Non-Hodgkin's lymphoma can present as either a nodal form with local or
regional lymphadenopathy or, less commonly, as an extranodal form outside the
lymphatic
system1,2.
Musculoskeletal involvement of non-Hodgkin's lymphoma occurs in 25% of
patients, typically as metastasis and, rarely, as primary lymphoma of bone or
soft
tissue3,4.
Non-Hodgkin's lymphoma involving the synovium is rare, with only thirteen
reported
cases4-15.
Additionally, all of these reported patients presented with some symptoms
suggestive of inflammatory arthropathy, including pain, intra-articular
effusion with joint swelling, or radiographic changes (see Appendix). None of
the previous reports described lymphoma involving the elbow joint.
We report an unusual case of synovial non-Hodgkin's B-cell lymphoma of the
elbow, with joint contracture being the primary presenting complaint. The
patient was informed that data concerning this case would be submitted for
publication.
A thirty-three-year-old right-hand-dominant man who worked as an inventory
manager was referred to us after being treated for progressive loss of motion
following a hyperextension injury. The primary care physician had performed
the initial evaluation and treatment four months after the injury. At that
time, there had been lateral and anterior tenderness on clinical examination,
without motion restriction. The patient was referred for physical therapy but
gradually had development of activity-related pain and progressive loss of
elbow motion that limited his ability to perform activities of daily life.
The patient was referred to us nine months after the initial injury of the
elbow. At that time, the patient complained of progressive loss of motion,
pain along the medial aspect of the elbow, and intermittent paresthesias in
the distribution of the ulnar nerve. The clinical examination revealed a
healthy young man with an unremarkable medical history except for a previous
problem with gastritis. The patient denied any constitutional symptoms such as
night sweats, fever, weight loss, or any other systemic complaints. Aside from
the involved elbow, the general physical examination was unremarkable.
Examination of the right elbow revealed a flexion contracture of 40°
and a 10° loss of flexion as compared with the left elbow. The patient had
normal forearm rotation bilaterally. No evidence of active synovitis of the
elbow was noted, no swelling was observed, and no masses were palpable. The
ulnar nerve was tender to palpation, but no motor or sensory deficits were
detected clinically.
Routine anteroposterior and lateral radiographs of the elbow did not show
any abnormality. An electromyographic study of the right upper extremity,
performed because of symptoms of numbness and tenderness of the ulnar nerve,
revealed unremarkable findings.
In spite of vigorous physical therapy and the application of a turnbuckle
splint, the elbow motion deteriorated to a 60° flexion contracture with
active flexion to only 90°. Because of the progressive impairment, an
elbow capsulectomy was advised.
With the patient under general anesthesia and with use of a sterile
tourniquet, the elbow joint was approached through a straight posterior
incision. A complete anterior and posterior capsulectomy was performed. A
small amount of fleshy, tan tissue was observed in the olecranon fossa at the
end of the procedure and was sent for permanent histopathological evaluation.
Frozen-section analysis was not performed. The distal part of the humerus and
the surrounding soft tissues were of normal macroscopic appearance. Full elbow
extension was achieved at the end of the procedure.
Histological analysis of the tissue specimen revealed a diffuse infiltrate
of large atypical lymphoid cells with oval, irregular, indented, or lobated
nuclei; inconspicuous nucleoli; and scant cytoplasm (Figs.
1-A and
1-B). There also were areas of
overlying hyperplastic synovium with chronic synovitis. Immunohistochemical
stains showed that the atypical cells were CD20+ (B cells) and that many
coexpressed CD10 and bcl-2. A stain for CD30 was negative, but many mixed
small T cells (CD3+) were seen. These results confirmed the diagnosis of a
diffuse large B-cell non-Hodgkin's lymphoma with areas of chronic
synovitis.
There were no postoperative complications. Six weeks after surgery, the
range of motion of the elbow had improved to 125° of flexion with a
10° flexion contracture.
The patient was referred for treatment and a staging workup with elbow
magnetic resonance imaging and computed tomographic scans as well as a gallium
scan. The gallium scan revealed isolated gallium uptake in the elbow without
evidence of disease elsewhere. Magnetic resonance imaging showed a small joint
effusion with a bone-marrow abnormality involving the distal metaphysis and
epiphysis, with low T1 signal and high T2 signal and with patchy enhancement
following the administration of gadolinium. There was also enhancement
surrounding the ulnar nerve at the level of the cubital tunnel. The findings
were consistent with the diagnosis of lymphoma (Figs.
2-A, 2-B, and
2-C). The patient met the
criteria for stage-IAE diffuse large B-cell non-Hodgkin's lymphoma of the
right elbow and was managed with three courses of CHOP (cyclophosphamide,
doxorubicin, vincristine, and prednisone) chemotherapy followed by field
radiation therapy. The humerus and the right elbow were treated with a total
of 48.6 Gy with use of an anteroposterior/posteroanterior field technique with
6-MV photons over thirty-six consecutive days.
At the time of the most recent follow-up, fifty-four months after treatment
with chemotherapy and radiation therapy, the patient was in complete remission
without evidence of recurrent disease. The patient had returned to his
previous occupation and reported no limitation of his daily activities. There
was an almost complete pain-free range of motion of the right elbow, with
125° of flexion on the right side as compared with 145° on the left
side. There was full elbow extension and forearm rotation bilaterally.
A review of the English-language literature revealed thirteen cases of
non-Hodgkin's lymphoma presenting in a joint and involving the synovium (see
Appendix). The age of the patients ranged from thirteen to seventy-six years,
with seven female and six male patients being affected. The knee was affected
in eleven of the thirteen patients, whereas the wrist and sternoclavicular
joint were involved in the remaining two. All patients presented with symptoms
of pain, swelling, and decreased range of motion or systemic complaints. The
association of non-Hodgkin's lymphoma with immunosuppressive diseases is well
established8 and was
present in five of these
patients4,8,9,12,14.
Five patients had no evidence of disease on plain radiographs, and additional
imaging studies or procedures were needed for diagnosis. In one patient,
malignant disease was only recognized after a total knee replacement had been
performed4.
Chemotherapy and radiation therapy, in combination or individually, was used
in all patients for whom treatment was recorded. The clinical outcome varied
from complete remission and resolution of symptoms to death one month after
diagnosis. However, those with a poor prognosis had either an associated
immunosuppressive co-morbidity or were treated before the development of
modern techniques of diagnosis and
management6.
The typical workup for posttraumatic elbow stiffness involves radiographs
to assess for deformity, heterotopic ossification, or fracture-healing.
Sometimes, stress radiographs can provide additional assessment of
instability. Additional imaging with computed tomography scanning is indicated
to help assess complex osseous architecture in the setting of deformity, large
osteophytes, and fracture-healing in order to help with surgical planning.
Also, a magnetic resonance imaging scan is useful to assess medial and lateral
collateral ligament integrity or focal articular cartilage damage. A full
understanding of the pathology of the stiff elbow is necessary before
proceeding to
surgery16.
The case of our patient presented a challenging diagnostic problem not only
because of the rarity of the condition but also because of the presentation of
the disease without symptoms usually associated with a malignant condition.
The absence of local signs of inflammation or swelling in a healthy young
adult with normal radiographic findings did not suggest the necessity of
additional imaging studies such as magnetic resonance imaging before surgery.
In addition, the intraoperative findings were quite limited and therefore
frozen-section analysis was not performed.
Our patient had a good outcome, with complete remission and resolution of
symptoms. Radiation therapy around or to a joint can be associated with
complications such as fracture, contracture, chronic pain, edema, decreased
muscle strength, and
stiffness17.
However, a review of the literature indicates that doses of up to 65 Gy around
joints, as in this case, usually are not associated with substantial long-term
morbidity17.
Radiation to the lower extremity may be associated with worse functional
outcomes than radiation to the upper extremity
is17,18,
and radiation in combination with chemotherapy may be associated with more
acute tissue injury than radiation alone
is17-19.
Ultimately, the treatment of a malignant lesion in an extremity requires a
high level of suspicion and a team approach. If there is a suspicion of a
malignant lesion on the basis of symptoms, comorbidities, and plain
radiographs, additional workup should include laboratory studies, magnetic
resonance imaging, and computed tomography scans of the affected joint as well
as additional staging studies, including bone scans and chest and abdominal
computed tomography
scans4. A computed
tomography-guided or open biopsy is typically indicated to make a definitive
diagnosis. Furthermore, a team approach with orthopaedic, medical, and
radiation oncologists as well as musculoskeletal radiologists and pathologists
is essential for the proper care of the patient. In retrospect, our patient
would have benefited from a more thorough workup of the elbow stiffness with
preoperative magnetic resonance imaging and computed tomography scans.
Additionally, if a change in diagnosis will influence intraoperative
management, or if it is anticipated that fresh tissue may be required for
special studies, then frozen-section analysis followed by evaluation of
permanent histological sections should be performed.
A table showing all previously reported cases of synovial non-Hodgkin's
lymphoma is available with the electronic versions of this article, on our web
site at
(go to the article citation and click on "Supplementary Material")
and on our quarterly CD-ROM (call our subscription department, at
781-449-9780, to order the CD-ROM). ?