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 Table of Contents  
REVIEW ARTICLE: REPUBLICATION
Year : 2017  |  Volume : 3  |  Issue : 3  |  Page : 44-46

Retroperitoneal sarcomas


OPUS 12 Foundation, Bethlehem, PA, USA

Date of Web Publication21-Apr-2017

Correspondence Address:
Stanislaw P Stawicki
Department of Research and Innovation, St. Luke's University Health Network, 801 Ostrum Street, Bethlehem, Pennsylvania 18015
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJAM.IJAM_93_16

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  Abstract 


Retroperitoneal sarcomas (RS) are malignant tumors arising from mesenchymal cells, which are usually located in adipose, muscle, or connective tissue. RSs represent approximately 15% of all sarcomas and approximately 33–55% of all retroperitoneal tumors. The most common variants of RS include liposarcoma (40%), leiomyosarcoma (30%), and malignant fibrous histiocytoma (<10%). This article reviews the most important clinical characteristics of RSs, focusing on high-yield facts frequently encountered on standardized exams.
The following core competencies are addressed in this article: Medical knowledge, Patient care.
Republished with permission from: Stawicki SP. Retroperitoneal sarcomas. OPUS 12 Scientist 2007;1(1):17.

Keywords: In-Training Exam, review, retroperitoneal sarcomas


How to cite this article:
Stawicki SP. Retroperitoneal sarcomas. Int J Acad Med 2017;3, Suppl S1:44-6

How to cite this URL:
Stawicki SP. Retroperitoneal sarcomas. Int J Acad Med [serial online] 2017 [cited 2017 Oct 23];3, Suppl S1:44-6. Available from: http://www.ijam-web.org/text.asp?2017/3/3/44/204974




  Introduction Top


Retroperitoneal sarcomas (RSs) represent approximately 15% of all sarcomas and approximately 33–55% of all retroperitoneal tumors. The most common variants of RS include liposarcoma (40%), leiomyosarcoma (30%), and malignant fibrous histiocytoma (<10%).[1],[2]

RSs are malignant tumors arising from mesenchymal cells, which are usually located in adipose, muscle, or connective tissue. Median patient age is approximately 50 years although patients of any age can be affected. Incidence is equal among men and women. RSs metastasize through hematogenous route, typically to the liver or lung.[3],[1],[2],[4],[5]

The most common clinical presentation is that of an abdominal mass. This is due to the fact that RSs smaller than 5 cm usually do not cause clinically significant complaints. Other symptoms and signs include nonspecific, vague abdominal discomfort or pain, weight loss, and early satiety. Some cases of RSs may present with intestinal obstruction or bleeding. Lower extremity swelling or pain has also been reported as the presenting symptom.[6],[7],[8]

Computed tomography (CT) is the initial test of choice in the evaluation of suspected RS [Figure 1]. CT facilitates accurate estimation of tumor location and size, its relationship to surrounding anatomic structures, and identification of metastatic lesions within the abdomen. Tumor characterization (i.e., fatty tumor) may also be possible using the CT imaging.[1],[4],[5],[8]
Figure 1: Computed tomographic appearance of retroperitoneal liposarcoma. Adapted from Radiology Picture of the Day (http://www.radpod.org/)

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Magnetic resonance imaging (MRI) can also provide useful information with regard to the tumor location, involvement of surrounding structures, and the presence of metastatic disease. The importance of axial MR images in addition to sagittal and coronal views has been emphasized. Large-scale comparisons of CT and MRI techniques in the setting of RS are scarce.[1],[4],[5],[8]

Following identification of a retroperitoneal tumor, a broad list of differential diagnoses should be considered. This list includes germ cell tumor, teratoma, cyst, hematoma and abscess, functioning and nonfunctioning adrenal tumor, renal tumor, pancreatic tumor, advanced gastrointestinal carcinoma, soft tissue sarcoma, and other neoplasms. Further workup should include detailed physical examination (including testicular examination and ultrasound) and measurement of serum beta human chorionic gonadotropin and alpha-fetoprotein if testicular mass is suspected or found. In cases of lymphadenopathy, core needle or excisional-node biopsy may show a lymphoma. For suspected gastrointestinal tumors, appropriate contrast studies and endoscopy may be helpful. Once diagnostic list is “narrowed down” to RS, the role of biopsy is controversial. Biopsy may be best performed at the beginning of definitive resection for the purpose of diagnostic confirmation.[1],[4],[5],[8],[9]

Surgery is the standard treatment for RS. While complete margin-negative resection may be difficult to achieve, it is the only hope for cure. Difficult surgical management is related largely to the late presentation of RSs, their large size, and the complexities of retroperitoneal anatomy. Surgical failures are related to the large tumor size on presentation, the inability to achieve wide surgical margins, and the limitations of existing adjuvant radiation and chemotherapy regimens.[3],[6],[7] Local failure is seen in nearly 90% of patients who die of RS and can be seen even 5–10 years after resection. The overall long-term recurrence rate for RS is thought to be over 70%. In general, the prognosis is worse for RS than for other trunk or extremity sarcomas.[2],[4],[7],[10],[11]

No significant survival benefit has been seen with incomplete resection, which is further complicated by >30% incidence of vascular involvement of these lesions, as well as the requirement for multivisceral resections (colon, kidney, small bowel, pancreas, bladder) in over 60% of cases. This accentuates the need for very careful preoperative planning to prevent suboptimal surgical results and potentially serious operative complications.[1],[2],[4],[6],[7],[5],[10],[11],[9]

Retroperitoneal liposarcomas may represent an exception to this rule, with reports of multiple and incomplete resections for repeated recurrences. Some also report effective symptom palliation and prolonged survival following partial resections of liposarcomas as compared to those who only underwent biopsy. Retroperitoneal liposarcomas have lower incidence of distant metastases (<10%) as compared to other RS subtypes (15–34%).[1],[2],[4],[6],[7],[5],[10],[11],[9]

Neoadjuvant chemotherapy may be used in an attempt to “shrink” the tumor. Postoperative radiotherapy may be of benefit. However, based on limited clinical evidence, it should only be conducted in the setting of a clinical trial. Adjuvant chemotherapy, when added to surgical therapy, may produce a modest improvement in the recurrence-free survival rate (approximately 10%). Unfortunately, the experience with both radio- and chemo-therapy in the setting of RS continues to be limited. Toxicities of both chemo- and radio-therapy have to be considered carefully before proceeding with these options.[2],[4],[7]

Local RS recurrences can be treated with resection in the absence of metastatic disease. Surgical treatment of local recurrences has been reported to produce disease-free survival when all grossly evident recurrent diseases can be removed. Not unexpectedly, subsequent recurrences have progressively lower chances of successful resection. The role of chemo- and radio-therapy in the setting of local recurrence is still controversial. In general, adjuvant treatment following re-resection should be considered for all patients who did not previously undergo chemotherapy.[1],[2],[4],[6],[5],[8],[10],[9]

Metastatic workup for RS includes plain film or CT evaluation of the thorax. Compared to patients with extremity sarcomas, those with RS have greater propensity for local recurrence and disseminated abdominal disease. Approximately 80% of recurrences are seen within 5 years of initial treatment. Treatment of pulmonary metastases is extrapolated largely from experience with sarcomas in general. Pulmonary metastases are associated with a median survival of 6–12 months. However, some reports indicate that resection of often multiple pulmonary metastases may associated with prolonged survival in 25–39% of patients. Here, the prognosis seems to be related to complete resection of all lesions, a disease-free interval of more than 12 months, or a low-grade primary tumor. Four of fewer metastatic lesions may be considered a positive prognostic factor. Negative prognostic factors include a greater number of metastatic lesions and rapid tumor doubling times. For hepatic metastases, survival rates following resection have been less than those seen after resection of pulmonary metastases. Although recurrence rates are very high for patients with hepatic metastases, an increase in median survival from approximately 11 months to about 30 months has been reported following resection.[1],[2],[4],[6],[5],[8],[10],[9]

For unresectable metastatic disease, chemotherapy may provide some clinical efficacy. Doxorubicin, ifosfamide, and dacarbazine have been demonstrated to have significant activity in the setting of metastatic soft tissue sarcomas. Reports also indicate that combinations of agents may be more effective than single-agent chemotherapy. Appropriate clinical trials should be considered for all patients with advanced and/or unresectable disease.[3],[6]

Postoperative surveillance for RS, according to the NCCN guidelines, is based on low- versus high-grade tumor categories. Patients with low-grade disease should undergo physical examination with CT scan of chest, abdomen, and pelvis for every 3–6 months for 2–3 years, and then annually. For patients with high-grade disease, physical examination and CT scan of chest, abdomen, and pelvis should be performed for every 3–4 months for 3 years, then for every 6 months for 2 years, and then annually.[8]

Acknowledgement

Justifications for re-publishing this scholarly content include: (a) The phasing out of the original publication after a formal merger of OPUS 12 Scientist with the International Journal of Academic Medicine and (b) Wider dissemination of the research outcome(s) and the associated scientific knowledge.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Brennan MF, Lewis JJ, editors. Diagnosis and Management of Soft Tissue Sarcoma. London: Martin Dunitz, Florence, Ky, Distributed in the USA by Taylor & Francis; 2002.  Back to cited text no. 1
    
2.
Catton CN, O'Sullivan B, Kotwall C, Cummings B, Hao Y, Fornasier V. Outcome and prognosis in retroperitoneal soft tissue sarcoma. Int J Radiat Oncol Biol Phys 1994;29:1005-10.  Back to cited text no. 2
    
3.
Antman KH, Ryan L, Elias A, Sherman D, Grier HE. Response to ifosfamide and mesna: 124 previously treated patients with metastatic or unresectable sarcoma. J Clin Oncol 1989;7:126-31.  Back to cited text no. 3
    
4.
Glenn J, Sindelar WF, Kinsella T, Glatstein E, Tepper J, Costa J, et al. Results of multimodality therapy of resectable soft-tissue sarcomas of the retroperitoneum. Surgery 1985;97:316-25.  Back to cited text no. 4
    
5.
Moley JF, Eberlein TJ. Soft-tissue sarcomas. Surg Clin North Am 2000;80:687-708.  Back to cited text no. 5
    
6.
Hensley ML, Maki R, Venkatraman E, Geller G, Lovegren M, Aghajanian C, et al. Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: Results of a phase II trial. J Clin Oncol 2002;20:2824-31.  Back to cited text no. 6
    
7.
Jones JJ, Catton CN, O'Sullivan B, Couture J, Heisler RL, Kandel RA, et al. Initial results of a trial of preoperative external-beam radiation therapy and postoperative brachytherapy for retroperitoneal sarcoma. Ann Surg Oncol 2002;9:346-54.  Back to cited text no. 7
    
8.
NCCN Clinical Practice Guidelines in Oncology. Available from: http://www.nccn.org/professionals/physician_gls/PDF/sarcoma.pdf. [Last accessed 2007 Sep 30].  Back to cited text no. 8
    
9.
Windham TC, Pisters PW. Retroperitoneal sarcomas. Cancer Control 2005;12:36-43.  Back to cited text no. 9
    
10.
Spira AI, Ettinger DS. The use of chemotherapy in soft-tissue sarcomas. Oncologist 2002;7:348-59.  Back to cited text no. 10
    
11.
van Oosterom AT, Mouridsen HT, Nielsen OS, Dombernowsky P, Krzemieniecki K, Judson I, et al. Results of randomised studies of the EORTC Soft Tissue and Bone Sarcoma Group (STBSG) with two different ifosfamide regimens in first- and second-line chemotherapy in advanced soft tissue sarcoma patients. Eur J Cancer 2002;38:2397-406.  Back to cited text no. 11
    


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