Case Author(s): Charles Pringle, M.D., Mike Roarke, M.D., and Keith Fischer, M.D. , 11/16/95 . Rating: #D3, #Q4

Diagnosis: Maffucci's syndrome

Brief history:

History witheld.


Anterior and posterior delayed images in two intensity settings.

View main image(bs) in a separate image viewer

View second image(bs). Delayed images of the right lower extremity

View third image(xr). Anterior view right tibia and fibula

View fourth image(mr). single axial T2 weighted image through the proximal tibia and fibula

Full history/Diagnosis is available below

Diagnosis: Maffucci's syndrome

Full history:

26-year old woman with history of Maffucci¹s syndrome (nonhereditary enchondromatosis and multiple soft tissue cavernous hemangiomas), for routine follow-up examination.


20.7 mCi Tc-99m MDP


There is increased activity in the soft tissues of the lower extremities on the immediate images consistent with hemangiomas in these areas. See plain films. On delayed images, multiple focal areas of abnormal tracer accumulation throughout the skeleton, not significantly changed since the previous study of 4-19-94 (not shown). These lesions correspond to multiple enchondromata. Also, bowing of bilateral forearms and lower legs, without change.


This patient undergoes yearly follow-up examinations to evaluate for malignant transformation of the enchondromata.

References: Donald Resnick, M.D. Bone and Joint Imaging. 1989



Major teaching point(s):

Maffucci¹s syndrome is a nonhereditary, rare cartilaginous dysplasia resulting in multiple enchondromas and soft tissue hemangiomas. A unilateral skeletal distribution is present in approximately 50% of cases. The metacarpals and phalanges of the hands are most frequently involved. When the extremities are involved, there may be resultant limb length discrepancies. Malignant transformation is the most severe complication and generally occurs in adults after the age of 40 years. The approximate frequency is 20%. There is potential for sarcomatous degeneration of both bone and soft tissue lesions. Chondrosarcoma is the most common, but other reported neoplasms include hemangiosarcoma, lymphangiosarcoma, and fibrosarcoma.

Differential Diagnosis List

Based on the bone scintigraphy alone, without any history, a differential diagnosis would include metastases or a metabolic bone disease.

ACR Codes and Keywords:

References and General Discussion of Bone Scintigraphy (Anatomic field:Skeletal System, Category:Normal, Technique, Congenital Anomaly)

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Case number: bs042

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