Table of Contents

Clear cell renal cell carcinoma

Clear cell renal cell carcinoma intracranial metastases

see Clear cell renal cell carcinoma intracranial metastases

Diagnosis

The diagnosis of metastatic ccRCC should be made using tissue biopsy of the primary tumor or a metastatic site with the inclusion of markers and/or stains to support the diagnosis.

Treatment

The International Metastatic RCC Database Consortium risk criteria should be used to inform treatment. Cytoreductive nephrectomy may be offered to select patients with kidney-in-place and favorable- or intermediate-risk disease. For those who have already had a nephrectomy, an initial period of active surveillance may be offered if they are asymptomatic with a low burden of disease. Patients with favorable-risk disease who need systemic therapy may be offered an immune checkpoint inhibitor (ICI) in combination with a vascular endothelial growth factor receptor (VEGFR) tyrosine kinase inhibitor (TKI); patients with intermediate or poor risk should be offered a doublet regimen (no recommendation was provided between ICIs or an ICI in combination with a VEGFR TKI). For select patients, monotherapy with either an ICI or a VEGFR TKI may be offered on the basis of comorbidities. Interleukin-2 remains an option, although selection criteria could not be identified. Recommendations are also provided for second- and subsequent-line therapy as well as the treatment of bone metastases, brain metastases, or the presence of sarcomatoid features. Participation in clinical trials is highly encouraged for patients with metastatic ccRCC.Additional information is available at www.asco.org/genitourinary-cancer-guidelines 1)

IMDC (International Metastatic RCC Database Consortium) Risk Model for Metastatic Renal Cell Carcinoma

https://www.mdcalc.com/calc/3008/imdc-international-metastatic-rcc-database-consortium-risk-model-metastatic-renal-cell-carcinoma

Outcome

Modern surgical planning and a more precise, tailored approach with modern radiosurgery techniques are able to improve the outcome and prolong survival even in aggressive types of renal cell carcinomas that give rise to brain metastases. In more severe cases and even in the case of multiple brain metastases, cranial surgery can be recommended 2)

Case report from the HGUA

A 79-year-old male. The patient has a history of chronic coronary artery disease, hypertension, hypercholesterolemia, gastric ulcer, and benign prostatic hyperplasia. A family history of colon and skin cancer. The patient has a history of clear cell renal carcinoma and pulmonary metastasis of melanoma melanocytic of unknown origin, which was treated with surgery and adjuvant nivolumab. The patient has a current diagnosis of an enlarged cerebellar lesion with associated edema, with a pattern of enhancement and diffusion facilitation, and microhemorrhages, with a possible vascular origin. There is also evidence of chronic moderate to severe vascular lesions in the white matter. The patient's current treatment includes telmisartan, acetylsalicylic acid, bisoprolol, solifenacin/tamsulosin, rosuvastatin, calcium carbonate/cholecalciferol, and calcifediol.

Case report

A 64-year-old female presented with headache and dizziness, along with abdominal pain for 2 weeks before being admitted. The abdominal computed tomography (CT) scan showed a kidney tumor. The patient developed left hemiplegia, and the brain CT scan showed an intracranial tumor. The patient suggested radical nephrectomy and craniotomy tumor removal. Histopathology of the kidney and brain tumor revealed two different features, which showed RCC and GBM. Immunohistochemistry results confirmed the diagnosis of GBM and IDH1 wild type; coexistent with clear cell RCC.

The coexistence of carcinoma and glioma should be regarded as coincidental cases if they did not accomplish the criteria for tumor-to-tumor metastasis or were proven to be a genetic syndrome. This case report provides an addition to the literature about double primary malignancy in a single patient. More studies are needed to confirm whether they have a causal relationship or merely coincidental findings 3).


A 63-year-old man presented with repeated epistaxis, nasal obstruction and hyposmia of 2-month duration. Radiological studies revealed a mass of the left ethmoid sinus involving anterior skull base.

The patient was treated with craniofacial resection, bifrontal craniotomy combined with an endonasal endoscopic approach. Intraoperatively, a hypervascular paranasal mass invading the dura mater was removed en block. Histologically, the tumor resembled a clear cell renal cell carcinoma, with cuboidal shaped cells having clear cytoplasm. The tumor cells were positive for CK7, S100, vimentin and PAX-8 and negative for CD10 and PAX-2 by immunohistochemistry. No evidence of renal malignancy was found by radiological and clinical examinations.

Following local radiation therapy, the patient was in good health without recurrence for 15 months after the operation.

To the best of Kim et al. knowledge, this is the first reported case of Sinonasal renal cell like adenocarcinoma (SNRCLA) in Korea. Because of its histological feature of clear cytoplasm, SNRCLA needs to be differentiated from clear cell renal cell carcinoma and other salivary clear cell carcinomas. The prognosis of SNRCLA is generally favorable as shown in the previously reported cases. Considering the limited number and follow-up periods of the cases, however, delayed recurrence should be kept in mind for clinicians. 4).

1)
Rathmell WK, Rumble RB, Van Veldhuizen PJ, Al-Ahmadie H, Emamekhoo H, Hauke RJ, Louie AV, Milowsky MI, Molina AM, Rose TL, Siva S, Zaorsky NG, Zhang T, Qamar R, Kungel TM, Lewis B, Singer EA. Management of Metastatic Clear Cell Renal Cell Carcinoma: ASCO Guideline. J Clin Oncol. 2022 Sep 1;40(25):2957-2995. doi: 10.1200/JCO.22.00868. Epub 2022 Jun 21. PMID: 35728020.
2)
Mencser Z, Tóth T, Kis D, Varga Á, Tiszlavicz L, Barzó P. Oligometasztatikus vesesejtes karcinóma idegsebészi ellátásának technikái és indikációi agyi áttétek esetén [Neurosurgical management for metastatic brain tumors in renal cell carcinoma]. Magy Onkol. 2023 Apr 22;67(1):32-37. Hungarian. Epub 2023 Feb 14. PMID: 37086455.
3)
Simanjuntak KAT, Al Fauzi A, Christi AY, Budiono PS, Susilo RI, Haq IBI, Suroto NS, Fauziah D, Djatisoesanto W. Clear-cell renal cell carcinoma and glioblastoma multiforme coexistence: Double primary malignancy, does it have a causal relationship? Surg Neurol Int. 2022 Aug 12;13:361. doi: 10.25259/SNI_598_2022. PMID: 36128116; PMCID: PMC9479614.
4)
Kim NI, Yang JI, Kim SS, Lee JS, Lim SC, Jung S, Lee JH, Moon KS, Lee KH. Sinonasal renal cell-like adenocarcinoma, a unique variant of primary clear cell carcinoma of the head and neck: The first reported case in Korea. Medicine (Baltimore). 2017 Aug;96(31):e7711. doi: 10.1097/MD.0000000000007711. PubMed PMID: 28767609.