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Myxoid Liposarcoma of the Scrotal Wall
0022-5347/95/1536-1938$03.00/0
Vol. 153, 1938-1939, June 1995 Printed in U.S.A.
"HE JOURNAL OF UROLQGY Copyright 0 1995 by AMERICAN URO~ICAL ASSOC~ATION,h c .
MYXOID LIPOSARCOMA OF THE SCROTAL WALL JOHN J. BAUER, ISABELL A. SESTERHENN
AND
RAYMOND A. COSTABILE
From the Urology Service, Department of Surgery, Walter Reed Army Medical Center and Armed Forces Institute of Pathology, Washington, D. C.
ABSTRACT
Liposarcomas of the scrotum are rare with 5 cases reported previously. We report a case of myxoid liposarcoma of the scrotal wall, and discuss pathological classification and treatment of genitourinary liposarcomas. KEY WORDS:scrotum, liposarcoma Liposarcoma accounts for 7.5 to 25% of all soft tissue sarcomas and 1% of all cancer.l.2 Sarcoma comprises 1to 2% of all malignant genitourinary tumors.3 Liposarcoma can develop anywhere in the body where fatty tissue exists but it occurs most commonly in the popliteal fossa, gluteal area and retroperitoneum. Liposarcoma is 1.5 times more common in male than female patients.2 Paratesticular malignancies are rare, comprising mainly sarcoma originating from the spermatic cord. Undifferentiated sarcoma and embryonal rhabdomyosarcoma predominate in children while fibrosarcoma, leiomyosarcoma and malignant fibrous histiocytoma develop in adults.4 Liposarcoma is the most common soft tissue sarcoma of adulthood. Goodman et a1 reviewed 92 cases of spermatic cord liposarcoma reported up to 1990.4 Liposarcoma of the scrotum that does not originate from the spermatic cord is extremely rare with only 5 cases reported.2.5 Sporadic cases of scrotal wall liposarcoma appeared in the literature from 1930 to 1950, including 4 of spindle cell sarcoma of the scrotal wall.5 In 1991 Lissmer et a1 reported liposarcoma of the perineum and scrotum.2 We report a case of scrotal wall liposarcoma that did not involve the spermatic cord. CASE REPORT
FIG.1. Scrotal ultrasound shows large heterogeneous scrotal mass separate from testis and spermatic cord. vealed no signs of lymphadenopathy. Left hemi-scrotectomy was performed, and the remaining adipose tissue and scrotal dartos fascia were removed. Frozen section of a n area near the original inguinal incision was suspicious for spindle cell sarcoma. The patient underwent extensive excision of intrascrotal tissue, partial removal of the external oblique muscle and excision of the old incisional scar with 1 cm. margins. Permanent section showed no evidence of malignancy in any of the tissue removed. The procedure was well tolerated without complications. Followup 1 year later showed no recurrence.
A 42-year-old white man presented with a painless left scrotal mass noted 3 years previously on routine physical examination. At that time it was 1 cm. in diameter. During the next 3 years the mass remained unchanged until growth was noted 3 months before the current presentation. The patient described rapid growth to 5 cm. during the previous 4 weeks. The remainder of the medical history was negative. Physical examination revealed a 5 cm. firm, lobulated left scrotal mass. The spermatic cord, epididymis and testicle DISCUSSION were palpated as separated structures and were normal. The mass was nontender and transmitted light. No inguinal adLipomas are extremely common benign tumors, found prienopathy was found. Scrotal ultrasound showed a solid mul- marily in subcutaneous tissues. In contrast to lipomas most tilobular mass separate from a normal testicle (fig. l).Within liposarcomas develop in deep structures, such as the retrothe mass a 1cm. area of calcification was noted. peritoneum, mesentery and axilla. The gross appearance of The scrotum was explored through a n inguinal incision. liposarcoma is opaque grayish-white tissue compared to the The mass and testicle were delivered through the inguinal yellowish appearance of lipoma. Liposarcomas are poorly incision without difficulty. The mass was noted to be part of delineated nonencapsulated lesions that may grow large and the lateral dartos fascia, well delineated and encapsulated have been noted to invade surrounding structures.6 Liposarwithout local invasion. The spermatic cord, epididymis and coma is histologically subdivided into 4 types, including well testicle were separate from the mass, which did not invade differentiated, myxoid, round cell lipoblastic and pleomorthe tunica vaginalis. The mass was bivalved and benign phi^.^ Myxoid liposarcoma is the most common variant and it appearing adipose tissue comprised the bulk of the specimen. rarely metastasizes.8 One area was brownish-gray and firm. Frozen section demTreatment of soft tissue sarcoma involves surgical excionstrated lipoma. Permanent section diagnosis revealed sion, surgery and radiation therapy or surgery with radiation spindle cell sarcomatous elements. Diagnosis was myxoid therapy and chemotherapy. Before 1950 excisional biopsies liposarcoma (fig. 2). were performed with local failure rates of 60 t o 80%. ThereComputerized tomography of the abdomen and pelvis re- after, surgical resection resulted in removal of the tumor with wide margins of normal tissue. Local recurrence rates Accepted for publication December 12, 1994. decreased to 25 t,o 30%. Lymphatic spread is uncommon and 1938
MYXOID LIPOSARCOMA OF SCROTAL WALL
1939
FIG. 2. A, fat cells of varying sizes, and tumor cells with nuclear pleomo hism and signet cell mo hology, lipoblasts, typical Y-shaped capillaries and myxoid background. B, spindle cells, myxoid background anTY-shaped capillaries. H% E, reduced from x150.
presents late in the course of the disease. Therefore, lymphadenectomy is not a routine part of staging or therapy for liposarcoma.2 Sarcoma is relatively radiosensitive but radiation alone is less effective than combination therapy. Myxoid liposarcoma appears to be the most radiosensitive of all subtypes.2 Radiation is recommended as sole therapy in cases in which surgical resection is not possible secondary to invasion of major organs or blood vessels. Neoadjuvant and adjuvant radiation therapy is currently performed only in a protocol environment. Doxorubicin based chemotherapy is used for disseminated soft tissue sarcomas with complete response rates in the 10 to 15% range.s
REFERENCES
1. Lewis, D.
J., Moul, J. W., Williams, S. C., Sesterhenn, I. A and
Colon, E.: Perirenal liposarcoma containing extramedullary hematopoiesis associated with renal cell carcinoma. Urology, 43:106, 1994. 2. Lissmer, L., Kaneti, J., Klain, J., Peiser, J., Maor, E. and Cohen, Y.: Liposarcoma of the perineum and scrotum. Int. Urol. Nephrol., 24: 205, 1992. 3. Lehmann,K, Stock, U., Musy, J. P. and Schmucki, 0.: Primary paratesticular liposarcoma. Urol. Int., 61: 54, 1993. 4. Goodman, F. R., Staunton, M. D. and Rees,H. C.: Liposarcoma of the spermatic cord. J. Roy. Soc. Med., &I. 499, 1991. 5. Waller, J. I.: Liposarcoma of the scrotum. J. Urol., 81: 139,
CONCLUSIONS
1962. 6. Rosenberg, A. E.: Skeletal system and soft tumors. In: Pathologic
Liposarcoma of the scrotum is extremely rare. Treatment is complete surgical resection with wide margins. If postoperative margins are positive, repeat resection and postoperative radiation are indicated. Myxoid liposarcoma, the most common subtype, has a much better prognosis (80% 5-year survival and 50% 10-year survival) than the more aggressive round cell and pleomorphic types (20% 5-year survival and 5% 10-year survival).* If surgically unresectable, radiation is the initial mode of therapy with possible surgical excision after radiation. Widely disseminated sarcoma is treated with doxorubicin based chemotherapy with dismal results.
Basis of Disease, 5th ed. Edited by R. S. Cotran, V. Kumar, S. L. Robbins and F. J. Schoen. Philadelphia: W. B. Saunders Co., chapt. 27, pp. 1262-1263,1994, 7. Davis, K R. and Waner, M.: Soft tissue sarcomas of the head and neck. In: Textbook of Uncommon Cancer. Edited by C. J. Williams, J. G. Krikorian, M. R. Green and D. Raghaven. New York John Wiley & Sons, chapt. 53, p. 1009, 1988. 8. Yang, J, C., Glatstein, E. J., Rosenberg, S. A and Antman K H.: Sarcomas of soft tissues. In: Cancer: Principles and Practice of Oncology, 4th ed. Edited by V. T. DeVita, Jr., S. Hellman and S. A. Rosenberg. Philadelphia: J. B. Lippincott Co., vol., 2, chapt. 42, pp. 1454-1467, 1993.
Vol. 153, 1938-1939, June 1995 Printed in U.S.A.
"HE JOURNAL OF UROLQGY Copyright 0 1995 by AMERICAN URO~ICAL ASSOC~ATION,h c .
MYXOID LIPOSARCOMA OF THE SCROTAL WALL JOHN J. BAUER, ISABELL A. SESTERHENN
AND
RAYMOND A. COSTABILE
From the Urology Service, Department of Surgery, Walter Reed Army Medical Center and Armed Forces Institute of Pathology, Washington, D. C.
ABSTRACT
Liposarcomas of the scrotum are rare with 5 cases reported previously. We report a case of myxoid liposarcoma of the scrotal wall, and discuss pathological classification and treatment of genitourinary liposarcomas. KEY WORDS:scrotum, liposarcoma Liposarcoma accounts for 7.5 to 25% of all soft tissue sarcomas and 1% of all cancer.l.2 Sarcoma comprises 1to 2% of all malignant genitourinary tumors.3 Liposarcoma can develop anywhere in the body where fatty tissue exists but it occurs most commonly in the popliteal fossa, gluteal area and retroperitoneum. Liposarcoma is 1.5 times more common in male than female patients.2 Paratesticular malignancies are rare, comprising mainly sarcoma originating from the spermatic cord. Undifferentiated sarcoma and embryonal rhabdomyosarcoma predominate in children while fibrosarcoma, leiomyosarcoma and malignant fibrous histiocytoma develop in adults.4 Liposarcoma is the most common soft tissue sarcoma of adulthood. Goodman et a1 reviewed 92 cases of spermatic cord liposarcoma reported up to 1990.4 Liposarcoma of the scrotum that does not originate from the spermatic cord is extremely rare with only 5 cases reported.2.5 Sporadic cases of scrotal wall liposarcoma appeared in the literature from 1930 to 1950, including 4 of spindle cell sarcoma of the scrotal wall.5 In 1991 Lissmer et a1 reported liposarcoma of the perineum and scrotum.2 We report a case of scrotal wall liposarcoma that did not involve the spermatic cord. CASE REPORT
FIG.1. Scrotal ultrasound shows large heterogeneous scrotal mass separate from testis and spermatic cord. vealed no signs of lymphadenopathy. Left hemi-scrotectomy was performed, and the remaining adipose tissue and scrotal dartos fascia were removed. Frozen section of a n area near the original inguinal incision was suspicious for spindle cell sarcoma. The patient underwent extensive excision of intrascrotal tissue, partial removal of the external oblique muscle and excision of the old incisional scar with 1 cm. margins. Permanent section showed no evidence of malignancy in any of the tissue removed. The procedure was well tolerated without complications. Followup 1 year later showed no recurrence.
A 42-year-old white man presented with a painless left scrotal mass noted 3 years previously on routine physical examination. At that time it was 1 cm. in diameter. During the next 3 years the mass remained unchanged until growth was noted 3 months before the current presentation. The patient described rapid growth to 5 cm. during the previous 4 weeks. The remainder of the medical history was negative. Physical examination revealed a 5 cm. firm, lobulated left scrotal mass. The spermatic cord, epididymis and testicle DISCUSSION were palpated as separated structures and were normal. The mass was nontender and transmitted light. No inguinal adLipomas are extremely common benign tumors, found prienopathy was found. Scrotal ultrasound showed a solid mul- marily in subcutaneous tissues. In contrast to lipomas most tilobular mass separate from a normal testicle (fig. l).Within liposarcomas develop in deep structures, such as the retrothe mass a 1cm. area of calcification was noted. peritoneum, mesentery and axilla. The gross appearance of The scrotum was explored through a n inguinal incision. liposarcoma is opaque grayish-white tissue compared to the The mass and testicle were delivered through the inguinal yellowish appearance of lipoma. Liposarcomas are poorly incision without difficulty. The mass was noted to be part of delineated nonencapsulated lesions that may grow large and the lateral dartos fascia, well delineated and encapsulated have been noted to invade surrounding structures.6 Liposarwithout local invasion. The spermatic cord, epididymis and coma is histologically subdivided into 4 types, including well testicle were separate from the mass, which did not invade differentiated, myxoid, round cell lipoblastic and pleomorthe tunica vaginalis. The mass was bivalved and benign phi^.^ Myxoid liposarcoma is the most common variant and it appearing adipose tissue comprised the bulk of the specimen. rarely metastasizes.8 One area was brownish-gray and firm. Frozen section demTreatment of soft tissue sarcoma involves surgical excionstrated lipoma. Permanent section diagnosis revealed sion, surgery and radiation therapy or surgery with radiation spindle cell sarcomatous elements. Diagnosis was myxoid therapy and chemotherapy. Before 1950 excisional biopsies liposarcoma (fig. 2). were performed with local failure rates of 60 t o 80%. ThereComputerized tomography of the abdomen and pelvis re- after, surgical resection resulted in removal of the tumor with wide margins of normal tissue. Local recurrence rates Accepted for publication December 12, 1994. decreased to 25 t,o 30%. Lymphatic spread is uncommon and 1938
MYXOID LIPOSARCOMA OF SCROTAL WALL
1939
FIG. 2. A, fat cells of varying sizes, and tumor cells with nuclear pleomo hism and signet cell mo hology, lipoblasts, typical Y-shaped capillaries and myxoid background. B, spindle cells, myxoid background anTY-shaped capillaries. H% E, reduced from x150.
presents late in the course of the disease. Therefore, lymphadenectomy is not a routine part of staging or therapy for liposarcoma.2 Sarcoma is relatively radiosensitive but radiation alone is less effective than combination therapy. Myxoid liposarcoma appears to be the most radiosensitive of all subtypes.2 Radiation is recommended as sole therapy in cases in which surgical resection is not possible secondary to invasion of major organs or blood vessels. Neoadjuvant and adjuvant radiation therapy is currently performed only in a protocol environment. Doxorubicin based chemotherapy is used for disseminated soft tissue sarcomas with complete response rates in the 10 to 15% range.s
REFERENCES
1. Lewis, D.
J., Moul, J. W., Williams, S. C., Sesterhenn, I. A and
Colon, E.: Perirenal liposarcoma containing extramedullary hematopoiesis associated with renal cell carcinoma. Urology, 43:106, 1994. 2. Lissmer, L., Kaneti, J., Klain, J., Peiser, J., Maor, E. and Cohen, Y.: Liposarcoma of the perineum and scrotum. Int. Urol. Nephrol., 24: 205, 1992. 3. Lehmann,K, Stock, U., Musy, J. P. and Schmucki, 0.: Primary paratesticular liposarcoma. Urol. Int., 61: 54, 1993. 4. Goodman, F. R., Staunton, M. D. and Rees,H. C.: Liposarcoma of the spermatic cord. J. Roy. Soc. Med., &I. 499, 1991. 5. Waller, J. I.: Liposarcoma of the scrotum. J. Urol., 81: 139,
CONCLUSIONS
1962. 6. Rosenberg, A. E.: Skeletal system and soft tumors. In: Pathologic
Liposarcoma of the scrotum is extremely rare. Treatment is complete surgical resection with wide margins. If postoperative margins are positive, repeat resection and postoperative radiation are indicated. Myxoid liposarcoma, the most common subtype, has a much better prognosis (80% 5-year survival and 50% 10-year survival) than the more aggressive round cell and pleomorphic types (20% 5-year survival and 5% 10-year survival).* If surgically unresectable, radiation is the initial mode of therapy with possible surgical excision after radiation. Widely disseminated sarcoma is treated with doxorubicin based chemotherapy with dismal results.
Basis of Disease, 5th ed. Edited by R. S. Cotran, V. Kumar, S. L. Robbins and F. J. Schoen. Philadelphia: W. B. Saunders Co., chapt. 27, pp. 1262-1263,1994, 7. Davis, K R. and Waner, M.: Soft tissue sarcomas of the head and neck. In: Textbook of Uncommon Cancer. Edited by C. J. Williams, J. G. Krikorian, M. R. Green and D. Raghaven. New York John Wiley & Sons, chapt. 53, p. 1009, 1988. 8. Yang, J, C., Glatstein, E. J., Rosenberg, S. A and Antman K H.: Sarcomas of soft tissues. In: Cancer: Principles and Practice of Oncology, 4th ed. Edited by V. T. DeVita, Jr., S. Hellman and S. A. Rosenberg. Philadelphia: J. B. Lippincott Co., vol., 2, chapt. 42, pp. 1454-1467, 1993.