Primary Dedifferentiated Liposarcoma of the Colon - Report of a Rare Case with Review of Literature

R. Sukumaran, Preethy T Ramadas, S. Raveendran
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Abstract

Liposarcoma is the most common malignant soft tissue tumor in adults. Extremities and retroperitoneum are the common sites of involvement of liposarcoma. Liposarcoma of the gastrointestinal tract is rare and the colon is an extremely uncommon site. Mesenchymal tumors of the bowel are mainly represented by gastrointestinal stromal tumors (GIST). A sixty-one-year-old male patient presented with abdominal pain and distension. Contrast-enhanced computerized tomography (CECT) showed a circumferentially proliferative mass lesion in the mid ascending colon causing acute colonic obstruction with multiple large enhancing exophytic mass lesions of varying sizes on the peritoneal surface of the intestine. A right hemicolectomy was done. Gross examination showed polypoidal growth of 3.5x3x1.5 cm in the luminal aspect of ascending colon and a large exophytic firm mass in the transverse colon of 15x5.5x5 cm. There were multiple smaller exophytic nodules on the serosal aspect of the intestine. Microscopy showed neoplasm composed of atypical spindle cells in ill-defined fascicles and whorls. Foci showing aggregates of adipocytes of varying sizes with occasional lipoblasts were also noted. The spindly cells were Ckit negative, DOG 1 negative, and showed positivity for SMA. Based on morphology and immunoprofile, a diagnosis of dedifferentiated liposarcoma was given. This case represents dedifferentiated liposarcoma presenting as single endophytic and multiple exophytic masses and highlights the fact that, although rare, dedifferentiated liposarcoma can present as multiple intestinal mass lesions.
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结肠原发性去分化脂肪肉瘤1例报告并文献复习
脂肪肉瘤是成人最常见的软组织恶性肿瘤。四肢和腹膜后是脂肪肉瘤的常见受累部位。发生在胃肠道的脂肪肉瘤是罕见的,而发生在结肠的脂肪肉瘤更是罕见。肠间质肿瘤主要以胃肠道间质肿瘤(GIST)为代表。一名六十一岁男性病人以腹痛及腹胀为主诉。造影增强ct (CECT)显示升结肠中部呈环状增殖性肿块病变,引起急性结肠梗阻,并在肠腹膜表面出现多个大小不等的大增强外生肿块病变。行右半结肠切除术。大体检查:升结肠管腔面息肉样生长3.5x3x1.5 cm,横结肠外生硬块大,15x5.5x5 cm。肠浆膜面有多个较小的外生结节。镜检显示肿瘤由非典型梭形细胞组成,呈不明确的束状和螺旋状。病灶显示大小不一的脂肪细胞聚集,偶见成脂细胞。细长细胞Ckit阴性,dog1阴性,SMA阳性。根据形态学和免疫图谱,诊断为去分化脂肪肉瘤。本病例为去分化脂肪肉瘤,表现为单个内生和多个外生性肿块,并强调了尽管罕见,但去分化脂肪肉瘤可表现为多个肠道肿块病变的事实。
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