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SRPS PS - Plastic Surgery Internal

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unknown. The lesions might arise de novo or might<br />

occur secondary to a benign process. Osteosarcomas in<br />

general tend to occur more frequently in association<br />

with irradiated bone, Paget’s disease, fibrous dysplasia<br />

of bone, giant cell tumor, solitary enchondroma,<br />

multiple enchondromatosis, and multiple<br />

osteochondromas.<br />

Radiographically, the borders of an osteosarcoma<br />

are indistinct, but the lesion invariably involves the<br />

cortex and generally transgresses it. Often, a large<br />

contiguous soft-tissue mass is present. A combination<br />

of destructive and proliferative new bone usually is<br />

present, showing a streaked texture and a<br />

characteristic sunburst pattern. Histologically,<br />

osteosarcoma has a typical spindle-shaped cell pattern.<br />

Treatment has changed from amputation to excision<br />

with a wide margin plus adjuvant therapy. In a study<br />

presented by Okada et al., 320 local control was achieved<br />

in five of six patients by using this protocol; one<br />

patient died as a result of metastatic disease.<br />

Chondrosarcomas—Chondrosarcomas are<br />

uncommon in the hand, where they occasionally are<br />

associated with osteochondromas and, to a lesser<br />

degree, with multiple enchondromatosis, 323,324 although<br />

the vast majority of cases include no preexisting<br />

lesion. 325,326 Chondrosarcomas characteristically occur in<br />

older patients (60–80 years) in the epiphyseal area of<br />

the proximal phalanx or metacarpal. The clinical<br />

course is slow, and metastasis is late. 327,328 The tumor<br />

presents as a progressively painful large mass near the<br />

metacarpophalangeal joint. Treatment of choice is<br />

amputation or ray resection. Histological interpretation<br />

of cartilaginous lesions of the hand is difficult, and<br />

clinical and radiological appearance (bone expansion,<br />

lytic areas of bone destruction, soft-tissue swelling)<br />

often are more reliable indicators of malignancy.<br />

Prognosis is good if metastasis has not occurred. 325,326<br />

Soft-Tissue Sarcomas<br />

Rosenberg and Schiller 302 have provided an excellent<br />

review of soft-tissue sarcomas of the hand. Soft-tissue<br />

sarcomas are an uncommon but important group of<br />

hand tumors. They tend to occur in young patients, are<br />

innocuous in presentation, often leading to an incorrect<br />

diagnosis, and have protracted clinical courses. They<br />

<strong>SR<strong>PS</strong></strong> Volume 10, Issue 25, 2009<br />

are prone to local recurrence, have an unusually high<br />

incidence of lymphatic spread and regional node<br />

metastases, and often metastasize systemically late in<br />

their course. Deep tumors that are firm and are 5 cm or<br />

larger should be considered to be possible sarcomas<br />

until proven otherwise. 313 CT and MRI often are used<br />

to define the anatomy. Standard treatment is wide<br />

surgical excision with or without adjunctive<br />

radiotherapy and/or chemotherapy. The prognosis<br />

generally is poor.<br />

Epithelioid sarcomas—Epithelioid sarcomas are the<br />

most common soft-tissue sarcomas of the hand. 329 A<br />

posttraumatic origin has been proposed by some. 330<br />

Lesions are notoriously insidious and often mistaken<br />

for a benign inflammatory condition. 331 Most lesions in<br />

the hand arise on the palm or volar surface of the<br />

digits. 332,333 Local recurrence is common, as is distant<br />

metastasis. Treatment recommendations are radical<br />

excision (often necessitating a partial amputation 313 ) and<br />

node dissection. 334 Adjuvant therapy can be of benefit.<br />

Malignant fibrous histiocytomas—Malignant<br />

fibrous histiocytomas are among the more common<br />

soft-tissue sarcomas in the adult upper extremity. 313<br />

Lesions can be superficial or deep, single or<br />

multinodular. They extend along tissue planes and<br />

metastasize via the lymphatics and bloodstream. 335<br />

Treatment primarily is surgical, with radiotherapy<br />

added unless there has been a generous margin. The<br />

value of chemotherapy has yet to be defined.<br />

Alveolar rhabdomyosarcomas—Alveolar<br />

rhabdomyosarcomas tend to involve the thenar and<br />

hypothenar musculature. 336,337 An alveolar<br />

rhabdomyosarcoma is a highly malignant, devastating<br />

tumor that presents as a rapidly growing, deep mass in<br />

the palm of a child. 313 Local recurrence is common, and<br />

it is invariably fatal if not adequately treated. The<br />

incidence of nodal spread and distant metastases is<br />

high. The prognosis for alveolar rhabdomyosarcoma has<br />

improved with multi-modality therapy but is still poor.<br />

Synovial sarcomas—Synovial sarcomas arise in the<br />

juxta-articular soft tissues (tendon, tendon sheath, and<br />

bursa). 338 A synovial sarcoma presents as a slowgrowing<br />

tumor on the volar surface of the hand, and<br />

delay to presentation often is measured in years.<br />

Synovial sarcoma has a poor prognosis and a high<br />

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