Primary Intraosseous Granular Cell Tumor of the Sphenoid and Central Skull Base in a Pediatric Patient.

IF 0.6 4区医学 Q4 PATHOLOGY

Fetal and Pediatric Pathology Pub Date : 2024-05-01 Epub Date: 2024-02-12 DOI:10.1080/15513815.2024.2315455

Delaney Sheehan, Belinda Mantle, Ashley Kraft, Randall Craver, Christopher Arcement, Renee Gardner, Ellen Zakris, Daniel Nuss

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引用次数: 0

Abstract

Background: Granular cell tumors occur in all ages and many anatomic sites. In the craniofacial region, they typically arise in soft tissue, not bone. We present a primary intra-osseous granular cell tumor of the sphenoid and central skull base arising in a 12- year- old girl.

Case report: A 12-year-old female with sickle cell disease and Jeavons syndrome presented with seizures. Imaging and partial resection revealed an expansile benign granular cell tumor (GCT) involving the sphenoid body, pterygoid process, and central skull base. The disease has remained stable after 36-month follow up.

Discussion: GCT primarily involving the osseous sphenoid/skull base has not been previously reported in a child. Although mostly benign, some are aggressive, with malignant transformation in 1-2%. Surgery is the mainstay of treatment, but in the skull base this may be limited by adjacent critical structures. Decision-making is guided by anatomic extent, histology, and clinical behavior.

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一名儿童患者的鼻骨和中央颅底原发性骨内颗粒细胞瘤

背景：颗粒细胞瘤发生在各个年龄段和许多解剖部位。在颅面部，颗粒细胞瘤通常发生在软组织而非骨骼。我们为您介绍的是一名 12 岁女孩的鼻骨和中央颅底原发性骨内颗粒细胞瘤：病例报告：一名患有镰状细胞病和杰文斯综合征的 12 岁女孩出现癫痫发作。影像学检查和部分切除术显示，该患者的蝶骨体、翼突和中央颅底长有扩张性良性颗粒细胞瘤（GCT）。经过36个月的随访，病情保持稳定：讨论：主要累及骨性蝶骨/颅底的儿童颗粒细胞瘤以前从未报道过。讨论：主要累及骨性蝶骨/颅底的 GCT 以前从未有过儿童病例的报道。手术是治疗的主要手段，但在颅底，手术可能会受到邻近重要结构的限制。在做出决定时，应根据解剖学范围、组织学和临床表现。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Fetal and Pediatric Pathology 医学-病理学

CiteScore

3.00

自引率

0.00%

发文量

审稿时长

6-12 weeks

期刊介绍： Fetal and Pediatric Pathology is an established bimonthly international journal that publishes data on diseases of the developing embryo, newborns, children, and adolescents. The journal publishes original and review articles and reportable case reports. The expanded scope of the journal encompasses molecular basis of genetic disorders; molecular basis of diseases that lead to implantation failures; molecular basis of abnormal placentation; placentology and molecular basis of habitual abortion; intrauterine development and molecular basis of embryonic death; pathogenisis and etiologic factors involved in sudden infant death syndrome; the underlying molecular basis, and pathogenesis of diseases that lead to morbidity and mortality in newborns; prenatal, perinatal, and pediatric diseases and molecular basis of diseases of childhood including solid tumors and tumors of the hematopoietic system; and experimental and molecular pathology.