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

IF 1.6 4区 医学 Q3 PATHOLOGY Acta Cytologica Pub Date : 2024-04-10 DOI:10.1159/000538809
A. Khan, Sana Ahuja, S. Zaheer
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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.
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细胞形态学对胚胎性横纹肌肉瘤的启示:中耳罕见病例
导言:横纹肌肉瘤虽然在中耳罕见,但需要及时识别,以便进行最佳治疗。横纹肌肉瘤是一种恶性间充质肿瘤,由横纹骨骼肌的胚胎间充质细胞产生。病例介绍 我们接诊了一例右乳突肿大并伴有血性耳分泌物的 5 岁儿童。通过细针穿刺活检进行的细胞学检查发现了恶性间质瘤的特征,经影像学检查和随后的组织病理学检查证实为胚胎性横纹肌肉瘤(ERMS)FNCLCC 2 级。特征性细胞学检查结果包括肌样基质中的纺锤形细胞和虎纹状背景,有助于早期诊断。尽管进行了乳突根治性切除术和辅助化疗,但由于中耳ERMS具有侵袭性和潜在并发症,因此仍然具有挑战性。结论 本病例强调了细胞学评估对识别 ERMS 等罕见软组织肿瘤的重要性,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中耳等罕见部位 ERMS 的复杂性至关重要。
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来源期刊
Acta Cytologica
Acta Cytologica 生物-病理学
CiteScore
3.70
自引率
11.10%
发文量
46
审稿时长
4-8 weeks
期刊介绍: With articles offering an excellent balance between clinical cytology and cytopathology, ''Acta Cytologica'' fosters the understanding of the pathogenetic mechanisms behind cytomorphology and thus facilitates the translation of frontline research into clinical practice. As the official journal of the International Academy of Cytology and affiliated to over 50 national cytology societies around the world, ''Acta Cytologica'' evaluates new and existing diagnostic applications of scientific advances as well as their clinical correlations. Original papers, review articles, meta-analyses, novel insights from clinical practice, and letters to the editor cover topics from diagnostic cytopathology, gynecologic and non-gynecologic cytopathology to fine needle aspiration, molecular techniques and their diagnostic applications. As the perfect reference for practical use, ''Acta Cytologica'' addresses a multidisciplinary audience practicing clinical cytopathology, cell biology, oncology, interventional radiology, otorhinolaryngology, gastroenterology, urology, pulmonology and preventive medicine.
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