Barroca Helena, Farinha N J, Lobo Ana, Monteiro Joaqum, Lopes José Mahuel
Service of Pathology, Department of Pediatrics, Faculty of Medicine, Hospital de S. João, Instituto de Patologia e Imunologia da Univesidade do Porto, Portugal.
Acta Cytol. 2007 May-Jun;51(3):473-6. doi: 10.1159/000325771.
Juvenile xanthogranuloma (JXG) is a rare, benign non-Langerhans cell histiocytosis that usually occurs in the head, neck or upper trunk of neonates and young children. Lesions appear most frequently as solitary cutaneous nodule, but in 12% of cases they are multiple and in 5%, subcutaneous or deep-seated. In 4% of cases they are systemic. Histopathologically, deep lesions tend to be more cellular and monotonous, with fewer Touton cells. Independent of its location, the prognosis is excellent, even after incomplete resection. The lesions usually resolve spontaneously within 3 years of diagnosis.
A child was born with a large, deep-seated cervical mass that was initially evaluated by fine needle aspiration biopsy, which disclosed vague, granulomatous aggregates with monotonous, histiocytic CD68-positive cells. The surgical specimen histology revealed a JXG with skeletal muscle invasion. Complete reexcision was performed 2 months later after regrowth of the tumor.
Deep-seated neonatal JXGs are infrequent and may demonstrate aggressive behavior. In this setting fine needle aspiration cytology is a useful tool for the initial differential diagnostic procedure and management.
幼年性黄色肉芽肿（JXG）是一种罕见的良性非朗格汉斯细胞组织细胞增生症，通常发生于新生儿和幼儿的头、颈或上躯干。病变最常表现为孤立性皮肤结节，但12%的病例为多发，5%为皮下或深部病变。4%的病例为系统性病变。组织病理学上，深部病变往往细胞更多且更单一， Touton细胞较少。无论其位置如何，预后都很好，即使不完全切除也是如此。病变通常在诊断后3年内自行消退。
一名儿童出生时颈部有一个巨大的深部肿块，最初通过细针穿刺活检进行评估，结果显示有模糊的肉芽肿性聚集物，伴有单一的、组织细胞性CD68阳性细胞。手术标本组织学显示为伴有骨骼肌侵犯的幼年性黄色肉芽肿。肿瘤复发2个月后进行了完整的再次切除。
深部新生儿幼年性黄色肉芽肿并不常见，可能表现出侵袭性。在这种情况下，细针穿刺细胞学检查是初步鉴别诊断和处理的有用工具。
