Transumbilical single-site laparoscopic resection of splenic papillary angioendothelioma in a pediatric patient: a rare case report and review of literature

BackgroundSplenic papillary angioendothelioma (PILA) is a rare borderline vascular tumor that is often asymptomatic and typically identified incidentally during imaging studies. Owing to its nonspecific clinical presentation and diagnostic complexity, optimal management strategies, particularly in p...

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Main Authors: Meng Kong, Qingfei Zhai, Shisong Zhang, Hongzhen Liu, Yuexia Bai, Shuai Chen, Jinhua Jia, Dong Wang, Xiang Ma
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-07-01
Series:Frontiers in Oncology
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Online Access:https://www.frontiersin.org/articles/10.3389/fonc.2025.1571209/full
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author Meng Kong
Meng Kong
Qingfei Zhai
Shisong Zhang
Shisong Zhang
Hongzhen Liu
Hongzhen Liu
Yuexia Bai
Shuai Chen
Shuai Chen
Jinhua Jia
Jinhua Jia
Dong Wang
Xiang Ma
author_facet Meng Kong
Meng Kong
Qingfei Zhai
Shisong Zhang
Shisong Zhang
Hongzhen Liu
Hongzhen Liu
Yuexia Bai
Shuai Chen
Shuai Chen
Jinhua Jia
Jinhua Jia
Dong Wang
Xiang Ma
author_sort Meng Kong
collection DOAJ
description BackgroundSplenic papillary angioendothelioma (PILA) is a rare borderline vascular tumor that is often asymptomatic and typically identified incidentally during imaging studies. Owing to its nonspecific clinical presentation and diagnostic complexity, optimal management strategies, particularly in pediatric patients, remain understudied. This report describes a case of pediatric splenic PILA managed with spleen-preserving minimally invasive surgery.Case descriptionA 5-year-old boy presented with an incidentally detected splenic mass during abdominal ultrasound six months prior to admission. The patient was asymptomatic and had no prior medical history. The patient was 115 cm tall and weighed 20.5 kg when admitted to the hospital. In the past half year, there has been no obvious growth and development delay, nor have there been any significant changes in diet or weight. Preoperative imaging, including contrast-enhanced ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI), revealed a well-circumscribed lesion (6.0 cm × 5.5 cm × 5.0 cm) at the splenic upper pole, exhibiting heterogeneous enhancement patterns suggestive of a vascular tumor. Following multidisciplinary evaluation, a transumbilical single-port laparoscopic exploration was performed. Intraoperative frozen-section histopathology revealed benign characteristics, suggesting the need for a spleen-sparing partial splenectomy. Definitive postoperative histopathological and immunohistochemical analyses confirmed the diagnosis of PILA, which was characterized by CD31, CD34, and ERG positivity.ConclusionsPediatric splenic PILA is diagnostically challenging because of its rarity and overlapping radiological features with other vascular neoplasms. Complete surgical excision and longitudinal surveillance are critical to mitigate the risk of local recurrence or metastasis, given the tumor’s borderline malignant potential. Single-port laparoscopic partial splenectomy represents a feasible, minimally invasive approach for pediatric patients, balancing oncological safety with preservation of splenic function. This case underscores the importance of integrating intraoperative frozen-section analysis and advanced imaging to guide surgical decision-making, particularly in children.
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spelling doaj-art-780c32f6466e4aba822d88725b6bcda02025-08-20T02:37:06ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2025-07-011510.3389/fonc.2025.15712091571209Transumbilical single-site laparoscopic resection of splenic papillary angioendothelioma in a pediatric patient: a rare case report and review of literatureMeng Kong0Meng Kong1Qingfei Zhai2Shisong Zhang3Shisong Zhang4Hongzhen Liu5Hongzhen Liu6Yuexia Bai7Shuai Chen8Shuai Chen9Jinhua Jia10Jinhua Jia11Dong Wang12Xiang Ma13Department of Pediatric Surgery, Children’s Hospital Affiliated to Shandong University, Jinan, ChinaDepartment of Pediatric Surgery, Jinan Children’s Hospital, Jinan, ChinaDepartment of Ultrasound Medicine, Jining No.1 People’s Hospital, Jining, ChinaDepartment of Pediatric Surgery, Children’s Hospital Affiliated to Shandong University, Jinan, ChinaDepartment of Pediatric Surgery, Jinan Children’s Hospital, Jinan, ChinaDepartment of Pediatric Surgery, Children’s Hospital Affiliated to Shandong University, Jinan, ChinaDepartment of Pediatric Surgery, Jinan Children’s Hospital, Jinan, ChinaDepartment of Pathology, Children’s Hospital Affiliated to Shandong University, Jinan, ChinaDepartment of Pediatric Surgery, Children’s Hospital Affiliated to Shandong University, Jinan, ChinaDepartment of Pediatric Surgery, Jinan Children’s Hospital, Jinan, ChinaDepartment of Pediatric Surgery, Children’s Hospital Affiliated to Shandong University, Jinan, ChinaDepartment of Pediatric Surgery, Jinan Children’s Hospital, Jinan, ChinaDepartment of Pediatric Surgery, Shandong Provincial Maternal and Child Health Care Hospital Affiliated to Qingdao University, Jinan, ChinaDepartment of Respiratory Disease, Children’s Hospital Affiliated to Shandong University, Jinan, ChinaBackgroundSplenic papillary angioendothelioma (PILA) is a rare borderline vascular tumor that is often asymptomatic and typically identified incidentally during imaging studies. Owing to its nonspecific clinical presentation and diagnostic complexity, optimal management strategies, particularly in pediatric patients, remain understudied. This report describes a case of pediatric splenic PILA managed with spleen-preserving minimally invasive surgery.Case descriptionA 5-year-old boy presented with an incidentally detected splenic mass during abdominal ultrasound six months prior to admission. The patient was asymptomatic and had no prior medical history. The patient was 115 cm tall and weighed 20.5 kg when admitted to the hospital. In the past half year, there has been no obvious growth and development delay, nor have there been any significant changes in diet or weight. Preoperative imaging, including contrast-enhanced ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI), revealed a well-circumscribed lesion (6.0 cm × 5.5 cm × 5.0 cm) at the splenic upper pole, exhibiting heterogeneous enhancement patterns suggestive of a vascular tumor. Following multidisciplinary evaluation, a transumbilical single-port laparoscopic exploration was performed. Intraoperative frozen-section histopathology revealed benign characteristics, suggesting the need for a spleen-sparing partial splenectomy. Definitive postoperative histopathological and immunohistochemical analyses confirmed the diagnosis of PILA, which was characterized by CD31, CD34, and ERG positivity.ConclusionsPediatric splenic PILA is diagnostically challenging because of its rarity and overlapping radiological features with other vascular neoplasms. Complete surgical excision and longitudinal surveillance are critical to mitigate the risk of local recurrence or metastasis, given the tumor’s borderline malignant potential. Single-port laparoscopic partial splenectomy represents a feasible, minimally invasive approach for pediatric patients, balancing oncological safety with preservation of splenic function. This case underscores the importance of integrating intraoperative frozen-section analysis and advanced imaging to guide surgical decision-making, particularly in children.https://www.frontiersin.org/articles/10.3389/fonc.2025.1571209/fullsplenic papillary angioendotheliomapediatriclaparoscopysingle-sitepartial splenectomy
spellingShingle Meng Kong
Meng Kong
Qingfei Zhai
Shisong Zhang
Shisong Zhang
Hongzhen Liu
Hongzhen Liu
Yuexia Bai
Shuai Chen
Shuai Chen
Jinhua Jia
Jinhua Jia
Dong Wang
Xiang Ma
Transumbilical single-site laparoscopic resection of splenic papillary angioendothelioma in a pediatric patient: a rare case report and review of literature
Frontiers in Oncology
splenic papillary angioendothelioma
pediatric
laparoscopy
single-site
partial splenectomy
title Transumbilical single-site laparoscopic resection of splenic papillary angioendothelioma in a pediatric patient: a rare case report and review of literature
title_full Transumbilical single-site laparoscopic resection of splenic papillary angioendothelioma in a pediatric patient: a rare case report and review of literature
title_fullStr Transumbilical single-site laparoscopic resection of splenic papillary angioendothelioma in a pediatric patient: a rare case report and review of literature
title_full_unstemmed Transumbilical single-site laparoscopic resection of splenic papillary angioendothelioma in a pediatric patient: a rare case report and review of literature
title_short Transumbilical single-site laparoscopic resection of splenic papillary angioendothelioma in a pediatric patient: a rare case report and review of literature
title_sort transumbilical single site laparoscopic resection of splenic papillary angioendothelioma in a pediatric patient a rare case report and review of literature
topic splenic papillary angioendothelioma
pediatric
laparoscopy
single-site
partial splenectomy
url https://www.frontiersin.org/articles/10.3389/fonc.2025.1571209/full
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