Department of Pathology, The Ohio State University Wexner Medical Center/James Cancer Hospital, Columbus, Ohio, USA.
Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.
Diagn Cytopathol. 2024 May;52(5):E111-E115. doi: 10.1002/dc.25288. Epub 2024 Feb 16.
Myxoid neurofibromas (NF) are uncommon, benign spindle cell tumors that originate from peripheral nerve sheaths, often posing a diagnostic challenge due to their hypocellularity on cytology specimens. Distinguishing myxoid spindle cell lesions can be challenging, given the broad range of potential differential diagnoses.
A 26-year-old female with a past medical history of embolized inguinal, flank, and retroperitoneal venolymphatic malformation presented with a left pelvic pain causing significant disability. CT scan showed an extensive 8.7 cm × 6.6 cm retroperitoneal mass. FNA was performed and alcohol-fixed papanicolaou-stained smears showed a hypocellular specimen with loosely arranged clusters of bland spindle cell proliferation in the background of a mucoid matrix. Spindle cells showed scant cytoplasm and elongated oval-shaped regular nuclei. Prominent nucleoli were not seen. An excisional biopsy revealed a bland spindle cell proliferation in a myxoid background associated with shredded collagen bundles. Immunohistochemical staining showed diffuse positivity for S100 and CD34. Based on the overall findings, a definitive diagnosis of myxoid neurofibroma was rendered.
Cytological features of myxoid neurofibroma include the presence of hypocellular spindle-shaped cells arranged in small, loosely organized groups within a myxoid matrix background. Cells exhibit scant cytoplasm with regular oval and elongated nuclei. Nucleoli are typically not identified. The differential diagnosis includes myxoid neurofibroma, myxoma, myxoid liposarcoma, myxoid chondrosarcoma, myxoid dermatofibrosarcoma protuberans, low-grade fibromyxoid sarcoma, and low-grade myxo-fibrosarcoma.
We aim to highlight the importance of considering myxoid neurofibroma in the differential diagnosis of hypocellular myxoid spindle cell lesions encountered on fine-needle aspiration cytology.
黏液样神经纤维瘤（NF）是一种罕见的良性梭形细胞肿瘤，起源于周围神经鞘，由于细胞学标本的细胞稀少，常导致诊断困难。由于潜在的鉴别诊断范围广泛，区分黏液样梭形细胞病变具有挑战性。
一位 26 岁女性，既往有腹股沟、腰部和腹膜后血管淋巴管畸形栓塞病史，因左盆腔疼痛导致严重残疾就诊。CT 扫描显示广泛的 8.7×6.6cm 腹膜后肿块。进行了细针抽吸活检（FNA），并对酒精固定巴氏染色的涂片进行了检查，显示细胞稀少的标本中，背景为黏液基质的松散排列的细胞簇中存在良性梭形细胞增生。梭形细胞细胞质稀少，长椭圆形规则核。未见明显核仁。切除活检显示黏液背景下的良性梭形细胞增生，伴有切碎的胶原束。免疫组织化学染色显示 S100 和 CD34 弥漫阳性。根据整体发现，明确诊断为黏液样神经纤维瘤。
黏液样神经纤维瘤的细胞学特征包括存在在黏液基质背景中排列成小而松散的细胞簇的少细胞性梭形细胞。细胞表现为稀少的细胞质，具有规则的椭圆形和长形核。通常不识别核仁。鉴别诊断包括黏液样神经纤维瘤、黏液瘤、黏液样脂肪肉瘤、黏液样软骨肉瘤、黏液样隆突性皮肤纤维肉瘤、低度纤维黏液肉瘤和低度黏液纤维肉瘤。
我们旨在强调在细针抽吸细胞学检查中遇到的少细胞性黏液样梭形细胞病变的鉴别诊断中考虑黏液样神经纤维瘤的重要性。
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