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Malignant fibrous histiocytoma of spermatic cord
MALIGNANT FIBROUS HISTIOCYTOMA OF SPERMATIC CORD GAETANO CIANCIO, M.D. FRANCISCO CIVANTOS, M.D. NORMAN L. BLOCK, M.D.
From the Departments of Urology and Pathology, University of Miami School of Medicine and Jackson Memorial Hospital, Miami, Florida ABSTRACT-Malignant fibrous histiocytoma occurs rarely in the genitourinary organs, and is exceptional in the spermatic cord. The prognosis of deep malignant fibrous histiocytoma not occurring in subcutaneous tissue or fascia is generally poor. We report a case, of malignant fibrous histiocytoma giant cell type, arising around the spermatic cord. The patient is alive without metastasis ten years after initial therapy. To OUTknowledge, this is the longest reported survivor of this disease. Radical inguinal orchiectomy along with wide en bloc local resection is the therapy of choice.
Malignant fibrous histiocytoma (MFH) is the most common sarcoma of the soft tissues encountered in late adult life. MFH manifests primarily in the extremities (legs 49%) arms 19 % ), or retroperitoneum or abdomen (16 % ) . l MFH occurs rarely in genitourinary organs. This tumor has been reported arising from the kidney,2-5 bladder,ev7 prostate,6.g and scrotum.‘O Weiss and Enzinger in 1978,’ and Kearney, Soule, and Ivins in 1980,” analyzed 200 and 167 cases of MFH, respectively, but did not mention specifically any case arising from the spermatic cord. Sixteen cases of MFH of the spermatic cord have been reported.12-24 MFH arising from the spermatic cord thus appears to be rare, and the relative rarity of this entity has been stressed in the 16 reported cases. Case Report A forty-year-old white man noted a lump in his left groin in September 1980. The area was slightly tender and gradually increased in size. The patient reported having had a physical examination in January 1980, with normal findings. On examination the patient had a 3 x 4 x 4 cm plum-sized firm, mobile lesion, high in the left groin. There was no palpable inguinal adenopathy.
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Results of urinalysis, complete blood count, and blood chemistry values were normal. Chest x-ray film was unremarkable. Ultrasound of the pelvis and abdomen was unremarkable for any metastatic lesions. He underwent a left radical orchiectomy and insertion of a testicular prosthesis on September 19, 1980. The mass was just inside the external inguinal ring and appeared to arise from the spermatic cord. There were no additional tumor implants along the cord at the time of surgery, and the testicle was normal. Histologic evaluation revealed a malignant fibrous histiocytoma, giant cell variant. The patient has been free of disease for ten years. He has been followed up with computerized tomography (CT) scan of chest, abdomen, and pelvis initially at three-month intervals and later, yearly. The last one was negative for evidence of disease recurrence. Pathologic findings The specimen was an oval tumor measuring 3.5 x 2.7 x 3.0 cm. The tumor weighed 48 g. The spermatic cord was densely adherent and appeared to be in close proximity to the tumor. Cut section revealed firm, grayish white tissue with focal yellowish areas; the surface was smooth.
55
FIGURE 2. Photomicrograph showing tumor nodule with numerous giant cells and stellate-like histiocytic cells and abundant vascularity (oTigina1 low-power magnification x 100).
able. The vas deferens was encased within dense fibrous tissue adjacent to tumor. Previously, this tumor had been called malignant giant cell tumor of soft parts, As reported by Enzinger and Weiss,e5 the presence of areas between nodules with classic storiformpleomorphic pattern (Fig. 2)) clearly noticeable in this patient, establishes the tumor as a variant of malignant fibrous histiocytoma. FIGURE 1. Photomicrographs: (A) showing numerous osteoclast-like giant cells typical of giant cell type of malignant fibrow histiocytoma admixed with mononuclear histiocytes and elongated fibroblast-like cells more numerous near blood vessels, and (B) atea with fibroblast-like cells arranged in classic storiform pattern. (Original high-power magnification x 400, x 400, respectively).
Microscopic sections revealed a malignant solid tumor composed of a mixture of fibroblasts, histiocytes, and numerous osteoclasttype giant cells. Dense fibrous bands with abundant blood vessels and areas with storiform-pattern separated tumor nodules where numerous giant cells were seen. The hallmark of this variety of malignant fibrous histiocytoma was the presence of numerous giant cells resembling normal osteoclasts (Fig. 1). No osteoid was present. These giant cells had voluminous eosinophilic cytoplasm with numerous small uniform nuclei. The osteoclast-like giant cells were surrounded by proliferating mononuclear histiocyte-like cells and in the periphery of the nodules by spindle-shaped fibroblast-like cells. Multiple mitotic figures were present in the tumor, many of them polypoid. The testis and adjacent cord structures were unremark-
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Comment Malignant fibrous histiocytoma also has been called fibrous xanthoma and fibroxanthosarcoma, and had often been confused with other sarcomas, such as pleomorphic rhabdomyosarcoma and liposarcoma, because of its highly variable morphologic pattern. Some spermatic cord tumors reported as fibrosarcoma show the classic histology of malignant fibrous histiocytoma.2e2* As a result, the true incidence and biologic potential of this neoplasm have not been clearly determined. The relative rarity of this entity has been stressed in the extensive review of O’Brien and Stout in 1964.2e Only 15 patients were found with MFH among 1,516 cases of fibrous tumors, including 979 cases of histiocytoma and dermatofibrosarcoma. In a later analysis by Weiss and Enzinger in 1978,’ MFH is the most common soft tissue sarcoma of late adult life. Kearney, Soule, and Ivins in 1980n found that MFH and liposarcoma were the most common soft tissue sarcomas. The aforementioned series1J1*2g do not report any case of MFH arising in the spermatic cord.
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excision in 6 cases. Of these latter 6 patients, 4 subsequently underwent radical orchiectomy after pathologic diagnosis was established. Adjunct therapies were given to 7 patients, including chemotherapy,15320 radiotherapy,e0,27 both 1e~17,23 and bilateral retroperitoneal lymphadenkctomy. 2o No additional therapies were given to 9 patients. In all 17 cases no evidence of distant metastases was found after intensive evaluation. Local radiation therapy has not been shown to be effective. Systemic chemotherapy has been effective in reducing the size of the larger and more anaplastic tumors facilitating surgical excision and also may be useful in selected patients with demonstrated locally recurrent or me&static disease.11,13,16,17,31Despite surgery, chemotherapy, and radiotherapy, tumor recurrences and metastases were observed. Metastases, when they occur are usually by hematogenous dissemination,12-15Je and regional lymphatic involvement is less common.’ Most of these tumors grow by direct extension. Banowsky and Shultz,32 suggested that retroperitoneal lymphadenectomy is justified for sarcoma of the cord in selected patients who are good surgical risk, have a histologic diagnosis of sarcoma with a known tendency for rapid, distant metastases, and without hematogenous metastases preoperatively. Weiss and Enzinger’ analyzed 290 cases of MFH and reported a 12percent rate of lymph node metastasis. Regional lymph node metastasis is not common in their cases. The incidence of lymph node infiltration in MFH of the spermatic cord has not yet been reported. In none of the 17 cases reviewed was lymph node metastasis found at the time of diagnosis. There was only 1 patient with local tumor recurrence and me&stases in 2 local lymph nodes eighteen months after previous surgery. I6 Sclama et uZ.~Oreported 2 cases of MFH of spermatic cord treated with radical inguinal orchiectomy and bilateral retroperitoneal lymphadenectomy. The lymph nodes were negative in both cases. One of their patients is free of disease after five years, while the other died six years later of retroperitoneal tumor recurrence. Bissada, Finkbeiner, and Redman reported that retroperitoneal lymphadenectomy in MFH of the spermatic cord is controversial. With this low incidence of lymph node metastases of MFH of the spermatic cord, it is difficult to justify retroperitoneal lymphadenectomy. Different variables have been used to aid in the prognosis of this disease. In superficial MFH
After the early description of MFH by O’Brien and Stout,29 Cole, Straus, and GillI reported on the first case of MFH arising in the spermatic cord. MFH of the spermatic cord usually appears in males of advanced age although it has been reported in a thirty-twoyear-old male. I9 The age range was from thirtytwo to eighty-one years (average 58 years). There is no predominance for right (8 cases) or left cord (9 cases). Patients with MFH of the spermatic cord present with a gradually enlarging mass in the inguinal region or scrotum. All reported cases have had a clinical picture of a fast-growing painless tumor, with the exception of 2 cases that presented with a painful growing mass. 22,23Clinical diagnosis of a spermatic cord tumor may be difficult, and MFH as primary paratesticular lesion is indeed uncommon. Paratesticular tumors usually manifest as firm, sometimes rubbery, non-tender irregular masses. They should be considered in any intrascrotal swelling that is thought to be discrete from the testis, regardless of the growth rate, the presence or absence of pain, and degree of transilluminability, The lesion can be confused with inguinal hernia,1s*20*24multiloculated spermatocele,20 strangulated hernia,22 or epididymo-orchitis.23 The tumor can be identified by physical examination, and accurate anatomic localization can be provided by CT or magnetic resonance imaging (MRI). Regional extension as well as nodal metastases and spread can be excluded by CT, MRI, or gallium-67 scan.3o Because of the rarity of MFH, diagnosis is established by histologic examination of the surgically resected material. Grossly, the tumor is a grayish-white, fleshy mass. Microscopically, MFH classically demonstrates a storiform pattern of spindle and round cells accompanied by pleomorphic giant cells and inflammatory cell infiltration. Most cases reported are of the storiform vaand two of these have a stoririety, 12-14~16~19*21-23 form pattern with inflammatory infiltrates consisting of polymorphonuclear and eosinophilic leukocytes. 22*23Ours is the first case report of giant cell type of malignant fibrous histiocytoma occurring in the spermatic cord. Treatment of this tumor is by no means standardized, and the unpredictable biologic behavior of this tumor makes assessment of the results of any therapy difficult. In addition, experience with these uncommon tumors is limited. Initial treatment was radical orchiectomy in 10 cases, orchiectomy in 1 case,23 and local
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of the dermal structures, depth of tumor is a prognostic variable,’ but this parameter is not useful in spermatic cord MFH. Lately, flow cytometric DNA analysis of MFH tumors has been suggested as a prognostic variable.24 Survival information indicated that the majority of deaths from this disease occurred within the first two years. Seventy-nine (40%) of 200 patients died within the first two years.’ Kearney et al.” correlated the level of tumor with survival. Patients with superficial tumors had a four-year survival of 65 percent, with deep tumor a four-year survival of 40 percent, and with retroperitoneal tumors a four-year resurvival of only 14 percent. Sclama et ~121.~~ ported that the average survival of 6 patients with MFH of the spermatic cord was twentyfive months. Superficial malignant fibrous histiocytomas of the giant cell type involving subcutis or fascia have a better prognosis with two-thirds recurring and only one-sixth metastasizing. However, of the deep tumors about half metastasize, a proportion comparable to that of ordinary forms of malignant fibrous histiocytoma. The average follow-up of the 17 cases of MFH of the spermatic cord was 3.2 years (range 0.3 to 10 years). Ten patients had a twoyear follow-up, and two-year survival was 100 percent, while 3 patients had a five-year follow-up and five-year survival was 66.3 percent (2 of 3 cases). In the present case, ten years after surgery no tumor recurrences or metastases have been observed, and the patient is periodically evaluated. In the spermatic cord, malignant fibrous histiocytoma tend to be large (mean, 7.0 cm) and moderately circumscribed. Of 7 cases with follow-up data, Epstein35 reported 4 patients alive with no evidence of disease at 1.3 to four years. This patient, therefore, has the longest survival which perhaps can be attributed to the smaller size of the tumor when detected (3.5 x 2.7 x 3.0 cm) and the radical excision. High survival rate presumably is related to early tumor detection and appropriate surgical treatment given before distant metastases occurred. Reported cases are few in number, and some follow-up is not well reported; therefore, correlation of survival rate is difficult. Local tumor recurrence rates vary from 26 to 51 percent,‘*” for malignant fibrous histiocytoma in general. The rate of local tumor recurrence of the 17 reviewed cases was 21 percent (3 of 14 cases), 3 patients were excluded due to
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lack of follow-up. To avoid local recurrence these patients should be treated with nothing less than a radical operative procedure. In conclusion, radical inguinal orchiectomy and appropriate wide local excision of the tumor arising from the spermatic cord is the treatment of choice. Further management must be individualized. Department of Urology (D-l) University of Miami PO. Box 016217 Miami, Florida 33101 (DR. CIANCIO) ACKNOWLEDGMENT. To Dr. Wade Weems and Dr. James Katsikas for referring this patient to us, and the Department of Pathology, Broward General Medical Center, for supplying the original pathology material to us. References 1. Weiss SW, and Enzinger FM: Malignant fibrous histiocytoma, Cancer 41: 2250 (1978). 2. Klugo RC, Farah RN, and Cerny JC: Renal malignant histiocytoma, J Urol 112: 727 (1974). 3; Raghavaiah NV, Mayer RE.Hagitt R, and Soloway M: Malignant fibrous histiccvtoma of the kidnev. 1 Uroll23: 951 (1989). -4. Striven RR, Thrasher TV, Smith DC, and Stewart SC: Primary renal malignant fibrous histiocytoma: a case report and literature review, J Urol 131: 948 (1984). 5. Kollias G, and Giannopoulus T: Primary malignant fibrous histiocytoma of the kidney: report of a case, J Urol 138: 499 (1987). 6. Henriksen OB, Mogensen P, and Engelholm AJ: Inflammatory fibrous histiocytoma of the urinary bladder, Ada Path01 Microbiol Immunol Stand, sect. A 99: 333 (1982). 7. Goodman AJ, and Greaney MG: Malignant fibrous histiocytoma of the bladder, Br J Uro157: 196 (1985). 8. Chin W, Fay R, and Ortega P: Malignant fibrous histiocytoma of prostate, Urology 27: 363 (1986). 9. Bain GO, et al: Malignant fibrous histiocytoma of prostate gland, Urology 26: 89 (1985). 10. Watanabe K. et al: Malienant fibrous histiocvtoma of the scrotal wall: a case’report, J Ur& 146: 151 (1988). ’ 11. Kearney MM, Soule EH, and Ivins JC: Malignant fibrous histiocytoma: a retrospective study of 167 cases, Cancer 45: 167 (1989). 12. Cole AT, Straus FH, and Gill WB: Malignant fibrous histiocytoma: an unusual inguinal tumor, J Urol 107: 1995 (1972). 13. Farah RN, and Bohne AW: Malignant fibrous histiocytoma of spermatic cord, Urology 3: 782 (1974). 14. Dias R, Fernandes M, and Gaetz HP: Malignant fibrous histiocytoma of spermatic cord, Urology 12: 365 (1978). 15. Sogani PC, Grab&d H, and Whitmore WF Jr: Spermatic cord sarcoma in adults, J Urol 120: 301 (1978).’ 16. Williamson JC, Johnson JD, Lamm DL, and Tio F: Malignant fibrous histiocytoma of the spermatic cord, J Urol 123: 785 (1979). 17. Adolphs HD, Helpap B, and Koischwitz D: Retroperitoneal and inguinal manifestation of malignant histiocytoma, Urology 29: 639 (1982). 18. Wheatley KK, and Rauschkolb E: Malignant fibrous histiocytoma of the spermatic cord, J Comput Assist Toruogr 6: 1035 (1982). 19. Smailowitz Z, et al: Malignant fibrous histiocytoma of the spermatic cord, J Urol 130: 150 (1983). 20. Sclama AO, Berger BW, Cherry JM, and Young JD Jr:
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28. Ashby BS, and MacGillivray JB: Paratesticular lipoma, Br J Surg 53: 828 (1966). 29. O’Brien JE, and Stout AP: Malignant fibrous xanthomas, Cancer 17: 1445 (1964). 30. Zazzaro PF, Bosworth JE, Schneider V, and Zelenak JJ: Gallium scanning in malignant fibrous histiocytoma, AJR 135: 775 (1980). 31. Malek RS, Utz DC, and Farrow GM: Malignant tumors of the spermatic cord, Cancer 29: 1198 (1972). 32. Banowsky LH, and Schultz GN: Sarcoma of the spermatic cord and tunics: review of the literature, case report and discussion of the role of retroperitoneal lymph node dissection, J Urol 103: 628 (1970). 33. Bissada NK, Finkbeiner AE, and Redman JF: Paratesticular sarcomas: review of management, J Urol 116: 198 (1976). 34. Radio SJ, Wooldridge TN, and Linder J: Flow cytometric DNA analysis of malignant fibrous histiocytoma and related fibro-histiocytic tumors, Human Path01 19: 74 (1988). 35. Epstein JI: Diseases of the spermatic cord and paratesticular tissue, in Murphy WM (Ed): Urological Pathology, Philadelphia, W.B. Saunders, chap 4, 1989, p 236.
Malignant fibrous histiocytoma of the spermatic cord: the role of retroperitoneal lymphadenectomy in management, J Urol 130: 577 (1983). 21. Botma JP De Kock MLS, and Laubscher WML: Malignant fibrous histiocytoma of the spermatic cord, S Afr Med J 71: 326 (1987). 22. Nistal M, Regadera J, Jareno E, and Paniagua R: Inflammatory malignant fibrous histiocytoma of the spermatic cord, Urol Int 43: 188 (1988). 23. Aigaba F, Trias I, and Castro C: Inflammatory malignant fibrous histiocytoma of the spermatic cord with eosinophilia, Histiopathology 14: 319 (1989). 24. De Bruin MJFM, Pelger RCM, Meijer WS, and Giard RWM: Malignant fibrous histiocytoma of the spermatic cord, J Urol 142: 131 (1989). 25. Enzinger FM, and Weiss SW: Soft Tissue ‘lbmors, St. Louis, C.V. Mosby Co., ed 2, chap 11, 1988, p 291. 26. Sherwin B, and Bergman H: Primary malignant neoplasms of the spermatic cord and epididymis, J Urol 67: 208 (1952). 27. Powell HDW: Fibrosarcoma of the spermatic cord, Br J Urol 28: 194 (1956).
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From the Departments of Urology and Pathology, University of Miami School of Medicine and Jackson Memorial Hospital, Miami, Florida ABSTRACT-Malignant fibrous histiocytoma occurs rarely in the genitourinary organs, and is exceptional in the spermatic cord. The prognosis of deep malignant fibrous histiocytoma not occurring in subcutaneous tissue or fascia is generally poor. We report a case, of malignant fibrous histiocytoma giant cell type, arising around the spermatic cord. The patient is alive without metastasis ten years after initial therapy. To OUTknowledge, this is the longest reported survivor of this disease. Radical inguinal orchiectomy along with wide en bloc local resection is the therapy of choice.
Malignant fibrous histiocytoma (MFH) is the most common sarcoma of the soft tissues encountered in late adult life. MFH manifests primarily in the extremities (legs 49%) arms 19 % ), or retroperitoneum or abdomen (16 % ) . l MFH occurs rarely in genitourinary organs. This tumor has been reported arising from the kidney,2-5 bladder,ev7 prostate,6.g and scrotum.‘O Weiss and Enzinger in 1978,’ and Kearney, Soule, and Ivins in 1980,” analyzed 200 and 167 cases of MFH, respectively, but did not mention specifically any case arising from the spermatic cord. Sixteen cases of MFH of the spermatic cord have been reported.12-24 MFH arising from the spermatic cord thus appears to be rare, and the relative rarity of this entity has been stressed in the 16 reported cases. Case Report A forty-year-old white man noted a lump in his left groin in September 1980. The area was slightly tender and gradually increased in size. The patient reported having had a physical examination in January 1980, with normal findings. On examination the patient had a 3 x 4 x 4 cm plum-sized firm, mobile lesion, high in the left groin. There was no palpable inguinal adenopathy.
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Results of urinalysis, complete blood count, and blood chemistry values were normal. Chest x-ray film was unremarkable. Ultrasound of the pelvis and abdomen was unremarkable for any metastatic lesions. He underwent a left radical orchiectomy and insertion of a testicular prosthesis on September 19, 1980. The mass was just inside the external inguinal ring and appeared to arise from the spermatic cord. There were no additional tumor implants along the cord at the time of surgery, and the testicle was normal. Histologic evaluation revealed a malignant fibrous histiocytoma, giant cell variant. The patient has been free of disease for ten years. He has been followed up with computerized tomography (CT) scan of chest, abdomen, and pelvis initially at three-month intervals and later, yearly. The last one was negative for evidence of disease recurrence. Pathologic findings The specimen was an oval tumor measuring 3.5 x 2.7 x 3.0 cm. The tumor weighed 48 g. The spermatic cord was densely adherent and appeared to be in close proximity to the tumor. Cut section revealed firm, grayish white tissue with focal yellowish areas; the surface was smooth.
55
FIGURE 2. Photomicrograph showing tumor nodule with numerous giant cells and stellate-like histiocytic cells and abundant vascularity (oTigina1 low-power magnification x 100).
able. The vas deferens was encased within dense fibrous tissue adjacent to tumor. Previously, this tumor had been called malignant giant cell tumor of soft parts, As reported by Enzinger and Weiss,e5 the presence of areas between nodules with classic storiformpleomorphic pattern (Fig. 2)) clearly noticeable in this patient, establishes the tumor as a variant of malignant fibrous histiocytoma. FIGURE 1. Photomicrographs: (A) showing numerous osteoclast-like giant cells typical of giant cell type of malignant fibrow histiocytoma admixed with mononuclear histiocytes and elongated fibroblast-like cells more numerous near blood vessels, and (B) atea with fibroblast-like cells arranged in classic storiform pattern. (Original high-power magnification x 400, x 400, respectively).
Microscopic sections revealed a malignant solid tumor composed of a mixture of fibroblasts, histiocytes, and numerous osteoclasttype giant cells. Dense fibrous bands with abundant blood vessels and areas with storiform-pattern separated tumor nodules where numerous giant cells were seen. The hallmark of this variety of malignant fibrous histiocytoma was the presence of numerous giant cells resembling normal osteoclasts (Fig. 1). No osteoid was present. These giant cells had voluminous eosinophilic cytoplasm with numerous small uniform nuclei. The osteoclast-like giant cells were surrounded by proliferating mononuclear histiocyte-like cells and in the periphery of the nodules by spindle-shaped fibroblast-like cells. Multiple mitotic figures were present in the tumor, many of them polypoid. The testis and adjacent cord structures were unremark-
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Comment Malignant fibrous histiocytoma also has been called fibrous xanthoma and fibroxanthosarcoma, and had often been confused with other sarcomas, such as pleomorphic rhabdomyosarcoma and liposarcoma, because of its highly variable morphologic pattern. Some spermatic cord tumors reported as fibrosarcoma show the classic histology of malignant fibrous histiocytoma.2e2* As a result, the true incidence and biologic potential of this neoplasm have not been clearly determined. The relative rarity of this entity has been stressed in the extensive review of O’Brien and Stout in 1964.2e Only 15 patients were found with MFH among 1,516 cases of fibrous tumors, including 979 cases of histiocytoma and dermatofibrosarcoma. In a later analysis by Weiss and Enzinger in 1978,’ MFH is the most common soft tissue sarcoma of late adult life. Kearney, Soule, and Ivins in 1980n found that MFH and liposarcoma were the most common soft tissue sarcomas. The aforementioned series1J1*2g do not report any case of MFH arising in the spermatic cord.
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excision in 6 cases. Of these latter 6 patients, 4 subsequently underwent radical orchiectomy after pathologic diagnosis was established. Adjunct therapies were given to 7 patients, including chemotherapy,15320 radiotherapy,e0,27 both 1e~17,23 and bilateral retroperitoneal lymphadenkctomy. 2o No additional therapies were given to 9 patients. In all 17 cases no evidence of distant metastases was found after intensive evaluation. Local radiation therapy has not been shown to be effective. Systemic chemotherapy has been effective in reducing the size of the larger and more anaplastic tumors facilitating surgical excision and also may be useful in selected patients with demonstrated locally recurrent or me&static disease.11,13,16,17,31Despite surgery, chemotherapy, and radiotherapy, tumor recurrences and metastases were observed. Metastases, when they occur are usually by hematogenous dissemination,12-15Je and regional lymphatic involvement is less common.’ Most of these tumors grow by direct extension. Banowsky and Shultz,32 suggested that retroperitoneal lymphadenectomy is justified for sarcoma of the cord in selected patients who are good surgical risk, have a histologic diagnosis of sarcoma with a known tendency for rapid, distant metastases, and without hematogenous metastases preoperatively. Weiss and Enzinger’ analyzed 290 cases of MFH and reported a 12percent rate of lymph node metastasis. Regional lymph node metastasis is not common in their cases. The incidence of lymph node infiltration in MFH of the spermatic cord has not yet been reported. In none of the 17 cases reviewed was lymph node metastasis found at the time of diagnosis. There was only 1 patient with local tumor recurrence and me&stases in 2 local lymph nodes eighteen months after previous surgery. I6 Sclama et uZ.~Oreported 2 cases of MFH of spermatic cord treated with radical inguinal orchiectomy and bilateral retroperitoneal lymphadenectomy. The lymph nodes were negative in both cases. One of their patients is free of disease after five years, while the other died six years later of retroperitoneal tumor recurrence. Bissada, Finkbeiner, and Redman reported that retroperitoneal lymphadenectomy in MFH of the spermatic cord is controversial. With this low incidence of lymph node metastases of MFH of the spermatic cord, it is difficult to justify retroperitoneal lymphadenectomy. Different variables have been used to aid in the prognosis of this disease. In superficial MFH
After the early description of MFH by O’Brien and Stout,29 Cole, Straus, and GillI reported on the first case of MFH arising in the spermatic cord. MFH of the spermatic cord usually appears in males of advanced age although it has been reported in a thirty-twoyear-old male. I9 The age range was from thirtytwo to eighty-one years (average 58 years). There is no predominance for right (8 cases) or left cord (9 cases). Patients with MFH of the spermatic cord present with a gradually enlarging mass in the inguinal region or scrotum. All reported cases have had a clinical picture of a fast-growing painless tumor, with the exception of 2 cases that presented with a painful growing mass. 22,23Clinical diagnosis of a spermatic cord tumor may be difficult, and MFH as primary paratesticular lesion is indeed uncommon. Paratesticular tumors usually manifest as firm, sometimes rubbery, non-tender irregular masses. They should be considered in any intrascrotal swelling that is thought to be discrete from the testis, regardless of the growth rate, the presence or absence of pain, and degree of transilluminability, The lesion can be confused with inguinal hernia,1s*20*24multiloculated spermatocele,20 strangulated hernia,22 or epididymo-orchitis.23 The tumor can be identified by physical examination, and accurate anatomic localization can be provided by CT or magnetic resonance imaging (MRI). Regional extension as well as nodal metastases and spread can be excluded by CT, MRI, or gallium-67 scan.3o Because of the rarity of MFH, diagnosis is established by histologic examination of the surgically resected material. Grossly, the tumor is a grayish-white, fleshy mass. Microscopically, MFH classically demonstrates a storiform pattern of spindle and round cells accompanied by pleomorphic giant cells and inflammatory cell infiltration. Most cases reported are of the storiform vaand two of these have a stoririety, 12-14~16~19*21-23 form pattern with inflammatory infiltrates consisting of polymorphonuclear and eosinophilic leukocytes. 22*23Ours is the first case report of giant cell type of malignant fibrous histiocytoma occurring in the spermatic cord. Treatment of this tumor is by no means standardized, and the unpredictable biologic behavior of this tumor makes assessment of the results of any therapy difficult. In addition, experience with these uncommon tumors is limited. Initial treatment was radical orchiectomy in 10 cases, orchiectomy in 1 case,23 and local
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of the dermal structures, depth of tumor is a prognostic variable,’ but this parameter is not useful in spermatic cord MFH. Lately, flow cytometric DNA analysis of MFH tumors has been suggested as a prognostic variable.24 Survival information indicated that the majority of deaths from this disease occurred within the first two years. Seventy-nine (40%) of 200 patients died within the first two years.’ Kearney et al.” correlated the level of tumor with survival. Patients with superficial tumors had a four-year survival of 65 percent, with deep tumor a four-year survival of 40 percent, and with retroperitoneal tumors a four-year resurvival of only 14 percent. Sclama et ~121.~~ ported that the average survival of 6 patients with MFH of the spermatic cord was twentyfive months. Superficial malignant fibrous histiocytomas of the giant cell type involving subcutis or fascia have a better prognosis with two-thirds recurring and only one-sixth metastasizing. However, of the deep tumors about half metastasize, a proportion comparable to that of ordinary forms of malignant fibrous histiocytoma. The average follow-up of the 17 cases of MFH of the spermatic cord was 3.2 years (range 0.3 to 10 years). Ten patients had a twoyear follow-up, and two-year survival was 100 percent, while 3 patients had a five-year follow-up and five-year survival was 66.3 percent (2 of 3 cases). In the present case, ten years after surgery no tumor recurrences or metastases have been observed, and the patient is periodically evaluated. In the spermatic cord, malignant fibrous histiocytoma tend to be large (mean, 7.0 cm) and moderately circumscribed. Of 7 cases with follow-up data, Epstein35 reported 4 patients alive with no evidence of disease at 1.3 to four years. This patient, therefore, has the longest survival which perhaps can be attributed to the smaller size of the tumor when detected (3.5 x 2.7 x 3.0 cm) and the radical excision. High survival rate presumably is related to early tumor detection and appropriate surgical treatment given before distant metastases occurred. Reported cases are few in number, and some follow-up is not well reported; therefore, correlation of survival rate is difficult. Local tumor recurrence rates vary from 26 to 51 percent,‘*” for malignant fibrous histiocytoma in general. The rate of local tumor recurrence of the 17 reviewed cases was 21 percent (3 of 14 cases), 3 patients were excluded due to
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lack of follow-up. To avoid local recurrence these patients should be treated with nothing less than a radical operative procedure. In conclusion, radical inguinal orchiectomy and appropriate wide local excision of the tumor arising from the spermatic cord is the treatment of choice. Further management must be individualized. Department of Urology (D-l) University of Miami PO. Box 016217 Miami, Florida 33101 (DR. CIANCIO) ACKNOWLEDGMENT. To Dr. Wade Weems and Dr. James Katsikas for referring this patient to us, and the Department of Pathology, Broward General Medical Center, for supplying the original pathology material to us. References 1. Weiss SW, and Enzinger FM: Malignant fibrous histiocytoma, Cancer 41: 2250 (1978). 2. Klugo RC, Farah RN, and Cerny JC: Renal malignant histiocytoma, J Urol 112: 727 (1974). 3; Raghavaiah NV, Mayer RE.Hagitt R, and Soloway M: Malignant fibrous histiccvtoma of the kidnev. 1 Uroll23: 951 (1989). -4. Striven RR, Thrasher TV, Smith DC, and Stewart SC: Primary renal malignant fibrous histiocytoma: a case report and literature review, J Urol 131: 948 (1984). 5. Kollias G, and Giannopoulus T: Primary malignant fibrous histiocytoma of the kidney: report of a case, J Urol 138: 499 (1987). 6. Henriksen OB, Mogensen P, and Engelholm AJ: Inflammatory fibrous histiocytoma of the urinary bladder, Ada Path01 Microbiol Immunol Stand, sect. A 99: 333 (1982). 7. Goodman AJ, and Greaney MG: Malignant fibrous histiocytoma of the bladder, Br J Uro157: 196 (1985). 8. Chin W, Fay R, and Ortega P: Malignant fibrous histiocytoma of prostate, Urology 27: 363 (1986). 9. Bain GO, et al: Malignant fibrous histiocytoma of prostate gland, Urology 26: 89 (1985). 10. Watanabe K. et al: Malienant fibrous histiocvtoma of the scrotal wall: a case’report, J Ur& 146: 151 (1988). ’ 11. Kearney MM, Soule EH, and Ivins JC: Malignant fibrous histiocytoma: a retrospective study of 167 cases, Cancer 45: 167 (1989). 12. Cole AT, Straus FH, and Gill WB: Malignant fibrous histiocytoma: an unusual inguinal tumor, J Urol 107: 1995 (1972). 13. Farah RN, and Bohne AW: Malignant fibrous histiocytoma of spermatic cord, Urology 3: 782 (1974). 14. Dias R, Fernandes M, and Gaetz HP: Malignant fibrous histiocytoma of spermatic cord, Urology 12: 365 (1978). 15. Sogani PC, Grab&d H, and Whitmore WF Jr: Spermatic cord sarcoma in adults, J Urol 120: 301 (1978).’ 16. Williamson JC, Johnson JD, Lamm DL, and Tio F: Malignant fibrous histiocytoma of the spermatic cord, J Urol 123: 785 (1979). 17. Adolphs HD, Helpap B, and Koischwitz D: Retroperitoneal and inguinal manifestation of malignant histiocytoma, Urology 29: 639 (1982). 18. Wheatley KK, and Rauschkolb E: Malignant fibrous histiocytoma of the spermatic cord, J Comput Assist Toruogr 6: 1035 (1982). 19. Smailowitz Z, et al: Malignant fibrous histiocytoma of the spermatic cord, J Urol 130: 150 (1983). 20. Sclama AO, Berger BW, Cherry JM, and Young JD Jr:
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28. Ashby BS, and MacGillivray JB: Paratesticular lipoma, Br J Surg 53: 828 (1966). 29. O’Brien JE, and Stout AP: Malignant fibrous xanthomas, Cancer 17: 1445 (1964). 30. Zazzaro PF, Bosworth JE, Schneider V, and Zelenak JJ: Gallium scanning in malignant fibrous histiocytoma, AJR 135: 775 (1980). 31. Malek RS, Utz DC, and Farrow GM: Malignant tumors of the spermatic cord, Cancer 29: 1198 (1972). 32. Banowsky LH, and Schultz GN: Sarcoma of the spermatic cord and tunics: review of the literature, case report and discussion of the role of retroperitoneal lymph node dissection, J Urol 103: 628 (1970). 33. Bissada NK, Finkbeiner AE, and Redman JF: Paratesticular sarcomas: review of management, J Urol 116: 198 (1976). 34. Radio SJ, Wooldridge TN, and Linder J: Flow cytometric DNA analysis of malignant fibrous histiocytoma and related fibro-histiocytic tumors, Human Path01 19: 74 (1988). 35. Epstein JI: Diseases of the spermatic cord and paratesticular tissue, in Murphy WM (Ed): Urological Pathology, Philadelphia, W.B. Saunders, chap 4, 1989, p 236.
Malignant fibrous histiocytoma of the spermatic cord: the role of retroperitoneal lymphadenectomy in management, J Urol 130: 577 (1983). 21. Botma JP De Kock MLS, and Laubscher WML: Malignant fibrous histiocytoma of the spermatic cord, S Afr Med J 71: 326 (1987). 22. Nistal M, Regadera J, Jareno E, and Paniagua R: Inflammatory malignant fibrous histiocytoma of the spermatic cord, Urol Int 43: 188 (1988). 23. Aigaba F, Trias I, and Castro C: Inflammatory malignant fibrous histiocytoma of the spermatic cord with eosinophilia, Histiopathology 14: 319 (1989). 24. De Bruin MJFM, Pelger RCM, Meijer WS, and Giard RWM: Malignant fibrous histiocytoma of the spermatic cord, J Urol 142: 131 (1989). 25. Enzinger FM, and Weiss SW: Soft Tissue ‘lbmors, St. Louis, C.V. Mosby Co., ed 2, chap 11, 1988, p 291. 26. Sherwin B, and Bergman H: Primary malignant neoplasms of the spermatic cord and epididymis, J Urol 67: 208 (1952). 27. Powell HDW: Fibrosarcoma of the spermatic cord, Br J Urol 28: 194 (1956).
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