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Angiomyolipoma of the Spermatic Cord: Case Report and Literature Review
0022-534 7 /89/1425-1308$02.00/0 Vol. 142, November
THE JOURNAL OF UROLOGY Copyright© 1989 by AMERICAN UROLOGICAL ASSOCIATION, INC.
Printed in U.S.A.
ANGIOMYOLIPOMA OF THE SPERMATIC CORD: CASE REPORT AND LITERATURE REVIEW THOMAS A. CASTILLENTI
AND
ANTHONY P. BERTIN
From the Department of Surgery, Division of Urology, Doctors Hospital of Stark County Inc., Massillon, Ohio
ABSTRACT
A case of an extrarenal angiomyolipoma in the spermatic cord is presented. Angiomyolipomas are uncommon tumors usually found in the kidney. The common embryological origin of kidney and cord structures offers a theoretical basis for the occurrence of such a neoplasm at this remote site. Complete wide excision is curative and recurrences are infrequent. (J. Ural., 142: 1308-1309, 1989) The kidney is the most common location for angiomyolipoma, which comprises only 0.3% of all renal neoplasms. 1 They are hamartomas of mixed mesenchymal origin and are composed of varying amounts of blood vessels, smooth muscle, adipose tissue and connective tissue stroma. They usually appear in 2 clinical settings: 1) with the tuberous sclerosis complex and 2) in middle-aged women. The tumor is benign but has characteristics of multicentricity and local extension that sometimes lead to a misdiagnosis of malignancy. Primary extrarenal angiomyolipomas are even more rare. We report a unique case in that the tumor occurred at a site anatomically remote from the kidney and in a young man without tuberous sclerosis. CASE REPORT
DISCUSSION
Extrarenal angiomyolipomas rarely have been described, with 25 cases reported to date: 13 in the liver2 • 3 and 7 in the uterus,4 while microscopic splenic involvement was noted in a patient with a left renal angiomyolipoma, 5 1 tumor was found in the retroperitoneum arising between the duodenum and pancreas,6 and 3 other cases involved the hard palate, 7 penis8 and abdominal wall. 9 The exact nature of these tumors is unclear. Since they arise simultaneously from multiple foci and primitive embryonal elements are present, they may represent incomplete mesenchymal maturation rather than a true neoplasm. 10 Therefore, the malignant nature of these tumors is controversial. If they
B. R., a 26-year-old white man, presented with left testicular pain and scrotal swelling several weeks in duration. A tender, high riding testis with an associated paratesticular mass was palpated in the left hemiscrotum and lower groin. There were no stigmata of tuberous sclerosis. Serum human chorionic gonadotropin and a-fetoprotein levels as well as computerized tomograms of the abdomen were normal. Radical orchiectomy was performed, removing an irregular neoplasm involving the distal portion of the spermatic cord to just below the internal inguinal ring. The tumor consisted of a well circumscribed mass, soft and lobulated, that measured 7 x 4 x 2 cm. and contained dense fibrous septae. The testis and epididymis were not involved (fig. 1). Microscopic sections revealed mature adipose tissue, capillary proliferation and smooth muscle cells. Characteristic spindle cell formation and large vascular channels also were noted. Occasional mitotic figures were present (fig. 2). Accepted for publication April 21, 1989.
FIG. 1. Angiomyolipoma of spermatic cord
FIG. 2. A, photomicrograph shows vascular components as well as smooth muscle and adipose tissue. Reduced from X200. B, spindle cell proliferation with adjacent endothelial lined channels. Reduced from X400. 1308
1309
A1'JGI0l\1:YOLIP0hh'.A OF SPERIVL!.!/I'IC CORD
are a developmental fault, they may not have a malignant counterpart. The nature of extension into adjacent tissue has been debated. Whether the tumor is benign but multicentric or malignant with metastasis is not clear. However, most researchers believe that this behavior represents the former type with complete local excision being the recommended treatment. 5· 11-13 No deaths of metastatic angiomyolipoma have been reported, 10 The urinary and reproductive systems are intimately associated in origin, development and certain final anatomical relationships, Both systems arise from mesoderm in the urogenital ridge of the embryo. The kidney and paratesticular cord structures are derived from mesenchymal tissue along this ridge. Existence of the most cephalad structure, the pronephros, is short and it degenerates into a vestigial remnant. The more caudally located mesonephros gives rise to the cord structures, including the epididymis, ductus deferens and seminal vesicles, The most caudad structure, the metanephros, develops into the adult kidney. 14 The common embryological origin of the kidney and cord structures offers an explanation for the development of an angiomyolipoma at an atypical location, such as the spermatic cord, CONCLUSION
An extrarenal angiomyolipoma arising in the spermatic cord in a young man without tuberous sclerosis is presented. An explanation for this development is offered based on similar embryological origin of the kidney and cord structures. Drs. J. E. Richard, Doctors Hospital, Massillon, and ff S. Levine, Cleveland Clinic Foundation, Cleveland, Ohio, and F. K Mostofi and L Sesterhenn, Armed Forces Institute of Pathology, Washington, D. C., helped to interpret the pathological findings in this case.
REFERENCES
L Hajdu, 8. I. and Foote, F. W., Jr.: Angiomyolipoma of the kidney: report of 27 cases and review of the literature. J. UroL, 102: 396, 1969. 2. Goodman, Z. D. and Ishak, K. G.: Angiomyolipomas of the liver. Amer. J. Surg. Path., 8: 745, 1984. 3. Takayasu, K., Shima, Y., Muramatsu, Y., Moriyama, N., Yamada, T., Makuuchi, M. and Hirohashi, S.: Imaging characteristics of large lipoma and angiomyolipoma of the liver: case reports. Cancer, 59: 916, 1987. 4. Demopoulos, R. I., Denarvaez, F. and Kaji, V.: Benign mixed mesodermal tumors of the uterus: a histogenetic study. Amer. J. Clin. Path., 60: 377, 1973. 5. Hulbert, J. C. and Graf, R.: Involvement of the spleen by renal angiomyolipoma: metastasis or multicentricity? J. Urol., 130: 328, 1983. 6. Friis, J. and Hjortrup, A.: Extrarenal angiomyolipoma: diagnosis and management. J. UroL, 127: 528, 1982. 7. Gutmann, J., Cifuentes, C., Vicuna, R. and Balzarini, M. A.: Intraoral angiomyolipoma. Oral Surg., 39: 945, 1975. 8. Chaitin, B. A., Goldman, R. L. and Linker, D. G.: Angiomyolipoma of penis. Urology, 23: 305, 1984. 9. Chen, KT. K. and Bauer, V.: Extrarenal angiomyolipoma. J. Surg. OncoL, 25: 89, 1984. 10. Allen, T. D. and Risk, W.: Renal angiomyolipoma. J. Urol., 94: 203, 1965. 11. Scott, M. B., Halpern, M. and Cosgrove, M. D.: Renal angiomyolipoma: two varieties. Urology, 6: 768, 1975. 12. Sant, G. R., Ucci, A. A., Jr. and Meares, E. M., Jr.: Multicentric angiomyolipoma: renal and lymph node involvement. Urology, 28: 111, 1986. 13. Busch, F. M., Bark, C. J. and Clyde, H. R.: Benign renal angicmyolipoma with regional lymph node involvement. J. Urol., 116: 715, 1976. 14. Arey, L.B.: The urinary system. In: Developmental Anatomy, 7th ed. Philadelphia: W. B. Saunders Co., chapt, XVII, p. 301, 1974.
THE JOURNAL OF UROLOGY Copyright© 1989 by AMERICAN UROLOGICAL ASSOCIATION, INC.
Printed in U.S.A.
ANGIOMYOLIPOMA OF THE SPERMATIC CORD: CASE REPORT AND LITERATURE REVIEW THOMAS A. CASTILLENTI
AND
ANTHONY P. BERTIN
From the Department of Surgery, Division of Urology, Doctors Hospital of Stark County Inc., Massillon, Ohio
ABSTRACT
A case of an extrarenal angiomyolipoma in the spermatic cord is presented. Angiomyolipomas are uncommon tumors usually found in the kidney. The common embryological origin of kidney and cord structures offers a theoretical basis for the occurrence of such a neoplasm at this remote site. Complete wide excision is curative and recurrences are infrequent. (J. Ural., 142: 1308-1309, 1989) The kidney is the most common location for angiomyolipoma, which comprises only 0.3% of all renal neoplasms. 1 They are hamartomas of mixed mesenchymal origin and are composed of varying amounts of blood vessels, smooth muscle, adipose tissue and connective tissue stroma. They usually appear in 2 clinical settings: 1) with the tuberous sclerosis complex and 2) in middle-aged women. The tumor is benign but has characteristics of multicentricity and local extension that sometimes lead to a misdiagnosis of malignancy. Primary extrarenal angiomyolipomas are even more rare. We report a unique case in that the tumor occurred at a site anatomically remote from the kidney and in a young man without tuberous sclerosis. CASE REPORT
DISCUSSION
Extrarenal angiomyolipomas rarely have been described, with 25 cases reported to date: 13 in the liver2 • 3 and 7 in the uterus,4 while microscopic splenic involvement was noted in a patient with a left renal angiomyolipoma, 5 1 tumor was found in the retroperitoneum arising between the duodenum and pancreas,6 and 3 other cases involved the hard palate, 7 penis8 and abdominal wall. 9 The exact nature of these tumors is unclear. Since they arise simultaneously from multiple foci and primitive embryonal elements are present, they may represent incomplete mesenchymal maturation rather than a true neoplasm. 10 Therefore, the malignant nature of these tumors is controversial. If they
B. R., a 26-year-old white man, presented with left testicular pain and scrotal swelling several weeks in duration. A tender, high riding testis with an associated paratesticular mass was palpated in the left hemiscrotum and lower groin. There were no stigmata of tuberous sclerosis. Serum human chorionic gonadotropin and a-fetoprotein levels as well as computerized tomograms of the abdomen were normal. Radical orchiectomy was performed, removing an irregular neoplasm involving the distal portion of the spermatic cord to just below the internal inguinal ring. The tumor consisted of a well circumscribed mass, soft and lobulated, that measured 7 x 4 x 2 cm. and contained dense fibrous septae. The testis and epididymis were not involved (fig. 1). Microscopic sections revealed mature adipose tissue, capillary proliferation and smooth muscle cells. Characteristic spindle cell formation and large vascular channels also were noted. Occasional mitotic figures were present (fig. 2). Accepted for publication April 21, 1989.
FIG. 1. Angiomyolipoma of spermatic cord
FIG. 2. A, photomicrograph shows vascular components as well as smooth muscle and adipose tissue. Reduced from X200. B, spindle cell proliferation with adjacent endothelial lined channels. Reduced from X400. 1308
1309
A1'JGI0l\1:YOLIP0hh'.A OF SPERIVL!.!/I'IC CORD
are a developmental fault, they may not have a malignant counterpart. The nature of extension into adjacent tissue has been debated. Whether the tumor is benign but multicentric or malignant with metastasis is not clear. However, most researchers believe that this behavior represents the former type with complete local excision being the recommended treatment. 5· 11-13 No deaths of metastatic angiomyolipoma have been reported, 10 The urinary and reproductive systems are intimately associated in origin, development and certain final anatomical relationships, Both systems arise from mesoderm in the urogenital ridge of the embryo. The kidney and paratesticular cord structures are derived from mesenchymal tissue along this ridge. Existence of the most cephalad structure, the pronephros, is short and it degenerates into a vestigial remnant. The more caudally located mesonephros gives rise to the cord structures, including the epididymis, ductus deferens and seminal vesicles, The most caudad structure, the metanephros, develops into the adult kidney. 14 The common embryological origin of the kidney and cord structures offers an explanation for the development of an angiomyolipoma at an atypical location, such as the spermatic cord, CONCLUSION
An extrarenal angiomyolipoma arising in the spermatic cord in a young man without tuberous sclerosis is presented. An explanation for this development is offered based on similar embryological origin of the kidney and cord structures. Drs. J. E. Richard, Doctors Hospital, Massillon, and ff S. Levine, Cleveland Clinic Foundation, Cleveland, Ohio, and F. K Mostofi and L Sesterhenn, Armed Forces Institute of Pathology, Washington, D. C., helped to interpret the pathological findings in this case.
REFERENCES
L Hajdu, 8. I. and Foote, F. W., Jr.: Angiomyolipoma of the kidney: report of 27 cases and review of the literature. J. UroL, 102: 396, 1969. 2. Goodman, Z. D. and Ishak, K. G.: Angiomyolipomas of the liver. Amer. J. Surg. Path., 8: 745, 1984. 3. Takayasu, K., Shima, Y., Muramatsu, Y., Moriyama, N., Yamada, T., Makuuchi, M. and Hirohashi, S.: Imaging characteristics of large lipoma and angiomyolipoma of the liver: case reports. Cancer, 59: 916, 1987. 4. Demopoulos, R. I., Denarvaez, F. and Kaji, V.: Benign mixed mesodermal tumors of the uterus: a histogenetic study. Amer. J. Clin. Path., 60: 377, 1973. 5. Hulbert, J. C. and Graf, R.: Involvement of the spleen by renal angiomyolipoma: metastasis or multicentricity? J. Urol., 130: 328, 1983. 6. Friis, J. and Hjortrup, A.: Extrarenal angiomyolipoma: diagnosis and management. J. UroL, 127: 528, 1982. 7. Gutmann, J., Cifuentes, C., Vicuna, R. and Balzarini, M. A.: Intraoral angiomyolipoma. Oral Surg., 39: 945, 1975. 8. Chaitin, B. A., Goldman, R. L. and Linker, D. G.: Angiomyolipoma of penis. Urology, 23: 305, 1984. 9. Chen, KT. K. and Bauer, V.: Extrarenal angiomyolipoma. J. Surg. OncoL, 25: 89, 1984. 10. Allen, T. D. and Risk, W.: Renal angiomyolipoma. J. Urol., 94: 203, 1965. 11. Scott, M. B., Halpern, M. and Cosgrove, M. D.: Renal angiomyolipoma: two varieties. Urology, 6: 768, 1975. 12. Sant, G. R., Ucci, A. A., Jr. and Meares, E. M., Jr.: Multicentric angiomyolipoma: renal and lymph node involvement. Urology, 28: 111, 1986. 13. Busch, F. M., Bark, C. J. and Clyde, H. R.: Benign renal angicmyolipoma with regional lymph node involvement. J. Urol., 116: 715, 1976. 14. Arey, L.B.: The urinary system. In: Developmental Anatomy, 7th ed. Philadelphia: W. B. Saunders Co., chapt, XVII, p. 301, 1974.