Green – 1987 introducere cu principii..

BENIGN SOFT TISSUE LESIONS

Ganglions and Mucous Cysts (KAS)

- De obicei apare intre TELP si ECD deasupra ligam scafolunat

Clinical Characteristics
Ganglion cysts are the most common soft tissue tumors of the hand. These mucin-filled cysts are usually attached to
the adjacent underlying joint capsule, tendon, or tendon sheath. Ganglions are most prevalent in women and
generally occur (70%) between the second and fourth decades of life. They are not rare in children and have been
reported from the first to the eighth decades. Ganglions usually occur singly and in very specific locations; however,
they have been reported to arise from almost every joint of the hand and wrist (Table 59.4).

The less common ganglions are often associated with other conditions of the hand (e.g., bossing of the
second and third carpometacarpal joints, de Quervain disease, and Heberden nodes of the DIP joint).
Ganglion cysts have also been reported to cause clinically symptomatic pressure on the median and ulnar
nerves of the hand. Because of their high incidence and prevalence, ganglion cysts should readily be
recognized by most hand surgeons.
Other conditions that cause diffuse swelling over the dorsum of the wrist, such as extensor tenosynovitis,
lipomas, and other hand tumors, also must be considered in the differential diagnosis.
The history, physical examination, techniques of transillumination, and aspiration should allow a
conclusive diagnosis in most instances. Patients usually seek medical attention because of the cosmetic
appearance of the mass, pain, weakness, and concern for potential malignancy. A specific antecedent
traumatic event is present in at least 10% of cases, and repeated minor trauma may be an etiologic factor
in their development. There is no obvious correlation with patient occupation. Malignant degeneration has
never been reported; however, malignant soft tissue
tumors are frequently misdiagnosed as ganglion cysts. Ganglions can appear quite suddenly or develop
over a period of several months. They may subside with rest, enlarge with activity, and rupture or
disappear spontaneously. Although recurrences are infrequent with proper excision, >50% may recur if
incompletely excised.
Radiographs of the involved region are often unremarkable, although intraosseous cysts are occasionally
present at the wrist.
Osteoarthritic changes are commonly seen with cysts at the DIP or carpometacarpal joints.
Communication between the wrist joint and cyst has been demonstrated with arthrograms but not with
cystograms. A one-way valvular mechanism has therefore been postulated to connect the wrist joint to the
cyst.

ANATOMY
The microscopic description of ganglions is well known. The main cyst may be single or multiloculated
and appears smooth, white, and translucent. The wall is made up of compressed collagen fibers and is
sparsely lined with flattened cells without evidence of an epithelial or synovial lining.
The capsular attachment of the main cyst reveals mucin filled “clefts,” which have been shown by serial
sections to intercommunicate through a tortuous continuous duct connecting the main cyst with the
adjacent underlying joint. The stroma surrounding the intracapsular ducts may show tightly packed
collagen fibers or sparsely cellular areas with broken collagen fibers and mucin-filled intercellular and
extracellular lakes.
The contents of the cyst are characterized by a highly viscous, clear, sticky, jelly-like mucin made up of
glucosamine, albumin, globulin, and high concentrations of hyaluronic acid. In some cases, the mucin
may be blood tinged. The contents of the cyst are decidedly more viscous than normal joint fluid.

Nonoperative Treatment
Nonsurgical treatment has included digital pressure, injections of hyaluronidase or sclerosing solutions,
subcutaneous tenotome dissection, and cross fixation with a heavy suture.
The most effective nonsurgical treatment is patient reassurance.
An explanation of the condition and assurance of its nonmalignant nature are often the only treatment
sought or required. Surgery is best reserved for patients with persistently symptomatic ganglions.
Operative Treatment
Open procedures are most commonly done for excision of ganglion cysts. Treatment principles include
attempts to minimize scar formation and loss of range of motion. Data on arthroscopic approaches to both
dorsal and volar carpal ganglion cysts have been published.

Dorsal Wrist Ganglion

Clinical Characteristics.
The main cyst is usually located directly over the scapholunate ligament and is
easily seen and diagnosed. The cyst may occur anywhere else between the extensor tendons, however,
and can be connected to the ligament through a long pedicle.
Failure to identify this pedicle and excise its attachment to the scapholunate ligament increases the
likelihood of recurrence. Careful preoperative palpation of the cyst with digital compression often reveals
its extent and the direction of the pedicle. Transillumination or aspiration confirms the diagnosis
preoperatively.
Although ganglions have been reported in other carpal joints, they are rare and attachments to the
scapholunate joint must be ruled out before a dissection is considered complete.
Review of the patient’s preoperative radiographs to rule out an interosseous component is wise.

AUTHOR’S PREFERRED METHOD OF TREATMENT

Operative Technique. Most dorsal ganglions can be approached through a transverse incision over the
proximal carpal row, but a modified incision or second transverse incision may be necessary for
ganglions not directly over the scapholunate ligament (Figure 59.17).
The diagnosis of ganglion cyst should be made before commitment to a transverse incision because this
type of incision is not readily incorporated into a limb-sparing incision in the event of a subsequent
diagnosis of a malignant soft tissue tumor. Typically, a dorsal ganglion appears between TELP and ECD
tendons, which are retracted radially and ulnarly, respectively.
The main cyst and its pedicle are mobilized down to the underlying joint capsule. With the wrist in volar
flexion, the joint capsule is opened along the border of the radius and scaphoid’s proximal pole. The
capsule is elevated and retracted distally to expose the capsular attachments to the scapholunate ligament
(Figure 59.20).

Smaller intraarticular cysts are often seen attached to the scapholunate ligament. The capsular incision is
then continued around the ganglion, but all capsular attachments to the ligament are left intact. The
capsular incision is extended more laterally if any capsular ducts, which can be identified by small
amounts of mucin drainage, are encountered during the dissection. The ganglion and its capsular
attachments are then tangentially excised off the scapholunate ligament (Figure 59.22).
A small, mucin-filled duct is invariably seen piercing the transverse fibers of the scapholunate ligament.
This duct appears to connect the underlying scapholunate joint with the main cyst. Synovial and capsular
attachments along the distal margin of the scapholunate ligament are also excised to give an unobstructed
view of the head and neck of the capitate (Figure 59.23). If the ganglion ruptures and its anatomic
features are lost during the dissection, it should continue until all attachments to the scapholunate
ligament have been excised.

The excised portion of the joint capsule usually measures approximately 1.0 to 1.5 cm. It is neither
necessary nor desirable to cut into the scapholunate ligament, nor is curettage of the scapholunate joint
necessary.
The tourniquet is released and hemostasis obtained, then the wound is closed and a dressing applied.
Attempts to close the joint capsule either primarily or with fibrous flaps are contraindicated.
Subcuticular closure with a monofilament, nonabsorbable pull-out suture may minimize scar formation.
Splinting the wrist in slight flexion may reduce postoperative flexion loss.

Postoperative Care. A bulky dressing extending from the proximal part of the forearm to the MP joints
is applied and the hand elevated. Early finger motion is encouraged. The dressing and sutures are
removed between 7 and 10 days postoperatively. Wrist motion should be initiated and encouraged,
especially volar flexion. Hand therapy is continued until a full range of motion has been obtained.

Complications.
Early recurrences, the most common complication of ganglion surgery, are usually the result of
inadequate and incomplete excision and rarely should occur. Ganglions appearing at the same site years
after excision may in fact be new ganglions.
Stiffness of the wrist can be avoided by early motion, physical therapy if necessary, and splinting of the
wrist in slight flexion during the immediate postoperative period. It is imperative that early volar flexion
be stressed.
To avoid keloid or hypertrophic scars, longitudinal incisions across the wrist joint should be avoided.
Awareness and respect for the sensory branches of the radial and ulnar nerves prevent neuroma
formation, which often defies (sfideaza) effective treatment. I have not seen avascular necrosis of either
the lunate or scaphoid or scapholunate dissociation after the surgical technique just described. Ganglions
and sprains of the intercarpal ligaments can occur concomitantly after trauma to the wrist and must be
distinguished preoperatively. Identification of concomitant wrist pathology may be facilitated with
arthroscopic treatment.

Occult Dorsal Carpal Ganglion

Unlike protruding dorsal ganglions, smaller, occult dorsal ganglions are easily overlooked and can often
only be palpated with the involved wrist in marked volar flexion. Comparison with the opposite
normal wrist is helpful. An occult ganglion may be the cause of unexplained wrist pain and is
disproportionately tender. MRI is useful in confirming the diagnosis and differentiating pain related to
scapholunate ligament injury. Dorsal ganglions occasionally do occur in association with an underlying
scapholunate diastasis, and they may be blamed for the carpal instability after their excision. Dorsal
prominence of the proximal pole of the scaphoid secondary to intercarpal instability may be confused
with a painful occult ganglion and must be diagnosed with appropriate radiographic studies to avoid a
delay in proper treatment. Excising the ganglion alone might not alleviate all the patient’s preoperative
pain. Excision of the posterior interosseous nerve at the level of the radiocarpal joint may help alleviate
the pain and add to the patient’s postoperative comfort. If other causes of wrist pain and tenderness,
especially directly over the scapholunate ligament, can be excluded, an occult dorsal ganglion is best
initially treated conservatively by immobilization and steroid injections directly into the dorsal capsule,
which can also aid in diagnosis. In cases in which further diagnostic studies are necessary, some authors
have found the use of MRI and ultrasonography helpful.
Chronic tenosynovitis of the extensor tendons can be confused with a dorsal wrist ganglion but can be
easily distinguished by the diffuse nature of the swelling and the puckering (încrețire) seen with digital
extension, the so-called tuck sign.

Volar Wrist Ganglion

Clinical Characteristics.
The second most common ganglion of the hand and wrist is a volar wrist ganglion (18-20%). The
majority of volar ganglions occur either directly over the distal edge of the radius or slightly more distally
over the scaphoid tubercle. The former arises from the capsular and ligamentous fibers of the radiocarpal
joint and occurs under the volar wrist crease between the FRC and LAP (Figure 59.24).
The main cyst may be intertwined with bifurcating branches of the radial artery, thus making delicate
dissection imperative. The other type of volar ganglion arises from the capsule of the scaphotrapezial
joint. Although volar ganglions may appear small clinically, they can be surprisingly extensive at surgery.
Multiloculated cysts extending under the thenar muscles, along the flexor carpi radialis tendon, into the
carpal canal, and under the first extensor compartment adjacent to the dorsal branch of the radial artery
and as far dorsally as the first web space may be encountered. These extensions can often be appreciated
preoperatively by careful palpation and digital compression of the ganglion. It is important to assess the
patency of the radial and ulnar arteries. The Allen test should be performed routinely and ulnar artery
occlusion excluded. The surgeon must be aware of the importance of preserving the radial artery,
particularly in patients with a radial-dominant circulation.

AUTHOR’S PREFERRED METHOD OF TREATMENT

Operative Technique.
Although the surgical technique of excision of a volar ganglion is similar to that for a dorsal ganglion,
exposure and precise identification of the capsular attachments of a volar ganglion are more difficult. The
incision must be planned to allow for extension into the carpal tunnel or base of the thenar muscles
(Figure 59.25).

Longitudinal incisions allow for optimal visualization. With the skin flaps retracted, the forearm
fascia is incised longitudinally and the dome of the cyst identified and mobilized. Particular care
should be taken to identify and protect the radial artery, which is frequently intimately attached
to the wall of the ganglion and may even be completely encircled by the ganglion (Figure 59.26).
Loupe magnification aids in this dissection. The pedicle is traced to the volar joint capsule
(usually the scaphotrapezial or radiocarpal ligament). The joint is opened and explored and the
capsular attachments are excised (approximately 3 ± 4 mm). Once the ganglion has been excised,
the surrounding tissues can be compressed digitally to rule out further mucin-filled pockets. If
unidentified extensions are present, they must be excised.
Hemostasis, wound lavage, and a simple skin closure (preferably subcuticular) complete the
operation. Again, capsular closure is unnecessary and only delays early mobilization.
Postoperative Care. A bulky bandage and elevation of the hand ensure early postoperative
comfort. Follow-up care is similar to that for dorsal ganglions, and motion of the wrist should
begin within the first 2 weeks after surgery. Complications. The complications are similar to
those of dorsal ganglions. Unexpected branches of the radial sensory or lateral antebrachial
cutaneous nerves may be injured and lead to troublesome neuromas. Extensions of the routine
incision into the carpal canal must avoid injury to the palmar cutaneous branch of the median
nerve. Injuries to the radial artery can be repaired microscopically. Some authors recommend
leaving a portion of the cyst wall attached to the artery to avoid arterial injury. Stiffness of the
wrist is less common than with dorsal ganglions, but it can occur if early motion is not
encouraged. Unpleasant scars are not an uncommon problem on the volar aspect of the wrist and
often defy “plastic” revisions. Curved incisions appear to consistently provide more attractive
scars, especially near the volar wrist creases (see Figure 59.25).

Volar Retinacular (Flexor Tendon Sheath) Ganglion

Clinical Characteristics.
The third most common ganglion (10-12%) of the hand is the volar retinacular one, which arises
from the proximal annular ligament (A1 pulley) of the flexor tendon sheath. This ganglion is
invariably a small (3-8 mm), firm, tender mass palpable under the MP flexion crease (Figure
59.27).

The cyst is attached to the tendon sheath and does not move with the tendon. Needle rupture
followed by a steroid injection and digital massage to disperse the cyst’s contents can frequently
delay or obviate the need for surgery. Several attempts at conservative treatment are
recommended before surgery, with the patient’s understanding that recurrences might happen.
The proximity of the digital nerves must be taken into account.

AUTHOR’S PREFERRED METHOD OF TREATMENT

Operative Technique. The ganglion is approached through an oblique incision over the mass
(see Figure 59.27). Transverse incisions do not allow adequate exposure without undue skin
traction and are not easily incorporated into an extensile incision, which also must allow
identification and mobilization of the radial and ulnar neurovascular bundles. The ganglion can
then be traced to the tendon sheath and excised with a small portion of the sheath (Figure 59.28).

After skin closure, a simple dressing is applied and early motion allowed.

Mucous Cyst
Clinical Characteristics. A mucous cyst is a ganglion of the DIP joint that generally occurs
between the fifth and seventh decades. The earliest clinical sign may be longitudinal grooving of
the nail, without a visible mass, caused by pressure on the nail matrix (Figure 59.29).

Usually, however, the patient is seen after the cyst has enlarged and attenuated the overlying
skin. The 3- to 5-mm cyst typically lies to one side of the extensor tendon and between the dorsal
distal joint crease and eponychium. The patient often has Heberden nodes and radiographic
evidence of osteoarthritic changes in the joint. The cyst and osteophytes both should be treated to
ensure a satisfactory result.

AUTHOR’S PREFERRED METHOD OF TREATMENT

Operative Technique. The cyst historically has been approached through an “L”-shaped or
curved incision (Figure 59.30, A), and any attenuated or involved skin that cannot be easily
separated from the cyst wall is excised elliptically (see Figure 59.30, B). The cyst is mobilized,
traced to the joint capsule, and excised with the joint capsule (Figure 59.31, A). From a practical
point of view, however, all soft tissue between the retracted extensor tendon and adjacent
collateral ligament needs to be excised and the DIP joint left exposed (see Figure 59.31, B).

Care should be taken to not disturb the insertion of the extensor tendon or nail matrix. With the
joint extended and the tendon retracted dorsally, the opposite side is explored and occult cysts or
hypertrophied synovial tissue is excised. Osteophytes can be excised with a rongeur (Figure
59.32). Skin closure may require rotation and advancement of a dorsal skin flap or a full-
thickness skin graft.
An alternative and my current preferred approach is to make a transverse incision centered over
the DIP joint. The base of the mucous cyst is identified and excised while leaving the distal and
superficial portion of the cyst intact. Osteophytes and joint capsule are excised and the skin
closed. The remaining portion of the cyst will involute over the course of several weeks, and the
skin complications seen with marginal excision are avoided. I have used this technique since its
report by Gingrass and colleagues and have not needed to perform any skin coverage. Patients
must be educated preoperatively to expect slow resolution of the cyst. Personal experience has
confirmed the results that were published in the report.

Complications.
Recurrences may be because of inadequate excision of the ganglion’s capsular attachments and
failure to recognize extensions of it under the extensor tendon to the opposite side. The
underlying arthritic process persists and can result in new ganglion formation. Relief of pressure
on the nail matrix by decompression or excision of the ganglion usually restores the nail to its
normal appearance. Stiffness rarely causes a functional problem.

Carpometacarpal Boss
Clinical Characteristics. Dorsal wrist ganglions can be confused with carpal bosses, which are
osteoarthritic spurs or prominences that develop la baza articulatiilor CMC II-III (or both)
(Figure 59.33).
A firm, bony, nonmobile, tender mass is visible and palpable at the base of the carpometacarpal
joints, especially when the wrist is flexed. Radiologically, the mass is best visualized with the
hand in 30 to 40 degrees of supination and 20 to 30 degrees of ulnar deviation (“carpal boss
view”). Bosses are more common in women, in the right hand, and between the third and fourth
decades. The mass may be asymptomatic, or the patient may complain of considerable pain and
aching. A small ganglion is associated with a carpal boss in 30% of cases, thus adding to
confusion of it with the more common dorsal wrist ganglion.
Every effort should be made to treat the carpal boss nonoperatively. Splinting, nonsteroidal
antiinflammatory medications, and ultrasound-guided cortisone injections should be used or
strongly considered prior to proceeding with surgery. The potential for persistent symptoms
following surgery must be emphasized.

AUTHOR’S PREFERRED METHOD OF TREATMENT

Operative Technique. The mass is approached through a transverse or oblique incision over the
bony prominence (Figure 59.34).

The extensor digitorum communis and extensor indicis proprius tendons are retracted ulnarward.
A ganglion, if present, can be mobilized and excised with its capsular attachments. The
osteophytes and carpometacarpal joints are approached through a separate longitudinal incision
over each involved joint. Subperiosteal dissection exposes the involved joint to the adjacent
osteophytes, which are then excised down to normal cartilage with small osteotomes. The
dissection may involve the second and third carpometacarpal joints individually or all four
opposing surfaces together (Figure 59.35). The surgical area is palpated through the skin to
ensure excision of all palpable prominences. The capsule, periosteum, and any adjacent
overlying tendon fibers (ECRL, ECRB) are reapproximated over the joint with a few fine
inverting sutures, which should not be palpable through the skin.
Postoperative Care. A cast or splint should be worn for 4 to 6 weeks to allow adequate
ligamentous healing and pain relief.

Complications. The most common complication is persistence of a mass. Pain may persist
unless all abnormal abutting surfaces have been excised. Dorsal wrist ganglions can occur over
the carpometacarpal joints and must be distinguished from carpal bosses with an associated
ganglion. Avoidance of injury to branches of the radial and ulnar sensory nerves is again
stressed.

Ganglions of the Proximal Interphalangeal Joint

Clinical Characteristics. Similar to a mucous cyst of the DIP joint, ganglions also occur dorsally
over the PIP joint on either side of the extensor tendon. They arise from the joint capsule and
usually pierce the oblique fibers between the central slip and lateral band. These cysts are small
(3-5 mm) and tender and may interfere with joint motion.
AUTHOR’S PREFERRED METHOD OF TREATMENT
Operative Technique. A curved or longitudinal incision over the PIP joint exposes the ganglion
(Figure 59.36).

The lateral margin of the lateral band is released from the transverse retaining ligament and
retracted dorsally to expose the PIP joint (Figure 59.37).
The pedicle from the main cyst usually can be followed through the extensor system into the
joint capsule. A small, elliptic incision through the oblique extensor fibers mobilizes the cyst and
pedicle. The entire joint capsule and synovial lining are excised between the collateral ligament
and extensor insertion on the middle phalanx. Postoperative Care. A simple skin closure and
dressing followed by early motion are all that is required.

Ganglions of Extensor Tendons

Clinical Characteristics. Ganglions do arise on or within extensor tendons. They typically occur
over the metacarpals and are distinguished by their proximal motion with the fingers in
extension.
They can, however, be confused with dorsal wrist ganglions, carpal bossing, or extensor
tenosynovitis. The patient may complain of tenderness, aching, or snapping of the tendon with
motion.

AUTHOR’S PREFERRED METHOD OF TREATMENT

Operative Technique. The ganglion is approached through a transverse incision (Figure 59.38),
and the intimate broad attachment to the extensor tendon is readily recognized. The ganglion
should be dissected off the extensor tendon with all the synovial tissue surrounding the involved
tendon. Rupture of the ganglion is difficult to avoid, but recurrences are rare.
Miscellaneous Locations Ganglions have been reported to occur in numerous other locations of
the hand and wrist and are often associated with various conditions, including those described in
the following subsections.

First Extensor Compartment (Dorsal Retinacular) Ganglion. Ganglions attached to the first
extensor compartment, similar to volar retinacular ganglions, are seen in patients with acute
stenosing tenosynovitis (de Quervain disease) (Figure 59.39). Therefore, in addition to releasing
the tendons (see Chapter 56), the involved tendon sheath and the ganglion can be excised. Injury
to the radial sensory nerve should be avoided, and a separate compartment of the extensor
pollicis brevis may be present and require release.
Carpal Tunnel. Symptoms consistent with compression of the median nerve have been ascribed
to ganglions arising from the volar carpus within the carpal canal. A volar wrist ganglion also
can extend distally and ulnarward to compress the median nerve. All carpal tunnel releases
should be accompanied by exploration of the canal to rule out extrinsic masses, including
ganglions.
Guyon Canal/Distal Ulnar Nerve. Ganglions within the ulnar canal (loge de Guyon) can
compress the ulnar nerve and cause motor or sensory loss, or both. These ganglions generally
arise from joints around the hamate and dissect through the hypothenar muscles or along the
motor branch. Ganglions in the palm can lead to isolated atrophy of the first dorsal interosseous
muscle. Early excision is imperative and avoids permanent injury to the ulnar nerve.
Intraosseous Ganglions Although rare and usually an incidental radiographic finding,
intraosseous ganglions are increasingly being recognized as a source of wrist pain. Surgical
treatment is best delayed, however, until all other possible causes of the patient’s discomfort
have been excluded. When indicated, curettage and bone grafting adequately treat the condition.
Careful exploration of the joint at the time of surgery helps rule out other undiagnosed causes of
the patient’s symptoms. Communication between the joint cavity and the intraosseous cyst has
not been consistently demonstrated. The histologic features of intraosseous ganglions are
identical to those of their soft tissue counterparts.

Epidermal Inclusion Cysts Epidermal inclusion cysts are thought to result from traumatic
implantation of epithelial cells into the underlying soft tissue or bone. The cells grow over an
interval of months to years to produce a painless swelling, most commonly in the fingertip.
Erythematous and painful lesions have been described. These lesions most frequently occur in
the distal phalanx of the left long finger and thumb in men in the third and fourth decades of life.
Epidermal inclusion cysts may be seen after traumatic or surgical amputation of the fingertip.
Metacarpal involvement is rare, and there are no reported cases occurring in the carpus. Patients
typically note a slowly growing mass on the fingertip or thumb tip, even though a history of
trauma (penetrating injury) may not be recalled. When confined to soft tissue, the cyst is usually
well circumscribed, firm, and slightly movable. The differential diagnosis can be more difficult
when the cyst involves bone, in which case radiographs generally show a welldemarcated lytic
lesion. Occasionally, cortical penetration can be seen, and the lesion may mimic a malignant or
infectious process. More than a fourth of reported lesions have been treated by primary
amputation, before pathologic diagnosis, out of concern for an aggressive neoplasm or advanced
bone destruction. Patients with a lytic lesion of the distal phalanx should undergo biopsy before
consideration of an ablative procedure. Epidermal inclusion cysts are readily treated by marginal
excision or by curettage and bone grafting for lesions involving bone. In rare cases with
advanced bone destruction, amputation may be an alternative. Recurrence is uncommon, and
malignant transformation has not been reported.

Foreign Body Lesions

Foreign body lesions are traumatic reactive masses rather than true neoplasms. These lesions are
a response to implanted foreign material. The tissue’s reaction to foreign material varies with the
nature of the foreign substance deposited, the anatomic site involved, the duration of the implant,
and the presence or absence of tissue hypersensitivity to the material. This response is the host’s
method of isolating recognized extraneous substances. Glass, wood, and metal are the most
common types of particulate matter associated with a foreign body reaction. These lesions are
usually relatively superficial in location. It is important to establish the cause of the lesion
because the differential diagnosis includes malignant soft tissue neoplasms. A history of
penetrating trauma or identification of a foreign body with radiographs or ultrasonography can
provide this information. It may be impossible to preoperatively distinguish between an inclusion
cyst, a foreign body reaction, or a soft tissue neoplasm. Excisional biopsy of these relatively
small lesions allows pathologic analysis and confirmation of the diagnosis and results in a cure
for the problem.

Lipomas
Lipomas are common benign fat tumors that typically occur in several locations in the
hand,14,20,139 most commonly subcutaneously or intramuscularly. Lipomas have been
reported to arise in association with tendon sheaths, in the carpal tunnel, in the Guyon canal,
and in the deep palmar space. They are soft nontender masses that do not transilluminate.
Symptoms of nerve compression may be noted if the tumor is in the deep palmar space or in
proximity to a major nerve. Such lesions are frequently present for several years and may grow
slowly. When the lesion is subcutaneous, it is readily recognized by its welldemarcated
appearance and rubbery consistency. Plain radiographs may reveal a soft tissue shadow, and
MRI may be particularly useful by demonstrating signal characteristics that are significantly
suggestive of fat, which is often similar to surrounding subcutaneous fat. Lipomas are usually
well demarcated and are readily dissected from surrounding soft tissues, but occasionally the
tumor may be immediately adjacent to nerves, thus making dissection difficult. When the
patient’s physical examination and radiographs are extremely suggestive of a lipoma, a small
lesion can be treated by excisional biopsy. Deep lesions, such as those occurring in the carpal
tunnel, should be studied preoperatively with MRI. If the signal characteristics are not clearly
suggestive of lipoma, a small incisional biopsy should be done to establish the diagnosis and
prevent operative contamination of major nerves. Marginal excision of lipomas is generally
curative, although recurrence may be seen.

Lipofibromatous Hamartomas Lipofibromas or lipofibromatous hamartomas are unusual

tumors of peripheral nerves that most commonly involve the median nerve when seen in the
upper extremity. Rare instances of ulnar or radial nerve involvement have been reported.
Patients usually complain of slowly progressive swelling in the distal part of the forearm or
palm, and symptoms of nerve compression may be present. The mass is most commonly noted
in childhood or adolescence and is thought to be congenital or developmental in origin. MRI
findings can be diagnostic. Surgical exploration reveals either diffuse fusiform swelling of the
nerve without extension into perineural tissues or a more circumscribed area of involvement.
The mass is closely associated with the nerve fibers and is not readily dissected free. Attempts
at partial excision or interfascicular dissection frequently result in permanent loss of motor or
sensory nerve function and should be avoided in the presence of characteristic MRI
findings.91,161,191 Biopsy of a cutaneous branch of the nerve can be done to confirm the
diagnosis if MRI findings are not diagnostic.161 Additional surgical treatment is directed only
at decompressing the nerve. Spontaneous decrease in tumor size and improved nerve function
have been reported after nerve decompression only. If the size of the lesion requires that it be
excised, Houpt and associates have advocated complete excision and nerve grafting in children
and immediate tendon transfers in adults.91 Histologic examination of the lesion reveals nerve
tissue with surrounding fat cells and a smaller component of fibrous tissue. Long-term follow-up
of patients with such lesions has demonstrated slow but progressive loss of nerve function.

Giant Cell Tumors of the Tendon Sheath (Pigmented Villonodular Tenosynovitis)

A giant cell tumor (GCT) of a tendon sheath is a benign soft tissue tumor and is the second most
common tumor seen in the hand (after ganglions). Several terms have been used to describe this
lesion, including localized nodular synovitis, fibrous xanthoma, and pigmented villonodular
tenosynovitis. Giant cell tumor of the tendon sheath is not a particularly good term because the
lesion does not uniformly contain giant cells and is not necessarily associated with a tendon
sheath. The tumor generally occurs on the volar surface of the fingers or hand, although dorsal
involvement is not uncommon. There is a propensity for involvement of the radial three digits
and the DIP joint region.145 Clinically, the tumor is most often firm, nodular, and nontender.
Because it grows slowly, it may be present for a long time before the patient is seen. A sensory
deficit may be present when the lesion occurs in proximity to the digital nerves. The lesion does
not transilluminate, which helps distinguish it from a ganglion. Plain radiographs may
demonstrate a soft tissue mass or pressure erosion of underlying bone.104 Bone invasion, as
can be seen with diffuse pigmented villonodular tenosynovitis, is not typical and suggests a
more aggressive neoplasm. Actual bone invasion is rare but has been described.198 Marginal
excision is the recommended treatment, although the reported incidence of recurrence is 5 to
50%.83,145 The high risk for recurrence is thought to be caused by the presence of satellite
lesions or incomplete excision. Statistically significant risk factors for local recurrence include
degenerative joint disease, location at the DIP joint, and radiographic evidence of pressure
erosion.166 If extensive joint involvement or degenerative changes are seen, arthrodesis may be
necessary after excision. Radiation can be considered for multiply recurrent or aggressive
lesions.40 A malignant form of GCT of the tendon sheath has been adequately described.

Schwannomas (Neurilemomas) Neurilemoma, or schwannoma, is the most common benign

nerve tumor occurring in the upper extremity. This lesion arises from Schwann cells and
produces a slowly growing, well-circumscribed, eccentric lesion in the peripheral nerve.
Neurilemomas are most commonly seen on the flexor surface of the forearm or hand in the
fourth, fifth, or sixth decades. Patients most frequently notice a painless mass, but less com
monly there may be a neurologic deficit. Palpation or compression of the lesion can produce
radiating pain in a specific nerve distribution. The lesion is often mobile in a transverse direction
but not longitudinally. It is frequently misdiagnosed as a ganglion and may have a similar
consistency. MRI can be useful in delineating the lesion, but it may not be possible to
distinguish neurilemoma from a neurofibroma or malignant peripheral nerve sheath tumor
(Figure 59.42).189 Neurilemoma can be dissected free or “shelled out” from the surrounding
nerve. Interfascicular involvement may be seen rendering dissection more difficult. Optical
magnification and microsurgical techniques have been advocated to reduce the risk for
postoperative neurologic deficit. The incidence of postoperative neurologic deficit is
approximately 5 to 15%,182 with a significantly greater risk of a deficit occurring with
procedures done after incisional biopsy or recurrence.50 In the overwhelming majority of cases,
the lesion can be completely removed and neurologic symptoms, if present preoperatively, are
improved. Recurrence is uncommon. Multiple lesions in a single peripheral nerve have been
described. There are rare reports of malignant transformation.

Neurofibromas Neurofibromas are benign nerve tumors that arise within nerve fasciculi and
are typically more difficult to excise than neurilemomas. 50 Solitary lesions may be seen, but
multiple neurofibromas are more common in the setting of neurofibromatosis (von
Recklinghausen disease). Symptoms and clinical findings may be similar to those seen with
neurilemomas. At surgery, a more prominent nerve fascicle or group of fascicles can be seen
entering and exiting the lesion if both proximal dissection and distal dissection are done under
magnification.50 Excision of the lesion requires transection of the entering and exiting fascicles.
Postoperative neurologic deficit is more common than after excision of neurilemomas.
Progression of motor and sensory nerve deficits has been noted after surgical treatment of
plexiform neurofibromas in patients with neurofibromatosis. 50 Surgical treatment of
neurofibromas in patients with neurofibromatosis should be reserved for lesions that are
growing or producing progressive symptoms. There is a known risk for malignant degeneration
of neurofibromas in patients with neurofibromatosis.

Granular Cell Tumors (Granular Cell Myoblastomas) A granular cell tumor is a neoplasm
that is rarely seen in the forearm and hand.58,64,132 This lesion appears to arise from neural
elements rather than from myoblasts, as originally believed, and it has been found to occur in
close association with nerves (Figure 59.43).58 Patients usually note a painless mass.
Multicentricity has been reported in 10 to 15% of cases. Excision of the tumor has been
curative, although there has been no critical analysis of the need for wide margins at excision. A
malignant variant of a granular cell tumor has been reported; it is rare and difficult to distinguish
from the benign lesion.58 Malignant lesions are highly aggressive and require wide excision
and consideration of systemic treatment.

Fibromatoses
Digital Fibroma of Infancy Digital fibroma of infancy is a benign but very aggressive fibrous
lesion seen virtually exclusively in the fingers or toes (Figure 59.44), with more than 80% of
cases arising before the age of one.19,61,94 Digital fibroma of infancy generally occurs in the
region of the interphalangeal joints of the fingers. The lesions are usually small, dome shaped,
skin colored, and painless. The true natural history of this condition is not known. Observation
has documented progression with interphalangeal contracture, angular deviation, and bone
deformity in severely affected children. 19,61 There are reports of spontaneous resolution of the
skin lesions, with some patients having residual deformity.19 Microscopic evaluation has
demonstrated intracytoplasmic inclusion bodies, which has resulted in speculation of a viral or
myofibroblast cause of the lesions. Treatment has included observation, marginal excision,
wide excision with coverage, and amputation. No documented cases of metastasis have been
reported, but the incidence of local recurrence after excision is approximately 60%. Although no
firm treatment guidelines exist, it seems prudent to observe these lesions until spontaneous
resolution occurs or early signs of deformity or contracture are seen. Wide excision and skin
grafting appear to give the best local control and functional results, but there is a significant risk
for local recurrence and even subsequent involvement of adjacent digits.61
Juvenile Aponeurotic Fibroma Fibrous lesions in the hand are commonly seen in childhood
and adolescence and are frequently difficult to classify and treat. Juvenile aponeurotic fibroma,
or calcifying aponeurotic fibroma, is a benign, aggressive fibrous lesion seen during childhood
or adolescence that most commonly affects the palm.3,34,107,114 This lesion is manifested as
a small painless mass, often closely asso ciated with tendons or neurovascular structures. The
histologic appearance can be difficult to distinguish from fibrosarcoma. Wide excision with
sparing of functional or vital structures is the recommended treatment, but the recurrence rate
has been reported to be higher than 50%.34 On rare occasion, amputation has been necessary to
control the disease. There is one reported case of metastasis after recurrence of juvenile
aponeurotic fibroma in the hand, although the metastasis was classified as fibrosarcoma.114
Local and systemic surveillance should be considered for patients with this lesion. Recurrent
lesions can be observed if they do not produce functional impairment and the diagnosis is not in
question.

Nodular Fasciitis

Pseudosarcomatous fasciitis, subcutaneous pseudosarcomatous fibromatosis, and infiltrative

fasciitis are terms that have been used to describe the entity currently called nodular fasciitis. It
is an uncommon but well-described reactive lesion that is poorly understood and may simulate a
sarcoma.17,187 This lesion most commonly occurs on the volar surface of the forearm and
rarely can occur in the hand and digits.105 Patients usually note a rapidly growing small nodule
that has been present for a very short time, frequently less than a month. There may be a history
of local trauma and tenderness on palpation. The histologic appearance has been confused with
fibrosarcoma and myxoid liposarcoma, which has led to overtreatment.187 The diagnosis of
nodular fasciitis is rarely anticipated preoperatively. The natural history of nodular fasciitis
appears to be self-limited. Recurrence after marginal excision should raise the question of an
alternative diagnosis because even incompletely excised lesions have not been noted to recur.17
The most important issue in treating patients with nodular fasciitis is to differentiate this lesion
from a soft tissue sarcoma to avoid unnecessary aggressive surgical treatment. Molecular
genetic testing of pathologic specimens can assist in establishing a diagnosis as genetic studies
have identified frequent presence of the USP6 gene rearrangement by interphase fluorescence in
situhybridization (FISH).

MALIGNANT SOFT TISSUE LESIONS

Soft Tissue Sarcomas

Soft tissue sarcomas are relatively rare in the hand and forearm.
The most common histologic subtypes of soft tissue sarcoma seen in the hand are epithelioid
sarcoma, synovial sarcoma, and malignant fibrous histiocytoma.

Clinical Picture The clinical characteristics of the various types of soft tissue sarcomas in the
upper extremity and hand can be similar. Most frequently, patients note a painless mass that may
have been present for a long time with recent growth.28,194 Less commonly, a painful mass
may be reported. Epithelioid sarcoma is notable for manifestation as an ulcerating nodule that
can be mistakenly treated for infection.185 Soft tissue sarcomas in the hand are frequently
misdiagnosed as infection, ganglion, and lipoma. This can lead to inappropriate treatment or to a
delay in treatment. Physical examination of a patient with a suspicious lesion includes careful
assessment of size, location, depth, mobility, and relationship to major nerves, vessels, tendons,
and bone. Regional lymph nodes are assessed. Plain radiographs are obtained and may
demonstrate soft tissue calcification, fat density, or unexpected bone involvement, which can
assist in making a diagnosis (Figure 59.45). MRI has become increasingly valuable in defining
the pathologic anatomy, determining the local extent of disease, and aiding preoperative
planning. Biopsy Soft tissue masses that are symptomatic, enlarging, or not readily diagnosed
by physical examination should be considered for biopsy. Very small lesions that can be excised
with a surrounding cuff of normal tissue can be treated by excisional biopsy before a known
diagnosis. Large lesions or lesions in proximity to major nerves, vessels, or tendons are best
treated with incisional biopsy. The decision of how to perform the biopsy is particularly
important in the hand because of the Incisional biopsies or biopsies in which the tumor is
inadvertently violated result in contamination of all tissues included in the exposure. Poorly
planned biopsies may compromise the ability to perform limb-sparing surgery. It is best to plan
the biopsy incision to be in line with or incorporated into the definitive limb salvage or
amputation procedure that may be required in the event of a high-grade malignant diagnosis.
Transverse, oblique, and zigzag (Bruner) incisions should specifically be avoided. Staging
After the diagnosis of a soft tissue sarcoma, systemic staging should be done with CT of the
chest and axilla. Soft tissue sarcomas of the upper extremity most commonly metastasize to the
lungs or regional lymph nodes.68,74 Lymph node metastasis occurs in less than 5% of soft
tissue sarcomas of the extremities, however.136 Epithelioid sarcoma is a notable exception,
with a risk for regional lymph node metastasis as high as 42%.22,162,165,185 Sentinel lymph
node biopsy for epithelioid sarcoma, clear cell sarcoma, angiosarcoma, and rhabdomyosarcoma
is currently being investigated and may play a role in the staging of patients with these
diseases.106,131 Soft tissue sarcomas are generally staged on the basis of grade, size, and the
presence of metastasis. Prognostic factors that have been incorporated into the AJCC staging
system include grade, size, depth, and presence of metastasis.77 Histologic grade is particularly
important in predicting the biologic behavior of the lesion and its risk for metastasis.118 The
tumor should also be staged with the MSTS staging system.55 Operative Treatment Surgery
is the primary form of local treatment for patients with soft tissue sarcoma. Treatment of either
bone or soft tissue sarcoma requires the surgeon to think and plan for two parts of the operation:
resection and reconstruction. At times, a twoteam approach may be best. Resection of the lesion
is of primary importance. Concerns regarding reconstruction must be given secondary
consideration within the context of the particular patient or aggressiveness of the lesion.
Compromise of resection margins out of concern for reconstruction increases the risk for local
recurrence and may increase the risk for metastasis and death.28,124,194 Positive margins in the
treatment of soft tissue sarcomas in the hand are not readily salvaged or compensated for by the
use of external beam radiation and have been found to be predictive of recurrence.28,194 The
goal of surgery is complete removal of the tumor with a surrounding cuff of normal tissue. Wide
excision with 2 to 3 cm of normal tissue surrounding the tumor has been advocated. 118,185
Deliberate sacrifice of major neurovascular structures can often be avoided, provided that there
is meticulous attention to dissection, a margin of normal tissue can be obtained, and
neurovascular structures are not surrounded by tumor.118,172 If negative margins cannot be
achieved with wide excision, amputation should be very strongly considered. Extensive
reconstruction may be required after wide excision of soft tissue sarcomas of the hand. Talbot
and colleagues developed an anatomic, site-specific reconstruction algorithm that can help in
decision making during complex soft tissue reconstruction after resection of soft tissue
sarcomas. Pag 2015