Giant retroperitoneal liposarcoma
© Herrera-Gómez et al; licensee BioMed Central Ltd. 2008
Received: 06 June 2008
Accepted: 31 October 2008
Published: 31 October 2008
Liposarcoma is the most frequent histopathological variety of the retroperitoneum, surgery is the gold standard for treatment.
We present the case of a 24-year-old male who was diagnosed with a giant retroperitoneal liposarcoma. The patient received palliative treatment due to non-resectability on the basis of chemotherapy. We decided to perform surgery after no benefit was received with systemic treatment. Complete macroscopic resection of the tumor was performed, without multi-organ resection. The patient is currently alive and disease free at 14 months of evolution.
Retroperitoneal liposarcomas represent a unique situation and require a more aggressive surgical approach including multiple resections for recurrences. Based on the ability of the patient to tolerate the procedure, surgery is suggested to evaluate resectability of the tumor. We must take into consideration whether prolonged survival will be attained and tumor removal will result in palliation of symptoms.
The most frequent subtypes are liposarcoma (41%), leiomyosarcoma (28%), malignant fibrous histiocytoma (7%), fibrosarcoma (6%) and tumors of the peripheral nerve sheath (3%) . Retroperitoneal liposarcomas alone comprise 0.07–0.2% of all neoplasias4. Approximately 85% of these are malignant, with soft-tissue sarcomas representing 35% of this group. Liposarcoma is the most frequent histopathological variety of the retroperitoneum . It presents with inherent characteristics in relation to its deep localization and slow expansive growth. Average diameter of the tumor is 20–25 cm with a weight of 15–20 kg . There is compromise of the adjacent organs in up to 80% of the cases [4, 5]. Surgery is the gold standard for treatment of liposarcoma. Retroperitoneal liposarcoma is a distinct clinical entity that requires a more aggressive surgical approach, including multiple resections or multiorgan resection with recurrences. There is a low incidence of distance metastasis (7%) compared to other histological subtypes that range from 15 to 34% . The objective of this study is to report a case of giant retroperitoneal sarcoma.
Liposarcoma is the most frequent histological type of retroperitoneal sarcoma, corresponding to 41% of these tumors [4, 5]. It has been reported that 20% of the tumors are > 10 cm at the time of diagnosis ; however, few cases of retroperitoneal liposarcomas exist that can be considered as giant [4, 6–9]. The case we present may be considered among the largest tumors reported for this histology. Clinically, these tumors tend to present with diffuse abdominal pain accompanied by anorexia and weight loss and increase in abdominal girth. The most characteristic sign is a painless abdominal mass detected in ~78% of the cases. Abdominal symptomatology is due to compression of the organs, similar to that reported with the present case [3, 4]. It is clear that our patient presented all the signs and symptoms specific for these tumors due to the size of the abdominal mass.
The tumor was initially considered nonresectable and, therefore, systemic treatment with chemotherapy was decided upon, with the goal of reducing tumor load. Various chemotherapy regimens have been described based on mesna, doxorubicin, ifosfamide, dacarbazine and paclitaxel. However, their use is limited for recurrent metastatic disease or palliation. Survival benefits have not been demonstrated . Due to the large tumor load and probable multiorgan involvement, we initially considered palliative treatment for our patient. Because the patient did not demonstrate any beneficial effects from chemotherapy, the joint decision of the hospital committee, along with the patient, was to perform surgery. Complete resection of the tumor was performed. We are in agreement with Patrik et al.  who demonstrated that in liposarcomas > 10 cm, complete resection can be carried out in up to 70% of cases; however, in up to 50% of these cases, multiorgan resection is necessary in order to reach this goal . The most frequent organ resected is the kidney (30%). In the case we report here, even with the large size of the tumor, organ resection was not necessary, because there was no infiltration to neighboring structures, only lax adhesions allowing adequate dissection of the tumor, as shown in Fig. 3.
Radiotherapy (RT) was not considered in this case for two principal reasons: the first was due to the large tumor load and no demonstrable reduction in tumor size and, second, because of the probable gastrointestinal morbidity associated with such an extensive field as in this case. With regard to RT as complementary treatment, there is agreement for its palliative use in non-operable tumors or in cases of incomplete resection [7, 8]. Although mesodermic tumors are radioresistant, liposarcoma is more radiosensitive . Although it has been noted that RT may increase survival and disease-free interval [3, 7, 8], other authors reported that this treatment has not demonstrated long-term improvement in survival or specific disease in cases of complete macroscopic resections [6, 8]. This occurs despite using intraoperative RT with the goal of increasing efficacy of the local dose with 50–60 Gy  and of minimizing toxicity to adjacent organs. In our case, because we were dealing with a well-differentiated primary liposarcoma that allowed complete resection, 5-year survival of 75–100% [9, 11] has been described. Local failure occurs within 5 to 10 years after resection in up to 90% of the cases related to size of tumor, inability to achieve free margins and limitations of adjuvant treatment such as chemotherapy or RT [3, 4]. We cannot compare these data with our report because our patient is disease free at 14 months of follow-up.
Retroperitoneal liposarcomas are a unique situation and require a more aggressive surgical approach including, when necessary, multiorgan resection or multiple resections with recurrences. In accordance with the ability of the patient to tolerate the procedure, surgery is suggested to evaluate tumor resectability, taking into consideration prolonged survival. After tumor removal, palliation of symptoms will be accomplished.
Written informed consent was obtained from the patient for publication of this case report.
- Kilkenny JW, Bland KI, Copeland EM: Retroperitoneal Sarcoma. The University of Florida Experience. J Am Coll Surg. 1996, 182: 329-39.PubMed
- Hassan I, Park SZ, Donohue JH, Nagorney DM, Kay PA, Nasciemento AG, Schleck CD, Ilstrup DM: Operative management of primary retroperitoneal sarcomas. A reappraisal of an institute experience. Ann Surg. 2004, 239: 244-50. 10.1097/01.sla.0000108670.31446.54.PubMed CentralView ArticlePubMed
- 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: 355-65. 10.1097/00000658-199809000-00008.PubMed CentralView ArticlePubMed
- Echenique-Elizondo M, Amodarain-Arratibel JA: Liposarcoma retroperitoneal gigante. Cir Esp. 2005, 77: 293-5.View ArticlePubMed
- Jaques DP, Coit DG, Hajdu SI, Bennan MF: Management of primary and recurrent soft-tissue sarcoma of the retroperitoneum. Ann Surg. 1990, 212: 51-9.PubMed CentralView ArticlePubMed
- McGrath PC, Neifeld , Lawrence W, DeMay RM, Kay S, Horsley JS, Parker DA: Improved survival following complete excision of retroperitoneal sarcomas. Ann Surg. 1984, 200: 200-4. 10.1097/00000658-198408000-00014.PubMed CentralView ArticlePubMed
- Azpiazu Arnaiz P, Muro Bidaurre I, De Frutos Gomero A, Castro Esnal E, Martin Lopez A, Asesnsio Gallego JI, Rivera Garbayo JR: Tumores retroperitoneales. Liposarcoma mixoide retroperitoneal. Presentación de un nuevo caso. Arch Esp de Urol. 2000, 53: 170-3.
- Romero Pérez P, Rafie Mazketli W, Amat Cecilia M, Merenciano Cortina FJ, Gonzalez Devesa M: Tumores adiposos retroperitoneales. A propósito de un liposarcoma mixoide gigante. Actas Urol Esp. 1996, 20: 79-84.PubMed
- Guzman Martinez-Valls Pl, Ferrero Doria R, López Alba J, Tomas Ros M, Rodenas Moncfada FJ, Rico Galiano JL, Rodríguez de Ledesma Vega JM, Fontana Compiano LO: Liposarcoma retroperitoneal. A propósito de tres casos. Arch Esp de Urol. 1997, 50: 529-31.
- Yoshida Y, Inoue K, Ohsaco T, Nagamoto N, Tanaka E, Tsuruzoe S: Weekly paclitaxel therapy is curative for patients with retroperitoneal liposarcoma. Gan To Kagaku Ryoho. 2007, 34: 465-7.PubMed
- Mehrotra PK, Ramachandran CS, Goel D, Arora V: Inflammatory variant of a well-differentiated retroperitoneal liposarcoma: case report of a rare giant variety. Indian J Cancer. 2006, 43: 36-8.View ArticlePubMed
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