- Hemangioendothelioma and angiosarcoma are a group of primary malignant vascular tumors of the bone, which are extremely rare
- Common locations: femur, tibia, pelvis and vertebra
- Age group: between 4th and 5th decade
- Imaging appearance: lytic lesion without sclerotic border, multilocular, bone expansion and laminated periosteal reaction
- Common differential diagnosis = aneurysmal bone cyst, simple bone cyst
- A 50 years old female presented with pain and swelling at left foot with no history of trauma. On clinical examination local tenderness at heal was present.
- Radiograph taken in oblique (fig 1) positions, showed a well defined, lobulated lytic lesion with a few internal septa in the anterior part of Calcaneum. The lesion extends and breaches the medial and superior cortex of calcaneum
- MRI study of foot and ankle using 1.5T Siemens Magnetom Essenza machine, which included T1, T2, PD and T2 fat suppressed axial, sagittal and coronal sequences were performed. On PDW sagittal and T1 coronal images the lesion appears well defined, lobulated , with few internal septas in the anteroinferior part of calcaneum and breaches the medial and superior cortex and involves the adjacent myofascial planes. The lesion is hyperintense on PDW images [figure 2 A] and hypointense in T1W coronal images [figure 2 B] Tibiotalar joint shows minimal joint effusion.
- On histopathological examination of the material obtained after intraoperative curettage of calcaneum findings were in favor of tumor of vascular origin.
- On the basis of the clinical, radiological and histopathological findings, presumptive diagnosis of Calcaneal Hemangioendothelioma was considered.
- Hemangioendothelial sarcoma includes hemangioendothelioma and angiosarcoma, which encompasses a group of primary malignant vascular tumors in bone that vary from the malignant capillary and cavernous blood vessel formation to the proliferative endothelial sarcomas. Hemangioendothelioma is a tumor of blood vessels, in which endothelial cells are seen as predominant cell
- Primary malignant vascular tumors in bone are extremely rare(less than 1% of all bone tumors). It can occur in all age groups; however most of the patients are between 4th and 5th decades of life. Most commonly affected bone is Femur (16%), followed by Tibia(14%), pelvis(12%), vertebra(10%). Other rare sites are foot, hand, forearm bones and clavicle. The patient may not experience any specific symptoms or signs. Patient may present with Pain or occasionally swelling. Hemangioendothelioma shows multicentricity of lesions in the bones of the same extremity. On Radiographs, the solitary lesion is well circumscribed, lytic with no surrounding sclerosis or matrix mineralization, which shows internal septa which may be scant or incomplete. Occasionally it may show multilocular appearance. The tumor causes expansion, thinning and erosion of the cortex and often associated with a mild laminated periosteal reaction.
- On MR imaging, the lesion appears well defined, multilocular which is hyperintense on T2W and PDW images and hypointense on T1W images with internal septa. The lesion involves the surrounding soft tissue and involvement of multiple bones can occur.
- The etiology of unicameral bone cysts of the calcaneum is an enigma, just as it is with these lesions in other bones. Popular theories regarding the origin of bone cysts have been related to the long bones and the juxtaposition of the cyst to the growth plate
- Most commonly considered differential diagnoses are Simple Bone Cyst, Aneurysmal Bone Cyst. That can be differentiated by moth eaten erosion pattern and irregular margins of Hemangioendothelial sarcoma.
1. Ackerman LV, Spujat HJ. Tumors of bones and cartilage. Atlas of tumor pathology. Armed Forces Institute of Pathology, Washington, DC, 1962
2. Jaffe HL, Lichtenstein L. Solitary unicameral bone cyst with emphasis on the roentgen picture. Arch Surg 1942;44:1004-1025
3. Smith RW, Smith CF. Solitary unicameral bone cyst of the calcaneum: a review of twenty cases. J Bone Joint Surg Am 1974;56:49-56.