Rimma Finkel and Morton Kasdan
DEFINITION
Vascular tumors are diverse, ranging from benign vascular malformations to malignant lesions.
The incidence of vascular tumors is about 2% to 6%.14,16
About 26% of vascular and lymphatic tumors are found in the extremities.17
When found in the upper extremity, they are more common in the hand and forearm.
Vascular tumors are fourth in frequency of upper extremity tumors, after ganglions, giant cell tumors, and inclusion cysts.
Most vascular tumors are congenital, and 10% of pediatric tumors involve the upper extremity. Of these, 90% can be classified as hemangiomas or vascular malformations.23
Benign vascular tumors can be congenital or acquired and include hemangiomas, lymphangiomas, congenital arteriovenous fistulas, aneurysms, vascular leiomyomas, glomus tumors, and pyogenic granulomas.
Malignant vascular tumors include hemangioendotheliomas, hemangiosarcomas, glomangiosarcomas, and malignant hemangiopericytomas.
ANATOMY
The ulnar and radial arteries form the superficial and deep arches of the hand, which then branch into the common digital arteries. There are multiple anatomic variants.5 The common digital arteries then branch into the proper digital arteries that course along the midlateral aspect of each digit, slightly volar to midline.
The arteries terminate at either a capillary bed or a glomus body. The glomus is a neuromyoarterial mechanoreceptor— that is, a specialized arteriovenous shunt. It lies in the stratum reticulum of the skin, especially in the subungual region and distal pads of the digits. The glomus body acts as a thermoregulator, and it regulates peripheral blood flow in the digits and possibly controls peripheral blood pressure. It contains the glomus cells surrounding the Sucquet-Hoyer canals, which are narrow vascular anastomotic channels.
PATHOGENESIS
The theory is that vascular tumors occur as a failure of differentiation of the common embryonic vascular channels, which results in the congenital lesions.17 These are more commonly seen in the pediatric population, but they may be discovered late, in adults.
Acquired vascular tumors are usually due to trauma that induces aneurysms or fistulas.
NATURAL HISTORY
Congenital Lesions
Hemangiomas
Thirty percent of hemangiomas are visible at birth, but this increases to 70% to 90% before the infant is 4 weeks old.
These lesions show rapid growth, then slower growth that is proportional to the child. Next a slow involutional process occurs.29 Fifty percent of hemangiomas will involute by the time the child is 5 years old and 70% will involute by the age of 7.
Hemangiomas consist of plump endothelial cells with high turnover rates.14 They may be classified by histology, location (superficial, subcutaneous, or intramuscular), or involutional status.17 Thirty percent of upper extremity hemangiomas will ulcerate. This becomes a problem with these hand and finger tumors that may present with acute or chronic paronychia, especially in children who suck their fingers.23
They present as reddish lesions that become raised during the growth phase.
Congenital Aneurysms
The histologic classification of congenital aneurysms includes capillary hemangiomas, sclerosing hemangiomas, and venous or cavernous hemangiomas (FIG 1).17
Capillary hemangiomas consist primarily of proliferated capillaries. There is a compact mass of endothelial cells where there are small or no capillary lumina. These extend from the dermis into the subcutaneous tissue and make up about 57% of subcutaneous hemangiomas.18
FIG 1 • A. Hemangioma of the volar fourth web space of the left hand. This patient had a raised, ulcerated lesion and pathology showing a polypoid lesion with central capillary and slightly larger vascular spaces. B. Cavernous hemangioma of the thumb. There is a pinkish hue to the skin without any raised tissue. Although the lesion appears ulcerated, on pathologic examination there were no ulcerations and large vascular spaces were found immediately beneath the epidermis.
If the hemangioma is associated with thrombocytopenia and consumptive coagulopathy, it is termed Kasabach-Merritt syndrome. This is unrelated to the size of the hemangioma and may be life-threatening if untreated.
If there are thin-walled sinuses secondary to widely dilated thin-walled spaces with little stroma, they become cavernous or venous hemangiomas. These make up about 23% of hemangiomas.
Sclerosing hemangiomas contain a perivascular thickening of the lymphatic cells. There is a fibrous, not hematogenous, origin to these lesions. This type represents 10% of all hemangiomas.17
Lymphangioma
Lymphangiomas are rare and classified as simple, cavernous, and cystic. The most common variety is cavernous lymphangiomas. These present at birth or soon thereafter and they are composed of dilated lymphatic sinuses.
Congenital Arteriovenous Fistula
Congenital arteriovenous fistulas (AVFs) develop early in the embryo. The upper extremity is the second most common location for these lesions after the head and neck. They have several arteriovenous communications at birth and are associated with syndromes such as Parkes-Weber syndrome and Klippel-Trenaunay syndrome.9
Parkes-Weber syndrome is a combination of multiple AVFs, vascular malformations, and skeletal hypertrophy of the affected limb.
Klippel-Trenaunay syndrome is characterized by a combined type of vascular malformation and limb enlargement due to hypertrophy of soft tissue and bone.
Both of these syndromes may have significant medical sequelae, including congestive heart failure, pulmonary embolism, venous thrombosis, bleeding, and cellulitis.
Vascular Malformations
Vascular malformations are uniformly present at birth but may not be visible until childhood, adolescence, or adulthood. Most appear by ages 2 to 5 years.29 They enlarge proportionately with the child unless they are stimulated by trauma, hormones, infection, or surgery.2 These lesions have an equal sex distribution. Malformations generally have flat, slowly dividing endothelial cells.
They can be categorized as low-flow or high-flow lesions based on their hemodynamic features at the time of angiography.
High-flow lesions have an arterial component. Marked enlargement and increased number of arteries, small vessels, and veins are consistent findings.3
Low-flow malformations have large channels without intervening parenchyma and often with associated phleboliths. These lesions are more common than high-flow lesions. They are subdivided into capillary, venous, lymphatic, and combined.
Capillary malformations (port wine stain, nevus flammeus) show dilated capillaries and postcapillary venules in the upper dermis. They are dark red to purple and may have another associated vascular lesion. Over time, they become darker and have a cobblestone appearance. They may be associated with limb or digit overgrowth.23
Seventy-five percent of venous malformations are recognized at birth. They are the most common anomaly of the lowflow group (40%).14 It is important to differentiate them from hemangiomas because venous malformations do not involute. They, like lymphatic malformations, present with a mass or skin discoloration. They enlarge shortly after birth and grow with the child.23 Slow commensurate growth, compressibility, and phleboliths are pathognomonic for venous malformation (FIG 2).14
Patients with vascular malformations will complain of the mass effect of the lesion, increased size with exercise, or pain due to thrombosis. Elevation of the extremity eases symptoms. They may lead to nerve compression at the forearm and wrist, and digital compression may be seen with localized thrombosis.23
Lymphatic malformations enlarge secondary to fluid accumulation, cellulitis, or inadequate drainage of lymphatic channels.14 They can limit hand motion, and infections are common.23 They can cause bone hypertrophy.
Mixed vascular malformations share the characteristics of their combination of vascular malformations.
High-flow malformations present early as a painless mass. They have a bimodal occurrence: 40% show up at birth and another 34% after 10 years old.23 They are not compressible. These lesions can lead to distal ischemia or even high-output heart failure if large and untreated. They have been divided into three types (FIG 3):
Type A lesions have single or multiple arteriovenous fistulas, aneurysms, or ectasias of the arterial side.24,29
Type B lesions consist of arteriovenous anomalies with microfistulas or macrofistulas that are localized to a single limb, hand, or digit. They have stable flow characteristics and provoke minimal to no distal symptoms. As with type A lesions, they remain localized to a specific anatomic region.23,24,29
Type C lesions enlarge slowly. They are diffuse, with microfistulas and macrofistulas involving all limb tissues. With increasing size, vascular steal occurs. The lesions and associated symptoms worsen with pregnancy and do not reverse with delivery. They can cause distal ischemic pain, tachycardia, and congestive heart failure. Compartment syndrome, compression neuropathies, and ulceration secondary to ischemia or attempted surgical interventions can also occur. The result can be unrelenting, progressive pain, eventually leading to amputation.23,24
FIG 2 • Venous malformation of the ulnar side of the left hand. Notice the blue color and slightly raised appearance.
Acquired Lesions
Acquired lesions comprise both true and false aneurysms of the vessels, glomus tumors, pyogenic granulomas, fistulas, and vascular leiomyomas.
True aneurysms contain all three layers of the vessel wall: intima, media, and adventitia. False or pseudoaneurysms do not contain all three layers of the vessel wall.
True Aneurysms
True aneurysms account for 6% of all tumors of the hand.17
True aneurysms, most notably hypothenar hammer syndrome, usually follow blunt trauma in the area of the vessel. The trauma may be a single event or repeated injury. The vessel dilates in response to injury to the arterial media, leading to a fusiform vessel.
Aneurysms occur secondary to other disease processes such as arteriosclerosis, metabolic disorders, Kawasaki disease, Buerger disease, hemophilia, osteogenesis imperfecta tarda, granulomatous arteritis, and cystic adventitial disease (FIG 4).14
Pseudoaneurysms
False or pseudoaneurysms account for most (83%) aneurysms of the hand and generally occur on the palmar surface of the hand.
They may be secondary to a puncture wound (such as from a knife or pencil lead) or complete rupture of the vessel wall with continuity maintained by the surrounding soft tissues.14,17
Pseudoaneurysms occur slowly over time and are usually not evident for weeks to months after the injury.
A bruit may be noted on examination. Like true aneurysms, the most common site is in the ulnar artery.
Acquired Arteriovenous Fistulas
Acquired AVFs occur secondary to trauma or surgical intervention. AVFs consist of a communication between an artery and a vein that shunts away from the higher-resistance capillary system.
FIG 3 • A. Arteriovenous malformation of the digit. The margins are indistinct and it is difficult to dissect from the surrounding tissues. B. Arteriovenous malformation of the palm at the ulnar artery. There is a bulbous region where the malformation has occurred.
FIG 4 • A. Venous aneurysm of the palm. Again, a bluish tinge is noticeable over the lesion. B. Intraoperative view of a venous aneurysm. There is dilatation present at the vein. C. Ulnar digit artery false aneurysm. The patient sustained a traumatic injury at work and noted an increase in the size of the lesion over the ensuing 6 weeks. D. Hypothenar hammer syndrome. The patient was releasing a mechanical latch of a machine by using the heel of his hand, which caused a sharp pain. The patient presented with coolness of the ring fingertip and associated pain.
FIG 5 • A. Glomus tumor of the left ring finger, subungual region. The patient presented with minimal discoloration and sensitivity to heat and cold. B. Glomus tumor of the left thumb. The patient had more significant discoloration of the subungual region consistent with a glomus tumor. C. Glomus tumor of the left ring finger after removal of the nail plate. Although the patient had minimal discoloration with the nail plate on, the bluish hue becomes more discernible after the nail is off.
Traumatic AVFs occur when there is penetrating injury to an artery and the adjacent vein, leading to a hematoma and shunting. This may occur secondary to injury with such objects as small knives or pencils, but it may also be due to venipuncture, arterial cannulation, or catheterization procedures. AVFs secondary to iatrogenic vascular injuries tend to occur slowly, while those that occur secondary to trauma are usually rapid in onset. This may be secondary to the size of the puncture that occurs; iatrogenic injuries tend to be smaller punctures than traumatic ones.25 Patients with intrinsic coagulation deficiencies are more vulnerable to this complication.
Surgical AVFs are formed for dialysis access in renal failure patients and can cause similar symptoms, including steal, ischemia, venous arterialization, and hand edema.
Glomus Tumors
Glomus tumors make up 8% of the vascular tumors of the hand and 1% to 4.5% of all hand tumors.17,27 They arise in the neuromyoarterial apparatus that was first described by Wood30 in 1812 and then again by Masson13 in 1924. These lesions have been found in the stomach, trachea, and retina but are most commonly found in the digits. Glomus tumors are more consistent with a hamartoma than a true tumor.14Sixty-five percent of these lesions are found in women 30 to 50 years old.
Between 26% and 90% of solitary glomus tumors are located in the subungual region.14,17,27 These lesions tend to be small—normally 5 mm and usually less than 1 cm. They are encapsulated and contain numerous small lumina when found as single tumors. Multiple tumors tend to be unencapsulated, rarely subungual, with larger-shaped vascular spaces.
Multiple glomus tumors tend to be asymptomatic and present earlier in life, whereas solitary tumors often go undiagnosed or misdiagnosed for years because the lesions are small and not palpable and with varying presentations (FIG 5).15
Vascular Leiomyomas
Vascular leiomyomas are very rare tumors of the hand. They arise in the smooth muscle of the tunica media of veins in 50% of cases. These masses are typically well encapsulated, small, round, firm, colorless, and curable (FIG 6).10
Pyogenic Granulomas
Pyogenic granulomas make up 20% of the vascular tumors of the hand and may be a variation of a capillary hemangioma. They appear as a circumscribed lesion.
They develop rapidly and become a pedunculated, friable lesion that is easily traumatized and bleeds. In children, these lesions are more commonly found on the glabrous portion of the palm and digits as well as in the mouth and around the lips and face. In adults, these are more commonly found on the fingers and toes.
They may occur spontaneously but are more frequently present as an overgrowth of granulation tissue in an area of previous penetrating trauma (FIG 7).6,17,23,29
Malignant Tumors
Malignant vascular tumors account for less than 1% of all vascular hand and forearm tumors.17 There are several types of malignant vascular tumors: hemangioendothelioma, glomangiosarcoma (malignant glomus tumors), angiosarcoma, Kaposi sarcoma, lymphangiosarcoma, and hemangiopericytoma.
Hemangioendotheliomas tend to arise adjacent to or within veins. They extend centrifugally from the vessel. They are slow-growing tumors, and tumors that show more than one mitosis per high-power field on histology are more likely to metastasize. Metastasis may occur locally to nodes or be distant to the lungs, liver, or bone.29
Glomangiosarcomas are extremely rare and were first described in 1972 by Lumley and Stansfield. They tend to be low-grade tumors that are locally invasive. They occur in adults ages 20 to 89 years. There are three categories of glomangiosarcoma: locally infiltrative glomus tumor (LIGT), glomangiosarcoma arising in a benign glomus tumor (GABG), and de novo glomangiosarcoma (GADN).
FIG 6 • A. Vascular leiomyoma of the right index finger. The patient presented after a 7-month history of having a trauma at work. She stated that the growth appeared 3 months later and had increased in size since then. B. Intraoperative photograph of the above vascular leiomyoma. It is a well-circumscribed lesion that is difficult to differentiate from an aneurysm except on pathology.
FIG 7 • A. Pyogenic granuloma of the left ring finger. The patient developed an open lesion of the cuticle that progressively swelled and then blistered over the nail bed. B. Pyogenic granuloma of the left index finger. Notice the granular, raised appearance.
LIGT is identical to solitary glomus tumors except that it has infiltrating growth and tends to recur with resection.
GABG is a sarcomatous tumor in association with a benign glomus tumor.
GADN is a sarcoma with round cells and features of a benign glomus tumor.12,19,20
Angiosarcomas are rare and aggressive and metastasize early. They may occur after radiation therapy or long-term exposure to polyvinyl chloride. They are sometimes mistaken for hemangioendotheliomas on histology. The prognosis is extremely poor with these tumors, with survival times averaging 2.5 years.14,17,29
First described by Kaposi in 1872 in elderly men of Jewish and Mediterranean heritage, Kaposi sarcoma present as small, purple macules. They are a malignant degeneration of the reticuloendothelial system. These lesions tend to start on the hands or lower extremities, progress onto the trunk, and coalesce into large papules. In this patient population, the disease has an indolent course and may be treatable with surgery and radiation. In the age of HIV/AIDS, however, the disease is much more aggressive, with a larger number of lesions. In these patients, it is associated with human herpes virus 8.14,17,29
Lymphangiosarcoma is a rare cancer that occurs after longstanding lymphedema, as seen in some postmastectomy patients. These lesions metastasize rapidly.
Hemangiopericytoma is a diffuse proliferation of capillaries, encased in connective tissue and surrounded by pericytes. They have no nerve elements and are generally painless. Patients tend to delay treatment secondary to lack of pain. They may present as a nonpigmented bleeding mole, an ulceration with prominent telangiectasia, or a dark blue, hemorrhagic swelling. Histologically, they have sheets of spindle cells surrounding capillaries, regular oval nuclei without anaplasia, indistinct cytoplasmic borders, and a reticulin sheath surrounding each cell on silver stain.11,28 Pathologists have described three histologic grades based on the above criteria: benign, borderline malignant, and malignant. It has an unpredictable behavior and may metastasize years after excision; therefore, long-term (5 to 10 years) follow-up is recommended (FIG 8).
PATIENT HISTORY AND PHYSICAL FINDINGS
It is imperative to get a complete history and physical examination of the patient and family.
Determine whether the lesion was present at birth or infancy or whether it appeared later in adolescence or adulthood.
Rate of growth should be sought. This may help to differentiate between a hemangioma and an arteriovenous malformation in early childhood. Hemangiomas grow out of proportion to the growth of the child.
Hemangiomas
Hemangiomas will appear as a reddish lesion that becomes raised. Lesions of the axilla or interdigital region will be chronically macerated. Fingertip hemangiomas may present with findings similar to an acute or chronic paronychial infection, especially in children who suck on their fingers.23
Vascular malformations
Low-flow malformations most commonly present as a mass or skin discoloration. If a capillary component is present, there may be a reddish stain of the skin. The physician should ascertain whether there are any compressive symptoms from the lesion consistent with a mass effect, distention, or pain with exercise that would indicate a venous malformation.
Ulceration is uncommon in these lesions.
If there is a lymphatic component, patients may present with intralesional infections secondary to ruptured vesicles and maceration of large lesions.
They may also be found in association with syndromes such as Parkes-Weber, Klippel-Trenaunay, proteus (capillary malformations, venous malformations, macrodactyly, hemihypertrophy, lipomas, scoliosis, and pigmented nevi), and Mafucci (lymphaticovenous malformations and enchondromas).23
FIG 8 • A. Hemangiopericytoma of the right forearm. The patient presented with a large mass of the forearm that had been present for 46 years. B. Intraoperative view of hemangiopericytoma. The lesion was 9 × 6.6 × 5 cm and weighed 168 g.
High-flow malformations tend to be painless early on but then progress to be warm, painful masses with palpable thrills and bruits as the child grows.
Asking the patient if he or she gets relief of the pain with elevation, if there is increased pain with exercise, and increased warmth in the lesion may help to distinguish these from low-flow lesions.
It is also important to ask about any symptoms of congestive heart failure, which may occur as sequelae of an untreated high-flow malformation.23
Any patient evaluated in the office for a suspected arteriovenous malformation should be evaluated for other lesions, Nicoldani sign (decrease in pulse with occlusion of the fistula), and any evidence of distal ischemia.14
Aneurysms and pyogenic granulomas
For evaluation of possible aneurysms and pyogenic granulomas, it is important to know whether there is a history of trauma in the region, how long the lesion has been present, whether it is a pulsatile mass, and whether it has bled.
Glomus tumors
The classic triad of paroxysmal pain, pinpoint tenderness, and temperature intolerance, especially cold, should be elicited if glomus tumors are in the differential.
On physical examination, the physician should look for a bluish discoloration (found in 28% of patients) and a pulp nodule or nail deformity (found in 33% of patients).15
The length of time that the patient has had symptoms can assist in differentiating glomus tumors from other tumors of the upper extremities, since most patients tend to have symptoms for more than 10 years. If the patient has had previous excisions of glomus tumors, it is necessary to find out the amount of time between resection and recurrence. This can help to determine whether the lesion is an incomplete excision or a new tumor.26
During the physical examination, the patient should also be evaluated for multiple glomus tumors, which tend to be less symptomatic.
Patients with lesions of the hand, wrist, or distal forearm should have an Allen's test performed.
The hand is elevated and the patient is asked to make a tight fist for about 30 seconds. The ulnar and radial arteries are occluded and the patient opens his or her hand slowly. The ulnar artery is then released and the color should return in 5 seconds. If color returns to the radial aspect of the hand within 5 seconds, the superficial arch is complete and the radial artery may be ligated.
A thorough evaluation of the rest of the patient's medical history, including a history of axillary dissections, HIV/AIDS status, and irradiation, is also necessary if the patient presents with a lesion that may be cancerous.
Methods for examining the vascular lesions of the hand
The examiner should look at the hand to check for blue spots, nail ridging, reddish, raised lesions, pulsatile masses, or traumatic injury, which helps to differentiate between malformations, aneurysms, pyogenic granulomas, and glomus tumors.
A stethoscope is gently placed over the lesion to listen for bruits or thrills. In fast-flow arteriovenous malformations, a bruit or thrill may be heard, which would not be found in other vascular lesions.
The mass is gently palpated. If a pulsatile mass is felt, the examiner should ascertain whether the lesion is compressible and whether there is associated pain.
Love pin test: The head of a pin or paperclip is gently pressed against the tender area to localize the pain. This locates a glomus tumor. In subungual tumors, the pin is placed on the nail plate at various locations to find the tumor.15
Hildreth test: The digit is exsanguinated by placing a tourniquet at its base or the hand is exsanguinated by elevating it and making a tight fist. The point of tenderness located by the Love pin test is then repalpated. If the patient has diminished or resolved pain with this maneuver, then the test is considered positive for a glomus tumor.8
IMAGING AND OTHER DIAGNOSTIC STUDIES
Plain radiographs of the digits and hands
Phleboliths (in 6%) and bony hypertrophy may be noted.14,17
There may be evidence of a soft tissue mass or signs of bone erosion or destruction of the cortical surface, which is seen in about 6% of patients with hemangiomas.17
Doppler ultrasonic flow detection is a noninvasive study that does not require the use of contrast.
It has been used to confirm high-flow anomalies and to help differentiate between hemangiomas and malformations.24 Doppler ultrasonography will show these lesions to be monophasic with low-flow velocity averaging 0.22 kHz.22
Computed tomography with contrast enhancement may show bony involvement of the tumor, especially in type A highflow malformations.24
MRI can be used to evaluate the site, size, flow rate, and characteristics of the lesion as well as involvement of contiguous structures.24
It may be used to determine whether a malformation is low-flow or high-flow and can also distinguish between dense parenchymal lesions and malformations with large vascular channels.14
It can also be used to evaluate glomus tumors, which have a high signal intensity on T2-weighted spin-echo MRI or after gadolinium injection.15
MRI has a sensitivity of 90% and a specificity of 50% for glomus tumors, so that it cannot be used as the single diagnostic study for glomus tumors, especially if they are less than 2 to 3 mm in size.1
Hemangiomas will appear as well-circumscribed mass lesions that enhance with gadolinium and will have a high T1 signal secondary to infiltrative margins and fatty tissue overgrowth as well an extremely high, heterogeneous T2 signal. A serpentine pattern in the mass may also be seen on MRI.29
MR angiography may be performed at the time of MRI to evaluate lesions in patients who are unable to undergo angiography secondary to renal problems or contrast allergies. It can be used to define the anatomic extent of lesions and their relationship with the surrounding tissue. It can be used to evaluate for both arterial and venous tumors without contrast enhancement.7
Technetium-99m red blood cell perfusion and blood pool scintigraphy will show increased activity on early and late blood pool images with increased perfusion in hemangiomas and may be useful in their operation or embolization.24 It may show a cluster of anomalous arterial branches with multiple communications with venous trunks draining the site of involvement.16 diagnosis.29
Angiography is the gold-standard evaluation of certain tumors, including vascular malformations. No longer routinely used for diagnosis of a lesion, it is used as an evaluation for operation or embolization.24It may show a cluster of anomalous arterial branches with multiple communications with venous trunks draining the site of involvement.16
FIG 9 • A. Angiogram of hypothenar hammer syndrome. The ulnar artery flow is absent and collaterals have formed to allow for flow in the palmar arch. This patient was relatively asymptomatic until a trauma to the hand. B. Angiogram of a second patient with hypothenar hammer syndrome. In this patient, there are no collaterals present, and he presented with coldness of the ulnar distribution digits.
Closed venous angiography uses contrast injected into the venous system distal to a proximal arterial tourniquet applied on the upper arm. Dye is injected into the exsanguinated extremity distal to the tumor and radiographs are taken as the vascular tumor fills to get an accurate assessment of the anatomy.14 Arterial angiography is performed through a stick into the femoral artery with a catheter that is fed into the involved extremity. Dye is then injected and both the arterial and venous phases of circulation are evaluated. This can be used to evaluate the size of the tumor, locate the feeding vessels, and embolize feeding vessels before operation (FIG 9).14
DIFFERENTIAL DIAGNOSIS
Foreign body
Bacillary angiomatosis
Pyogenic granuloma
Glomus tumor
Hemangioma
Arteriovenous or lymphatic malformation
AVFs (traumatic, congenital, iatrogenic)
Traumatic aneurysm (true or false)
Mycotic aneurysm (hematogenous or exogenous)
Arteriosclerotic aneurysm
Congenital aneurysm
Metabolic aneurysm (eg, osteogenesis imperfecta, granulomatous arteritis, Buerger disease)
Vascular leiomyomas
Glomangiosarcoma
Angiosarcoma
Hemangioendothelioma
Hemangiopericytoma
Kaposi sarcoma
Lymphangiosarcoma
NONOPERATIVE MANAGEMENT
Observation is important for hemangiomas. Up to 70% of these lesions will involute by the age of 7.
Large venous or capillary malformations should be observed for limb growth disturbances and a possible underlying high-flow lesion.
Limb compression garments can be used to compress massive congenital arteriovenous fistulas that are inoperable, giant venous malformations, lymphatic malformations, or large hemangiomas in the arm and forearm.17,23 For larger lymphatic lesions, home compression pumps can be used to decrease edema at night.23
Antibiotic prophylaxis is indicated in patients who have recurrent infections in lymphatic malformations. The bacteria most commonly responsible for these infections is penicillinsensitive beta-hemolytic streptococcus.23
If a patient with venous malformations or capillary-venolymphatic malformation has recurrent intralesional thrombosis, then low-dose aspirin may be added to the compression garments for effective therapy.23
Local wound care and dressings may be required if ulcerations occur in the periungual regions or the central portions of large lesions during the involutional phase.23
Pulsed-dye laser or argon laser may be used with some hemangiomas to treat the pigmented lesion without damaging the overlying skin, sweat glands, and hair follicles. Lasers of 585-nm wavelength work well on vascular lesions, such as hemangiomas, which are rich in hemoglobin. The laser heats the hemoglobin, causing coagulation of the vessels in the dermis. Scar formation ensues and replaces the damaged blood vessels.17
Sclerotherapy with 1% sodium tetradecylsulfate, for small superficial lesions, or 100% ethanol, for large, deep saccular lesions, may be used in treating venous malformations.
With the larger lesions, there is a possibility of skin ulceration, necrosis, inflammatory changes, and contracture due to the treatment, and patients should be warned to watch for these sequelae.23
In arteriovenous malformations interventional radiology may be used for embolization of selectively catheterized vessels with polyvinyl alcohol foam or tissue adhesive. This may be helpful if surgical resection is performed 24 to 48 hours later. If the lesion is small, this may completely occlude the malformation and destroy the lesion, eliminating the need for surgical resection. Several embolizations may be necessary to fully destroy small lesions.14
Embolization may lead to residual tissue loss, neurologic deficit, and enlargement of the malformation if the lesion is large and not excised promptly.9,16
Either intralesional or systemic steroids may be useful for the treatment of hemangiomas, and a 6-week course may help to treat life-threatening or tissue-threatening lesions. This is also true for interferon alpha-2a or 2b. However, neither of these medications has been shown to have any effects on malformations, and the morbidity (neutropenia, elevation of liver enzymes, and spastic diparesis) of interferon must be considered before its use.24,29
Radiation therapy was used in the past for sclerosis of hemangiomas; however, it leads to atrophic changes in the skin and subcutaneous tissue as well as arrest of skeletal growth.17
SURGICAL MANAGEMENT
Indications for surgery include pain, intralesional thrombi, episodic bleeding or ulceration, recurrent infection, or functional problems related to the size or weight of the extremity. It is important to consider whether the extremity will be functional after the proposed surgical treatment; in many cases amputation may be a better option.23
Lymphatic malformations have the added difficulties of beta-hemolytic streptococcal septicemia, skin maceration, and vesicular eruptions. This makes the planning of surgical resection complex. Complications occur in 25% of all procedures. The surgeon should be aware that tumor-free tissues, such as grafts or flaps, may be necessary for coverage.23
Preoperative Planning
Radiographic studies should be reviewed carefully to plan resection of large or complex lesions.
An Allen's test should be performed on the patient to evaluate for the patency of the superficial palmar arch and to see if the patient has an adequate ulnar artery.
If the Allen's test is positive, reconstruction of the radial artery is necessary if it is to be resected.
Positioning
The patient should be placed in supine position with the arm abducted.
A proximal arm tourniquet is used, but the arm should not be exsanguinated with an Esmarch bandage to avoid the proximal spread or localized compression of the tumor. Exsanguination with the Esmarch bandage may also obscure the margins of hemangiomas and malformations.
Injections around the tumor should also be avoided to reduce the risk of local spread and compression of the mass, which could cause incomplete resection.
Approach
The technique chosen is based on the location of the lesion and the access necessary for excision.
Ligation of Feeding Vessel
For lesions that are small, with few feeder vessels, direct exploration and ligation of the feeding vessels can lead to involution of the lesion without significant tissue loss.
If tissue loss occurs, excision of the area and either primary closure, skin grafting, or flap reconstruction can be performed.
Staged Excision
Staged excision is useful for venous malformations, lymphatic malformations, combined malformations, and types A and B high-flow malformations.24
For larger lesions, the interventional radiologist may be helpful in embolizing feeding vessels. This will decrease or limit the amount of open exposure necessary in the first stage.
In this approach, the extremity is not exsanguinated completely to allow identification of the vessels more readily.
In the first stage, the tributary and exiting vessels are ligated proximal and distal to the tumor. It is possible that ligation of the vessels may induce distal ischemia. If this occurs, the surgeon should be prepared to bypass the anatomic defect with autogenous vein grafts.
At a second stage, the lesion is removed after the above procedure and depending on the condition of the patient. If necessary, the second procedure may be delayed. If the tumor is adherent to the skin, that portion of tissue is excised as well and the area is covered with grafts or flaps.17
Amputation
Amputation is the treatment choice for highly aggressive malignancies such as hemangiosarcoma, lymphangiosarcoma, aggressive hemangioendothelioma, and massive arteriovenous malformations that have created a nonfunctional extremity.
This should be performed with a proximal tourniquet for operative hemostasis.
If the lesion is too proximal for a tourniquet, an internal vascular balloon can be used to occlude the feeding vessel or vessels.
Guillotine amputation is an option if infection is present; otherwise, closure should be performed at the time of amputation.
The most common error we have seen after amputation of a digit or hand is failure to obtain adequate, tension-free soft tissue coverage.
Wide local excision may be considered for less aggressive hemangioendothelioma, hemangiopericytomas, malformations, and hemangiomas that have not involuted.
TECHNIQUES
TRANSUNGUAL EXCISION
Transungual excision is an approach to subungual lesions, such as glomus tumors.
Make small radial and ulnar corner incisions over the nail fold (TECH FIG 1A,B).
Half the nail is then elevated and folded over, allowing for visualization of the nail matrix (TECH FIG 1C).
The nail can be completely removed with a Freer elevator if necessary for access to the tumor (TECH FIG 1D).
Make a longitudinal incision with a no. 15 blade into the nail matrix, directly over the tumor, and excise the lesion circumferentially down to the phalanx (TECH FIG 1E,F).
Curette the bone before the nail bed is closed with 6-0 plain gut.
Replace the nail into the eponychial fold as a dressing for the nail bed and suture the corner incision closed (TECH FIG 1G).15,21
TECH FIG 1 • A. Radial and/or ulnar incisions of the nail fold are drawn. If the lesion is proximal in the nail bed, one or both of these incisions may be necessary to access the lesion. B. The incisions are at oblique angles to the nail fold to avoid contracture of the area. C,D.The nail plate is elevated off the nail bed with a Freer elevator. Half the nail is elevated primarily (C), but the entire nail may be removed to allow for access to the lesion (D). Incision(s) are then extended, if necessary, to allow for visualization. E,F. A longitudinal incision is made in the nail bed to allow for removal of the lesion. The bone is curetted to remove any tumor and the nail bed is then closed with 6-0 or 7-0 plain gut. G. The nail plate is then replaced as the dressing and the incision(s) are closed with 5-0 or 6-0 nylon or chromic.
LATERAL INCISION
This is an alternative to the transungual excision and allows exposure of the dorsal distal phalanx without violating the nail matrix. Because the view of the tumor is narrower, we do not recommend this approach.15
If this approach is to be used, then a longitudinal midaxial incision slightly dorsal to the neurovascular bundle is used (TECH FIG 2A).
The incision is placed on the radial or ulnar surface of the digit, based on the location of the lesion. Sharp dissection is carried out to the distal phalanx without manipulating the surrounding soft tissue.
A small, sharp elevator is used to create a subperiosteal dorsal flap (TECH FIG 2B).
A small curette or elevator is used to excise the lesion.
The flap is replaced and the incision is closed with interrupted or running nylon suture.21,27
TECH FIG 2 • A. A midlateral incision is drawn just dorsal to the midaxial line. The incision is carried sharply down to the bone, keeping the neurovascular bundle volar to the incision and dissection. B. A Freer elevator is then used to create a subperiosteal flap to allow removal of the lesion. The incision is then closed with 5-0 or 6-0 nylon.
EPIPHYSIODESIS
Epiphysiodesis, destroying the growth plate by scraping or drilling, may help to diminish hypertrophy in patients whose digits have reached adult size.
Make a midaxial incision sharply, with dissection continued to the bone.
Retract the neurovascular bundle volarly to avoid injury (TECH FIG 3A).
The dorsal branches may be transected if it is necessary to gain access to the dorsal aspect of the phalanx.
Use a drill to destroy the growth plate of the phalanx (TECH FIG 3B).
Close the incision with 5-0 or 6-0 nylon.
TECH FIG 3 • A. A midlateral incision is made sharply and dissection continues to the level of the bone. The neurovascular bundle is retracted with the volar flap to ensure that it is not injured during dissection. The dorsal branches may be ligated or left intact, if it does not interfere with the exposure of the phalanx. B. A drill is used to annihilate the growth plate of the phalanx to halt its growth. The incision is then closed with 5-0 or 6-0 nylon.
POSTOPERATIVE CARE
After excision of the lesion, most patients will require a bulky dressing, and most will be able to return to their normal activity within 1 to 2 weeks.
Patients with partial resection of arteriovenous malformations may need to continue wearing compressive garments postoperatively when the dressings are removed.
If patients required skin grafts or flaps, dressings and splints can be left in place to keep the patient from shearing the graft or pulling at the flap until the incisions are healed.
Graft bolsters or splints should be left in place for about 3 to 5 days to allow the graft to adhere well.
For patients who require amputations, prosthetics may be formed, depending on the level of the amputation. These are more readily available for patients who have belowor aboveelbow amputations, although patients who have forequarter amputations may also be candidates for specialized prosthetics.
Patients will require physical therapy to teach them how to use prosthetics or to relearn hand function, if wide excisions were necessary.
OUTCOMES
The prognosis of hemangiomas is not affected by race, gender, tumor site, size, or presence at birth.16
Attempts to excise arteriovenous malformations may lead to serious complications.
Complications are seen in about 22% of slow-flow lesions and 28% of fast-flow lesions. Wound dehiscence, seromas, and hematomas are noted early on. Partial skin loss and incision site infection are seen in the late postoperative period.
In fast-flow malformations, episodic bleeding and wound breakdown are more common.23
After resection of venous malformations and lymphatic malformations, persistent edema and swelling are more frequent. Patients with type C malformations more consistently require multiple operative procedures due to complications.
Disseminated intravascular coagulation has been reported, and coagulation studies should be obtained before any intervention.
In the study by Mendel and Louis,16 13 of 17 lesions persisted after excision through extension or recurrence. Ten of these lesions were diffuse. Thus, two fifths of lesions that are thought to be localized are diffuse and will require more than one procedure for complete excision.
In view of the high recurrence rate, excision should be considered in specific situations. Partial resection might be chosen to provide relief of symptoms, but as a balance between aggressive resection and preservation of function.16
Patients who had wide local excision of venous malformations were found to have a 2% recurrence rate.14
It is generally accepted that primary tumor excision is the treatment of choice in all adults with venous malformations and children who have been observed for 1 year without regression of the lesion.
Glomus tumors recur in 15% to 24% of patients, with an average time before recurrence of 2.9 years.
Late presentation of recurrence is thought to be due to a new tumor near the site of excision. Patients who had incomplete excisions had recurrence of the tumor within weeks of surgery.
In patients who had transungual excisions, nail deformities were noted in 26% of patients postoperatively.
The prognosis of hemangioendothelioma depends on the grade of the tumor. Patients with low-grade lesions have a good long-term survival rate, and those with aggressive tumors may not survive longer than 2 years.11
Kaposi sarcoma in elderly non-HIV patients may be cured with wide-local excision; however, the accepted treatment for these patients is chemoradiation and alpha-interferon therapy. The 5-year survival rate of these patients is only 19%. In patients with HIV/AIDS, the mortality rate of Kaposi sarcoma was 80% at 2 years, but this has improved with highly active antiretroviral therapy (HAART).17
For patients with hemangiosarcoma, early radical amputation is the treatment of choice. Palliative radiation has also been used. The average survival is 2.5 years, and the 5-year survival rate is less than 20%. One third of patients with hemangiosarcoma have hemorrhage or coagulopathy, and 45% have nodal metastases.4,17
Glomangiosarcomas are believed to be low-grade malignancies; however, more than 25% of reported cases develop metastases.12 Wide local excision is the treatment of choice for these lesions, and close long-term follow-up is necessary.
COMPLICATIONS
High-output cardiac failure
Consumptive coagulopathy
Bacterial endocarditis
Distal ischemia
Tissue loss
Local infection
Compartment syndrome
Arterial steal
Hematoma
Seroma
Partial wound dehiscence
Cellulitis at the operative site
Hypertrophic scarring
Joint contracture
Neuromas
Reflex sympathetic dystrophy
Pain
Partial or total extremity gangrene
Vesicle formation
Recurrence
Amputation
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