Downes K A, Goldblum J R, Montgomery E A, Fisher C
Department of Anatomic Pathology, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA.
Mod Pathol. 2001 Mar;14(3):179-84. doi: 10.1038/modpathol.3880280.
Pleomorphic liposarcoma is a variant of liposarcoma defined morphologically by the presence of pleomorphic lipoblasts. Because of its rarity, there are limited studies with long-term follow-up information. Nineteen pleomorphic liposarcomas were studied. Unequivocal pleomorphic lipoblasts were required for inclusion. In each case, the following features were noted: tumor site; tumor size; tumor depth; predominant histologic pattern (epithelioid or malignant fibrous histiocytoma [MFH]-like); extent of necrosis (absent, less than 15%, or at least 15%); mitotic counts; treatment and clinical follow-up. Patients were 11 females and 8 males, aged 33-87 years (mean, 64.5 y; median, 70 y). Tumors involved the extremities (13 patients: intramuscular in 10, subcutaneous in 2, depth unknown in 1), retroperitoneum (4 patients), mediastinum (1 patient), and paratesticular region (1 patient). Size ranged from 4.5--31 cm (mean, 11.9 cm; median, 12.0 cm). Predominant pattern was epithelioid in 7 and MFH-like in 12. Necrosis was present in 15 (79%) and was extensive (36 15%) in 14 patients. Mitotic counts ranged from 0.2--3.4/10 high-power fields (mean, 1.4; median, 1.4) by the average-count method and from 1--6/10 high power fields by the highest count method (mean, 2.9; median, 3.0). All patients were treated surgically; 10 patients received adjuvant chemotherapy and/or radiation therapy. On follow-up of 18 patients (range, 2--129 mo; mean, 35.4 mo; median, 23 mo) nine (50%) were dead of disease (range, 2--48 mo; mean, 20.1 mo; median, 12 mo), one died of other causes 2 months after diagnosis, two were alive with disease, five were disease free, and one was alive at 129 months (tumor status unknown). Five had recurrences (range, 3--28 mo; mean, 14.4 mo; median, 8 mo), and four of five (80%) with recurrences were dead of disease. Metastases developed in eight patients (range, 4--48 mo; mean, 19.5 mo; median, 11.5 mo), most commonly to the lungs. In conclusion, pleomorphic liposarcoma is a rare tumor of adulthood that occurs most commonly in the deep, soft tissues of the extremities. It behaves as a high-grade sarcoma that frequently metastasizes, most commonly to the lungs. Although this tumor has a wide range of histologic appearances, no clinical or pathologic feature is predictive of a more aggressive clinical course.
多形性脂肪肉瘤是脂肪肉瘤的一种变体，在形态学上由多形性脂肪母细胞的存在来定义。由于其罕见性，长期随访信息的研究有限。对19例多形性脂肪肉瘤进行了研究。纳入标准为明确存在多形性脂肪母细胞。在每例病例中，记录了以下特征：肿瘤部位；肿瘤大小；肿瘤深度；主要组织学模式（上皮样或恶性纤维组织细胞瘤[MFH]样）；坏死程度（无、小于15%或至少15%）；有丝分裂计数；治疗及临床随访情况。患者中女性11例，男性8例，年龄33 - 87岁（平均64.5岁；中位数70岁）。肿瘤累及四肢（13例患者：10例位于肌肉内，2例位于皮下，1例深度不明）、腹膜后（4例患者）、纵隔（1例患者）和睾丸旁区域（1例患者）。大小范围为4.5 - 31 cm（平均11.9 cm；中位数12.0 cm）。主要模式为上皮样的有7例，MFH样的有12例。15例（79%）存在坏死，14例患者坏死广泛（≥15%）。通过平均计数法，有丝分裂计数范围为0.2 - 3.4/10高倍视野（平均1.4；中位数1.4），通过最高计数法为1 - 6/10高倍视野（平均2.9；中位数3.0）。所有患者均接受了手术治疗；10例患者接受了辅助化疗和/或放疗。对18例患者进行随访（范围2 - 129个月；平均35.4个月；中位数23个月），9例（50%）死于疾病（范围2 - 48个月；平均20.1个月；中位数12个月），1例在诊断后2个月死于其他原因，2例带瘤生存，5例无病生存，1例在129个月时存活（肿瘤状态不明）。5例出现复发（范围3 - 28个月；平均14.4个月；中位数8个月），5例中有4例（80%）复发患者死于疾病。8例患者发生转移（范围4 - 48个月；平均19.5个月；中位数11.5个月），最常见转移至肺部。总之，多形性脂肪肉瘤是一种成人罕见肿瘤，最常发生于四肢深部软组织。它表现为高级别肉瘤，常发生转移，最常见转移至肺部。尽管该肿瘤具有广泛的组织学表现，但没有临床或病理特征可预测更具侵袭性的临床病程。
