Sarcomatoid Morphology in Pediatric Langerhans Cell Neoplasm Does Not Always Predict Aggressive Clinical Course.

IF 1.3 4区 医学 Q3 PATHOLOGY Pediatric and Developmental Pathology Pub Date : 2024-09-28 DOI:10.1177/10935266241281517
Sam Sirotnikov, Louis P Dehner, José E Velázquez Vega, Jinjun Cheng
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Abstract

Langerhans cell sarcoma (LCS), a rare malignant neoplasm in the general category of myeloid neoplasms characterized by overtly malignant Langerhans cells (LC) with conspicuous mitotic activity including atypical forms. Although most cases occur in adults, rare examples of LCS have been reported in children with variable clinical outcome. We present 2 childhood cases of Langerhans cell neoplasm with high grade sarcomatous features and OSBPL9::BRAF fusion and BRAF V600E mutation.

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小儿朗格汉斯细胞肿瘤中的肉瘤样形态并不总能预测侵袭性临床病程。
朗格汉斯细胞肉瘤(Langerhans cell sarcoma,LCS)是一种罕见的恶性肿瘤,属于髓系肿瘤的一般类别,其特征是明显恶性的朗格汉斯细胞(Langerhans cell,LC)具有明显的有丝分裂活性,包括非典型形式。虽然大多数病例发生在成人身上,但也有罕见的儿童朗格汉斯细胞瘤病例报道,而且临床结果各不相同。我们报告了两例具有高级别肉瘤特征、OSBPL9::BRAF融合和BRAF V600E突变的朗格汉斯细胞肿瘤儿童病例。
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来源期刊
CiteScore
3.70
自引率
5.30%
发文量
59
审稿时长
6-12 weeks
期刊介绍: The Journal covers the spectrum of disorders of early development (including embryology, placentology, and teratology), gestational and perinatal diseases, and all diseases of childhood. Studies may be in any field of experimental, anatomic, or clinical pathology, including molecular pathology. Case reports are published only if they provide new insights into disease mechanisms or new information.
期刊最新文献
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