CASE REPORTS
HOARSENESS;AUNIQUE
CLINICALPRESENTATION
FOR RENAL CELL CARCINOMA RICHARD E. GREENBERG, JEFFERY COOPER, M.D. ROBERT L. KRIGEL, M.D.
M.D.
R. MELVYN RICHTER, M.D. HOWARD KESSLER, M.D. ROBERT 0. PETERSEN, M.D.
From the Departments of Surgery/Urology, Medical Oncology, Radiation Therapy, Radiology, and Pathology, The Fox Chase Cancer Center, Philadelphia, and the Division of Otolaryngology Abington Memorial Hospital, Abington, Pennsylvania
of
ABSTRACT-The first reported case of an isolated metastasis to the larynx from a regionally localized renal cell carcinoma presenting clinically as hoarseness is discussed. Aggressive management and outcome are presented.
Renal cell carcinoma is a common genitourinary malignancy known both for its propensity for early, widespread metastases and its associated poor survival rates. It is unresponsive to radiation and chemotherapy such that aggressive surgery represents the only possibility for cure. Renal cell carcinoma has multiple and diverse methods of presentation. It has been referred to as the “internist’s tumor” in that next to syphilis, renal cell carcinoma is the great imitator.’ The clinical triad of flank pain, mass, and hematuria is as unusual as its presentation secondary to metastases outside the genitourinary system. The world literature has multiple reports of renal cell carcinoma metastasizing to the head and neck region.2-5 However, we believe this is the first report of an isolated metastasis to the larynx from a renal cell carcinoma. Case Report The patient was a relatively healthy fiftyfive-year-old white man with mild hypertension. He presented for evaluation of hoarseness which had been present for approximately six weeks. Physical examination at that time was unremarkable. Indirect laryngoscopy demonstrated only a general subglottic fullness, but no obvious mucosal lesions. This was confirmed at the time of direct laryngoscopy and biopsy.
UROLOGY
i AUGUST1992
/ VOLUME40,NUMBER2
Prior to the biopsy, the patient had a computerized axial tomography (CAT) scan of the neck which showed a 1.5 cm subglottic tumor on the left side (Fig. 1A). Microscopic review of biopsy tissue demonstrated clear cell carcinoma. Retrospective questioning still failed to generate any significant urinary symptoms, or any significant constitutional symptoms. The patient then underwent a CAT scan of the abdomen which demonstrated a localized left renal mass consistent with a renal cell carcinoma (Fig. 1B). Extensive additional metastatic evaluation failed to reveal any other sites of me&stases. Following the original biopsy the patient required a tracheostomy. Since the major clinical problem at this point was the laryngeal lesion, the patient underwent local irradiation using a 6 MeV radiation source for a total treatment dose of 5,000 rad to the subglottic region. This was given in 25 fractions over forty-four days. Next, the patient had a repeat extent-of-disease evaluation which included CAT scans of the head, neck, thorax, and abdomen, bone scan, and full chemical profile. All of these studies continued to show only the primary left renal mass and the solitary laryngeal lesion, both without change. Six months after his initial presentation the patient underwent a total laryngectomy. Obvious residual metastatic clear cell carcinoma was seen in the submucosal
159
tissues. Three months later a repeat evaluation again failed to demonstrate any evidence of either locally recurrent disease in the neck, progression of the primary left renal tumor, or new metastases. The patient subsequently underwent a left radical nephrectomy including a para-aortic lymphadenectomy. The final pathologic diagnosis was renal cell carcinoma, clear cell type, confined to the kidney. The patient’s postoperative course was entirely without complication. He is now five years status post nephrectomy and five and one-half years since his initial presentation. He remains clinically free of disease being re-evaluated for evidence of both locally recurrent disease as well as new metastases every three months.
Comment The natural history of renal cell carcinoma is certainly unpredictable for the individual patient. Despite the increased incidence of finding incidental, localized and therefore unexpected renal cancers by the newer imaging modalities, approximately 40 percent of malignant renal tumors will have multiple metastases at the time of presentation. Equally disheartening for the urologist is the extremely poor prognosis that this advanced stage of renal cell carcinoma portends. Indeed, the median survival of metastatic renal carcinoma is less than eight months, with 80 percent of patients dead within one year and virtually no survivors at two years. Furthermore, in 50 percent of those patients surgically treated for clinically “curable” disease metastases will develops; yet, prolonged survival is documented. Two reports in the mid1960s demonstrated the delayed clinical onset of metastatic disease of over thirty years.7,8 The role of the “adjunctive nephrectomy” has been discussed in detail in the literature. Initially done for purposes of symptomatic palliation, it is now rarely performed except in the context of specific experimental protocols where large amounts of tissue are required or where debulking of tumor volume is thought to be of some benefit. There does not appear to be any statistical survival advantage in performing a nephrectomy in the presence of multiple metastatic lesions.e-12 The exception to this general rule may be multiple osseous metastases where adjuvant nephrectomy has been reported to significantly prolong life.13 Solitary metastasis from a renal cell carcinoma is a rare finding and reported in approximately 2 percent of large series. It is in this small subgroup of those patients with metastases that aggressive surgical management appears to offer the best possibility for long-term survival and possibly cure.14-le Following removal of the primary tumor and a solitary metastasis 45 percent of patients survived three years and 34 percent at least five years.17 Solitary synchronous metastasis from renal cell carcinoma that present clinically with signs and symptoms referable to the metastasis, in the context of an undiagnosed renal cell carcinoma primary, as exemplified by this case, are even more uncommon. There are 110 cases reported in the literature.rs Of these, only approximately 10 percent of patients are alive after two years. This poor outcome in part may be secondary to a lack of surgical aggressiveness
160
UROLOGY
FICURE~. (A) CT scan of neck at level of true cord. Exophytic mass displacing left true cord medially (arrows). Destruction of arytenoid and cricoid cartilage is also seen (arrowheads) in addition to destruction of thyroid cartilage (curved arrow). (B) Contrast-enhanced CT scan of abdomen demonstrates solid, partially necrotic 6 x 8 cm-mass originating from inferior aspect of left kidney (arrows); l-cm cyst is seen in medial aspect of right kidney.
I AUGUST1992
/ VOLUME40,NUMBER2
in the management of these patients. For our patient, who has already outlived the predicted survival for an individual with metastatic renal cell carcinoma, and others with an isolated metastasis, the surgically aggressive approach taken in this case seems justified. Fox Chase Cancer Center 7701 Burholme Avenue Philadelphia, Pennsylvania 19111 References 1. Ochsner MC: Renal cell carcinoma: five year follow-up study of 70 cases, J Uro193: 361 (1965). 2. Enerath C-M, Martensson G, and Thulin A: Profuse epistaxsis in hypernephroma metastasis, Acta Otolaryngol 53: 546 (1961). 3. Sellstrom LG: Hypernephroma metastases in the ear and nose region, Acta Otolaryngol 55: 545 (1962). 4. Friedmann I, and Osborn DA: Metastatic tumor in the ear, nose and throat region, J Laryngol Otol 79: 576 (1965). 5. Stankiewicz C, and Mostowski L: Laryngeal metastasis of clear cell renal carcinoma, Otolaryngol Po133: 543 (1979). 6. deKernion JB, Ramming KP, and Smith RB: The natural history of renal cell carcinoma: a computer analysis, J Urol 120: 148 (1978).
UROLOGY
/
AUGUST
1992
I
VOLUME
40, NUMBER
2
7. Kradjian RM, and Bennington JL: Renal carcinoma recurrent 31 years after nephrectomy, Arch Surg 99: 192 (1965). 8. Takats LJ, and Czapo Z: Death from renal cancer 37 years after original recognition. Cancer 19: 1172 (1966). 9. Johnson DE, Kaesler KE, and Samuels ML: Is nephrectomy justified in patients with me&static renal cell carcinoma? J Urol 114: 27 (1975). 10. Freed SZ: Nephrectomy for renal cell carcinoma with metastases, Urology 9: 613 (1977). 11. Waters WB, and Richie JP: Aggressive surgical approach to renal cell carcinoma with solitary metastases, J Urol 122: 306 (1979). 12. deKernion JB: Treatment of advanced renal cell carcinoma, J Urol 130: 2 (1983). 13. Montie JE, et al: The role of adjunctive nephrectomy in patients with metastatic renal cancer, J Urol 117: 272 (1977). 14. Middleton RG: Surgery for metastatic renal cell carcinoma, J Urol 97: 973 (1967). 15. Weigensberg IJ: Metastatic renal cancer: unusual and deceptive features, South Med J 65: 611 (1972). 16. O’Dea MJ, Zincke H, Utz, DC, and Bernatz PE: The treatment of renal cell carcinoma with solitary metastases, J Uroll20: 540 (1978). 17. Appelquist P: The role and value of surgery in metastatic renal adenocarcinoma: a retrospective study of 106 cases, J Surg Oncol 26: 138 (1984). 18. Petersen RO: Renal Cell Carcinoma, in; Urologic Pathology Philadelphia, JB Lippincott Co, 1986, chap 1, pp 58-76.
161
HOARSENESS;AUNIQUE
CLINICALPRESENTATION
FOR RENAL CELL CARCINOMA RICHARD E. GREENBERG, JEFFERY COOPER, M.D. ROBERT L. KRIGEL, M.D.
M.D.
R. MELVYN RICHTER, M.D. HOWARD KESSLER, M.D. ROBERT 0. PETERSEN, M.D.
From the Departments of Surgery/Urology, Medical Oncology, Radiation Therapy, Radiology, and Pathology, The Fox Chase Cancer Center, Philadelphia, and the Division of Otolaryngology Abington Memorial Hospital, Abington, Pennsylvania
of
ABSTRACT-The first reported case of an isolated metastasis to the larynx from a regionally localized renal cell carcinoma presenting clinically as hoarseness is discussed. Aggressive management and outcome are presented.
Renal cell carcinoma is a common genitourinary malignancy known both for its propensity for early, widespread metastases and its associated poor survival rates. It is unresponsive to radiation and chemotherapy such that aggressive surgery represents the only possibility for cure. Renal cell carcinoma has multiple and diverse methods of presentation. It has been referred to as the “internist’s tumor” in that next to syphilis, renal cell carcinoma is the great imitator.’ The clinical triad of flank pain, mass, and hematuria is as unusual as its presentation secondary to metastases outside the genitourinary system. The world literature has multiple reports of renal cell carcinoma metastasizing to the head and neck region.2-5 However, we believe this is the first report of an isolated metastasis to the larynx from a renal cell carcinoma. Case Report The patient was a relatively healthy fiftyfive-year-old white man with mild hypertension. He presented for evaluation of hoarseness which had been present for approximately six weeks. Physical examination at that time was unremarkable. Indirect laryngoscopy demonstrated only a general subglottic fullness, but no obvious mucosal lesions. This was confirmed at the time of direct laryngoscopy and biopsy.
UROLOGY
i AUGUST1992
/ VOLUME40,NUMBER2
Prior to the biopsy, the patient had a computerized axial tomography (CAT) scan of the neck which showed a 1.5 cm subglottic tumor on the left side (Fig. 1A). Microscopic review of biopsy tissue demonstrated clear cell carcinoma. Retrospective questioning still failed to generate any significant urinary symptoms, or any significant constitutional symptoms. The patient then underwent a CAT scan of the abdomen which demonstrated a localized left renal mass consistent with a renal cell carcinoma (Fig. 1B). Extensive additional metastatic evaluation failed to reveal any other sites of me&stases. Following the original biopsy the patient required a tracheostomy. Since the major clinical problem at this point was the laryngeal lesion, the patient underwent local irradiation using a 6 MeV radiation source for a total treatment dose of 5,000 rad to the subglottic region. This was given in 25 fractions over forty-four days. Next, the patient had a repeat extent-of-disease evaluation which included CAT scans of the head, neck, thorax, and abdomen, bone scan, and full chemical profile. All of these studies continued to show only the primary left renal mass and the solitary laryngeal lesion, both without change. Six months after his initial presentation the patient underwent a total laryngectomy. Obvious residual metastatic clear cell carcinoma was seen in the submucosal
159
tissues. Three months later a repeat evaluation again failed to demonstrate any evidence of either locally recurrent disease in the neck, progression of the primary left renal tumor, or new metastases. The patient subsequently underwent a left radical nephrectomy including a para-aortic lymphadenectomy. The final pathologic diagnosis was renal cell carcinoma, clear cell type, confined to the kidney. The patient’s postoperative course was entirely without complication. He is now five years status post nephrectomy and five and one-half years since his initial presentation. He remains clinically free of disease being re-evaluated for evidence of both locally recurrent disease as well as new metastases every three months.
Comment The natural history of renal cell carcinoma is certainly unpredictable for the individual patient. Despite the increased incidence of finding incidental, localized and therefore unexpected renal cancers by the newer imaging modalities, approximately 40 percent of malignant renal tumors will have multiple metastases at the time of presentation. Equally disheartening for the urologist is the extremely poor prognosis that this advanced stage of renal cell carcinoma portends. Indeed, the median survival of metastatic renal carcinoma is less than eight months, with 80 percent of patients dead within one year and virtually no survivors at two years. Furthermore, in 50 percent of those patients surgically treated for clinically “curable” disease metastases will develops; yet, prolonged survival is documented. Two reports in the mid1960s demonstrated the delayed clinical onset of metastatic disease of over thirty years.7,8 The role of the “adjunctive nephrectomy” has been discussed in detail in the literature. Initially done for purposes of symptomatic palliation, it is now rarely performed except in the context of specific experimental protocols where large amounts of tissue are required or where debulking of tumor volume is thought to be of some benefit. There does not appear to be any statistical survival advantage in performing a nephrectomy in the presence of multiple metastatic lesions.e-12 The exception to this general rule may be multiple osseous metastases where adjuvant nephrectomy has been reported to significantly prolong life.13 Solitary metastasis from a renal cell carcinoma is a rare finding and reported in approximately 2 percent of large series. It is in this small subgroup of those patients with metastases that aggressive surgical management appears to offer the best possibility for long-term survival and possibly cure.14-le Following removal of the primary tumor and a solitary metastasis 45 percent of patients survived three years and 34 percent at least five years.17 Solitary synchronous metastasis from renal cell carcinoma that present clinically with signs and symptoms referable to the metastasis, in the context of an undiagnosed renal cell carcinoma primary, as exemplified by this case, are even more uncommon. There are 110 cases reported in the literature.rs Of these, only approximately 10 percent of patients are alive after two years. This poor outcome in part may be secondary to a lack of surgical aggressiveness
160
UROLOGY
FICURE~. (A) CT scan of neck at level of true cord. Exophytic mass displacing left true cord medially (arrows). Destruction of arytenoid and cricoid cartilage is also seen (arrowheads) in addition to destruction of thyroid cartilage (curved arrow). (B) Contrast-enhanced CT scan of abdomen demonstrates solid, partially necrotic 6 x 8 cm-mass originating from inferior aspect of left kidney (arrows); l-cm cyst is seen in medial aspect of right kidney.
I AUGUST1992
/ VOLUME40,NUMBER2
in the management of these patients. For our patient, who has already outlived the predicted survival for an individual with metastatic renal cell carcinoma, and others with an isolated metastasis, the surgically aggressive approach taken in this case seems justified. Fox Chase Cancer Center 7701 Burholme Avenue Philadelphia, Pennsylvania 19111 References 1. Ochsner MC: Renal cell carcinoma: five year follow-up study of 70 cases, J Uro193: 361 (1965). 2. Enerath C-M, Martensson G, and Thulin A: Profuse epistaxsis in hypernephroma metastasis, Acta Otolaryngol 53: 546 (1961). 3. Sellstrom LG: Hypernephroma metastases in the ear and nose region, Acta Otolaryngol 55: 545 (1962). 4. Friedmann I, and Osborn DA: Metastatic tumor in the ear, nose and throat region, J Laryngol Otol 79: 576 (1965). 5. Stankiewicz C, and Mostowski L: Laryngeal metastasis of clear cell renal carcinoma, Otolaryngol Po133: 543 (1979). 6. deKernion JB, Ramming KP, and Smith RB: The natural history of renal cell carcinoma: a computer analysis, J Urol 120: 148 (1978).
UROLOGY
/
AUGUST
1992
I
VOLUME
40, NUMBER
2
7. Kradjian RM, and Bennington JL: Renal carcinoma recurrent 31 years after nephrectomy, Arch Surg 99: 192 (1965). 8. Takats LJ, and Czapo Z: Death from renal cancer 37 years after original recognition. Cancer 19: 1172 (1966). 9. Johnson DE, Kaesler KE, and Samuels ML: Is nephrectomy justified in patients with me&static renal cell carcinoma? J Urol 114: 27 (1975). 10. Freed SZ: Nephrectomy for renal cell carcinoma with metastases, Urology 9: 613 (1977). 11. Waters WB, and Richie JP: Aggressive surgical approach to renal cell carcinoma with solitary metastases, J Urol 122: 306 (1979). 12. deKernion JB: Treatment of advanced renal cell carcinoma, J Urol 130: 2 (1983). 13. Montie JE, et al: The role of adjunctive nephrectomy in patients with metastatic renal cancer, J Urol 117: 272 (1977). 14. Middleton RG: Surgery for metastatic renal cell carcinoma, J Urol 97: 973 (1967). 15. Weigensberg IJ: Metastatic renal cancer: unusual and deceptive features, South Med J 65: 611 (1972). 16. O’Dea MJ, Zincke H, Utz, DC, and Bernatz PE: The treatment of renal cell carcinoma with solitary metastases, J Uroll20: 540 (1978). 17. Appelquist P: The role and value of surgery in metastatic renal adenocarcinoma: a retrospective study of 106 cases, J Surg Oncol 26: 138 (1984). 18. Petersen RO: Renal Cell Carcinoma, in; Urologic Pathology Philadelphia, JB Lippincott Co, 1986, chap 1, pp 58-76.
161
