Is Pseudomyogenic hemangioendothelioma a cancer?
In conclusion, the pseudomyogenic hemangioendothelioma is a recently classified low-grade malignant soft tissue tumor of vascular origin [6]. It tends to affect young adults and the majority of cases are diagnosed affecting the skin and deep soft tissue of the extremities.
What causes epithelioid hemangioendothelioma?
What is the genetic cause of EHE? As is the case with many other cancers, EHE is caused by a genetic malfunction, a specific chromosomal rearrangement or translocation (swapping of genetic material) called the ‘WC Fusion. ‘ This discovery was made by dedicated scientists studying the molecular biology of EHE.
Is hemangioendothelioma malignant?
The term hemangioendothelioma describes several types of vascular neosplasms and includes both non-cancerous (benign) and cancerous (malignant) growths. The term has also been applied to those that show “borderline” behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas.
What is Pseudomyogenic hemangioendothelioma?
Pseudomyogenic hemangioendothelioma (PMHE) is a rare vascular tumor that was added to the World Health Organization classification of soft tissue tumors. These tumors have a unique clinical presentation and microscopic appearance as compared to other vascular tumors in the differential diagnosis.
How long can you live with EHE?
Although localized epithelioid hemangioendothelioma can be surgically resected, there is no effective therapy for systemic disease, and mortality from the cancer ranges between 13% and 18% when confined to soft tissue, but life expectancy in metastatic cases is unpredictable and ranges from a couple of months to 15 to …
What is the meaning of hepatoblastoma?
Hepatoblastoma is a very rare cancerous (malignant) tumor that starts in the liver. Symptoms include a lump (mass), swelling, or pain in the belly (abdomen). Diagnosis is done with blood tests, imaging, and biopsy.
Can a hemangioma become malignant?
Because hemangiomas very rarely become cancerous, most do not require any medical treatment. However, some hemangiomas can be disfiguring, and many people seek a doctor’s care for cosmetic reasons. In most cases of hemangioma, treatment does not involve surgery.
What is Kaposiform Hemangioendothelioma?
Kaposiform hemangioendothelioma, or KHE, is a type of tumor that grows in the blood vessels. Blood vessels help move blood around the body. This tumor usually grows just underneath the skin, but it can also grow deeper inside the chest or abdomen.
What is Hemangioendothelioma?
What is epithelioid hemangioendothelioma? Epithelioid hemangioendothelioma, or EHE, is a rare cancer that grows from the cells that make up the blood vessels. This cancer can occur anywhere in the body with the most common sites being the liver, lungs, and bone.
When does pseudomyogenic hemangioendothelioma ( pmhe ) occur in young adults?
Pseudomyogenic hemangioendothelioma (PMHE) is a rarely metastasizing endothelial neoplasm that most commonly occurs in young adults and often presents as multiple discontiguous nodules in different planes of a limb.
Why is wide excision recommended for pseudomyogenic hemangioendothelioma?
SERPINE1 is highly expressed in vascular tissues and acts as the promoter for FOSB expression when fused ( Cancer Genet 2011;204:211 ) Wide excision is recommended as first line ± adjuvant radio or chemotherapy because of the high risk of local recurrence
Is it rare to have retiform hemangioendothelioma?
Likewise, for individuals with retiform hemangioendothelioma, local recurrence is common but metastatic disease is rare. [1] On the other hand, a follow-up study of 30 patients with cutaneous epithelioid hemangioendothelioma showed that 21% of the affected individuals developed systemic metastases, and 17% of these patients died as a consequence.
What kind of treatment is there for hemangioendothelioma?
Treatment depends on the type of hemangioendothelioma present but typically includes surgical excision (removal). Treatment for hemangioendothelioma may depend on the type of hemangioendothelioma present in the affected individual and the risk of recurrence or metastases.