Summary
We report a rare case of Maffucci's syndrome combined with dedifferentiated chondrosarcoma in the right shoulder girdle developing from pre-existing enchondroma. In this case, magnetic resonance imaging was useful in diagnosing dedifferentiated chondrosarcoma before surgery. T2-weighted imaging was used to distinguish between the cartilaginous component and the dedifferentiated one. Histologically, there was enchondroma in the humerus and grade 2 chondrosarcoma in the scapula. Further, the dedifferentiated tumor had three mesenchymal elements: osteosarcoma, malignant fibrous histiocytoma, and fibrosarcoma. This histological heterogenicity may be due to mesodermal dysplasia of Maffucci's syndrome.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Broders AC, Hargrave R, Meyerding HW (1939) Pathological features of soft tissue fibrosarcoma. With special reference to the grading of its malignancy. Surg Gynecol Obstet 69:267–280
Campanacci M, Bertoni F, Capanna R (1979) Dedifferentiated chondrosarcomas. Ital J Orthop Traumatol 5:331–341
Capanna R, Bertoni F, Bettelli G, Picci P, Bacchini P, Present D, Giunti A, Campanacci M (1988) Dedifferentiated chondrosarcoma. J Bone Joint Surg [Am] 70:60–69
Carleton A, Elikington JSTC, Greenfield JG, Robb-Smith AHT (1942) Maffucci's syndrome (dyschondroplasia with haemangiomata). Quart J Med 11:203–228
Dahlin DC, Beabout JW (1971) Dedifferentiation of low-grade condrosarcomas. Cancer 28:461–466
Dahlin DC, Unni KK (1986) Bone tumors. General aspects and data on 8542 cases, 4th edn. Thomas, Springfield, Ill, pp 245–253
Dervan PA, O'Loughlin J, Hurson BJ (1988) Dedifferentiated chondrosarcoma with muscle and cytokeratin differentiation in the anaplastic component. Histopathology 12:517–526
Evans HL, Ayala AG, Romsdahl MM (1977) Prognostic factors in chondrosarcoma of one. A clinicopathologic analysis with emphasis on histological grading. Cancer 40:818–831
Frassica FJ, Unni KK, Beabout JW, Sim FH (1986) Dedifferentiated chondrosarcoma. J Bone Joint Surg [Am] 68:1197–1205
Johnson S, Tetu B, Ayala AG, Chawla SP (1986) Chondrosarcoma with additional mesenchymal component (dedifferentiated chondrosarcoma). I. A clinicopathologic study of 26 cases. Cancer 58:278–286
Lewis RJ, Ketcham AS (1973) Maffucci's syndrome: functional and neoplastic significance. J Bone Joint Surg [Am] 55A:1465–1479
Linberg BE (1928) Interscapulo-thoracic resection for malignant tumors of the shoulder joint region. J Bone Joint Surg 10:344–349
Maffucci A (1881) Di un caso di enchondroma ed angioma multiplo. Mov Med Chir Nap 3:399–412, 565–575
McCarthy EF, Dorfman HD (1982) Chondrosarcoma of bone with dedifferentiation. A study of eighteen cases. Hum Pathol 13:36–40
Mirra JM, Gold RH, Marcove RC (1980) Bone tumors: diagnosis and treatment. Lippincott, Toronto, pp 186–188
Niezabitowski A, Edel G, Roessner A, Grundmann E (1987) Rhabdomyosarcomatous component in dedifferentiated chondrosarcoma. Path Res Pract 182:275–279
Schwartz HS, Zimmerman NB, Simon MA, Wroble RR, Millar EA, Bonfigho M (1987) The malignant potential of enchondromatosis. J Bone Joint Surg [Am] 69:269–274
Sun T, Swee RG, Shives TC, Unni KK (1985) Chondrosarcoma in Maffucci's syndrome. J Bone Joint Surg [Am] 67:1214–1219
Unger EC, Kessler HB, Kowalyshyn MJ, Lackman RD, Morea GT (1988) MR imaging of Maffucci syndrome. Am J Radiol 150:351–353
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Tsuchiya, H., Tomita, K., Yasutake, H. et al. Maffucci's syndrome combined with dedifferentiated chondrosarcoma. Arch Orthop Trauma Surg 110, 269–272 (1991). https://doi.org/10.1007/BF00572886
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00572886