620634
research-articleXXXX
FASXXX10.1177/1938640015620634Foot <italic>&</italic> Ankle SpecialistFoot & Ankle Specialist
58 Foot & Ankle Specialist
〈 Review 〉
Primary Tumors of the Foot
and Ankle
Abstract: Tumors of the foot and
ankle are rarely encountered in the
general patient population. Even
among studies of tumor patients, foot
and ankle neoplasms are uncommon.
Given the weight-bearing demands of
the foot and its relatively small area,
even small masses are likely to be
symptomatic and/or palpable to both
patient and physician. Only 3% of
osseous neoplasms are found in this
region, while 8% of benign soft tissue
tumors and 5% of malignant soft tissue
tumors are localized to the foot and
ankle. Despite the rarity of presentation,
it is important for orthopaedic surgeons
to be familiar with the diagnostic
criteria and therapeutic options for
these patients, as each tumor varies in
its presentation, level of aggressiveness,
and natural history of the disease.
With appropriate diagnostic tests and
treatment, patients can anticipate a
reasonable chance of survival and
preservation of function. In this review
article, the authors survey the current
literature regarding the presentation,
diagnostic workup, and treatment
for the most common benign and
malignant tumors of the foot and ankle.
Levels of Evidence: Level IV:
Literature Review
DOI: 10.1177/1938640015620634. From the Division of Foot and Ankle Surgery, Hospital for Special Surgery, New York, New York (JGK, KAR, NAS), and University of
Pittsburgh School of Medicine, Pittsburgh, Pennsylvania (MVH, CDM). Address correspondence to John G. Kennedy, MD, FRCS (Orth), Division of Foot and Ankle Surgery,
Hospital for Special Surgery, 523 East 72nd Street, Suite 507, New York, NY 10021; e-mail: kennedyj@hss.edu.
For reprints and permissions queries, please visit SAGE’s Web site at http://www.sagepub.com/journalsPermissions.nav.
Copyright © 2015 The Author(s)
Keywords: foot; ankle; primary
tumor; soft tissue neoplasm; bone
neoplasm
T
umors of the foot and ankle are
rarely encountered in the general
patient population. Even among
studies of tumor patients, foot and ankle
neoplasms are uncommon. Only 3% of
osseous neoplasms are found in this
region, while 8% of benign soft tissue
tumors and 5% of malignant soft tissue
tumors are localized to the foot and
ankle.1-3 In a review of
“
2660 tumors, only 153
(5.75%) occurred in the
foot and ankle, 60.8% of Although most soft tissue and
which were benign.4 The
anatomy of the distal osseous foot tumors are benign, it is
lower extremity is
complex; consequently,
prudent to exercise a “malignant until
the presentation of proven otherwise” approach.”
tumors in this region may
be difficult to interpret
and easy to misdiagnose.
Despite the rarity of presentation, it is
important for the orthopaedist to be
familiar with the diagnostic criteria and
therapeutic options for these diseases.
A patient who presents with a mass in
the foot or ankle is a diagnostic challenge
for any physician. This review of primary
Feb 2016
John G. Kennedy, MD, FRCS (Orth),
Keir A. Ross, BS, Niall A. Smyth, MD,
MaCalus V. Hogan, MD,
and Christopher D. Murawski, BS
tumors presenting in the foot and ankle
identifies the most common lesions the
orthopaedist may encounter in clinical
practice. Based on our review, we
recommend a standard approach to
diagnosing a foot and ankle mass,
beginning with a thorough history and
physical examination and supplemented
by diagnostic imaging as guided by the
clinical impression. In certain cases,
pathology can be used to confirm the
diagnosis.
Given the weightbearing demands of
the foot and its relatively small area,
even small masses are likely to be
symptomatic and/or palpable to both
patient and physician. Although most
soft tissue and osseous foot tumors are
benign, it is prudent to exercise a
“malignant until proven otherwise”
approach. Thus, with appropriate
vol. 9 / no. 1
Figure 1.
Subungual exostosis is shown on (A) lateral radiograph of the left forefoot
(arrowheads) arising from the lateral aspect of the first distal phalanx nail bed. The
same lesion is shown on (B) sagittal magnetic resonance imaging (MRI) (fast spin
echo [FSE] TR 3317/TE 27) and is deforming the nail (arrow).
diagnostic tests driven by clinical
findings and treatment as described in
this review article, patients can anticipate
a reasonable chance of survival and
preservation of function.
This extensive review of the most
common primary tumors presenting in
the foot and ankle is necessary in order
to familiarize orthopaedic surgeons with
this group of rare, but important
diseases. In this article, we survey the
current literature regarding the
presentation, diagnostic workup, and
treatment for the most common benign
and malignant tumors of the foot and
ankle, occurring in both the soft tissue
and in bone.
Benign Tumors
Subungual Exostosis
This benign lesion usually presents in
the young adult or adolescent who has
suffered past trauma to a distal phalanx,
although it has been reported in patients
who recently underwent nail removal.5-7
The great toe is the digit most commonly
involved, and classically presents with
ambulatory pain or difficulty wearing
shoes. On examination, an erythematous
or flesh-colored bony growth is palpated
on the dorsum of the distal phalanx, and
may cause onycholysis of the overlying
nail. The mass begins as a
fibrocartilaginous proliferation
underneath the nail bed, which
undergoes endochondral ossification to
form a core of trabeculated bone. The
radiographic appearance of a well-
defined bony growth extending away
from the distal phalanx confirms the
diagnosis (Figure 1A and B).8,9
T1-weighted magnetic resonance imaging
(MRI) can demonstrate an area of high
signal intensity encircled by low signal
intensity fibrocartilage.10 Invasion of the
nail bed usually requires removal of the
nail with a direct approach to the tumor.
Less invasive tumors that spare the nail
bed can be approached via a simple fish
mouth–type incision. Once the
fibrocartilaginous cap is completely
excised, risk of recurrence is
minimized.10,11 The recurrence rate after
complete excision is up to 12.5%.7
Chondroblastoma
Chondroblastoma is a rare, usually
benign, but locally invasive, cartilaginous
neoplasm that often occurs in the long
bones. However, it may occasionally
present in the foot and ankle, and
malignant cases have been reported.12 A
Foot & Ankle Specialist 59
review of 322 chondroblastomas
determined that only 42 tumors (13%)
were found in the foot and ankle
region.13 The most common locations in
the foot are the posterior subchondral
areas of the talus and calcaneus. The
typical presentation is a young adult or
adolescent. Symptoms gradually increase
in intensity, and may culminate in a
pathologic fracture. On examination,
local swelling and tenderness are typical,
but soft tissue mass, skin changes, or
palpable lymph nodes are not often
present. On conventional radiograph,
chondroblastoma tends to be a well-
circumscribed radiolucent lesion without
matrix formation. Minimal septations may
be appreciated. Scalloping or expansion
of the cortex is seen in up to 69% of
lesions, and cortices may be disrupted if
a pathologic fracture has occurred.
Subchondral fractures have been
reported in up to 45% of patients.13 Some
authors have described an association
with aneurysmal bone cyst.14-16 Treatment
most often involves curettage and
augmentation with bone grafting or
cementing, as needed.17 Recurrence
occurs locally in approximately 18% of
cases after curettage and bone
grafting18,19 but studies have indicated
that cryosurgery as a local adjuvant
therapy may decrease recurrence rates to
10% and improve functional results.20,21
Osteoblastoma
This bone-forming tumor is considered
benign, but invariably progressive and
can be locally aggressive.22 Up to 12.5%
of osteoblastomas occur in the foot and
ankle. These tumors tend to involve the
calcaneus and talus, but any bone may
be affected including the distal tibia/
fibula, midfoot, or forefoot bones.
Patients are typically young adults, with
a male predominance, only a quarter of
whom will have periarticular swelling on
physical examination, with history of
trauma in some cases. The lesion is well
visualized on plain radiography or
computed tomography (CT) (Figure 2).
Subperiosteal localization (41%) is
commonly seen, and is usually found in
association with a soft tissue mass and
60 Foot & Ankle Specialist
Figure 2.
Osteoblastoma is shown on an axial
computed tomography (CT) section
of the right lower leg of a patient
with multiple osteoblastomas. There
are several lytic lesions in the distal
tibia just proximal to the malleolus
(arrows).
variable matrix production. Medullary
lesions (54%) may take on a bubbly,
cystic appearance. Cortical lesions are
the least common (5%). Diffuse or
regional osteopenia may be observed,
and patients may present with a
pathologic fracture. Treatment for benign
tumors consists of intralesional curettage
with or without bone graft.23 Malignant
transformation, although rare, has been
reported. Treatment for aggressive or
malignant tumors usually requires en
bloc resection, with bone graft if
necessary. Although some series have
reported no local recurrences, patients
should be monitored for recurring
lesions.22,24
Osteoid Osteoma
This is a benign, bone-forming tumor
usually found in adolescents and young
adults. Pain is characteristically relieved
by nonsteroidal anti-inflammatory
drugs.25-27 Physical examination may
demonstrate swelling, erythema, and
tenderness to palpation. Reduced range
of motion may be observed, especially if
the osteoid osteoma occurs in an intra-
articular location.28 In the foot and
ankle, authors report a high incidence of
Figure 3.
Enchondroma is shown on sagittal
magnetic resonance imaging
(MRI) (fast spin echo [FSE], TR
4217/TE 20) of the right forefoot
demonstrating an expansile
intraosseous lesion (arrowheads) at
the proximal aspect of the second
toe. The lobulated heterogeneous
appearance is typical of a chondral
tumor.
lesions in the talus and subtalar joint,
but calcaneal, navicular, metatarsal, and
phalangeal tumors occur as well.26,27
Radiographs classically reveal a rim of
cortical sclerosis surrounding a
radiolucent nidus, but in some cases
plain films may not show the lesion. In
these situations, a technetium bone scan
may be helpful in showing an intense
“hot spot” secondary to increased
vascularity. A T2-weighted MRI can
demonstrate a focal area of abnormal
signal intensity. In one study, CT
identified the nidus in 89% of lesions
and MRI identified high-grade edema in
bone and soft tissues in all cases.29
Treatment has historically been
curettage, but good results with
minimally invasive arthroscopic
techniques have been reported.30
Radiofrequency ablation, when possible,
is the recommended treatment. Primary
clinical success rates are between 89.6%
and 94% with recurrence rates between
0% and 10%.31,32
Feb 2016
Enchondroma
This benign cartilaginous tumor is not a
true neoplasm, but rather results from
islands of normal cartilage aberrantly
incorporated into the medullary cavity of
the involved bone without undergoing
the proper sequence of endochondral
ossification.33 Most patients are young
adults and may present with associated
fracture, and in rare cases, transformation
to malignant chondrosarcoma may
occur.34 Regarding the foot and ankle,
phalanges are the most commonly
involved (77% of lesions in one series).33
Plain radiographs show a radiolucent
area in the affected bone. An associated
soft tissue mass, cortical involvement, or
endosteal scalloping can be appreciated,
but may be indicative of a malignant
process. MRI may help in further defining
the extent of the lesion (Figure 3).
Individuals found to have multiple
enchondromas (Ollier’s disease) may be
at increased risk of malignant
degeneration; secondary
chondrosarcoma was reported in 4.1% of
patients with Ollier’s disease in one
series.34 The combination of
enchondromatosis and hemangiomatosis,
or Maffucci’s syndrome, is associated
with an increased risk of malignancy. For
this reason, physical examination should
include inspection for cutaneous
hemangioma.18 Treatment should consist
of curettage, with subsequent bone
grafting. Some authors report good
results without augmentation or with
plaster of Paris or hematoma filling of
the defect, instead of bone grafting.35-37
Unicameral Bone Cyst
This benign, membrane-lined serous or
sanguineous cyst is rarely described in the
foot and ankle but there are several reports
of patients with lesions of the calcaneus
presenting with insidious onset of heel
pain with limp.38,39 Palpation may elicit
tenderness over the affected area. Plain
radiographs show a lytic lesion in the
affected bone, and CT scan may help to
precisely delineate the margins of the cyst
and the presence of intracystic bony
pillars. MRI is also a useful study for
vol. 9 / no. 1
Figure 4.
Unicameral bone cyst is shown on
(A) sagittal magnetic resonance
imaging (MRI) (fast spin echo
[FSE], TR 4000/TE 32) of the right
calcaneus (arrow). (B) MRI of the
same lesion with inversion recovery
(TR 4717/TE 20/TI 150) is also
provided. Note that the lesion does
not lose signal with fat-suppression,
in contrast to lipoma (see Figure 5).
evaluating the lesion (Figure 4A and B).39
Surgical treatment requires evacuation of
the fluid-filled cavity and removal of the
lining membrane to prevent recurrence.
Larger cysts may require bone grafts.
Recently, minimally invasive arthroscopic
techniques have been described with good
results.38 According to a 175-patient series,
complete cyst resection is paramount.
Incomplete resection increases the risk of
recurrence and recorded recurrence rates
are up to 30%.40 Fluid samples should be
sent for culture evaluation as some cysts
may become infected.38,39
Intraosseous Lipoma
Intraosseous lipoma tends to occur
about the ankle, especially in the
calcaneus,41 and may also lead to
confusion of the diagnosis with plantar
fasciitis. Physical examination reveals
local tenderness to palpation but with
good range of motion about the joint.
Plain radiographs demonstrate a well-
demarcated lytic area in the affected
bone with a surrounding rim of sclerosis.
Intralesional septations may be evident.
MRI may be helpful in distinguishing
lipoma from other causes of lytic lesions;
an increased signal intensity on
T1-weighted imaging with a decreased
intensity on T2-weighted imaging is
consistent with a lipomatous lesion
(Figure 5A and B).41 Surgical treatment
with curettage and bone grafting is
indicated for large or symptomatic
lesions and one series demonstrated 0%
recurrence and graft consolidation at 5
months postoperatively.42
Epidermal Inclusion Cyst
This lesion does not arise from a
neoplastic transformation within bone,
but rather may result from the traumatic
implantation of epidermal tissue into the
underlying soft tissue or bone.43 It has,
however, been reported without history
of trauma.44 This epidermal tissue may
continue to grow in its new location,
causing local destruction. Often there is a
history of local trauma or surgery.
Physical examination demonstrates local
tenderness to palpation. Plain films or
MRI demonstrate an expansile,
destructive lytic lesion of the involved
bone.43,44 Curettage is typically curative.
Nonossifying Fibroma
This benign tumor is often described
in children and adolescents.45-47 Plain
radiographs reveal a well-circumscribed
lytic lesion with osteosclerotic margins
(Figure 6). Typically, curettage and bone
grafting is performed in larger lesions
(>50% tranverse cortical diameter) to
reduce the incidence of pathologic
fracture. One retrospective study of 22
cases of nonossifying fibroma meeting
the size criteria for operative
intervention reported only 9 pathologic
fractures (41%), indicating that not all
large lesions necessarily need
Foot & Ankle Specialist 61
Figure 5.
Intraosseous lipoma is shown on
(A) sagittal magnetic resonance
imaging (MRI) (fast spin echo [FSE],
TR 4583/TE 26) of the left ankle
for evaluation of a calcaneal lesion
(black arrowheads). (B) Sagittal MRI
of the same lesion with inversion
recovery (TR 4167/TE 15/TI 150)
is provided. Note the loss of signal
from the lesion in the fat-suppressed
image (white arrowheads). The
finding of a well-defined, high-
signal, intraosseous mass (A) that is
suppressed with fat saturation (B),
indicates it is a lipoma.
treatment.46,48 The natural history of
these lesions is to remit spontaneously;
in the same study, all 13 subjects who
did not sustain fractures were
successfully treated with observation
alone. Adamantinoma accounts for less
than 1% of primary bone tumors but
most commonly occurs in the tibia,
fibula, radius, and ulna. These malignant
tumors may mimic fibrous dysplasia and
other bone tumors and must be
considered on differential diagnosis.49
Fibrous dysplasia is a developmental
bone abnormality that typically presents
as an isolated finding but can also be a
component of McCune-Albright
62 Foot & Ankle Specialist
Figure 6.
Anteroposterior radiograph view
of the left ankle demonstrates a
heterogeneous, bubbly mass (arrow)
in the lateral cortex of the distal
tibial metaphysis. There is mild
expansion of the bone. The finding
is typical for a nonossifying fibroma.
syndrome. These lesions should also be
considered on diagnosis. The deformity
is the result of a genetic mutation that
affects the alpha subunit of a G-protein
receptor. Lesions appear as a
radiolucent area with no visible
trabecular matrix and a “ground glass”
pattern on radiograph and should be
carefully examined histologically to rule
out differential diagnoses and
malignancy.50
Giant Cell Tumor of Bone
Giant cell tumors (GCTs) are
reportedly the most common foot and
ankle tumor, primarily found in the
distal tibia and fibula, talus, calcaneus,
and cuneiforms.4,51,52 Seen on plain
radiographs or CT, GCTs demonstrate an
expansile, metaepiphyseal lytic lesion
with or without evidence of cortical
erosion or extension into the joint
space.51-53 GCTs are seen predominantly
Figure 7.
Axial magnetic resonance imaging
(MRI) (fast spin echo [FSE], TR
5067/TE 17) of the right ankle
demonstrates a giant cell tumor
(GCT) of bone with a superimposed
aneurysmal bone cyst (ABC) at the
level of the distal tibial. The lesion is
seen extending focally to the lateral
articular surface. The area of low
signal indicates the GCT component
of the mass (black arrow). The area
of high signal (black arrowheads)
contains a fluid level (white arrow),
reflecting the ABC component.
in adults once the epiphyseal plate has
fused. T1- or T2-weighted MRI is most
useful in demonstrating a low-intensity
expansile lesion.54 Interestingly,
coincident aneurysmal bone cyst may
occur in association with GCT of bone
(Figure 7).53 In 1 series of 12 cases, 3
tumors were associated with a soft tissue
mass, and three developed a pathologic
fracture.52 These tumors are considered
benign, but can be locally aggressive
and tend to recur. In the aforementioned
12-patient series, 4 cases treated with
primary excision remained disease-free,
but 3 out of 8 patients treated with
intralesional curettage had local
recurrences. A recent case series of 20
patients indicated complete local
excision may help minimize recurrence,
though the recurrence rate in this series
was still 20%.54
Feb 2016
Ganglion Cyst
Although these cystic soft tissue masses
are benign lesions, local compression of
nerves or mechanical interference with
the associated joint can make ganglia
painful and irritating.55-57 For example,
patients with lesions associated with the
tarsal tunnel and the plantar aspect of
the first toe have demonstrated poorer
functional outcomes after excision than
patients with lesions in other anatomic
locations.58 These lesions may develop
after direct or indirect trauma and a soft
mass may be palpable on physical
examination.59 Plain radiographs may not
show the cyst, but T2-weighted MRI
typically demonstrates a cystic lesion of
bright signal intensity, which may have
loculations. Therapy involves
corticosteroid injections, aspiration of the
mucinous fluid within the cyst, or simple
excision of the cyst, but several authors
report multiple recurrences.56 A newer
approach involves injection of the
ganglion with OK-432 (Picibanil), a
lyophilized incubation mixture of group
A Streptococcus pyogenes of human
origin, which has demonstrated final
resolution in all 18 patients in 1
series.57,58,60 Tetracycline injections after
cyst aspiration were also effective in 16
patients, with 2 cases of recurrence.61
Peripheral Nerve Tumors
Schwannoma or neurilemmoma is a
benign, encapsulated tumor that arises
from the Schwann cells of peripheral
nerves. The lesions typically present in
isolation but can also be associated with
neurofibroma.62 The posterior tibial
nerve is the most common peripheral
nerve affected by schwannoma in the
foot and ankle.63 Peripheral nerve tumors
are often undiagnosed until they are
notable on inspection, palpable, cause
pain due to impingement of peripheral
nerves or surrounding structures, or
cause pain with shoe wear. As a result,
these tumors are often misdiagnosed as
entrapment neuropathy. Identifiable
imaging characteristics include fusiform
shape, identification of an entering and
exiting nerve, low attenuation at CT, and
associated muscle atrophy. A true
vol. 9 / no. 1
capsule encompasses the tumor and the
affected nerve.64 Malignant tumors can
be distinguished based on radiographic
signal heterogeneity. Treatment should
consist of surgical excision. The nerve
and its function may be spared as
recurrence and malignant transformation
are rare.3
Interdigital neuromata, or Morton’s
neuromata, are nonneoplastic nervous
tissue swellings, which typically occur at
the level of the metatarsal heads, most
commonly at the third intermetatarsal
space.65 They are characterized by neural
degeneration, vascular hyalinization and
peripheral fibrosis. Several theories have
been advanced as to their etiology such
as repetitive trauma and mechanical
compression by the adjacent transverse
intermetatarsal ligament just dorsal to the
nerve. The lesion is typically diagnosed
clinically but is best confirmed by MRI or
ultrasound if the pain or presentation is
atypical or when preoperative planning
is being performed. Conservative
modalities are usually attempted initially
such as combination of shoe
modifications, pads, orthoses, physical
therapy, anti-inflammatory medications,
and alcohol65 and corticosteroid
injections.10 When symptoms continue
despite conservative therapy, operative
treatment is warranted. Surgical excision
is the definitive mode of treatment but
carries with it a high rate of recurrence.
One report by Nashi et al reported 80%
of patients undergoing excision through
a dorsal incision and 65% of patients
undergoing excision through a plantar
excision reported a greater than 50%
decrease in pain over a 3.1-year mean
follow-up period.66 Of late, a minimally
invasive neuroma decompression
procedure has been proposed which
demonstrates promising results.67 In this
regard, Zelent et al67 reported the results
of 17 minimally invasive decompression
in 14 patients, of which 11 reported
alleviation of symptoms, 2 had
recurrence of symptoms and the final
had a further injury, negating further
follow-up.
Neurofibroma is a benign soft-tissue
tumor that arises from the nerve sheath
and may occur as a solitary lesion68,69 or
in association with multiple
neurofibromas as seen in von
Recklinghausen disease. Concerning the
foot and ankle, these lesions are most
commonly located in the tibia and along
the flexor surfaces of the foot.70 Large,
invasive plexiform neurofibromas have
been described in the foot and ankle,
and may cause significant pain and
ambulatory dysfunction secondary to
invasion of adjacent tendons and
neurovascular structures. A palpable
mass is usually present on examination.
MRI is the preferred method of imaging
for these tumors.71 T1-weighted images
show an often nodular-appearing mass
that is hypointense with respect to
adjacent musculature. T2-weighted
imaging shows areas of high and low
signal intensity, corresponding with
myxoid areas and compressed nerves,
respectively. A “target sign” may be seen,
in which a high-intensity area surrounds
a relatively hypointense center. Surgical
excision can be difficult, depending on
the degree of involvement of adjacent
tendons and neurovasculature, significant
reconstruction may be necessary for
larger tumors. Subtotal and total
resection without functional destruction
is often possible in superficial cases.48
Recurrence of this tumor is common.72
Pigmented Villonodular Synovitis
This condition is an inflammatory
proliferation of the synovial tissue, which
can occur either as a localized, nodular
form or as a more diffuse disease.73,74
Multiple case series report predominance
in women, over a wide age range, and
no association with a history of trauma.
The foot and ankle is the third most
commonly involved location after the
knee and hip.74 Up to 20% of pigmented
villonodular synovitis occurs in the foot
and ankle. MRI is the preferred imaging
modality, with T1- and T2-weighted
images both displaying a lobular mass
with areas of low signal intensity
consistent with the high hemosiderin
content of the lesion.71 These lesions are
typically monoarticular, but can become
very destructive and affect more than
one joint in the foot and ankle.74 PVNS
Foot & Ankle Specialist 63
may also spread into synovial fluid,
eventually affecting tendon sheaths as
well as spreading through the extremities
both proximally and distally. Joint
erosion may be appreciated in more
extensive disease, leaving ankle
arthrodesis as the sole treatment option.
Localized disease is adequately treated
with wide surgical excision of the
synovium. However, in diffuse disease, it
can be difficult to remove all of the
affected synovial tissue, and this can
contribute to an increased rate of
recurrence. Despite surgical intervention,
recurrence rates of up to 45% have been
reported. Adjuvant radiotherapy has
been reported with variable success.75
Giant Cell Tumor of Tendon Sheath
This is an inflammatory cellular
proliferation that arises from peripheral
tendons or the periarticular soft
tissue.76,77 The foot and ankle is the
second most common location after the
hand. Specifically regarding the lower
extremity, small joints of the foot are
most frequently affected. There may be a
history of local trauma. Plain films show
a soft tissue mass and may demonstrate
calcifications, or cortical erosion of
underlying bone. Ultrasonography has
also been reported as a useful initial
imaging study, and may help
characterized the size, vascularity, and
degree of local infiltration of the lesion.78
T1- and T2-weighted MRI
characteristically show low-intensity
signal consistent with hemosiderin
deposition. Enhancement with
gadolinium is usually demonstrated.
Multiple authors report series in which
all patients received curative resection;
however, these tumors may recur at a
rate of up to 25% with inadequate local
excision.52,76,77
Hemangioma
This tumor consists of a benign
proliferation of vascular channels within
the soft tissues, most often occurring in
the lower extremities. These lesions can
increase in size with activity as they
become engorged with blood, and may
bleed profusely if protrusion through the
64 Foot & Ankle Specialist
Figure 8.
Sagittal magnetic resonance
imaging (MRI) (fast spin echo [FSE],
TR 4000/TE 20) of the forefoot
demonstrates a lobulated soft
tissue mass (arrowheads) extending
between the 2nd and 3rd metatarsal
shafts, involving the interosseous
muscles, with focal extension into
the distal dorsal subcutaneous soft
tissues. The morphology is typical of
a soft tissue hemangioma.
skin occurs. Intramuscular hemangiomas
may lack some of these classic features
and can be particularly difficult to
diagnose clinically.79 Rarely, more serious
complications occur; one case report
describes a patient whose initial
presentation was a lower extremity
compartment syndrome, secondary to a
thrombosed hemangioma.80 MRI is the
most definitive imaging modality, which
demonstrates increased signal on both
T1- and T2-weighted images (Figure 8).80
Ultrasound is also useful for visualizing
these lesions.81 Surgical resection is the
definitive treatment; however, in the foot
and ankle this made be difficult due to
the intimate association of the tumor
with adjacent neurovasculature and other
structures. For this reason, several
authors propose injection with sclerosing
agents such as ethanolamine oleate to
shrink the tumor, which has been
reported with good results.79
Plantar Fibromatosis
This condition, also known as
Ledderhose disease, is a nodular lesion
that may be associated with a history of
Figure 9.
Sagittal magnetic resonance
imaging (MRI; fast spin echo) of
the right forefoot demonstrates a
superficial plantar mass (arrow)
contiguous with the plantar fascia,
reflecting plantar fibromatosis.
prior trauma to the underside of the foot.
The patient may have concurrent palmar
fibromatosis (Dupuytren’s contracture),
Peyronie’s disease, or a family history
may be positive for plantar fibromatosis.
MRI demonstrates one or more poorly
defined nodules arising from the plantar
aponeurosis, and signal intensity may
vary depending on the degree of
cellularity82 (Figure 9). Initial therapy
includes orthoses and corticosteroid
injections, but highly symptomatic and
recalcitrant lesions may require surgical
excision. Unfortunately, multiple series
report recurrence rates as high as 84% to
90%, which may be due to the difficulty
of achieving clear surgical margins.83,84 A
series of 27 patients reported 25%
recurrence after fasciectomy and 100%
recurrence after local resection.85
However, adjuvant radiotherapy has
been used with promising results in
treating recurrent disease.84
Glomus Tumor
Glomus tumors are benign neoplasms
that develop from the glomus body.86
These tumors are rare and thought to
represent less than 1.5% of all benign
soft tissue neoplasms of the extremities.
Subungual lesions may present with a
bluish hue beneath the nail bed and the
lesion may lead to abnormal nail growth.
The Love test involves the use of an
instrument to elicit pinpoint tenderness,87
Feb 2016
Hildreth’s sign describes relief of pain
following placement of a tourniquet
proximally on the extremity, which
relates to the vascular nature of glomus
tumors.10 Plain radiograph images of
affected extremity are usually
unremarkable while on MRI, these
lesions appear as dark, well-delineated
masses on T2-weighted images.88 MRI
may also help diagnose lesions earlier as
delay in diagnosis is common with a
reported range from 4 months to 1 year
from onset of symptoms.89,90 Surgical
excision is the definitive treatment.91,92
Recurrence is rare and suggests
incomplete excision. There are no cases
reported of an invasive appearance or
metastasis. Grossly, the lesion is well
encapsulated and of a gray-pink color.
The presence of small concentrations of
nerve fibers is thought to be responsible
for the extreme pain, which can be
associated with these lesions.91
Malignant Tumors
Chondrosarcoma
These malignant cartilaginous
neoplasms typically present in the
middle-aged male, with possible history
of local trauma. The tarsal bones and
short tubular bones of the feet may be
involved.93 The radiographic findings are
unequivocally malignant, featuring
endosteal erosion, cortical destruction,
indistinct margins, matrix calcification,
and an associated soft tissue mass in
most cases. Joint extension and
pathologic fractures have also been
reported.94 Distinguishing from
endochondroma may be difficult and
concern for malignant change is
indicated for tumors exceeding 5 cm2.27,94
A heterogenous, multilobulated
appearance on MRI is also helpful in
making the diagnosis. Surgical treatment
varies depending on the site of the
tumor. Ray resection is usually adequate
for disease involving the short tubular
bones, whereas calcaneal and talar
tumors likely require below-knee
amputation. Inappropriately limited
surgical treatment risks ignoring the
potential for metastatic disease; in 1
series of 11 patients, 3 died from
vol. 9 / no. 1
subsequent pulmonary or brain
metastases.93 Unfortunately, traditional
radiotherapy or chemotherapy is not
effective for many of these lesions.94 Use
of cryotherap as a local adjuvant to
resection may have a role in decreasing
recurrence and improving postoperative
function.95
Melanoma
This malignant melanocytic neoplasm
is typically seen in the white, middle-
aged patient, with a history of blistering
sunburns. However, melanoma has also
been reported in darker skinned
individuals, and acral lentiginous
melanoma is of particular consideration
in this patient demographic. A recent
retrospective analysis of 38 patients with
histopathologically confirmed foot
melanoma, acral lentiginous was the
most common subtype (46%).14 Early
detection and complete local excision
with wide margins are emphasized;
however, Mohs excision, toe amputation,
or ray amputation may be preferential
depending on the location and
characteristics of the individual
lesion.14,96,97 Patients with poor
prognostic factors (depth greater than 1
mm, ulceration, Clark level IV) may
benefit from sentinel lymph node biopsy
to rule out metastatic disease.14
Clear Cell Sarcoma
This rare malignancy is found most
commonly in the feet of young adults,
with pain as an associated feature in 8 of
17 patients in one retrospective study.95
In direct contrast to the aggressive nature
of the tumor, MRI demonstrates a lesion
that looks relatively benign.82 A well-
defined, strongly enhancing mass of
slightly higher signal intensity than
muscle is the typical finding on
T1-weighted images. The natural history
of this lesion is typically poor.98 In a
retrospective analysis of 75 tumors, a
total 52 patients developed metastatic
disease, even after treatment.99 The
overall survival was 47% at 5 years and
36% at 10 years. Tumor size greater than
4 to 5 cm is a poor prognostic factor.99,100
Recognizing the aggressive behavior of
Foot & Ankle Specialist 65
these tumors and the propensity for
metastasis, early definitive therapy is Figure 10.
paramount. Surgical excision of the Sagittal magnetic resonance
primary is the treatment of choice, but imaging (MRI) (fast spin echo [FSE],
some patients may require amputation or TR 3800/TE 32) of the right forefoot
disarticulation. Several combinations of is shown (A) demonstrating a large,
neoadjuvant or adjuvant chemotherapy high-signal soft tissue mass (“M”),
were used in one series, including representing synovial sarcoma. It
doxorubicin, ifosfamide, cisplatin, is seen invading the overlying bone
dacarbazine, cyclophosphamide, (arrowhead). MRI with inversion
vincristine, bleomycin, interferon-α, and recovery (TR 5117/TE 20) of the
caffeine in various combinations, which
same lesion (B) seen in (A) is
significantly improved 5-year survival in provided.
localized disease.99 When metastatic
cases were also analyzed, 5-year survival
was not significantly improved by
chemotherapy or radiotherapy. Only
tumor size remained a prognostic factor
on multivariate analysis. Chemotherapy
may remain important in treating primary
tumors greater than 4 to 5 cm, which are
more likely to have micrometastases
present at diagnosis. However, a
52-patient series indicated that these
tumors may be chemoresistant in up to
71.15% of cases. The benefit of
neoadjuvant radiotherapy is less clear.100
Synovial Sarcoma
This malignant soft tissue neoplasm is
a highly aggressive disease, and in the
foot and ankle, may commonly be
mistaken for a more benign
process.101-103 The lower extremity is the
most common location, with the foot
and ankle accounting for 3.08% and
2.31% of lower extremity synovial a mass of heterogeneous signal intensity
sarcomas, respectively.80,104 There may that may include a halo of increased
be a history of local trauma. The signal, indicating reactive edema (Figure
appearance of the tumor can occur 10A and B). Staging is particularly
insidiously (range of 1-48 months in important with these lesions; a
one series), which may confuse the retrospective analysis of 14 cases
diagnosis. Several studies demonstrate involving the foot and ankle reports that
that this tumor may be the “great 2 patients had pulmonary metastases on
mimicker,” with patients initially CT scan at the time of diagnosis.103 The
presenting with what appeared to be goal of surgical treatment is to attain a
gouty arthritis,101 ganglia, plantar wide surgical margin while maintaining
fasciitis, synovitis, or plantar fibroma, a sensate plantigrade foot. However, this
but was subsequently shown to be is often not a practical option, and most
synovial sarcoma on tissue biopsy. MRI patients will require more radical
is the best study for determining the soft surgery. Adjuvant chemotherapy and
tissue extent of the tumor. T1-weighted radiation are indicated for tumors
imaging demonstrates a hypointense greater than 5 cm in size, as these
mass, while T2-weighted imaging shows primaries are more likely to
66 Foot & Ankle Specialist
Figure 11.
Sagittal magnetic resonance
imaging (MRI) (fast spin echo
[FSE], TR 4000/TE 20) of the right
distal tibia demonstrates a mass
contiguous with the tibial nerve,
reflecting a nonaggressive nerve
sheath tumor (arrow).
metastasize.102 These lesions have a
tendency for late local recurrence and
metastasis to the lungs.105 Surgery and
radiation therapy have achieved
excellent local control, but the presence
or absence of distant metastasis remains
the most accurate prognostic factor of
survival.106 In the aforementioned report
of 14 foot or ankle synovial sarcomas, 8
patients received below-knee
amputation, 2 had Chopart amputation,
and 2 had wide local resection (the
remaining 2 patients had metastatic
disease at diagnosis and opted for
chemoradiation).103 Six of these patients
ultimately developed pulmonary
metastases (56% total incidence of
metastatic disease). Every patient who
developed metastases in this series died
of their disease.
Differential Diagnosis
Other masses that may present in the
foot and ankle and can be mistaken for
neoplastic lesions, including rheumatoid
nodules, accessory muscles, nerve sheath
tumors (Figure 11), lipomata, and gout.
Therefore, maintaining a working
knowledge of the differential diagnosis for
masses about the foot and ankle is
paramount.
Conclusion
Because of the complex nature of the
distal lower extremity, the presentation
of tumors about the foot and ankle is a
diagnostic challenge for any physician,
consequently leaving the possibility of
misdiagnosis. A distinct scarcity of
literature is available concerning the
diagnosis and treatment of primary
tumors in the foot and ankle. With the
prompt diagnosis and treatment plans
outlined in this review, patients should
anticipate a reasonable chance of
survival and return to function.
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