Age/sex: 14-year-old male
Size: 14.3 x 8.5 x 2.5 cm
Two fingers have been cut longitudinally to show several irregularly shaped white areas (W) where there should be darker bone marrow. Some are clearly continuous with normal cartilage (short arrow).
Enchondromas
Enchondromas are cartilaginous tumors that grow within rather than on the surface of a bone. They are usually found in the hands or feet and can be solitary or multiple. The latter situation―Ollier disease―is rare, affecting only about 1 in 100,000 individuals in North America. The condition in which multiple enchondromas are associated with hemangiomas (benign blood vessel tumors) in soft tissue and viscera is even rarer and is called Maffucci syndrome.
Patients have palpable, hard nodules on a finger or hand. Although usually present at birth, these often do not become clinically evident until childhood. The affected bones can be short and somewhat deformed. Solitary enchondromas are almost always benign. However, those that develop in one of the two-named syndromes not uncommonly undergo malignant transformation (chondrosarcoma). The syndromes are named after French surgeon Louis Léopold Ollier and the Italian pathologist Angello Maffucci; each described their eponymous condition in the late 1800s.
Below: The hand of a patient with Maffucci syndrome.
Source: Bluish swellings on the dorsum of the left hand. Hand of individual with Maffuci’s syndrome. From “Mafficci’s syndrome associated with hyperparathyroidism,” by AG Rao, D Indira, and TN Rekha. (2011), Indian J Dermatol, 52(2), 203-205. doi: 10.4103/0019-5154.80418
