Hemangioendothelioma
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]
Synonyms and keywords: Infantile hemangioendothelioma; Kaposiform hemangioendothelioma; Retiform hemangioendothelioma; Epithelioid hemangioendothelioma
Overview
Hemangioendothelioma is a rare group of benign or malignant vascular neoplasms. Hemangioendotheliomas commonly present with an enlarging mass and have been reported in the head and neck, intestines, lungs, lymph nodes, pleura, retroperitoneum,or stomach. Surgical resection, radiotherapy, and chemotherapy is the treatment of choice for hemangioendothelioma.
Historical Perspective
- Hemangioendothelioma was first described by Sharon Weiss, an American pathologist, in 1986.
Classification
- Hemangioendothelioma may be classified into 4 groups:
- Epithelioid hemangioendothelioma
- Kaposiform hemangioendothelioma
- Retiform hemangioendothelioma
- Infantile hemangioendothelioma
- Type I
- Multiple vascular channels
- Formed by an immature endothelial lining
- Stromal separation from bile ductules
- Type II:
- Disorganized appearance and hypercellular
- There are no bile ductules
Pathophysiology
- The pathogenesis of hemangioendothelioma is characterized by
- There are no genetic mutations associated with the development of hemangioendothelioma.
- On gross pathology, characteristic findings of hemangioendothelioma, include:
- No hallmark features
- Size may range up to 18 cm.
- On microscopic histopathological analysis, characteristic findings of hemangioendothelioma, include:
- Large epithelioid perivascular cells
- Abundant pale eosinophilic cytoplasm
- Cytoplasmic vacuolation (some cells) (also known as "blister cells") - key feature.
- May form lumen and have red blood cells within
- Vesicular nucleus with prominent nucleolus in some cells
- Tuft-like projections into capillaries.
- On immunohistochemistry, characteristic findings of hemangioendothelioma, include:
- Positive CD31
- Positive CD34
- Positive Factor VIII
Causes
- Common causes of hemangioendothelioma, include:
Differentiating hemangioendothelioma from other Diseases
- Hemangioendothelioma must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
- [Differential dx1]
- [Differential dx2]
- [Differential dx3]
Epidemiology and Demographics
- The prevalence of hemangioendothelioma is approximately [number or range] per 100,000 individuals worldwide.
- In [year], the incidence of hemangioendothelioma was estimated to be [number or range] cases per 100,000 individuals in [location].
Age
- Patients of all age groups may develop hemangioendothelioma.
- Hemangioendothelioma is more commonly observed among patients aged [age range] years old.
- Hemangioendothelioma is more commonly observed among [elderly patients/young patients/children].
Gender
- Hemangioendothelioma affects men and women equally.
- [Gender 1] are more commonly affected with hemangioendothelioma than [gender 2].
- The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
Race
- There is no racial predilection for hemangioendothelioma.
Risk Factors
- Common risk factors in the development of hemangioendothelioma, include:
- Oral contraceptives
- Polyvinyl chloride
Natural History, Complications and Prognosis
- The majority of patients with hemangioendothelioma remain asymptomatic for [duration/years].
- Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
- If left untreated, [#%] of patients with hemangioendothelioma may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
- Common complications of hemangioendothelioma include [complication 1], [complication 2], and [complication 3].
- Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with hemangioendothelioma is approximately [#%].
Diagnosis
Diagnostic Criteria
- The diagnosis of hemangioendothelioma is made when at least [number] of the following [number] diagnostic criteria are met:
- [criterion 1]
- [criterion 2]
- [criterion 3]
- [criterion 4]
Symptoms
- Hemangioendothelioma is usually asymptomatic.
- Symptoms of hemangioendothelioma may include the following:
- [symptom 1]
- [symptom 2]
- [symptom 3]
- [symptom 4]
- [symptom 5]
- [symptom 6]
Physical Examination
- Patients with hemangioendothelioma usually may be well-appearing.
- Physical examination may be remarkable for:
- [finding 1]
- [finding 2]
- [finding 3]
- [finding 4]
Laboratory Findings
- There are no specific laboratory findings associated with hemangioendothelioma.
Imaging Findings
- There are no [imaging study] findings associated with hemangioendothelioma.
- [Imaging study 1] is the imaging modality of choice for hemangioendothelioma.
- On [imaging study 1], hemangioendothelioma is characterized by [finding 1], [finding 2], and [finding 3].
- [Imaging study 2] may demonstratedemonstate [finding 1], [finding 2], and [finding 3].
Other Diagnostic Studies
- Hemangioendothelioma may also be diagnosed using [diagnostic study name].
- Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
Treatment
Medical Therapy
- There is no treatment for hemangioendothelioma; the mainstay of therapy is supportive care.
- The mainstay of therapy for hemangioendothelioma is [medical therapy 1] and [medical therapy 2].
- [Medical therapy 1] acts by [mechanism of action1].
- Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
Surgery
- Surgery is the mainstay of therapy for hemangioendothelioma.
- [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of hemangioendothelioma.
- [Surgical procedure] can only be performed for patients with [disease stage] hemangioendothelioma.
Prevention
- There are no primary preventive measures available for hemangioendothelioma.
- Effective measures for the primary prevention of hemangioendothelioma include [measure1], [measure2], and [measure3].
- Once diagnosed and successfully treated, patients with hemangioendothelioma are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].