异源苗勒管囊肿的变异型假囊性结缔组织增生小圆细胞瘤。

IF 1.3 4区医学 Q3 PATHOLOGY

Pediatric and Developmental Pathology Pub Date : 2025-01-09 DOI:10.1177/10935266241312435

Eric I Nayman, Carole Brathwaite, Felipe Pedroso, Maggie E Fader, Farres Obeidin, Louis P Dehner

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引用次数: 0

摘要

结缔组织增生小圆细胞瘤（DSRCT）提出了一个13岁的女性急腹症由于扭转输卵管囊肿。在腹腔镜检查中发现盆底偶发有一个2厘米的带梗实心多囊肿块。肿瘤呈不同的黏液样和梭形细胞，小细胞巢和索状，形成假性囊肿，真囊肿内衬纤毛上皮，PAX-8+和ER+/PR+。肿瘤为EWSR1::WT1融合。3个月后再次腹腔镜检查发现大量小于1cm的腹膜结节。这些具有相似的特征，包括纤毛上皮囊肿。我们的病例显示了另一种可能的DSRCT变异模式，伴有缪勒氏样纤毛上皮囊肿。

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Variant Pseudocystic Desmoplastic Small Round Cell Tumor With Heterologous Mullerian Cysts.

A desmoplastic small round cell tumor (DSRCT) presented in a 13-year-old female with an acute abdomen due to torsion of a fallopian tube cyst. She was found to have an incidental 2 cm pedunculated, solid, and multicystic mass attached to the pelvic floor on laparoscopy. The neoplasm had a variably myxoid and spindle cell pattern with nests and cords of small cells, forming pseudocysts, and true cysts lined by ciliated epithelium which were PAX-8+ and ER+/PR+. The tumor had the EWSR1::WT1 fusion. Numerous peritoneal nodules less than 1 cm were noted on repeat laparoscopy 3 months later. These had similar features including the ciliated epithelial cysts. Our case illustrates yet another potential variant pattern of DSRCT with Mullerian-like ciliated epithelial cysts.

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来源期刊

Pediatric and Developmental Pathology 医学-病理学

CiteScore

3.70

自引率

5.30%

发文量

审稿时长

6-12 weeks

期刊介绍： The Journal covers the spectrum of disorders of early development (including embryology, placentology, and teratology), gestational and perinatal diseases, and all diseases of childhood. Studies may be in any field of experimental, anatomic, or clinical pathology, including molecular pathology. Case reports are published only if they provide new insights into disease mechanisms or new information.