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Chondrosarcoma Presenting in a Rheumatoid Arthritic Foot Abstract: The
authors present a case of chondrosarcoma involving the left foot of a
71-year-old patient with long-standing rheumatoid arthritis.
Chondrosarcoma is a malignant tumor that rarely occurs in the
foot. The average duration
of symptoms is usually 15 months, with pain and swelling being the most
common. ( 1 ) In this case, the patient’s tumor was locally excised,
but metastatic disease developed concurrently.
The tumor was poorly responsive to chemotherapy and, despite a
temporary response to radiation therapy, it eventually let to the
patient’s death.
Bone tumors represent less then 1% of all tumors diagnosed in the
United States. ( 2 ) It is estimated that 1-2% of these tumors arise in
the foot. ( 3
)
Benign neoplasms outnumber malignant neoplasms by approximately
four to one. ( 4 ) Chondrosarcoma is a malignant tumor involving both cartilage and bone.
These tumors represent the third most common primary malignant
tumors of bone and are rarely located in the foot. ( 5
& 6 ) Over a 12 year period in Japan, only 4 cases of
chondrosarcoma involving the foot were noted in a review of 2,658
malignant bone tumors. ( 7
) Berlin also noted that chondrosarcomas rarely arise on the
small bones of the feet and are more frequently seen involving pelvic
bones and the femur. ( 8
)
The incidence of chondrosarcomas is less than 3% in the feet and
is not well understood. Chondrosarcomas are more common in males than females, with a
3:1 ratio. Chondrosarcomas
of the bones of the hands and feet are rare, and the criteria for
differentiating these lesions from the relatively common chondromas of
the bones of the hands and feet are not well delineated. ( 9 ) Clinical
features include dull pain of several years’ duration, local swelling
without inflammation, and disability of a contiguous joint.
The tumor typically appears as a gradually enlarging, painful
mass.( 10
) The mortality rate is unknown in chondrosarcomas in the feet
because of the rarity of the disease.
The histologic grade, location of the tumor, and adequacy of
surgical excision have a significant effect of survival. Recurrence of the tumor is highly suggestive of possible
malignancy. Prognosis for
survival in chondrosarcoma of the foot appears to be better than for
proximally located lesions. ( 10
) Treatment consists of chemotherapy, radiation, surgical
excision, and amputation of the affected extremity.
Early diagnosis and proper medical treatment while the tumor is
localized is the greatest chance for survival.
Rheumatoid arthritis is a systemic disease primarily affecting
the joints, whit upper and lower extremity manifestations.
Swelling, pain and limitation of motion are everyday problems to
the person living with this disease.
It is marked by inflammatory changed in the synovial membranes
and articular structures. Contracture,
deviation, and subluxation of the joint in the hands and feet are common
occurrences in the rheumatoid patient.
Painful rheumatoid nodules are often present in the upper and
lower extremities.
We present a case of a man who developed a myxoid chondrosarcoma
involving the dorsal left foot in the setting of long-standing
rheumatoid arthritis. A
Case Report
A 71-year-old man was first seen in consultation by the Medical
Oncology Service at the Salt Lake City Veterans Affairs Medical Center
in December 1992. The
patient had a past medical history of rheumatoid arthritis dating to
1985, for which he had received multimodality medical therapy, including
low-dose weekly methotrexate. Several
upper and lower extremity subcutaneous nodules had been discovered
during that time period that were consistent with rheumatoid nodules.
Recently, however, a nodule that had been present on the dorsal left
foot for at least 1 year rapidly increased in size.
Evidence of skin breakdown in that area led to resection of the
nodule, which had the gross histologic appearance of a rheumatoid
nodule. Within 2 weeks of
the resection, however, the nodule had regrown to its original size.
At that time solitary erythematous subcutaneous nodules were also
noted bilaterally on the anterior thighs.
The foot lesion was re-excised, and both thigh nodules were
biopsied. Histologic
examination of the nodules revealed a high-grade tumor with areas
containing predominantly myxoid or spindle components adjacent to each
other. Tumor necrosis was
noted, as were a number of mitotic figures.
The features of the tumor were most consistent with a high-grade
myxoid chondrosarcoma.
On physical examination the patient was noted to have bilateral 2
cm x 3 cm erythematous anterior thigh masses and a 3-cm ulcer on the
dorsal left foot, with central nodularity.
Further staging revealed the presence of bilateral lung nodules.
The patient was started on combination chemotherapy consisting of
mesna, doxorubicin, ifosfamide, and dacarbazine, ( 11) and
after one cycle her had slight improvement in the thigh nodules.
However, the foot mass grew rapidly despite the chemotherapy.
The thigh and foot lesions were then treated with external beam
photon irradiation, which led to the rapid regressing and near-complete
resolution of all three lesions for approximately 8 months.
The tumor subsequently recurred in all three areas, as well as in
the lung in the form of isolated nodules.
At that time the patient had significant pain in his left foot
and had difficulty ambulating.
Photographs of the foot lesion at the time of the recurrence are
presented in Figures 1-3. The skin over the proximal dorsal left foot
was thickened and immobile. A 6 cm x 6 cm irregular exophytic tumor mass was present,
with areas of central ulceration, necrosis, and bleeding. Multiple smaller subcutaneous nodules surrounded the larger
tumor. A photograph of the
right thigh mass is presented in Figure 4.
Figure 5 demonstrates the changes in the soft tissues of the
foot, as well as erosive changed in the superior aspect of the talus and
posterior aspect of the calcaneus. Amputation below the knee was recommended to the patient for
pain control and wound care reasons, but the patient refused.
Intravenous and oral morphine were administered for pan control,
and the patient expired related to the progressive tumor in March 1994.
Discussion Bone
tumors presenting in the feet should be evaluated to determine if the
neoplasms are benign or malignant.
The differential diagnosis for benign tumors involving the feet
should include osteoid osteoma, osteoblastoma, enchondroma,
osteochondroma, giant cell tumor, unicameral bone cyst, chondroblastoma,
chondromyxoid fibroma, and aneurysmal bone cyst.
The differential diagnosis for malignant tumors of the feet
should include chondrosarcoma, Ewing’s sarcoma, osteogenic sarcoma,
Kaposi’s sarcoma, synovial sarcoma, malignant melanoma, and
fibrosarcoma. Taking a
careful history and completing a thorough physical examination of the
patient will often lead to the use of the appropriate diagnostic
procedure. Biopsy and / or
surgical excision followed by histologic examination of the tissue are
essential for establishing a diagnosis.
Although most bone tumors in the feet are benign, malignant
tumors do occur and can be difficult to manage for both the physician
and the patient.
In the patient described in this report, the relationship of the
development of the chondrosarcoma to the long-standing rheumatoid
arthritis, or the treatment thereof, is unclear.
There is a clinical association between the treatment of patients
with autoimmune diseases such as rheumatoid arthritis, systemic lupus
erythematosus, polymyaglia rheumatica, or temporal arteritis with
long-term immunosuppressive regimens and the development of Kaposi’s
sarcoma. ( 12
) This form of Kaposi’s sarcoma, which is unrelated to HIV-1
infection, usually presents clinically and pathologically like the
classic of African (epidemic) form. ( 13 )
The histologic features of the tumor found in this patient were
not consistent with the diagnosis of
Kaposi’s sarcoma, but the pattern of the spread of the tumor
noted in this case (subcutaneous nodules) is distinctly different from
that of typical soft tissue of bone sarcomas, which metastasize
predominantly to the lungs. We
are unaware of an association between the development of other types of
sarcomas and either rheumatoid arthritis of long-term immunosuppressive
therapy, such as the weekly methotrexate and corticosteroids used to
treat this patient.
Chondrosarcomas are malignant tumors that occur rarely in the
bones of the feet. But when
evaluation bone tumors, chondrosarcomas should be included in the
differential diagnosis. Early
diagnosis and proper medical management are essential to achieve good
local tumor control and to reduce the risk of the subsequent development
of distant metastases. Standard
surgical approaches for well-localized tumors include wide local
excision of amputation, but should be considered prior to the use of
extensive local surgical procedures alone. Summary
The authors presented a rare case study of a chondrosarcoma
involving the left foot of a patient with rheumatoid arthritis.
The patient was unresponsive to chemotherapy but responded to
radiation therapy for a short time.
He subsequently developed metastases to the lower extremities and
left lung, which led to his death.
The prognosis is unfavorable in most such metastatic tumors.
It is very important to establish the diagnosis early and to
treat the tumor aggressively while it is still localized. Acknowledgment
We thank Marva Jensen, MEd, for reviewing the manuscript. References 1. Pachter MR, Alpert M: Chondrosarcoma of the foot
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© 2001, Dr. Brian Richman, All Rights Reserved |