Department of Pathology, Chacha Nehru Bal Chikitsalaya, New Delhi, India.
Department of Radiodiagnosis, Chacha Nehru Bal Chikitsalaya, New Delhi, India.
Cytopathology. 2020 Mar;31(2):115-121. doi: 10.1111/cyt.12786. Epub 2020 Feb 7.
Fibroblastic/myofibroblastic tumors constitute 12% of all pediatric soft tissue tumors with the majority of them belonging to the benign and intermediate prognostic categories. They are often misdiagnosed owing to their variable clinical presentation and unusual microscopic features. The diagnosis, specially cytological diagnosis of benign and intermediate categories is difficult due to paucity of cellular component and increased amount of extracellular matrix as compared to malignant ones. We hereby discuss the Fine needle aspiration cytology (FNAC) findings of non-malignant fibroblastic/myofibroblastic lesion in the pediatric age group encountered at our institute.
All the benign and intermediate fibroblastic/myofibroblastic/fibroadipocytic lesions (age 0-12 years) diagnosed on FNAC over a period of 3½ years (Jan 2016- July 2019), with availability of corresponding histopathology were included in the study.
A total of seven pediatric benign and intermediate fibroblastic/myofibroblastic lesions with histopathological confirmation were identified which included Infantile digital fibromatosis (IDF) (n = 2), Lipofibromatosis (n = 1), Fibrous hamartoma of infancy (FHI) (n = 1), Fibromatosis colli (FC) (n = 2) and myofibroma/myofibromatosis (n = 1). FNAC smears were mainly paucicellular with presence of benign spindle shaped cells in a collagenous stroma common to almost all the cases. A few additional findings such as degenerated skeletal muscle fibres, muscle giant cells and mature adipose tissue were also present in some cases.
Fibroblastic/myofibroblastic tumors although uncommon, form an important category that must be considered in the differential diagnosis of pediatric soft tissue tumors. FNAC cytology features when assessed in a proper clinical setting (specially the age and site of presentation) are helpful in suggesting probable preoperative diagnosis in these lesions.
成纤维细胞/肌纤维母细胞瘤构成所有儿科软组织肿瘤的 12%，其中大多数属于良性和中间预后类别。由于其多变的临床表现和不寻常的显微镜特征，它们经常被误诊。与恶性肿瘤相比，良性和中间类别由于细胞成分较少，细胞外基质较多，因此诊断，特别是细胞学诊断具有一定难度。我们在此讨论在我院遇到的儿科年龄组中非恶性成纤维细胞/肌纤维母细胞瘤的细针抽吸细胞学（FNAC）发现。
在 3 年半的时间内（2016 年 1 月至 2019 年 7 月），对所有在 FNAC 上诊断为良性和中间成纤维细胞/肌纤维母细胞/纤维脂肪细胞病变（年龄 0-12 岁）的患儿进行了研究，且均有相应的组织病理学检查结果。
共发现 7 例经组织病理学证实的儿科良性和中间成纤维细胞/肌纤维母细胞病变，包括婴儿指纤维瘤病（IDF）（n=2）、脂肪纤维瘤病（n=1）、婴儿纤维性错构瘤（FHI）（n=1）、颈纤维瘤病（FC）（n=2）和肌纤维瘤/肌纤维母细胞瘤（n=1）。FNAC 涂片主要为细胞稀少，几乎所有病例中均存在良性梭形细胞，存在胶原基质。一些附加发现，如退变的骨骼肌纤维、肌巨细胞和成熟脂肪组织，在一些病例中也存在。
成纤维细胞/肌纤维母细胞瘤虽然不常见，但构成了一个重要的类别，在儿科软组织肿瘤的鉴别诊断中必须考虑。在适当的临床环境下评估 FNAC 细胞学特征（特别是年龄和发病部位）有助于提示这些病变的术前可能诊断。
