PARATESTICULAR LIPOSARCOMA: A REPORT OF TWO CASES AND REVIEW OF THE LITERATURE James F. Littles, Jr, MD, Richard C. Matter, MD, and Gill Herman, MD Ann Arbor, Michigan
Paratesticular liposarcoma is extremely rare. Only 41 cases have been reported in the literature. This article reports two cases, reviews the literature, discusses the pathology, and proposes the use of combined surgery and radiation therapy in selected cases. (J NatI Med Assoc. 1992;84:951-955.) Key words * paratesticular liposarcoma * testis * spermatic cord Liposarcomas are soft tissue sarcomas that originate from adipose tissue. They appear to produce the largest of the soft tissue tumors. In 1940, Wells reported a liposarcoma that weighed 32 kg.1 Because of the difficulty in determining the exact site of origin, paratesticular liposarcomas include all liposarcomas arising in the structures around the testis, including the lower end of the cord.2 Paratesticular liposarcoma most commonly originates in the spermatic cord. It is felt to arise from the subserous retroperitoneal fat that becomes continuous distally with the fatty tissue of the cord.3 Only two out of 39 previously reported paratesticular liposarcoma cases have not originated in the spermatic cord. The therapy of paratesticular liposarcoma is unsettled mainly because of the rarity of this disease. A review of the literature revealed that this disease is of relatively From the Department of Radiation Oncology, University of Michigan Medical School, Ann Arbor, Michigan; the Department of Radiation Oncology, William Beaumont Hospital, Royal Oak, Michigan; and the Department of Pathology, Sinai Hospital, Detroit, Michigan. Presented at the 94th Annual Convention and Scientific Assembly of the National Medical Association, July 15-20, 1989, Orlando, Florida. Requests for reprints should be addressed to Dr James F. Littles, Jr, Radiation Oncology Department, Department of Veterans Affairs Medical Ctr, 221 Fuller Rd, Ann Arbor, Ml 48105. JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 84, NO. 11
low malignancy when compared with other paratesticular malignancies.3 Therefore, orchiectomy with high ligation and excision of the spermatic cord may be adequate. Because liposarcoma appears to be among the most radiosensitive of the soft tissue sarcomas, there are some instances where postoperative radiation therapy may be advantageous.4 This article reports two cases and reviews the literature. The pathology of the disease is discussed, and a treatment protocol is proposed.
CASE REPORT Case 1 A 73-year-old male first noticed a right-sided scrotal mass in 1986. The patient described the mass as being 5 cm to 7 cm in diameter, mobile, firm, painless, and nontender. He was referred to a urologist by his family doctor. In May 1987, the scrotal mass was excised. A 4-in vertical scrotal incision was made. The right scrotal mass was easily resectable. The mass weighed approximately 900 g and appeared to arise from the subcutaneous tissues of the scrotum. A 3 X 3 cm left scrotal mass also was noted and was easily resectable. The pathology report showed the right scrotal mass to be a well-differentiated liposarcoma and the left scrotal mass to be lobulated adipose and fibrovascular tissue, without neoplasm. A metastatic workup at this time included a computed tomographic scan of the abdomen and pelvis, a chest radiograph, and bone scan. No evidence of metastatic disease was found. In August 1987, the patient was referred for a possible adjuvant course of radiation therapy to the scrotum. The patient had a significant past medical history. In October 1972, a large mass was removed from his left scrotum. The mass consisted of multilobulated portions of encapsulated fat. The combined weight of the mass was 595 g. The pathology report indicated a lipoma of 951
PARATESTICULAR LIPOSARCOMA
Study Dreyfuss5
Stout6
Herbert8 Angeli9 Dreyfuss & Goodsitt10
TABLE 1. SUMMARY OF PARATESTICULAR LIPOSARCOMAS CASE REPORTS Patient Age Duration of Treatment Year (Years) Symptoms Pathologic Diagnosis 14 years Radical orchiectomy then local 54 Lipo-osteofibrosarcoma 1940 excision plus "deep x-ray therapy" 2 months Partial excision Round cell liposarcoma 1944 62 4 years Excision 67 Myxoid liposarcoma 2 years 1945 66 Liposarcoma, spermatic cord Orchiectomy, ligation cord 1 year Liposarcoma, spermatic cord Orchiectomy, ligation radiation 71 1956 8 years 1960 80 Liposarcoma, spermatic cord Orchiectomy, ligation cord
Waller'1
Wide local excision
58 29
-
Liposarcoma, scrotum Pleomorphic liposarcoma
42 60 55
1 year 6 months
Pleomorphic liposarcoma Orchiectomy, ligation cord Lipofibromyxosarcoma Liposarcoma, spermatic cord Orchiectomy, ligation cord
43 55
5 years 3 months
hezzi17 1968 Alexander18 Garcia & Martin19 1968
50 32
4 years 5 years
Hauseld & Guira20 1968 1971 Castellano2l
52 56
6 weeks 5 years
Liposarcoma, spermatic cord Hemiscrotectomy Orchiectomy, ligation cord Liposarcoma, epididymus radiation Liposarcoma, spermatic cord Orchiectomy, ligation cord, Orchiectomy, chemotherapy, Liposarcoma radiation Liposarcoma, spermatic cord Orchiectomy, ligation cord Wide local excision plus Lipofibromyxosarcoma,
Datta et a13 Malek et a122
1971 1972
60 62
11 months 4 months
scrotum Liposarcoma, spermatic cord Fibroblastic liposarcoma
Ray et a123
D'Abrera24 Dimacopoulos25
1972 1973 1974
50 60 74
6 years 7 years 10 years
Liposarcoma Liposarcoma Liposarcoma
MacKenzie &
1974
68
2 months
Liposarcoma
Bissada et a12 Bellinger et a14
1976 1978
29 27
2 years 3 years
Johnson et a127
1978
68 60
3 months
Liposarcoma Well-differentiated liposarcoma, spermatic cord Liposarcoma, spermatic cord Myxoid liposarcoma
54
5 to 6 years Myxoid liposarcoma
65
13 years
Gowing & Morgan12
Graf13 Samellas14 Orri et a115 Lundblad et a116 Pedroni & Seg-
1962 1964 1964 1964 1965 1967 1967
3 months
radiation Orchiectomy, ligation cord Radical orchiectomy, then local excision plus 6000 rad, then local excision times 2 Right hemiscrotectomy Scrotectomy Scrotectomy with bilateral orchiectomy Orchiectomy, cord ligation
Roberts26
Senoh et a128
952
1978
77
Poorly differentiated liposarcoma Well-differentiated liposarcoma, spermatic cord
Hemiscrotectomy Radical orchiectomy, left hemiscrotectomy Radical orchiectomy Local excision, then radical orchiectomy plus hemiscrotectomy plus superficial inguinal lymphadenectomy Radical orchiectomy plus extensive removal of the lower abdominal wall fascia Local excision, then left radical orchiectomy plus 6000 rad, then right radical orchiectomy plus chemotherapy Radical orchiectomy
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 84, NO. 11
PARATESTICULAR LIPOSARCOMA
TABLE 1. SUMMARY OF PARATESTICULAR LIPOSARCOMAS CASE REPORTS (CONTINUED) Patient Age Duration of Year (Years) Symptoms Study Treatment Pathologic Diagnosis 47 1978 7 years Sogani et a129 Lipoblastic liposarcoma Orchiectomy, cord ligation 6 months 35 Liposarcoma Hemiscrotectomy 57 Reyes30 1980 Well-differentiated liposarNone-postmortem finding coma 2 weeks 67 Well-differentiated liposarRadical orchiectomy coma 65 10 years Well-differentiated liposarRadical orchiectomy coma Blitzer et a17 1981 51 10 months Myxoid liposarcoma Radical orchiectomy 71 6 months Liposarcoma Radical orchiectomy, then 4800 rad in 25 fractions for hemiscrotum and 3070 rad in 19 fractions for para-aortic and bilateral iliac nodes, followed by retroperitoneal lymph node dissection Reitan3l 1985 2 to 3 years Well-differentiated liposarLittles et al Present 73 Local excision, then radical study coma orchiectomy plus radiation therapy, 5400 rad 1 month 69 Well-differentiated liposarRadical orchiectomy coma
the left spermatic cord. In July 1981, tumors were removed from the patient's right and left scrotum. The right scrotal mass measured 8 X 5 X 3 cm. The left scrotal mass involved the left testicle intricately. The left testicle was removed along with the right and left scrotal mass. The pathology report showed a welldifferentiated liposarcoma in the right scrotal mass and a questionable sarcoma, malignant mesenchymoma in the left scrotal mass. On physical examination, the patient was a welldeveloped, well-nourished white male. General physical examination was unremarkable. There was no evidence of submandibular, supraclavicular, axillary, or inguinal lymphadenopathy. Scrotal exam showed the patient to be status postleft orchiectomy. The right testicle was normal sized and nontender. There were no masses or induration of the scrotal sac. The patient was treated with an adjuvant course of external beam radiation therapy to his scrotum. He was treated with 9 MEV electrons to a total dose of 54 gray at 1.8 gray/fraction. The patient tolerated the treatment well.
Case 2 A 54-year-old male was referred to a urologist for evaluation in February 1973. Physical examination revealed a well-developed, well-nourished male. JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 84, NO. 11
Lymph glands were without adenopathy. Examination of the scrotum revealed a left-sided fluctuant mass. The left testicle was palpable. A hard, nodular, 1½/2 to two times normal mass was noted at the upper pole of the left testicle. The right testicle was hypoplastic and tender to palpation. A chest radiograph and intravenous pyelogram failed to reveal evidence of distant metastaSiS.
On February 23, 1973, a left radical orchiectomy was performed. The pathological specimen revealed a 7.5-cm yellow lipomatous tumor in the adjacent soft tissue of the spermatic cord. A well-differentiated liposarcoma was diagnosed after histologic evaluation. The patient was disease-free 15 years later.
LITERATURE REVIEW In 1940, Dreyfuss and Lubash5 reported the first documented liposarcoma of the spermatic cord in a 54-year-old male who had a scrotal mass for 14 years prior to surgery. He was treated with a radical orchiectomy. The tumor recurred 2 months after surgery. The recurrence was treated with local excision and "deep x-ray therapy." No additional follow-up information was documented.5 Paratesticular liposarcomas usually occur in patients more than 40 years old. As in Table 1, the youngest 953
PARATESTICULAR LIPOSARCOMA
TABLE 2. TREATMENT RESULTS FOR PARATESTICULAR LIPOSARCOMA Average Follow-Up Type of Treatment No. of Patients No. of Recurrences (%) Period (Months) Radical orchiectomy 2 (11) 18 42 Wide local excision 3 (50) 6 32 4 Hemiscrotectomy 11 0 (0) Radical orchiectomy plus radiation 3 0 (0) 17 therapy 2 Scrotectomy 0 0(0) 1 Radical orchiectomy plus 0 (0) 0 chemotherapy and radiation therapy 1 Wide local excision plus radiation 0 (0) 60 therapy
reported patient was 27 years old, and the oldest was 80 years.4'5 The average age of patients is 56.9 years, and the usual presenting symptom is a painless mass in the scrotum. The duration of symptoms in the reported cases ranges from 1 month to 14 years. Liposarcoma appears to be a disease of relatively low grade.6'7 These tumors are often well-differentiated, and only 40% of the poorly differentiated liposarcomas metastasize.6 Two of the 41 (5%) reported paratesticular liposarcoma patients died of distant metastasis, and six out of 41 (15%) patients developed a local recurrence. However, it should be noted that 32 of 41 patients (78%) have been followed for less than 5 years postoperatively. Treatment of these patients has varied. Initial treatment in 18 of the 41 reported cases consisted of orchiectomy with high ligation of the spermatic cord at the inguinal canal (radical orchiectomy). Six patients were treated with wide local excision only. Four patients were treated with a hemiscrotectomy. Three patients were treated with combined radical orchiectomy and radiation therapy. As for the treatment to the remaining 10 patients, no information regarding treatment was given about six of these patients. Two patients were treated with a scrotectomy, one with an orchiectomy, chemotherapy, and radiation therapy, and one other patient was treated with wide local excision and radiation. Because of the rarity of this disease and the absence of documented long-term follow-up, it is difficult to evaluate the efficacy of the different treatments used. Table 2 compares the results of the different treatments and makes reference to the average follow-up time. The role of retroperitoneal lymph node dissection is uncertain. The procedure was only used in one of the 41 reported cases. In a review by Blitzer et a17 of all spermatic cord sarcomas, including liposarcoma, lymph node involvement occurred in a third of the patients 954
with medium- to high-grade lesions. Again, liposarcomas are usually neoplasms of low-grade malignancy.7
CONCLUSION A well-differentiated liposarcoma histologically simulates a lipoma except for a scattering of lipoblasts, irregularly shaped mesenchymal cells with hyperchromatic nuclei, and lipocytes that show a slightly greater variation in size and shape than those of normal fat.4 Not infrequently, there are areas that are virtually indistinguishable from those of a lipoma. Paratesticular liposarcomas are usually neoplasm of low-grade malignancy. Therefore, in most cases, surgery alone will be adequate. The following guidelines for treating paratesticular liposarcomas are offered: 1. Radical orchiectomy with high ligation of the spermatic cord at the inguinal ring will be adequate for uncomplicated cases of local infiltration. In cases presenting with local scrotal infiltration, a radical orchiectomy with high ligation of the spermatic cord as well as wide local scrotal excision will be required. 2. A postoperative course of external beam radiation therapy may be indicated in the following instances: local recurrent disease and histologically proven intermediate or high-grade lesions. 3. Retroperitoneal lymph node dissection is recommended in only a few cases. It may be advantageous in intermediate or high-grade lesions with evidence of lymph node involvement by computed tomographic scan or lymphangiography when the remainder of the metastatic workup is negative. 4. Patients should receive follow-up examinations every 3 months during the first year, every 6 months during the second year, and yearly thereafter. Literature Cited 1. Wells HG. Adipose tissue: a neglected subject. JAMA. 1940;1 14:2177-2187. JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 84, NO. 11
PARATESTICULAR LIPOSARCOMA
2. Bissada NK, Finkbeiner AE, Redman JF. Paratesticular sarcomas: review of management. J Urol. 1976;1 16:198-200. 3. Datta NS, Singh SM, Bapna BC. Liposarcoma of the spermatic cord: report of a case and review of the literature. J
Urol. 1971;106:888-889. 4. Bellinger MF, Gibbons MD, Koontz WW, Graff M. Paratesticular liposarcoma. J Urol. 1978;1 1:285-288. 5. Dreyfuss ML, Lybash S. Malignant mixed tumor of the spermatic cord. J Urol. 1940;44:314-324. 6. Stout AP. Liposarcoma-the malignant tumor of lipoblasts. Ann Surg. 1944;1 19:86-107. 7. Blitzer P, Dosoretz DE, Proppe KH, Shipley WU. Treatment of malignant tumors of the spermatic cord: a study of 10 cases and a review of the literature. J Urol. 1981;126:611614. 8. Herbert PA. Urological Pathology. 1st ed. Philadelphia, Pa: Lea & Febiger; 1952. 9. Angeli A. Su di un caso di liposarcoma del funiculo spermatico. Arch Ital Chir. 1956;81:427-436. 10. Dreyfuss W, Goodsitt E. Tumors of the spermatic cord. J Urol. 1960;84:658-665. 11. Waller J. Liposarcoma of the scrotum. J Urol. 1962;87:139-141. 12. Gowing NF, Morgan AD. Paratesticular tumours of connective tissue and muscle. Br J Urol. 1964;36:78-84. 13. Graf R. Malignant tumors of the spermatic cord: a brief review presentation of a lipofibromyxosarcoma of the spermatic cord. J Urol. 1965;93:74-76. 14. Samellas W. Malignant neoplasms of the spermatic cord: liposarcoma. New York J Med. 1964;64:1213-1215. 15. Orri T, Sasano N, Sato S, Ouchi K, Watanabe T. A case of liposarcoma of the spermatic cord. Jap J Canc Clin. 1965;1 1:167-169. 16. Lundblad R, Mellinger GT, Gleason DF Spermatic cord
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 84, NO. 11
malignancies. J UroL 1967;98:393-396. 17. Pedroni G, Seghezzi R. Osservazioni cliniche ed anatomicopathologiche su un raro caso di lipsarcoma primitivo dell' epidimo. Chir Ital. 1967;19:1310-1319. 18. Alexander F. Intrascrotal sarcomas. Br J Cancer. 1968; 1 2:486-497. 19. Garcia AE, Martin GG. Liposarcoma del cordon espermatico. Rev Argent Urol. 1968;37:44-48. 20. Hauseld K, Guira A. Liposarcoma of the spermatic cord. Ohio Med J 1968;64:1036-1037. 21. Castellano G. Lipofibromyxosarcoma of the scrotum: a case report and review of literature. Arch Surg. 1972;104:842844. 22. Malek RS, Utz DC, Farrow GM. Malignant tumors of the spermatic cord. Cancer 1972;29:1108-1113. 23. Ray B, Huvos AG, Whitmore WF. Unusual malignant tumors of the scrotum: review of five cases. J Urol. 1972; 1 08:760-766. 24. D'Abrera VS. A giant scrotal liposarcoma. Med J Aust. 1973; 18:854-856. 25. Dimacopoulos DG. Paratesticular liposarcoma. Br J Urol. 1974;46:347. 26. MacKenzie I, Roberts GH. Liposarcoma of the paratesticular origin: a case report. Br J Urol. 1974;46:467-470. 27. Johnson DE, Harris JD, Ayala AG. Liposarcoma of the spermatic cord. Urology. 1 978;1 1:190-192. 28. Senoh K, Osada Y, Kawachi J. Spermatic cord liposarcoma: case report. Br J UroL 1978;50:429. 29. Sogani P, Grabstald H, Whitmore WF. Spermatic cord sarcoma adults. J Urol. 1978; 120:301-305. 30. Reyes CV. Spermatic cord liposarcoma. Urology. 1980;15:416-417. 31. Reitan JB, Kaalhus 0, Brennhoud I0, et al. Prognostic factors in liposarcoma. Cancer. 1985;55:2482-2490.
955
Paratesticular liposarcoma is extremely rare. Only 41 cases have been reported in the literature. This article reports two cases, reviews the literature, discusses the pathology, and proposes the use of combined surgery and radiation therapy in selected cases. (J NatI Med Assoc. 1992;84:951-955.) Key words * paratesticular liposarcoma * testis * spermatic cord Liposarcomas are soft tissue sarcomas that originate from adipose tissue. They appear to produce the largest of the soft tissue tumors. In 1940, Wells reported a liposarcoma that weighed 32 kg.1 Because of the difficulty in determining the exact site of origin, paratesticular liposarcomas include all liposarcomas arising in the structures around the testis, including the lower end of the cord.2 Paratesticular liposarcoma most commonly originates in the spermatic cord. It is felt to arise from the subserous retroperitoneal fat that becomes continuous distally with the fatty tissue of the cord.3 Only two out of 39 previously reported paratesticular liposarcoma cases have not originated in the spermatic cord. The therapy of paratesticular liposarcoma is unsettled mainly because of the rarity of this disease. A review of the literature revealed that this disease is of relatively From the Department of Radiation Oncology, University of Michigan Medical School, Ann Arbor, Michigan; the Department of Radiation Oncology, William Beaumont Hospital, Royal Oak, Michigan; and the Department of Pathology, Sinai Hospital, Detroit, Michigan. Presented at the 94th Annual Convention and Scientific Assembly of the National Medical Association, July 15-20, 1989, Orlando, Florida. Requests for reprints should be addressed to Dr James F. Littles, Jr, Radiation Oncology Department, Department of Veterans Affairs Medical Ctr, 221 Fuller Rd, Ann Arbor, Ml 48105. JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 84, NO. 11
low malignancy when compared with other paratesticular malignancies.3 Therefore, orchiectomy with high ligation and excision of the spermatic cord may be adequate. Because liposarcoma appears to be among the most radiosensitive of the soft tissue sarcomas, there are some instances where postoperative radiation therapy may be advantageous.4 This article reports two cases and reviews the literature. The pathology of the disease is discussed, and a treatment protocol is proposed.
CASE REPORT Case 1 A 73-year-old male first noticed a right-sided scrotal mass in 1986. The patient described the mass as being 5 cm to 7 cm in diameter, mobile, firm, painless, and nontender. He was referred to a urologist by his family doctor. In May 1987, the scrotal mass was excised. A 4-in vertical scrotal incision was made. The right scrotal mass was easily resectable. The mass weighed approximately 900 g and appeared to arise from the subcutaneous tissues of the scrotum. A 3 X 3 cm left scrotal mass also was noted and was easily resectable. The pathology report showed the right scrotal mass to be a well-differentiated liposarcoma and the left scrotal mass to be lobulated adipose and fibrovascular tissue, without neoplasm. A metastatic workup at this time included a computed tomographic scan of the abdomen and pelvis, a chest radiograph, and bone scan. No evidence of metastatic disease was found. In August 1987, the patient was referred for a possible adjuvant course of radiation therapy to the scrotum. The patient had a significant past medical history. In October 1972, a large mass was removed from his left scrotum. The mass consisted of multilobulated portions of encapsulated fat. The combined weight of the mass was 595 g. The pathology report indicated a lipoma of 951
PARATESTICULAR LIPOSARCOMA
Study Dreyfuss5
Stout6
Herbert8 Angeli9 Dreyfuss & Goodsitt10
TABLE 1. SUMMARY OF PARATESTICULAR LIPOSARCOMAS CASE REPORTS Patient Age Duration of Treatment Year (Years) Symptoms Pathologic Diagnosis 14 years Radical orchiectomy then local 54 Lipo-osteofibrosarcoma 1940 excision plus "deep x-ray therapy" 2 months Partial excision Round cell liposarcoma 1944 62 4 years Excision 67 Myxoid liposarcoma 2 years 1945 66 Liposarcoma, spermatic cord Orchiectomy, ligation cord 1 year Liposarcoma, spermatic cord Orchiectomy, ligation radiation 71 1956 8 years 1960 80 Liposarcoma, spermatic cord Orchiectomy, ligation cord
Waller'1
Wide local excision
58 29
-
Liposarcoma, scrotum Pleomorphic liposarcoma
42 60 55
1 year 6 months
Pleomorphic liposarcoma Orchiectomy, ligation cord Lipofibromyxosarcoma Liposarcoma, spermatic cord Orchiectomy, ligation cord
43 55
5 years 3 months
hezzi17 1968 Alexander18 Garcia & Martin19 1968
50 32
4 years 5 years
Hauseld & Guira20 1968 1971 Castellano2l
52 56
6 weeks 5 years
Liposarcoma, spermatic cord Hemiscrotectomy Orchiectomy, ligation cord Liposarcoma, epididymus radiation Liposarcoma, spermatic cord Orchiectomy, ligation cord, Orchiectomy, chemotherapy, Liposarcoma radiation Liposarcoma, spermatic cord Orchiectomy, ligation cord Wide local excision plus Lipofibromyxosarcoma,
Datta et a13 Malek et a122
1971 1972
60 62
11 months 4 months
scrotum Liposarcoma, spermatic cord Fibroblastic liposarcoma
Ray et a123
D'Abrera24 Dimacopoulos25
1972 1973 1974
50 60 74
6 years 7 years 10 years
Liposarcoma Liposarcoma Liposarcoma
MacKenzie &
1974
68
2 months
Liposarcoma
Bissada et a12 Bellinger et a14
1976 1978
29 27
2 years 3 years
Johnson et a127
1978
68 60
3 months
Liposarcoma Well-differentiated liposarcoma, spermatic cord Liposarcoma, spermatic cord Myxoid liposarcoma
54
5 to 6 years Myxoid liposarcoma
65
13 years
Gowing & Morgan12
Graf13 Samellas14 Orri et a115 Lundblad et a116 Pedroni & Seg-
1962 1964 1964 1964 1965 1967 1967
3 months
radiation Orchiectomy, ligation cord Radical orchiectomy, then local excision plus 6000 rad, then local excision times 2 Right hemiscrotectomy Scrotectomy Scrotectomy with bilateral orchiectomy Orchiectomy, cord ligation
Roberts26
Senoh et a128
952
1978
77
Poorly differentiated liposarcoma Well-differentiated liposarcoma, spermatic cord
Hemiscrotectomy Radical orchiectomy, left hemiscrotectomy Radical orchiectomy Local excision, then radical orchiectomy plus hemiscrotectomy plus superficial inguinal lymphadenectomy Radical orchiectomy plus extensive removal of the lower abdominal wall fascia Local excision, then left radical orchiectomy plus 6000 rad, then right radical orchiectomy plus chemotherapy Radical orchiectomy
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 84, NO. 11
PARATESTICULAR LIPOSARCOMA
TABLE 1. SUMMARY OF PARATESTICULAR LIPOSARCOMAS CASE REPORTS (CONTINUED) Patient Age Duration of Year (Years) Symptoms Study Treatment Pathologic Diagnosis 47 1978 7 years Sogani et a129 Lipoblastic liposarcoma Orchiectomy, cord ligation 6 months 35 Liposarcoma Hemiscrotectomy 57 Reyes30 1980 Well-differentiated liposarNone-postmortem finding coma 2 weeks 67 Well-differentiated liposarRadical orchiectomy coma 65 10 years Well-differentiated liposarRadical orchiectomy coma Blitzer et a17 1981 51 10 months Myxoid liposarcoma Radical orchiectomy 71 6 months Liposarcoma Radical orchiectomy, then 4800 rad in 25 fractions for hemiscrotum and 3070 rad in 19 fractions for para-aortic and bilateral iliac nodes, followed by retroperitoneal lymph node dissection Reitan3l 1985 2 to 3 years Well-differentiated liposarLittles et al Present 73 Local excision, then radical study coma orchiectomy plus radiation therapy, 5400 rad 1 month 69 Well-differentiated liposarRadical orchiectomy coma
the left spermatic cord. In July 1981, tumors were removed from the patient's right and left scrotum. The right scrotal mass measured 8 X 5 X 3 cm. The left scrotal mass involved the left testicle intricately. The left testicle was removed along with the right and left scrotal mass. The pathology report showed a welldifferentiated liposarcoma in the right scrotal mass and a questionable sarcoma, malignant mesenchymoma in the left scrotal mass. On physical examination, the patient was a welldeveloped, well-nourished white male. General physical examination was unremarkable. There was no evidence of submandibular, supraclavicular, axillary, or inguinal lymphadenopathy. Scrotal exam showed the patient to be status postleft orchiectomy. The right testicle was normal sized and nontender. There were no masses or induration of the scrotal sac. The patient was treated with an adjuvant course of external beam radiation therapy to his scrotum. He was treated with 9 MEV electrons to a total dose of 54 gray at 1.8 gray/fraction. The patient tolerated the treatment well.
Case 2 A 54-year-old male was referred to a urologist for evaluation in February 1973. Physical examination revealed a well-developed, well-nourished male. JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 84, NO. 11
Lymph glands were without adenopathy. Examination of the scrotum revealed a left-sided fluctuant mass. The left testicle was palpable. A hard, nodular, 1½/2 to two times normal mass was noted at the upper pole of the left testicle. The right testicle was hypoplastic and tender to palpation. A chest radiograph and intravenous pyelogram failed to reveal evidence of distant metastaSiS.
On February 23, 1973, a left radical orchiectomy was performed. The pathological specimen revealed a 7.5-cm yellow lipomatous tumor in the adjacent soft tissue of the spermatic cord. A well-differentiated liposarcoma was diagnosed after histologic evaluation. The patient was disease-free 15 years later.
LITERATURE REVIEW In 1940, Dreyfuss and Lubash5 reported the first documented liposarcoma of the spermatic cord in a 54-year-old male who had a scrotal mass for 14 years prior to surgery. He was treated with a radical orchiectomy. The tumor recurred 2 months after surgery. The recurrence was treated with local excision and "deep x-ray therapy." No additional follow-up information was documented.5 Paratesticular liposarcomas usually occur in patients more than 40 years old. As in Table 1, the youngest 953
PARATESTICULAR LIPOSARCOMA
TABLE 2. TREATMENT RESULTS FOR PARATESTICULAR LIPOSARCOMA Average Follow-Up Type of Treatment No. of Patients No. of Recurrences (%) Period (Months) Radical orchiectomy 2 (11) 18 42 Wide local excision 3 (50) 6 32 4 Hemiscrotectomy 11 0 (0) Radical orchiectomy plus radiation 3 0 (0) 17 therapy 2 Scrotectomy 0 0(0) 1 Radical orchiectomy plus 0 (0) 0 chemotherapy and radiation therapy 1 Wide local excision plus radiation 0 (0) 60 therapy
reported patient was 27 years old, and the oldest was 80 years.4'5 The average age of patients is 56.9 years, and the usual presenting symptom is a painless mass in the scrotum. The duration of symptoms in the reported cases ranges from 1 month to 14 years. Liposarcoma appears to be a disease of relatively low grade.6'7 These tumors are often well-differentiated, and only 40% of the poorly differentiated liposarcomas metastasize.6 Two of the 41 (5%) reported paratesticular liposarcoma patients died of distant metastasis, and six out of 41 (15%) patients developed a local recurrence. However, it should be noted that 32 of 41 patients (78%) have been followed for less than 5 years postoperatively. Treatment of these patients has varied. Initial treatment in 18 of the 41 reported cases consisted of orchiectomy with high ligation of the spermatic cord at the inguinal canal (radical orchiectomy). Six patients were treated with wide local excision only. Four patients were treated with a hemiscrotectomy. Three patients were treated with combined radical orchiectomy and radiation therapy. As for the treatment to the remaining 10 patients, no information regarding treatment was given about six of these patients. Two patients were treated with a scrotectomy, one with an orchiectomy, chemotherapy, and radiation therapy, and one other patient was treated with wide local excision and radiation. Because of the rarity of this disease and the absence of documented long-term follow-up, it is difficult to evaluate the efficacy of the different treatments used. Table 2 compares the results of the different treatments and makes reference to the average follow-up time. The role of retroperitoneal lymph node dissection is uncertain. The procedure was only used in one of the 41 reported cases. In a review by Blitzer et a17 of all spermatic cord sarcomas, including liposarcoma, lymph node involvement occurred in a third of the patients 954
with medium- to high-grade lesions. Again, liposarcomas are usually neoplasms of low-grade malignancy.7
CONCLUSION A well-differentiated liposarcoma histologically simulates a lipoma except for a scattering of lipoblasts, irregularly shaped mesenchymal cells with hyperchromatic nuclei, and lipocytes that show a slightly greater variation in size and shape than those of normal fat.4 Not infrequently, there are areas that are virtually indistinguishable from those of a lipoma. Paratesticular liposarcomas are usually neoplasm of low-grade malignancy. Therefore, in most cases, surgery alone will be adequate. The following guidelines for treating paratesticular liposarcomas are offered: 1. Radical orchiectomy with high ligation of the spermatic cord at the inguinal ring will be adequate for uncomplicated cases of local infiltration. In cases presenting with local scrotal infiltration, a radical orchiectomy with high ligation of the spermatic cord as well as wide local scrotal excision will be required. 2. A postoperative course of external beam radiation therapy may be indicated in the following instances: local recurrent disease and histologically proven intermediate or high-grade lesions. 3. Retroperitoneal lymph node dissection is recommended in only a few cases. It may be advantageous in intermediate or high-grade lesions with evidence of lymph node involvement by computed tomographic scan or lymphangiography when the remainder of the metastatic workup is negative. 4. Patients should receive follow-up examinations every 3 months during the first year, every 6 months during the second year, and yearly thereafter. Literature Cited 1. Wells HG. Adipose tissue: a neglected subject. JAMA. 1940;1 14:2177-2187. JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 84, NO. 11
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2. Bissada NK, Finkbeiner AE, Redman JF. Paratesticular sarcomas: review of management. J Urol. 1976;1 16:198-200. 3. Datta NS, Singh SM, Bapna BC. Liposarcoma of the spermatic cord: report of a case and review of the literature. J
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