Adamantinoma of the posterior tibial cortex in a child

Adamantinoma : The first report from Syria

“…Adamantinomas represent 0.1 to 0.5 % of all primary bone tumors. In the huge majority of cases, they involve the anterior cortex of the tibial diaphysis [1] . In their initial stage they are located at the affected bone's cortex and they can be asymptomatic or have a very slight symptomatology, while during the advanced stage they affect the perilesional soft tissue and the bone marrow, and symptoms such as pain and limb deformity increase [5] .…”

“…In histopathological terms, an adamantinoma is a tumor characterized by the presence of a variable proportion of epithelial cells within osteofibrous tissue. There are two histological sub-types: osteofibrous dysplasia-like adamanti-noma (predominance of the osteofibrous component) and classic adamantinoma (predominance of the epithelial component) [1] . Four histological patterns have been described for classic adamantinoma: basaloid, spindle, tubular, and squamous, of which the basaloid and tubular patterns are the most common [2] .…”

“…At a more advanced stage, the tumor has a ‘soap bubbles’ appearance. Peripheral osteosclerosis is common, and cortical destruction and aperiosteal reaction may also be associated [1] . In our case the lesion was involved to the anterior cortex of the tibia but without periosteal reaction.…”

“…MRI is the firstline examination to perform to determine the extension of the tumor into the bone marrow and neighboring structures. Two types of presentation have been described: a solitary or bifocal lobulated lesion or many small nodules grouped into one or more foci [1] . Adamantinoma metastasizes in about 15 to 30 % of cases by both hematogenous and lymphatic routes to other parts of the body, usually to the lungs or lymph nodes; bone and abdominal viscera make up a minority [3] .…”

“…It is a biphasic locally aggressive or malignant tumor characterized by a variety of morphological patterns, with a variable epithelial component within a bland osteofibrous component. The first case report is attributed to Maier in the year 1900 [1] . In 1913, Fisher reported the first tibia adamantinoma [2] .…”