Year : 1999 | Volume
: 9 | Issue : 3 | Page : 133--135
Case report: Multifocal osteosarcoma
Gaurav Saigal, Jeevan Vernekar, Sanjay Sardessai
Department of Radiology, Goa Medical College, Goa-403202, India
Department of Radiology, Goa Medical College, Goa-403202
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Saigal G, Vernekar J, Sardessai S. Case report: Multifocal osteosarcoma.Indian J Radiol Imaging 1999;9:133-135
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Saigal G, Vernekar J, Sardessai S. Case report: Multifocal osteosarcoma. Indian J Radiol Imaging [serial online] 1999 [cited 2020 Jun 4 ];9:133-135
Available from: http://www.ijri.org/text.asp?1999/9/3/133/28319
We would like to report a case of multifocal osteogenic sarcoma in a young boy. The presence of multiple osteoblastic lesions suggests the possibility of multicentricity. However in this case, one lesion was larger indicating the origin of the disease process at that site. The presence of pulmonary involvement in the form of multiple nodules favored a diagnosis of unicentric osteosarcoma with metastases. The case is reported because of its unique presentation.
An eighteen-years-old boy presented with a two months history of swelling in the right knee and pain in the lower back. Local examination revealed a non-tender 5.0cm x 5.0cm swelling of bony hard consistency on the medial aspect of the right upper tibia. The movements of the right knee joint were normal. Examination of the lower spine revealed a swelling in the sacral region with some fullness in the lower gluteal cleft. Routine blood examination was within normal limits. The serum alkaline phosphatase levels were elevated.
A radiograph of the tibia revealed dense sclerosis involving the metaphysis of the upper tibia extending into the proximal diaphysis[Figure 1]. Proximally subarticular cortex was involved. The overlying cortex showed discontinuity and spiculated periosteal new bone posteromedially. Thin periosteal new bone was noted along the lateral cortex of the upper tibia. The zone of transition between the normal and abnormal bone was relatively wide. Diffuse soft tissue swelling was seen in the region of the upper leg. The radiographs of both ankles revealed a small focal sclerotic lesion in the right lower tibia [Figure 2]. The radiograph of the pelvis revealed multiple focal areas of bony sclerosis on the iliac aspect of the left sacroiliac joint and ala of the sacrum [Figure 3]. The lumbar vertebrae appeared normal. The radiograph of the sacrum showed a small presacral mass with a bony matrix arising from segments S3 and S4 [Figure 4]. The outline of this extra-osseous mass was ill-defined. The chest radiograph revealed multiple pulmonary nodules of varying sizes distributed in the lung parenchyma bilaterally, suggesting pulmonary metastases [Figure 5]. A CT of the sacrum confirmed the above findings [Figure 6],[Figure 7]. Fine needle aspiration of the upper tibia showed marked hypercellularity with large pleomorphic hyperchromatic cells arranged singly, interspersed with tumor giant cells. The cytological features were suggestive of malignant osteogenic sarcoma.
A lot of controversy exists regarding the entity of multifocal osteosarcomas. Although the occurrence of osteosarcomas at multiple sites is rare, it has been seen. Attempts have been made towards classification of multifocal cases into syndromes. However the etiology of multifocal osteosarcomas is rather varied and considerable disagreement exists regarding the accuracy of any classification system.
One of the earliest attempts at describing these tumors was made by Amstutz in 1969 . He classified multifocal osteosarcomas based on the age of the patient, distribution and symmetry of the lesions and presence or absence of pulmonary metastases. This differentiation by age and symmetry as proposed by Amstutz has been considered as arbitrary and is no longer followed .
It has also been conclusively proved that the presence or absence of pulmonary lesions cannot be used as a single differentiating factor between multifocal osteosarcomas and unicentric metastatic osteosarcomas. This is because rapid appearance of secondary skeletal lesions without pulmonary metastases occurs via the Batson's vertebral venous plexus or lymphatics .
The presently accepted classification divides these lesions into three types .
Unicentric, dominant osteosarcomas with subsequent skeletal metastases. Multiple, metachronous osteosarcomas occurring typically in adolescents and young adults. These are distributed asymmetrically, vary in size and are not necessarily osteosclerotic. They may represent new primary tumors or metastases from a single original neoplasm. Osteosarcomatosis. This is a distinct rare form of disease restricted to children between the ages of six and nine. The lesions appear simultaneously, are bilaterally symmetrical of nearly the same size and densely opaque affecting the metaphyses of long bones. The absence of a single, large predominant lesion is characteristic. Many theories have been proposed to explain this rare entity. Silverman in 1936 proposed that the origin of these tumors was in 'multipotent preosseus tissue of the periosteum' and this theory has found wide acceptance .
The debate on the entity of multifocal osteosarcomas continues. It is however generally agreed upon that multifocal osteosarcomas can be either metachronous or synchronous. The synchronous variety represents a distinct form of disease called osteosarcomatosis characteristically occurring in children with symmetrical metaphyseal involvement with osteosclerotic lesions. All other varieties of multifocal osteosarcomas irrespective of age, presence or absence of metastases can be grouped under the metachronous type representing a dominant lesion with subsequent metastases. The case depicted above is of the metachronous type.
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