Organ-sparing Surgery in Treating Patients with Liposarcoma of the Spermatic Cord: Institutional Experience and Pooled Analysis

Background: Liposarcoma of the spermatic cord is rare and frequently misdiagnosed. The standardtherapeutic approach has been radical inguinal orchiectomy with wide local resection of surrounding softtissues. The current trend of organ preservation in the treatment of several cancers has started to evolve. Hereinwe present our testis-sparing surgery experience in the treatment of spermatic cord liposarcoma and a pooledanalysis on this topic. Materials and Methods: Clinical information from patient receiving organ-sparing surgerywas described. Clinical studies evaluating this issue were identified by using a predefined search strategy, e.g.,Pubmed database with no restriction on date of published papers. The literature search used the followingterms: epidemiology , surgery , chemotherapy , radiotherapy , testis sparing surgery, spermatic cord sarcomas/liposarcomas. Results: Patient received a complete excision of the lesion, preserving the spermatic cord and thetestis. The final pathological report showed a well differentiated liposarcoma with negative surgical marginsand no signs of local invasion. After 2-year of follow-up, there was no evidence of local recurrence. Since thefirst case reported in 1952, a total of about 200 well-documented spermatic cord liposarcoma cases have beenpublished in English literature. Among these patients, only three instances were reported to have received anorgan-sparing surgery in the treatment of spermatic cord liposarcoma. Conclusions: Radical inguinal orchiectomyand resection of the tumor with a negative microscopic margin is the recommended treatment for liposarcomaof the spermatic cord. But for small, especially well-differentiated, lesions, testis-sparing surgery might be agood option if an adequate negative surgical margin is assured.     

Retroperitoneal sarcomas: A single center experience.]

PURPOSE: To analyse the management and clinical outcome of patients treated for retroperitonal soft tissue sarcoma and to identify prognosis factors. PATIENTS AND METHODS: This is a retrospective study of 20 adults ; 11 women and nine men treated between 1980 and 2000 in our institution. RESULTS: There were seven liposarcomas and 13 leiomyosarcomas. The mean tumor size was 26cm. Three patients underwent a complete resection (R0), 14 patients an incomplete resection (R1/R2) with microscopic or macroscopic tumour remained after surgery. Only three patients had a surgical biopsy. Median follow-up was 18 months. Overall survival rates was 45%, 10% at two and five years, respectively. Among the following factors, sex, age, symptom duration, tumor size, histologic type, histologic grade, resection type, mitotic index, necrosis extension, histologic differentiation, and AJCC stage, only mitotic index factor (scale 1,2 versus scale 3) significantly influenced patient survival (p=0.02). CONCLUSIONS: This study re-emphasizes the poor outcome of patients with retroperitoneal sarcoma. Survival rates appeared low when compared with other studies. These results incite for a more aggressive approach.     

Retroperitoneal liposarcomas: follow-up analysis of dedifferentiation after clinicopathologic reexamination of 86 liposarcomas and malignant fibrous histiocytomas

BACKGROUND: Dedifferentiated liposarcoma (DDL) juxtapose components of well-differentiated liposarcoma (WDL) and nonlipogenic sarcoma. Malignant fibrous histiocytoma (MFH) is no longer considered a homogeneous entity, but rather as the common morphologic appearance of various subtypes of sarcomas. The objectives of the current retrospective study were: 1( to analyze the relation between DDLs and tumors previously diagnosed as MFHs; 2) to trace the evolution of liposarcomas, and 3) to assess the consequences of dedifferentiation. METHODS: Between 1974 and 2001, 86 patients with retroperitoneal liposarcoma (61 patients) or MFH (25 patients) underwent surgery at Institut Bergonie in Bordeaux, France. Histologic review was performed and tumors reclassified as WDL or DDL were retained for further clinicohistologic study. Subsequently, initial presentation and all recurrences were analyzed. RESULTS: The 61 liposarcomas consisted of 21 WDLs and 35 DDLs; 17 MFHs were reclassified as DDL. In all, approximately half of the retroperitoneal liposarcomas and MFHs were found to be DDLs. The DDLs presented with a smaller size (20 cm vs.30 cm; P = .05) but a lower rate of complete resection (72% vs.90%; P = .015) and remission (72% vs. 100%; P = .0015). Dedifferentiated recurrence was evidenced in 7 WDLs. Ten DDLs presented with metastatic evolution. DDLs demonstrated a tendency toward lower rates of 5-year overall survival (55% vs. 82%; P = .075). CONCLUSIONS: Most occurrences of retroperitoneal liposarcomas and MFHs are in fact DDLs and dedifferentiated recurrence of WDLs is frequent. Retroperitoneal DDLs present a lower rate of complete remission than WDLs and a risk of metastatic recurrence. Therefore, extensive histologic analysis of WDLs is required to identify an undifferentiated component and avoid misdiagnosis of DDL.     

无脂肪的腹膜后脂肪肉瘤的 CT 和 MRI 表现

目的：探讨并分析影像学评价无脂肪的腹膜后脂肪肉瘤（lipid poor retroperitoneal liposarcomas, LPRL）的 CT 和 MRI 表现及其临床诊断、鉴别价值。方法回顾性分析21例经病理证实的无脂肪的腹膜后脂肪肉瘤患者的临床及 CT、MRI 影像学资料,并与病理和组织学表现对照分析。结果21例患者病变表现为腹膜后软组织肿物,大小为（12.5±8.0）cm,范围为1.9～21.0 cm。肿瘤边界清晰者12例,边界不清者9例。病变呈分叶状或形态不规则。肿瘤位于肾周间隙者12例,位于肾旁前间隙者6例,位于肾旁后间隙者3例。9例患者的肿瘤伴粗大血管影,11例伴分隔,7例伴坏死,4例伴钙化（粗大、散在、多发钙化）。该病有特征性的CT、MRI 表现,多表现为软组织实性肿物;平扫密度或信号不均匀,增强后实性部分不均匀强化,病变多伴坏死或分隔、血管影。结论 CT 及 MRI 对无脂肪的腹膜后脂肪肉瘤患者的临床诊断和鉴别有重要的参考价值。     

Spermatic Cord Sarcoma in Adults

The cases of 16 adult patients with spermatic cord sarcoma were retrospectively reviewed. Patient ages had a bimodal distribution (median 57.5 years). Presentation was scrotal mass, inguinal mass or both, with an average diameter of 6.7 cm. Radical orchiectomy was performed in 9 patients, and simple tumorectomy in 7. Lymph node dissection was performed in 6 patients, including retroperitoneal dissection in 4 patients. Various types of soft tissue sarcomas were found. Embryonal rhabdomyosarcomas were evident only in young patients. Lymph node metastases were found in 2 out of 6 cases. Testicular atrophy was observed in 3 young patients, germline destruction by the tumor in 2, and partial spermatogenesis in 2 patients. The median time to first relapse in 13 patients was 5.5 months. Surgery was the primary treatment and the best salvage modality. The role of chemotherapy and radiation therapy as true adjuvant or post-salvage-surgery adjunctive treatments remains unclear.     

An operative case of spermatic cord rhabdomyosarcoma--with a review of similar cases in Japan

A 17-year-old Japanese boy was admitted with the complaint of painless, progressive enlargement of the left scrotal contents during the preceding two months. The tumor was firm and nontender without transillumination. Orchitectomy with high ligatation of the spermatic cord was performed. The tumor, weighing 13.1 g, was gray, solid, located at the distal part of the spermatic cord, and separated from the testis and epididymis. The pathological diagnosis showed left spermatic cord rhabdomyosarcoma of the combined embryonal and alveolar type. All of the retroperitoneal lymph nodes dissected were negative for metastasis. The patient was treated with actinomycin D and vincristin postoperatively, and is well three years after the operation. Ninety-two cases of intrascrotal rhabdomyosarcoma reported in Japan are reviewed.     

Atypical lipoma, atypical intramuscular lipoma, and well differentiated retroperitoneal liposarcoma: a reappraisal of 30 cases formerly classified as well differentiated liposarcoma.

Thirty cases formerly diagnosed as Grade I lipogenic liposarcoma (well differentiated liposarcoma) were reviewed. The basic histologic pattern in all was that of adult fat modified by the presence of cells with enlarged, hyperchromatic nuclei; in most cases there was also a component of myxoid and/or fibrous tissue. The length of follow-up ranged from two to 30 years. Nine of the tumors were located in the subcutaneous layer. None of these recurred after excision, not even those which were simply "shelled out," and none metastasized. The term "atypical lipoma" is proposed for this group. Thirteen were located within or between muscles of the limbs, limb girdles, and head and neck. Nine of these recurred at least one, but there were no metastases and no deaths due to tumor. These were designated "atypical intramuscular lipoma." The remaining eight originated in the retroperitoneum. Although none of these patients developed metastases, five suffered inoperable recurrence and three died as a result of the neoplasm. It is suggested that the term "well differentiated retroperitoneal liposarcoma" be retained for cases of this type.     

Giant malignant mesenchymoma of the spermatic cord with bidirectional differentiation.

BACKGROUND: Spermatic cord neoplasms are a rare tumor entity and, moreover, of benign behavior. Malignant tumors of the spermatic cord are mostly of mesenchymal origin. We present the unusual case of a giant malignant mesenchymoma of the spermatic cord with bidirectional differentiation into a liposarcoma and a leiomyosarcoma. CASE REPORT: A 84-year-old male patient presented with a scrotal mass on the left side which was observed growing since 1 year and misdiagnosed as scrotal hernia or testicular hydrocele. Ultrasound and computed tomography demonstrated a solid tumor suggesting a spermatic cord tumor. The patient underwent hemiscrotectomy, and the histological examination of the 2,500-gram specimen revealed a malignant mesenchymoma originating from the spermatic cord with two distinct histopathological compartments of liposarcoma and leiomyosarcoma. Because an adjuvant therapy protocol is of questionable effect and because of the patient's age no further therapy was applied. The patient was closely followed and is now, 5 years after surgery, still free of disease. CONCLUSION: Even in older patients, scrotal masses should be considered malignant tumors as long as no benign diagnosis has been proven. Although malignant mesenchymomas are rare tumors with poor prognosis, in selected cases even large tumor masses, as presented, can be cured by surgery.     

腹膜后脂肪肉瘤的影像学和病理学分析

目的探讨不同组织学亚型腹膜后脂肪肉瘤的影像学和病理组织学特征。方法回顾性分析21例腹膜后原发性脂肪肉瘤的CT和MRI表现,按不同病理组织学亚型进行对照观察。结果21例腹膜后原发性脂肪肉瘤中,高分化11例,黏液性4例,圆形细胞性3例,多形性2例,去分化脂肪肉瘤1例。分化型脂肪肉瘤主要由脂肪瘤样和硬化性成分组成,脂肪瘤样成分的CT密度、MRI信号与脂肪成分相似,而硬化性成分与肌肉的密度和信号相似。黏液性脂肪肉瘤CT平扫密度和MRI信号同水相似,CT增强扫描表现为网状延迟强化。圆形细胞性和多形性脂肪肉瘤表现为无明显脂肪、但有灶性坏死的软组织肿块。去分化脂肪肉瘤表现为分化良好的脂肪瘤样成分与明显强化的软组织肿块成分,二者分界清楚。结论不同病理组织学亚型的脂肪肉瘤,其CT和MRI表现有所不同,这取决于肿瘤所含的主要组织学成分。了解肿瘤不同的组织学亚型与影像学表现之问的关系,有助于腹膜后脂肪肉瘤的准确诊断。     

A case of metastatic carcinoma of the spermatic cord due to cholangioma

Tumor in the spermatic cord is infrequent. Further, among such cases, it is extremely rare to encounter a carcinoma other than a nonepithelial malignant tumor; almost all are metastatic tumors. We recently experienced an interesting case of spermatic cord tumor in a 78-year-old male. He entered our hospital with the chief complaint of a right inguinal tumor. He underwent 2 operations to excise the tumor. The extirpated tumor mass from the right spermatic cord was composed of adenocarcinoma with abundant fibrous stroma, which is considered to be metastasis from the other viscerae. We were unable to find the primary focus in spite of extensive clinical and pathological examinations. In an autopsy about four months after the 2nd operation, the tumor was confirmed to be primary intrahepatic bile duct carcinoma, which is called cholangioma. There have been only 20 cases of metastatic spermatic cord tumor reported in Japan. However, we considered that this is the 1st case caused by cholangioma in Japan and even in other countries.     

Primary lymphoma of spermatic cord.

Primary lymphomas of spermatic cord are extremely rare. In a review of the world medical literature, until now, only fourteen cases of spermatic cord lymphoma have been reported, and, furthermore, they have a poor prognosis even in patients with stage I disease. Herein, we report a new case of primary non-Hodgkin's lymphoma of the spermatic cord. In August, 1993, 76-year-old man visited an urological hospital with a compaint of a right intrascoral mass, and underwent orchiectomy. Macroscopically no invasive lesion in the testis was observed, and the tumorous lesion was restricted to the epididymis. The histopathological study indicated that he suffered from primary malignant lymphoma of the spermatic cord (B-cell, diffuse medium-sized cell type). As radiographic investigations showed no other invasive lesion, the patient was diagnosed to be in stage IE. He was followed only with clinical observation, and, in August, 1996, relapsed with extensive disease in the abdoninal cavity, and was transferred to our hospital. Fourty months after the orchiectomy, he died of progression of disease irrespective of the salvage radio-chemotherapies given to him.     

Derangement of adipose tissue: a case report of multicentric retroperitoneal liposarcomas, retroperitoneal lipomatosis and multiple subcutaneous lipomas

No aetiological factors are at present known in the pathogenesis of liposarcomas. Benign lipomatous disorders, such as lipomas and lipomatosis, generally do not predispose to the development of liposarcomas. A case is presented of a patient with multicentric retroperitoneal liposarcomas, associated with retroperitoneal lipomatosis and preceded by a long history of multiple subcutaneous lipomas. In the literature this combination has not been described. This rare combination of lipomatous disorders seems to indicate a common derangement of adipose tissue.     

Large ulcerated cecal lipoma mimicking malignancy

Colonic lipomas are relatively uncommon tumors of mesenchymal origin, composed of well-differentiated adipose tissue supported by fibrous tissue, that usually occur in cecum and ascending colon. Colonic lipomas rarely cause symptoms and are usually detected incidentally. However, if the lesion is large, it may produce symptoms, such as abdominal pain, rectal bleeding, obstruction, intussusception, and even weight loss. Large colonic lipomas can be mistaken for malignancy, which may result in extensive surgical operations. We report a large broad-based ulcerated cecal lipoma in a 68-year-old woman, who presented with abdominal pain and weight loss. The ulcerated lesion was highly suspicious for malignancy radiologically and endoscopically. The patient underwent laparoscopic right-hemicolectomy, and the lesion was diagnosed as a cecal submucosal lipoma. The surgical approach remains the treatment of choice for large and complicated cases.     

Surgical treatment of 43 retroperitoneal sarcomas

Forty-three cases of primary retroperitoneal sarcomas, observed and treated from 1970 to 1983 at this Institute, were analysed. The series consisted of 16 liposarcomas (37%), 10 leiomyosarcomas (23%), 7 rhabdomyosarcomas (16%), 5 fibrosarcomas (12%), 2 malignant histiocytomas (5%), 2 sarcomas NOS (5%) and 1 mesenchymoma (2%). All the patients underwent surgery. In order to evaluate the results of surgery, the patients were divided into three groups, according to the type of operation performed, that is open biopsy, resection and excision. Survival of the patients in the first group never exceeded 24 months. The symptom-free period for the patients treated by incomplete removal of the tumour (second group) lasted 3-24 months. Further surgery, in three cases for this group, did not result in a useful control of the disease. As regards radically treated cases, local recurrence was observed in 3 of the 7 liposarcomas, 2 of the 5 leiomyosarcomas, 1 of the 2 rhabdomyosarcomas and 1 fibrosarcoma. Out of 16 cases of the third group, regularly followed up, only 3 patients (liposarcomas) were alive and free of disease at 5 years from first operation. Overall 5-year survival for this group was 31.9%; disease-free survival was 18.7%. For the whole series of 43 cases, overall 5-year survival was 11.4%. As far as histology was concerned, liposarcomas showed the highest operability rate: out of 16, 7 were resected and 9 radically excised. There is a lack of convincing evidence for the utility of post-operative chemotherapy. On the contrary, post-operative radiotherapy seems to be worthwhile for liposarcomas, especially after non-radical operations.     

Intraspinal Lumbosacral Lipoma: Review of Literature and Report of Three Cases

Three cases of intraspinal lumbosacral lipoma are reported and the existing English literature is reviewed. Intraspinal lumbosacral lipoma presents a distinct clinical and radiological pat tern. It should be suspected if, in a child, a soft tissue mass in the lumbosacral region is associated with a neurological deficit in the lower limbs, unilateral talipes equinovarus or bony developmental defects of the vertebral bodies and sacrum. The presence of radio-lucency in the vertebral canal in plain films is pathognomonic of intraspinal lipoma. At myelography, the presence of a large thecal sac, a low position of the conus medullaris and demonstration of extension of the lesion outside the vertebral canal are characteristic of in traspinal lipoma. In infancy, these lesions may superficially resemble sacrococcygeal teratoma.     

Aggressive angiomyxoma mimicking inguinal hernia in a man

We report the case of a 37-year-old Japanese man who presented with a left lower abdominal mass that was initially interpreted clinically as an inguinal hernia. The patient reported a swelling in his left lower abdomen over the past 6 months. Magnetic resonance imaging revealed a left inguinal mass extending from the left spermatic cord to the left inguinal subcutaneous layer. Local excision was performed, with a preoperative diagnosis of benign soft tissue tumor. Histopathological diagnosis revealed aggressive angiomyxoma (AAM), and no recurrence was observed 6 months after surgery. AAM is a rare tumor which most commonly occurs in the pelvis or perineum of premenopausal women, with extremely rare cases in men. The findings from previously reported cases and our case suggest that AAM should be considered in patients with an inguinal or scrotal swelling with a suspicious soft tissue tumor.     

Liposarcoma of the spermatic cord: case report and review of the literature

This report describes a patient with liposarcoma of the spermatic cord. The clinical presentation, pathogenesis, and prognosis of this unusual sarcoma are reviewed. The low-grade malignant potential and irregular growth characteristics of this liposarcoma render preoperative diagnosis difficult. The role of radical orchiectomy, retroperitoneal lymph node dissection, radiation therapy, and chemotherapy in the treatment of spermatic cord sarcomas is discussed.     

The role of standard and novel radiotherapy approaches in management of retroperitoneal sarcomas

Primary non-metastatic retroperitoneal soft tissue sarcoma patients can be cured by radical surgery. However there remains a risk for patients to develop a local recurrence. To minimize this risk, patients with low grade liposarcomas might benefit from preoperative radiotherapy. This review summarizes all issues that should be considered for the irradiation of patients with retroperitoneal soft tissue sarcoma.     

Expression of epidermal growth factor receptor, ERBB2 and KIT in adult soft tissue sarcomas: a clinicopathologic study of 281 cases

BACKGROUND: Little is known about the expression of receptor tyrosine kinases in adult soft tissue sarcomas (STS). In the current study, the authors analyzed the expression of epidermal growth factor receptor (EGFR), ERBB2, and KIT in 281 patients with STS who were treated in a single institution. Verification of the presence of an association with prognosis was performed. METHODS: The current study included 281 adult patients with STS of the extremity and trunk who were diagnosed and treated in the National Cancer Center, Tokyo. Expression was assessed using immunohistochemical stains for EGFR, ERBB2, and KIT on formalin-fixed, paraffin-embedded tissue sections by standard avidin-biotin peroxidase complex technique and EGFR detection system. RESULTS: Positive staining of EGFR was observed in 168 of 281 (60%) patients. Positive staining was common in pleomorphic malignant fibrous histiocytomas (89%), myxofibrosarcomas (89%), synovial sarcomas (76%), malignant peripheral nerve sheath tumors (89%), and leiomyosarcomas (73%). It was less common in well differentiated liposarcomas (38%), fibrosarcomas (36%), and myxoid liposarcomas (6%). In contrast, positive staining of ERBB2 and KIT was very limited. Increased levels of EGFR were significantly associated with a decreased probability of overall survival (P = 0.01), although by univariate analysis; probability of overall survival at 5 years was 64% in patients with increased levels of EGFR and 79% in patients without such overexpression. The overexpression of EGFR was significantly associated with histologic grade (P < 0.001). Moreover, stratified log-rank test revealed that there is an interrelation between EGFR overexpression and histologic grade. CONCLUSIONS: EGFR overexpression was found to be a negative prognostic factor of adult STS, which is strongly associated with histologic grade. STS patients with EGFR overexpression may benefit from treatment with currently available biospecific inhibitors for EGFR.     

Cytogenetic findings in liposarcoma correlate with histopathologic subtypes.

The cytogenetic findings in 31 liposarcomas from 26 patients are reported. Four other tumors did not grow. Three histologic types are represented in this analysis. The well-differentiated liposarcomas were characterized by telomeric associations, large marker chromosomes and ring chromosomes, and in some cases, double minutes. The pleomorphic liposarcomas contained very high clonal chromosomal numbers with near-tetraploid modes and numerous variable, often unidentifiable, chromosomal abnormalities. The myxoid liposarcomas were characterized primarily by a t(12;16)(q13;p11) as the sole abnormality or additional changes. These results indicate that cytogenetic findings may provide a new criterion, not only for establishing the diagnosis of liposarcoma, but also for differentiating confusing histologic types of liposarcoma and these lesions from other types of sarcomas.