Malignant Fibrous Histiocytoma of the Spermatic Cord: Case Report and Literature Review

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1 The Journal of International Medical Research 2012; 40: Malignant Fibrous Histiocytoma of the Spermatic Cord: Case Report and Literature Review LW XU, YL YU AND G-H LI Department of Urology, Affiliated Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China This case study reports on a rare case of malignant fibrous histiocytoma arising in the right spermatic cord. An 80-yearold male presented with a 2-week history of a right inguinal mass, with mild pain in the same region. Ultrasonography and computed tomography showed a mass arising from the right spermatic cord. A right radical orchiectomy was performed with wide dissection of the mass; the right testicle and epididymis were found not to be grossly involved. The patient had an uneventful postoperative course and recovered well. The patient and his family refused further adjuvant radiotherapy. There was no evidence of recurrence or metastasis during 20 months of regular follow-up. The diagnosis, histological classification, treatment and prognosis of this case are presented, together with a review of the literature. KEY WORDS: MALIGNANT FIBROUS HISTIOCYTOMA; SPERMATIC CORD; TREATMENT; CASE REPORT Introduction Soft tissue sarcomas are rare malignant tumours that represent < 1% of malignancies. 1 Malignant fibrous histio - cytoma is the most commonly diagnosed subtype of soft tissue sarcoma, and was first reported in 1964 by O Brien and Stout. 2 Some pathologists consider malignant fibrous histiocytoma to be a discrete entity that may be subclassified into specific sarcoma types. 3 Malignant fibrous histiocytoma arises mainly in the deep soft tissues of the extremities (70%) and retroperitoneum (16%) and, occasionally, in the inguinal region; 4 it rarely involves the spermatic cord, and was first described at this site by Cole et al. in A literature search revealed that 33 cases of spermatic cord malignant fibrous histiocytoma have been reported in English-language journals The rarity of spermatic cord malignant fibrous histiocytoma has made its diagnosis, staging and treatment difficult. This report describes the clinical and histological features of one case of spermatic cord malignant fibrous histiocytoma, and discusses the recommended management of, and prognosis for, this tumour. Case report An 80-year-old male was admitted to the Department of Urology, Affiliated Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China, in 816

2 February The patient presented with a 2-week history of a firm mass in the right inguinal region and mild, vague pain in this region, especially when walking. He had a 2- year history of well-controlled insulindependent diabetes mellitus and a 5-year history of cholecystolithiasis. The patient had undergone colonectomy for colon carcinoma when aged 50 years and had recovered well. Physical examination revealed a painless mass in the right inguinal region, which was fixed to the right spermatic cord. The surrounding skin was of normal appearance and no superficial inguinal lymph nodes were palpated. Physical examination was otherwise unremarkable. Blood chemistry revealed no abnormalities. Levels of tumour markers (such as carcinoembryonic antigen, α-fetoprotein and prostate specific antigen) were within normal ranges. Ultrasonography revealed a hypoechoic mass ( cm), with a clear border, arising from the right spermatic cord. The testes and epididymides were of normal appearance, with the exception of several small epididymal cysts. A subsequent abdominal and pelvic computed tomography (CT) scan revealed a welldefined soft-tissue density mass within the right inguinal canal (Fig. 1A). The mass showed marked enhancement, with diminished attenuation in the centre of the lesion (Figs 1B and 1C). The patient underwent right inguinal exploratory surgery. A tumour involving the spermatic cord was identified, and a frozen section was examined and considered to be malignant. Right radical orchiectomy was subsequently performed with wide dissection of the mass. Removal of any spermatic cord remnant was performed as high as possible up to the internal inguinal ring, and the proximal extent of the cord was labelled for pathological margin evaluation. The testicle was found to be of normal size and was not involved grossly by the tumour. The tumour was cm with an intact external surface. The cut surface of the tumour was grey-yellow and elastic. Pathological examinations were completed in the pathology laboratory of Sir Run Run Shaw Hospital. Tumour samples were fixed in 10% formalin and embedded in paraffin; sections were stained with haematoxylin and eosin. Microscopic examination revealed bizarre allomorphic spindle cells, marked nuclear swelling, scattered mitoses and polynucleated giant cells (Fig. 2). Immunohistochemical analysis was performed via the EnVision system (Dako, Glostrup, Denmark) using the horseradish peroxidase diaminobenzidine complex method with antibodies to CD68 (1 : 200 dilution), vimentin (1 : 1200), smooth muscle actin (1 : 3000), HMBE-1 (1 : 200), CD117 (1 : 1200), CD34 (1 : 500), DOG1 (1 : 600), epithelial membrane antigen (1 : 1500), cytokeratin (1 : 500), desmin (1 : 4000) and S100 (1 : 4000) (all antibodies from Dako). Tumour cells were positive for CD68 (Fig. 3), vimentin, smooth muscle actin and HMBE-1, and were negative for CD117, CD34, DOG1, epithelial membrane antigen, cytokeratin, desmin and S100. The pathological diagnosis was malignant fibrous histiocytoma. Surgical margins were noted to be tumour negative, and the postoperative recovery was uneventful. The patient and his family refused any further adjuvant radiotherapy due to his advanced age. He was followed up regularly for 20 months, with no evidence of recurrence or metastasis. Discussion Since malignant fibrous histiocytoma was first reported, its status as a tumour entity 817

3 A B C FIGURE 1: Pelvic computed tomography images of an 80-year-old man with a 2-week history of a firm mass in the right inguinal region accompanied by mild, vague pain. (A) Well-defined soft tissue density mass within the right inguinal canal (arrow); (B) showing marked enhancement of the mass (arterial phase; arrow); (C) showing marked enhancement of the mass (venous phase; arrow) 818

4 A 100 µm B 100 µm FIGURE 2: Light photomicrographs of haematoxylin and eosin-stained sections of tumour tissue excised from an 80-year-old man with a 2-week history of a firm mass in the right inguinal region accompanied by mild, vague pain. (A) Low-power photomicrograph showing tumour cells with unusual morphology and severe nuclear pleomorphism (arrow). (B) High-power photomicrograph showing bizarre pleomorphic spindle cells with mitoses and giant cells (arrow) has been the subject of debate and reassessment. 17 Malignant fibrous histiocytoma, particularly in its storiformpleomorphic and myxoid forms, has been regarded as the most common type of soft tissue sarcoma for the majority of the last 30 years. 3 Malignant fibrous histiocytoma arising from the spermatic cord is rare and has been reported to represent only 11% of adult spermatic cord sarcomas. 18 A literature search of the PubMed database found that 33 cases of spermatic cord 819

5 100 µm FIGURE 3: Light photomicrograph of anti-cd68 immunohistochemical staining of tumour tissue excised from an 80-year-old man with a 2-week history of a firm mass in the right inguinal region accompanied by mild, vague pain. Tumour cells showed CD68 positive histiocytes (arrow). Cells were counterstained with haematoxylin and eosin malignant fibrous histiocytoma have been reported in English, 4 16 with the majority documented in case studies or in small series reports. The pathogenesis of malignant fibrous histiocytoma is still unknown. Tumour cells have features suggestive of fibroblastic, myofibroblastic and/or histiocytic origin, and may have developed from the fibrous tissue of the spermatic cord. 12 Staiman et al. 7 reported the case of a male Holocaust survivor who received an intratesticular injection of an unknown substance in a Nazi concentration camp 40 years before developing paratesticular malignant fibrous histiocytoma. Histopatho logically, spermatic cord malignant fibrous histiocytoma can be divided into four morphological subtypes, depending on the predominant cellular components (in decreasing order of prevalence): storiform pleomorphic type (83%), giant-cell type (9%), inflammatory type (6%) and, very rarely, myxoid type. 12 The rarity of malignant fibrous histiocytoma of the spermatic cord has led to difficulties in its diagnosis and management. Characteristics of the 33 cases of spermatic cord malignant fibrous histio cytoma reported in the literature, together with the present case, are given in Table The age distribution was one case aged < 40 years; six cases aged years; eight cases aged years; eight cases aged years; eight cases aged years; and three cases aged > 80 years. Overall, almost 80% of spermatic cord malignant fibrous histiocytoma occurred in patients who were 50 years old (Table 1). Spermatic cord malignant fibrous histiocytoma generally presented as a slowly growing, 820

6 TABLE 1: Characteristics of patients with malignant fibrous histiocytoma of the spermatic cord identified in a PubMed search of Englishspeaking journals, 4 16 together with the current case (n = 34) Age Mean age at diagnosis, years 62.1 (32 87) 50 years 27 (79) Growth pattern Single mass 26 (76) Multiple lesions 8 (24) Duration of symptoms, 2 weeks 15 years range Tumour diameter Range < 1 27 cm 2 10 cm 26 (76) > 10 cm 7 (21) Treatment Radical surgery 32 (94) Adjuvant radiotherapy 12 (35) Local recurrence or metastasis 7 (21) Data presented as n (%) of patients, mean (range) or range, as indicated. painless solitary mass in the left or right inguinal region, although eight cases had multiple tumours or satellite nodules. 4,5,12 Several patients felt mild pain in the tumour region. The largest spermatic cord malignant fibrous histiocytoma recorded in the literature was a well-encapsulated mass, measuring cm. 7 The unusually large size of this tumour may have been caused by the intratesticular injection of an unknown substance. Preoperative diagnosis of spermatic cord malignant fibrous histiocytoma is difficult, as clinical presentation and physical examination alone usually cannot determine the origin and nature of an inguinal mass. Indeed, it is easy to misdiagnose spermatic cord malignant fibrous histiocytoma as a hernia, spermatocele, hydrocele or other benign condition. The majority of tumours in the published cases showed nonspecific radiological findings, further complicating the diagnosis. Ultrasonography is helpful in defining neoplasms and can reveal satellite nodules. It is accurate in distinguishing testicular from nontesticular masses and eliminating other diagnostic possibilities. 4,19 Although the imaging findings are rather nonspecific, CT and magnetic resonance imaging (MRI) studies are essential for preoperative staging and surgical planning in patients with spermatic cord malignant fibrous histiocytoma. 20,21 CT is necessary for staging of the pelvis and abdomen, especially when the tumour is not well differentiated. 19 MRI is an excellent modality for evaluating the extent of inguinal masses; 10 such images often show heterogeneous signal intensity on all pulse sequences, reflecting the complex histological components of the tumour. 22 A definitive diagnosis of soft tissue sarcoma always requires pathological examination. Biopsy is an essential component of the preoperative diagnostic work-up and is the only way to establish whether a soft tissue tumour is malignant, 17 although care should be taken to ensure that the biopsy does not interfere with subsequent optimal definitive surgery. The use of fine needle aspiration in the diagnosis of soft tissue sarcoma is controversial and is, therefore, relatively underused; although it is reliable in making an initial diagnosis of sarcoma, specific subtyping as malignant fibrous histiocytoma is more problematic. 23 Bosch-Príncep et al. 9 reported the use of fineneedle aspiration and imprint cytology in a case of spermatic cord malignant fibrous histiocytoma. They found that fine-needle aspiration of a spermatic cord lesion may reveal a pleomorphic sarcoma, and concluded that a pleomorphic appearance (together with some spindle elements and compatible immunocytochemistry) could 821

7 help to diagnose spermatic cord malignant fibrous histiocytoma. Spermatic cord malignant fibrous histiocytoma has a high propensity for local recurrence and metastasis. Of the 34 reported cases, seven recurred after surgery (21%). The most common site for meta stasis in malignant fibrous histiocytoma is the lung (90%). 24 Prognostic factors of spermatic cord malignant fibrous histio cytoma are difficult to assess because of the relative paucity of cases and the short follow-up. Coleman et al. 18 did not identify any specific prognostic factors associated with recurrence or disease-free survival in adult spermatic cord sarcomas. Based on a careful review of previous reports and the present case study, we suggest that tumour biology, adequacy of surgical resection and adjuvant treatment could be the major factors that affect local control rates. Histopathological grade and size are known to be significant prognostic factors for malignant fibrous histiocytoma, 24,25 and the presence of satellite nodules at initial surgery increases the overall recurrence rate. The current treatment strategy for all soft tissue sarcomas begins with complete local resection: radical inguinal orchiectomy and removal of all the spermatic structures are necessary components of surgical management and were performed in 94% (32/34) of the reported cases. Wide local excision or partial scrotectomy were performed in six patients whose tumours extended into adjacent tissues or who had satellite nodules. In the reported cases, seven patients developed local recurrence and lung or mesentery metastasis, indicating a need for additional treatment The addition of radiotherapy substantially and significantly decreased the risk of local relapse. 25,26 Localized radiotherapy was used as an adjuvant therapy in 12 cases, 4,6,12,13,15,16 with a dose range of Gy. It is recommended that the radiation field includes the entire tumour bed, scar, drain sites and a margin of 5 cm around the tumour bed. 27 Of these 12 patients, 10 remained tumour free (duration of followup, 0 months 5 years), one died after 27 months and one developed local recurrence after 18 months. Chemotherapy is generally reserved for metastatic disease. The two most commonly used chemotherapeutic agents doxorubicin and ifosfamide have response rates ranging from 17 to 33% as single-agent therapy and from 55 to 66% when used in combination. 1,27 In conclusion, the optimum treatment strategy for spermatic cord malignant fibrous histiocytoma is radical orchiectomy and wide excision of surrounding soft tissue structures, followed by radiation therapy. Conflicts of interest The authors had no conflicts of interest to declare in relation to this article. Received for publication 18 October 2011 Accepted subject to revision 9 November 2011 Revised accepted 25 January 2012 Copyright 2012 Field House Publishing LLP References 1 Skubitz KM, D Adamo DR: Sarcoma. Mayo Clin Proc 2007; 82: O Brien JE, Stout AP: Malignant fibrous xanthomas. Cancer 1964; 17: Fletcher CD, Gustafson P, Rydholm A, et al: Clinicopathologic re-evaluation of 100 malignant fibrous histiocytomas: prognostic relevance of subclassification. J Clin Oncol 2001; 19: Glazier DB, Vates TS, Cummings KB, et al: Malignant fibrous histiocytoma of the spermatic cord. J Urol 1996; 155: Cole AT, Straus FH, Gill WB: Malignant fibrous histiocytoma: an unusual inguinal tumor. J Urol 1972; 107:

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