Hayes T C, Britton H A, Mewborne E B, Troyer D A, Saldivar V A, Ratner I A
Department of Pathology, Santa Rosa Children's Hospital, San Antonio, TX 78207, USA.
Pediatrics. 1998 May;101(5):E10. doi: 10.1542/peds.101.5.e10.
An 11-year-old girl with low-grade fever, night sweats, thrombocytopenia, and an 8-year history of progressive splenomegaly underwent an elective splenectomy. Pathologic diagnosis was multiple splenic hamartoma. The patient's symptoms resolved after the splenectomy. Since first described by Rokitansky in 1861, approximately 140 cases of splenic hamartoma have been described in the literature. Most of the splenic hamartomas were discovered incidentally. A minority of these lesions were associated with hematologic symptoms such as pancytopenia, anemia, and thrombocytopenia. Only 20 of the reported cases of splenic hamartoma occurred in pediatric patients. However, compared with the adult patients, nearly half of these cases in pediatric patients was associated with symptoms. Splenectomy and partial splenectomy have relieved these symptoms. With advances in imaging, splenic hamartomas are being discovered with increasing frequency. A multimodal radiologic work-up has enabled some cases of splenic hamartoma to be diagnosed preoperatively. Inclusion of this benign entity in the differential diagnoses of symptomatic splenomegaly in a pediatric patient is important in the preoperative management and counseling of the patient and family. In patients who have discrete lesions, consideration of this entity preoperatively may avoid total splenectomy.
一名11岁女孩,伴有低热、盗汗、血小板减少,并有8年进行性脾肿大病史,接受了择期脾切除术。病理诊断为多发性脾错构瘤。脾切除术后患者症状缓解。自1861年罗基坦斯基首次描述以来,文献中已报道了约140例脾错构瘤病例。大多数脾错构瘤是偶然发现的。少数这些病变与血细胞减少、贫血和血小板减少等血液学症状有关。报道的脾错构瘤病例中只有20例发生在儿科患者中。然而,与成年患者相比,儿科患者中近一半的病例与症状有关。脾切除术和部分脾切除术缓解了这些症状。随着影像学的发展,脾错构瘤的发现频率越来越高。多模式影像学检查已使一些脾错构瘤病例能够在术前得到诊断。在儿科患者有症状性脾肿大的鉴别诊断中纳入这种良性病变,对于患者及其家属的术前管理和咨询非常重要。对于有离散病变的患者,术前考虑这种病变可能避免全脾切除术。