“恶性间质瘤”再谈

Sanhong Yu, J. Hornick
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引用次数: 2

摘要

平滑肌肉瘤(LMS)是成人中最常见的肉瘤。LMS很少去分化为未分化肉瘤。很少有LMS合并异源骨肉瘤分化(OS)的病例被报道。本研究的目的是评估LMS合并OS的临床病理特征。2006 - 2022年诊断的5570例LMS中,有15例(0.2%)LMS合并OS,其中女性13例,男性2例;年龄32 ~ 66岁(中位53岁),子宫10例,腹膜后2例,肠系膜、纵隔和直肠各1例。原发肿瘤范围7 ~ 20 cm(平均16 cm)。LMS成分多数呈常规梭形细胞形态;3例有明显的多形性;3例含有上皮样成分;1例表现为黏液样征。5例患者在原发肿瘤中发现OS, 10例患者在转移瘤中首次发现OS。1例转移性LMS和2例原发性LMS同时表现为骨肉瘤和软骨肉瘤分化。11例骨OS部位可见明显的破骨巨细胞。有丝分裂活性为17 ~ 61/10 HPF, 10例肿瘤坏死。12例患者发生转移;部位包括肺、膈、肾、肾上腺、结肠、小肠、肝、骨和胰腺。最后随访8例死亡,4例转移生存。OS和死亡之间的时间间隔为3周到18个月(中位数:6.5个月)。在LMS中开发操作系统是非常罕见的。这种形式的异源分化可能发生在原发肿瘤和转移瘤中。LMS合并OS具有高度侵袭性,预后较差。认识这种现象对于避免误诊为骨肉瘤是很重要的。
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“Malignant Mesenchymoma” Revisited
Leiomyosarcoma (LMS) is the most common sarcoma in adults. Rarely, LMS dedifferentiates into an undifferentiated sarcoma. Very few cases of LMS with heterologous osteosarcomatous differentiation (OS) have been reported. The purpose of this study was to evaluate the clinicopathologic features of LMS with OS. Of 5570 LMS cases diagnosed from 2006 to 2022, 15 cases (0.2%) of LMS with OS were identified, affecting 13 females and 2 males; ages ranged from 32 to 66 years (median: 53 y). Ten tumors arose in the uterus, 2 in the retroperitoneum, and 1 each in the mesentery, mediastinum, and rectum. Primary tumors ranged from 7 to 20 cm (mean: 16 cm). The LMS components showed conventional spindle cell morphology in most cases; 3 cases showed marked pleomorphism; 3 cases contained an epithelioid component; and 1 case showed myxoid features. In 5 cases OS was identified in the primary tumor, whereas in 10 cases OS was first detected in metastases. One metastatic and 2 primary LMS showed both OS and chondrosarcomatous differentiation. Prominent osteoclastic giant cells were seen in the OS components in 11 cases. Mitotic activity ranged from 17 to 61/10 HPF with tumor necrosis in 10 cases. Twelve patients developed metastases; sites included lungs, diaphragm, kidney, adrenal glands, colon, small intestine, liver, bone, and pancreas. At last follow-up, 8 patients had died of disease, and 4 patients were alive with metastases. The interval between OS and death ranged from 3 weeks to 18 months (median: 6.5 mo). Development of OS in LMS is exceptionally rare. This form of heterologous differentiation may occur in both primary tumors and metastases. LMS with OS is highly aggressive with poor outcomes. Awareness of this phenomenon is important to avoid misdiagnosis as osteosarcoma.
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