Renal cell carcinomaA CT scan showing bilateral renal cell carcinomas
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Renal cell carcinoma

Renal cell carcinoma, also known by the eponym Grawitz tumor, is the most common form of kidney cancer arising from the renal tubule. It is the most common type of kidney cancer in adults. Initial therapy is with surgery. more...

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It is notoriously resistant to radiation therapy and chemotherapy, although some cases respond to immunotherapy.

Signs and symptoms

The classic triad is hematuria (blood in the urine), flank pain and an abdominal mass. This "classic triad" is infrequently present when the patient first presents for medical attention.

Other signs may include:

  • Abnormal urine color (dark, rusty, or brown) due to blood in the urine
  • Weight loss of more than 5% of body weight with emaciated, thin, malnourished appearance
  • More and more frequently, renal cell carcinoma is identified as an incidental finding on a medical imaging study of the abdomen (e.g. computed tomography a.k.a. CT) done for an unrelated purpose
  • The presenting symptom may be due to to metastatic disease, such as a pathologic fracture of the hip due to a metastasis to the bone
  • Enlargement of one testicle (usually the left, due to blockage of the left gonadal vein by tumor invasion of the left renal vein -- the right gonadal vein drains directly into the inferior vena cava)
  • Paraneoplastic phenomena (not directly due to the mass, but due to secreted substances with hormonal activity):
    • Vision abnormalities
    • Pallor or plethora
    • Excessive hair growth (females)
    • Constipation
    • Cold intolerance
  • High blood pressure

Causes

Renal cell carcinoma affects about three in 10,000 people, resulting in about 31,000 new cases in the US per year. Every year, about 12,000 people in the US die from renal cell carcinoma. It is more common in men than women, usually affecting men older than 55.

Why the cells become cancerous is not known. A history of smoking greatly increases the risk for developing renal cell carcinoma. Some people may also have inherited an increased risk to develop renal cell carcinoma, and a family history of kidney cancer increases the risk.

People with von Hippel-Lindau disease, a hereditary disease that also affects the capillaries of the brain, commonly also develop renal cell carcinoma. Kidney disorders that require dialysis for treatment also increase the risk for developing renal cell carcinoma.

Pathology

Gross examination shows a hypervascular lesion in the renal cortex, which is frequently multilobulated, yellow (because of the lipid accumulation) and calcified.

Light microscopy shows tumor cells forming cords, papillae, tubules or nests, and are atypical, polygonal and large. Because these cells accumulate glycogen and lipids, their cytoplasm appear "clear", lipid-laden, the nuclei remain in the middle of the cells, and the cellular membrane is evident. Some cells may be smaller, with eosinophilic cytoplasm, resembling normal tubular cells. The stroma is reduced, but well vascularized. The tumor grows in large front, compressing the surrounding parenchyma, producing a pseudocapsule.

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A case of renal cell carcinoma metastatic to the nose and tongue - Original Article
From Ear, Nose & Throat Journal, 5/1/03 by Emer E. Lang

Abstract

Reported cases of renal cell carcinoma metastatic to the nose or to the tongue are rare. No case of renal cell carcinoma metastatic to both sites has been previously described. In this article, we report what to our knowledge is the first such case. The nasal metastasis grew rapidly and was treated with radiotherapy. Subsequently, a rapidly enlarging tongue metastasis developed, and it was removed via laser excision. Despite treatment, the patient died within 4 months of the diagnosis.

Introduction

Renal cell carcinoma is the third most common neoplasm reported to metastasize to the head and neck region; only breast and lung carcinomas do so more often. (1-3) Renal cell carcinoma is (1) the most common tumor of infraclavicular origin to metastasize to the paranasal sinuses, larynx, and skin of the head and neck region, (2) the second most common neoplasm to metastasize to the jaws and temporal bones, and (3) the third most common tumor to metastasize to the thyroid and parotid glands. (1-3) In contrast, the literature contains only 26 cases of renal cell carcinoma metastatic to the nose and only eight cases metastatic to the tongue. (4,5) In this article, we describe the case of a patient whose renal cell carcinoma metastasized to both the nose and tongue. To our knowledge, this is the first report of such a case.

Case report

A 45-year-old man came to the Beaumont Hospital in Dublin, Ireland, with a 2-month history of dyspnea, productive cough, lethargy, and night sweats. Chest x-ray detected a diffuse nodular shadowing that was consistent with a pulmonary metastasis. Computed tomography of the abdomen identified a left renal mass, and biopsy confirmed that it was a clear-cell carcinoma. The patient was started on a course of interleukin and interferon therapy.

Four weeks later, the patient began to experience epistaxis. Examination of his nose revealed the presence of a large fungating mass that had arisen from the right side of the nasal septum. Biopsy examination identified the mass as a metastatic renal cell carcinoma. The patient was treated with a 2-week course of radiotherapy, but the lesion continued to enlarge.

Eight weeks after his initial diagnosis, the patient developed a pedunculated fungating mass at the tip of his tongue (figure). Biopsy confirmed that this lesion was also a metastatic renal cell carcinoma. Histology identified the typical pattern of large polygonal cells with central round or oval, hyperchromatic nuclei and abundant clear cytoplasm. The patient's general condition deteriorated, and the tongue mass enlarged rapidly, impairing his speech and swallowing and threatening his airway. The tongue lesion was successfully removed via laser excision. The patient died of his disease shortly thereafter.

Discussion

Renal cell carcinoma typically affects men between the ages of 30 and 60 years. (5) It can metastasize to any location in the body, and its propensity to metastasize to unusual sites has been well documented. The most common sites of metastasis are the lungs (75% of cases), regional lymph nodes (65%), bone (40%), and liver (40%). (6) Approximately 15% of renal cell carcinomas metastasize to the head and neck region--specifically, to the paranasal sinuses, larynx, jaws, temporal bones, thyroid gland, and parotid glands. (6,7) Renal cell carcinoma is the most common metastasis reported 'in the paranasal sinuses. One explanation for the relatively high incidence of spread to the head and neck appears to be that tumor emboli bypass the pulmonary filters by proceeding via Batson's venous plexus between the valveless vertebral and epidural venous systems. (7)

Nasal metastases can cause swelling, nasal obstruction, and pain, but epistaxis is the chief symptom (>70% of cases). (8) The previously reported cases of metastasis to the tongue occurred in patients with generalized dissemination, particularly to the lung. (9) Possible routes of metastasis to the tongue include the systemic, venous, and lymphatic circulations. (7) The most common site of lingual metastasis is the basal region of the tongue; this is believed to be a result of either the tongue's rich vascular supply (via the dorsal lingual arteries) or to the relative immobility of this area compared with other areas of the tongue. (9) The metastatic deposits from renal cell carcinoma are vascular in nature, and they often bleed.

The histopathologic characteristics of metastatic renal cell carcinoma include the presence of encapsulating connective tissue, clear cell borders, round- or oval-shaped nuclei, and abundant clear cytoplasm.

The clinical course of the primary tumor is often unpredictable. Primary tumors vary in their growth rate, in the reported rates of metastasis, and in the patterns of metastatic spread. Spontaneous regression has been noted, as has the occurrence of metastasis several years after the primary has been resected. (10)

The overall prognosis for patients with multiple metastases is poor, and 5-year survival rates have been reported to range from 0 to 7%. (11) Most patients with metastases to the tongue died of their disease within 6 months of diagnosis. (9) Treatment should be individualized according to the location of the tumor and the general health of the patient. If a lesion can be resected without causing gross disturbance of function or form, substantive palliation can ensue. (9)

References

(1.) Batsakis JG. Tumors of the Head and Neck: Clinical and Pathological Considerations. 2nd ed. Baltimore: Williams and Wilkins, 1979:240.51.

(2.) Bernstein JM, Montgomery WW, Balogh K, Jr. Metastatic tumors to the maxilla, nose, and paranasal sinuses. Laryngoscope 1996;76:621-50.

(3.) Nahum AM, Bailey BJ. Malignant tumors metastatic to the paranasal sinuses: Case report and review of the literature. Laryngoscope 1963;73:942-53.

(4.) Cheng ET, Greene D, Koch RJ. Metastatic renal cell carcinoma to the nose. Otolaryngol Head Neck Surg 2000;122:464.

(5.) Lim RY, Bastug DF, Caldwell BL. Metastatic renal cell carcinoma of the nasal septum. WV Med J 1989;85: 143-5.

(6.) Som PM, Norton KI, Shugar JM, et al. Metastatic hypernephroma to the head and neck. AJNR Am J Neuroradiol 1987;8:1103-6.

(7.) Boles R, Cemy J. Head and neck metastases from renal carcinomas. Mich Med 1971;70:616-18.

(8.) Sgouras ND, Gamatsi IE, Porfyris EA, et al. An unusual presentation of a metastatic hypernephroma to the frontonasal region. Ann Plast Surg 1995;34:653-6.

(9.) Okabe Y, Ohoka H, Miwa T, et al. View from beneath: Pathology in focus. Renal cell carcinoma metastasis to the tongue. J Laryngol Otol 1992;106:282-4.

(10.) Pantuck AJ, Zisman A, Belldegrun A. Gene and immune therapy for renal cell carcinoma. Int J Urol 2001:8:S1-4.

(11.) Hefer T, Joachims HZ, Golz A. Metastatic renal cell carcinoma to the nose. Eur Arch Otorhinolaryngol 1994;251:127-9.

From the Department of Otolaryngology--Head and Neck Surgery (Dr. Lang, Dr. Patil, Dr. R. Walsh, and Dr. M. Walsh) and the Department of Pathology (Dr. Leader), Beaumont Hospital, Dublin, Ireland.

Reprint requests: Dr. E.E. Lang, Department of Otolaryngology, The Emory Clinic, Emory University Hospital, 1365A Clifton Rd. N.E., Atlanta, GA 30322. Phone: (404) 778-5329; fax: (404)727-9643; e-mail: emerlang@eircom.net

COPYRIGHT 2003 Medquest Communications, LLC
COPYRIGHT 2003 Gale Group

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