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Hemangioendothelioma

Name  l  Define  l  Risk  l  Detect  l  Treatment  l  Stage/Grade  l  Followup  l  Mets/Recur  l  Link

Names - Synonyms

Hemangioendothelioma, haemangioendothelioma, cellular hemangioma, histiocytoid hemangioma, and angioendothelioma are are phrases that may be used interchangeably.

Types, that may be considered benign, borderline, or malignant, that I found references to during my research, include:   infantile hepatic, composite, cutaneous, Dabska's tumor, endovascular papillary, epithelioid, histiocytic, Kaposiform, mammary, polymorphous, pulmonary epithelioid, sarcoma-like, spindle cell, and retiform.

Definition

Neoplasm of vascular origin, characterized by proliferation of endothelial cells in and about the vascular lumen.  Some forms of this disease are considered benign, others borderline, still others are considered malignant.  Tumors of this type can develop in soft tissues or in internal organs, such as the liver or lungs. Hemangioendotheliomas may invade nearby tissues or metastasize.

Classifications that I have found for hemangioendothelioma would include:

Benign:
Benign infantile hemangioendothelioma - aka cellular hemangioma of infancy
Hemangioendothelioma - aka angioendothelioma
Kaposiform hemangioendothelioma - may be locally aggressive
Epithelioid hemangioendothelioma, not otherwise specified
Masson's vegetant intravascular hemangioendothelioma - intravascular endothelial hyperplasia
Spindle cell hemangioendothelioma - aka spindle cell angioendothelioma, spindle cell hemangioma

Low Grade Malignancy or Borderline:
Endovascular papillary angioendothelioma - aka Dabska tumor
Retiform hemangioendothelioma
Composite hemangioendothelioma
Polymorphous hemangioendothelioma

Malignant:
Epithelioid hemangioendothelioma, malignant
Malignant Hemangioendothelioma - aka Hemangioendothelial sarcoma

Risk Factors - Causes

There are no known specific risk factors for these diseases.

Detection

Angiography, CT scan, Xray, MRI, and blood tests may all be used to diagnose this disease.  

Treatment

Several treatments are used, depending on the severity of the tumor.  For many benign tumors, a 'wait and see' stance will be taken or steroids will be administered.  Wide surgical excision may be used to remove borderline or malignant tumors.  Transplant surgery may be used for hepatic involvement.  Radiotherapy, radiofrequency ablation, vascular embolization, and chemotherapy have all been used for this disease.

Stage - Grade

Many forms of hemangioendothelioma are considered intermediate in grade, between hemangioma and hemangiosarcoma.   Grade I - abundant vascular channelslined by only mildly atypical neoplastic endothelial cells and rare mitoses. Grade II - reduced numbers of neoplastic vascular channels, prominent nuclear atypia, and easily identifiable mitoses.  Grade III - dedifferentiation to epithelial areas resembling metastatic adenocarcinoma, fewer vascular channels, more striking nuclear atypia, including tumor giant cells, numerous mitotic figures, and tumor necrosis.

Stage I and Stage II references were found.

Suggested Followups

Follow-up requirements will be based solely on the type of hemangioendothelioma diagnosed.  You may need regular blood tests for platelet counts.  Several forms of hemangioendothelioma will require lifelong follow-up exams.

Metastasis - Recurrence

Tumors with significant cellular atypia and mitotic activity are associated with more aggressive clinical behavior.  A large percentage of hemangioendotheliomas may recur at the original site, or in close proximity.  Almost a third of epithelioid hemangioendotheliomas develop metastases in regional lymph nodes or in the lungs, liver or bones.

Link

Hemangioendothelioma Support Forum