Uterine Neurotrophic Tyrosine Receptor Kinase Rearranged Spindle Cell Neoplasms: Three Cases of an Emerging Entity.

IF 1.6 4区 医学 Q3 OBSTETRICS & GYNECOLOGY International Journal of Gynecological Pathology Pub Date : 2024-07-01 Epub Date: 2023-09-22 DOI:10.1097/PGP.0000000000000988
Lucy Grant, William Boyle, Sarah Williams, Jennifer Pascoe, Raji Ganesan
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Abstract

Uterine sarcomas are rare; most are either smooth muscle or endometrial stromal in origin. Recent molecular advances have identified several, genetically defined entities with specific morphologic, clinicopathological associations, and therapeutic options. We report 3 cases of uterine neurotrophic tyrosine receptor kinase ( NTRK )-rearranged spindle cell neoplasms," currently classified as "emerging entities" in the WHO Classification of Female Genital Tract Tumors, 2020, Fifth Edition. The affected patients were 32, 34, and 42 years of age. Two patients presented with vaginal bleeding; the third presented with a cervical mass found incidentally during laparoscopy for an ectopic gestation. All 3 tumors were polypoid masses that distorted the cervix. Microscopically, they comprised cellular, fascicular, and storiform, plump spindle cells, with occasional rounded cells, and frequent mitoses (4-48/10 high power fields) in a myxoid stroma. All 3 cases showed entrapment of benign cervical glands. Inflammatory cell infiltrates, including plasma cells, were noted in all 3 tumors. One case had tumor cell necrosis, osteoid-like material, and osteoclast-like giant cells and showed lymphovascular invasion. Immunohistochemically, our cases showed patchy S100 (2/3) and CD34 (3/3) positivity. CD10 was positive in 2/3 cases. 3/3 cases showed pan-tropomyosin receptor kinase positivity (cytoplasmic). The NTRK -translocations demonstrated were: NTRK1::TMP3, NTRK1::TPR, and NTRK3::SPECC1L . Two of the patients had extensive disease and underwent chemotherapy. Larotrectinib was approved for one patient who demonstrated a striking reduction in tumor volume upon initiation of this treatment.

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子宫神经营养酪氨酸受体激酶重排梭形细胞肿瘤:三例新发实体瘤。
子宫肉瘤是罕见的;大多数起源于平滑肌或子宫内膜间质。最近的分子进展已经确定了几个具有特定形态学、临床病理学关联和治疗选择的基因定义实体。我们报告3例子宫神经营养酪氨酸受体激酶(NTRK)重排梭形细胞肿瘤,“目前被归类为”新兴实体“世界卫生组织女性生殖道肿瘤分类,2020,第五版。受影响的患者年龄分别为32岁、34岁和42岁。两名患者出现阴道出血;第三名患者出现宫外孕腹腔镜检查时意外发现的宫颈肿块。所有3个肿瘤都是扭曲宫颈的息肉样肿块。显微镜下,它们包括细胞、束状ar,黏液样间质中有储存状、肥大的梭形细胞,偶尔有圆形细胞,有频繁的有丝分裂(4-48/10高倍视野)。3例均表现为良性宫颈腺体包夹。在所有3个肿瘤中均发现炎症细胞浸润,包括浆细胞。1例出现肿瘤细胞坏死、类骨物质和破骨细胞样巨细胞,并显示淋巴管浸润。免疫组化显示,我们的病例显示斑片状S100(2/3)和CD34(3/3)阳性。CD10阳性2/3例。3/3例显示泛原肌球蛋白受体激酶阳性(细胞质)。证明的NTRK易位为:NTRK1::TMP3、NTRK1::TPR和NTRK3::SPECC1L。其中两名患者患有广泛的疾病,并接受了化疗。拉罗替尼被批准用于一名患者,该患者在开始该治疗时肿瘤体积显著减少。
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来源期刊
CiteScore
3.90
自引率
12.50%
发文量
154
审稿时长
6-12 weeks
期刊介绍: International Journal of Gynecological Pathology is the official journal of the International Society of Gynecological Pathologists (ISGyP), and provides complete and timely coverage of advances in the understanding and management of gynecological disease. Emphasis is placed on investigations in the field of anatomic pathology. Articles devoted to experimental or animal pathology clearly relevant to an understanding of human disease are published, as are pathological and clinicopathological studies and individual case reports that offer new insights.
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