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Neuroradiology Case of the Week

Case 449

October 2009

Hossam El-Khouli, MD, David Tuttle, MD,
and Per-Lennart Westesson, MD, PhD, DDS

Clinical Presentation: A 71-year-old man with a history of renal cell carcinoma diagnosed 19 years ago, status post left nephrectomy, now presents with complex partial seizures.

Imaging Findings: CECT shows a mass occupying the right masticator space with associated osseous destructive changes to the lateral wall of the orbit. MRI demonstrates a large soft tissue mass within the right masticator space, with heterogenous signal on T2, and showing T1 post-contrast enhancement. The lesion is hypermetabolic on PET.

Diagnosis: Pathologically proven metastatic renal cell carcinoma to the right masticator space, 19 years post-left nephrectomy.

Discussion: Renal cell carcinoma (RCC) represents 2% of all adult malignant cancers [1,2]. According to the American Cancer Society, in 2007 there will be 51,590 cases (31,990 in males and 19,600 in females) of malignant tumors of the kidney diagnosed in the United States, with 12,890 deaths (8,080 in males and 4,810 in females); renal cell cancer accounted for 80% of this incidence and mortality [1].

• RCC is the eighth or ninth leading cause of cancer death in the United States [3].
• It is more common in people of Northern European ancestry than in those of Asian or African descent. In the United States, the incidence has been equivalent among whites and African Americans, but the incidence among African Americans is increasing rapidly [2].
• RCC has a male-to-female preponderance of 1.6:1.
• It occurs most commonly in the fourth to sixth decades of life [3], but has been reported in younger people who belong to family clusters
• At least 4 hereditary syndromes associated with RCC are recognized: (1) von Hippel-Lindau (VHL) syndrome, (2) hereditary papillary renal carcinoma (HPRC), (3) familial renal oncocytoma (FRO) associated with Birt-Hogg-Dube syndrome (BHDS), and (4) hereditary renal carcinoma (HRC).
• RCC may remain clinically occult. The classic triad of flank pain, hematuria, and flank mass is uncommon (10%) and is indicative of advanced disease.
• RCC metastasizes in about 33% of patients at initial presentation.
• In 30% of patients metastatic lesions develops during the course of the disease [4], and locally recurrence happens in about 5% [5].
• The most frequent sites of RCC metastatic deposites are lung (50-60%), bone (30-40%), liver (30-40%) and brain (5%).
• Less common sites include the contralateral kidney, adrenal glands, pancreas, mesentry and abdominal wall.
• The head and neck are unusual sites for metastases, but skin, skeletal muscle, thyroid, nasal cavity and paranasal sinus metastases have been reported [4-6].
• The time interval between the diagnosis of the primary tumor and the occurence of the metastases to sites such as the brain spans several years (0 - 190 months) [7,8 ].

     Common presenting symptoms of metastatic RCC in the head and neck include enlarged solitary mass, epistaxis, facial tenderness and nasal obstruction, depending on the tumor location and extent of invasion.

References:

1. Levine E, King BF Jr. Adult malignant renal parenchymal neoplasms. In: Pollack HM, McClennan BL, Eds. Clinical Urography. 2nd ed., Philadelphia: Saunders, 2000:1440–1559.
2. American Cancer Society. Cancer facts and figures 2000. Atlanta, Ga: American Cancer Society, 2000.
3. American Cancer Society. Statistics for 2007. American Cancer Society. Available at http://www.cancer.org/docroot/STT/STT_0.asp. Accessed November, 2007.
4. Flanigan RC, Campbell SC, Clark JI, Picken MM. Metastatic renal cell carcinoma. Curr Treat Options Oncol. 2003 Oct;4(5):385-90. PMID: 12941198 [PubMed]
5. Russo P. Seeking the problem of metastatic renal carcinoma. Cancer. 2003 Jun 15;97(12):2941-4. PMID: 12784326 [PubMed]
6. Janzen NK,Kim HL, Figlin RA, Belldgrun AS. Surveillance after radical or partial nephrectomy for localized renal cell carcinoma and management of recurrent disease. Urol Clin North Am. 2003 Nov;30(4):843-52. PMID: 14680319 [PubMed]
7. Harada Y, Nonomura N, Kondo M, et al Clinical study of brain metastasis of renal cell carcinoma. Eur Urol. 1999 Sep;36(3):230-5. PMID: 10450008 [PubMed]
8. Postler E, Meyermann R. Brain metastasis in renal cell carcinoma: clinical data and neuropathological differential diagnoses. Anticancer Res. 1999 Mar-Apr;19(2C):1579-81. PMID: 10365149 [PubMed]