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(Circulation. 2001;104:1693.)
© 2001 American Heart Association, Inc.

Images in Cardiovascular Medicine

Maffucci’s Syndrome

M. Jermann, MD; K. Eid, MD; T. Pfammatter, MD; R. Stahel, MD

Departments of Oncology, Traumatology, and Radiology, University Hospital, Zurich, Switzerland.

Correspondence to Monika Jermann, MD, Department of Oncology, University Hospital, Ramistrasse 100, 8091 Zurich, Switzerland. E-mail monika.jermann{at}dim.usz.ch

A 30-year-old woman presented with painful hemangiomas (Figure, A and B) and enchondromas (Figure, C) in her arms, predominantly in the left hand. The hemangiomas had existed since childhood. They were slowly increasing in size, especially the girth of the left thumb. Multiple resections of enchondromas and transarterial carpal embolizations of the hemangiomas with polyvinyl alcohol particles had been performed during the previous 9 years. At the age of 22, the small finger of the left hand was removed. At the age of 25, a pathological fracture of the left humerus occurred due to an enchondroma.

View larger version (79K): [in this window] [in a new window]   A, Left hand of patient showing extensive hemangiomas. The fifth finger had to be removed 8 years previously. B, Radiograph of the left hand showing multiple calcifications within the hemangiomas (eg, first finger, base of fifth finger) and multiple enchondromas (eg, metacarpophalangeal joint of the fourth finger, proximal and distal interphalangeal joints of second through fourth fingers). C, Arterial angiograph of the left hand showing multiple hemangiomas.

A contour reduction and partial excision of the most important nodules was performed. Serum levels of vascular endothelial growth factor and basic fibroblast growth factor were elevated 2-fold and 7-fold, respectively. The patient is in good clinical health and works part-time as a secretary.

Maffucci‘s syndrome is a rare nonhereditary mesodermal dysplasia. It consists of multiple hemangiomas of the soft tissue and multiple enchondromas, which are most often found in the phalanges and long bones. The bone and vascular lesions exist at birth or occur during childhood and may be progressive. The syndrome can be associated with benign or malignant tumors (goiter, parathyroid adenoma, pituitary adenoma, hemangioendothelioma, adrenal tumor, ovarian tumor, chondrosarcoma, breast cancer, astrocytoma).

Footnotes

The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospotal and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.

Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MCI–267, Houston, TX 77030.

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