Working Diagnosis:
Osteosarcoma

Treatment:
Patient was seen by pediatric oncology and began antineoplastic chemotherapy. He wore a sling and avoided activities with fall risk. Repeat imaging showed a new fracture of the proximal humeral diaphysis. Pediatric orthopedic surgery recommended limb sparing local control surgery.

Outcome:
Limb sparing surgery was not possible due to the fracture and swelling. As a result, the patient underwent forequarter amputation. Repeat imaging showed inoperable metastatic disease. He was placed on hospice and succumbed to his illness shortly after.

Author's Comments:
Osteosarcoma, a rare tumor characterized by the production of osteoid by malignant cells, is the most common primary malignant bone tumor in children and young adults. Peak incidence occurs in patients ages 10-19 years old and most commonly affects the long bones. Patients typically present with a gradual and persistent pain that is present for weeks. Pain may be worse with movement but is present at rest and at night. Patients may associate pain with a past minor traumatic event. Once osteosarcoma is suspected the patient should be urgently referred to a multidisciplinary team with experience in childhood cancers.

Editor's Comments:
Osteosarcoma may occur as a sporadic mutation or the result of tumor suppressor malfunction.
Infrequently, it can occur with certain syndromes such as Li Fraumani Syndrome. Patients often present with pain but no specific injury that persists during sleep. Often times, the initial presentation is a non-traumatic fracture due to compromise of the bone by the tumor. Plain radiographs are suggestive of the diagnosis, and biopsy is required for confirmation and to determine tumor histology. Work up should include blood work prior to starting chemotherapy and evaluation for metastasis. The most frequent location is in the lungs. Treatment includes a combination of chemotherapy and surgical resection. Five year survival for those with high grade osteosarcoma is around 60-65% depending on the staging of the tumor and presence of metastasis.

References:
Kumar, V., Abbas, A. K., & Aster, J. C. (2015). Robbins and Cotran pathologic basis of disease (Ninth edition.). Philadelphia, PA: Elsevier/Saunders.

Huvos A. Bone Tumors: Diagnosis, Treatment, Prognosis, 2nd, WB Saunders, Philadelphia 1991.

Sissons HA. The WHO classification of bone tumors. Recent Results Cancer Res 1976;:104.

Durfee, R. A., M. Mohammed, and H. H. Luu. "Review of Osteosarcoma and Current Management." Rheumatol Ther 3.2 (2016): 221-43. Print.

Simpson E, Brown HL. Understanding Osteosarcomas. JAAPA. 2018 Aug;31(8):15-19.

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