Kanthala Lalith, Ray Samrat, Aurobindo Prasad Das Sri, Nundy S, Mehta N
Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi, 110060, India.
Ann Med Surg (Lond). 2021 Apr 17;65:102329. doi: 10.1016/j.amsu.2021.102329. eCollection 2021 May.
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.
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.
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.
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.
腹膜后脂肪肉瘤(RPLS)通常生长较大且易复发。完整手术切除仍是原发性甚至复发性肿瘤的金标准治疗方法。其预后取决于组织学类型和分级。我们报告一例重达18.55千克的复发性巨大去分化RPLS,已被完整切除。据我们所知,这是文献中报道的最大脂肪肉瘤之一。
一名40岁女性,腹部逐渐增大的肿块持续1年。她过去曾两次出现类似的大肿块,并在其他地方接受过肿瘤切除。全腹可触及质地硬、无压痛的肿块,腹壁及双侧下肢有水肿。PET CT显示巨大的不均匀强化肿块几乎占据整个腹腔盆腔。一个50×40×40cm的肿瘤被完整切除,活检显示为2级去分化脂肪肉瘤(DDLS)。她正在密切随访中,12个月时无复发。
DDLS远处转移风险较低,但局部复发风险较高。这些肿瘤最重要的有利预后因素是切缘阴性的完整切除。由于目前化疗无效且所需放疗剂量高得难以耐受,即使对于RPLS的局部复发,手术切除仍是最有效的治疗方法。
对于局部侵袭性RPLS应怀疑去分化亚型。密切随访、早期发现复发并及时进行切缘阴性的切除可降低复发风险并提高生存率。