Davi Gabriel Bellan, MD; Reynaldo Jesus-Garcia, MD, PhD; Maria Teresa de Seixas Alves, MD, PhD; Marcelo de Toledo Petrilli, MD; Antonio Sérgio Petrilli, MD, PhD; Marcos Korukian, MD; Dan Carai Maia Viola, MD; Murillo Ferri Schoedl, MD; Andreza Almeida Senerchia, MD.

JBJS Case Connect, 2013 Jan 23; 3 (1): e6 . http://dx.doi.org/10.2106/JBJS.CC.L.00004


 

Extraskeletal osteosarcoma (ESOS) is a well-defined and rare soft-tissue neoplasm characterized by osteoid production with no association with bone (1). It represents 1% of all soft-tissue sarcomas and 4% to 5% of all osteosarcomas (1-3) .

ESOS mainly arises in the deep soft tissue of the lower limbs and is extremely uncommon in young people. The median age of affected patients in most series has been in the fifth to seventh decades of life; the number of reported pediatric cases is low (3-9). The prevalence of ESOS in the upper extremity is between 15% and 23%; 7% of these cases have occurred in the hand (1,3,4,10,11).

Previous series, primarily including patients treated with surgery alone, have reported five-year survival rates ranging from 24% to 50%, with high rates of distant metastasis (2,4,12-14). Several reports have found no difference in overall or event-free survival between patients treated with resection and those treated with amputation (5,6). Uncertainty remains as to whether ESOS should be treated with use of neo or adjuvant chemotherapy (5-7,15).

We report the clinical and pathological findings of an unusual case of an ESOS arising from the hypothenar muscles of the left hand in an eighteen-year-old woman. The patient was informed that data concerning the case would be submitted for publication, and she provided consent.


Case Report

An eighteen-year-old woman presented with a rapidly enlarging, painless mass of the left hand. The patient had noticed the mass four months earlier. She reported no injury to the hand or prior history of radiation therapy. She denied any health problems and family history of cancer. Physical examination confirmed a firm, immobile, painless mass, …


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