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unknown. The lesions might arise de novo or might occur secondary to a benign process. Osteosarcomas in general tend to occur more frequently in association with irradiated bone, Paget’s disease, fibrous dysplasia of bone, giant cell tumor, solitary enchondroma, multiple enchondromatosis, and multiple osteochondromas. Radiographically, the borders of an osteosarcoma are indistinct, but the lesion invariably involves the cortex and generally transgresses it. Often, a large contiguous soft-tissue mass is present. A combination of destructive and proliferative new bone usually is present, showing a streaked texture and a characteristic sunburst pattern. Histologically, osteosarcoma has a typical spindle-shaped cell pattern. Treatment has changed from amputation to excision with a wide margin plus adjuvant therapy. In a study presented by Okada et al., 320 local control was achieved in five of six patients by using this protocol; one patient died as a result of metastatic disease. Chondrosarcomas—Chondrosarcomas are uncommon in the hand, where they occasionally are associated with osteochondromas and, to a lesser degree, with multiple enchondromatosis, 323,324 although the vast majority of cases include no preexisting lesion. 325,326 Chondrosarcomas characteristically occur in older patients (60–80 years) in the epiphyseal area of the proximal phalanx or metacarpal. The clinical course is slow, and metastasis is late. 327,328 The tumor presents as a progressively painful large mass near the metacarpophalangeal joint. Treatment of choice is amputation or ray resection. Histological interpretation of cartilaginous lesions of the hand is difficult, and clinical and radiological appearance (bone expansion, lytic areas of bone destruction, soft-tissue swelling) often are more reliable indicators of malignancy. Prognosis is good if metastasis has not occurred. 325,326 Soft-Tissue Sarcomas Rosenberg and Schiller 302 have provided an excellent review of soft-tissue sarcomas of the hand. Soft-tissue sarcomas are an uncommon but important group of hand tumors. They tend to occur in young patients, are innocuous in presentation, often leading to an incorrect diagnosis, and have protracted clinical courses. They SRPS Volume 10, Issue 25, 2009 are prone to local recurrence, have an unusually high incidence of lymphatic spread and regional node metastases, and often metastasize systemically late in their course. Deep tumors that are firm and are 5 cm or larger should be considered to be possible sarcomas until proven otherwise. 313 CT and MRI often are used to define the anatomy. Standard treatment is wide surgical excision with or without adjunctive radiotherapy and/or chemotherapy. The prognosis generally is poor. Epithelioid sarcomas—Epithelioid sarcomas are the most common soft-tissue sarcomas of the hand. 329 A posttraumatic origin has been proposed by some. 330 Lesions are notoriously insidious and often mistaken for a benign inflammatory condition. 331 Most lesions in the hand arise on the palm or volar surface of the digits. 332,333 Local recurrence is common, as is distant metastasis. Treatment recommendations are radical excision (often necessitating a partial amputation 313 ) and node dissection. 334 Adjuvant therapy can be of benefit. Malignant fibrous histiocytomas—Malignant fibrous histiocytomas are among the more common soft-tissue sarcomas in the adult upper extremity. 313 Lesions can be superficial or deep, single or multinodular. They extend along tissue planes and metastasize via the lymphatics and bloodstream. 335 Treatment primarily is surgical, with radiotherapy added unless there has been a generous margin. The value of chemotherapy has yet to be defined. Alveolar rhabdomyosarcomas—Alveolar rhabdomyosarcomas tend to involve the thenar and hypothenar musculature. 336,337 An alveolar rhabdomyosarcoma is a highly malignant, devastating tumor that presents as a rapidly growing, deep mass in the palm of a child. 313 Local recurrence is common, and it is invariably fatal if not adequately treated. The incidence of nodal spread and distant metastases is high. The prognosis for alveolar rhabdomyosarcoma has improved with multi-modality therapy but is still poor. Synovial sarcomas—Synovial sarcomas arise in the juxta-articular soft tissues (tendon, tendon sheath, and bursa). 338 A synovial sarcoma presents as a slowgrowing tumor on the volar surface of the hand, and delay to presentation often is measured in years. Synovial sarcoma has a poor prognosis and a high 19
SRPS Volume 10, Issue 25, 2009 incidence of metastases. Treatment usually consists of surgery (often involving a partial amputation 313 ), radiotherapy, and chemotherapy. Fibrosarcomas—Fibrosarcomas arise within the deep subcutaneous space, fascial septa, or muscle and present as insidiously growing deep masses. 339,340 Lymph node metastases are less common with fibrosarcoma, but hematogenous spread frequently occurs. Treatment is wide excision or amputation when neurovascular structures are compromised. 341 Adjuvant therapy can be of benefit. Clear cell sarcomas—Clear cell sarcomas (malignant melanomas of soft parts) are uncommon tumors. A clear cell sarcoma presents as a slowgrowing, deep-seated mass attached to tendons, aponeuroses, or fascia. 342,343 Prognosis is poor, with a very high rate of local recurrence and both lymphatic and hematogenous dissemination. Surgery with node dissection usually is combined with radiotherapy and chemotherapy. Kaposi sarcomas—Kaposi sarcomas are malignant tumors that often involve bone and can originate in bone. The hand and foot are the most common locations of occurrence and early detection. 344 Patients of all ages can be affected, from very small children to the elderly, with peaks in the 4th and 5th decades. The male-to-female ratio is 10:1, and Kaposi sarcoma is strongly associated with acquired immunodeficiency syndrome. 345 The first clinical signs are dark blue to violaceous macules on the skin that are later replaced by infiltrative plaques and finally by nodules measuring 0.5 to 3 cm in diameter. Some of the lesions heal, and others coalesce and ulcerate. Initially, the skin lesions correspond to the distal end of the tumor in the bone, but in time, the skin manifestations appear at progressively more proximal levels. Radiographic examination reveals the affected bones to be decalcified in a trabecular pattern, with cortical thinning as the tumor expands. Cystic erosion shows as bites taken out of the bone. Treatment is by a combination of radiotherapy and chemotherapy. The prognosis varies according to the behavior of the tumor. Fulminating lesions have a fatal outcome within 6 to 12 months of diagnosis, 20 whereas slower growing tumors are compatible with 20-year survival. Metastatic Tumors Hand metastases are very uncommon and usually are associated with a primary carcinoma in the lung 346–348 or kidney. Despite their rarity, metastatic tumors should be considered in the differential diagnosis of inflammatory processes of the hand. The distal phalanges are most often involved, and metastases in those locations often are mistaken for felons or paronychia. 349–351 Amadio and Lombardi 352 recommend palliative treatment considering the median survival time of only 5 months. Amputation of a phalanx, digit, or ray is recommended for most solitary phalangeal or metacarpal lesions when survival is expected to exceed a few months. 348 SOFT-TISSUE RECONSTRUCTION Fingertips The treatment objectives of fingertip amputations are as follows: close the wound maximize sensory return preserve length maintain joint function obtain a satisfactory cosmetic appearance 353–356 Many variables affect the reconstructive choice: mechanism of injury; size of defect; location and status of wound; associated injuries to other parts of hand; and age, sex, general health, and occupation of patient. Healing by Secondary Intention If the skin loss is no larger than approximately 1.5 cm 2 , the wound can be allowed to granulate and heal spontaneously. 357–359 Such treatment is especially well suited to children and the elderly. All devitalized tissue should undergo débridement, and any exposed bone should be trimmed to lie below the level of the soft tissue. The wound is covered with a semi-occlusive 360 or alginate dressing, which can be left intact for 5 to 7 days and can then be changed as necessary. Complete healing usually is achieved in 3 to 4 weeks. Mennen and Wiese 360 treated extensive fingertip defects by using this method and reported excellent functional
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