Chondromyxoid fibroma is a rare benign bony tumor
classically occurring in the metaphyseal region of the long bones surrounding
the knee, but also found with relative frequency in other long bones, the
pelvis, ribs, and small foot bones. The small bones of the hand, however, are
rarely involved. The tumor is considered a physeal plate remnant and may
involve the epiphysis, diaphysis, or both along with its metaphyseal origin. It
may cause cortical expansion and destruction, but consistently respects the
We report a case of histopathologically confirmed
chondromyxoid fibroma involving a metacarpal, proximal phalanx, and synovial
tissues of the metacarpophalangeal joint. A 29-year-old woman presented with
pain, weakness, and a mass of the right ring finger metacarpophalangeal joint
that had persisted since prior surgery to remove an extraosseous chondroma.
Prominent soft tissue swelling and limited range of motion were noted,
corresponding to magnetic resonance imaging findings of lytic changes in the
right ring finger metacarpal and proximal phalanx, surrounded by an irregularly
enhancing mass with soft tissue extension. Open biopsy revealed calcified
cartilaginous synovial deposits suspicious for synovial chondromatosis, with
erosion into surrounding bones. Histologic examination revealed an unusual
chondromyxoid fibroma with joint involvement. The patient is >15 months out
from synovectomy, intralesional curettage, and placement of demineralized bone
matrix and allograft cancellous chips, and is without signs of recurrence. This
benign, curable lesion should be included in the differential diagnosis of a
bony lesion, even when a joint and adjacent bones are involved.
Chondromyxoid fibroma is a rare, benign bone lesion
accounting for <1% of all bony tumors.1-9 It was first described
in 1948 by Jaffe and Lichtenstein,10 who differentiated the
histologic findings from that of chondrosarcoma and enchondroma. Since then,
approximately 700 cases have been reported in the literature, primarily in long
bones and the small bones of the foot. However, <30 reported cases involve
the hand, and none of those document joint involvement. We present what we
believe to be the first case of chondromyxoid fibroma involving a metacarpal,
the adjacent phalanx, and the connecting synovial tissues of the
A 29-year-old woman presented with moderate dull pain,
weakness, and a mass of the metacarpophalangeal joint of the right ring finger
for over 2 years. She had similar symptoms approximately 3 years prior, at
which time she presented to another hospital and underwent excision of a mass
via an extended palmar Z incision. Final pathologic diagnosis was reported as
consistent with extraosseous chondroma and slides were not
available for review. The patient reported persistent pain unchanged since this
procedure, but increasing weakness and swelling. There was no history of trauma
and no symptoms suggestive of infection.
Physical examination revealed no visible mass but
prominent soft tissue swelling in the anteroposterior dimension. The patient
had tenderness to palpation over the metacarpophalangeal joint and a painful,
limited active range of motion of 25° to 45°. Radiographs of the right
hand revealed a lobulated but geographic lytic lesion of the metacarpal head of
the ring finger and the base of the adjacent proximal phalanx with surrounding
sclerosis and cortical destruction (Figure 1). Magnetic resonance imaging
showed an irregularly enhancing mass, dark on T1-weighted and bright on
T2-weighted images. There were lytic changes in the head of the ring finger
metacarpal and the base of the proximal phalanx with extension into the soft
tissues of the joint, but no involvement of the surrounding tendons (Figure 2).
Laboratory tests documented an elevated erythrocyte sedimentation rate and
C-reactive protein with negative rheumatologic antibody titers.
|Figure 1: Preoperative AP radiograph of the metacarpophalangeal joint of the patients right ring finger shows the lobulated, lytic lesion involving both the metacarpal and proximal phalanx. Soft tissue involvement is also noted. Figure 2: Preoperative MRI sections show the patients right ring finger metacarpophalangeal joint lesion. The lesion appears dark on T1 (A) and bright on fat-suppressed T2 (B), and demonstrates irregular enhancement after administration of gadolinium contrast (C).
An open biopsy was performed using a dorsal approach to
avoid complications from scar tissue from the patients prior surgery. The
incision was made centrally over the joint with subsequent division of the
extensor tendons. The synovium was noted to be involved by grossly calcified
cartilaginous deposits. These were removed with care given not to disrupt the
collateral ligaments. Secondary to these findings, an intraoperative diagnosis
of synovial chondromatosis was suspected. The lesions in the metacarpal head
and proximal phalanx were then extensively curetted and irrigated. Both lesions
were carefully packed with demineralized bone matrix and cancellous allograft
chips, which proved challenging due to the fragility of the remaining cortical
bone. Examination of the joint surface revealed articular cartilage loss at the
edges of the joint, likely due to the extensive tumor involvement of the
synovium, but adequate cartilage coverage remained centrally. Postoperative
examination under anesthesia revealed full passive range of motion of all
joints of the ring finger. Intraoperative cultures were negative and the
patient experienced no complications from the surgery.
On pathologic examination, the specimen grossly
consisted of multiple irregular fragments of rubbery tanyellow to
graywhite tissue. Histologic examination revealed a neoplasm that was
variably comprised of nodules of maturing cartilage (Figure 3A) and lobules of
spindle cells. The latter were admixed with occasional stellate cells and
contained a prominent myxoid background (Figure 3B). Within the spindle cell
regions were rare moderately enlarged and hyperchromatic cells (Figure 3B,
inset). The periphery of the lobules was partially rimmed by areas of
osteoclasttype giant cells (Figure 3B, arrows). There was focal
dystrophic calcification. There was no evidence of mitotic activity, necrosis,
or lymphovascular space invasion. Despite the unusual location, the morphologic
features were indicative of the diagnosis of chondromyxoid fibroma.
|Figure 3: Photomicrographs of the patients chondromyxoid fibroma demonstrate foci of various stages of cartilaginous differentiation (A; hematoxylin-eosin 200), lobules of spindle cells rimmed by osteoclasttype giant cells (B; arrows; hematoxylin-eosin 200), and enlarged hyperchromatic cells (B; inset; hematoxylin-eosin 400). Figure 4: AP radiograph of the patients right hand was taken 4 months postoperatively. There is no evidence of recurrence or loss of bone graft signal.
At 9-month follow-up, the patient was experiencing
occasional sharp, activity-related pain, but reported no night pain or
functional limitations. Physical examination revealed a slightly limited but
improved and painless range of motion at the metacarpophalangeal joint,
measured to be 10° to 70°, and slightly diminished but functional grip
strength. Postoperative radiographs taken at 4 months showed no new lytic
lesions and no loss of bone graft signal (Figure 4).
Accounting for >1% of all biopsy-proven bone lesions,
chondromyxoid fibroma occurs classically as a metaphyseal lesion of long bones
surrounding the knee, with the proximal tibia being the most common site of
occurrence.2,4-8,11-14 The tumor has also been noted with relative
frequency in other long bones, the pelvis, ribs, and the small bones of the
foot.1,2,5,7,8,11-14 Mandibular involvement has been reported with
some frequency, but due to differences in histologic features is thought to be
a separate process of odontogenic origin.4 Although small bone
involvement accounts for approximately 19% of all lesions,4,9,11-13
chondromyxoid fibroma is rarely found distal to the carpus. Large case series
and individual case reports document only 11 metacarpal lesions and 14 cases of
upper extremity phalangeal involvement.2,6,7,11-25 An isolated case
of carpal involvement in the trapezoid bone also exists.26
Patients with chondromyxoid fibroma are generally in
their second or third decade of life, with between 50% to 82% of patients
falling in this age range.1,3,4,6,9,13,14 A slight male predominance
is normally agreed upon, but male-to-female ratios between 1:1 and 2:1 are
found in the literature.1-8,11-14,27 The most common presenting
symptoms are pain in up to 100% of cases, and swelling in up to
75%.5,27 Swelling is more likely to be noted when areas of tumor
involvement have minimal soft tissue coverage, such as in the hands and feet.
Radiographic findings in chondromyxoid fibroma are
consistent with a benign process, but vary depending on the bone involved.
Overall, the tumors will appear lytic, round to elongated, and sharply defined
with a sclerotic rim and frequently scalloped borders, and may appear to have
gross trabeculations.2-5,8,11,14 There is rarely radiologic evidence
of calcification, though histologic calcification is not
uncommon.2,3,5,9,11,13,27 Reactive new bone formation may occur in
up to 39% of cases.12 In long bones, the classic eccentric
metaphyseal location is most clearly visible. However, expansile full-width and
even full-bone involvement is noted in the small and flat
Due to its histologic combination of chondroid, fibrous,
and myxoid components, it has been suggested that chondromyxoid fibroma is
related to physeal development.2,3,28,29 This impression is enhanced
by the generally metaphyseal location when present in a long bone. Initial
reports documented the tumors respect for the epiphyseal
plate,3,30 but it was later reported to cross even open growth
plates, involving both the metaphysis and epiphysis in a number of
cases.1,2,4,5,11,12,28,31 Cases have been documented to extend fully
through the epiphysis to the articular cortex.12 This is especially
true in the small bones; Wu et al13 documented a rate of epiphyseal
involvement in the small tubular bones of 42%. Although the capability of the
tumor to involve multiple areas of a bone has now been recognized, the
literature is still limited to cases of single bone involvement.
Dahlins1 case series contained a large metatarsal lesion
causing mechanical erosion of the adjacent phalanx. Wilson et al12
described the erosion of another metatarsal lesion into the metatarsophalangeal
joint, causing loose body formation. However, true tumor involvement of
adjacent bones and the connecting synovial tissues seen in our patient has not
heretofore been described.
The gross appearance of the tumor tissue has been
described as grayish- to bluish-white, glistening, lobulated, and firm, and may
strongly represent its chondroid origins.1-3,5,6,8,11,14,27
Histologically, stellate or spindle-shaped cells are arranged in a lobular
pattern on a background of most commonly myxoid, fibrous, or least commonly
chondroid matrix.3,5,10,11,13,28 Areas fitting all of these
descriptions may be found within a single lesion, in varying
proportions.1,4,5,10,11,14,27,28 The lobular appearance is made of
hypocellular central areas with a condensation of nuclei at the periphery of
each pseudolobule.1,11,13,14,28 Benign-appearing cells with oval
nuclei may be present in the interlobular areas, along with blood vessels and
multinucleated giant cells.2-4,10,13 Mitotic figures and bizarre
cytological features, such as hyperchromatic nuclei, pleomorphism, and nuclear
vacuolization, may also be seen,2,4,9-11 with a higher percentage of
cellular atypia found in the small bones.13 It is due to these
features that chondromyxoid fibroma is sometimes misdiagnosed as a malignant
lesion, though it has only rare mitoses.3,11,14 Aneurysmal bone
cysts may also be present within the lesion.11,13
Treatment options for chondromyxoid fibroma include en
bloc resection, simple curettage, and curettage with bone grafting or
polymethylmethacrylate (PMMA) placement. Simple curettage offers the least
morbidity but the highest risk of recurrence, with rates between 20% and 80%
documented in the literature.2,3,5-7 This recurrence is thought to
be a result of residual tumor left after surgical removal. En bloc resection
has an almost negligible recurrence rate, but is associated with high morbidity
and marked functional loss depending on the location of the tumor. Some studies
advocate curettage combined with placement of corticocancellous bone graft or
PMMA and report a low recurrence rate for these procedures.2,5,7
Overall recurrence rate for chondromyxoid fibroma, independent of surgical
technique, is approximately 20%.1,2,4-8,11,13,27 Before presentation
to our center, our patient initially underwent excision of a soft tissue
lesion, which recurred within 1 year.
Chondromyxoid fibroma is a rare bony tumor which,
although commonly found in the foot, involves the bones of the hand
infrequently. Initially considered to respect the physeal scar, the tumor is
now recognized to cross that boundary and involve the epiphyseal or diaphyseal
regions, but is considered not to violate the periosteum. Our case was a
previously unreported incident in which a chondromyxoid fibroma crossed the
metacarpophalangeal joint of the hand, involving both the metacarpal and
proximal phalanx as well as the synovial tissues. This suggests that the
behavior of this tumor may not yet be fully defined, and one must thus include
this benign, curable lesion on the differential diagnosis even when a joint and
adjacent bones are involved.
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Drs Slotcavage and Ogilvie are from the Department of
Orthopedic Surgery, University of Pennsylvania, and Dr Dickson is from the
Department of Pathology and Laboratory Medicine, Pennsylvania Hospital,
Dr Slotcavage received a research fellowship from
Stryker Orthopaedics. Drs Dickson and Ogilvie have no relevant financial
relationships to disclose.
Correspondence should be addressed to: Christian M.
Ogilvie, MD, Garfield Duncan Bldg, Ste 2C, 301 S 8th St, Philadelphia, PA