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Renal Parenchymal Neurilemoma: A Rare and Unusual Kidney Tumor
0022-534 7/88/1391-0109$02.00/0 THE JOURNAL OF UROLOGY
Vol. 139, January
Copyright © 1988 by The Williams & Wilkins Co.
Printed in U.S.A.
RENAL PARENCHYMAL NEURILEMOMA: A RARE AND UNUSUAL KIDNEY TUMOR WILLIAM J. SOMERS, BUCKLEY TERPENNING, FRANKLIN C. LOWE NICHOLAS A. ROMAS*
AND
From the Departments of Urology and Pathology, St. Luke's/Roosevelt Hospital Center and Department of Urology, College of Physicians and Surgeons of Columbia University, New York, New York
ABSTRACT
We report a case of a neurilemoma arising from the renal parenchyma. Renal neurilemoma is an extremely rare tumor, with only 5 cases previously reported. Because so little is known of its natural history and potential for malignancy we recommend radical nephrectomy as the treatment of choice. (J. Ural., 139: 109-110, 1988) Although neurilemoma or schwannoma is a common tumor of peripheral and auditory nerves only a few cases have been described arising from the kidney. 1- 5 There are many reports of retroperitoneal neurilemoma6 but to date only 5 cases of true renal neurilemoma have been described previously. Of these 5 tumors 2 arose from the collecting system, 2 apparently arose from the parenchyma and 1 was described as having developed from the renal capsule. We report the third case of a parenchymal neurilemoma.
hyalinized blood vessels and an occasional Verocay body were seen. DISCUSSION
Verocay first used the term neurinoma in 1910 to describe a nerve sheath tumor. 7 In 1932 Masson showed that the origin of this type of tumor was probably the Schwann cell and, therefore, he named it a schwannoma.8 In 1935 Stout suggested the
CASE REPORT
L. S., a 55-year-old black woman, was referred to the urology service for evaluation of a mass in the upper pole of the left kidney discovered incidently on excretory urography as part of a gynecological study for uterine myomas. Arteriography revealed the solid nature of the lesion (fig. 1). The mass appeared to be carcinoma of the kidney that did not involve any other structures. Urinalysis, complete blood count and chemistry profile were within normal limits. Metastatic studies, including a chest radiograph, liver function tests and bone scan, were completely negative. Urine cytology was negative for malignancy. Physical examination was unremarkable, without hypertension, flank mass or costovertebral angle tenderness. Left radical nephrectomy was performed through an 11th rib extraperitoneal extrapleural approach. Examination of the specimen revealed a well defined, 5 X 5 cm. mass replacing the upper pole of the kidney. There was no involvement of the collecting system, hilar region or renal vein. Lymphadenopathy was absent and there was no involvement of adjacent retroperitoneal tissue. Convalescence was uneventful and the patient was discharged from the hospital 8 days postoperatively. At 18month followup there was no evidence of recurrence.
FIG. 1. A, arteriogram indicates upper pole mass (arrows). B, nephrogram phase of arteriogram demonstrates upper pole tumor.
PATHOLOGICAL DESCRIPTION
Gross examination of the specimen revealed a 12.5 x 3.2 cm. left kidney with a large, tense, bulging mass arising from the upper pole. Sectioning of the mass demonstrated a 5.1 x 5 cm. unencapsulated well defined tumor circumscribed by renal parenchyma and capsule. The tumor arose from the upper pole and it compressed the collecting system but the epithelium of the collecting system was not involved. The cut surface of the tumor showed soft yellow-white granular tissue in small lobules. Microscopically, there were numerous punctate areas of compact spindle cells in whorls with palisades, consistent with Antoni type A tissue (fig. 2). Antoni type B tissue also was present in the same specimen. Thickened Accepted for publication May 6, 1987. * Requests for reprints: Department of Urology, St. Luke's Hospital, 114th St. and Amsterdam Ave., New York, New York 10025.
FIG. 2. Histology of tumor reveals Antoni type A tissue (arrows). Reduced from X250. 109
110
SOMERS AND ASSOCIATES
term neurilemoma as a compromise between the 2 names, since the actual embryological origin was unknown. 9 Hence, the 2 names are correctly interchangeable and both refer to the same type of tumor arising from the nerve sheath. Neurilemoma has been described in many different areas of the body, although most of the tumors have been found along peripheral nerve roots. It is found rarely in the retroperitoneum and even less rarely in the kidney. There have been only 5 cases of renal neurilemoma reported in the world literature to date, 1- 5 although neurilemoma arising from the perineum, penis, psoas muscle, stomach, cauda equina and tunica vaginalis of the testicle has been reported. 10- 15 When these tumors are found in the retroperitoneum they frequently are misdiagnosed as either sarcoma or renal cell carcinoma. They present as large masses that can displace the ureters and the kidneys. There is no accurate nonoperative method of differentiating these tumors from carcinoma. In 1965 Fein and Hamm reported a case of neurilemoma of the renal pelvis. 1 Their review of the literature included 3 other cases of neurilemoma: a tumor described by Phillips and Baumrucker originating from the renal pelvis, 2 a tumor reported by Kuzmina arising from the renal capsule,3 and a case reported by Martinot and associates apparently arising from the parenchyma but capsular origin could not definitely be excluded. 4 In 1982 Polianichko and associates described a parenchymal tumor that initially was thought to be a perirenal abscess. 5 Since 1965 there have not been any additional cases reported in the English literature. It is generally accepted by pathologists that neurilemoma tissue can be distinguished by the presence of Antoni type A and type B cells in the same specimen. 16 Type A cells are densely packed and ovoid, possess intact nucleii and show condensed peripheral chromatin. There are no myelinated nerve fibers. Verocay bodies are present and characteristic of type A cells. Antoni type B cells are separated widely, and they contain dense osmophilic granules and whorls of membranes similar to collapsed myelin sheaths. 16 They often contain cystic tissue and thickened hyalinized blood vessels. Antoni, who first described the types of tissue, believed that type B cells represented a step in nerve cell degradation. These findings were supported by the work of Sian and Ryan who found edematous transition zones along the borders between types A and B tissue, suggesting an area of cell destruction. 16 The cell of origin is still in question but most pathologists believe it is the Schwann cell. Myelinated axons are not seen in neurilemoma.
In each of the previously reported cases radical nephrectomy was performed, since the preoperative diagnosis was considered to be carcinoma and the final diagnosis was not known until after surgery. The natural history of the tumor remains unknown. The tumor usually is benign when it involves peripheral nerves, although malignant neurilemomas have been reported. 1 - 4 Radical surgery is the preferred method of treatment for renal neurilemomas. We recommend radical nephrectomy, since it is not likely that the diagnosis will be established before or during surgery. REFERENCES 1. Fein, R. L. and Hamm, F. C.: Malignant schwannoma of the renal pelvis: a review of the literature and a case report. J. Urol., 94: 356, 1965. 2. Phillips, C. A. S. and Baumrucker, G.: Neurilemmona (arising in the hilus of left kidney). J. Urol., 73: 671, 1955. 3. Kuzmina, V. E.: Neurinoma of the kidney capsule. Urologiia, 27: 52, 1962. 4. Martinot, M., Dupont, A. and Demaille, A.: Malignant schwannoma of the kidney. J. Urol. Med. Chir., 66: 748, 1960. 5. Polianichko, M. F., Zaderin, V. P. and Ratner, 0. N.: Neurilemmoma pochki. Urol. Nefrol. (Mosk), No. 5, p. 60, Sept.-Oct. 1982. 6. Miller, P. L., Tessler, A., Alexander, S. and Pinck, B. D.: Retroperitoneal neurilemmoma. Urology, 11: 619, 1978. 7. Verocay, J.: Zur Kenntnis der "Neurofibrome." Beitr. z. Path. Anat. u.z. Alig. Path., 48: 1, 1910. 8. Masson, P.: Experimental and spontaneous schwannomas. Amer. J. Path., 8: 367, 1932. 9. Stout, A. P.: The peripheral manifestations of the specific nerve sheath tumor (neurilemmoma). Amer. J. Cancer, 24: 751, 1935. 10. Carlson, H. E.: Neurilemmoma of the perineum. J. Urol., 94: 158, 1965. 11. Peral Aranda, E.: Neurinoma of the penis. Arch. Espana Urol., 10: 309, 1954. 12. Syred, D. B.: Neurilemmoma within psoas. Brit. J. Rad., 25: 108, 1952. 13. Gronbaeck, P.: Two cases of gastric neurinoma and a case of neurinoma in rectovaginal septum. Ugesk. Iaeger., 114: 246, 1952. 14. Fowler, M.: Malignant neurilemmoma arising in cauda equina. Med. J. Aust., 1: 236, 1955. 15. Cowen, R.: Tumor of the tunica vaginalis testis: case report of neurilemmoma. J. Urol., 77: 59, 1977. 16. Sian, C. J. and Ryan, S. F.: The ultrastructure of neurilemmoma with emphasis on Antoni B tissue. Hum. Path., 12: 145, 1981.
Vol. 139, January
Copyright © 1988 by The Williams & Wilkins Co.
Printed in U.S.A.
RENAL PARENCHYMAL NEURILEMOMA: A RARE AND UNUSUAL KIDNEY TUMOR WILLIAM J. SOMERS, BUCKLEY TERPENNING, FRANKLIN C. LOWE NICHOLAS A. ROMAS*
AND
From the Departments of Urology and Pathology, St. Luke's/Roosevelt Hospital Center and Department of Urology, College of Physicians and Surgeons of Columbia University, New York, New York
ABSTRACT
We report a case of a neurilemoma arising from the renal parenchyma. Renal neurilemoma is an extremely rare tumor, with only 5 cases previously reported. Because so little is known of its natural history and potential for malignancy we recommend radical nephrectomy as the treatment of choice. (J. Ural., 139: 109-110, 1988) Although neurilemoma or schwannoma is a common tumor of peripheral and auditory nerves only a few cases have been described arising from the kidney. 1- 5 There are many reports of retroperitoneal neurilemoma6 but to date only 5 cases of true renal neurilemoma have been described previously. Of these 5 tumors 2 arose from the collecting system, 2 apparently arose from the parenchyma and 1 was described as having developed from the renal capsule. We report the third case of a parenchymal neurilemoma.
hyalinized blood vessels and an occasional Verocay body were seen. DISCUSSION
Verocay first used the term neurinoma in 1910 to describe a nerve sheath tumor. 7 In 1932 Masson showed that the origin of this type of tumor was probably the Schwann cell and, therefore, he named it a schwannoma.8 In 1935 Stout suggested the
CASE REPORT
L. S., a 55-year-old black woman, was referred to the urology service for evaluation of a mass in the upper pole of the left kidney discovered incidently on excretory urography as part of a gynecological study for uterine myomas. Arteriography revealed the solid nature of the lesion (fig. 1). The mass appeared to be carcinoma of the kidney that did not involve any other structures. Urinalysis, complete blood count and chemistry profile were within normal limits. Metastatic studies, including a chest radiograph, liver function tests and bone scan, were completely negative. Urine cytology was negative for malignancy. Physical examination was unremarkable, without hypertension, flank mass or costovertebral angle tenderness. Left radical nephrectomy was performed through an 11th rib extraperitoneal extrapleural approach. Examination of the specimen revealed a well defined, 5 X 5 cm. mass replacing the upper pole of the kidney. There was no involvement of the collecting system, hilar region or renal vein. Lymphadenopathy was absent and there was no involvement of adjacent retroperitoneal tissue. Convalescence was uneventful and the patient was discharged from the hospital 8 days postoperatively. At 18month followup there was no evidence of recurrence.
FIG. 1. A, arteriogram indicates upper pole mass (arrows). B, nephrogram phase of arteriogram demonstrates upper pole tumor.
PATHOLOGICAL DESCRIPTION
Gross examination of the specimen revealed a 12.5 x 3.2 cm. left kidney with a large, tense, bulging mass arising from the upper pole. Sectioning of the mass demonstrated a 5.1 x 5 cm. unencapsulated well defined tumor circumscribed by renal parenchyma and capsule. The tumor arose from the upper pole and it compressed the collecting system but the epithelium of the collecting system was not involved. The cut surface of the tumor showed soft yellow-white granular tissue in small lobules. Microscopically, there were numerous punctate areas of compact spindle cells in whorls with palisades, consistent with Antoni type A tissue (fig. 2). Antoni type B tissue also was present in the same specimen. Thickened Accepted for publication May 6, 1987. * Requests for reprints: Department of Urology, St. Luke's Hospital, 114th St. and Amsterdam Ave., New York, New York 10025.
FIG. 2. Histology of tumor reveals Antoni type A tissue (arrows). Reduced from X250. 109
110
SOMERS AND ASSOCIATES
term neurilemoma as a compromise between the 2 names, since the actual embryological origin was unknown. 9 Hence, the 2 names are correctly interchangeable and both refer to the same type of tumor arising from the nerve sheath. Neurilemoma has been described in many different areas of the body, although most of the tumors have been found along peripheral nerve roots. It is found rarely in the retroperitoneum and even less rarely in the kidney. There have been only 5 cases of renal neurilemoma reported in the world literature to date, 1- 5 although neurilemoma arising from the perineum, penis, psoas muscle, stomach, cauda equina and tunica vaginalis of the testicle has been reported. 10- 15 When these tumors are found in the retroperitoneum they frequently are misdiagnosed as either sarcoma or renal cell carcinoma. They present as large masses that can displace the ureters and the kidneys. There is no accurate nonoperative method of differentiating these tumors from carcinoma. In 1965 Fein and Hamm reported a case of neurilemoma of the renal pelvis. 1 Their review of the literature included 3 other cases of neurilemoma: a tumor described by Phillips and Baumrucker originating from the renal pelvis, 2 a tumor reported by Kuzmina arising from the renal capsule,3 and a case reported by Martinot and associates apparently arising from the parenchyma but capsular origin could not definitely be excluded. 4 In 1982 Polianichko and associates described a parenchymal tumor that initially was thought to be a perirenal abscess. 5 Since 1965 there have not been any additional cases reported in the English literature. It is generally accepted by pathologists that neurilemoma tissue can be distinguished by the presence of Antoni type A and type B cells in the same specimen. 16 Type A cells are densely packed and ovoid, possess intact nucleii and show condensed peripheral chromatin. There are no myelinated nerve fibers. Verocay bodies are present and characteristic of type A cells. Antoni type B cells are separated widely, and they contain dense osmophilic granules and whorls of membranes similar to collapsed myelin sheaths. 16 They often contain cystic tissue and thickened hyalinized blood vessels. Antoni, who first described the types of tissue, believed that type B cells represented a step in nerve cell degradation. These findings were supported by the work of Sian and Ryan who found edematous transition zones along the borders between types A and B tissue, suggesting an area of cell destruction. 16 The cell of origin is still in question but most pathologists believe it is the Schwann cell. Myelinated axons are not seen in neurilemoma.
In each of the previously reported cases radical nephrectomy was performed, since the preoperative diagnosis was considered to be carcinoma and the final diagnosis was not known until after surgery. The natural history of the tumor remains unknown. The tumor usually is benign when it involves peripheral nerves, although malignant neurilemomas have been reported. 1 - 4 Radical surgery is the preferred method of treatment for renal neurilemomas. We recommend radical nephrectomy, since it is not likely that the diagnosis will be established before or during surgery. REFERENCES 1. Fein, R. L. and Hamm, F. C.: Malignant schwannoma of the renal pelvis: a review of the literature and a case report. J. Urol., 94: 356, 1965. 2. Phillips, C. A. S. and Baumrucker, G.: Neurilemmona (arising in the hilus of left kidney). J. Urol., 73: 671, 1955. 3. Kuzmina, V. E.: Neurinoma of the kidney capsule. Urologiia, 27: 52, 1962. 4. Martinot, M., Dupont, A. and Demaille, A.: Malignant schwannoma of the kidney. J. Urol. Med. Chir., 66: 748, 1960. 5. Polianichko, M. F., Zaderin, V. P. and Ratner, 0. N.: Neurilemmoma pochki. Urol. Nefrol. (Mosk), No. 5, p. 60, Sept.-Oct. 1982. 6. Miller, P. L., Tessler, A., Alexander, S. and Pinck, B. D.: Retroperitoneal neurilemmoma. Urology, 11: 619, 1978. 7. Verocay, J.: Zur Kenntnis der "Neurofibrome." Beitr. z. Path. Anat. u.z. Alig. Path., 48: 1, 1910. 8. Masson, P.: Experimental and spontaneous schwannomas. Amer. J. Path., 8: 367, 1932. 9. Stout, A. P.: The peripheral manifestations of the specific nerve sheath tumor (neurilemmoma). Amer. J. Cancer, 24: 751, 1935. 10. Carlson, H. E.: Neurilemmoma of the perineum. J. Urol., 94: 158, 1965. 11. Peral Aranda, E.: Neurinoma of the penis. Arch. Espana Urol., 10: 309, 1954. 12. Syred, D. B.: Neurilemmoma within psoas. Brit. J. Rad., 25: 108, 1952. 13. Gronbaeck, P.: Two cases of gastric neurinoma and a case of neurinoma in rectovaginal septum. Ugesk. Iaeger., 114: 246, 1952. 14. Fowler, M.: Malignant neurilemmoma arising in cauda equina. Med. J. Aust., 1: 236, 1955. 15. Cowen, R.: Tumor of the tunica vaginalis testis: case report of neurilemmoma. J. Urol., 77: 59, 1977. 16. Sian, C. J. and Ryan, S. F.: The ultrastructure of neurilemmoma with emphasis on Antoni B tissue. Hum. Path., 12: 145, 1981.