Ramirez Daniel C, Hwang Sinchun, Athanasian Edward A, Wang Lu, Hameed Meera
Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
Department of Pathology and Laboratory Medicine, Hospital For Special Surgery, New York, NY, USA.
Skeletal Radiol. 2018 May;47(5):703-709. doi: 10.1007/s00256-017-2832-1. Epub 2017 Dec 5.
Atypical lipomatous tumor/well-differentiated liposarcoma is the most common sarcoma of soft tissue in adults. We describe the clinical, radiologic, and pathologic features of an atypical lipomatous tumor arising within the soft tissue of the left hand of a 68-year-old female that underwent transformation to dedifferentiated liposarcoma and eventually metastasized. At initial presentation, imaging demonstrated an extensively calcified fatty soft tissue mass with displacement of the digits. Following biopsy and staged debulking, the patient subsequently developed local recurrence, dedifferentiation, and widespread metastases to the lungs, pancreas, bone, and soft tissues. To our knowledge, this is the first case of a cytogenetically proven atypical lipomatous tumor of the hand that has undergone dedifferentiation with widespread metastases.
非典型脂肪瘤性肿瘤/高分化脂肪肉瘤是成人最常见的软组织肉瘤。我们描述了一名68岁女性左手软组织中出现的非典型脂肪瘤性肿瘤的临床、放射学和病理学特征,该肿瘤发生了去分化并最终转移。初次就诊时,影像学显示一个广泛钙化的脂肪性软组织肿块,伴有手指移位。活检及分期减瘤术后,患者随后出现局部复发、去分化,并广泛转移至肺、胰腺、骨和软组织。据我们所知,这是首例经细胞遗传学证实的手部非典型脂肪瘤性肿瘤发生去分化并广泛转移的病例。