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Pediatric case of the day. Mesenchymal chondrosarcoma

1987, Radiographics

.., . of the Dciy . Pediatric Robert A. Kaufman, M.D. Case Day 1986 Annual Meeting, Richard B. Towbin, Alan History: of the E. Oestreich, William M.D. S. Ball, M.D. M.D. This patient was first found to have a lesion in the left hemithorax in 1983 when a screening chest radiograph was made prior to surgery for appendicitis. Three years later, in I98#{243}, the patient, then 14 years of age, was seen complaining of left sided chest pain after blunt trauma. He had no other symptoms and the physical examination was normal. Images shown below include chest nadiographs from 1983 (Figure 1) and 1986 (Figure 2), and computed tomography from 1986 (Figure 3). The patient was taken to surgery and the lesion was resected. Postoperatively, a large amount of blood accumulated in the left hemithorax, and life-threatening bleeding persisted for a number of days. Figure 1 Figure 2 From the Department of Radiology. University of Cincinnati Medical Center, Cincinnati, Ohio. Address reprint requests to RadioGraphics. Suite IA 822 College Pine Street, Volume of Medicine and Philadelphia. 7, Number 3 Division of Radiology. Children’s Hospital PA 19107. May, #{149} 1987 RadioGraphics #{149} 597 Figure 3A Figure 3B Course The original rib lesion, first noted, in 1983 was thought to represent a benign “chondroma” of the nib and no action was taken. When the patient returned in 1986, the lesion was much larger and surgical evaluation was performed prior to resection. Computed tomography of the lesion showed incomplete calcification of the mass peripherally, with a more regular cantilaginous matrix centrally. The lateral margins of the lesion seemed poorly organized. At surgery, the 598 Radio raphics May, #{149} 1987 Volume #{149} tumor, ribs 2, 3 and 4, and part of the adjacent lung were resected. Postoperatively, coagulation studies, clotting factors, and hepanin levels suggested grossly elevated hepanin was present, yet no anticoagulant had been given. It appears that the tumor itself had liberated a hepanin or hepanin-like anticoagulant substance, a thesis confirmed by careful histologic examination of the tumor. 7, Number 3 Diagnosis: Mesenchymal chondrosancoma Discussion chondrosancoma may appear to have a peniosteal origin. When located in the soft tissues, the following diagnoses are suggested: extraskeletal osteosarcoma, synovial sarcoma, myositis ossificans, and tumonal calcinosis. The histological differential diagnosis is mainly malignant hemangiopenicytoma and Ewing malignant lymphoma; but the lesion must also be differentiated from small cell osteosarcoma. In this particular case, there were areas that histologically resembled hemangiopenicytoma, Ewing tumor, chondroblastoma, and some areas bore a slight resemblance to osteosarcoma. Treatment is radical surgical removal of the pnimany neoplasm. Pulmonary metastases are common. Adjuvant chemotherapy may be used, but its efficacy is not yet clear. The prognosis is poor, and late metastasis is common. Mesenchymal chondrosarcoma is a rare tumor of bone, and is even rarer in the soft tissues. Most of these tumors occur in young adults in the third and fourth decades of life. Radiologically, there is aggressive osteolysis with irregular granular calcifications within the tumor and sometimes poorly defined streaks of ossification. Multicentnic skeletal lesions have been descnibed. Soft tissue tumors also contain granular and irregular calcifications and ossifications. Among chondrosancomas, mesenchymal chondrosancoma is very rare, accounting for approximately 2-4% of chondrosancomas in large series. It is even less common in the soft tissues. There is a slight predilection for females, and the current patient is considerably younger than the usual victim. The granular calcification and poorly defined matrix suggest a malignant cantilaginous tumor, and rarely the mesenchymal References 1. Bertoni F, Picci P. Bacchini P. et al. Mesenchymal 3, Salvador AH. Beabout JW, Dahlin DC. Mesenchymal drosarcoma: Observation on 30 new cases. Cancer 28:605-615. chon- drosarcoma of bone and soft tissues. Cancer 1983; 52:553-541. 2. Dahlin DC. Henderson ED. Mesenchymal chondrosarcoma: Further observation on a new entity. Cancer 1962; 15:410-417. The authors wish to thank Ms. Juanita Volume Hunter 7, Number for secretarial 3 May, #{149} chon1971; assistance. 1987 RadioGraphics #{149} 599