Superficial plexiform schwannoma of the skin and subcutis: Clinicopathological study of 48 cases.

IF 4.1 2区医学 Q2 CELL BIOLOGY

Histopathology Pub Date : 2025-08-27 DOI:10.1111/his.15544

David I Suster, John M Gross, Steven D Billings, Jennifer S Ko, Saul M Suster, Shira Ronen

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引用次数: 0

Abstract

Aims: To study the clinicopathological characteristics of a large cohort of cutaneous plexiform schwannoma.

Methods and results: A total of 48 cases of plexiform schwannoma were collected. Clinical and pathological features including age, location, location within the dermis, histology, immunohistochemical features, treatment and clinical follow-up were collected by reviewing glass slides or abstracted from the medical record. The patients were 22 women and 26 men aged 6-84 years (mean: 43). The tumours arose in the extremities in 17 patients, trunk in 17, head and neck in 12, and buttock in 2. The tumours were all superficially located and confined to the dermis, subcutis, or both and measured from 0.3 to 6.0 cm (mean: 2.0 cm). The tumours were all well-circumscribed and showed a multinodular, plexiform pattern of growth. Histologically they showed features of Antoni type A in 30 cases, Antoni type B in 3, and a combination of type A and type B in 15. Immunohistochemical stains in all cases were positive for S100 and SOX10 and showed peripheral condensation of EMA and CD34 positive perineurial cells. The Ki-67 index was low in all cases (1%-2%). Seven patients (12.5%) had type 2 neurofibromatosis (NF2). All patients were treated by complete surgical excision. Clinical follow-up from 13 months to 5 years was available in 16 patients; there was no evidence of recurrence or metastases.

Conclusions: Plexiform schwannomas arising in the skin and subcutis are indolent. A subset shows an association with neurofibromatosis type 2.

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皮肤及皮下浅表丛状神经鞘瘤48例临床病理分析。

目的：探讨一大批皮肤丛状神经鞘瘤的临床病理特点。方法与结果：共收集48例丛状神经鞘瘤。通过回顾玻片或从病历中摘录，收集患者的年龄、体位、真皮内位置、组织学、免疫组化特征、治疗及临床随访等临床病理特征。女性22例，男性26例，年龄6 ~ 84岁（平均43岁）。17例患者的肿瘤发生在四肢，17例发生在躯干，12例发生在头颈部，2例发生在臀部。肿瘤均位于浅表，局限于真皮、皮下或两者，尺寸为0.3 ~ 6.0 cm（平均2.0 cm）。肿瘤边界清晰，呈多结节、丛状生长。组织学上表现为Antoni A型30例，Antoni B型3例，A型和B型合并15例。所有病例的免疫组化染色均为S100和SOX10阳性，并显示EMA和CD34阳性的周围神经细胞凝聚。Ki-67指数均较低（1% ~ 2%）。2型神经纤维瘤病（NF2） 7例（12.5%）。所有患者均行完全手术切除。16例患者的临床随访时间为13个月至5年；没有复发或转移的证据。结论：发生于皮肤和皮下的丛状神经鞘瘤是无痛的。一个亚群显示与2型神经纤维瘤病有关。

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来源期刊

Histopathology 医学-病理学

CiteScore

10.20

自引率

4.70%

发文量

239

审稿时长

1 months

期刊介绍： Histopathology is an international journal intended to be of practical value to surgical and diagnostic histopathologists, and to investigators of human disease who employ histopathological methods. Our primary purpose is to publish advances in pathology, in particular those applicable to clinical practice and contributing to the better understanding of human disease.