Presentation
Large left forearm swelling, tender. Exclude compartmental syndrome.
Patient Data
Extensively globular, complex tumor containing heterogeneous solid and hypoechoic fluid pockets and macro to micro-calcification foci some of which spike and lobulate from the radio-ulnar diaphyses with resultant extrinsic splaying of the flexor pollicis longus and the flexor digitorium profundus musculature is noted. There is regional subcutaneous tissue edema with engorged distal basilic and cephalic veins and the radial-ulnar arterial systems. No thrombosis.
Diffusely dramatic osseous tumoral involvement at the proximal to the medial radial-ulnar diaphyses with numerous osteoblastic extensions into the soft tissues, almost reminiscing the sunrays and or sunburst appearance. There is surrounding subcutaneous tissue edema.
The posterior-anterior erect chest radiograph exhibits focal radiodense right proximal perihilar opacity with gently lobulated peripheral borders.
Contrast-enhanced CT chest characterization (images not shown), revealed: Large poorly enhancing, and partially calcified lesions at the superior, middle, and posterior right mediastinal and hila regions and a right upper posterior partially calcified pleural mass; with the CECT findings ruling in a possibility of metastatic nodes and a likely partially calcified metastatic pleural mass.
Histopathology report:
Specimen: Left forearm.
Gross: Above elbow amputation- 40cm length. Other dimensions- 28x16cm. Cut surface, large, hemorrhagic, necrotic bone tumor, 6cm from the proximal margin. Representative sections processed in cassettes (1-10)-tumor.
Microscopy: Tumor of variegated pattern with areas of fibrous dysplasia identified. Large areas of the lobulated tumor with bland fibroblastic proliferation, exhibiting minimal atypia and low mitoses, <5/10HPF, admixed with new bone islands. Tumor-associated with extensive necrosis, hemorrhage, and bone destruction.
Diagnosis: Left arm, above elbow amputation, malignant transformation of fibrous dysplasia with a large component of low-grade osteogenic sarcoma.
Case Discussion
Operatively, above-the-elbow amputation was done, and subsequent histopathological findings favored a low-grade osteogenic sarcoma primarily attributed to the malignant transformation of fibrous dysplasia. Attendant mediastinal, pleural, and hila lesions visualized may portend early distant metastases.
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