Giant retroperitoneal liposarcoma extending into the scrotum: a case report

V Genç A Çakmak C Akyol, S Baskan, SM. Hazinedaroglu

Ankara University School of Medicine, Department of Surgery, Ankara, Turkey.

Corresponding Address:

Selcuk M. Hazinedaroglu, Altay Mah. 97. Sokak İrem Sitesi , No: 2M-10 Eryaman Ankara Turkey 

e-mail:selcukhazinedaroglu@yahoo.com

SMJ 2009 54(2): 

 

Abstract

Liposarcomas are the most common soft-tissue sarcomas in adults. Soft tissue sarcomas can be seen almost every localisation in the body, 10-15% of these are located in the retroperitoneum. Due to their slow growth and lack of specific anatomical compartments, retroperitoneal liposarcomas frequently reach an enormous size long before diagnosis. We describe a case of giant retroperitoneal liposarcoma successfully treated by surgical resection. The interest of our report is that the dimension alone should not be considered a contraindication for surgery and to remind the effectiveness of the first operation is the most important part of the therapy.

Key Words: Retroperitoneum, Soft-tissue sarcoma, Surgery

 

Introduction

Soft tissue sarcomas predominantly arise from embryonic mesoderm with a tremendous diversity of histopathologic characteristics. Sarcomas account for 0.8% of all new diagnosed malignancies. Soft tissue sarcomas can be seen almost every localisation in the body, but 10-15% of are located in the retroperitoneum.1 Liposarcomas are the most common soft-tissue sarcomas in adults, with accounting for 20% of all sarcomas.2 We report a case of giant retroperitoneal liposarcoma treated successfully by surgical resection.

 

Case-Report

A 60-year-old male presented with abdominal distention. He had umbilical and scrotal masses and also reported a vague sensation of lower abdominal pain, dyspnea, weight gain that became gradually apparent with in the last 12 months. He had no fever and no gastrointestinal symptoms such as nausea, vomiting or change in bowel habits. Six-months previously, he had received an operation for a giant left inguinal hernia six months ago. The lesion was found to be an adipose mass lesion and left untouched by the surgeon.

On examination, he was found to have overt abdominal distention and irreducible umbilical and scrotal masses. A full blood count and blood chemistry were within normal limits. Computerised tomographic and ultrasonographic examination revealed a giant retroperitoneal solid tumour extending into the scrotum. Intraperitoneal organs were compressed to the right side of the abdomen, the left kidney was surrounded by the tumoural mass and there was no evidence of liver or lung metastasis (figure 1).

 

Figure 1: The Computerised tomographic appearance of a giant retroperitoneal solid tumour  pre-operatively.

            

He underwent a midline laparotomy and a 65x40x30 cm; 30 kg retroperitoneal tumour was completely resected together with the anterior capsule of the left kidney (figure 2). Scrotal and umbilical extensions of the tumour were resected from the same incision and the defects were repaired. The postoperative period was unremarkable. Examination of the tumour revealed a well-differentiated liposarcoma with a microscopically negative margin. The patient was discharged on the 10th postoperative day without chemotherapy or radiotherapy. There was no evidence of local recurrence or distant metastasis at the fourth postoperative year neither clinically nor with tomography (figure 3).

 

Figure 2: Totally excised giant lipomatous tumour.

 

Figure 3:  The Computerised tomographic appearance of a giant retroperitoneal solid tumour 2 years after operation.

Discussion

Due to their relative rarity and slow growth there is often a delay in the diagnosis of retroperitoneal soft tissue sarcomas. As such, it is essential that these sarcomas are known by physicians since clinical outcomes might be challenging if the initial management is not optimum.

Liposarcoma is the most common histopathological type of retroperitoneal soft tissue sarcoma.  Retroperitoneal liposarcoma is principally seen in adults, with peak incidence occurring between the ages of 40-60 years.1 

 

Pre-disposing factors such as genetic, environmental (radiation, chemicals, chronic irritation) and iatrogenic (lymphedema after mastectomy, immunosupression after transplantation) have been well documented in the literature. However, the causes of soft tissue sarcomas remain little understood.3 

 

The diagnosis is usually the incidental result of finding a soft tissue mass in the retroperitoneum on abdominal computed tomography. There is no adequate staging system for retroperitoneal liposarcomas. In the retroperitoneum, CT is usually sufficient, unless midline vascular structures are not involved. In such cases magnetic resonance imaging may better delineate the relationship of the tumour with the midline vasculature. Fine needle aspiration biopsy has a limited role and needle biopsy is indicated if abdominal lymphoma is strongly suspected as a part of differential diagnosis.

Due to their slow growth, retroperitoneal liposarcomas frequently reach an enormous size long before diagnosis. However to our best knowledge this is the third largest case reported in the English literature.2,4 

 

The liver is the primary site for metastasis and together with lung and as such, should be carefully screened especially in large tumours cases.3

All histopathologically distinct subtypes of liposarcoma namely, well-differentiated (WD), myxoid, pleomorphic and dedifferentiated (DD) can be seen in their pure forms or in combination within the retroperitoneum. Histological grade is the most important factor affecting survival of patients with liposarcomas.5

Soft tissue sarcomas are often treated with surgical resection. With retroperitoneal liposarcomas, surgical resection remains the only choice of treatment. The absence of specific anatomic boundaries in the retroperitoneum makes the determination of negative microscopic margins difficult. Studies have shown that complete resection may increase overall 5-year survival to 58% from 16.7%.6 However, despite resections with curative intent, local recurrence is common. Surgeons have to decide a surgical strategy with an acceptable risk of postoperative complications to obtain a clear margin resection. It is recommended that surgeons should be in close collaboration with radiology and pathology departments. This collaboration is very important not only for preoperative evaluation but also postoperative follow up. 

Although radiotherapy is effective especially in extremity lesions, due to the adjacent vulnerable structures, such as intestine, and the local extensions of the tumour, it is invaluable in the retroperitoneum. Chemotherapy is ineffective and still investigational.7 Nevertheless complete surgical resection is reported to be possible only in a subgroup of cases and usually necessitates the removal of adjacent organs.8 In our case, we were able to excise this giant liposarcoma without resection of any organ except the anterior capsule of the kidney.

 

As locally recurrent disease occurs frequently and is the most common cause of death in patients after resection of retroperitoneal soft tissue sarcomas, it is recommended that patients have an abdominal tomography every 6 months postoperatively.1 Mussi et al. detected the local recurrence free survival rate was 31.2% at 5 years and the median time to local progression was 29 months in a series of 148 patients. Nevertheless they determined the presence of a DD component was the main independent prognostic factor for local recurrence.9 

 

Conclusion

The success of the first surgical resection is the most important part of the treatment for retroperitoneal liposarcoma. This case represents this success and the patient remains disease-free three years after operation. 

 

References

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  2. McCallum OJ, Burke JJ 2nd, Childs AJ, Ferro A, Gallup DG. Retroperitoneal liposarcoma weighing over one hundred pounds with review of the literature. Gynecol Oncol. 2006;103(3):1152-4.

  3.  Savarese DMF, DeLaney TF.Overview of soft tissue sarcomas. In: Rose BD, editor. UpToDate. Wellesley, MA; 2006.

  4. Yol S, Tavli S, Tavli L, Belviranli M, Yosunkaya A. Retroperitoneal and scrotal giant liposarcoma: report of a case. Surg Today. 1998;28(3):339-42.

  5. Nijhuis PH, Sars PR, Plaat BE, Molenaar WM, Sluiter WJ, Hoekstra HJ. Clinico-pathological data and prognostic factors in completely resected AJCC stage I-III liposarcomas. Ann Surg Oncol. 2000;7(7):535-43.

  6. Bradley JC, Caplan R. Giant retroperitoneal sarcoma: a case report and review of the management of retroperitoneal sarcomas. Am Surg 2002; 68(1):52–6.

  7. Mendenhall WM, Zlotecki RA, Hochwald SN, Hemming AW, Grobmyer SR, Cance WG.  Retroperitoneal soft tissue sarcoma. Cancer. 2005;104(4):669-75.

  8. Lewis JJ, Leung D, Woodruff JM, Brennan MF. Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution. Ann Surg. 1998; 228(3):355-65.

  9. Mussi C,  Collini P, Miceli R, Barisella M, Mariani L, Fiore M, Casali PG, Gronchi A. The prognostic impact of dedifferentiation in retroperitoneal liposarcoma: a series of surgically treated patients at a single institution. Cancer. 2008;113(7):1657-65.

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