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Vascular Lesions Several authors offer excellent reviews of vascular tumors of the hand and upper extremity. 182–186 Upton and Coombs 187 discussed pediatric vascular tumors. Glomus tumors—Glomus tumors are benign hamartomas of the normal glomus apparatus, which consists of arteriovenous anastomoses involved in the regulation of cutaneous circulation. 188–191 Glomus tumors usually are smaller than 1 cm in diameter (often measuring only a few millimeters) and classically present with the triad of pain, pinpoint tenderness, and cold sensitivity. Transillumination is a simple and useful clinical test. 192 The most common site of presentation is subungual, but glomus tumors occasionally occur on the volar surface of a digit. Approximately one-fourth of all glomus tumors are multiple. 193,194 Ultrasonography 195–197 and MRI 198–200 are used as aids in diagnosis and also to detect multiple tumors. Treatment is by excision. If subungual, care should be taken to repair the nail bed after removal of the tumor. The major problems after surgical treatment are a high recurrence rate and a residual nail deformity. 201–203 Ulnar artery aneurysms—Ulnar artery aneurysms are almost always posttraumatic (“hypothenar hammer syndrome” 204,205 ) and occur predominantly in male patients. 206,207 Typical clinical features are a pulsatile mass accompanied by digital ischemic changes, with or without distal emboli. 208,209 An ulnar nerve Tinel sign often is present. 210 An Allen’s test should be performed to ascertain patency of the ulnar artery, and arteriography can rule out thrombosis of the ulnar artery and embolic showering. Management consists of aneurysm resection and ligation of the ulnar artery, with autogenous vein grafting in cases of inadequate collateral circulation. 211,212 Regional thrombolysis can also be considered in cases with embolization. 209 Peripheral Nerve Tumors True neural cell tumors in the hand are rare (1%–5% incidence). 213 All lesions arise from Schwann cells. 214–216 Although no uniform classification of peripheral nerve tumors exists, most clinicians refer to five general types. Neurilemmomas—Neurilemmomas are the most common solitary tumors of neural cell origin in the hand and are particularly prevalent in middle-aged patients. 217,218 Neurilemmomas begin as asymptomatic SRPS Volume 10, Issue 25, 2009 nodular swellings without associated sensory or motor abnormalities. When exposed surgically, they are seen to have a dumbbell shape and to lie extrinsic to the nerve fiber proper. Histologically, they are made up almost exclusively of Schwann cells. Excision involves enucleation under magnification so as to preserve nerve fibers that fan out over the tumor. Recurrences are rare, and malignant degeneration is not a clinical feature. Neurofibromas—Unlike neurilemmomas, neurofibromas can intimately proliferate within nerve fibers, producing functional abnormalities and making excision more difficult without division of the nerve. Histologically, neurofibromas are difficult to differentiate from neurilemmomas, although they exhibit mast cells, lymphocytes, mucoid material, and xanthoma cells in addition to Schwann cells. 218 Solitary lesions usually occur before age 10 years. Neurofibromas can cause gigantism of the affected part. 219,220 Neurofibromatosis—Neurofibromatosis (von Recklinghausen disease) is an autosomal dominant condition characterized by multiple peripheral and central neurofibromata (acoustic neuromas, meningiomas, optic gliomas). Temporal lobe involvement can erode the greater wing of the sphenoid, producing pulsatile exophthalmos. 220 Extremity involvement can produce gigantism of the limb. 221 Diagnostic café au lait spots, numbering more than six, occur on the skin. The individual tumors manifest a plexiform pattern. Sarcomatous degeneration has been reported in 2% to 3% of lesions. 222 Neurofibrosarcomas—Neurofibrosarcomas (neurosarcomas or malignant schwannomas) account for 2% to 3% of malignant hand tumors and usually are associated with von Recklinghausen disease. 223 Local extension and metastases are common, resulting in 90% mortality. Wide excision or amputation of the extremity is the recommended treatment. Intraneural tumors of non-neural origin— Intraneural tumors of non-neural origin include lipofibromatous hamartomas, hemangiomas, ganglion cysts, and lipomas. 224 Lipofibromatous hamartomas commonly occur within the 1st decade of life and usually involve the median nerve. They can result in macrodactyly, especially of the index and middle fingers. 225 Treatment involves release of the carpal tunnel after excision of the tumor under magnification. 15
SRPS Volume 10, Issue 25, 2009 Malignant degeneration has not been reported. Epidermal Inclusion Cysts Epidermal inclusion cysts commonly occur on the palmar surface of the hand or digits of patients whose work or leisure activities predispose them to penetrating hand injuries. The time from the traumatic incident to cyst development varies from months to years. Clinically, the lesions are firm, spherical, and nontender. The cyst wall consists of squamous epithelium with laminated keratin, and the cyst material contains protein, cholesterol, fat, and fatty acids. Spontaneous rupture is common, but the lesion often persists unless the cyst lining, contents, and overlying puckered skin are surgically removed. Local complications include infections and bone erosion. Malignant Skin Tumors Malignant tumors of the skin of the hand make up a very small percentage of upper extremity neoplasms226- 228 and are primarily squamous cell carcinomas (SCC). 229–232 SCC predominate among people with fair skin and light hair color. The usual origin of SCC is ionizing solar radiation. Other less common causes of SCC are previous irradiation, 233 burn scars, exposure to arsenic compounds, and inherited genetic disorders. 234 The dorsum of the hand, with the highest actinic exposure, is the most common site for SCC, although the tumor has been reported to also occur on the palms235 and subungually. 236–239 Appropriate treatment consists of a 4-mm margin for tumors with a diameter of less than 2 cm. When the size of the tumor exceeds those dimensions, a margin of 6 mm is necessary. 240 If evidence exists of nodal metastasis or local recurrence, axillary lymphadenectomy is recommended. The role of sentinel node biopsy in cases of SCC is not yet defined in the literature. SCC of the hand is an aggressive tumor prone to recurrence and metastasis. The metastatic rate for SCC of the hand is higher than elsewhere on the body, particularly if the primary lesion involves the digital web space. 230 Basil cell carcinomas—Basal cell carcinomas (BCC) are very uncommon tumors on the finger. 241 Palmar variants have been observed, 242 especially in cases of Gorlin’s syndrome (multiple nevoid BCC syndrome), 243 and BCC has been reported to also occur 16 subungually, 244 in which case differentiation from a subungual melanoma must be made. 245 Although BCC do not metastasize, they are locally aggressive. Excision is the usual form of treatment. Melanomas—Melanomas of the hand can occur on the palm 246–255 or subungually. 256–259 A study by Ridgeway et al. 255 showed that the acral histological subtype does not affect the disease-free and overall survival. Tumor thickness remains the only prognostic indicator. Slingluff et al. 252 found that acral melanoma has a strong racial predilection, carries a grave prognosis, and arises from glabrous skin. In that study, no survival difference was shown between volar and subungual sites, nor did amputation make a difference. Melanoma requires wide excision or amputation of the digit or hand, depending on location and depth. 260–262 The appropriate level of amputation has not been determined. Papachristou and Fortner 256 advocated amputation through the carpometacarpal joint, whereas Finley et al. 258 performed seven finger amputations distal to the metacarpophalangeal joint (four just proximal to the distal interphalangeal joint and three just proximal to the proximal interphalangeal joint), with no local recurrences. Quinn et al. 259 showed no difference in local recurrence for subungual melanomas whether amputations are performed proximal or distal to the interphalangeal joint of the thumb or the middle of the middle phalanx in the fingers. Similarly, no prospective study to date has shown a survival or local control benefit to prophylactic lymph node dissection, regional perfusion, or immunotherapy. 263–265 The use of sentinel lymph node biopsy has grown significantly in recent years. 266,267 Bony Tumors Several authors have presented excellent reviews of bony tumors of the hand. 268–270 Treatment is based on accurate diagnosis and staging of the lesions (Table 3). Chondromas Chondromas are the most common benign cartilaginous tumors of the hand. 271,272 Chondromas that remain within the substance of the bone or cartilage are called enchondromas. 273–275 Enchondromas favor the tubular bones of the hand, especially the middle and proximal phalanges. Congenital cartilaginous rests are
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