Importance of preoperative diagnosis for management of patients with suspected retroperitoneal sarcoma

Soft tissue sarcoma is an umbrella term which encompasses over 60 histological tumour types. Approximately 15% of soft tissue sarcomas arise in the retroperitoneum. This complex group of tumours poses unique management challenges due to their often large size, histological heterogeneity and complexity of anatomical relationships. This review discusses the management of retroperitoneal tumours including the need for preoperative diagnosis, the evidence for neoadjuvant radiotherapy, the role of multivisceral resection and the importance of a multidisciplinary team approach.     

Retroperitoneal and scrotal giant liposarcoma: Report of a case

(Received for publication on Aug. 5, 1996; accepted on Mar. 4, 1997)     

腹膜后脂肪肉瘤的生物学特性及其诊断和治疗

目的 探讨腹膜后脂肪肉瘤生物学行为及诊断与治疗的有关问题。方法 回顾性分析1970—2005年中国医科大学附属第一医院收治的32例（共接受手术43例次）原发及复发腹膜后脂肪肉瘤病人的临床和病理资料。结果 该病主要临床表现是腹胀、腹部包块进行性增大。复发病例较原发病例生物学行为差，肿瘤侵袭力强，组织学亚型恶性程度高，手术完全切除率低。多次复发者复发间期逐渐缩短。难治性大出血是术后主要且致命的并发症。结论 腹膜后脂肪肉瘤术后易复发，很少转移，手术切除是最有效的治疗手段。完全切除者复发间期长，控制术中出血是减少术后死亡的关键。放、化疗对腹膜后脂肪肉瘤的作用有限。     

Demographics,management and treatment outcomes of benign and malignant retroperitoneal tumors in Japan

Objectives

To show the demographics, type of treatment and clinical outcomes of patients with retroperitoneal tumors in Japan.

Methods

We carried out a retrospective analysis of patients with retroperitoneal tumors treated between 2000 and 2012 at 12 university hospitals in Japan. Histology was re‐evaluated using the 2013 World Health Organization classification.

Results

A total of 167 patients were included in the analysis. The number of diagnosed patients increased over the 12‐year study period. Liposarcoma and schwannoma were the most common histological types among intermediate/malignant and benign tumors, respectively. The intermediate/malignant tumors were larger and were more frequently found in older people. Surgical resection was the primary treatment for 151 patients. The median survival duration for patients with malignant tumors was 91 months, and was significantly shorter than that for patients with benign and intermediate tumors (P < 0.01). R2 resection was associated with significantly shorter survival than R0/R1 resection for malignant tumors (P < 0.01), but not for intermediate. Grossly complete resection of the recurrent tumors improved survival.

Conclusion

The number of patients diagnosed with retroperitoneal tumors increased over time. R2 resection of primary tumors was found to be associated with poor prognosis in malignant tumors, but not in intermediate tumors. Complete surgical resection of recurrent tumors was associated with a better oncological outcome.     

肾周脂肪肉瘤的诊断和外科治疗

目的 探讨肾周脂肪肉瘤的外科治疗方法。方法 总结分析2000—2006年间收治的28例肾周脂肪肉瘤病人经手术治疗的临床资料。结果 肾周脂肪肉瘤临床主要表现为进行性增大的腹部包块与消化道症状；起源于右肾周17例，左肾周11例。28例全部行手术切除。联合患侧肾脏切除19例，结肠部分切除10例，小肠部分切除8例，胃部分切除2例，脾切除2例，部分膈肌切除、胸腔闭式引流5例。平均输血1870mL。结论 手术切除是目前治疗肾周脂肪肉瘤惟一有效的方法，根治性手术必须包括肿瘤、肾脏和肾周脂肪囊在内的整块切除。     

Retroperitoneal tumours: review of management

INTRODUCTION

The retroperitoneum can host a wide spectrum of pathologies, including a variety of rare benign tumours and malignant neoplasms that can be either primary or metastatic lesions. Retroperitoneal tumours can cause a diagnostic dilemma and present several therapeutic challenges because of their rarity, relative late presentation and anatomical location, often in close relationship with several vital structures in the retroperitoneal space.

MATERIALS AND METHODS

A comprehensive literature search was conducted using PubMed. Relevant international articles published in the last ten years were assessed. The keywords for search purposes included: retroperitoneum, benign, sarcoma, neoplasm, diagnosis and surgery, radiotherapy, chemotherapy. The search was limited to articles published in English. All articles were read in full by the authors and selected for inclusion based on relevance to this article.

RESULTS

Tumours usually present late and cause symptoms or become palpable once they have reached a significant size. Retroperitoneal tumours are best evaluated with good quality cross-sectional imaging and preoperative histology by core needle biopsy is required when imaging is non-diagnostic. Sarcomas comprise a third of retroperitoneal tumours. Other retroperitoneal neoplasms include lymphomas and epithelial tumours or might represent metastatic disease from known or unknown primary sites. The most common benign pathologies encountered in the retroperitoneum include benign neurogenic tumours, paragangliomas, fibromatosis, renal angiomyolipomas and benign retroperitoneal lipomas.

CONCLUSIONS

Complete surgical resection is the only potential curative treatment modality for retroperitoneal sarcomas and is best performed in high-volume centres by a multidisciplinary sarcoma team. The ability completely to resect a retroperitoneal sarcoma and tumour grade remain the most important predictors of local recurrence and disease-specific survival.     

原发性腹膜后软组织肉瘤诊治中国专家共识（2019版）

正软组织肿瘤主要是指一大类源于间叶组织的肿瘤,传统上还包括周围神经源性的肿瘤。其胚胎发生上主要源于中胚层和神经外胚层。腹膜后腔是指位于横膈以下和盆膈以上,后壁层腹膜与腹横筋膜间的潜在腔隙,是软组织肿瘤的好发部位之一。原发性腹膜后肿瘤是指源于腹     

隆凸性皮肤纤维肉瘤的诊断和治疗

目的 探讨隆凸性皮肤纤维肉瘤（DFSP）的诊断和治疗方法。方法 分析第二军医大学长海医院收治的DFSP病人11例,均由手术后病理确诊。其中DFSP纤维肉瘤型（DFSP-FS）4例。肿瘤位于躯干部9例,位于四肢2例。2例行局部常规切除,其余9例均行扩大切除术。3例在术后行放射治疗,平均剂量55Gy（50—65Gy）。结果 切除11例标本,CD34阳性10例,阴性1例。术后随访1—10年（平均4．3年）,失访1例。复发6例,平均复发时间2、6年（10个月至5年）。11例均未见有远处转移,无死亡。结论 DFSP在临床上易与其他软组织肿瘤相混淆。诊断主要依据病理。治疗DFSP最主要的方法是扩大的手术切除,切缘阳性或肿块太靠近切缘的辅以放疗。单独使用放疗可以作为偶见的无法切除肿块的治疗方式。     

原发性腹膜后副神经节瘤14例报告并文献复习

目的：探讨原发性腹膜后副神经节瘤的临床和病理特点、诊治方法及预后。方法：回顾性分析14例病理检查证实为腹膜后副神经节瘤患者的临床资料：男8例,女6例,年龄14～66岁。有高血压症状9例,其中尿香草扁桃酸（VMA）升高8例,尿儿茶酚胺升高9例。14例均接受手术治疗。术后随访13个月～5年。结果：术中见肿瘤位于肾门区4例,肾上极区5例,肾下极区2例,肾上腺前外上方3例。13例手术完全切除肿瘤,1例伴肝转移者行肿瘤大部切除及肝尾叶切除。病理检查诊断为良性肿瘤13例,恶性肿瘤1例。术后随访12例,9例术前高血压者有7例血压恢复正常;9例无瘤生存,1例复发,2例死亡。结论：手术完全切除肿瘤是腹膜后副神经节瘤最有效的治疗方法,充分的术前准备和围手术期处理是手术成功的关键。术后生存时间取决于肿瘤是否发生淋巴结或远处转移,复发病例可再次手术切除。     

Inflammatory Leiomyosarcoma of the Ankle: A Case Report and Review of the Literature

Inflammatory leiomyosarcoma is a rare morphologic expression of smooth muscle tumor, which is more often found in the trunk, extremities, and retroperitoneoum. We present a case of inflammatory leiomyosarcoma arising near the ankle joint, superior to the medial malleolus, in a 55-year-old man. Immunohistochemistry, electron microscopy, and cytogenetics were used in the differentiation of this rare morphologic expression of leiomyosarcoma. As in all soft tissue leiomyosarcomas, the accurate prediction of the clinical behavior of this morphologic expression often proves to be difficult. Age, vascular invasion, depth, grade, tumor disruption, size, and stage have prognostic value. Nevertheless, prognosis of inflammatory leiomyosarcoma seems to be favorable when treated early with wide excision of the tumor as in the case presented. Sixteen months after excision of the tumor the patient is doing well with no signs of recurrence.     

原发性腹膜后黏液性囊腺瘤1例报告并文献复习

目的：探讨原发性腹膜后黏液性囊腺瘤的临床及病理特点。方法：回顾性分析1例原发性腹膜后黏液性囊腺瘤患者的临床资料：患者女,29岁。19个月前体检时B超发现腹膜后右肾下极有3．5cm×2．4cm无回声占位性病变,内无明显血流信号。患者每6个月进行一次影像学复查,肿物体积未见明显变化。血清Ca199、Ca125、CEA等指标均在正常范围。患者在全麻下行腹腔镜腹膜后肿瘤切除术。并检索Pubmed和CBM数据库,对该疾病相关文献进行复习。结果：术后病理检查诊断为腹膜后交界性黏液性囊腺瘤。术后随访2个月,未见肿瘤复发。结论：腹膜后黏液性囊腺瘤是一种罕见的腹膜后肿瘤,诊断该病主要依靠术后病理检查。该肿瘤的体积及生长速度与肿瘤的恶性程度无确定关系。腹腔镜手术治疗腹膜后黏液性囊腺瘤安全有效。     

Multicentric liposarcoma: Report of two cases

We present herein two unusual cases of multicentric liposarcoma which highlight the problems associated with the management of this disease entity. When the surgeon is confronted with multicentric liposarcomas, it is necessary to define whether they are secondary tumors or independently arising multicentric liposarcomas, since the latter situation merits a more aggressive approach. Guidelines for this differentiation are suggested in the discussion following the case reports. Surgery, which is often multiple, remains the mainstay of treatment, although adjuvant chemotherapy and radiotherapy are also usually required.     

Atypical lipomatous tumor/well-differentiated liposarcoma of the extremity and trunk wall: Importance of histological subtype with treatment recommendations

Background This study defines the behavior and classification of atypical lipomatous tumors (ALT) and well-differentiated liposarcomas (WDLS) of the extremity and trunk. Methods A total of 91 well-differentiated lipomatous tumors of the extremity and trunk were identified from a soft tissue tumor database between July 1982 and June 2001. A soft tissue pathologist, blinded to prior diagnosis and clinical outcome, reviewed histology. Those composed predominantly of mature adipose tissue with scattered atypical stromal cells and scant lipoblasts or fibrosis were ALTs. Tumors with lipoblast but <25% fibrosis were termed lipoma-like WDLS, and those with >-25% fibrosis were identified as sclerosing WDLS. Clinical factors were analyzed to assess effects on local recurrence-free survival (LRFS). Results Histological review identified 34 ALTs and 57 WDLSs. Of the WDLSs, 29 were lipoma-like and 28 were sclerosing. Five-year and 10-year LRFS were 100%±0% and 78%±9%, respectively. Factors evaluated were age, sex, tumor site, tumor size, histology, presentation status, margin status, and adjuvant radiotherapy. Positive resection margins and sclerosing histology were associated with reduced LRFS. Dedifferentiation was observed in three tumors (3%) Conclusions Lipoma-like WDLSs and ALTs share similar histological features and favorable behavior. Margin-positive sclerosing WDLSs have a 10-year LRFS of only 17% and should undergo function-preserving re-excision when possible, or adjuvant radiotherapy.     

Retroperitoneal leiomyosarcoma mimicking acute appendicitis: laparoscopic management.

BACKGROUND: Retroperitoneal leiomyosarcomas (RLMS) are a challenging clinical entity. The vast majority of patients are operated on when tumors are advanced. We report herein a case of RLMS, mimicking acute appendiceal disease and treated successfully via laparoscopy. METHODS: A 37-year-old woman, para 1, was admitted to our department for right lower quadrant abdominal pain, fever, and leukocytosis. She had no changes in gastrointestinal and urologic function. A physical examination revealed the presence of abdominal guarding, rebound, and a tender mass in the right lower quadrant. The abdominal ultrasound showed an inhomogeneous ovoid mass (6 cm in diameter) located below the cecum, with no definite margins, and consistent with an appendiceal abscess. The patient was referred for laparoscopy. The procedure was performed with the aid of 3 ports: a 12-mm trocar in the umbilicus (open technique), a 10-mm trocar in the left iliac fossa, and a 5-mm one in the supra-pubic space. On inspection of the abdominal cavity, a retroperitoneal 6-cm mass was immediately found below the cecum and the appendix. Neither intraperitoneal seeding nor suspected lymph nodes were present. After dissection of the parietal peritoneum, the mass appeared to be encapsulated and well demarcated from all surrounding structures. It was eventually dissected and removed via a plastic bag. A standard appendectomy was also performed. The postoperative course was uneventful, and the patient was discharged on the 3rd day. The histology analysis of the resected specimen showed a totally excised G2 leiomyosarcoma. The appendix had no signs of inflammation. Postoperatively, the patient underwent a total-body CT-scan, which had no signs of residual or distant disease. No adjuvant therapy was necessary. At an 18-month follow-up, the patient was doing well and was disease free. CONCLUSION: Surgery represents the main therapeutic option for resectable RLMS. Laparoscopy is a useful diagnostic tool that allows safe resection of incidentally discovered, small and well encapsulated RLMS.     

Retroperitoneal sarcoma: Time for a change in attiude?

Background : Retroperitoneal sarcoma (RPS) is considered a disease with poor prognosis partly because of the difficulty with diagnosis at an early stage. This review assesses the current best practice principles for RPS and finds evidence suggesting a better outlook for appropriately managed cases. Recommendations are made for improving diagnostic certainty before laparotomy and inappropriate transperitoneal biopsy occur. Methods : A critical review of the English language literature was conducted using MEDLINE software and searching the terms ‘retroperitoneal sarcoma’ alone or in combination with ‘prognosis’, ‘surgery’ and ‘adjuvant therapy’. Conclusions : Retroperitoneal sarcoma is a rare disease but when appropriately managed the disease‐free survival can be improved and may even approach that of extremity soft tissue sarcoma. One of the greatest barriers to improving outcome is the misinterpretation of clinical signs and an over‐reliance on ultrasound diagnosis in pelvic presentations, or misinterpretation of clinical signs and/or computer tomography (CT) scans in abdominal masses. Physicians referring patients with a retroperitoneal mass should consider more frequently the less common differential diagnoses of an abdominopelvic mass including retroperitoneal sarcoma. This is especially true in circumstances where there is a circumscribed, predominantly solid tumour, with clinical or radiological signs of vascular or rectal displacement, ureteric obstruction and/or classic renal rotational displacement. The more frequent use of CT scans with intravenous and oral contrast with referral prior to inappropriate transperitoneal biopsy is recommended. In atypical cases where preoperative biopsy is necessary, extraperitoneal routes are preferable. Complete en bloc surgical excision at the first laparotomy is the treatment of choice in RPS. Macroscopic clearance may necessitate resection of adjacent viscera, neurovascular structures or abdominopelvic walls but, if achieved, may lead to long‐term survival depending on individual tumour biology.     

Clinical outcomes of deep atypical lipomas (well-differentiated lipoma-like liposarcomas) of the extremities

AIM: Controversy exists with regard to the nomenclature, treatment and outcome of a group of well-differentiated lipomatous tumours sometimes labelled as atypical lipomas. The purpose of the present paper is to attempt to clarify these controversies by reporting our experience with this lesion. METHODS: The clinical features and follow up of 61 patients with the diagnosis of deep atypical lipoma and a minimum two-year follow up were examined. RESULTS: All patients were treated by marginal excision alone. A local recurrence was seen in five patients (8%). Three recurred once and two recurred twice. No patient had a metastasis or died as a result of the tumour. No lesion dedifferentiated. CONCLUSION: We believe the term atypical lipoma is appropriate for these tumours, as they appear not to have any metastatic potential, merely a propensity to recur locally. The chance of dedifferentiation is small and radiotherapy may play a role in its causation. We suggest that a simple marginal resection (shelling-out) is adequate treatment for these lesions. Radiotherapy should not be used.     

Clinical diagnosis of soft tissue tumors

In the clinical diagnosis of a soft tissue tumor, it is important to determine whether the lesion is malignant as soon as possible. To establish such a diagnosis we perform aspiration cytology and needle biopsy simultaneously with computed radiography (CR) and echosonogram at the first visit. After the first day's investigations, it is usually possible to select the surgical procedures based on the working diagnosis in almost all patients. When the lesion is clearly benign by diagnosis, excision can be performed. For large lesions or lesions that are suspected to be malignant, CT, magnetic resonance imaging , isotope-investigation, and arteriography are also performed, effectively providing precision in an already established diagnosis. These imaging findings are useful for establishing a surgical plan by anticipating the surgical line of the safety margin. For these few patients in whom the diagnosis still remains uncertain or when preoperative treatment is planned, an incisional or excisional biopsy may be necessary to establish the final diagnosis. When preoperative treatment is performed, the effectiveness of this treatment can be evaluated by medical imaging. Received for publication on Nov. 16, 1997