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胚胎性横纹肌肉瘤的细胞形态学观察:中耳罕见病例。

Cytomorphological Insights into Embryonal Rhabdomyosarcoma: A Rare Case in the Middle Ear.

机构信息

Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.

Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India,

出版信息

Acta Cytol. 2024;68(4):309-313. doi: 10.1159/000538809. Epub 2024 Apr 17.

DOI:10.1159/000538809
PMID:38599199
Abstract

INTRODUCTION

Rhabdomyosarcoma, though rare in the middle ear, necessitates prompt recognition for optimal management. They are malignant mesenchymal neoplasms arising from the embryonic mesenchymal cells of striated skeletal muscles.

CASE PRESENTATION

We present a case of a 5-year-old child with massive right mastoid swelling and bloody ear discharge. Cytological examination via fine needle aspiration biopsy revealed features suggestive of a malignant mesenchymal tumor, confirmed on imaging and subsequent histopathology as embryonal rhabdomyosarcoma (ERMS) FNCLCC grade 2. Characteristic cytological findings included spindle-shaped cells in a myxoid stroma with tigroid background, aiding in early diagnosis. Despite radical mastoidectomy and adjuvant chemotherapy, ERMS in the middle ear remains challenging due to its aggressive nature and potential complications.

CONCLUSION

This case underscores the importance of cytological evaluation in identifying rare soft tissue tumors like ERMS, facilitating timely intervention and improved outcomes. Early recognition and multidisciplinary management are crucial in addressing the complexities of ERMS in uncommon sites like the middle ear.

摘要

简介

横纹肌肉瘤虽在中耳罕见,但为实现最佳治疗,需及时识别。其是起源于骨骼肌胚胎间充质细胞的恶性间叶性肿瘤。

病例介绍

我们介绍了一例 5 岁儿童,其右侧乳突巨大肿胀伴血性耳漏。细针抽吸活检的细胞学检查显示提示恶性间叶性肿瘤的特征,影像学和随后的组织病理学检查证实为胚胎性横纹肌肉瘤(ERMS)FNCLCC 分级 2 级。特征性的细胞学发现包括黏液样基质中的梭形细胞伴网织状背景,有助于早期诊断。尽管进行了根治性乳突切除术和辅助化疗,但由于其侵袭性和潜在并发症,中耳 ERMS 仍然具有挑战性。

结论

该病例强调了细胞学评估在识别 ERMS 等罕见软组织肿瘤中的重要性,有助于及时干预和改善预后。早期识别和多学科管理对于解决 ERMS 在中耳等罕见部位的复杂性至关重要。

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Cytomorphological Insights into Embryonal Rhabdomyosarcoma: A Rare Case in the Middle Ear.胚胎性横纹肌肉瘤的细胞形态学观察:中耳罕见病例。
Acta Cytol. 2024;68(4):309-313. doi: 10.1159/000538809. Epub 2024 Apr 17.
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