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The Management of Spermatic Cord Liposarcomas
0022-534 7/84/1311-0066$02.00 /0
THE
Vol. 131, January Printed in U.S.A.
JOURNAL OF UROLOGY
Copyright© 1984 by The Williams & Wilkins Co.
THE MANAGEMENT OF SPERMATIC CORD LIPOSARCOMAS BERT VORSTMAN,* NORMAN L. BLOCKt
AND
VICTOR A. POLITANO
From the Department of Urology, University of Miami School of Medicine, Miami, Florida
ABSTRACT
We review 3 cases of spermatic cord liposarcoma that have been followed for 12 months to 5 years after diagnosis. Inguinal orchiectomy rendered adequate treatment. Preoperative diagnosis of liposarcoma of the spermatic cord is unusual although gray scale sonography may be of some assistance. A review of the literature reveals that adjunctive medical or surgical treatment appears to be superfluous. The term liposarcoma describes a group of fatty sarcomatous tumors that may occur wherever adipose tissue normally is present. 1 The rarity of spermatic cord liposarcoma is evidenced by a review of the literature, 52 cases having been reported previously. 2-31 We review the clinical and pathological data, and the management of 3 patients with histologically proved primary liposarcoma of the spermatic cord.
peritoneal fat) or as a malignant transformation of a preexisting lipoma. 1· 15 There also has been some speculation regarding contributory factors, such as trauma, on the origin of these lesions but no definite etiologic factors have been implicated. We have added 3 cases of liposarcoma of the spermatic cord to the 52 reported previously to improve the understanding of the management. The natural history of the untreated spermatic cord liposarcoma is not known but in the case of other liposarcomas there often is a long history of a lipoma-like lesion that suddenly begins to increase in size. 1 These tumors tend to be slow growing, with clinical presentations in the literature varying from 2 weeks to 13 years. Recurrences, virtually always local, also show slow progression and may manifest several years later. The primary lesion usually is a large, firm tumor in the inguinal canal or in the scrotum adjacent to the epididymis and testis. Of the 52 tumors 31 were documented adequately as to site, with 14 in the groin and 17 adjacent to the testes. Commonly associated symptoms are a feeling of heaviness or a dragging sensation. 15 The differential diagnosis should include malignancies, such as testicular tumor, and benign lesions, such as inguinal hernia, hydrocele, epididymal cyst, epididymitis, heµiatocele and tubercular epididymo-orchitis. There are no ancillary studies that will assist in the preoperative diagnosis of spermatic cord liposarcomas. However, gray scale sonography has been shown to be of some limited use in reported studies. 26 • 27 Paraneoplastic syndromes, such as hypoglycemia, have not been recorded with spermatic cord liposarcomas as they have in other sarcomas. 34 The gross appearance of the tumor resembles lipoma, especially the lipoblastic types, but the cut surface may show foci of mucinous appearance and may feel firmer. 1· 29 Large tumors often are multilobulated and have a variegated appearance. Changes of necrosis with hemorrhage, and of myxoid and cystic degeneration are frequent with large tumors. 17 The majority of spermatic cord liposarcomas are of low grade malignancy but, as with liposarcomas in other sites, there may be some correlation between size and biologic activity. 1 Histologically, the tumor contains foci of mature adipose tissue together with areas of sarcomatous change suggested by the appearance ofpleomorphic giant cells and areas of sclerosis. Although embryonal lipoblasts are diagnostic, additional features are mucinous foci with a plexiform type of vascular pattern. 18 Well differentiated liposarcomas may be difficult to distinguish from lipomas, and they exhibit commonly a variety of microscopic features and lack of homogeneity so that the value of subclassification has been questioned.' Hajdu has classified all liposarcomas into 5 categories: 1) well differentiated, 2) myxoid, 3) lipoblastic, 4) fibroblastic and 5) pleomorphic.1 This classification also would seem applicable to spermatic cord liposarcoma. Spermatic cord liposarcomas are predominantly well differentiated and recurrences nearly al-
MATERIALS AND METHODS
The hospital records from 1972 to 1982 were reviewed and 3 patients with spermatic cord liposarcomas were found. The outcome of these patients was correlated with treatment. RESULTS
Mean patient age was 65.6 years (40, 72 and 85 years, respectively). The clinicopathological data are summarized in the table. These patients presented with a long antecedent tumor history of many months, with nonspecific local symptoms and without any metastatic disease. The lesions were painless and without an associated hydrocele, and they were opaque to transmitted light. On gross examination the tumors were fatty and multilobular, resembling lipomas. Histology in all 3 cases revealed low grade myxoliposarcoma. One patient had local recurrence after a simple excision but no recurrence was detected 5 years after a more aggressive local resection. No distant metastatic disease was recorded. DISCUSSION
Paratesticular tumors are rare and are distinguished from the larger group in which the testis is the primary lesion. About 72 per cent of the paratesticular tumors begin in the cord, with some 90 per cent of these being of mesodermal origin. 32 Of the spermatic cord tumors 20 per cent are malignant, with the majority being sarcomas. 9 Liposarcoma comprises approximately 7 per cent of previously reported primary tumors of the spermatic cord. 9· 30 Many types of spermatic cord sarcomas have been reported in the literature. 21 Malignancies of the spermatic cord are of further interest because their presentation often is confused with testicular tumors. In the review by Williams and Banerjee the 26 paratesticular tumors comprised 7 per cent of all intrascrotal tumors seen during a 57-year interval. 14 The majority of these lesions occur in men after the fourth decade of life, although in our review the ages ranged from 27 to 88 years. The neoplasms were more common on the right side (see table). This lesion is believed to arise as either a de novo cancer of the tissue of the cord (which may be an extension of retroAccepted for publication May 27, 1983. * Current address: 5559 Old Guard Cres, Campus East Virginia Beach,, Virginia 23462. ' .t R~quests for repr~n~s: Department of Urology (D-1), University of M1am1 School of Med1cme, P. 0. Box 016217, Miami, Florida 33101. 66
SPERMATIC CORD LIPOSARCOMAS Reference Herbut
W-66
Angeli Dreyfus and Goodsitt4
W-71 ?-80
Gowing and Morgan•
W-29
Samellas 6
W-42 W-55
Graf' Orii and associates•
W-65 Japanese-42
Lundblad and associates•
W-43
Alexander'°
W-50
Garcia and associates 11
Latin-32
Hausfeld and Guira 12 Castria and associates 13 Williams and Banerjee"
W-52 Latin-67
2
3
Clinical Findings
Race-Age
Rt. testicular swelling, 2 yrs. Rt. side swelling, 10 mos. Rt. inguinal swelling, 8 yrs. ?
Treatment
Histology
Results
Radical orchiectomy
Liposarcoma
Radical orchiectomy Radical orchiectomy
High grade liposarcoma Low grade liposarcoma
3-yr. followup, no metastases ?
Radical orchiectomy
Liposarcoma
1 alive and 1 had local recur-
Radical orchiectomy Radical orchiectomy
Liposarcoma Low grade !iposarcoma
Radical orchiectomy Radical orchiectomy
Lipofibromyxosarcoma Liposarcoma
Lt. hemiscrotectomy and excision of cord
Low grade liposarcoma
Radical orchiectomy
Liposarcoma
No recurrence 4 yrs. later
Rt. radical orchiectomy
High grade liposarcoma
Radical orchiectomy Rt. radical orchiectomy Local excision
Low grade !iposarcoma Liposarcoma Fibroliposarcoma
Orchiectomy Radical orchiectomy
Fibroliposarcoma Low grade liposarcoma
Died of metastatic disease 10 mos. after 6,000 rad, although no histological confirmation No recurrence at 3 yrs. Died 11 mos. later, cause unknown Recurrence despite radiotherapy but alive 5 yrs. later ? No recurrence at 5 yrs.
?
rence
W-30s
? Rt. hemiscrotal mass, 6 yrs. Lt. inguinal mass Rt. inguinal mass, initial local excision, rapid growth 2 yrs. later 300 gm. lipoma excised from groin, then It. orchiectomy 1 yr. later, recurrent mass in It. hemiscrotum 4 yrs. later Lt. groin swelling 6 wks., local excision with recurrence 4 yrs. later 13-yr. history, then sudden increase in size for 3 yrs. Lt. groin swelling, 6 wks. Swelling in old hernia scar ?
No recurrence at 1 yr. ? Local recurrence excised 12 mos. later, free of recurrence 1 yr. later Died of leukemia, no recurrence of liposarcoma
Datta and associates 15
W-23 Indian-60
? Swelling in rt. scrotum, 11
Malek and associates 16
W-62
Rt. groin mass, 4 mos.
Radical orchiectomy
Grade III fibroblastic liposarcoma
D' Abrera and BurfittWilliams 17
W-60
Enlarging It. scrotal mass, 7 yrs.
Orchiectomy
Undifferentiated liposarcoma
Mostofi and Price18
14 cases-3988 W-74
Radical orchiectomy Bilat. orchiectomy
Low grade, usually well differentiated liposarcoma Liposarcoma
W-68
Slow progressive enlargement Rt. scrotal swelling, 10 yrs. Lt. groin swelling, 1 mo.
Radical orchiectomy
Liposarcoma
?
W-29
Lt. hydrocele, 2 yrs.
Radical orchiectomy
Liposarcoma
?
B-27
Lt. scrotal mass
Radical orchiectomy
W-68
Lt. inguinal mass, 3 mos.
Local excision of mass
Low grade, well differentiated liposarcoma Liposarcoma
W-60
Rt. groin mass
Initial local excision of
Myxoid liposarcoma
mos.
Dimacopoulos
19
MacKenzie and Roberts20 Bissada and associates21 Bellinger and associates22 Johnson and associates23
mass
W-54
Lt. groin mass, 5-6 yrs.
Radical orchiectomy
Myxoid liposarcoma
W-65
Lt. groin mass, 13 yrs.
Local excision
Liposarcoma
Senoh and associates24
Japanese-77
Lt. scrotal mass, 1 mo.
Radical orchiectomy
Sogani and associates25
47
Rt. scrotal mass, 7 yrs.
Radical orchiectomy
Low grade well differentiated liposarcoma Lipoblastic liposarcoma
35
Rt. scrotal mass, 6 mos.
70 W-58
Rt. scrotal mass, 2 yrs. Rt. supratesticular mass
Radical orchiectomy, hemiscrotectomy Radical orchiectomy Radical orchiectomy
Gould and associates26 Turetz and associates 27
Excision of recurrence with radiotherapy at 10 yrs., excisions of further recurrences at 11 and 16 yrs. Hemiparesis at 5 mos. Died of multiple metastatic disease, no histological confirmation Recurrences in 4 pts. (2 pts. had multiple recurrences) ?
Hemiscrotectomy for added margin of safety After diagnosis underwent radical orchiectomy, no recurrence at 5 yrs. Initially diagnosed as neurofibroma, underwent radical orchiectomy and partial scrotectomy with excision of superficial inguinal nodes, well 8 yrs. later No recurrence 12 mos. later Recurrence at 1 yr., It. radical orchiectomy and 6,000 rad, recurrence at 4 yrs. in rt. upper scrotum treated by contralat. radical orchiectomy, pathology findings showed poorly differentiated liposarcoma, local recurrence 8 mos. later, chemotherapy with dimethyl-triazeno imidazole carboxamide, vincristine, doxorubicin and cyclophosphamide, alive with local disease in rt. groin Died 1 mo. later of heart failure No recurrence at 3½ yrs.
Liposarcoma
No recurrence at 18 mos.
Myxoliposarcoma Liposarcoma, blood vessel invasion, lymph node metastases
? Invasion and metastases, not elaborated upon
68
VORSTMAN, BLOCK AND POLITANO Clinical Findings
Reference Molina Sanchez and associates28
Latin-76
Reyes 29
W-59 W-67 W-65
Blitzer and
51
associates30
Treadwell and associates 31 Present study
Rt. scrotal swelling after excision of scrotal lipoma 13 yrs. before Rt., incidental at autopsy Rt. scrotal mass, 2 wks. Rt. scrotal swelling, 10 yrs. Lt. testicular mass, 10
Treatment Radical orchiectomy
Myxoid liposarcoma
? Radical orchiectomy
Liposarcoma Liposarcoma
Radical orchiectomy
Liposarcoma
Radical orchiectomy
Multiple local nodules, grade II myxoid liposarcoma Lobulated mass, lower cord, grade I liposarcoma
mos.
71
Rt. cord mass, 6 mos.
W-59
Lt. scrotal mass, several yrs. Excision of lipoma at time of vasectomy 2½ yrs. earlier Rt. inguinal mass believed to be recurrent inguinal hernia Rt. scrotal discomfort with slowly enlarging scrotal mass, 14 mos.
W-40 W-72
W-85
Histology
Radical orchiectomy with postop. radiotherapy and retroperitoneal lymphadenectomy Radical orchiectomy Radical orchiectomy
Results ?
? No recurrence at 5 mos., lost to followup No recurrence at 3 mos.
24-pound bilobed mass, well differentiated Jiposarcoma Low grade myxoliposarcoma
No recurrence at 3 yrs. No recurrence at 15 mos.
No recurrence 5 yrs. later
Radical orchiectomy
Low grade myxoliposarcoma of cord
No recurrence 4 yrs. later
Radical orchiectomy
Low grade myxoliposarcoma infiltrating into epididymis
No recurrence at 1 yr.
ways are local. For this reason, if the diagnosis of the cell type is certain a metastatic diagnostic study in these patients is unwarranted, especially in cases of low grade lesions. The treatment of choice is radical orchiectomy and, unlike other sarcomas of the spermatic cord, adjuvant surgical treatment is not required because of the excellent prognosis of this lesion. Retroperitoneal lymphadenectomy is not indicated, since dissemination to the retroperitoneum by this tumor has not been documented. Wide, local excision providing a good margin about the lesion is imperative but hemiscrotectomy need not be performed routinely. If a local excision has been performed previously en .bloc removal of skin and subcutaneous tissues around the wound margin and inguinal contents, and orchiectomy and/or partial scrotectomy are mandatory to prevent local recurrence. The anatomy and possible pathways of spread have been outlined previously. 30 Liposarcomas have a propensity to recur locally, and a proportion will change toward higher grades and less differentiated forms. 1 Two patients have died with a clinical impression of widespread metastatic disease. Both patients had high grade tumors but in neither was there histological confirmation of metastatic disease at autopsy. 11 • 17 Of the 16 patients in whom reported followup was ~12 months 4 had recurrences and 12 had none. Of the former 4 patients the interval to documented local recurrence varied from 1 to 16 years after excision of the initial lesion: 2 had local excision of the primary lesion, while 2 had a radical orchiectomy as the primary procedure. Of these 4 patients 2 had multiple regional recurrences over time. Only 4 cases of high grade lesions have been recorded, with 3 having recurrences and 2 patients died <1 year after unconfirmed metastatic liposarcoma. Recurrences may be treated by wide excision and, because of its relative radiosensitivity, adjunctive postoperative radiotherapy.1·30 However, in 2 cases the documented recurrences were documented after regional radiotherapy following excision of the primary lesion. Chemotherapy in the treatment of recurrent spermatic cord liposarcoma has not been documented adequately, since persistent recurrences refractory to local treatment are uncommon. Johnson and associates have induced remission in 1 patient with a poorly differentiated local recurrence with combination chemotherapy consisting of dimethyl-triazeno imidazole carboxamide, vincristine, cyclophosphamide and doxorubicin. 23 W allijn and associates have determined that the over-all mortality rate in patients with malignant paratesticular tumors is 56 per cent. 32 Since mortality from spermatic cord liposar-
coma has never been confirmed this tumor should not be included in these statistics. Because of the predilection of this lesion for local recurrence these patients must be followed periodically. REFERENCES
1. Hajdu, S. I.: Pathology of Soft Tissue Tumors. Philadelphia: Lea & Febiger, pp. 252-289, 1979. 2. Herbut, P. A.: Urological Pathology, 1st ed. Philadelphia: Lea & Febiger, pp. 1029-1030, 1952. 3. Angeli, A.: Su di un caso di lipo-sarcoma del funicolo spermatico. Arch. Ital. Chir., 81: 427, 1956. 4. Dreyfus, W. and Goodsitt, E.: Tumors of the spermatic cord. J. Ural., 84: 658, 1960. 5. Gowing, N. F. C. and Morgan, A. D.: Paratesticular tumors of connective tissue and muscle. Brit. J. Ural., suppl., 36: 78, 1964. 6. Samellas, W.: Malignant neoplasms of spermatic cord. Liposarcoma. New York J. Med., 64: 1213, 1964. 7. Graf, R. A.: Malignant tumors of the spermatic cord: a brief review and presentation of a lipofibromyxosarcoma of the spermatic cord. J. Ural., 93: 74, 1965. 8. Orii, T., Sasano, N., Sato, S., Ouchi, K. and Watanabe, T.: A case of liposarcoma of the spermatic cord. Jap. J. Cancer Clin., 11: 167, 1965. 9. Lundblad, R. R., Mellinger, G. T. and Gleason, D. F.: Spermatic cord malignancies. J. Urol., 98: 393, 1967. 10. Alexander, F.: Intrascrotal sarcomas. Brit. J. Cancer, 22: 486, 1968. 11. Garcia, A. E., Martin, G. G., Torrenti, H. and Monserrat, J. M.: Liposarcoma del cordon espermatico. Rev. Argent. Ural., 37: 44, 1968. 12. Hausfeld, K. F. and Guira, A. C.: Liposarcoma of the spermatic cord: case report. Ohio State Med. J., 64: 1036, 1968. 13. Castria, M. A., Otero, J. M., Casazza, M. and Piegari, N. S.: Liposarcoma del cordon espermatico. Rev. Argent. Ural., 38: 243, 1969. 14. Williams, G. and Banerjee R.: Paratesticular tumours. Brit. J. Ural., 41: 332, 1969. 15. Datta, N. S., Singh, S. M. and Bapna, B. C.: Liposarcoma of the spermatic cord: report of a case and review of the literature. J. Ural., 106: 888, 1971. 16. Malek, R. S., Utz, D. C. and Farrow, G. M.: Malignant tumors of the spermatic cord. Cancer, 29: 1108, 1972. 17. D'Abrera, V. S. and Burfitt-Williams, W.: A giant scrotal liposarcoma. Med. J. Aust., 2: 854, 1973. 18. Mostofi, F. K. and Price, E. B., Jr.: Tumors of the male genital system. In: Atlas of Tumor Pathology. Washington, D. C.: Armed Forces Institute of Pathology, 2nd series, fasc. 8, pp. 166-167, 1973.
69
SPERMATIC CORD LIPOSARCOMAS
19. Dimacopoulos, D. G.: Paratesticular liposarcoma. Brit. J. Urol., 46: 347, 1974. 20. MacKenzie, I. and Roberts, G. H.: Liposarcoma of paratesticular origin: a case report. Brit. J. Urol., 46: 467, 1974. 21. Bissada, N. K., Finkbeiner, A. E. and Redman, J. F.: Paratesticular sarcomas: review of management. J. Urol., 116: 198, 1976. 22. Bellinger, M. F., Gibbons, M. D., Koontz, W. W., Jr. and Graff, M.: Paratesticular liposarcoma. Urology, 11: 285, 1978. 23. Johnson, D. E., Harris, J. D. and Ayala, A. G.: Liposarcoma of spermatic cord. Urology, 11: 190, 1978. 24. Senoh, K., Osado, Y. and Kawachi, ,T.: Spermatic cord liposarcoma. Brit. J. Urol., 50: 429, 1978. 25. Sogani, P. C., Grabstald, H. and Whitmore, W. F., Jr.: Spermatic cord sarcoma in adults. J. Urol., 120: 301, 1978. 26. Gould, L., Klein, K. and Patel, N.: Ultrasound evaluation of spermatic cord myxoliposarcoma. J. Med. Soc. New Jersey, 76: 203, 1979. 27. Turetz, F., Smulewicz, J. J. and Hecht, H.: Liposarcoma of the spermatic cord: report of a case and pitfalls in sonographic differential diagnosis. Mt. Sinai J. Med., 46: 199, 1979. 28. Molina Sanchez, J., Bermal Ortiz-Repiso, J.M., Fernandez Cuervo, L. and Lopez Pardo, R.: Liposarcoma myxoide de! cordon espermatico. Acta Urol. Esp., 4: 159, 1980. 29. Reyes, C. V.: Spermatic cord liposarcoma. Urology, 15: 416, 1980.
30. Blitzer, P. H., Dosoretz, D. E., Proppe, K. H. and Shipley, W. U.: Treatment of malignant tumors of the spermatic cord: a study of 10 cases and a review of the literature. J. Urol., 126: 611, 1981. 31. Treadwell, T., Treadwell, M.A., Owen, M., McConnell, T. H. and Ashworth, C. T.: Giant liposarcoma of the spermatic cord. South. Med. J., 74: 753, 1981. 32. Wallijn, E., Popelier, G. and Renders, G.: Malignant paratesticular tumors. Discussion of the problems of dissemination and metastasis and the treatment of 2 patients. Ann. Urol., 9: 159, 1975. 33. Waldenstrom, J. G.: Paraneoplasia: Biological Signals in the Diagnosis of Cancer. New York: John Wiley & Sons, Inc., pp. 8082, 1978.
EDITORIAL COMMENT Although not specifically addressed this review suggests that the biological behavior of liposarcomas in the spermatic cord is in common with liposarcomas arising elsewhere. There is a correlation with histologic type of tumor, that is low grade, well differentiated and myxoid liposarcomas have favorable prognoses, whereas liposarcomas with multiple recurrences or metastases are likely to be of the high grade, round cell, pleomorphic or mixed variety. W. A. G.
I I I
i !
j
!
I I
11 I
THE
Vol. 131, January Printed in U.S.A.
JOURNAL OF UROLOGY
Copyright© 1984 by The Williams & Wilkins Co.
THE MANAGEMENT OF SPERMATIC CORD LIPOSARCOMAS BERT VORSTMAN,* NORMAN L. BLOCKt
AND
VICTOR A. POLITANO
From the Department of Urology, University of Miami School of Medicine, Miami, Florida
ABSTRACT
We review 3 cases of spermatic cord liposarcoma that have been followed for 12 months to 5 years after diagnosis. Inguinal orchiectomy rendered adequate treatment. Preoperative diagnosis of liposarcoma of the spermatic cord is unusual although gray scale sonography may be of some assistance. A review of the literature reveals that adjunctive medical or surgical treatment appears to be superfluous. The term liposarcoma describes a group of fatty sarcomatous tumors that may occur wherever adipose tissue normally is present. 1 The rarity of spermatic cord liposarcoma is evidenced by a review of the literature, 52 cases having been reported previously. 2-31 We review the clinical and pathological data, and the management of 3 patients with histologically proved primary liposarcoma of the spermatic cord.
peritoneal fat) or as a malignant transformation of a preexisting lipoma. 1· 15 There also has been some speculation regarding contributory factors, such as trauma, on the origin of these lesions but no definite etiologic factors have been implicated. We have added 3 cases of liposarcoma of the spermatic cord to the 52 reported previously to improve the understanding of the management. The natural history of the untreated spermatic cord liposarcoma is not known but in the case of other liposarcomas there often is a long history of a lipoma-like lesion that suddenly begins to increase in size. 1 These tumors tend to be slow growing, with clinical presentations in the literature varying from 2 weeks to 13 years. Recurrences, virtually always local, also show slow progression and may manifest several years later. The primary lesion usually is a large, firm tumor in the inguinal canal or in the scrotum adjacent to the epididymis and testis. Of the 52 tumors 31 were documented adequately as to site, with 14 in the groin and 17 adjacent to the testes. Commonly associated symptoms are a feeling of heaviness or a dragging sensation. 15 The differential diagnosis should include malignancies, such as testicular tumor, and benign lesions, such as inguinal hernia, hydrocele, epididymal cyst, epididymitis, heµiatocele and tubercular epididymo-orchitis. There are no ancillary studies that will assist in the preoperative diagnosis of spermatic cord liposarcomas. However, gray scale sonography has been shown to be of some limited use in reported studies. 26 • 27 Paraneoplastic syndromes, such as hypoglycemia, have not been recorded with spermatic cord liposarcomas as they have in other sarcomas. 34 The gross appearance of the tumor resembles lipoma, especially the lipoblastic types, but the cut surface may show foci of mucinous appearance and may feel firmer. 1· 29 Large tumors often are multilobulated and have a variegated appearance. Changes of necrosis with hemorrhage, and of myxoid and cystic degeneration are frequent with large tumors. 17 The majority of spermatic cord liposarcomas are of low grade malignancy but, as with liposarcomas in other sites, there may be some correlation between size and biologic activity. 1 Histologically, the tumor contains foci of mature adipose tissue together with areas of sarcomatous change suggested by the appearance ofpleomorphic giant cells and areas of sclerosis. Although embryonal lipoblasts are diagnostic, additional features are mucinous foci with a plexiform type of vascular pattern. 18 Well differentiated liposarcomas may be difficult to distinguish from lipomas, and they exhibit commonly a variety of microscopic features and lack of homogeneity so that the value of subclassification has been questioned.' Hajdu has classified all liposarcomas into 5 categories: 1) well differentiated, 2) myxoid, 3) lipoblastic, 4) fibroblastic and 5) pleomorphic.1 This classification also would seem applicable to spermatic cord liposarcoma. Spermatic cord liposarcomas are predominantly well differentiated and recurrences nearly al-
MATERIALS AND METHODS
The hospital records from 1972 to 1982 were reviewed and 3 patients with spermatic cord liposarcomas were found. The outcome of these patients was correlated with treatment. RESULTS
Mean patient age was 65.6 years (40, 72 and 85 years, respectively). The clinicopathological data are summarized in the table. These patients presented with a long antecedent tumor history of many months, with nonspecific local symptoms and without any metastatic disease. The lesions were painless and without an associated hydrocele, and they were opaque to transmitted light. On gross examination the tumors were fatty and multilobular, resembling lipomas. Histology in all 3 cases revealed low grade myxoliposarcoma. One patient had local recurrence after a simple excision but no recurrence was detected 5 years after a more aggressive local resection. No distant metastatic disease was recorded. DISCUSSION
Paratesticular tumors are rare and are distinguished from the larger group in which the testis is the primary lesion. About 72 per cent of the paratesticular tumors begin in the cord, with some 90 per cent of these being of mesodermal origin. 32 Of the spermatic cord tumors 20 per cent are malignant, with the majority being sarcomas. 9 Liposarcoma comprises approximately 7 per cent of previously reported primary tumors of the spermatic cord. 9· 30 Many types of spermatic cord sarcomas have been reported in the literature. 21 Malignancies of the spermatic cord are of further interest because their presentation often is confused with testicular tumors. In the review by Williams and Banerjee the 26 paratesticular tumors comprised 7 per cent of all intrascrotal tumors seen during a 57-year interval. 14 The majority of these lesions occur in men after the fourth decade of life, although in our review the ages ranged from 27 to 88 years. The neoplasms were more common on the right side (see table). This lesion is believed to arise as either a de novo cancer of the tissue of the cord (which may be an extension of retroAccepted for publication May 27, 1983. * Current address: 5559 Old Guard Cres, Campus East Virginia Beach,, Virginia 23462. ' .t R~quests for repr~n~s: Department of Urology (D-1), University of M1am1 School of Med1cme, P. 0. Box 016217, Miami, Florida 33101. 66
SPERMATIC CORD LIPOSARCOMAS Reference Herbut
W-66
Angeli Dreyfus and Goodsitt4
W-71 ?-80
Gowing and Morgan•
W-29
Samellas 6
W-42 W-55
Graf' Orii and associates•
W-65 Japanese-42
Lundblad and associates•
W-43
Alexander'°
W-50
Garcia and associates 11
Latin-32
Hausfeld and Guira 12 Castria and associates 13 Williams and Banerjee"
W-52 Latin-67
2
3
Clinical Findings
Race-Age
Rt. testicular swelling, 2 yrs. Rt. side swelling, 10 mos. Rt. inguinal swelling, 8 yrs. ?
Treatment
Histology
Results
Radical orchiectomy
Liposarcoma
Radical orchiectomy Radical orchiectomy
High grade liposarcoma Low grade liposarcoma
3-yr. followup, no metastases ?
Radical orchiectomy
Liposarcoma
1 alive and 1 had local recur-
Radical orchiectomy Radical orchiectomy
Liposarcoma Low grade !iposarcoma
Radical orchiectomy Radical orchiectomy
Lipofibromyxosarcoma Liposarcoma
Lt. hemiscrotectomy and excision of cord
Low grade liposarcoma
Radical orchiectomy
Liposarcoma
No recurrence 4 yrs. later
Rt. radical orchiectomy
High grade liposarcoma
Radical orchiectomy Rt. radical orchiectomy Local excision
Low grade !iposarcoma Liposarcoma Fibroliposarcoma
Orchiectomy Radical orchiectomy
Fibroliposarcoma Low grade liposarcoma
Died of metastatic disease 10 mos. after 6,000 rad, although no histological confirmation No recurrence at 3 yrs. Died 11 mos. later, cause unknown Recurrence despite radiotherapy but alive 5 yrs. later ? No recurrence at 5 yrs.
?
rence
W-30s
? Rt. hemiscrotal mass, 6 yrs. Lt. inguinal mass Rt. inguinal mass, initial local excision, rapid growth 2 yrs. later 300 gm. lipoma excised from groin, then It. orchiectomy 1 yr. later, recurrent mass in It. hemiscrotum 4 yrs. later Lt. groin swelling 6 wks., local excision with recurrence 4 yrs. later 13-yr. history, then sudden increase in size for 3 yrs. Lt. groin swelling, 6 wks. Swelling in old hernia scar ?
No recurrence at 1 yr. ? Local recurrence excised 12 mos. later, free of recurrence 1 yr. later Died of leukemia, no recurrence of liposarcoma
Datta and associates 15
W-23 Indian-60
? Swelling in rt. scrotum, 11
Malek and associates 16
W-62
Rt. groin mass, 4 mos.
Radical orchiectomy
Grade III fibroblastic liposarcoma
D' Abrera and BurfittWilliams 17
W-60
Enlarging It. scrotal mass, 7 yrs.
Orchiectomy
Undifferentiated liposarcoma
Mostofi and Price18
14 cases-3988 W-74
Radical orchiectomy Bilat. orchiectomy
Low grade, usually well differentiated liposarcoma Liposarcoma
W-68
Slow progressive enlargement Rt. scrotal swelling, 10 yrs. Lt. groin swelling, 1 mo.
Radical orchiectomy
Liposarcoma
?
W-29
Lt. hydrocele, 2 yrs.
Radical orchiectomy
Liposarcoma
?
B-27
Lt. scrotal mass
Radical orchiectomy
W-68
Lt. inguinal mass, 3 mos.
Local excision of mass
Low grade, well differentiated liposarcoma Liposarcoma
W-60
Rt. groin mass
Initial local excision of
Myxoid liposarcoma
mos.
Dimacopoulos
19
MacKenzie and Roberts20 Bissada and associates21 Bellinger and associates22 Johnson and associates23
mass
W-54
Lt. groin mass, 5-6 yrs.
Radical orchiectomy
Myxoid liposarcoma
W-65
Lt. groin mass, 13 yrs.
Local excision
Liposarcoma
Senoh and associates24
Japanese-77
Lt. scrotal mass, 1 mo.
Radical orchiectomy
Sogani and associates25
47
Rt. scrotal mass, 7 yrs.
Radical orchiectomy
Low grade well differentiated liposarcoma Lipoblastic liposarcoma
35
Rt. scrotal mass, 6 mos.
70 W-58
Rt. scrotal mass, 2 yrs. Rt. supratesticular mass
Radical orchiectomy, hemiscrotectomy Radical orchiectomy Radical orchiectomy
Gould and associates26 Turetz and associates 27
Excision of recurrence with radiotherapy at 10 yrs., excisions of further recurrences at 11 and 16 yrs. Hemiparesis at 5 mos. Died of multiple metastatic disease, no histological confirmation Recurrences in 4 pts. (2 pts. had multiple recurrences) ?
Hemiscrotectomy for added margin of safety After diagnosis underwent radical orchiectomy, no recurrence at 5 yrs. Initially diagnosed as neurofibroma, underwent radical orchiectomy and partial scrotectomy with excision of superficial inguinal nodes, well 8 yrs. later No recurrence 12 mos. later Recurrence at 1 yr., It. radical orchiectomy and 6,000 rad, recurrence at 4 yrs. in rt. upper scrotum treated by contralat. radical orchiectomy, pathology findings showed poorly differentiated liposarcoma, local recurrence 8 mos. later, chemotherapy with dimethyl-triazeno imidazole carboxamide, vincristine, doxorubicin and cyclophosphamide, alive with local disease in rt. groin Died 1 mo. later of heart failure No recurrence at 3½ yrs.
Liposarcoma
No recurrence at 18 mos.
Myxoliposarcoma Liposarcoma, blood vessel invasion, lymph node metastases
? Invasion and metastases, not elaborated upon
68
VORSTMAN, BLOCK AND POLITANO Clinical Findings
Reference Molina Sanchez and associates28
Latin-76
Reyes 29
W-59 W-67 W-65
Blitzer and
51
associates30
Treadwell and associates 31 Present study
Rt. scrotal swelling after excision of scrotal lipoma 13 yrs. before Rt., incidental at autopsy Rt. scrotal mass, 2 wks. Rt. scrotal swelling, 10 yrs. Lt. testicular mass, 10
Treatment Radical orchiectomy
Myxoid liposarcoma
? Radical orchiectomy
Liposarcoma Liposarcoma
Radical orchiectomy
Liposarcoma
Radical orchiectomy
Multiple local nodules, grade II myxoid liposarcoma Lobulated mass, lower cord, grade I liposarcoma
mos.
71
Rt. cord mass, 6 mos.
W-59
Lt. scrotal mass, several yrs. Excision of lipoma at time of vasectomy 2½ yrs. earlier Rt. inguinal mass believed to be recurrent inguinal hernia Rt. scrotal discomfort with slowly enlarging scrotal mass, 14 mos.
W-40 W-72
W-85
Histology
Radical orchiectomy with postop. radiotherapy and retroperitoneal lymphadenectomy Radical orchiectomy Radical orchiectomy
Results ?
? No recurrence at 5 mos., lost to followup No recurrence at 3 mos.
24-pound bilobed mass, well differentiated Jiposarcoma Low grade myxoliposarcoma
No recurrence at 3 yrs. No recurrence at 15 mos.
No recurrence 5 yrs. later
Radical orchiectomy
Low grade myxoliposarcoma of cord
No recurrence 4 yrs. later
Radical orchiectomy
Low grade myxoliposarcoma infiltrating into epididymis
No recurrence at 1 yr.
ways are local. For this reason, if the diagnosis of the cell type is certain a metastatic diagnostic study in these patients is unwarranted, especially in cases of low grade lesions. The treatment of choice is radical orchiectomy and, unlike other sarcomas of the spermatic cord, adjuvant surgical treatment is not required because of the excellent prognosis of this lesion. Retroperitoneal lymphadenectomy is not indicated, since dissemination to the retroperitoneum by this tumor has not been documented. Wide, local excision providing a good margin about the lesion is imperative but hemiscrotectomy need not be performed routinely. If a local excision has been performed previously en .bloc removal of skin and subcutaneous tissues around the wound margin and inguinal contents, and orchiectomy and/or partial scrotectomy are mandatory to prevent local recurrence. The anatomy and possible pathways of spread have been outlined previously. 30 Liposarcomas have a propensity to recur locally, and a proportion will change toward higher grades and less differentiated forms. 1 Two patients have died with a clinical impression of widespread metastatic disease. Both patients had high grade tumors but in neither was there histological confirmation of metastatic disease at autopsy. 11 • 17 Of the 16 patients in whom reported followup was ~12 months 4 had recurrences and 12 had none. Of the former 4 patients the interval to documented local recurrence varied from 1 to 16 years after excision of the initial lesion: 2 had local excision of the primary lesion, while 2 had a radical orchiectomy as the primary procedure. Of these 4 patients 2 had multiple regional recurrences over time. Only 4 cases of high grade lesions have been recorded, with 3 having recurrences and 2 patients died <1 year after unconfirmed metastatic liposarcoma. Recurrences may be treated by wide excision and, because of its relative radiosensitivity, adjunctive postoperative radiotherapy.1·30 However, in 2 cases the documented recurrences were documented after regional radiotherapy following excision of the primary lesion. Chemotherapy in the treatment of recurrent spermatic cord liposarcoma has not been documented adequately, since persistent recurrences refractory to local treatment are uncommon. Johnson and associates have induced remission in 1 patient with a poorly differentiated local recurrence with combination chemotherapy consisting of dimethyl-triazeno imidazole carboxamide, vincristine, cyclophosphamide and doxorubicin. 23 W allijn and associates have determined that the over-all mortality rate in patients with malignant paratesticular tumors is 56 per cent. 32 Since mortality from spermatic cord liposar-
coma has never been confirmed this tumor should not be included in these statistics. Because of the predilection of this lesion for local recurrence these patients must be followed periodically. REFERENCES
1. Hajdu, S. I.: Pathology of Soft Tissue Tumors. Philadelphia: Lea & Febiger, pp. 252-289, 1979. 2. Herbut, P. A.: Urological Pathology, 1st ed. Philadelphia: Lea & Febiger, pp. 1029-1030, 1952. 3. Angeli, A.: Su di un caso di lipo-sarcoma del funicolo spermatico. Arch. Ital. Chir., 81: 427, 1956. 4. Dreyfus, W. and Goodsitt, E.: Tumors of the spermatic cord. J. Ural., 84: 658, 1960. 5. Gowing, N. F. C. and Morgan, A. D.: Paratesticular tumors of connective tissue and muscle. Brit. J. Ural., suppl., 36: 78, 1964. 6. Samellas, W.: Malignant neoplasms of spermatic cord. Liposarcoma. New York J. Med., 64: 1213, 1964. 7. Graf, R. A.: Malignant tumors of the spermatic cord: a brief review and presentation of a lipofibromyxosarcoma of the spermatic cord. J. Ural., 93: 74, 1965. 8. Orii, T., Sasano, N., Sato, S., Ouchi, K. and Watanabe, T.: A case of liposarcoma of the spermatic cord. Jap. J. Cancer Clin., 11: 167, 1965. 9. Lundblad, R. R., Mellinger, G. T. and Gleason, D. F.: Spermatic cord malignancies. J. Urol., 98: 393, 1967. 10. Alexander, F.: Intrascrotal sarcomas. Brit. J. Cancer, 22: 486, 1968. 11. Garcia, A. E., Martin, G. G., Torrenti, H. and Monserrat, J. M.: Liposarcoma del cordon espermatico. Rev. Argent. Ural., 37: 44, 1968. 12. Hausfeld, K. F. and Guira, A. C.: Liposarcoma of the spermatic cord: case report. Ohio State Med. J., 64: 1036, 1968. 13. Castria, M. A., Otero, J. M., Casazza, M. and Piegari, N. S.: Liposarcoma del cordon espermatico. Rev. Argent. Ural., 38: 243, 1969. 14. Williams, G. and Banerjee R.: Paratesticular tumours. Brit. J. Ural., 41: 332, 1969. 15. Datta, N. S., Singh, S. M. and Bapna, B. C.: Liposarcoma of the spermatic cord: report of a case and review of the literature. J. Ural., 106: 888, 1971. 16. Malek, R. S., Utz, D. C. and Farrow, G. M.: Malignant tumors of the spermatic cord. Cancer, 29: 1108, 1972. 17. D'Abrera, V. S. and Burfitt-Williams, W.: A giant scrotal liposarcoma. Med. J. Aust., 2: 854, 1973. 18. Mostofi, F. K. and Price, E. B., Jr.: Tumors of the male genital system. In: Atlas of Tumor Pathology. Washington, D. C.: Armed Forces Institute of Pathology, 2nd series, fasc. 8, pp. 166-167, 1973.
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SPERMATIC CORD LIPOSARCOMAS
19. Dimacopoulos, D. G.: Paratesticular liposarcoma. Brit. J. Urol., 46: 347, 1974. 20. MacKenzie, I. and Roberts, G. H.: Liposarcoma of paratesticular origin: a case report. Brit. J. Urol., 46: 467, 1974. 21. Bissada, N. K., Finkbeiner, A. E. and Redman, J. F.: Paratesticular sarcomas: review of management. J. Urol., 116: 198, 1976. 22. Bellinger, M. F., Gibbons, M. D., Koontz, W. W., Jr. and Graff, M.: Paratesticular liposarcoma. Urology, 11: 285, 1978. 23. Johnson, D. E., Harris, J. D. and Ayala, A. G.: Liposarcoma of spermatic cord. Urology, 11: 190, 1978. 24. Senoh, K., Osado, Y. and Kawachi, ,T.: Spermatic cord liposarcoma. Brit. J. Urol., 50: 429, 1978. 25. Sogani, P. C., Grabstald, H. and Whitmore, W. F., Jr.: Spermatic cord sarcoma in adults. J. Urol., 120: 301, 1978. 26. Gould, L., Klein, K. and Patel, N.: Ultrasound evaluation of spermatic cord myxoliposarcoma. J. Med. Soc. New Jersey, 76: 203, 1979. 27. Turetz, F., Smulewicz, J. J. and Hecht, H.: Liposarcoma of the spermatic cord: report of a case and pitfalls in sonographic differential diagnosis. Mt. Sinai J. Med., 46: 199, 1979. 28. Molina Sanchez, J., Bermal Ortiz-Repiso, J.M., Fernandez Cuervo, L. and Lopez Pardo, R.: Liposarcoma myxoide de! cordon espermatico. Acta Urol. Esp., 4: 159, 1980. 29. Reyes, C. V.: Spermatic cord liposarcoma. Urology, 15: 416, 1980.
30. Blitzer, P. H., Dosoretz, D. E., Proppe, K. H. and Shipley, W. U.: Treatment of malignant tumors of the spermatic cord: a study of 10 cases and a review of the literature. J. Urol., 126: 611, 1981. 31. Treadwell, T., Treadwell, M.A., Owen, M., McConnell, T. H. and Ashworth, C. T.: Giant liposarcoma of the spermatic cord. South. Med. J., 74: 753, 1981. 32. Wallijn, E., Popelier, G. and Renders, G.: Malignant paratesticular tumors. Discussion of the problems of dissemination and metastasis and the treatment of 2 patients. Ann. Urol., 9: 159, 1975. 33. Waldenstrom, J. G.: Paraneoplasia: Biological Signals in the Diagnosis of Cancer. New York: John Wiley & Sons, Inc., pp. 8082, 1978.
EDITORIAL COMMENT Although not specifically addressed this review suggests that the biological behavior of liposarcomas in the spermatic cord is in common with liposarcomas arising elsewhere. There is a correlation with histologic type of tumor, that is low grade, well differentiated and myxoid liposarcomas have favorable prognoses, whereas liposarcomas with multiple recurrences or metastases are likely to be of the high grade, round cell, pleomorphic or mixed variety. W. A. G.
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