Saturday, March 8, 2008

Bubbly Lesion in the Hand

"Bubbly lesions of the bone."

Hearing that phrase will put me in the Wayback machine, and instantly transport me back a few years, to residency, and FEGNOMASHIC. Positively Proustian, the experience. Bubbly lesions and madeleines, there is a connection there somewhere....

One sees bubbly lesions of the bone on MRI episodically, alas, usually without the conventional radiograph, which has often been done elsewhere. Here is a young adult male, with a bubbly lesion of the ring finger metacarpal:

(click on image to enlarge)

Note that the lesion signal is isointense to hyaline cartilage of all pulse sequences. There is rind-like peripheral enhancement. This appearance is suggestive of an enchondroma, but tissue is necessary for a definitive diagnosis. This is a biopsy-proven enchondroma.

Maffucci's syndrome is a nonhereditary enchondromatoses, first described by Angelo Maffuci:

Angelo Mafucci
(courtesy R. Ciranni)
Virchows Arch 2006; 449:495-497

Here is the illustration from his original paper in 1881, describing his eponymous syndrome in the distal femur:

(courtesy R. Ciranni)
Virchows Arch 2006; 449:495-497

Maffucci was an Italian pathologist who not only described multiple enchondromatosis, but also made important contributions to the understanding of tuberculosis and anthrax. After a long and illustrious career, he succumbed to malaria in 1903.

Ollier described another enchondromatosis 19 years after Maffuci's original description. Both Maffuci and Ollier syndromes are characterized by multiple enchondromas. Patients with Maffucci's syndrome also have multiple hemangiomas and, less commonly, multiple lymphangiomas.

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