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Leiomyosarcoma of the Major Peripheral Arteries: Case Report and Review of the Literature
Leiomyosarcoma of the Major Peripheral Arteries: Case Report and Review of the Literature Joseph A. Blansfield, MD, Heiwon Chung, MD, Theodore R. Sullivan, Jr., MD, and Christopher M. Pezzi, MD, Abington, Pennsylvania
We describe a case of primary intramural, extraluminal leiomyosarcoma of the common iliac artery. The patient presented with pelvic and leg pain and pheochromocytoma-like symptoms. The diagnosis was made intraoperatively, the tumor was resected, vascular reconstruction was performed, and postoperative radiation therapy was administered. Primary leiomyosarcoma of a major peripheral artery is extremely rare, and this report examines the clinical presentation, treatment, and prognosis of these patients.
Although leiomyosarcomata of the central veins and pulmonary arteries have been widely reported, similar tumors of the aorta are much less common, and those arising from the peripheral arterial system are extremely rare.1 We present a case of a leiomyosarcoma of the left common iliac artery treated by surgical resection, vascular reconstruction, and postoperative radiation therapy, and we review the limited published experience with these tumors.
CASE REPORT A 42-year-old woman presented with a 1-year history of intermittent, severe, left groin pain with associated low back and buttock pain that radiated down her left anterior thigh. She described the pain as intermittently excruciating, deep in her pelvis, and worsening over time. Department of Surgery, Abington Memorial Hospital, Abington, PA, USA. Correspondence to: Theodore R. Sullivan, Jr., MD, Vascular Center of Excellence, Abington Memorial Hospital, 1200 Old York Road, Abington, PA 19001, USA, E-mail: [email protected] Ann Vasc Surg 2003; 17: 565-570 DOI: 10.1007/s10016-003-0038-6 Annals of Vascular Surgery Inc. Published online: 29 September 2003
The patient also reported episodic panic attacks, cold sweats, and episodes of diarrhea and constipation over this same time period. Her medical history included hypertension, hypothyroidism, lumbar disc herniation, a prior hysterectomy, and rotator cuff surgery. She had no family history of cancer or premature atherosclerosis. The patient denied exertional lower extremity symptoms, and review of systems was otherwise unremarkable. On physical examination, the patient was 5 feet, 11 inches tall and weighed 298 lbs. She was in no acute distress. Her blood pressure was 160/90 and symmetrical in both arms, with a heart rate of 70 and regular. There were no carotid bruits or jugular venous distention and her cardiopulmonary examination was normal. There were no abdominal masses, bruits, or inguinal adenopathy. Normal pulses were noted in both upper and lower extremities, and no evidence of lower extremity edema or venous congestion was detected. Laboratory values, including CBC, electrolytes, liver function tests, urine metanephrines, and catecholamines, were all within normal ranges. Her chest X-ray was normal. A computed tomography (CT) scan of the abdomen and pelvis showed a 6 · 5 · 3.5 cm soft tissue mass in the left lower retroperineum. The mass began just caudad to the aortic bifurcation and extended inferiorly along the left psoas muscle, inseparable along its medial border from the left common iliac artery (Fig. 1). The liver appeared normal, and there was no other evidence of
565
566 Case reports
Fig. 1. CT scan of the abdomen/pelvis with IV contrast showing a 6 · 5 · 3.5 cm soft tissue mass (arrow) in the left lower retroperitoneum extending along the left psoas muscle inseparable along its medial border from the left common iliac artery. lymphadenopathy in the para-aortic or right iliac regions. Magnetic resonance imaging (MRI) of the abdomen and pelvis demonstrated this same contrast-enhancing retroperitoneal mass enveloping the left common iliac artery with some medial displacement of the artery (Fig. 2). An aortogram demonstrated medial bowing of the left common and external iliac arteries, consistent with extrinsic compression from the adjacent hypervascular mass (Fig. 3). A left femoral venogram revealed extrinsic compression of the left common iliac vein with no evidence of invasion or thrombus. A octreotide-labeled nuclear medicine scan demonstrated a marked increase in somatostatin receptor activity corresponding to this pelvic mass, highly suggestive of a neuroendocrine tumor. The differential diagnosis preoperatively included (1) a neuroendocrine tumor, (2) a retroperitoneal sarcoma, (3) lymphadenopathy from lymphoma or metastatic disease, and (4) a primary vascular tumor. After abnormal catecholamine production was excluded, surgical resection for diagnosis and treatment was recommended. At surgical exploration through a left retroperitoneal approach, a smooth, well-circumscribed, firm mass was found enveloping the left common iliac artery and abutting, but not invading, the left common iliac vein. The left ureter was identified anterior and lateral to the mass, was not directly involved with the tumor, and was preserved. The tumor was inseparable from the wall of the left common iliac artery and appeared grossly to arise from that vessel. After obtaining proximal and distal vascular control of the left common, external, and internal iliac arteries, and after systemic heparinization, the tumor was resected en bloc with the common iliac artery leaving a 1-cm cuff of proximal artery. A 10-mm Dacron interposition graft was placed between the proximal left common iliac artery and both the external and internal iliac arteries to revascularize the left leg. Blood loss during the procedure was 700 cc, and the patient tolerated
Annals of Vascular Surgery
Fig. 2. Coronal section of an out-of-phase T2-weighted MRI of the abdomen/pelvis with contrast demonstrating contrast-enhancing mass intimately involved with the left common iliac artery.
the procedure well. Pulses in the left lower extremity were normal at the conclusion of the procedure. The gross specimen consisted of a 6-cm segment of the left common iliac artery with an enveloping ovoid, rubbery, pink/tan, fibrous mass measuring 6.5 · 4.5 · 4 cm. The mass had a swirling, dusty pink/tan parenchyma with central areas of hemorrhage. The tumor consisted of spindle cells with eosinophilic cytoplasm growing in interlacing bundles suggestive of smooth muscle differentiation (Fig. 4). No significant atypia was seen. Immunohistochemical stains for actin and desmin were positive, confirming the smooth muscle origin of the tumor. Mitotic activity varied with a maximum of 2 mitoses per 10 high-power fields. Focal necrosis was seen. The mass extended focally to the inked surgical resection margin. The final pathologic diagnosis was a low-grade leiomyosarcoma arising from the left common iliac artery. The postoperative recovery was uneventful, and the patient was discharged to home with a patent vascular graft on hospital day 4. Her preoperative pain was relieved completely. Because of the positive microscopic surgical margin, external beam irradiation was delivered, beginning at 4 weeks postoperatively, with anterior and posterior irradiation fields measuring 10 · 11 cm. The treatments were delivered to a dose of 4500 cGy in 25 fractions over 36 days. At 15-month follow-up, the patient is doing well, is back to work full-time, and is without pain. MRI at 1 year postoperatively demonstrated no evidence of recurrence or metastatic disease.
DISCUSSION In the vascular system, leiomyosarcomata arise from veins five times more commonly than from
Vol. 17, No. 5, 2003
Fig. 3. Aortogram showing extrinsic compression of the left common and external iliac arteries from an adjacent hypervascular mass (arrow).
the arteries. The most common site of occurrence is the inferior vena cava and comprises about half of all of the reported cases.1 Of those arising from arteries, the pulmonary artery is the site of origin in twice as many cases as the large systemic arteries.2 Few cases of leiomysarcomata of the major peripheral arteries, excluding those that involve the aorta, have been reported.1-15 Review of the published literature identified 18 previously reported cases of nonaortic leiomyosarcoma of a major peripheral artery. Including the present case, there are 9 women and 10 men have been discussed (Table I), suggesting no gender predilection. These 19 patients ranged in age from 33 to 79 years, with an average age of 58.6 years. Only four previous reports of a primary leiomyosarcoma tumor arising specifically from the common iliac artery have been described.2-5 Of the five cases of leiomyosarcomata arising from the iliac artery that have now been reported2-5 three of these five patients presented with symptoms of claudication.2,3,5 Our patient had no history of claudication and had a normal vascular examination prior to undergoing resection. Claudication will result when the tumor grows intraluminally or when tumor growth otherwise obstructs flow through compression. In the present case, the tumor growth was outward from the wall of the vessel encasing the artery, but antegrade flow was preserved.
Case reports 567
Fig. 4. Low-power view of smooth muscle neoplasm with the adjacent artery. The neoplastic spindle cells blend imperceptibly with the muscular wall of the artery seen in the upper left. Inset: High-power view of the neoplasm. The tumor is composed of interlacing fascicles of spindle cells with elongated nuclei consistent with a smooth muscle neoplasm.
All five patients had the diagnosis confirmed pathologically. One patient was diagnosed by biopsy and treated with radiation;3 two patients, including ours, underwent resection and vascular reconstruction;4 one patient had endarterectomy and bypass;5 and one patient underwent endartectomy alone.2 Three of the five patients (60%), including ours, were disease-free at the time of their report.2,4 Both of the patients treated by resection and vascular reconstruction survived free of disease recurrence, along with one patient treated by endarterectomy alone. The remaining two patients died of complications of their treatment, one from rupture of the common iliac artery 3 weeks after endarterectomy and femoral-tibial bypass.5 The other also died of a complication, after treatment by biopsy and radiation. These limited data suggest that the optimal surgical treatment associated with both the lowest risk of postoperative morbidity and mortality as well as the highest likelihood of disease-free survival is tumor resection and vascular reconstruction. In the 19 reported cases of leiomyosarcomata of a major peripheral artery, the presenting symptoms included pain, vascular insufficiency, and a mass. Because of the rarity of the disease, the diagnosis is usually not established preoperatively. These tumors may occasionally be confused on arteriogram with pseudoaneurysms, as demonstrated in two patients who were originally treated with arterial bypass without resection and only later proven to have leiomyosarcomata.10,15 Twelve of the 19 patients were treated by resection, usually with
Common iliac Femoral Common iliac Axillary Common iliac Popliteal Popliteal Popliteal Popliteal
F, 60 M, 55 M, 71 M, 55 F, 68 F, 55 M, 65 F, 65 M, 51 M, 70
Hopkins (1968)3 Kevorkian and Cento (1973)1
Birkenstock and Lipper (1976)4 Varela-Duran et al. (1979) 7 Gutman et al. (1986)2 Basu et al. (1988)14 Leeson et al. (1988)12 (patient 1) Lesson et al. (1988)12 (patient 2) Briggs et al. (1990)15
F, 42
Present case (2001)
Superficial femoral Subclavian Common carotid Common iliac
M, 79 M, 51 F, 33
Iliac
F, 72
Delin et al. (1990)5 (patient 2) Meulman and Graham (1991)13 Giangola et al. (1993)10 Mikami et al. (1997)8
F, 37
Femoral Femoral Inferior mesenteric Internal mammary Femoral
M, 66 F, 70 M, 49
Kevorkian and Cento (1973)1 Kevorkian and Cento (1973)1 Stringer (1977)11 (case III) Stringer (1977)11 (case I) Kevorkian and Cento (1973)1
Site
Sex age (years)
Reference
Table I. Leiomyosarcoma of the peripheral arterial system
Pain, pheochromocytomalike symptoms, mass
Pain, mass Pain, mass
Claudication, pain, and toe ulcer Pain, mass
Claudication, mass
Pain, mass
Claudication Pain, mass Pain, mass
Pain, hot flashes, mass
Claudication, mass Mass
Asymptomatic mass on chest X-ray
Pain, mass
Symptoms/size
Debulking, radiation Resection and graft placement Resection and bypass
Endarterectomy, femoral-tibial bypass Resection and bypass
Above-knee amputation
Endarterectomy Above-knee amputation Excision with bypass, radiation Above-knee amputation
Excision and radiation
Tumor debulking, radiation, and chemotherapy Sternotomy with local excision Resection with graft interposition Biopsy and radiation Resection with bypass, radiation Resection and bypass
Treatment
No evidence of disease
Metastasis to brain; died of disease No evidence of disease
Died of disease
Metastasis to lung and lymph nodes; died of disease Died of complication
Metastasis to lung; died of disease
No evidence of disease Metastasis to lung; alive with disease No evidence of disease
Alive at 6-year follow-up
No evidence of disease
Died of complication Metastasis to lung; died of disease
Metastasis to lung; alive with disease
No evidence of disease
Alive with disease; metastasis to bone Metastasis to lung; died of disease
Follow-up
568 Case reports Annals of Vascular Surgery
Vol. 17, No. 5, 2003
reconstruction, and three patients underwent above-knee amputation for popliteal artery leiomyosarcoma. Two patients were treated by endarterectomy, two by incomplete resection or debulking, and one by biopsy alone. Of the 18 patients with leiomyosarcoma of a major peripheral artery for whom follow-up data were reported, 7 (39%) were alive and had no evidence of metastasis at their follow-up interval. Two died of complications of their treatment. Nine patients (50%) developed metastatic disease. The lung was the most common site of metastatic disease, in six patients, with one case each of brain, bone, and lymph node metastasis. For soft tissue sarcomas, size, histological grade, location, and the ability to undergo complete resection are important prognostic factors.9 It is logical to assume that these same prognostic factors (size, grade, location, and resectability) will pertain to leiomyosarcomata of the peripheral arterial system. VanGulik et al. reported that patients with leiomyosarcomata that arise in the retroperitoneum and those with tumors larger than 5 cm have a worse prognosis than patients without these findings.9 In our review, there were eight patients in whom tumor size was reported: five patients with masses >5 cm and three with masses £ 5 cm. Of those patients with masses >5 cm, four out of five patients developed metastases and three of these patients died of their disease within 16 months of initial presentation. Of the patients with masses £ 5 cm, all three remain free of metastasis, which is consistent with the prognostic significance of size for soft tissue sarcomas of other locations. For leiomyosarcoma, complete surgical resection is the cornerstone of optimal treatment. Whenever possible, surgical resection should be used to completely remove the primary tumor intact and en bloc with any directly involved adjacent structures that can be sacrificed. Ideally, a margin of uninvolved normal tissue would be resected with the tumor, given the high incidence of local recurrence when lesser resections are performed. The necessary disruption of the arterial system will usually require that vascular reconstruction be performed after resection. The location of the primary tumor and its extent will dictate the type and complexity of the reconstruction required to reestablish arterial flow. Since involvement of the iliac vein in our case would have significantly complicated both the resection and the reconstruction, a venogram was obtained preoperatively. In many cases, however, a CT scan and MRI will suffice. A retroperitoneal approach was chosen to avoid the peritoneal cavity and lessen postoperative ileus, and because of the
Case reports 569
relatively small size of the retroperitoneal mass. For larger tumors, a transabdominal approach using a long midline incision may be preferable. A ureteral stent, although not used in this case, can be valuable, especially in patients who have undergone previous surgery or radiation, and if the tumor cannot be separated from the ureter on preoperative imaging. Preoperative embolization for iliac artery tumors would not be recommended without a histologic diagnosis, may carry excessive risk, and was not considered in this case. The role of adjuvant radiation therapy, either pre- or postoperatively, in decreasing the rates of local recurrence of soft tissue sarcomas is well established. While high doses are required, local recurrence rates can be significantly reduced with the addition of radiation therapy. As these tumors are rarely diagnosed preoperatively, adjuvant radiation therapy will generally be considered postoperatively. Given the proximity of the major peripheral arteries to adjacent veins, nerves, and bones, tumors arising from the peripheral arterial system will rarely be amenable to very wide surgical resection with generous margins, and therefore radiation therapy should often be considered to lower the rate of local recurrence. Effective systemic chemotherapy for the treatment of leiomyosarcomata of soft tissues in an adjuvant setting is, at best, controversial. Toxicity is significant, and systemic chemotherapy would only be considered for large high-grade tumors, recurrent tumors, and metastatic disease. Appropriate followup for these tumors should monitor for local recurrence (with CT or MRI every 3 to 4 months) and for pulmonary metastasis (with CT of the chest every 4 to 6 months) during the first 5 years postoperatively. In summary, leiomyosarcoma arising from a major peripheral artery is fortunately quite rare, with only 19 cases now reported. When such tumors occur, they usually present with vascular insufficiency, pain, or a mass. Optimal treatment involves complete surgical resection and vascular reconstruction, often followed by radiation therapy. Prognosis will depend on the size of the tumor, the grade, and the completeness of surgical resection possible. Overall, recurrent or metastatic disease has developed in 50% of cases, with 39% of patients alive and free of disease.
REFERENCES 1. Kevorkian J, Cento DP. Leiomyosarcoma of large arteries and veins. Surgery 1973;73:390-400. 2. Gutman H, Haddad M, Zelikovski A, Mor C, Reiss R. Primary leiomyosarcoma of the right common iliac artery—a rare
570 Case reports
3.
4. 5.
6.
7.
8.
finding and cause of occlusive vascular disorder. J Surg Oncol 1986;32:193-195. Hopkins GB. Leriche syndrome associated with leiomyosarcoma of the right common iliac artery. JAMA 1968; 18:1789-1790. Birkenstock WE, Lipper S. Leiomyosarcoma of the right common iliac artery: a case report. Br J Surg 1976;63:81-82. Delin A, Johansson G, Silfversward C. Vascular tumors in occlusive disease of the iliac-femoral vessels. Eur J Vasc Surg 1990;4:539-542. Wright EP, Glick AD, Virmani R, Page DL. Aortic intimal sarcoma with embolic metastases. Am J Surg Pathol 1985; 9:890-897. Varela-Duran J, Oliva H, Rosai J. Vascular leiomyosarcoma: the malignant counterpart of vascular leiomyoma. Cancer 1979;44:1684-1691. Mikami Y, Manabe T, Lie JT, Sakurai T, Endo K. Intramural sarcoma of the carotid artery with adventitial inflammation and fibrosis resembling ‘‘inflammatory aneurysm’’. Pathol Int 1997;47:569-574.
Annals of Vascular Surgery
9. vanGulik TM, Taat CW, Regroort M, Knomhout JG, Thunnissen FB. Vascular leiomyosarcoma—a report of two cases [letter]. Neth J Surg 1987;39:72-73. 10. Giangola G, Migaly J, Crawford B, Moskowitz P, Sebenick M. Leiomyosarcoma of the subclavian artery. J Vasc Surg 1995;22:496-500. 11. Stringer BD. Leiomyosarcoma of artery and vein. Am J Surg 1977;134:90-94. 12. Leeson MC, Malaei M, Makley JT. Leiomyosarcoma of the popliteal artery: a report of two cases. Clin Orthop 1990; 253:225-230. 13. Meulman NB, Graham JC. Leiomyosarcoma of the superficial femoral artery presenting as a ruptured aneurysm. Aust N Z J Surg 1991;61:853-855. 14. Basu SK, Scott TD, Wilmshurst CC, MacEachern AG, Clyne CA. Leiomyosarcomata of the popliteal vessels: rare primary tumors. Eur J Vasc Surg 1988;2:423-425. 15. Briggs PJ, Pooley J, Malcolm AJ, Chamberlain J. Popliteal artery leiomyosarcoma: a case report and review of the literature. Ann Vasc Surg 1990;4:365-369.
We describe a case of primary intramural, extraluminal leiomyosarcoma of the common iliac artery. The patient presented with pelvic and leg pain and pheochromocytoma-like symptoms. The diagnosis was made intraoperatively, the tumor was resected, vascular reconstruction was performed, and postoperative radiation therapy was administered. Primary leiomyosarcoma of a major peripheral artery is extremely rare, and this report examines the clinical presentation, treatment, and prognosis of these patients.
Although leiomyosarcomata of the central veins and pulmonary arteries have been widely reported, similar tumors of the aorta are much less common, and those arising from the peripheral arterial system are extremely rare.1 We present a case of a leiomyosarcoma of the left common iliac artery treated by surgical resection, vascular reconstruction, and postoperative radiation therapy, and we review the limited published experience with these tumors.
CASE REPORT A 42-year-old woman presented with a 1-year history of intermittent, severe, left groin pain with associated low back and buttock pain that radiated down her left anterior thigh. She described the pain as intermittently excruciating, deep in her pelvis, and worsening over time. Department of Surgery, Abington Memorial Hospital, Abington, PA, USA. Correspondence to: Theodore R. Sullivan, Jr., MD, Vascular Center of Excellence, Abington Memorial Hospital, 1200 Old York Road, Abington, PA 19001, USA, E-mail: [email protected] Ann Vasc Surg 2003; 17: 565-570 DOI: 10.1007/s10016-003-0038-6 Annals of Vascular Surgery Inc. Published online: 29 September 2003
The patient also reported episodic panic attacks, cold sweats, and episodes of diarrhea and constipation over this same time period. Her medical history included hypertension, hypothyroidism, lumbar disc herniation, a prior hysterectomy, and rotator cuff surgery. She had no family history of cancer or premature atherosclerosis. The patient denied exertional lower extremity symptoms, and review of systems was otherwise unremarkable. On physical examination, the patient was 5 feet, 11 inches tall and weighed 298 lbs. She was in no acute distress. Her blood pressure was 160/90 and symmetrical in both arms, with a heart rate of 70 and regular. There were no carotid bruits or jugular venous distention and her cardiopulmonary examination was normal. There were no abdominal masses, bruits, or inguinal adenopathy. Normal pulses were noted in both upper and lower extremities, and no evidence of lower extremity edema or venous congestion was detected. Laboratory values, including CBC, electrolytes, liver function tests, urine metanephrines, and catecholamines, were all within normal ranges. Her chest X-ray was normal. A computed tomography (CT) scan of the abdomen and pelvis showed a 6 · 5 · 3.5 cm soft tissue mass in the left lower retroperineum. The mass began just caudad to the aortic bifurcation and extended inferiorly along the left psoas muscle, inseparable along its medial border from the left common iliac artery (Fig. 1). The liver appeared normal, and there was no other evidence of
565
566 Case reports
Fig. 1. CT scan of the abdomen/pelvis with IV contrast showing a 6 · 5 · 3.5 cm soft tissue mass (arrow) in the left lower retroperitoneum extending along the left psoas muscle inseparable along its medial border from the left common iliac artery. lymphadenopathy in the para-aortic or right iliac regions. Magnetic resonance imaging (MRI) of the abdomen and pelvis demonstrated this same contrast-enhancing retroperitoneal mass enveloping the left common iliac artery with some medial displacement of the artery (Fig. 2). An aortogram demonstrated medial bowing of the left common and external iliac arteries, consistent with extrinsic compression from the adjacent hypervascular mass (Fig. 3). A left femoral venogram revealed extrinsic compression of the left common iliac vein with no evidence of invasion or thrombus. A octreotide-labeled nuclear medicine scan demonstrated a marked increase in somatostatin receptor activity corresponding to this pelvic mass, highly suggestive of a neuroendocrine tumor. The differential diagnosis preoperatively included (1) a neuroendocrine tumor, (2) a retroperitoneal sarcoma, (3) lymphadenopathy from lymphoma or metastatic disease, and (4) a primary vascular tumor. After abnormal catecholamine production was excluded, surgical resection for diagnosis and treatment was recommended. At surgical exploration through a left retroperitoneal approach, a smooth, well-circumscribed, firm mass was found enveloping the left common iliac artery and abutting, but not invading, the left common iliac vein. The left ureter was identified anterior and lateral to the mass, was not directly involved with the tumor, and was preserved. The tumor was inseparable from the wall of the left common iliac artery and appeared grossly to arise from that vessel. After obtaining proximal and distal vascular control of the left common, external, and internal iliac arteries, and after systemic heparinization, the tumor was resected en bloc with the common iliac artery leaving a 1-cm cuff of proximal artery. A 10-mm Dacron interposition graft was placed between the proximal left common iliac artery and both the external and internal iliac arteries to revascularize the left leg. Blood loss during the procedure was 700 cc, and the patient tolerated
Annals of Vascular Surgery
Fig. 2. Coronal section of an out-of-phase T2-weighted MRI of the abdomen/pelvis with contrast demonstrating contrast-enhancing mass intimately involved with the left common iliac artery.
the procedure well. Pulses in the left lower extremity were normal at the conclusion of the procedure. The gross specimen consisted of a 6-cm segment of the left common iliac artery with an enveloping ovoid, rubbery, pink/tan, fibrous mass measuring 6.5 · 4.5 · 4 cm. The mass had a swirling, dusty pink/tan parenchyma with central areas of hemorrhage. The tumor consisted of spindle cells with eosinophilic cytoplasm growing in interlacing bundles suggestive of smooth muscle differentiation (Fig. 4). No significant atypia was seen. Immunohistochemical stains for actin and desmin were positive, confirming the smooth muscle origin of the tumor. Mitotic activity varied with a maximum of 2 mitoses per 10 high-power fields. Focal necrosis was seen. The mass extended focally to the inked surgical resection margin. The final pathologic diagnosis was a low-grade leiomyosarcoma arising from the left common iliac artery. The postoperative recovery was uneventful, and the patient was discharged to home with a patent vascular graft on hospital day 4. Her preoperative pain was relieved completely. Because of the positive microscopic surgical margin, external beam irradiation was delivered, beginning at 4 weeks postoperatively, with anterior and posterior irradiation fields measuring 10 · 11 cm. The treatments were delivered to a dose of 4500 cGy in 25 fractions over 36 days. At 15-month follow-up, the patient is doing well, is back to work full-time, and is without pain. MRI at 1 year postoperatively demonstrated no evidence of recurrence or metastatic disease.
DISCUSSION In the vascular system, leiomyosarcomata arise from veins five times more commonly than from
Vol. 17, No. 5, 2003
Fig. 3. Aortogram showing extrinsic compression of the left common and external iliac arteries from an adjacent hypervascular mass (arrow).
the arteries. The most common site of occurrence is the inferior vena cava and comprises about half of all of the reported cases.1 Of those arising from arteries, the pulmonary artery is the site of origin in twice as many cases as the large systemic arteries.2 Few cases of leiomysarcomata of the major peripheral arteries, excluding those that involve the aorta, have been reported.1-15 Review of the published literature identified 18 previously reported cases of nonaortic leiomyosarcoma of a major peripheral artery. Including the present case, there are 9 women and 10 men have been discussed (Table I), suggesting no gender predilection. These 19 patients ranged in age from 33 to 79 years, with an average age of 58.6 years. Only four previous reports of a primary leiomyosarcoma tumor arising specifically from the common iliac artery have been described.2-5 Of the five cases of leiomyosarcomata arising from the iliac artery that have now been reported2-5 three of these five patients presented with symptoms of claudication.2,3,5 Our patient had no history of claudication and had a normal vascular examination prior to undergoing resection. Claudication will result when the tumor grows intraluminally or when tumor growth otherwise obstructs flow through compression. In the present case, the tumor growth was outward from the wall of the vessel encasing the artery, but antegrade flow was preserved.
Case reports 567
Fig. 4. Low-power view of smooth muscle neoplasm with the adjacent artery. The neoplastic spindle cells blend imperceptibly with the muscular wall of the artery seen in the upper left. Inset: High-power view of the neoplasm. The tumor is composed of interlacing fascicles of spindle cells with elongated nuclei consistent with a smooth muscle neoplasm.
All five patients had the diagnosis confirmed pathologically. One patient was diagnosed by biopsy and treated with radiation;3 two patients, including ours, underwent resection and vascular reconstruction;4 one patient had endarterectomy and bypass;5 and one patient underwent endartectomy alone.2 Three of the five patients (60%), including ours, were disease-free at the time of their report.2,4 Both of the patients treated by resection and vascular reconstruction survived free of disease recurrence, along with one patient treated by endarterectomy alone. The remaining two patients died of complications of their treatment, one from rupture of the common iliac artery 3 weeks after endarterectomy and femoral-tibial bypass.5 The other also died of a complication, after treatment by biopsy and radiation. These limited data suggest that the optimal surgical treatment associated with both the lowest risk of postoperative morbidity and mortality as well as the highest likelihood of disease-free survival is tumor resection and vascular reconstruction. In the 19 reported cases of leiomyosarcomata of a major peripheral artery, the presenting symptoms included pain, vascular insufficiency, and a mass. Because of the rarity of the disease, the diagnosis is usually not established preoperatively. These tumors may occasionally be confused on arteriogram with pseudoaneurysms, as demonstrated in two patients who were originally treated with arterial bypass without resection and only later proven to have leiomyosarcomata.10,15 Twelve of the 19 patients were treated by resection, usually with
Common iliac Femoral Common iliac Axillary Common iliac Popliteal Popliteal Popliteal Popliteal
F, 60 M, 55 M, 71 M, 55 F, 68 F, 55 M, 65 F, 65 M, 51 M, 70
Hopkins (1968)3 Kevorkian and Cento (1973)1
Birkenstock and Lipper (1976)4 Varela-Duran et al. (1979) 7 Gutman et al. (1986)2 Basu et al. (1988)14 Leeson et al. (1988)12 (patient 1) Lesson et al. (1988)12 (patient 2) Briggs et al. (1990)15
F, 42
Present case (2001)
Superficial femoral Subclavian Common carotid Common iliac
M, 79 M, 51 F, 33
Iliac
F, 72
Delin et al. (1990)5 (patient 2) Meulman and Graham (1991)13 Giangola et al. (1993)10 Mikami et al. (1997)8
F, 37
Femoral Femoral Inferior mesenteric Internal mammary Femoral
M, 66 F, 70 M, 49
Kevorkian and Cento (1973)1 Kevorkian and Cento (1973)1 Stringer (1977)11 (case III) Stringer (1977)11 (case I) Kevorkian and Cento (1973)1
Site
Sex age (years)
Reference
Table I. Leiomyosarcoma of the peripheral arterial system
Pain, pheochromocytomalike symptoms, mass
Pain, mass Pain, mass
Claudication, pain, and toe ulcer Pain, mass
Claudication, mass
Pain, mass
Claudication Pain, mass Pain, mass
Pain, hot flashes, mass
Claudication, mass Mass
Asymptomatic mass on chest X-ray
Pain, mass
Symptoms/size
Debulking, radiation Resection and graft placement Resection and bypass
Endarterectomy, femoral-tibial bypass Resection and bypass
Above-knee amputation
Endarterectomy Above-knee amputation Excision with bypass, radiation Above-knee amputation
Excision and radiation
Tumor debulking, radiation, and chemotherapy Sternotomy with local excision Resection with graft interposition Biopsy and radiation Resection with bypass, radiation Resection and bypass
Treatment
No evidence of disease
Metastasis to brain; died of disease No evidence of disease
Died of disease
Metastasis to lung and lymph nodes; died of disease Died of complication
Metastasis to lung; died of disease
No evidence of disease Metastasis to lung; alive with disease No evidence of disease
Alive at 6-year follow-up
No evidence of disease
Died of complication Metastasis to lung; died of disease
Metastasis to lung; alive with disease
No evidence of disease
Alive with disease; metastasis to bone Metastasis to lung; died of disease
Follow-up
568 Case reports Annals of Vascular Surgery
Vol. 17, No. 5, 2003
reconstruction, and three patients underwent above-knee amputation for popliteal artery leiomyosarcoma. Two patients were treated by endarterectomy, two by incomplete resection or debulking, and one by biopsy alone. Of the 18 patients with leiomyosarcoma of a major peripheral artery for whom follow-up data were reported, 7 (39%) were alive and had no evidence of metastasis at their follow-up interval. Two died of complications of their treatment. Nine patients (50%) developed metastatic disease. The lung was the most common site of metastatic disease, in six patients, with one case each of brain, bone, and lymph node metastasis. For soft tissue sarcomas, size, histological grade, location, and the ability to undergo complete resection are important prognostic factors.9 It is logical to assume that these same prognostic factors (size, grade, location, and resectability) will pertain to leiomyosarcomata of the peripheral arterial system. VanGulik et al. reported that patients with leiomyosarcomata that arise in the retroperitoneum and those with tumors larger than 5 cm have a worse prognosis than patients without these findings.9 In our review, there were eight patients in whom tumor size was reported: five patients with masses >5 cm and three with masses £ 5 cm. Of those patients with masses >5 cm, four out of five patients developed metastases and three of these patients died of their disease within 16 months of initial presentation. Of the patients with masses £ 5 cm, all three remain free of metastasis, which is consistent with the prognostic significance of size for soft tissue sarcomas of other locations. For leiomyosarcoma, complete surgical resection is the cornerstone of optimal treatment. Whenever possible, surgical resection should be used to completely remove the primary tumor intact and en bloc with any directly involved adjacent structures that can be sacrificed. Ideally, a margin of uninvolved normal tissue would be resected with the tumor, given the high incidence of local recurrence when lesser resections are performed. The necessary disruption of the arterial system will usually require that vascular reconstruction be performed after resection. The location of the primary tumor and its extent will dictate the type and complexity of the reconstruction required to reestablish arterial flow. Since involvement of the iliac vein in our case would have significantly complicated both the resection and the reconstruction, a venogram was obtained preoperatively. In many cases, however, a CT scan and MRI will suffice. A retroperitoneal approach was chosen to avoid the peritoneal cavity and lessen postoperative ileus, and because of the
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relatively small size of the retroperitoneal mass. For larger tumors, a transabdominal approach using a long midline incision may be preferable. A ureteral stent, although not used in this case, can be valuable, especially in patients who have undergone previous surgery or radiation, and if the tumor cannot be separated from the ureter on preoperative imaging. Preoperative embolization for iliac artery tumors would not be recommended without a histologic diagnosis, may carry excessive risk, and was not considered in this case. The role of adjuvant radiation therapy, either pre- or postoperatively, in decreasing the rates of local recurrence of soft tissue sarcomas is well established. While high doses are required, local recurrence rates can be significantly reduced with the addition of radiation therapy. As these tumors are rarely diagnosed preoperatively, adjuvant radiation therapy will generally be considered postoperatively. Given the proximity of the major peripheral arteries to adjacent veins, nerves, and bones, tumors arising from the peripheral arterial system will rarely be amenable to very wide surgical resection with generous margins, and therefore radiation therapy should often be considered to lower the rate of local recurrence. Effective systemic chemotherapy for the treatment of leiomyosarcomata of soft tissues in an adjuvant setting is, at best, controversial. Toxicity is significant, and systemic chemotherapy would only be considered for large high-grade tumors, recurrent tumors, and metastatic disease. Appropriate followup for these tumors should monitor for local recurrence (with CT or MRI every 3 to 4 months) and for pulmonary metastasis (with CT of the chest every 4 to 6 months) during the first 5 years postoperatively. In summary, leiomyosarcoma arising from a major peripheral artery is fortunately quite rare, with only 19 cases now reported. When such tumors occur, they usually present with vascular insufficiency, pain, or a mass. Optimal treatment involves complete surgical resection and vascular reconstruction, often followed by radiation therapy. Prognosis will depend on the size of the tumor, the grade, and the completeness of surgical resection possible. Overall, recurrent or metastatic disease has developed in 50% of cases, with 39% of patients alive and free of disease.
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Annals of Vascular Surgery
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