Rapid response of aggressive cranial Langerhans cell histiocytosis to combined intralesional and perilesional triamcinolone injection: a case report with video.

IF 1.2
Jesús A Sánchez Hernández, Mónica Rivero Garvia, Gloria Moreno Madueño, José I Gutiérrez Carrasco, Javier Márquez Rivas
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Abstract

Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm that predominantly affects bone in the pediatric population, with the skull being one of the most frequent sites of involvement. Local complications following diagnostic procedures in this context are exceptionally rare and poorly described. We report the case of a 14-year-old male with unifocal cranial LCH who developed aggressive local progression after a diagnostic biopsy, characterized by progressive bone destruction, soft tissue involvement, surgical wound dehiscence, and exposure of tumor tissue. Given the rapid evolution of the lesion and the limitations for conventional surgical management, intralesional and perilesional triamcinolone infiltration (40 mg) was performed. A prompt clinical response was observed, with a significant reduction in lesion volume within 4 days. Follow-up magnetic resonance imaging at that time demonstrated a 50-70% decrease in the soft tissue component. During subsequent follow-up, progressive skin healing and early signs of bone repair were noted. This case highlights an uncommon local complication following biopsy of pediatric cranial LCH and suggests that local corticosteroid infiltration may represent a minimally invasive and potentially useful therapeutic alternative in selected cases with aggressive local progression and limited surgical options.

侵袭性脑朗格汉斯细胞组织细胞增生症对局内外曲安奈德联合注射的快速反应:附视频的1例报告。
朗格汉斯细胞组织细胞增生症(LCH)是一种罕见的髓系肿瘤,主要影响儿童骨骼,颅骨是最常见的受累部位之一。在这种情况下,诊断程序后的局部并发症非常罕见且描述不佳。我们报告一例14岁男性单灶性颅内LCH患者,在诊断性活检后出现侵袭性局部进展,其特征是进行性骨破坏,软组织受损伤,手术伤口裂开和肿瘤组织暴露。鉴于病变的快速演变和传统手术治疗的局限性,我们进行了局内和局外曲安奈德浸润(40mg)。观察到迅速的临床反应,病变体积在4天内显着减少。当时的后续磁共振成像显示软组织成分减少了50-70%。在随后的随访中,皮肤逐渐愈合和骨修复的早期迹象被注意到。本病例强调了小儿颅内LCH活检后罕见的局部并发症,并提示局部皮质类固醇浸润可能是一种微创且潜在有用的治疗选择,适用于局部进展严重且手术选择有限的病例。
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