British Journal of Radiology 75 (2002),853-854 © 2002 The British Institute of Radiology
A boy with bilateral hip pain
T Yamamoto, MD
and
I Fujita, MD
Department of Orthopaedic Surgery, Kobe University School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan
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Introduction
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A 16-year-old boy presented with a 3-month history of bilateral hip pain. He was previously healthy. Serum alkaline phosphatase was 1855 IU l-1 (normal 100–303 IU l-1). Radiographs of both femora and hips and the right knee are shown (Figure 1
) as well as a 99Tcm bone scan (Figure 2). An open biopsy of both femora was performed. What is your diagnosis?
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Figure 1. (a, c) Anteroposterior radiographs of both femora showing multiple sclerotic lesions. (b) Anteroposterior radiograph of the right knee reveals a heavily ossified lesion in the distal femur and multiple spotty osteosclerosis in the proximal metaphysis of the tibia (arrows).
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Figure 2. 99Tcm scintigraphic scan showing multiple increased uptake of isotope in the axial and appendicular skeleton.
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Answer
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Radiographs of both femora (Figure 1a, c
) show diffuse, dense sclerosis, especially in the lower end of the right femur where there is periosteal reaction and extension of the bone lesion into the adjacent soft tissues. There is slight patchy sclerosis in the upper end of the right tibia (Figure 1b, arrows). The radionuclide bone scan (Figure 2) shows widespread increased uptake within the skeleton, especially in the right femur.
Biopsy specimens obtained from both femora showed tumour tissue consisting of atypical spindle and pleomorphic cells as well as heavy ossification. A diagnosis of multicentric osteosarcoma was made. Despite intensive chemotherapy, each lesion continued to enlarge. The patient developed pulmonary metastases 3 months post-operatively and died from metastatic disease 8 months after initial diagnosis.
Osteosarcoma is the most common skeletal malignancy in childhood and adolescence. Multicentric osteosarcoma is a rare type of osteosarcoma. Approximately 20% of all primary malignant bone tumours are osteosarcomas and only 1–2% of cases have multicentric tumours [1]. Patients with multicentric osteosarcoma synchronously or metachronously have multiple skeletal foci of osteosarcoma, usually with no pulmonary involvement [2–4]. Radiographically, these are often diffuse, dense sclerotic lesions in the metaphyses of long bones [2–4]. There have been two hypotheses for the pathogenesis of multicentric osteosarcoma. Some agree with the multicentric origin [3], whilst others consider the condition to be early bone-to-bone metastasis [2].
Amustuz [4] classified the synchronous and metachronous multicentric osteosarcoma into three types. Type 1 patients usually have multiple synchronous osteosarcomas in the metaphyses of long bones. This type occurs in young patients and carries an extremely poor prognosis. The current case corresponds to this type. Type 2 patients are usually adults and have synchronous osteosarcoma in the axial skeleton. Type 3 patients have metachronous osteosarcomas.
The radiographic differential diagnosis of multicentric osteosarcoma includes metastatic carcinoma, diffuse skeletal angiomatosis and multifocal chronic recurrent osteomyelitis. Metastatic carcinoma that give rise to osteoblastic lesions, such as breast and prostatic cancers, would be extremely unusual in young patients. Skeletal angiomatosis may radiographically show multiple, blastic, lytic or mixed foci in the axial and appendicular skeleton [5]. Chronic recurrent multifocal osteomyelitis is a rare condition in childhood and adolescence with unknown aetiology [6]. This disease involves different osseous sites and may be associated with palmar and plantar pustulosis. Bacterial cultures of affected bone marrow have been reported to be negative. Radiographs of affected bones may show lytic, mixed lytic-sclerotic, or pure sclerotic lesions [6].
Patients with multicentric osteosarcomas may present as polyarthralgia because of the metaphyseal distribution of the bony lesions.
Received for publication January 2, 2001.
Revision received April 17, 2001.
Accepted for publication April 20, 2001.
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References
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- Unni KK. Osteosarcoma. In: Unni KK, editor. Dahlin's bone tumors. General aspects and data on 11087 cases. Philadelphia, PA: Lippincott-Raven, 1996:143–96.
- Daffner RH, Kennedy SL, Fox KR, Crowley JJ, Sauser DD, Cooperstein LA. Synchronous multicentric osteosarcoma: the case for metastasis. Skeletal Radiol 1997;26:569–78.[Medline]
- Singh AD, Scudder IB. Multiple osteogenic sarcomas. Report of a case. J Bone Joint Surg 1965;47:542–47.
- Amstutz HC. Multiple osteogenic sarcoma: metastatic or multicentric? Report of two cases and review of literature. Cancer 1969;24:923–31.[Medline]
- Wallis LA, Asch T, Maisel BW. Diffuse skeletal hemangiomatosis. Report of two cases and review of literature. Am J Med 1964;37:545–63.[Medline]
- Demharter J, Bohndorf K, Michl W, Vogt H. Chronic recurrent multifocal osteomyelitis: a radiological and clinical investigation of five cases. Skeletal Radiol 1997;26:579–88.[Medline]
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