clear cell RCC. At gross pathologic analysis, chRCC is a solid well-defined mass with lobulated borders. Histologic findings vary by subtype but include large pale polygonal cells with abundant transparent cytoplasm, crinkled “raisinoid” nuclei with perinuclear halos, and prominent cell membranes. Pathologic analysis reveals only moderate vascularity. The most common imaging pattern is a predominantly solid renal mass with circumscribed margins and enhancement less than that of the renal cortex. The authors discuss chRCC with emphasis on correlative pathologic findings and illustrate the multimodality imaging appearances of chRCC by using cases from the Radiologic Pathology Archives of the American Institute for Radiologic Pathology.

^©RSNA, 2021

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SA-CME LEARNING OBJECTIVES

After completing this journal-based SA-CME activity, participants will be able to:

■ Describe the most common clinical features of chRCC.
■ List the main pathologic features of chRCC.
■ Describe the most common imaging findings of chRCC.

Introduction

Renal cell carcinoma (RCC) is a heterogeneous group of neoplasms derived from the renal tubular epithelial cells (1). Despite a common cellular origin, RCC is best thought of as a spectrum of diseases with different histologic subtypes, unique genetic and molecular alterations, and distinctive clinical courses (2). The 2016 World Health Organization classification of renal tumors includes 16 subtypes of renal cell tumors (Table 1) (3). The malignant renal epithelial tumor RCC includes multiple histologic subtypes, with clear cell RCC (ccRCC), papillary RCC (papRCC), and chromophobe RCC (chRCC) accounting for approximately 75%, 15%, and 5% of cases, respectively (4). The purpose of this article is to review the imaging and pathologic features of chRCC.

Table 1:

WHO Classification of Renal Cell Tumors

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Clinical Considerations

Presentation

chRCC is often detected as an incidental finding or less commonly manifests with clinical symptoms (5,6) (Fig 1). With increased use of cross-sectional abdominal imaging, there is a trend toward detection of asymptomatic renal masses, including chRCC (7–9). The reported clinical findings of chRCC include local symptoms such as abdominal or flank pain (34%–67% of cases), abdominal mass (27% of cases), and hematuria (17%–40% of cases) and systemic symptoms such as fever, cachexia, fatigue, and weight loss (20%–33% of cases) (5,6,10). Most chRCCs are low-stage cancers at diagnosis, reflecting both the indolent nature of chRCC and the trend toward incidental detection at imaging (5,6,10,11). Lymph node and distant metastases are infrequent, seen in 2%–4% and 1%–4% of cases, respectively (5–7,12). The most common sites of metastases are the liver and lungs (10,13).

Chromophobe renal cell carcinoma (chRCC) appearing as a small incidental renal mass in a 53-year-old man. Axial noncontrast (a), nephrographic phase (b), and excretory phase (c) CT images show a small solid renal mass (arrow) with enhancement less than that of the renal parenchyma. — Chromophobe renal cell carcinoma (chRCC) appearing as a small incidental renal mass in a 53-year-old man. Axial noncontrast **(a)**, nephrographic phase **(b)**, and excretory phase **(c)** CT images show a small solid renal mass (arrow) with enhancement less than that of the renal parenchyma.

Epidemiologic Features

The estimated worldwide incidence of RCC in 2018 was 400 000, with 175 000 related deaths (2). Of these 400 000 cases worldwide, approximately 65 000 occurred in the United States (14). Since chRCC accounts for approximately 5% of RCC cases, the estimated U.S. annual incidence is 3250 cases (4,10). The reported mean age of patients diagnosed with chRCC is 58–60 years (5–7,12,15). chRCC shows a slight male sex predilection (5,7,10,12).

chRCC is associated with hereditary Birt-Hogg-Dubé (BHD) syndrome (Fig 2), which is a rare autosomal dominant syndrome associated with germline mutations in the folliculin gene, FLCN.FLCN is a tumor suppressor gene located on the short arm of chromosome 17. BHD affects multiple organ systems, including the skin, lungs, and kidneys. In patients with BHD, one allele of the FLCN gene is mutated in the germline. When FLCN is biallelically inactivated in the kidney, there is loss of inhibition of the mammalian target of rapamycin pathway, which is one of the major pathways that regulates angiogenesis and tumor growth in RCC.

Birt-Hogg-Dubé (BHD) syndrome and bilateral chRCC in a 60-year-old woman. (a) Coronal CT image of the lungs shows multiple small lung cysts (arrows). (b, c) Coronal T2-weighted (b) and contrast-enhanced T1-weighted (c) MR images show bilateral solid renal masses (arrows). — Birt-Hogg-Dubé (BHD) syndrome and bilateral chRCC in a 60-year-old woman. **(a)** Coronal CT image of the lungs shows multiple small lung cysts (arrows). **(b, c)** Coronal T2-weighted **(b)** and contrast-enhanced T1-weighted **(c)** MR images show bilateral solid renal masses (arrows).

RCC is seven times more common in patients with BHD than in the general population. Approximately 25%–35% of patients with BHD develop renal tumors, with a mean age at diagnosis of 50 years (16). The RCCs that occur in BHD are frequently bilateral, multifocal, and slow growing. Approximately 90% of the renal tumors seen in BHD are either hybrid oncocytic tumors (mixed oncocytoma and chRCC) or pure chRCCs (10, 14). Other renal tumors seen in patients with BHD include oncocytoma, ccRCC, and papRCC (17).

Pathologic Features

Gross Pathologic Features

chRCCs are unencapsulated but well-circumscribed solid masses with lobulated surfaces (Fig 3) (3,10,17). They are characteristically tan-brown, similar to benign oncocytomas (18). Central scarring, necrosis, hemorrhage, and calcification may be seen at gross assessment (5). On the basis of data from large pathologic series (5–7,12), the mean size of a chRCC at surgical resection is 4.6–8.0 cm, with a downward trend in tumor size in recent years.

Histopathologic Features

Overall architectural features of chRCC most commonly include a well-circumscribed lesion without a fibrous capsule (Fig 4). Exceptions to this pattern include large tumors or tumors with sarcomatoid features, which may show an infiltrative pattern (5). The histologic appearance varies, with alveolar and solid patterns most common in tumors with typical histologic features and a nested pattern common in the eosinophilic variant (5). Tumor cells are typically supported by a delicate fibrous framework, and the vasculature is not usually prominent (5).

chRCC may contain two types of tumor cells. The first type is that of large, pale, polygonal cells with abundant transparent cytoplasm, crinkled “raisinoid” nuclei with perinuclear halos, and prominent cell membranes (18). The second cell type comprises smaller cells with granular and eosinophilic cytoplasm. Both cell types demonstrate irregular nuclei and commonly show binucleation and perinuclear halos. Different proportions of these two cell types lead to different histologic variants. In the classic variant, which comprises the majority of cases, pale cells predominate, although admixed eosinophilic cells are not uncommon. This variant is more frequently associated with necrosis and/or sarcomatoid changes, and these tumors are associated with a higher rate of metastases. In contrast, the eosinophilic variant, which accounts for a minority of the cases, is composed almost entirely of eosinophilic cells. The eosinophilic variant of chRCC is classified as an oncocytic renal neoplasm and may resemble oncocytoma. This variant shows a higher rate of multifocal and bilateral tumors than does the classic variant (10).

Immunohistochemical and Ancillary Studies

Immunohistochemical and ancillary studies may be helpful for confirming the diagnosis of chRCC. Diffuse positivity for cytokeratin 7, c-KIT/CD117, and kidney-specific cadherin is common, and diffuse cytoplasmic staining for Hale colloidal iron is a key diagnostic criterion (3,10,19).

Molecular Pathology Features

chRCC has been shown to commonly display a unique pattern of whole chromosome losses. In approximately 86% of cases, one-copy loss of chromosomes 1, 2, 6, 10, 13, and/or 17 is observed. Additional loses of chromosomes 3, 5, 8, 9, 11, and 18 and chromosome arm 21q can be seen in 12%–58% of cases (2). These patterns of chromosomal loss are typically present in chRCC with classic histologic features, while tumors with eosinophilic histologic features frequently show no chromosomal losses. Common genetic features of chRCC include TP53 mutations and alterations in phosphoinositide 3–kinase/protein kinase B/mammalian target of rapamycin pathway genes, which occur in 31.1% and 18.9% of cases, respectively. Loss of cyclin-dependent kinase inhibitor 2A (CDKN2A) also is a common alteration in chRCC, occurring in 19.8% of cases, and CDKN2A loss or PTEN mutation is associated with poorer survival in chRCC cases (2,20).

Imaging Features

The radiology literature contains multiple case series describing the imaging findings of chRCC. These imaging findings are consistent with the pathologic findings described earlier. chRCCs vary in size, with mean sizes ranging from 3.2 cm to 7.0 cm reported in radiologic case series (21–27). They are usually confined to the kidney, with local invasion or renal vein invasion uncommonly identified at imaging. Nearly all lesions are solid or mostly solid and sharply marginated. They may be homogeneous or heterogeneous, with the heterogeneous appearance predominating. Calcification and/or a central scar with or without a spoke-wheel pattern of enhancement is seen in a significant minority of cases. A dominant cystic component, hemorrhage, fat, and microscopic lipid are very uncommon findings. chRCC is a moderately vascular tumor. It enhances less than the renal cortex in all phases, as expected given the lack of prominent vascularity seen pathologically. In addition, peak enhancement occurs in the nephrographic phase.

US Findings

At US, chRCC is a predominantly solid mass (Figs 5, 6). Case series (27–29) have shown disparate findings regarding the predominant pattern of echogenicity, with chRCCs ranging from very hypoechoic to very hyperechoic. The majority of masses demonstrate detectable vascular flow at Doppler US (27). Reports of the appearance of chRCC on contrast-enhanced US images describe a solid mass with enhancement less than that of the renal cortex (30–32). The tumor-to-cortex ratio is lower than that of ccRCC but similar to that of papRCC (30,31). Enhancement is homogeneous compared with the enhancement of ccRCC (31).

chRCC in a 37-year-old man who presented with gross hematuria. (a, d) Sagittal US image of the kidney (a) shows a well-defined heterogeneous mass (arrow in a) with a central anechoic region (* in a) that was shown to be necrosis on the gross pathologic specimen (* in d), which also shows central scars (circles). (b) Color Doppler US image demonstrates vascular flow (arrow) within the mass. (c) Axial contrast-enhanced excretory phase CT image shows a hypoenhancing mass (arrow) with nonenhancing central necrosis (*). (e) Photomicrograph of an eosinophilic variant of chRCC shows smaller and more uniform cells with prominent eosinophilic (pink) cells and less conspicuous nuclear irregularity and perinuclear clearing. (Hematoxylin-eosin stain; original magnification, ×200.) — chRCC in a 37-year-old man who presented with gross hematuria. **(a, d)** Sagittal US image of the kidney **(a)** shows a well-defined heterogeneous mass (arrow in a) with a central anechoic region (* in a) that was shown to be necrosis on the gross pathologic specimen (* in d), which also shows central scars (circles). **(b)** Color Doppler US image demonstrates vascular flow (arrow) within the mass. **(c)** Axial contrast-enhanced excretory phase CT image shows a hypoenhancing mass (arrow) with nonenhancing central necrosis (*). **(e)** Photomicrograph of an eosinophilic variant of chRCC shows smaller and more uniform cells with prominent eosinophilic (pink) cells and less conspicuous nuclear irregularity and perinuclear clearing. (Hematoxylin-eosin stain; original magnification, ×200.)

chRCC in a 37-year-old woman with flank pain and gross hematuria of 2 weeks duration. (a, b) Longitudinal gray-scale (a) and transverse color Doppler (b) US images demonstrate a solid (black * in a) and cystic (white * in a) mass with internal Doppler flow (arrow in b). (c–e) Axial T2-weighted MR image (c) and noncontrast (d) and contrast-enhanced (e) T1-weighted fat-saturated MR images demonstrate small cystic spaces in the mass (* in c) and areas of enhancement (* in e). — chRCC in a 37-year-old woman with flank pain and gross hematuria of 2 weeks duration. **(a, b)** Longitudinal gray-scale **(a)** and transverse color Doppler **(b)** US images demonstrate a solid (black * in a) and cystic (white * in a) mass with internal Doppler flow (arrow in b). **(c–e)** Axial T2-weighted MR image **(c)** and noncontrast **(d)** and contrast-enhanced **(e)** T1-weighted fat-saturated MR images demonstrate small cystic spaces in the mass (* in c) and areas of enhancement (* in e).

CT Findings

At CT, chRCC is typically a well-circumscribed mass with smooth or lobular contours (Fig 7) (21,26,27). Most chRCCs are solid or mostly solid at CT (27). chRCCs may be homogeneous or heterogeneous, with the heterogeneous appearance more commonly reported (21,26) (Fig 8). Calcification is seen in 14%–34% of cases (Fig 9) (21,26,27). A central scar is present in 19%–34% of cases, with a spoke-wheel pattern of enhancement described in a minority of cases (19,21,27,33). On noncontrast CT images, chRCC tends to be isoattenuating to slightly hyperattenuating compared with the background renal parenchyma (21). chRCC shows moderate enhancement that peaks in the nephrographic phase or less commonly in the corticomedullary phase (21,25,26). Enhancement is less than that of the renal cortex during all phases (26). chRCC enhances less than ccRCC but more than papRCC (25,26).

chRCC in a 40-year-old man with an incidental renal mass. Axial noncontrast (a) and contrast-enhanced (b) corticomedullary phase and contrast-enhanced nephrogenic phase (c) and excretory phase (d) CT images show a well-circumscribed solid mass that enhances less than the adjacent renal cortex (*). — chRCC in a 40-year-old man with an incidental renal mass. Axial noncontrast **(a)** and contrast-enhanced **(b)** corticomedullary phase and contrast-enhanced nephrogenic phase **(c)** and excretory phase **(d)** CT images show a well-circumscribed solid mass that enhances less than the adjacent renal cortex (*).

chRCC in a 57-year-old man who presented to the emergency department for evaluation of sudden-onset urinary retention and intermittent hematuria. (a–c) Axial noncontrast (a) and axial (b) and coronal (c) contrast-enhanced CT images show a renal mass (*) with internal calcifications (arrow in a) and heterogeneous enhancement. (d) Axial contrast-enhanced CT image from below the level of the kidneys shows an enlarged retroperitoneal lymph node (*), compatible with metastatic disease. — chRCC in a 57-year-old man who presented to the emergency department for evaluation of sudden-onset urinary retention and intermittent hematuria. **(a–c)** Axial noncontrast **(a)** and axial **(b)** and coronal **(c)** contrast-enhanced CT images show a renal mass (*) with internal calcifications (arrow in a) and heterogeneous enhancement. **(d)** Axial contrast-enhanced CT image from below the level of the kidneys shows an enlarged retroperitoneal lymph node (*), compatible with metastatic disease.

chRCC in a 51-year-old woman with an incidental right renal mass. Axial noncontrast (a) and contrast-enhanced (b) CT images and coronal contrast-enhanced CT image (c) show a renal mass with calcification (arrow in a) and enhancement (* in b and c) less than that of the renal cortex (arrow in b and c). — chRCC in a 51-year-old woman with an incidental right renal mass. Axial noncontrast **(a)** and contrast-enhanced **(b)** CT images and coronal contrast-enhanced CT image **(c)** show a renal mass with calcification (arrow in a) and enhancement (* in b and c) less than that of the renal cortex (arrow in b and c).

MRI Findings

At MRI, chRCC is a peripherally located well-circumscribed mass (13,23). chRCCs are predominantly solid, without significant areas of necrosis or cystic change (13). These masses are uniformly hypointense on T1-weighted MR images. The T2 signal intensity is variable (22,23,27). The majority of chRCCs are heterogeneous at T2-weighted MRI (22,23). A central scar can be identified at MRI in 33%–40% of cases, with the scar appearing hyperintense in most cases (22,23). Similar to enhancement at CT, enhancement at MRI is less than that of the cortex during all phases and peaks during the nephrographic phase (23). The tumor-to-cortex ratio ranges from 0.5 to 0.6 in the corticomedullary phase and from 0.5 to 0.8 in the nephrographic phase (27,34) (Fig 10). Enhancement tends to be heterogeneous, and segmental enhancement inversion or a spoke-wheel pattern of enhancement can be identified in the minority of cases (13). As at CT, at MRI, chRCC enhances less than ccRCC but more than papRCC (34,35). chRCC has typically demonstrated lower apparent diffusion coefficient values than the more common ccRCC (13,36).

chRCC in a 53-year-old man. (a, b) Axial (a) and coronal (b) T2-weighted MR images show an isointense mass (*) centered at the renal hilum. (c, d) Axial diffusion-weighted image (c) and apparent diffusion coefficient map (d) show restricted diffusion (*). (e–h) Axial noncontrast T1-weighted fat-saturated (e) and contrast-enhanced T1-weighted multiphase fat-saturated (f–h) MR images show an intrinsically T1-hypointense mass (* in e) with enhancement less than that of the adjacent renal cortex (* in f–h). — chRCC in a 53-year-old man. **(a, b)** Axial **(a)** and coronal **(b)** T2-weighted MR images show an isointense mass (*) centered at the renal hilum. **(c, d)** Axial diffusion-weighted image **(c)** and apparent diffusion coefficient map **(d)** show restricted diffusion (*). **(e–h)** Axial noncontrast T1-weighted fat-saturated **(e)** and contrast-enhanced T1-weighted multiphase fat-saturated **(f–h)** MR images show an intrinsically T1-hypointense mass (* in e) with enhancement less than that of the adjacent renal cortex (* in f–h).

PET/CT Findings

Similar to other malignancies, renal cell tumors may show increased glucose metabolism at PET/CT (37). Despite the increased glucose metabolism, fluorine 18 fluorodeoxyglucose (FDG) PET/CT generally has limited utility in the evaluation of RCCs owing to the physiologic excretion of FDG from the kidneys. This physiologic excretion may obscure renal lesions, lowering the sensitivity of FDG PET/CT.

Key Features for Differentiating chRCC from Other Common Renal Masses

No single imaging finding is pathognomonic for chRCC; however, understanding the key imaging features of this cancer allows the radiologist to reliably distinguish chRCC from many important renal lesions that are encountered regularly in clinical practice (Table 2).

Table 2:

Key Features for Distinguishing chRCC from Common Renal Masses

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First, chRCCs are solid or nearly entirely solid lesions without significant regions of necrosis, hemorrhage, or cystic change (13,21,23,27). This finding allows easy differentiation of chRCC from both low– and high–Bosniak-class cystic lesions, including simple cysts, hemorrhagic cysts, multilocular cystic renal neoplasm of low malignant potential, and the mixed epithelial and stromal family of tumors.

Second, chRCCs do not contain macroscopic fat or microscopic lipid, allowing their differentiation from classic angiomyolipomas (23,27). chRCCs also lack the high attenuation at noncontrast CT and the consistently low homogeneous T2 signal intensity characteristic of lipid-poor angiomyolipomas (13,38). In addition, chRCCs show a later peak enhancement compared with lipid-poor angiomyolipomas, which tend to peak in the corticomedullary phase (38).

The differentiation of chRCC from the more common ccRCC relies largely on the reproducible differences in enhancement. At both CT and MRI, chRCC tends to enhance less than the renal cortex in all phases and shows peak enhancement in the nephrographic phase (25,34,35). ccRCC enhances earlier and more intensely, with peak enhancement greater than that of the cortex in the corticomedullary phase (25,34,35). In addition, chRCC is usually localized to the kidney and lacks necrosis, hemorrhage, or cystic change, while ccRCC more commonly shows aggressive behavior such as perinephric fat and/or renal vein invasion, distant metastases, or tumor necrosis (27).

The enhancement pattern also helps in differentiating chRCC from papRCC. Although the enhancement differences are less pronounced, chRCC enhances moderately, while papRCC enhances only mildly. This difference is evident on multiple imaging studies, although it does not always meet statistical significance (25,34,35). papRCC may show relatively low signal intensity on in-phase images compared with on opposed-phase images, a finding that is absent with chRCC due to the lack of hemosiderin (13). papRCC also has a low-signal-intensity capsule, while chRCC appears circumscribed but lacks a defined capsule (13). papRCC is more commonly bilateral and multifocal than is chRCC, unless chRCC occurs in the setting of BHD (13,27).

Last, distinguishing chRCC from oncocytoma remains a challenge at imaging. Studies (23) have shown overlapping qualitative MRI features, including location, margins, presence of a central scar, T2 signal intensity, and enhancement pattern. Quantitative image analysis shows promise for distinguishing these two related clinically indolent tumors (24,39).

RCC Staging

RCCs, including chRCC, are staged by using the American Joint Committee on Cancer TNM staging system. Similar to TNM staging systems for other tumors, the TNM system for RCC classification is based on the size and local extent of the primary tumor (T), regional lymph node metastases (N), and distant metastases (M).

Tumor stage I (T1) disease includes tumors that are limited to the kidney and less than or equal to 7 cm in greatest dimension. T1 tumors are subdivided into T1a and T1b substages by using 4 cm as the dividing criterion. Tumor stage II (T2) disease includes tumors that are limited to the kidney but greater than 7 cm in greatest dimension. Tumor stage III (T3) disease includes tumors that extend into major veins or perinephric fat but not into the ipsilateral adrenal gland and not beyond the Gerota fascia. Tumor stage IV (T4) disease includes tumors that invade beyond the Gerota fascia, including the ipsilateral adrenal gland (40). It is important to note that 40%–70% of chRCCs are stage T1 and 19%–38% are stage T1a at surgery. Extrarenal extension of tumor (stage T3 or T4), including extension into major veins or perinephric fat, is relatively uncommon (12%–23% of cases) (5–7,12).

The extent of disease described by using the TNM system may be used to classify patients with chRCC into clinical stage groups. These groups are early stage (stages I and II: T1–T3, N0, M0), intermediate stage (stage III: T1–T3, N1, M0), and advanced disease (stage IV: T4 and/or M1).

Treatment Considerations

The main treatment for RCC is surgery. For patients with T1a disease, there is a growing body of evidence to support active surveillance or percutaneous ablation in selected patients (41–43). Percutaneous ablation may be performed by using radiofrequency ablation, microwave ablation, or cryoablation (44). For stage I T1b disease, the treatment is partial nephrectomy when feasible. If this treatment is not feasible, then radical nephrectomy should be performed (45). The treatment for stage II and stage III disease is radical nephrectomy, with thrombectomy performed when necessary for stage III (46). Regional lymph node dissection is optional but recommended for patients who have adenopathy at preoperative imaging or at the time of surgery. Stage IV disease is treated with cytoreductive radical nephrectomy if the primary tumor is resectable, followed by systemic therapy (47).

For chRCC associated with BHD, a common treatment size threshold is 3 cm to preserve as much renal function for as long as possible. Treatment may include partial nephrectomy or ablation (14).

chRCC-related Prognoses

The clinical outcomes of patients with chRCC are good, with higher rates of recurrence-free survival (RFS), cancer-specific survival, and overall survival compared with those for patients with ccRCC (6,7,15,48). The rates of RFS in chRCC are 89% and 79% at 5 and 10 years, respectively (6,15). Despite the good prognosis associated with most chRCCs, large tumor size, high T stage, small or large vessel vascular invasion, sarcomatoid differentiation, and tumor necrosis have been identified as negative prognostic indicators in a minority of cases (5–7,12,15). Unlike ccRCCs and papRCCs, chRCCs are not routinely graded owing to their inherent nuclear atypia. Although a few chRCC-specific grading systems have been proposed in the pathologic literature, none have been widely accepted in clinical practice yet (18). The most common sites of disease recurrence or metastasis in chRCC are the liver, lungs, and retroperitoneal lymph nodes (10). Metastatic chRCC is associated with a median survival of approximately 29 months (10).

Conclusion

chRCC is the third most common subtype of RCC. Although it may occur in association with BHD, it frequently develops sporadically and is often an incidental finding discovered at imaging. The pathologic features, including well-circumscribed margins, moderate vascularity, and lack of internal fat or a significant cystic component, predict the imaging findings of a solid well-circumscribed mass with enhancement less than that of the renal cortex in all phases. Knowledge of the clinically indolent behavior and expected imaging pattern of chRCC will aid the radiologist in the evaluation of a solid renal mass detected at imaging.

For this journal-based SA-CME activity, the author A.M.U. has provided disclosures (see end of article); all other authors, the editor, and the reviewers have disclosed no relevant relationships.

The opinions and assertions contained herein are those of the authors and do not reflect those of the Uniformed Services University, the Department of Defense, or the National Institutes of Health.

Disclosures of Conflicts of Interest.— : A.M.U. Activities related to the present article: disclosed no relevant relationships. Activities not related to the present article: paid institution support for sponsored research from Ventana Medical Systems. Other activities: disclosed no relevant relationships.

Abbreviations:

BHD: Birt-Hogg-Dubé syndrome
ccRCC: clear cell RCC
chRCC: chromophobe RCC
papRCC: papillary RCC
RCC: renal cell carcinoma

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46. Luo JH , Zhou FJ , Xie D , et al . Analysis of long-term survival in patients with localized renal cell carcinoma: laparoscopic versus open radical nephrectomy . World J Urol 2010. ; 28 ( 3 ): 289 – 293 . [DOI] [PubMed] [Google Scholar]
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[r42] 42. Motzer RJ , Jonasch E , Michaelson MD , et al . NCCN guidelines insights: kidney cancer, version 2.2020 . J Natl Compr Canc Netw 2019. ; 17 ( 11 ): 1278 – 1285 . [DOI] [PubMed] [Google Scholar]

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[r44] 44. Rivero JR , De La Cerda J 3rd , Wang H , et al . Partial nephrectomy versus thermal ablation for clinical stage T1 renal masses: systematic review and meta-analysis of more than 3,900 patients . J Vasc Interv Radiol 2018. ; 29 ( 1 ): 18 – 29 . [DOI] [PubMed] [Google Scholar]

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[r47] 47. Culp SH , Tannir NM , Abel EJ , et al . Can we better select patients with metastatic renal cell carcinoma for cytoreductive nephrectomy? Cancer 2010. ; 116 ( 14 ): 3378 – 3388 . [DOI] [PubMed] [Google Scholar]

[r48] 48. Frees S , Kamal MM , Knoechlein L , et al . Differences in overall and cancer-specific survival of patients presenting with chromophobe versus clear cell renal cell carcinoma: a propensity score matched analysis . Urology 2016. ; 98 ( 81 ): 87 . [DOI] [PubMed] [Google Scholar]

PERMALINK

Chromophobe Renal Cell Carcinoma with Radiologic-Pathologic Correlation

Jamie Marko, MD

Ryan Craig, BS

Andrew Nguyen, BS

Aaron M Udager, MD, PhD

Darcy J Wolfman, MD

Abstract

SA-CME LEARNING OBJECTIVES

Introduction

Table 1:

Clinical Considerations

Presentation

Figure 1a.

Figure 1b.

Figure 1c.

Epidemiologic Features

Figure 2a.

Figure 2b.

Figure 2c.

Pathologic Features

Gross Pathologic Features

Figure 3a.

Figure 3b.

Figure 3c.

Figure 3d.

Figure 3e.

Histopathologic Features

Figure 4a.

Figure 4b.

Figure 4c.

Figure 4d.

Immunohistochemical and Ancillary Studies

Molecular Pathology Features

Imaging Features

US Findings

Figure 5a.

Figure 6a.

Figure 5b.

Figure 5c.

Figure 5d.

Figure 5e.

Figure 6b.

Figure 6c.

Figure 6d.

Figure 6e.

CT Findings

Figure 7a.

Figure 8a.

Figure 9a.

Figure 7b.

Figure 7c.

Figure 7d.

Figure 8b.

Figure 8c.

Figure 8d.

Figure 9b.

Figure 9c.

MRI Findings

Figure 10a.

Figure 10b.

Figure 10c.

Figure 10d.

Figure 10e.

Figure 10f.

Figure 10g.

Figure 10h.

PET/CT Findings

Key Features for Differentiating chRCC from Other Common Renal Masses

Table 2:

RCC Staging

Treatment Considerations

chRCC-related Prognoses

Conclusion

Abbreviations:

References

ACTIONS

PERMALINK

RESOURCES

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