Malignant Tumors of the
Hand and Wrist
Ann-Marie Plate, MD
German Steiner, MD
Martin A. Posner, MD
Dr. Plate is Assistant Professor, New
York University School of Medicine, and
Assistant Attending Physician, Hand
Service, Department of Orthopaedic
Surgery, NYU–Hospital for Joint
Diseases, New York, NY. Dr. Steiner is
Professor of Surgical Pathology, New
York University School of Medicine, and
Chairman, Department of Pathology,
NYU–Hospital for Joint Diseases.
Dr. Posner is Clinical Professor of
Orthopaedics, New York University
School of Medicine, and Chief of Hand
Service, Department of Orthopaedic
Surgery, NYU–Hospital for Joint
Diseases.
None of the following authors or the
departments with which they are
affiliated has received anything of value
from or owns stock in a commercial
company or institution related directly or
indirectly to the subject of this article:
Dr. Plate, Dr. Steiner, and Dr. Posner.
Reprint requests: Dr. Posner, 2 East
88th Street, New York, NY 10128.
J Am Acad Orthop Surg 2006;14:
680-692
Copyright 2006 by the American
Academy of Orthopaedic Surgeons.
680
Abstract
Malignant tumors in the hand and wrist compose a wide variety of
lesions involving skin, soft tissues, and bone. Although these
lesions are found elsewhere in the body, many have unique
characteristics at this anatomic location. Skin tumors predominate;
the most common are squamous cell carcinomas, followed in
frequency by basal cell carcinomas and malignant melanomas.
Other soft-tissue malignancies are less common but may present
more difficult diagnostic problems. They often appear as painless
masses that sometimes have been present for months or even years
and deceptively appear to be benign. A missed or delayed diagnosis
of these tumors can have devastating consequences. Bone
malignancies involve both primary lesions, of which
chondrosarcomas are the most common, and metastatic lesions.
Regardless of cell type, treatment of malignant tumors in the hand
and wrist requires special considerations because of the important
function of these structures. Orthopaedic surgeons should be
familiar with the spectrum of these tumors, the work-up necessary
to arrive at a precise diagnosis, and the treatment that will achieve
the most favorable outcome.
nerves or from actual tumor invasion
Diagnostic Workup
of nerves. Any family or personal his-
M alignant skin tumors present
with an obviously visible le-
sion, whereas tumors in deeper soft
tory of prior malignancies also
should be obtained. The physical ex-
amination should define the size,
tissues typically present with a pal- shape, mobility, consistency, and lo-
pable mass that may be painless. cation of the lesion, as well as any
When pain does occur, it tends to be tenderness or changes in the overly-
progressive in nature, worse at night, ing skin. Neurologic deficits affecting
and generally unrelated to activities. sensibility and/or muscle function
On occasion, there are systemic should be determined, along with
symptoms, such as fever. For bone any deficits in circulation. The pres-
tumors, swelling and discoloration of ence of epitrochlear and, more impor-
the skin often occur over the affected tantly, axillary lymph nodes also
bone. A complete history is impor- should be determined because they
tant and should include when the le- are common metastatic sites for car-
sion was first noted, its rate of cinomas and melanomas. However,
growth, and symptoms such as musculoskeletal sarcomas rarely
numbness that may be neurogenic spread to regional lymph nodes ex-
because of compression of adjacent cept for rare rhabdomyosarcomas, ep-
Journal of the American Academy of Orthopaedic Surgeons

ithelioid sarcomas, clear-cell sarco-
mas, and synovial sarcomas.1
Conventional radiographs provide
important information. In addition
to routine posteroanterior and later-
al radiographic views, we recom-
mend pronation and supination ob-
lique views. For lesions in or
adjacent to the pisiform and hook of
the hamate, a carpal tunnel view is
useful. A more definitive carpal tun-
nel view is obtained using computed
tomography. Other imaging studies
that may aid in the diagnosis and
surgical planning are magnetic reso-
nance imaging (MRI), bone scans
(scintigraphy), and positron emission
tomography.
Biopsy is the final step in the diag-
nostic workup. Generally, the sur-
geon performing the biopsy should
be capable of performing any subse-
quent surgery that is required. For
skin lesions, the biopsy is performed
using a punch, shave, or excision
technique. The shave technique is
most applicable for pedunculated le-
sions and involves an intradermal in-
jection of a local anesthetic that el-
evates the lesion and makes it more
accessible. The lesion is then shaved
off with a scalpel or razor blade.
For soft-tissue and bone tumors,
the biopsy is performed using either
a closed or an open technique. A
closed biopsy with a fine needle or
trephine has the advantage of mini-
mizing tissue contamination; how-
ever, the disadvantage of this tech-
nique is that the tissue sample may
be inadequate for the pathologist to
arrive at a definitive diagnosis or
may not be from a representative
area of the tumor, resulting in misdi-
agnosis. Needle biopsies are general-
ly successful except for very small
tumors.
Most biopsies are performed as
open surgical procedures, adhering
to several important principles. The
operation is performed under pneu-
matic tourniquet control unless
medically contraindicated. Prior to
tourniquet inflation, the arm is not
exsanguinated with an elastic or rub-
Volume 14, Number 12, November 2006
ber wrap because of the potential
risk of forcing malignant cells into
adjacent tumor-free tissues. (Al-
though this possibility has not been
proved to occur, taking this precau-
tion is prudent.) Intravenous region-
al anesthesia (Bier block) is therefore
avoided. Elevating the arm for sever-
al minutes before inflating the tour-
niquet will provide a relatively
bloodless surgical field. The surgical
approach to the lesion should be
planned to permit excision of the bi-
opsy scar and surrounding tissues if
additional surgery is required. Longi-
tudinal incisions are generally pref-
erable to transverse incisions.
There are two types of biopsies:
intralesional or incisional and mar-
ginal or excisional. An intralesional
(incisional) biopsy, as its name sug-
gests, consists of removal of tissue
from within the tumor. A marginal
(excisional) biopsy is through the
pseudocapsule (zone of reactive tis-
sue) that surrounds the tumor and
often consists of excision of the en-
tire lesion. An intralesional biopsy
can be excisional when it is used to
curet a presumably benign lesion in
bone before packing the tumor cav-
ity with a bone graft. Allografts and
commercially available bone substi-
tutes are being used with increasing
frequency instead of autogenous
grafts. When a malignant tumor is
suspected, an incisional biopsy is
preferred to determine the diagnosis
before proceeding with definitive
treatment.
Marginal excision is commonly
referred to as an excisional biopsy
because the tumor is “shelled out”
or excised from the surrounding soft
tissues. Although acceptable for a
benign lesion (eg, a ganglion), such
treatment of a malignant lesion is
unacceptable because tumor cells
usually remain in the pseudocapsule
(reactive zone). Generally, tumors
>3 cm in diameter in the hand are
not marginally excised; rather, they
are treated by wide local excision.
Lipomas are a notable exception,
however, because they rarely under-
Ann-Marie Plate, MD, et al
go malignant transformation. Mar-
ginal excisions for lipomas are ap-
propriate, even though these tumors
frequently exceed 8 cm in diameter,
provided they appear to be clinically
benign. When there is any doubt, an
incisional biopsy should be per-
formed and excision of the tumor de-
ferred until the permanent sections
are reviewed. Frozen sections may
provide important information, but
some pathologists avoid making a
definitive diagnosis based solely on
these biopsy specimens.
Once the tumor is identified as
malignant, a treatment plan is initi-
ated. In most cases, it involves either
a wide excision or a radical resec-
tion. The terms “excision” and “re-
section” are paired with “wide” and
“radical,” respectively, to emphasize
important differences between the
two procedures. A wide excision, of-
ten referred to as an en bloc resec-
tion, is excision of the tumor togeth-
er with its pseudocapsule (reactive
zone) and at least a 2- to 3-cm sur-
rounding margin of normal tissue.
The dissection is entirely within the
involved anatomic compartment; al-
though adjacent muscle tissue and
bone are excised, the excision does
not include the entire length of the
involved muscle or the entire bone.
For tumors outside the hand, a wide
excision often can be performed
without jeopardizing or injuring vi-
tal structures. In the hand, however,
it may be impossible to obtain an ad-
equate tumor-free margin without
sacrificing important nerves, ves-
sels, intrinsic muscles, and tendons.
A radical resection involves remov-
al of the tumor, its pseudocapsule
(reactive zone), and all involved
muscles and/or bone in their entire-
ty as a single block of tissue. In the
hand, a radical resection is often a
digit or ray amputation.
General Surgical
Principles
Soft-tissue sarcomas of the upper ex-
tremity are uncommon. Of approxi-
681
Malignant Tumors of the Hand and Wrist
mately 5,000 soft-tissue sarcomas
reported each year in the United
States, only 15% occur in the upper
extremity and most of these are
proximal to the wrist.1 These figures
are imprecise because no data are
available on the exact number of cas-
es or types of malignancies treated
each year. However, useful data are
provided by the Surveillance, Epide-
miology, and End Results Program
of the National Cancer Institute,
which collects data from tumor reg-
istries in the United States.2 The
overall incidence of upper extremity
sarcomas has remained relatively
constant since these data were first
collected in 1973, but changes have
occurred in some subtypes. The
great majority of patients are Cauca-
sian (83.7%), with males being more
at risk (55.3%) than females. The av-
erage patient age is 54.5 years; how-
ever, age varies depending on the
type of tumor.
When a soft-tissue sarcoma does
appear in the hand and wrist, it often
presents as a painless mass that de-
ceptively appears to be benign. Prior
to biopsy, the mass may be misdiag-
nosed as a ganglion, lipoma, soft-
tissue giant cell tumor, or even an
infection. Regardless of cell type, the
objective of treatment is wide exci-
sion of the tumor. When the tumor
is distal to the metacarpophalangeal
(MCP) joint, the only option is com-
plete or partial amputation. A soft-
tissue or bone sarcoma confined to
the distal segment is often treated by
amputation of that segment through
the next proximal joint. The carti-
lage over the end of the middle pha-
lanx is excised and the bone con-
toured to eliminate prominent
condyles. The digital nerves are cut
back to avoid tender neuromas, and
the wound is closed using dorsal and
volar skin flaps. Skin closure should
be loose; it is preferable to resect
more of the middle phalanx than to
have a tight skin closure that results
in a painful stump. When resection
of the middle phalanx proximal to
the insertion of the flexor digitorum
682
superficialis tendon (midportion of
the bone) is necessary, amputation at
the level of the proximal interpha-
langeal joint is generally performed
because active flexion of the joint is
no longer possible. Tumors involv-
ing the middle phalanx of a finger
usually require amputation through
the MCP joint or a ray resection. Re-
gardless of the level of amputation,
the permanent tissue sections must
be carefully inspected to ensure that
the margins are tumor-free.
When amputation of an index fin-
ger at the level of the MCP joint is
appropriate, a modified ray resection
is preferable to a disarticulation that
retains the metacarpal head. A mod-
ified ray resection eliminates that
bony prominence and, unlike a stan-
dard ray resection that removes the
entire metacarpal, preserves most of
the normal width of the palm. In a
modified ray resection, an oblique
osteotomy is made through the
head-neck area of the second meta-
carpal. This provides a smooth slope
of the web between the thumb and
middle finger that also enhances the
postoperative appearance of the
hand. A similar oblique osteotomy
through the head-neck area of the
fifth metacarpal (but with the angle
of the osteotomy in the opposite di-
rection) is used for a modified ray re-
section of the little finger.
When amputation of a thumb,
middle finger, or ring finger is neces-
sary, the base of the metacarpal of
that digit is preserved to permit sec-
ondary reconstruction. The pre-
ferred reconstruction following a
thumb amputation is pollicization
of the index finger. Following ampu-
tation of a middle or ring finger,
transposition of the adjacent border
ray is recommended to close the
space. Generally, pollicizations are
performed as secondary procedures,
whereas digital ray transpositions
are performed in conjunction with
amputation of a central finger to
close the gap in the palm. With any
amputation, the digital nerves must
be cut back as far proximally as pos-
Journal of the American Academy of Orthopaedic Surgeons
sible to avoid tender neuromas.
Although malignant tumors that
remain within the cortex of a meta-
carpal often can be treated by ray re-
section, treatment is more compli-
cated and extensive for bone tumors
that have broken through the cortex
into the surrounding soft tissues.
The priority of surgery is to obtain a
safe margin of normal tissue. An ag-
gressive malignant tumor of the
thumb metacarpal that has broken
through the cortex requires removal
of the entire first ray, the intrinsic
muscles, and occasionally the sec-
ond metacarpal. In such cases, it
may be possible to safely retain the
index finger distal to the second
metacarpal as a “floating” finger
that can then be pollicized onto an
autograft or allograft that is arthro-
desed to the trapezium.1
Aggressive malignant tumors of a
border finger metacarpal (ie, second
or fifth) require ray resection of the
involved finger and occasionally the
adjacent finger. Aggressive tumors of
a central finger (ie, middle or ring)
may require resection of three rays—
the affected finger and the finger on
either side. Resection of the ulnar
three rays will retain the thumb and
index finger, thus preserving reason-
ably good pinch. With resection of
the second, third, and fourth rays, a
rotational osteotomy of the fifth
metacarpal will improve tip-to-tip
pinch between the remaining little
finger and thumb. In some cases, all
four finger rays must be resected to
achieve a safe margin, leaving only
the thumb. Although prehension is
significantly impaired, a thumb that
is sensate and mobile is still more
functional than a total prosthesis.
The patient can be fitted with a par-
tial hand prosthesis that permits
pinch with the thumb. With any sur-
gery requiring resection of one or
more rays, soft-tissue coverage of the
surgical site can be problematic.
Soft-tissue closure can often be ac-
complished using the skin and sub-
cutaneous tissues of the amputated
finger as a fillet flap.

Figure 1
A, Papular, irregular skin lesion involving the second web space. B, Photomicro-
graph of a well-differentiated squamous cell carcinoma demonstrating malignant
epithelial cells with large nuclei and increased mitotic activity (hematoxylin-
eosin, original magnification ×80).
Although some soft-tissue sarco-
mas may arise in areas where they
are impossible to excise with safe
margins, satisfactory hand function
can sometimes still be maintained.
This is typical of sarcomas in the
palm or on the volar aspect of the
wrist or palm, where the median and
ulnar nerves and flexor tendons are
in close proximity. Although vascu-
lar grafts, tendon transfers, and inter-
position nerve grafts are available for
reconstruction as well as myocuta-
neous and fasciocutaneous flaps, the
surgeon must decide whether these
procedures are likely to restore use-
ful hand function. If not, total ampu-
tation and prosthetic replacement is
indicated.
For sarcomas involving the dorsal
aspect of the wrist, en bloc tumor re-
section and secondary reconstruc-
tion may be feasible. A wide en bloc
excision of the carpal bones, togeth-
er with the overlying skin and exten-
sor tendons, can be performed and
the wrist joint arthrodesed. General-
ly, a bone graft is not required for the
fusion, but when a large (>6 cm)
bone defect exists, a vascularized fib-
ula or vascularized iliac graft should
Volume 14, Number 12, November 2006
be considered. Following wide exci-
sion of the tumor, tendon transfers
are performed, and soft-tissue cover-
age is provided by a vascularized free
flap. Distant pedicle and local flaps
should be avoided because of the risk
of transferring malignant cells. Re-
gardless of tumor location, the mar-
gins of resection must never be com-
promised to preserve function; the
primary goal of treatment is patient
survival.
Treatment of Skin
Tumors
Squamous Cell Carcinoma
Squamous cell carcinoma is the
most common malignant skin tu-
mor of the hand; 11% of all such tu-
mors occur in the hand.3 They arise
from keratinocytes in the epidermis
and are generally induced by ultravi-
olet rays. They are often associated
with clinical signs of solar skin dam-
age, such as actinic keratosis. Squa-
mous cell carcinomas are potential-
ly serious tumors because of their
propensity to invade surrounding
structures and to metastasize, usual-
ly to regional lymph nodes. They
Ann-Marie Plate, MD, et al
range in size from small, slow-
growing, wart-like masses to large,
erythematous, ulcerated lesions (Fig-
ure 1). Tumor thickness correlates
with its biologic activity; lesions
>4 mm thick recur locally, and le-
sions >10 mm thick tend to metasta-
size.4 In some cases, the tumor is in
situ and appears as a small papule, a
condition known as Bowen’s disease.
Squamous cell carcinomas also oc-
cur in previously irradiated skin, in
burn scars (Marjolin’s ulcers), and at
sites of chronic osteomyelitis. Tu-
mors arising in areas of previous in-
jury have a poorer prognosis because
they tend to metastasize early,
whereas tumors arising in areas of
solar damage are less aggressive and
usually require only local excision.
They are excised with a 1-cm,
tumor-free margin. Because squa-
mous cell carcinomas spread by the
lymphatic system, careful examina-
tion for axillary lymph nodes is nec-
essary; node dissection is indicated
when they are palpable.
Squamous cell carcinomas also
can be subungual, a site most com-
monly seen in thumbs and index fin-
gers in elderly men.5 These carcino-
mas tend to be indolent; symptoms
often are present for years before the
patient seeks medical attention. The
typical clinical presentation is a
small, red, slow-growing lesion in
the nail bed. As the tumor enlarges,
it usually becomes painful and is as-
sociated with local swelling and
erythema. Secondary infections that
can obscure the diagnosis are com-
mon. The preferred treatment is am-
putation at the level of the distal in-
terphalangeal joint. An in situ lesion
can be treated by excision of the nail
bed and the dorsal cortex of the dis-
tal phalanx, with coverage using a
free, full-thickness skin graft, there-
by preserving the fingertip.
An important lesion in the differ-
ential diagnosis of squamous cell
carcinoma is a keratoacanthoma.
This benign tumor is seen in elderly
individuals and also has a predilec-
tion for sun-exposed skin on the dor-
683
Malignant Tumors of the Hand and Wrist
Figure 2
Keratoacanthoma. Firm, dome-shaped
nodule with a central depression.
(Case courtesy of Alfred Kopf, MD.)
sal surfaces of their hands. The tu-
mor is typically skin-colored or
pink, with a raised, central, keratin
plug–filled crater (Figure 2). The ini-
tial growth of the tumor for the first
2 to 8 weeks is generally rapid, fol-
lowed by an inactive phase for sever-
al weeks, and then a stage of involu-
tion that also lasts for several weeks.
During the last stage, the keratin
plug is expelled, resulting in a slight-
ly depressed alopecic scar. Some
keratoacanthomas in the hand be-
have in an aggressive manner in that
their initial growth phase does not
plateau and they do not regress.6 Ap-
proximately 10% of lesions thought
to be keratoacanthomas are actually
squamous cell carcinomas.7 There-
fore, all suspicious skin lesions re-
quire biopsy. Keratoacanthomas also
can appear subungually, where they
usually cause erosion of the distal
phalanx (Figure 3).
Basal Cell Carcinomas
Basal cell carcinomas also have a
predilection for sun-exposed areas,
especially the dorsal surfaces of
hands in fair-skinned individuals.
They are classified by gross and his-
tologic morphology. The nodulocys-
tic or noduloulcerative type ac-
counts for approximately 70% of
lesions. Typically, basal cell carcino-
mas are slow-growing tumors that
patients commonly neglect for
years. They generally appear in areas
of skin atrophy as pink-red discolor-
684
Figure 3
Posteroanterior radiograph of a
keratoacanthoma that produced an
erosive, punched-out lesion in the distal
phalanx. This is a benign lesion.
ations. Telangietatic changes fre-
quently develop, followed by ulcer-
ation. When the ulceration is large,
it is called a rodent ulcer (Figure 4).
Although basal cell carcinomas can
invade deeper structures and cause
extensive local destruction, metas-
tases are rare. Most lesions are
<2 mm in diameter and nodular.
They are often treated with curet-
tage and electrodessication, laser
therapy, or cryosurgery. The disad-
vantage of these techniques is that
they do not provide histologic con-
firmation of tumor-free margins.
Surgical excision with a 3- to 4-mm
margin of normal tissue is preferred.
Patients should be followed closely
because new lesions and recurrences
are common. The most difficult bas-
al cell carcinoma to treat is the su-
perficial spreading type because it
contains nests of cells that are sepa-
rate from the original tumor and of-
ten go undetected until new lesions
appear.8
Journal of the American Academy of Orthopaedic Surgeons
Figure 4
Noduloulcerative lesion, or rodent
ulcer. (Case courtesy of David Polsky,
MD, PhD, NYU Department of
Dermatology Photography Archives.)
Melanomas
Malignant melanoma is a com-
monly encountered tumor of in-
creasing incidence, doubling every 8
to 10 years.9 Although malignant
melanomas account for fewer than
5% of all skin malignancies, they ac-
count for 75% of deaths from skin
cancer. Approximately 2% of malig-
nant melanomas occur in the
hand.10
Four distinct varieties of melano-
mas have been identified: superficial
spreading is the most common type,
followed by nodular, lentigo maligna
(also known as melanotic freckle of
Hutchison), and acral lentiginous
melanoma. The first two types are
rare in the hand. Lentigo maligna, the
third variety of melanoma, accounts
for approximately 10% of cases and
is commonly seen on the dorsum of
the hand in elderly patients with ad-
vanced solar degeneration. These le-
sions are frequently ignored and often
become quite large before treatment
is sought. Fortunately, invasive
growth occurs late, and the progno-
sis for lentigo maligna is the best of
all types of melanomas.
Acral lentiginous, the fourth type
of melanoma, occurs on the palmar
surface of hands and in subungual
areas, usually in the thumb (Figure
5). The diagnosis of subungual mel-
anoma is frequently delayed because
they are often misdiagnosed as fun-

Figure 5
Subungual melanoma of a thumb
extending past the nail edge. (Case
courtesy of David Polsky, MD, PhD,
NYU Department of Dermatology
Photography Archives.)
gal infections. They are often amel-
anotic, which also delays the correct
diagnosis. Consequently, subungual
melanomas generally present at a
more advanced stage than do cutane-
ous melanomas; they tend to be
thicker and also are more likely to be
ulcerated (Figure 6). Amputation
through the proximal phalanx of the
thumb and at the level of the proxi-
mal interphalangeal joint of a finger
is the recommended treatment.11
Not all subungual pigmentations
are malignant, especially in African-
American individuals who com-
monly have light brown to black
streaks in the nail bed, known as
melanonychia striata longitudina-
lis. However, a longitudinal streak
>6 mm wide is cause for concern and
warrants biopsy. Similar treatment
is indicated for any dark discolora-
tion in the area of the lunula, com-
monly known as Hutchison’s sign.
Melanomas are graded according
to the extent of skin invasion and
thickness.12 Tumor thickness is
probably the more important prog-
nostic indicator; the thicker the tu-
mor, the greater the likelihood that
regional or distant metastases al-
ready have occurred. Thin melano-
mas (<0.76 mm) have a 98% 5-year
survival rate, compared with a sur-
vival rate of only 45% for thick mel-
anomas (>4.0 mm). The survival rate
Volume 14, Number 12, November 2006
Ann-Marie Plate, MD, et al
Figure 6
A, Ulcerative malignant melanoma of a thumb that began as a subungual lesion.
B, Photomicrograph of malignant melanoma arising from squamous cell epithelium
(arrow), demonstrating aggregates of polygonal cells with large nuclei and
prominent nucleoli (hematoxylin-eosin, original magnification ×100).
for intermediate-thickness melano- mediate thickness, a regional lym-
mas varies considerably, from 60% phadenectomy is indicated.
to slightly more than 90%, because Thin melanoma lesions have a
tumor thickness in this category has low incidence of positive nodes;
a wide range (0.76 to 4.0 mm).13 thus, elective lymph node dissec-
The size of a suitable excision tions are generally not recommend-
margin depends on the thickness of ed. For lesions >4.0 mm in thick-
the tumor. For thin melanomas, a 1- ness, however, the risk is significant
to 2-cm margin is suitable; for thick- that distant metastases already have
er melanomas, the margin should be occurred. In this setting, lymph node
at least 3 cm. A thin melanoma of a dissections may have some limited
digit can be treated by a disartic- value.15 Although sentinel node bi-
ulation; a thick melanoma requires a opsy has been effective in identify-
ray resection.9 ing metastatic spread of skin tu-
Frozen-section examination of mors, especially melanomas, it is
the sentinel lymph node is an effec- unclear whether it is effective for
tive technique for identifying occult other tumors.
metastases. Tracking of lymph
nodes has been facilitated by the ad- Other Skin Malignancies
dition of a radiolabeled isotope to Eccrine sweat gland malignancies
the dye. Approximately 1 to 2 hours usually present as slow-growing,
after the injection, a handheld gam- painless nodules in the palm. These
ma probe is applied to the axilla. The are locally aggressive tumors that
sentinel node is located and the site can metastasize. Wide local excision
marked for incision. For surgery, the is recommended, together with a
node is identified using a sterile lymphadenectomy when positive
gamma probe; it is also visibly nodes are found.3
stained.14 When the sentinel node is Merkel cell carcinoma, also re-
positive and the tumor is of inter- ferred to as trabecular or neuroendo-
685
Malignant Tumors of the Hand and Wrist

Figure 7
Merkel cell tumor appearing as a raised
nodule. These tumors rarely ulcerate.
(Case courtesy of David Polsky, MD,
PhD, NYU Department of Dermatology
Photography Archives.)
crine carcinoma, originally was
thought to be a variant of squamous
cell carcinoma (Figure 7). However,
immunohistochemical markers have
shown that the tumor is of neuroepi-
thelial differentiation. As with other
skin malignancies, they have a predi-
lection for sun-exposed areas. Merkel
cell carcinomas are usually seen in
the elderly. They are aggressive le-
sions that spare the epidermis while
infiltrating deeper structures. Surgi-
cal excision with a 3-cm margin is
recommended, together with pro-
phylactic regional lymph node dis-
section and adjuvant radiation ther-
apy. Local recurrences are common,
as are distant metastases; the prog-
nosis is worse than that for melano-
mas.
Treatment of
Soft-Tissue Tumors
Dermatofibrosarcoma protuberans is
a low-grade malignant tumor that
arises in the dermis. The lesion ap-
pears as a painless nodule that in
time may ulcerate and take on the
appearance of a pyogenic granulo-
ma.16 Dermatofibrosarcoma protu-
berans extends into the subcutane-
ous tissues, where it tends to spread
in a horizontal fashion. Consequent-
ly, the recurrence rate following ex-
cision of these tumors historically
has been high, approximately 50%.
That statistic is decreasing with im-
proved surgical planning, using MRI
to determine the extent of tumor
spread. Treatment is wide excision
of the lesion with a 3-cm margin, in-
cluding the underlying deep fas-
cia.17 Although adjuvant radiation
therapy has been used in patients
with positive margins, the preferred
treatment is excision of additional
tissue.
Epithelioid sarcoma is one of the
more common soft-tissue sarcomas
in the hand.18 Although the overall
incidence of the tumor is low, the
hand is frequently involved. The
typical presentation is a painless
mass on the volar aspects of fingers
or in the palms of young males, be-
tween the ages of 10 and 35 years
(Figure 8). In many cases, it presents
as an ulcerating nodule that is ini-
tially misdiagnosed as an infec-
tion.19 Generally, epithelioid sarco-
mas at surgery deceptively appear to
be innocent. They are solid, gray-
white, and seem to be encapsulated.
However, they are extremely malig-

Figure 8

Epithelioid sarcoma of soft tissue. A, Posteroanterior radiograph of a ring finger demonstrating a soft-tissue mass that had been
present for approximately 1.5 years. The deformity of the phalanx is secondary to the soft-tissue tumor. B, MRI scan of the same
ring finger demonstrating a soft-tissue tumor causing secondary bone deformity. C, Photomicrograph demonstrating a dense
proliferation of malignant epithelioid cells with areas of central necrosis (arrow).

686 Journal of the American Academy of Orthopaedic Surgeons


Figure 9
Synovial sarcoma of the hand. A, Posteroanterior radiograph of a soft-tissue mass
in the second web space. The mass was nontender, and had slowly increased in
size over a period of at least 4 months. B, Photomicrograph demonstrating a
monophasic synovial sarcoma composed of dense aggregates of spindle cells with
mitotic changes (hematoxylin-eosin, original magnification ×60).
nant tumors that spread in a capri-
cious fashion along the lymphatic
system, tendons, and fascial planes.
Treatment must be aggressive; when
wide excision is not feasible, ampu-
tation is necessary. Sentinel node bi-
opsy is warranted even in the ab-
sence of clinically palpable axillary
nodes. Forequarter amputation has
been curative in patients with axil-
lary node involvement.18
Synovial sarcoma is found in the
hand and wrist, but rarely in fingers.
Fewer than 10% of tumors are intra-
articular; they commonly arise in
para-articular tissues such as tendon
sheaths and bursae20 (Figure 9). Clin-
ically, synovial sarcomas are similar
to epithelioid sarcomas in that they
are often initially misdiagnosed as
benign lesions; this especially can
occur when they present as painless,
slow-growing masses that have been
present for many months or even
years, which may occur with gangli-
on cysts. Conventional radiographs
may show focal calcifications with-
in the tumor. Histologically, syn-
ovial sarcomas are either monopha-
sic or biphasic, consisting primarily
of fibrosarcoma-like spindle cells in-
termixed with epithelial cells. Syn-
Volume 14, Number 12, November 2006
ovial sarcomas behave in a manner
similar to that of epithelioid sarco-
mas; local recurrence and distant
spread are common. The surgical op-
tions are also similar to those of ep-
ithelioid sarcomas.
Malignant fibrous histiocytoma is
a primary soft-tissue sarcoma distal
to the elbow in adults that usually
appears between the ages of 50 and
70 years. Most arise in the muscles
and deep fascial tissues in the fore-
arm. They have also been reported in
the hand in the palm of a 3-year-old
child21 and in the finger of a 42-year-
old adult with multifocal tumors in
both the soft tissues and bone.22
Treatment is wide local excision or
amputation, combined with adju-
vant and sometimes neoadjuvant ra-
diation therapy. Chemotherapy also
has been used preoperatively and/or
postoperatively in an attempt to im-
prove survival.
Myxoid chondrosarcoma, also
known as tenosynovial sarcoma, is
associated with tenosynovial struc-
tures such as the flexor tendon
sheaths in digits (Figure 10). This tu-
mor rarely occurs in the hand and is
more likely to arise in deeper tis-
sues, particularly muscles. Because a
Ann-Marie Plate, MD, et al
morphologically identical tumor is
seen in bones, imaging studies such
as radiographs and MRI are neces-
sary to establish its soft-tissue ori-
gin. Myxoid chondrosarcomas usual-
ly occur in men between the ages of
50 and 70 years. The duration of
symptoms varies considerably, rang-
ing from weeks to years. The gross
appearance of the tumor is charac-
teristically gelatinous and multilob-
ular. Its color is largely dependent on
the amount of hemorrhagic tissue, a
common feature that in some cases
is so prominent that the tumor can
be mistaken for a hematoma. The
risk of misdiagnosis is more likely in
patients who report a history of trau-
ma before discovery of the lesion.
Myxoid chondrosarcomas are slow-
growing, but local recurrences and
pulmonary metastases are common,
sometimes years after the initial di-
agnosis. The only treatment option
is surgical; tumors in the hand have
been successfully treated with ray
resections.23
Leiomyosarcomas arise from
smooth muscle cells that in the ex-
tremities are found in skin (eg, arrec-
tores pilorum muscles, myoepithe-
lial cells of sweat glands) and
subcutaneous tissues (eg, vessel
walls). Although skin lesions never
metastasize, subcutaneous lesions
frequently do, usually to the lung by
hematogenous spread, probably be-
cause of their close association with
vascular structures. Although fewer
than 1% of leiomyosarcomas occur
in the hand and wrist, they are im-
portant to consider in the differen-
tial diagnosis of soft-tissue masses
because of their seemingly benign
presentation.24,25 The tumor is usual-
ly painless and mobile beneath the
skin and can easily be confused with
a ganglion. Treatment is wide exci-
sion and radiation therapy. In the ab-
sence of metastases, the survival
rate is high.
Rhabdomyosarcomas have been
classified into several histologic
types; the embryonal type has the
highest incidence, followed by alve-
687
Malignant Tumors of the Hand and Wrist
Figure 10
Myxoid chondrosarcoma. A, Posteroanterior radiograph demonstrating a soft-tissue
mass in an index finger with erosion of the radial aspect of the proximal phalanx.
B, Photomicrograph demonstrating round to ovoid tumor cells of uniform shape,
arranged in cords, and separated by a myxoid stroma with chondroid features
typical of myxoid chondrosarcoma (hematoxylin-eosin, original magnification ×200).
olar and pleomorphic. The alveolar
type is the most common one seen
in the hand and occurs in adolescent
patients (median age, 15 years). The
embryonal type occurs in younger
children (median age, 8 years).26
Rhabdomyosarcomas also can arise
in bone. In children, a rhabdomyosar-
coma in the hand is almost always a
metastatic lesion or represents
spread from a contiguous soft-tissue
lesion. The prognosis for these tu-
mors has improved with the use of
chemotherapy and radiation therapy
combined with surgery. Limb-
sparing surgery is now an option to
amputation.
Lymphangiosarcoma is a tumor
seen in women with chronic lym-
phedema following mastectomy for
breast carcinoma (Stewart-Treves
syndrome).27 It is an aggressive tumor
with a propensity to metastasize.
Kaposi’s sarcoma is a tumor of
688
lymphatic endothelial origin. Before
the appearance of acquired immune
deficiency syndrome (AIDS), it was
most commonly seen in elderly Jew-
ish men of Mediterranean descent.
The tumors in these individuals
tend to be slow-growing; long-term
survival (up to 25 years) is common.
Kaposi’s sarcoma in patients with
AIDS behaves much more aggres-
sively, with early dissemination to
the lymphatic system and viscera.
The sarcoma usually presents as
small, nontender, red-blue, pigment-
ed cutaneous nodules resembling
pyogenic granulomas. Eventually,
the lesions coalesce and form large
ulcerating plaques; frequently, pa-
tients have brawny edema and itch-
ing in the area. The tumor can also
present as an arteriovenous malfor-
mation.28 Surgery is necessary only
for tissue diagnosis because treat-
ment is nonsurgical. In patients
Journal of the American Academy of Orthopaedic Surgeons
without AIDS, radiation therapy is
effective and has a success rate
>90%. Patients with AIDS are treat-
ed with radiation therapy as well as
chemotherapy, and, more recently,
α-interferon. The prognosis is much
worse in these patients; the 2-year
survival is 20%.29
Malignant peripheral nerve
sheath tumor (MPNST) has replaced
such terms as malignant schwanno-
ma, neurosarcoma, and neurofibro-
sarcoma because of uncertainty that
these tumors arise from Schwann
cells, perineural cells, or nerve
sheath fibroblasts. MPNSTs usually
appear between the ages of 30 and
50 years. Approximately 20% occur
in the upper extremities; they com-
pose 3% of all malignant tumors of
the hand.30,31 They tend to involve
large peripheral nerves. Approxi-
mately half of MPNSTs develop in
patients with von Recklinghausen’s
disease and at an earlier age than
in patients without the disease.
MPNSTs are highly malignant tu-
mors and commonly spread along
the nerve. There is no standardized
treatment. Chemotherapy and radi-
cal surgery, either wide excision or
limb amputation when excision is
not feasible, are accepted treat-
ments. Radiation therapy generally
is not effective. Average survival is
3 years from time of diagnosis; the
10-year survival rate is slightly more
than 20%.31 The prognosis is even
worse for patients with von Reck-
linghausen’s disease.32
Treatment of Bone
Tumors
Chondrosarcoma is the most com-
mon malignant bone tumor in the
hand, usually affecting metacarpals
and proximal phalanges. Some tu-
mors represent malignant transfor-
mation of benign enchondromas in
patients with multiple enchon-
dromatosis (Ollier’s disease) or
Maffucci’s syndrome (multiple en-
chondromatosis with soft-tissue an-
giomatosis). A chondrosarcoma aris-
 Ann-Marie Plate, MD, et al

Figure 11

Chondrosarcoma of the index metacarpal. A, Posteroanterior radiograph demonstrating bone expansion with cortical irregularity
and multiple radiolucencies in the distal half of the second metacarpal. B, MRI scan demonstrating expansion and disruption
of the cortex, with tumor extension into the soft tissues. C, Photomicrograph demonstrating a moderately cellular chondro-
sarcoma with large cells lined within lacunae. Several multinucleated tumor cells are evident (hematoxylin-eosin, original
magnification ×120). (Panel A reproduced with permission from Cawte TG, Steiner GC, Beltran J, Dorfman HD:
Chondrosarcoma of the short tubular bones of the hands and feet. Skeletal Radiol 1998;27:625-632.)

ing from a preexisting solitary
enchondroma is exceedingly rare.33
Because enchondromas are the most
common benign cartilage tumors in
the hand, differentiating between be-
nign and malignant lesions is criti-
cally important. There are several
key differences. Chondrosarcomas
tend to be painful and generally ap-
pear in older individuals (ages 40 to
60 years) compared with benign en-
chondromas. A dramatic increase in
size of an enchondroma in a patient
with Ollier’s disease is an ominous
sign. Radiographs and MRI provide
important information; marked cor-
tical expansion and destruction ac-
companied by tumor extension into
the soft tissues usually is indicative
of a malignant lesion (Figure 11). Re-
currence following curettage of a
previously diagnosed enchondroma
also is cause for concern. Chondro-
sarcomas are radioresistant and
chemoresistant; the sole treatment
is surgery. In the hand, a ray resec-
tion is usually required. Fortunately,
chondrosarcomas are generally slow-
growing and have an excellent prog-
nosis. Metastases occur in <10% of
cases based on tumor grade. Chon-
drosarcomas are associated with lo-
cal recurrences following inadequate
intralesional curettages.
Although osteosarcoma is the
most common primary bone tumor
in children and adolescents, fewer
than 40 cases in the hand have been
reported. Generally, patients are old-
er, with a median age of 50 years.
Many lesions of osteosarcoma are
secondary to radiation, Paget’s dis-
ease, or multicentric metastatic dis-
ease. Radiographs show areas of scle-
rosis and periosteal reaction as
well as areas of bone destruction
(Figure 12). The extent of extra-
osseous soft-tissue extension gen-
erally corresponds to the size of the
intramedullary tumor and the extent
of cortical destruction. Some inves-
tigators have suggested that osteo-
genic sarcomas in the hand are less
aggressive than tumors at other sites
and have a better response to treat-
ment that combines surgery with
neoadjuvant and adjuvant chemo-
therapy.34,35 Ray amputation is the
procedure of choice for an osteosar-
coma of a phalanx. An osteosarcoma
of a metacarpal requires more exten-
sive surgery that usually involves re-
moving at least two rays.
Ewing’s sarcoma involves the up-
per extremity in approximately 20%
of cases. Fewer than 30 cases have
been reported in the hand; the young-
est patient was a 5-month-old infant
with a tumor in a distal phalanx.36

Volume 14, Number 12, November 2006 689

Malignant Tumors of the Hand and Wrist
Figure 12
Osteosarcoma. A, Posteroanterior radiograph demonstrating a predominately
radiodense lesion involving the medullary cavity of the proximal phalanx with soft-
tissue extension. B, Photomicrograph demonstrating a densely sclerotic tumor with
malignant osteoid and bone formation (hematoxylin-eosin, original magnification
×90). (Case courtesy of Howard D. Dorfman, MD.)
The classic presentation includes fe-
ver, pain, erythema, and swelling,
which may be present for weeks or
even months before the family seeks
medical attention. The problem is
frequently initially misdiagnosed as
a local soft-tissue infection; an ele-
vated erythrocyte sedimentation
rate, leukocytosis, and anemia con-
tribute to this misdiagnosis. Radio-
graphically, these are generally de-
structive osteolytic lesions that may
appear mottled and resemble osteo-
myelitis. However, in the small tu-
bular bones in the hand, the tumor
often has osteoblastic or mixed fea-
tures37 (Figure 13). The onion-skin
periosteal reaction commonly seen
in long bones rarely occurs in the
hand. MRI is useful in demonstrating
the extent of soft-tissue and in-
tramedullary involvement that may
not be apparent on radiographs, par-
ticularly when the bone lesion has no
cortical expansion. Chemotherapy
690
followed by external beam radiation
therapy or surgery has been used
with about equal success in treating
these tumors in most parts of the
body. In the hand, however, radiation
therapy is associated with serious
complications, such as soft-tissue
contractures, neuropathies, and epi-
physeal plate damage, which will sig-
nificantly damage hand function.38
Probably the best combination treat-
ment of a Ewing’s sarcoma in the
hand is immediate neoadjuvant che-
motherapy followed by wide excision
of the lesion (or even amputation) to
obtain local control of the tumor.
Analysis of the surgical specimen
will determine the effects of the che-
motherapy. When tumor-free mar-
gins are inadequate, adjuvant radia-
tion is administered.
Primitive neuroectodermal tu-
mor (PNET) or neuroepithelioma is
a rare tumor that has all of the mi-
croscopic features of Ewing’s sarco-
Journal of the American Academy of Orthopaedic Surgeons
Figure 13
Neuroepithelioma. Posteroanterior (A)
and oblique (B) radiographs
demonstrating a mixed osteolytic and
radiodense lesion involving the proximal
phalanx with a soft-tissue mass on the
volar surface of the bone. (Case
courtesy of the Bone Pathology Club
of New York.)
ma. It is distinguished from Ewing’s
sarcoma by electron microscopy and
immunohistochemical stains. PNET
has been reported in the hand as con-
genital tumors in two infants, both
of whom were treated with surgery
and postoperative chemothera-
py.39,40 Treatment was successful in
one patient, but the other rapidly
succumbed to disseminated meta-
static disease. PNET has also been
reported in the thumb metacarpal of
a young adult who was cured by neo-
adjuvant chemotherapy, followed by
ray resection and pollicization of the
index finger, and postoperative che-
motherapy.41
Treatment of Metastatic
Tumors
Metastases to the hand are rare and
represent only slightly more than
0.1% of all bone metastases.42 Bron-
chogenic carcinomas are the prima-
ry metastatic malignancy in nearly

Figure 14
Metastatic lesion in the hand. A, Posteroanterior radiographic view of the thumb demonstrating destructive lesion of nearly the
entire distal phalanx in a patient with metastatic carcinoma of the lung. B, Gross specimen demonstrating almost complete
destruction of the distal phalanx by tumor. Note the clear differences between the distal and middle phalanges. (Case courtesy
of Bruce Ragsdale, MD.)
50% of cases. When a metastasis ex-
ists, it most commonly occurs in a
distal phalanx and may be associat-
ed with clubbing of the finger43 (Fig-
ure 14). Breast, colon, kidney, and
prostate are other primary malignan-
cy sites. In some cases, the metasta-
sis is the first manifestation of an oc-
cult malignancy. Clinically, there is
generally local pain, swelling, and
erythema about the finger that can
be confused with an infection. Ra-
diographs usually show a radiolu-
cent lesion that has broken through
the cortex, although metastatic pros-
tate cancer can produce a sclerotic
lesion. The primary objective of
treatment is to relieve pain; this usu-
ally is achieved by amputation. The
overall prognosis is poor and is relat-
ed to the aggressiveness of the pri-
mary malignancy. Most patients
succumb to their disease within sev-
eral months after the metastasis is
diagnosed.
Summary
Orthopaedic surgeons should have a
solid understanding of potential ma-
lignancies that affect the hand and
wrist. Although malignant tumors
usually grow more rapidly than be-
Volume 14, Number 12, November 2006
nign lesions, some malignancies are
slow-growing and may be present for
years before the patient seeks medi-
cal attention. Many of these tumors
are pain-free and nontender. Sur-
geons should never be lulled into
complacency by a painless mass that
has been present for years. Incision-
al biopsy of the lesion is of critical
importance, and it must be per-
formed in a manner that minimizes
contamination of the surrounding
tissues. Once the diagnosis is estab-
lished, in collaboration with the ra-
diologist and pathologist, effective
medical and surgical treatment can
be planned, including referral to a
medical and radiation oncologist.
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