Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH, USA.
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
Hum Pathol. 2023 Dec;142:90-95. doi: 10.1016/j.humpath.2023.09.005. Epub 2023 Sep 22.
Myxofibrosarcoma is a locally aggressive sarcoma that characteristically arises in the extremities of older patients. Cases arising at a younger age are rare, leading to diagnostic challenges. Our aim was to study the clinicopathologic features of myxofibrosarcoma in patients aged ≤40 years. Cases of myxofibrosarcoma and myxoid malignant fibrous histiocytoma arising in patients aged ≤40 years with clinical follow-up were collected from multiple institutions. Hematoxylin and eosin slides were evaluated for mitoses, necrosis, and epithelioid areas. Seventeen cases were identified (13 females, 4 males; 16-39 years; median 32 years), tumors ranged from 2.2 to 34 cm (median 4.1 cm). Anatomic sites included proximal extremity (9), distal extremity (4), trunk (1), and head/neck (3). Ten were superficial, and 6 were deep-seated. Three cases were predominantly epithelioid. In untreated resection specimens, 6 were FNCLCC grade 1, 4 grade 2, and 2 grade 3. Follow-up (6-204 months, median 36 months) revealed that 2 patients experienced local recurrences, 1 distant metastasis, and 2 patients both. The 5-year overall survival (OS) and event-free survival (EFS) were 84% and 55.9%, respectively. Tumor depth and necrosis were correlated with inferior OS (P = .025, P = .005), while tumor depth was also associated with worse EFS (P = <.001). We conclude that myxofibrosarcomas arising in adolescents and young adults show similar behavior compared to their older adult counterparts. Tumor depth and necrosis are poor prognostic factors in myxofibrosarcoma in this age group. Awareness that myxofibrosarcoma can rarely present in this population is important for accurate diagnosis.
黏液纤维肉瘤是一种局部侵袭性肉瘤，特征性地发生于老年患者的四肢。年龄较小的患者中罕见发病，导致诊断具有挑战性。我们的目的是研究≤40 岁患者中黏液纤维肉瘤的临床病理特征。从多个机构收集了≤40 岁患者中发生的黏液纤维肉瘤和黏液样恶性纤维组织细胞瘤的病例，这些患者具有临床随访资料。评估苏木精和伊红染色切片的有丝分裂、坏死和上皮样区域。共确定了 17 例（13 名女性，4 名男性；年龄 16-39 岁；中位年龄 32 岁），肿瘤大小为 2.2-34cm（中位 4.1cm）。解剖部位包括近端肢体（9 例）、远端肢体（4 例）、躯干（1 例）和头颈部（3 例）。10 例为表浅性，6 例为深部。3 例主要为上皮样。在未经治疗的切除标本中，6 例为 FNCLCC 分级 1，4 例为分级 2，2 例为分级 3。随访（6-204 个月，中位随访 36 个月）显示 2 例患者出现局部复发，1 例出现远处转移，2 例患者均出现局部复发和远处转移。5 年总生存率（OS）和无事件生存率（EFS）分别为 84%和 55.9%。肿瘤深度和坏死与较低的 OS 相关（P=0.025，P=0.005），而肿瘤深度也与较差的 EFS 相关（P<.001）。我们得出结论，青少年和年轻成人中发生的黏液纤维肉瘤与老年患者相比具有相似的行为。在该年龄组中，肿瘤深度和坏死是黏液纤维肉瘤的不良预后因素。了解黏液纤维肉瘤在该人群中罕见发生对准确诊断很重要。
