Mremi Alex, Macheku Godwin Silas, Mondea Adam Pastory, Sadiq Adnan, Mesarieki Lobulu Vincent
Faculty of Medicine, Kilimanjaro Christian Medical University College, Moshi, Tanzania; Department of Pathology, Kilimanjaro Christian Medical Centre, Moshi, Tanzania; Kilimanjaro Clinical Research Institute, Moshi, Tanzania.
Department of Obstetrics and Gynecology, Mawenzi Regional Referral Hospital, Moshi, Tanzania.
Int J Surg Case Rep. 2024 Dec;125:110629. doi: 10.1016/j.ijscr.2024.110629. Epub 2024 Nov 19.
Atypical lipomatous tumor/well differentiated liposarcoma (ATL/WDL) is an intermediate, locally aggressive malignant mesenchymal neoplasm composed either entirely or in part of a mature adipocytic proliferation showing significant variation in cell size and at least focal nuclear atypia in both adipocytes and stromal cells. Symptoms related to these tumors depend on the anatomic site.
A 61-year-old female presented with a long-standing worsening abdominal distension. Her vital signs and blood workup tests were within normal ranges. CT scans revealed a gigantic soft tissue mass occupying the entire abdominal cavity, displacing visceral organs. An en block surgical resection was attempted. Histopathology report confirmed the diagnosis of ALT/WDL, incompletely excised. Postoperative period was uneventful. To date, a year of close follow-up has passed; she remains disease-free.
The most important prognostic factor includes anatomic location and tumor size. These tumors do not metastasize unless they dedifferentiate, which is associated with significantly shorter overall survival. Subcutaneous or intramuscular tumors may recur but typically do not dedifferentiate or metastasize. The risk of dedifferentiation is directly related to location and duration of growth. Wide local excision with negative margins is curative. The efficacy and safety of further therapeutic choices, such as chemotherapy and radiotherapy, are still controversial.
As clinicians, it is important that we have a thorough understanding of the clinico-pathology, diagnosis, treatment, and prognosis of this tumor. Regular follow-up is important after treatment due to the risk of recurrence.
非典型脂肪瘤性肿瘤/高分化脂肪肉瘤（ATL/WDL）是一种中间型、局部侵袭性恶性间叶性肿瘤，完全或部分由成熟脂肪细胞增殖构成，脂肪细胞大小差异显著，脂肪细胞和间质细胞均至少有局灶性核异型性。这些肿瘤相关症状取决于解剖部位。
一名61岁女性因长期腹胀加重前来就诊。她的生命体征和血液检查结果均在正常范围内。CT扫描显示一个巨大的软组织肿块占据整个腹腔，推移了内脏器官。尝试进行整块手术切除。组织病理学报告确诊为ALT/WDL，切除不完全。术后恢复顺利。迄今为止，已过去一年的密切随访时间；她仍无疾病复发。
最重要的预后因素包括解剖位置和肿瘤大小。这些肿瘤除非去分化，否则不会发生转移，而去分化与总体生存期显著缩短相关。皮下或肌内肿瘤可能复发，但通常不会去分化或转移。去分化风险与生长部位和持续时间直接相关。切缘阴性的广泛局部切除可治愈。化疗和放疗等进一步治疗选择的疗效和安全性仍存在争议。
作为临床医生，我们全面了解这种肿瘤的临床病理、诊断、治疗和预后非常重要。由于存在复发风险，治疗后定期随访很重要。
