Case Analysis Of Complex Vascular Tumor With Papillary Intralymphatic Angioendothelioma And Literature Review Of Papillary Intralymphatic Angioendothelioma

Background:Papillary intralymphatic angioendothelioma（PILA）is a rare vascular tumor,which can occur in both children and adults,and is more common in children.This disease is also called hobnail haemangioendothelioma,and Dabska tumor.They are classified as intermediate occasional metastatic vascular tumors in the WHO Classification of Bone and soft tissue tumors（5th edition）（ICD-O code:9135x1x ICD11 code:XH4SY7）^[1].Papillary intralymphatic angioendothelioma is extremely rare,and the number of cases related to papillary intralymphatic angioendothelioma is relatively small,and the related research data and diagnostic experience need to be further accumulated.Based on the analysis of the existing case data,summarizing its clinicopathological features will play an important role in the diagnosis and treatment of the disease.Objective:A case of complex vascular tumor with papillary intralymphatic angioendothelioma was analyzed,and the literature of papillary intralymphatic angioendothelioma was reviewed.the clinical manifestations,pathological features,treatment and prognosis were studied.Methods:This paper reports a case of complex vascular tumor with papillary intralymphatic angioendothelioma in the right thigh treated by operation in our hospital,and reviews the literature related to papillary intralymphatic angioendothelioma at home and abroad combined with the clinical history,examination report,imaging data and pathological diagnosis of patients with papillary intralymphatic angioendothelioma.To summarize the clinicopathological characteristics of papillary intralymphatic angioendothelioma,so as to promote the development of pathological diagnosis,clinical treatment and prognosis evaluation of papillary intralymphatic angioendothelioma.Results:All the literatures related to papillary intralymphatic angioendothelioma at home and abroad were searched,and a total of 97 cases were found（including this case）.The main characteristics of PILA are summarized as follows:（1）The age of onset:the patient has a large age span,ranging from 2 days to 83 years old,the average age of the patient is 28 years old,and the median age is 22 years old.（2）Population distribution:papillary intralymphatic angioendothelioma can be seen in both children and adults,and papillary intralymphatic angioendothelioma is more common in children than in young,middle-aged and old people.（3）Gender difference:so far,there are 53 female patients and 44 male patients,and female patients are slightly more common.（4）The location of papillary intralymphatic angioendothelioma can be found not only in limbs,torso,head and neck,but also in breast,spleen,small intestine,testis,bones and other parts of the body.（5）Clinical features:it often occurs in the skin or subcutaneous painless mass or mass,showing slow growth,and discoloration may occur in those covered with purple skin;（6）Naked eye observation:the maximum diameter of the tumor is 0.7 cm 50 cm,the average maximum diameter of the tumor is 6.7 cm,and the median is 4.1 cm;mostly located in the subcutaneous and dermis,the section is mostly pink,yellowish or light blue,the perimeter is unclear,and some may have cystic degeneration or surface ulcers.（7）Pathological diagnosis:vascular hyperplasia in the dermis was seen under light microscope,papillary process was seen in the lumen,and enlarged cubic or columnar endothelial cells were lined with match head or shoe nail protruding to the lumen,and the central papillary structure was glass-like axis,mitosis was rare;immunohistochemistry:tumor endothelial cells could express CD31,CD34,VEGFR-3 and FⅧ;Weibel-Palade bodies could be seen under electron microscope.At the same time,PILA should be differentiated from reticular hemangiendothelioma,shoe nail hemangioma and compound hemangioendothelioma.（8）At present,the main recommended treatment is local extensive resection,regional lymph node dissection is feasible for patients with suspected lymph node metastasis,and other treatments such as radiotherapy,chemotherapy or hyperthermia are given if necessary.（9）Follow-up time:15 days to 17 years,the average follow-up time was 43 months.Conclusion:Papillary intralymphatic angioendothelioma is very rare and belongs to intermediate occasional metastatic type tumors.It can be diagnosed according to histopathological morphology,immunohistochemical results and electron microscopic ultrastructure.At present,extensive local resection is the first choice,regional lymph node dissection is feasible for patients with suspected lymph node metastasis,and other treatments such as radiotherapy,chemotherapy or thermotherapy are given if necessary.At the same time,health education for patients should be strengthened and long-term follow-up should be done.