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Lymphaticovenous malformation (LVM)

Summary

Lymphaticovenous malformation is a rare benign tumor. This tumor is one of several tumors related to vascular malformation.

The lesion can be very painful.
Surgical removal is curative.
Complete Information on this Tumor
Introduction and Definition: 

Lymphaticovenous malformation (LVM) is a rare benign tumor. We describe a case that occurred in the skin and tissues of the dorsal portion of the forefoot. LVM is one of the combined vascular malformation tumors, a group which includes arterovenous malformation, port win stain, capillary-venous, capillary-lympatic and other tumors.

Incidence and Demographics: 
The tumors can occur in a variety of locations, such as the extremities, heart, external genitalia, craniofacial, periorbital tissues, intrathoracic/chest wall, and others.
Symptoms and Presentation: 

In the case presented, the lesion was very painful to the touch and the patient was unable to walk due to the pain.

Differential Diagnosis: 
Other vascular malformation tumors, such as hemangioma.
Preferred Biopsy Technique for this Tumor: 
Incisional
Histopathology findings: 
The tumor is comprised of multiple enlarged vascular and lymphatic channels in a fatty stroma.
Treatment Options for this Tumor: 
Complex lesions require a multidisciplinary team. Some of these tumors may be treated or managed with compression and/or sclerotherapy. Resectable lesions shgould be excised with the best possible margin.
Preferred Margin for this Tumor: 
Wide
Outcomes of Treatment and Prognosis: 
These lesions can be very infiltrative and therefore extremely difficult to remove. They can cause a variety of problems due to cosmesis and interference with the function of the nearby anatomy. They may be difficult to treat and cause cause continuing pain, dysfunction, and cosmetic problems. Tumors that do not involve important anatomical structures have an excellent prognosis.
Special and Unusual Features: 
Thes tumors are due to disordered proliferation of vascular tissues, which is controlled by a number of genes and related proteins. In general vascualr growth is controlled by paracrine signals using the receptor tyrosine kinases. Down-regulators include regulators are angiostatin, endostatin, and thrombospondin. Up-regulators include vascular endothelial growth factor (VEGF), fibronectin, 5-integrin, vascular endothelial cadherin, and transforming growth factor-1 or TGF-1.