Case Report Y. S. Chen, et al Paratesticular myxoid liposarcoma: Case Report Yi-Sheng Chen1, Shang-Jen Chang2, I-Ni Chiang1, Hong-Jeng Yu1, Yeong-Shiau Pu1 1
Department of Urology, National Taiwan University Hospital, Taipei, Taiwan, R.O.C. Department of Urology, Buddhist Tzu Chi General Hospital, Taipei Branch, Taipei Taiwan, R.O.C.
2
Paratesticluar myxoid liposarcoma is rare, and it shares the similar clinical presentation with hydrocele, inguinal hernia, and testicular tumor. Via adequate management, good prognosis can be obtained. Here, we presented a 62-yearold man with recurrent paratesticular myxoid liposarcoma, and reviewed the literatures about the disease. (JTUA 20:
95-7, 2009) Key words: scrotal tumor, paratesticular, myxoid liposarcoma
INTRODUCTION Liposarcomas (LPSs) are one of the most common soft tissue sarcomas. The extremities and retroperitoneum are the most commonly affected locations accounting for about 70% of cases.1 They can be further subdivided into 4 types, including well-differentiated, myxoid, round-cell, and pleomorphic LPSs. Myxoid LPSs (MLPSs) are the most common type and account for about 50% of all LPSs. Among them, the well-differentiated LPS has an indolent course, but the round-cell and pleomorphic variants are usually aggressive and frequently metastasize. The MLPS has intermediate malignant characteristics and a tendency to recur locally. Herein, we present a case of a recurrent MLPS at an unusual site.
testis was displaced laterally. No inguinal lymphadenopathy was detected. In our hospital, the laboratory study showed elevated lactate dehydrogenase (548 U/L; normal range, xx~xx U/L). However, the beta-human chorionic gonadotropin (< 1.0 mIU/ml) and alpha-fetoprotein (2.48 ng/ml) levels were within normal limits. The abdominal and pelvic computed tomographic scan showed a 9.2-cm heterogeneous scrotal mass with a fat component in the left scrotum (Fig. 2). No evidence of visceral metastasis or lymphadenopathy was seen. The bone scan showed no evidence of bony metastasis. We made an incision over the left scrotum and left inguinal area and dissected around the tumor. Local invasion of the left crus of the penis was found. We performed a wide excision, including the overlying skin (of the left
CASE REPORT A 62-year-old male initially presented to another hospital and received local tumor resection for a painless mass lesion in the left paratesticular region, 9 months before the recurrence occurred. The pathologic diagnosis was an MLPS. Unfortunately, the left scrotal painless mass appeared again, and he also complained of rapid growth of the tumor. This time he visited our hospital. A physical examination showed a huge, movable, elastic-firm, and painless mass in the left scrotum (Fig. 1). The left testis was impalpable, and the right Address reprint requests and correspondence to: Yeong-Shiau Pu Department of Urology, National Taiwan University Hospital, No.7 Chung San South Road, Taipei, Taiwan, R.O.C. Tel: 886-2-23123456 ext. 5249 Fax: 886-2-23219145 E-mail:
[email protected]
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Fig. 1.
A huge, movable, elastic-firm, and painless mass in left scrotum. Right testis is displaced laterally.
VR
scrotal tumor
scrotum), the left testis, left epididymis, left spermatic cord, and some of the crus. Then we closed the wound by V-Y plasty. Grossly, the tumor was yellow, elastic, encapsulated, and somewhat myxoid (Fig. 3). The overlying skin, testis, epididymis, and spermatic cord were free of tumor invasion. The pathologic report was a highgrade MLPS. The postoperative course was smooth. The
Fig. 2.
Abdominal and pelvic computer tomography showed a 9.2 cm mass with fat component in left scrotum (arrow head).
wound recovered well, and no tumor recurrence was found at the postoperative 2nd week of follow-up. He will receive regular follow-up for at least 6 months.
DISCUSSION LPSs are one of the most common soft tissue sarcomas. They are malignant neoplasms of adipose tissue. They are located in the extremities and retroperitoneum in about 70% of cases. About 12% of LPSs are paratesticular.1 In addition, more than 90% of primary spermatic cord malignancies are sarcomas, with about 7% of cases being LPSs.2-4 LPSs can be further subdivided into well-differentiated, myxoid, round-cell, and pleomorphic LPSs. MLPSs are the most common type, and account for about 50% of all LPSs. They often occur in middle age, between 40 and 60 years.5 Clinically, paratesticular MLPSs present as painless scrotal or inguinal masses. They are often confused with an inguinal hernia, hydrocele, and testicular tumors. Surgeons often perform conservative management with tumor excision. However, MLPSs have a tendency to recur locally, although there is little propensity to metastasize distally. At present, there is no gold standard management for MLPSs due to their rarity, but there is a general consensus that tumors should be managed by complete resection, including a radical orchiectomy, high ligation of the spermatic cord, and a hemiscrotectomy if necessary.6 In addition, MLPSs are the most radiosensitive form of LPSs. If complete tumor excision is not feasible, adjuvant radiotherapy apparently reduces the local recurrence rate.7 If it distally metastasizes, systemic chemotherapy with a doxorubicin-based regimen is an option, and it has shown complete response rates in 10%~15% for disseminated soft tissue sarcomas.8 A lymphadenectomy is not a routine procedure since lymphatic spread is uncommon for MLPSs. 6 With adequate treatment, patients with an MLPS have a good prognosis (80% 5-year survival and 50% 10-year survival).9 According to these principles, we performed extensive surgery to completely resect the tumor. The pathologic report showed that all the surgical margins were free of malignancy. Since complete resection was obtained, radiotherapy or other adjuvant therapies were not arranged. For this patient, regular follow-up is necessary and should be adequate.
CONCLUSIONS
Fig. 3.
VS
Gross picture of the tumor: the tumor is yellow, elastic, encapsulated, and somewhat myxoid.
Paratesticular myxoid liposarcomas are an extremely rare malignancy, and are easily confused with an inguinal hernia, hydrocele, and testicular tumors. They have a
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tendency for local recurrence, but distal metastasis is not common. The current consensus of management is complete resection with a radical orchiectomy, high ligation of the spermatic cord, and a hemiscrotectomy. If a definite diagnosis is not obtained and simple tumor excision is initially performed, adjuvant wide local excision with a radical orchiectomy is suggested. If complete tumor excision is not feasible, adjuvant radiotherapy can offer better local control.
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3. Sogani PC, Grabstald H, Whitmore WF Jr. Spermatic cord sarcoma in adults. J Urol 1978;120:301-5. 4. Bellinger MF, Gibbons MD, Koontz WW Jr, Graff M. Paratesticular liposarcoma. Urology 1978;11:285-8. 5. Hamdi O, Burak O, Bulent A, Bulent O, Haydar D, Zubeyr T. Recurrent paratesticular myxoid liposarcoma in a young man. J Urol 2004;171:343. 6. Khoubehi B, Mishra V, Ali M, Motiwala H, Karim O. Adult paratesticular tumours. BJU Int 2002;90:707-15. 7. Bauer JJ, Sesterhenn IA, Costabile RA. Myxoid liposarcoma of the scrotal wall. J Urol 1995;153:1938-9. 8. Yang JC, Glatstein EJ, Rosenberg SA, Antman KH. Sarcomas of soft tissues. In: Vol. 2, Chapt. 42. Cancer: Principles and Practice of Oncology, 4th ed. DeVita VT Jr, Hellman S, Rosenberg SA, eds. Philadelphia, PA: JB Lippincott. 1993:1454-67. 9. Lissmer L, Kaneti J, Klain J, Peiser J, Maor E, Cohen Y. Liposarcoma of the perineum and scrotum. Int Urol Nephrol 1992;24:205-10.
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