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Osteosarcoma in the Preadolescent Filipino Patient

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›ŽœŽ—ŽȱŠœȱ›ŽŽȱ™Š™Ž›ȱŠȱ‘ŽȱŜŖ‘ȱ‘’•’™™’—Žȱ›‘˜™Ž’Œȱœœ˜Œ’Š’˜—ȱ

˜—ŸŽ—’˜—ǰȱ˜ŸŽ–‹Ž›ȱşȬŗŚǰȱŘŖŖşǰȱȱ‘Š—›’Ȭ•Šȱ˜Ž•ǰȱŠ—Š•ž¢˜—ȱ

’¢ǰȱ‘’•’™™’—Žœǯȱ ȱ

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˜››Žœ™˜—’—ȱŠž‘˜›DZȱ Š›ȱǯǯȱŠ—ǰȱǰȱŒȱ

ޙЛ–Ž—ȱ˜ȱ›‘˜™ŠŽ’Œœȱ

‘’•’™™’—Žȱ ޗޛЕȱ˜œ™’Š•ȱ

—’ŸŽ›œ’¢ȱ˜ȱ‘Žȱ‘’•’™™’—ŽœȱŠ—’•Šȱ

ŠȱŸŽ—žŽǰȱ›–’ŠǰȱŠ—’•ŠȱŗŖŖŖȱ‘’•’™™’—Žœȱ

ޕޙ‘˜—ŽDZȱƸŜřŘȱśśŚŞŚŜŜȱ

Š¡ȱ˜DZȱƸŜřŘȱśŘŗŞśřŞȱȱ

–Š’•DZȱŽ‘– Š—ȓœ”¢‹›˜Š‹Š—ǯŒ˜–ǯ™‘

œŽ˜œŠ›Œ˜–Šȱ’—ȱ‘Žȱ›ŽŠ˜•ŽœŒŽ—ȱ’•’™’—˜ȱŠ’Ž—ȱ ȱ

 Š›ȱǯǯȱŠ—ǰ

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ȱž•’žœȱǯȱЕޗ£žŽ•Šǰ

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—Šȱ›’œ’—ŠȱǯȱŽŒŽ—ŽŒŽ˜ǰ

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ȱ–¢ȱ ˜•ŽŠȬ¢ǰ

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•ŒŠœŠ‹Šœǰ

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ȱŠǯȱ’Œ˜›’ŠȱǯȱŽ››Š—˜ǰ

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ŽœŠ›ȱǯȱ’–Š¢žŠ

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ȱŠ—ȱ•‹Ž›ȱŽ›˜–Žȱǯȱž’—˜œ

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؎Œ’˜—ȱ˜ȱŽ’Š›’Œȱ—Œ˜•˜¢ǰȱޙЛ–Ž—ȱ˜ȱŽ’Š›’Œœǰȱ˜••ސŽȱ˜ȱŽ’Œ’—ŽȱŠ—ȱ‘’•’™™’—Žȱ ޗޛЕȱ˜œ™’Š•ǰȱ—’ŸŽ›œ’¢ȱ˜ȱ‘Žȱ‘’•’™™’—ŽœȱŠ—’•Šȱ

řޙЛ–Ž—ȱ˜ȱŠ‘˜•˜¢ǰȱ˜••ސŽȱ˜ȱŽ’Œ’—Žǰȱ—’ŸŽ›œ’¢ȱ˜ȱ‘Žȱ‘’•’™™’—ŽœȱŠ—’•Šȱ

ȱ ȱ

ABSTRACT

Objective. Classic high-grade osteosarcoma is uncommon in preadolescents (≤10 years of age). The possibilities of clinicopathologic differences from the typical adolescent osteosarcoma patient have been raised. We sought to compare the presentation, treatment and survival of this subgroup of patients with published rates in order to determine if there is a need to use a treatment regimen different from that for regular adolescent osteosarcoma patients.

Methods. Records of the University of the Philippines–

Musculoskeletal Tumor Unit (UP-MuST) over a 15-year period (1993–2008) were reviewed and data collected on patients 10 years and younger with biopsy-proven classic high-grade intramedullary osteosarcoma who underwent complete treatment by the Unit. Demographics and survival rates were then compared with published rates for preadolescent and regular adolescent osteosarcoma cases.

Results. There were fourteen patients; (6M:8F; age: 4–10 years).

The most common presentation was a painful mass in the distal femur (8); the tumors most commonly had osteoblastic histology (12). Treatment consisted of neoadjuvant chemotherapy, wide surgical excision through ablation (9) or limb-saving surgery (5), and postoperative chemotherapy. There was a good histologic response (over 90% tumor necrosis) in four patients. Seven patients are ANED (alive no evidence of disease) 25 to 186 months after diagnosis. Five-year survival estimate is 52%, compared to a dismal 5 to 10% 15 years ago.

Conclusion. Clinicopathologic presentation, clinical course, and overall survival in this subgroup of patients are comparable with

published results for both preadolescent and adolescent osteosarcoma patients. There is no need to alter the present treatment regimen for this group of young patients.

Key Words: osteosarcoma, preadolescent, presentation, treatment, survival

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•Šœœ’Œȱ‘’‘Ȭ›ŠŽȱ’—›Š–Žž••Š›¢ȱ˜œŽ˜œŠ›Œ˜–ŠȱžœžŠ••¢ȱ

˜ŒŒž›œȱ ’—ȱ ™Š’Ž—œȱ ’—ȱ ‘Ž’›ȱ œŽŒ˜—ȱ ŽŒŠŽȱ ˜ȱ •’Žǯȱ ŠŠȱ ›˜–ȱ

‘Žȱ —’ŸŽ›œ’¢ȱ ˜ȱ ‘Žȱ ‘’•’™™’—ŽœȮžœŒž•˜œ”Ž•ŽŠ•ȱ ž–˜›ȱ

—’ȱ ǻȬžǼȱ ˜ŸŽ›ȱ Šȱ ŗśȬ¢ŽŠ›ȱ ™Ž›’˜ȱ ǻŗşşřȬŘŖŖŞǼȱ œ‘˜ ȱ Šȱ

™ŽŠ”ȱ’—Œ’Ž—ŒŽȱž›’—ȱ‘ŽȱŽŽ—АŽȱ¢ŽŠ›œǯŗȱ‘’•›Ž—ȱŗŖȱ¢ŽŠ›œȱ

˜•ȱ Š—ȱ ¢˜ž—Ž›ȱ ǻ™›ŽŠ˜•ŽœŒŽ—œǼȱ Š›Žȱ —˜ȱ Šœȱ ›ŽšžŽ—•¢ȱ

ŠŽŒŽȱ ‹žȱ ˜›–ȱ Šȱ ’œ’—Œȱ œž‹œŽȱ ˜ȱ ™Š’Ž—œȱ —˜ȱ ˜—•¢ȱ

‹ŽŒŠžœŽȱ ˜ȱ ™˜œ˜™Ž›Š’ŸŽȱ Œ˜—ŒŽ›—œȱ ˜ȱ •’–‹ȱ •Ž—‘ȱ

’œŒ›Ž™Š—Œ’Žœȱ ‹žȱ –˜›Žȱ ’–™˜›Š—•¢ȱ ‹ŽŒŠžœŽȱ ˜ȱ ‘Žȱ

™˜œœ’‹’•’¢ȱ˜ȱ’Ž›Ž—ŒŽœȱ›˜–ȱ‘Žȱ›Žž•Š›ȱŠ˜•ŽœŒŽ—ȱ™Š’Ž—ȱ

’—ȱ ȱ Ž›–œȱ ȱ ˜ȱ ȱ Œ•’—’ŒŠ•ȱ ȱ ȱ ™›ŽœŽ—Š’˜—ȱ ȱ Š—ȱ ȱ ™›˜—˜œ’œǯȱ ȱ Žȱȱȱ

‘Šœȱ‹ŽŽ—ȱ’–™•’ŒŠŽȱȱŠœȱȱŠȱȱ™›˜—˜œ’ŒŠ˜›ȱȱ’—ȱȱŒŠ—ŒŽ›œȱȱœžŒ‘ȱȱ

Šœȱ ŠŒžŽȱ •¢–™‘˜Œ¢’Œȱ •Žž”Ž–’Šǰȱ —Žž›˜‹•Šœ˜–Šǰȱ Š—ȱ

–Žž••˜‹•Šœ˜–ŠDzȱ‘ŽȱšžŽœ’˜—ȱ ‘Ž‘Ž›ȱ‘’œȱ’œȱ›žŽȱ˜›ȱ—˜ȱ˜›ȱ

˜œŽ˜œŠ›Œ˜–Šȱ‘Šœȱ‹ŽŽ—ȱ›Š’œŽȱ‹¢ȱœ˜–ŽȱŠž‘˜›œǯŘȱ

ŽŸŽ›Š•ȱŽŠ›•¢ȱœž’ŽœřǰŚǰśȱœžŽœȱŠȱ’Ž›Ž—ȱ™›˜—˜œ’œȱ’—ȱ

‘’œȱ›˜ž™ȱ˜ȱ¢˜ž—ȱ™Š’Ž—œǯȱ›ŽŠ–Ž—œǰȱ‘˜ ŽŸŽ›ǰȱ’—ȱ‘ŽœŽȱ

œž’Žœǰȱ  Ž›Žȱ ˜Ž—ȱ ’—‘˜–˜Ž—Ž˜žœȯ—˜ȱ Š••ȱ ™Š’Ž—œȱ

›ŽŒŽ’ŸŽȱ ‘Žȱ œŠ—Š›ȱ Œ˜–‹’—Š’˜—ȱ ˜ȱ Œ‘Ž–˜‘ޛЙ¢ȱ Š—ȱ

œž›Ž›¢ǯȱ —ȱ ‘Žȱ ˜‘Ž›ȱ ‘Š—ǰȱ –˜›Žȱ ›ŽŒŽ—ȱ •’Ž›Šž›ŽŘǰŜǰŝǰŞǰşȱ

›Ž™˜›ȱ —˜ȱ ’Ž›Ž—ŒŽȱ ’—ȱ œž›Ÿ’ŸŠ•ȱ ‹Ž ŽŽ—ȱ ‘Žȱ ™›ŽŠ˜•ŽœŒŽ—ȱ

Š—ȱ Š˜•ŽœŒŽ—ȱ ™Š’Ž—ȱ  ’‘ȱ ˜œŽ˜œŠ›Œ˜–Šǰȱ ‘ŽœŽȱ •ŠŽ›ȱ

Šž‘˜›œȱ Œ˜—Œ•ž’—ȱ ‘Šȱ ‘Ž›Žȱ ’œȱ ‘žœȱ —˜ȱ —ŽŽȱ ˜›ȱ Š—ȱ

Š•Ž›Š’˜—ȱ˜ȱȱœŠ—Š›ȱŒ‘Ž–˜‘Ž›Š™Žž’Œȱ™›˜˜Œ˜•ǯȱ

ž›ȱœž¢ȱ’œȱŠȱŒŠœŽȱœŽ›’Žœȱ˜ȱ™›ŽŠ˜•ŽœŒŽ—ȱ˜œŽ˜œŠ›Œ˜–Šȱ

™Š’Ž—œȱ  ‘˜ȱ ›ŽŒŽ’ŸŽȱ Œ˜–™•ŽŽȱ ›ŽŠ–Ž—ȱ ›˜–ȱ ‘Žȱ Ȭ

žœŒž•˜œ”Ž•ŽŠ•ȱ ž–˜›ȱ —’ȱ Šȱ ‘Žȱ ‘’•’™™’—Žȱ ޗޛЕȱ

˜œ™’Š•ǯȱ‘Ž’›ȱ™›ŽœŽ—Š’˜—ǰȱ™Š‘˜•˜’ŒȱŽŠž›Žœǰȱ›ŽŠ–Ž—ǰȱ

Š—ȱ œž›Ÿ’ŸŠ•ȱ ›ŠŽœȱ  ’••ȱ ‹Žȱ ŽœŒ›’‹Žȱ Š—ȱ Œ˜–™Š›Žȱ  ’‘ȱ

™ž‹•’œ‘Žȱ›Ž™˜›œȱ˜—ȱ˜œŽ˜œŠ›Œ˜–Šȱ’—ȱ‹˜‘ȱ‘Žȱ™›ŽŠ˜•ŽœŒŽ—ȱ

Š—ȱŠ˜•ŽœŒŽ—ȱ™Š’Ž—ǯȱ ȱ

Ž‘˜œȱ

‘Žȱ ŠŠ‹ŠœŽȱ ˜ȱ ‘Žȱ ȬžœŒž•˜œ”Ž•ŽŠ•ȱ ž–˜›ȱ —’ȱ ǻȬžȱ—’ǼȱŒ˜—Š’—œȱŠ••ȱ™Š’Ž—œȱœŽŽ—ȱ‹¢ȱ‘Žȱ—’ȱ›˜–ȱ

’œȱ ŽœŠ‹•’œ‘–Ž—ȱ ’—ȱ ŗşşřȱ ˜ȱ ‘Žȱ ™›ŽœŽ—ǯȱ••ȱ ™Š’Ž—œȱ  ’‘ȱ Šȱ

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