Recurrent giant retroperitoneal liposarcoma: Review of literature and a rare case report

复发性巨大腹膜后脂肪肉瘤:文献回顾及一例罕见病例报告

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Abstract

INTRODUCTION AND IMPORTANCE: Retroperitoneal liposarcomas (RPLS) are usually grow large with frequent recurrences. Complete surgical excision remains the gold standard treatment for primary and even recurrent tumours. Their prognosis depends on their histological type and grade. We report a recurrent giant de-differentiated RPLS weighing 18.55 kg which was completely excised. To the best of our knowledge, this is one of the largest liposarcoma reported in the literature. CASE PRESENTATION: A 40 year old female presented with a gradually progressing large abdominal lump for 1year. She had had a similar large lump twice in the past and undergone excision of the tumour elsewhere. Firm non-tender mass felt all over abdomen with edema noted over abdominal wall and bilateral lower limbs. PET CT showed large heterogeneously enhancing mass occupying almost the entire abdominopelvic cavity. 50 × 40 × 40cm tumour was completely excised and biopsy showed grade 2 dedifferentiated liposarcoma (DDLS). She is under close follow up with no recurrence at 12months. CLINICAL DISCUSSION: DDLS have lower risk of distant metastases but have a high risk of local recurrence. The most important favourable prognostic factor in these tumours is complete resection with negative margins. Because of the ineffectiveness of current chemotherapy and the requirement of intolerably high radiation doses, surgical excision remains the most effective treatment even for the localized recurrences of RPLS. CONCLUSION: The dedifferentiated subtype should be suspected in locally aggressive RPLS. Close follow up with early detection of recurrences and prompt excision with negative margins lowers the risk of recurrences and improves survival.

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