Clin Podiatr Med Surg 25 (2008) 167–181
Synovial Sarcoma: A Case Report Albert V. Armstrong, Jr, DPM, MS, FACPRa,*, Andrea Aedo, DPMb, Sarah Phelps, DPMc a
Division of Medicine, School of Podiatric Medicine, Barry University, 11300 NE 2nd Avenue, Miami Shores, FL 33161, USA b Department of Podiatric Medicine, Jackson North Medical Center, 160 NW 170th Street, North Miami Beach, FL 33169, USA c Mercy Hospital, 3659 South Miami Avenue, Suite 3008, Miami, FL 33133, USA
There are several bone and soft tissue tumors that have a predilection for the lower extremity. With regard to imaging, plain radiographs should always be the initial imaging modality because of its availability and low cost. If advanced imaging is necessary, CT is the imaging method of choice for bone tumors. For soft tissue tumors, however, MRI with and without contrast should be ordered to determine if there is a vascular component to the tumor. With this imaging protocol followed, the patient discussed in this article was diagnosed with a synovial sarcoma, and was treated before the tumor had the opportunity to metastasize. The condition, however, still had a devastating effect on him and his family.
Case report A 37-year-old white male presented to the clinic with chief complaint of swelling accompanied by heavy, dull pain of the right foot for approximately 5 days. He stated that he came in for evaluation urgently because his symptoms had been affecting his gait and activities of daily living, giving his wife and family much concern. The patient related that there had been a marked increase of swelling to the right foot at the end of the workday. He denied trauma or previous injury, change in activity, change in shoe gear, and so forth. The patient denied history of fever, malaise, or weight loss. The patient had been treating symptoms with ice and ibuprofen to
* Corresponding author. E-mail address:
[email protected] (A.V. Armstrong, Jr). 0891-8422/08/$ - see front matter Ó 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.cpm.2007.11.002 podiatric.theclinics.com
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decrease swelling and for pain control, respectively. These treatments provided no relief and were increasing in severity with time. His past medical history was unremarkable with no contributing family or social history. Past surgical history involved an appendectomy at 8 years old. At time of presentation, the patient was not taking any medications and had no known drug allergies. The patient was moderately active at work and in exercise. He was concerned that repetitive use of his right foot at work might be a contributing factor. On clinical examination, the patient was observed with an antalgic gait favoring his right lower extremity. A deep, palpable mass measured to be approximately 7 5 cm was noted on the plantar aspect of the right foot just proximal to the metatarsal heads. Pain on palpation was noted to the dorsal and plantar aspects of the right foot. Also noted was a gross enlargement or edema of the right forefoot in comparison with the contralateral foot. There was no local elevation of temperature, puncture wounds or open lesions, drainage, streaking, palpable cords, or other clinical signs of infection noted to the right foot. The patient’s neurovascular status was intact. The patient had full range of motion of all pedal joints, without crepitation. Muscle strength of the foot and ankle was not affected. Imaging A standard foot series of radiographs were taken of the right foot to rule out fractures, foreign body, and so forth. Upon radiographical evaluation, distortion of the cortex was observed along the medial aspect of the fourth metatarsal suggestive of an enlarging, soft tissue mass. An area of mild radiopacity was noted throughout transmetatarsal area (Fig. 1). No breaks in the cortical margins were noted. No foreign bodies were present radiographically. Bone density was within normal limits and evidence of soft tissue swelling was noted on lateral radiograph. Prescription for an MRI with and without contrast of the right foot was given to the patient to have done as soon as possible to visualize soft tissue pathology. Prescription was given for Motrin 600 mg for stronger pain management and antiinflammatory effects. Two days later, the patient went for an MRI at a nearby outpatient facility. From there he requested a copy of the MRI on compact disk and had a close friend and physician review it for his medical opinion. Noticing the urgency of what was seen on MRI, this physician referred him to a wellrespected orthopedic oncologist at a nearby university teaching hospital and medical complex to have further evaluation. On the T1-weighted MRI, a well-defined ovoid mass with gently lobulated margins and a homogeneous signal intensity similar to that of adjacent muscle was noted throughout the intermetatarsal compartment involving the second, third, and fourth metatarsals with displacement of the flexor tendons (Figs. 2 and 3). With short tau inversion recovery technique employed, the tumor is visualized with
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Fig. 1. Dorsoplantar and medial oblique radiographs of the right forefoot show bone erosion and pariosteal reaction of the medial aspect of the fourth metatarsal shaft.
a hyperintense signal distinguishing the pathologic nature of the mass as in comparison to the T1-weighted image (Figs. 4 and 5). Needle biopsy was performed at the physician’s office days after initial visit with podiatry to obtain a definitive diagnosis. Biopsy was sent to pathology and within 30 minutes results returned with diagnosis of synovial
Fig. 2. T1-weighted MR images, axial slices. The images demonstrate the homogeneous, lobulated mass, with signal intensity similar to that of muscle. (A) Plantar and (B) intermediate slices. (C) Distortion of the fourth metatarsal shaft medially, consistent with the radiographic images.
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Fig. 3. T1-weighted MR images, sagittal slices (A, B) and coronal slice (C). These images demonstrate the mass, with plantar displacement of the flexor tendons.
sarcoma. Full-body CT scan was also performed on this day to evaluate for distant metastases. Areas of concern were noted on vertebrae L4 and L5 and on the thyroid gland. With these findings, the orthopedic oncologist referred the patient to a sarcoma specialist within this medical community. This physician heads a weekly sarcoma conference discussing difficult or rare cases
Fig. 4. STIR MR images, axial slices. Compared with the T1 weighted images, these images demonstrate the tumor with high signal intensity, consistent with the high fluid content of the mass. (A, B, C) Plantar, intermediate and dorsal slices, respectively.
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Fig. 5. STIR MR images, sagittal slices (A, B), and coronal slice (C). These images demonstrate the lobulated nature of the mass.
and planning for treatment strategies. The sarcoma specialist ordered an ultrasound of the thyroid and an MRI of the lower back and vertebral column both returning without any significant findings.
Treatment course After conference review of the patient’s case and the accumulated findings thus far, the anticipated treatment was to undergo two treatments of inpatient chemotherapy, assess the percentage of tumor kill, and undergo a transmetatarsal amputation with possibility of adjuvant postoperative chemotherapy treatments. Within the following week, an intra-arterial port for chemotherapeutic treatment was placed inches proximal to tumor site, unfortunately resulting in a major complication after the first round of chemotherapy. Secondary to the close proximity of port placement, a backflow of the chemotherapeutic agents occurred leaving the patient with essentially a chemical burn from the knee distally from the inside out. This resulted in extreme pain and the inability to bear weight on the affected limb thus forcing him to use crutches for assistance in ambulation from this point forward. Pain was tolerable only with continuous use of morphine and percocet. After this major complication, the patient felt the physician had made a major oversight. It was brought to his attention
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that the port should have been placed just distal to the knee to prevent such a complication. The patient was persuaded by the orthopedic oncologist not to take any legal matter onto the sarcoma specialist being that he was a very prominent and successful physician in the community. Therefore, he was referred and pleased with another specialist who was also a part of the sarcoma conference to conclude his treatment. Meanwhile, the burn encompassing the lower extremity was being treated conservatively with silvadene cream and protective wrapping. Discussion was made regarding wound-healing effects of hyperbaric chambers versus doing an emergency below knee amputation. The patient made this decision easy for the doctors being that he had researched his condition via personal friends in the medical field, advice by prostheticians and orthopedists, and research of literature. After much deliberation, he elected for a below knee amputation with an Ertl [1] modification, which was to be done after the second round of chemotherapy. An Ertl modification allows for tibiofibular stabilization created by a bony bridge and also allows for the reestablishment of normal intramedullary pressure improving the surrounding bone and soft tissue blood supply. The Ertl procedure attempts to reconstruct the amputated lower extremity limb through anatomic and physiologic means in attempt to avoid the inactive residual extremity syndrome. This confirming that the amputation with additional modification would provide proper weight bearing allowing him to be more athletic and active postoperatively with a reliable prosthesis. With the worry of supporting a family including a 5-month-old son, this was a major concern for him. He also felt that with the extent of this type of amputation versus doing the transmetatarsal amputation, there would be a wider margin of tissue resected, decreasing his chances for recurrence. To improve the quality of treatment, the second round of chemotherapy was planned to be the same regimen of medicines but on an outpatient basis. The patient stated that this made the situation a much more bearable one being that he was comfortable going home at the end of treatment to deal with the post-chemo side effects. Another MRI was taken for preoperative planning of the surgery, to assess the percentage of tumor kill, and to see the extent of soft tissue damage secondary to the chemotherapy burn. Shortly thereafter, the below-knee amputation with Ertl modification was performed without any major complications. At this point the patient adds a personal story showing the frustration he has undergone. The patient stated then when he was discharged home shortly after his surgery, he decided to take a ride on his Harley motorcycle down the driveway for a little cathartic excitement. He quickly found out that when he needed to stop the bike, a second leg was not there to provide the support to do so and resulted in a fall without injury. At this time, the reality of knowing what it meant to have had a below-the-knee amputation set in and he had a ‘‘panic attack.’’ As a result, he began seeing a psychologist who placed him on antidepressants and coached him on the benefits of
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drinking mass amounts of fluid in combination with the use of antacids during his chemotherapy treatments. The patient stated the fluid/antacid combination advice helped a great deal with the nausea and therefore was never seen without a cooler full of drinks in the hospital. Three to 4 weeks postsurgery, the percentage of tumor kill was reassessed at unsatisfactory 60%. That being said, the patient went for groshong/umbrella port placement at the hospital to begin adjuvant chemotherapy. This time his physician changed the regimen to Ifex (Ifosfamide), the same chemotherapy Lance Armstrong underwent to battle testicular cancer. Ifex is an antineoplastic chemotherapy drug classified as an alkylating agent. The patient was advised that this was a last-resort chemotherapeutic agent used because of its extreme side effects. Side effects include hallucinations, nausea, vomiting, loss of appetite, blood in urine, low platelet count, and risk of developing leukemia. The plan was to undergo 4 inpatient treatments that were 5 days long with a 2-week rest period in between. The patient checked into the hospital Monday mornings and did not leave until late Friday afternoons. He described that in those weeks of chemotherapy he became ‘‘violently ill as though it was like an exorcism.’’ He also spoke of the extreme pain involved in the process for which he was on constant morphine, pain killers, and nausea medication. He stated that the week was ‘‘like a haze. a fog’’ where he didn’t ever know what day it was or remember which family or friends were there to visit him. Doctors quickly found nausea medications that were properly suited to better prepare him for the next three rounds of Ifex. During this process, he also experienced extreme constipation, which took him some time to figure out the proper amount of the nausea medication to lessen the severity. Another surprise encountered was to find that the chemotherapy caused his urine to turn red. And to top it all off and add some fear, during one of his treatments he witnessed a nurse drop a bottle of the chemotherapy medication and saw that it had burned through the floor because it was so caustic. Talks of how ironic it was that they were administering those same medications into his system as that was happening in front of him. He stated that much of the hospital staff was amazed he made it through all four treatments with the Ifex. They usually do not see patients make it past two, if not just one treatment. The last round of chemotherapy was in August 2005. Follow-up care consisted of chest radiograph, full-body CT scan, and full blood work done every 2 months for the next 2 years. In January 2006, there was a broken blood vessel visualized on his chest film. The following CT scan did not report any significant findings. There was concern by the patient’s friends as to why positron emission tomography (PET) scans were not being ordered, but the two sarcoma specialists strongly felt that this type of scan produced too many false positives and put patients through unneeded physical and mental hardship. Just before the last round of Ifex, the patient was molded for his prosthesis. His healing after surgery was much delayed secondary to the negative
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effects chemotherapy has on healing potentials. He chose the flex foot prosthesis for it has an integrated shock-absorbing system allowing for a painfree landing, once again enhancing activity with an amputation. The patient was eager to be there for his son and to begin working again. Soon after he finished chemotherapy, the surgical site healed and he began ambulating with the prosthesis. He used it so much that in time he developed a painful bone spur at the distal end of the stump putting a major decline on his abilities to function well in the prosthesis. Shortly after, in February 2006, he underwent a second surgery for exostectomy of the spur. In March 2006, complete healing was achieved and a new socket was molded to refurbish his prosthesis. The patient states he currently is about 90% of what he was before receiving his diagnosis and the associated symptoms. He is now able to climb stairs, has a perfect gait, and walks fast but unfortunately is not yet able to run. He is working on his goal to be able to run again by concentrating on his most recent Christmas present: DVDs made by a physical therapist for amputee athletes and the transtibial runners. Aside from the physical detriment the patient endured from beginning to end, he realized the crippling emotional and financial effects this process had on him and his family. He was forced to sell his company, his wife had to begin working to support the family and pay continuously mounting medical bills (over $1 million in insurance bills after chemotherapy was completed), and his parents moved to Florida to help support him and the family through all the hardships entailed. The patient relates how he was able to get through it all with more strength knowing that someone was with him every day. In the end, he is most of all thankful to be alive and be with his family and raise his son. Day by day he crosses his fingers to remain cancer free.
Synovial sarcoma research Synovial sarcoma most commonly affects adults in the third to fifth decades of life, but can occur in adolescents as well. Clinically and morphologically it is a well-defined soft tissue tumor that may or may not present as a painful lesion. It earned its name because of its anatomical location and histological resemblance to normal synovial tissue [2]. Studies now show it can be composed of variable epithelioid and spindle cell populations that initially grow quite slowly [3,4]. The tumors are encapsulated and frequently in contact with bone. Because there are often few anatomical barriers, malignant spread to surrounding nerves and vasculature is common. Soft tissue sarcomas in general are nearly 100 times less frequent than cancer of the breast, lung, or prostate [5]. Synovial sarcoma specifically represents only 8% to 10% of all soft tissue sarcomas. Ninety percent of these cases occur in the extremities, with the most common site being the knee,
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followed by the ankle and foot region. These lesions are usually painful when occurring on the plantar aspect of the foot. They may frequently be mistaken for a fibroma when occurring plantarly and a ganglionic cyst when dorsal [6]. Diagnosis of this malignant tumor can be made in several ways. Routine plain radiographs will be unremarkable unless the lesion has begun to invade bone. If bony involvement is present, CT scan can depict periosteal reaction, soft tissue calcifications, and bone invasion in general. Triple-phase bone scan will often demonstrate increased uptake in all three phases secondary to periosteal inflammation of adjacent bone. Because of the neovascularity associated with the tumor process, angiography is often quite useful to outline the margins of the lesion. MRI will provide useful information regarding the extent of tumor invasion and preoperative planning for excision [6]. Radical excision of the mass with wide margins is often required. Tendon sheaths are often involved, which may necessitate a more radical amputation. Sometimes localized irradiation is used along with multidrug adjuvant chemotherapy for systemic control. A study several years ago found that 11 of 18 patients with localized soft tissue sarcoma who received adjuvant immunotherapy were free of disease, compared with only 5 of 15 treated by surgery alone. Immunotherapy also prolonged the median disease-free interval from 7.3 months to 15 months in the patients who experienced recurrence of their disease [7]. Five-year survival rates overall are 23.5% to 64.0%; 10-year survival rates are 11.2% to 34.0% [3]. Literature review In a retrospective study, Kirby and colleagues [8] in 1989 analyzed the cases of 83 patients who had a soft tissue tumor or tumor-like lesion in the foot or ankle. Their research focused on identifying the relative frequency of the lesions and which factors, if any, could be used to identify them preoperatively. Prognostic predictors in the development of a soft tissue tumor, such as age and sex, were noted, as were the limitations of diagnostic technique. They established that 87% of the lesions were benign, with ganglionic cysts and plantar fibromatoses being the most common. Of the remaining 13%, which were malignant, 45% were synovial sarcomas. They found the age of the patient and the locations of the lesion were the two most important factors that characterized the malignant tumors. For only 12% of the total 83 patients, radiographs were helpful in identifying the nature of the lesion. Their study also established that the size of the lesion, the presence of pain, a history of trauma, the duration of the symptoms, and the sex of the patient were not useful in discriminating a benign lesion from a malignant tumor. Zeytoonjian and colleagues [5] in 2004 explored the relationship between leg-ankle-foot and other body sites for the type of soft tissue sarcoma, frequency, and patient survival rate. A computerized database of 14,000
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patients, mostly with tumors treated over a 25-year period, was used. Of these patients, 175 had sarcomatous lesions located in the distal lower extremity. These were compared with 2367 lesions of similar diagnoses in other body parts. They found the most frequent distal lower limb tumors to be synovial sarcoma, osteosarcoma, and Ewing’s tumor. They found strong evidence that sarcomatous tumors of the leg-anklefoot are less commonly encountered than those of other parts of the body. The benign lesions most frequently seen in the leg-ankle-foot include epithelioid inclusion cysts, lipomas, ganglion cysts, enchondromas, hemangiomas, non-ossifying fibromas, unicameral and aneurysmal cysts, osteoid osteomas, osteocartilaginous exostoses, and, less commonly, giant cell tumors, chondroblastomas, and chondromyxoid fibromas. Malignant lesions are rarer and consist of a group of soft tissue sarcomas that include synovial sarcoma, clear cell sarcoma, fibrosarcoma, malignant fibrous histiocytoma, leiomyosarcoma, and others less frequently. Zeytoonjian and colleagues [5] established the most frequent soft tissue lesion was synovial sarcoma in the distal lower extremity. That is, of the distal 175 sarcomas, 38 were synovial. The differences (ie, the percentage distribution) of the various tumors for the distal site as compared with the rest of the body were pronounced, with the greatest differences noted for synovial sarcoma. Only 37 of the 2367 sarcomas in other body parts were specifically synovial sarcomas. Regarding mortality, their study found 11% of the patients died of synovial sarcoma in the lower extremity versus a 13% mortality rate of synovial sarcoma in other body parts. In terms of distribution, the greater frequency of synovial sarcoma in the distal lower extremity than in other sites may be evidence for the unusual prevalence of the apparent tissues of origin as compared with fat or histiocytic tissue. There was a remarkable difference in outcome with the death rate for the lower limb tumors set at 10%, while the same tumors at other sites had a death rate of 27%. The rate of metastasis and death is markedly reduced for this site as compared with others. Their study demonstrated in general that malignant tumors occur less frequently in distal sites than in more proximal sites, and that these lesions, even considering histologic subtypes, are less likely to metastasize or lead to the death of the patient [5]. In 2002, Sobel and colleagues [9] presented the case of a patient who was treated for a high-grade, deeply seated synovial sarcoma greater than 5 cm. The lesion extended to the lateral aspect of the distal lower leg, involving the fibular bone. For 10 years she had recurrent episodes of pain and swelling of the leg, for which she had seen several physicians. Her symptoms in the last year included increased tenderness, swelling, and overlying warmth of the leg. Radiographs showed periosteal reaction and permeative destruction of the distal right fibula. According to the American Joint Committee on Cancer’s 1997 classification system, the tumor was consistent with stage III soft tissue malignancy, giving it a relatively poor prognosis. Lab studies revealed markers for
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cytokeratins, epithelial membrane antigens, vimentin, and CD99 (Ewing’s sarcoma marker). The lesion was negative for S100, smooth muscle actin, human muscle actin, desmin, markers for endothelium (CD31, CD34), CD68 (macrophages), and factor XIII (fibrohistiocytic tumors), making it a synovial sarcoma. A below-the-knee amputation was suggested by her physician in Jamaica, but the patient wanted an alternative treatment and came to the United States. Wide excision of the tumor, including the fibula, was performed, followed by adjuvant radiation and chemotherapy. Approximately 1 year later, multiple lesions of metastatic sarcoma were excised from the left lung. Chemotherapy was continued for the patient. Sobel and colleagues site another study that supports limb-preserving surgery by means of wide resection, followed by radiation and adjuvant chemotherapy, without adverse effects on recurrence. Survival rates resulted in 80% survival after 5 years [9]. Yet another study presented two cases of synovial sarcoma misdiagnosed as posterior tibial tendon dysfunction. Both patients complained of pain and swelling of the medial foot and ankle and, because synovial sarcomas are slow growing, a misdiagnosis of arthritis, bursitis, or synovitis, among others, could be made. Chou and colleagues [10] described two patients who had signs and symptoms similar to those of posterior tibial tendon dysfunction. The first patient was a 44-year-old woman with a 2-year history of pain with compensatory limping. Plain radiographs were unremarkable. MRI findings suggested chronic tendinopathy, for which she was treated with local anesthetic injections and a short-leg walking cast. After these modalities failed to improve her symptoms, surgery was chosen. A hypertrophic synovium was biopsied and identified as synovial sarcoma, 2 to 3 mm in diameter. Two months after the initial operation, the patient had wide excision of the synovial sarcoma, posterior tibial tendon, and a portion of the navicular. She had radiation therapy and at 15 months after surgery, she is free of local recurrence and metastasis. The other case involved a 31-year-old healthy woman who complained of foot pain of 5 years’ duration. In the preceding year she had noticed a quarter-size tender mass that grew noticeably, causing her to limp. She was diagnosed with posterior tibial tendonitis, plantar fasciitis, and enthesopathy at separate offices for which she received two cortisone injections, physical therapy, and orthotic devices without relief. An MRI showed tenosynovitis and subsequently she was placed in a cast for 6 weeks. Ultimately, she underwent surgery at which time the mass was discovered and excised and the posterior tibial tendon was reconstructed. Synovial sarcoma was diagnosed upon pathology results. The patient was treated with a below-the-knee amputation and after 1 year was free of metastatic disease [10]. Hirao and colleagues [11] in 2005 evaluated a case of a patient diagnosed with florid reactive periostitis (FRP), which is a rare juxtacortical soft tissue lesion. The patient’s symptoms were remarkably similar to those of synovial
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sarcoma. The patient had progressive pain, swelling, and calor with an illdefined, immobile, hard bony mass. Radiograph and MRI findings mimicked those of synovial sarcoma. Proliferating fibroblasts with foci of osteoid and cartilage in the lesion was pathognomonic for FRP. Hiraga and colleagues [2] described synovial sarcoma at histologic and genetic levels in 1999. They approached their study under the assumption that there was no convincing evidence that synovial sarcoma always originates from synovial tissue. They asserted that the lesion sometimes arises from primitive mesenchymal cells rather than from preformed synovial cells. Preexisting synovial tissue, they stated, was no longer necessary for the development of synovial sarcoma. They described the case of a patient who had synovial sarcoma of bone that was verified by the detection of a chimeric SYT-SSX transcript. This specific chimeric transcript does not occur normally and thus provides a specific marker for synovial sarcoma. Twentytwo reported cases of synovial sarcoma demonstrated a specific reciprocal translocation. Cloning of the DNA adjacent to the breakpoints resulted in a rearranged configuration of DNA, which was transcribed to messenger RNA and was called a chimeric SYT-SSX transcript. A cytogenetic study was performed on short-term cultures from fresh surgical specimens obtained from 41 patients with soft tissue sarcomas of various histologic origins. The results demonstrated that synovial sarcomas (six tumors) were associated with a specific translocation between the X chromosome and chromosome 18. These chromosomal data have been used to ascertain the diagnosis of synovial sarcoma in undifferentiated soft tissue sarcomas. The results to date indicate that identification of specific chromosomal changes in sarcomas may provide a new diagnostic criterion for these tumors and possibly improve prognostication with regard to survival and response to treatment [12]. Gibbs and colleagues [3] in 1997 reviewed the cases of 62 patients with subcutaneous sarcoma. The authors evaluated the rates of survival and local recurrence in relationship to specific details of the tumor and treatment. In their study, treatment consisted of repeat excision with the goal of obtaining wide margins. At a median of 56 months after the repeat excision, 81% of the total patients had been continuously disease free, one had no evidence of disease, eight had died of the disease, and three had died of other causes. The 5-year rate of disease-free survival was 85% of 62 patients. Six percent of their patients were diagnosed histologically as synovial sarcoma. The most common diagnosis was malignant fibrous histiocytoma. There were three local recurrences, all in patients who had had a marginal resection. A patient had been referred to us after an incidental marginal excision of a synovial sarcoma during an ulnar-nerve transposition. She was managed initially with irradiation alone, but had a recurrence at 34 months and a transhumeral amputation was performed. At the time of writing, she had no evidence of disease. No recurrences were noted in patients who had had a wide local excision of the tumor.
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Gibbs and colleagues’ study noted a worse prognosis occurred with a large tumor, only a marginal excision, and adjuvant radiation therapy. They suggested that carefully planned operative treatment alone can provide excellent rates of survival for patients who have a subcutaneous sarcoma, including those who have a large or high-grade tumor and those who have residual tumor following a previous operation. Their team recommended operative excision or repeat excision with wide margins because of the high prevalence of residual tumor. Size is the most important tumor-related factor, and the operative margin is the most important treatment-related factor. They stated the additional value of adjuvant radiation therapy remains unproven [3]. Tumor staging allows assessment of both prognosis and treatment. In 1999, Cheng and colleagues [13] evaluated a modification of the tumor-staging system proposed by the American Joint Committee on Cancer (AJCC). Established in 1959, this system addresses growth first, as manifested by the size of the tumor (T). Secondly, spread to the lymph nodes (N) is considered; and last, distant metastasis (M) is evaluated. Because sarcomas seldom spread to the lymph nodes, this factor is irrelevant to this review. In the original classification scheme of the AJCC, the size and grade of the tumor were the chief determinants of the stage in the absence of metastasis. The grading system distinguished among low-, intermediate-, and high-grade tumors. More recently, with adjuvant chemotherapy and radiation often preceding tumor excision and biopsy, accuracy of staging has become increasingly important. These prebiopsy treatment modalities may alter the size of the tumor and the amount of necrosis, thus reducing the accuracy of pathological staging based solely on the resected tumor. Therefore, the staging of tumors needed modification to include both clinical factors (ie, size, anatomical location, and the presence or absence of metastatic disease) at the time of the diagnosis and the grade of the tumor (number of mitotic figures, cytological atypia, and the extent of necrosis) on biopsy. The most recent modifications to the staging occurred in 1999 and include anatomic depth and the reduction in the number of grades from three to two. Low grade now indicates Stage I or II and high grade indicates Stage III or IV. Stage III is reserved for the worst tumors, that is, high grade, large, and deep and no longer distinguishes between subcategory A and B. Finally, IIC has been added to identify high-grade, large, but superficial tumors. This new system more effectively stratifies patients into different prognostic groups and is more reliable and reproducible [13]. Discussion Multiple studies have been undertaken and are continuing to be done on the extensive array of factors related to synovial sarcoma. There are innumerable ways to approach this pathology, as demonstrated by the vast amounts of varying literature. Factors such as prognostic predictors and specific clinical symptoms, to treatment and survival rates are all being
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explored. Some scientists choose to focus on the relationship of synovial sarcoma to other soft tissue sarcomas while others concentrate on specific details of synovial sarcoma, such as histologic and genetic markers. Different studies maintain amputation is curative, while others propose wide-margin excision can demonstrate an 85% disease-free 5-year survival rate [3]. Some authors state that it is important to consider quality of remaining life and perhaps a radical amputation is not the best treatment option for the patient [9]. Although research takes different approaches and results may vary between studies, one thing is certain: a patient’s well-being is always the first and foremost consideration. In fact, the patient’s health is the stimulus for such research at all, and indeed dictates the overall method taken by the team of researchers.
Summary With regard to imaging tumors of the lower extremity, plain radiographs are the initial imaging method one should employ. If a bone tumor is clearly benign on plain films, further imaging is not necessary. If the benignity of a bone tumor is uncertain, CT is next imaging method of choice. For soft tissue tumors, MRI with and without contrast should be ordered after plain radiographs. Diagnostic ultrasound is an imaging modality that will be researched by the authors with regard to tumor imaging. Upon review of several articles pertaining to synovial sarcoma, one can appreciate the breadth of the literature addressing such a topic. Various studies demonstrate the relative newness of the research. More studies will enable us to better understand the risk factors, clinical symptoms, laboratory findings, and, ultimately, the treatment for synovial sarcoma. As one of the most frequent lower extremity tumors, it needs to be considered in every suggestive clinical case by a lower extremity expert [5].
References [1] Ertl J. Prosthetic primer: pain and the inactive residual extremity syndrome. Amputation Coalition of America 2000;10(5). Available at: http://www.amputee-coalition.org/ inmotion/sep_oct_00/prosth.html. Accessed May, 2006. [2] Hiraga H, Nojima T, Isu K, et al. Histological and molecular evidence of synovial sarcoma of bone. A Case Report. J Bone Joint Surg Am 1999;81:558–63. [3] Gibbs C, Peabody T, Mundt A, et al. Oncological outcomes of operative treatment of subcutaneous soft-tissue sarcomas of the extremities. J Bone Joint Surg Am 1997;79:888–97. [4] Reale C, Sinclair G. Synovial sarcoma in the foot. J Foot Surg 1986;25:124–7. [5] Zeytoonjian T, Mankin H, Gebhardt M, et al. Distal lower extremity sarcomas: frequency of occurrence and patient survival rate. Foot Ankle Int 2004;25(5):325–30. [6] Duh M. Synovial sarcoma. eMedicine WebMD. Available at: http://www.emedicine.com/ radio/topic668.htm. Accessed May, 2006. [7] Townsend C Jr, Eilber F, Morton D. Skeletal and soft tissue sarcomas. Treatment with adjuvant immunotherapy. JAMA 1976;236(19):2187–9.
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[8] Kirby K, Shereff M, Lewis M. Soft-tissue tumors and tumor-like lesions of the foot. An analysis of eighty-three cases. J Bone Joint Surg Am 1989;71:621–6. [9] Sobel E, Giorgini R, Oropeza R, et al. Limb salvage in recurrent synovial sarcoma of the right ankle and lower leg. J Am Podiatr Med Assoc 2002;92(2):90–6. [10] Chou L, Malawer M. Synovial sarcoma presenting as posterior tibial tendon dysfunction: a report of two cases and review of the literature. Foot Ankle Int 2004;11:810–4. [11] Hirao K, Sugita T, Yasunaga Y, et al. Florid reactive periostitis of the metatarsal bone: a case report. Foot Ankle Int 2005;26(9):771–4. [12] Karakousis C, Dal Cin P, Turc-Carel C, et al. Chromosomal changes in soft-tissue sarcomas. A new diagnostic parameter. Arch Surg 1987;122(11):1257–60. [13] Cheng E, Thompson R. Instructional course lectures, the American academy of orthopaedic surgeonsdnew developments in the staging and imaging of soft-tissue sarcomas. J Bone Joint Surg Am 1999;81:882–92.
