Case Reports
Popliteal Artery Leiomyosarcoma: A Case Report and Review of the Literature P.J. Briggs, BSc, FRCS, J. Pootey, MD, A.J. M a l c o l m , M R C P a t h , J. C h a m b e r l a i n , F R C S , E d , Newcastle upon Tyne, England
The case of a 70-year-old man presenting with calf claudication due to a popliteal artery leiomyosarcoma is described, and previous reports are reviewed. (Ann Vasc Surg 1990; 4:365-369). KEY WORDS:
Popliteal artery; leiomyosarcoma; claudication.
CASE REPORT A 70-year-old man had a 15-month history of left calf claudication with a march tolerance of 200 yards. He was generally fit, a non-smoker, and had no significant medical history. Popliteal and foot pulses were absent from the left leg. The femoral pulses were normal and there were no bruits. Transfemoral aortography was performed, which showed occlusion of the left superficial femoral artery at the level of the adductor canal (Fig. 1). There was no filling of the popliteal artery, Distal vessels were filling through collaterals at the origin of the anterior tibial artery. Arterial reconstruction was not appropriate. He was reviewed three months later when he complained of deteriorating exercise tolerance, and the onset of rest pain in the left foot. Seven weeks before he had noted a rapidly enlarging swelling behind his left knee. He had a smooth, firm, immobile and non- pulsatile mass occupying the popliteal fossa. There was ischemic rubor in the foot and the leg was edematous below the knee. There was no palpable lymphadenopathy. The initial impression that he had developed a popliteal cyst was not supported by further investigations. Routine
blood tests and chest films were normal. An ultrasound scan of the popliteal fossa revealed a solid, lobulated mass around and lateral to the popliteal vessels. There was no evidence of pulsation within the mass. A computed tomographic (CT) scan confirmed the ultrasound findings, showing a homogeneous soft tissue mass occupying the popliteal fossa and lying deep to the gastrocnemius and soleus interiorly (Fig. 2). Adjacent fat planes were obliterated, but there was no evidence of bone destruction. The clinical suspicion of a malignant tumor was confirmed by an incisional biopsy which showed the features of a leiomyosarcoma. A left mid-thigh amputation was therefore performed. Three months later he developed a swelling in the left inguinal region which was excised. Histology showed similar appearances to the tumor from behind the knee. Lung metastases became evident on further chest films. The patient died 10 months after his initial operation. A postmortem examination was declined.
PATHOLOGY T h e a m p u t a t i o n s p e c i m e n w a s d i s s e c t e d (Fig. 3). T h e r e w a s a I0 x 7 x 6 c m m a s s c e n t e r e d in t h e popliteal fossa around the artery, displacing the tibial n e r v e . T h e r e w a s a s e c o n d n o d u l e m e a s u r i n g 1.5 x 1 x l c m s i t u a t e d on t h e p o p l i t e a l a r t e r y 7 c m p r o x i m a l to the l a r g e r l e s i o n . T h e a r t e r y w a s infiltrated and occluded by the tumor. The popliteal
From the Departments of General Surgel~, and Orthopaedic Surgery, Freeman Hospital, Newcastle upon Tyne, and the Department of Pathology, Royal Victoria Infirmary, Newcastle upon Tvne, England. Reprint requests: Mr. P. J. Briggs, Department of Orthopaedic Surgery, F'reeman Hospital, Newcastle upon Tyne, NE7 7DN, England. 365
366
ANNALS OF V ASCU LAR SURGERY
POPLITEA L A R TER Y LEIOM YOSARCOMA
spindle cell sarcoma with the cells arranged in curving bundles (Fig. 4a). The tumor was intimately associated with the arterial smooth muscle (Fig. 4b). There was little collagen and the tumor reacted positively with antibodies to actin and smooth muscle myosin. Electron microscopy showed poor cellto-cell junctions, dilated rough endoplasmic reticulum, and focal areas of filamentous material in the cytoplasm. All these features confirmed the diagnosis of a high-grade leiomyosarcoma.
DISCUSSION
Fig. 1. Arteriogram showing occlusion of left superficial femoral artery at level of adductor canal.
vein was compressed and distorted by the tumor but was not directly invaded by it. The main vein, although narrowed, was not occluded, but some of its tributaries were occluded by thrombus, Microscopy showed a high grade pleomorphic
Fig. 2. CT scan of knees showing homogeneous mass occupying left popliteal fossa.
Leiomyosarcomata arising from large arteries are rare. Eight cases were reviewed by Kevorkian and Cento [I], but three were considered to be of doubtful validity. Including our patient, a further eight have been described [2-6], giving a total of 13 definite cases. It is the least common variety of vascular leiomyosarcoma; those arising from the large veins are five times as common, and those from the pulmonary artery twice as common [I]. The age range of the reported cases is 37 to 77 years. There were eight men and five women. Their clinical details are summarized in Table I. Arterial occlusion was frequently seen among patients; four had claudication. Another four complained of abdominal pains mimicking visceral disease. However, only when a mass became evident clinically, as in our case or at laparotomy, was the true diagnosis made. Four other patients had a swelling, and another was discovered incidentally on a chest film. Six patients died after periods ranging from three weeks to 14 years after the diagnosis had been made. Where recorded, the length of history was longer than 18 months. All had extensive local spread and two were considered to be inoperable when seen initially. The other four patients in this group developed local recurrence or metastatic disease at times between three months and seven years postoperatively. Seven patients were alive at the time of reporting, four apparently tumor-free six months to 14 years postresection. Three had evidence of metastatic disease. The length of history was relatively shorter, although Varela-Duran and associates [5] described a patient with symptoms for two years prior to diagnosis. This patient developed local recurrence of a grade II tumor which was treated by further excision and radiotherapy. The patient remains well five years later. Local spread which involved structures such as muscle, vein, or bowel occurred in seven of the patients. Blood-borne spread occurred to the lungs in five patients, and to bone in two. It is interesting to note that lymph node spread occurred in our patient, an unusual occurrence in leiomyosarcoma
POPLITEAL ARTERY LEIOMYOSARCOMA
VOLUME 4 N o 4 - 1990
TA -
TUMOUR A
367
S -- S C I A T I C N E R V E
T B -- T U M O U R B
T -
TIBIAL NERVE
P -- P O P L I T E A L A R T E R Y
C -
COMMON PERONEAL NERVE
Fig. 3. D i s s e c t e d a m p u t a t i o n s p e c i m e n s h o w i n g two t u m o r s (TA and TB) arising from popliteal artery (P). Sciatic nerve (S), tibial nerve (T), a n d c o m m o n peroneal (C).
not previously described in those arising from arteries. Since this tumor arises in large arteries, intraarterial metastatic deposits seem more likely to occur distally. However, this occurrence has not been described. In our patient two separate tumors were in fact present in the popliteal artery, but the larger tumor was distally placed and seemed unlikely to be a metastasis from the proximal tumor. Both tumors could of course have been metastatic, but investigations did not reveal evidence this to be so. Delay in diagnosis would seem to be a factor in the high mortality rate associated with this condition. Considering its rarity the delay is perhaps not surprising. In our patient, the diagnosis was not considered until the tumor became palpable. Arteriography had shown no evidence of tumor circula-
tion, a typical feature of soft tissue leiomyosarcomata of the extremities [7]. The development of duplex ultrasound scanning as a technique for the investigation of peripheral vascular disease may detect such unusual causes of claudication earlier. These tumors, where recorded, appear to be histologically aggressive, usually grade II or III. Histological grade is an important prognostic indicator in arterial [5] and other soft tissue leiomyosarcomata [8]. Neither the site of origin of the tumor nor its mode of presentation appear to be related to patient survival. Surgical resection offers the best chance of cure. Although the number of reported cases is small, patient survival appears comparable with the 32% five-year survival of patients with other soft tissue leiomyosarcomata [8]. Earlier diagnosis may improve this.
1913 1925
1967
1968
1973
1976
1977
1977
1977
1979
1979
1986
1989
Ferrarini [1] Ajello [1]
Sadlinski [1]
Hopkins [1]
Kevorkian [1 ] Patient 2
Birkenstock [2]
Stringer [3] Patient 1
Stringer [3] Patient 3
Treiman's [3] patient
Hernandez [4]
Varela-Duran
Gutman [6]
Our patient
[5]
Date
Author
70
55
68
42
77
49
37
75
71
55
60
66 70
Age
M
F
F
M
M
M
F
M
M
M
F
F
Sex M
Common lilac Popliteal
Axillary
Aorta
Popliteal
Inferior mesenteric
Common iliac Internal mammary
Femoral
Common lilac
Femoral
Femoral Femoral
Arterial site
Claudication
Claudication
Swelling + cubital tunnel syndrome
Abdominal pains
Claudication
Asymptomatic shadow on Chest X-ray Abdominal pains
Abdominal pains
Swelling
Claudication
Swelling
Swelling
Symptoms
18 months
9 months
2 years
Several years
5 months
2 years
3 years
2 years
Length of history
Lymph node Lung
spine
Lung.&
Local
Bowel, ureter + vessels
Vein
Muscle Lung
Ureter
Lung
Femur
Spread
TABLE I.--Summary of reported cases
Amputation
Resection radiation therapy + chemotherapy at 7 years Resection and radiation therapy Radiation + chemotherapy Resection, further excision, radiation therapy at 10 months Endarterectomy
Resection
Excision Further excision, radiation therapy at 11 months Resection
Laparotomy and biopsy
Resection
Treatment
Ill
II
II
I
III
II or III
Histological grade
Alive and well at 1 year postop Died at 10 months postop
Died at 2 years postop Alive and well at 6 years postop
Died at 7 months postop
Died at 14 years postop
Alive and well 6 mos postop Alive and well at 14 years postop
Aortic thrombosis Died 16 months after primary treatment
Metastases to femur 10 months postresection Alive at 1 year after treatment Died 3 weeks postlaparotomy
Outcome
>
Popliteal Artery Leiomyosarcoma: A Case Report and Review of the Literature P.J. Briggs, BSc, FRCS, J. Pootey, MD, A.J. M a l c o l m , M R C P a t h , J. C h a m b e r l a i n , F R C S , E d , Newcastle upon Tyne, England
The case of a 70-year-old man presenting with calf claudication due to a popliteal artery leiomyosarcoma is described, and previous reports are reviewed. (Ann Vasc Surg 1990; 4:365-369). KEY WORDS:
Popliteal artery; leiomyosarcoma; claudication.
CASE REPORT A 70-year-old man had a 15-month history of left calf claudication with a march tolerance of 200 yards. He was generally fit, a non-smoker, and had no significant medical history. Popliteal and foot pulses were absent from the left leg. The femoral pulses were normal and there were no bruits. Transfemoral aortography was performed, which showed occlusion of the left superficial femoral artery at the level of the adductor canal (Fig. 1). There was no filling of the popliteal artery, Distal vessels were filling through collaterals at the origin of the anterior tibial artery. Arterial reconstruction was not appropriate. He was reviewed three months later when he complained of deteriorating exercise tolerance, and the onset of rest pain in the left foot. Seven weeks before he had noted a rapidly enlarging swelling behind his left knee. He had a smooth, firm, immobile and non- pulsatile mass occupying the popliteal fossa. There was ischemic rubor in the foot and the leg was edematous below the knee. There was no palpable lymphadenopathy. The initial impression that he had developed a popliteal cyst was not supported by further investigations. Routine
blood tests and chest films were normal. An ultrasound scan of the popliteal fossa revealed a solid, lobulated mass around and lateral to the popliteal vessels. There was no evidence of pulsation within the mass. A computed tomographic (CT) scan confirmed the ultrasound findings, showing a homogeneous soft tissue mass occupying the popliteal fossa and lying deep to the gastrocnemius and soleus interiorly (Fig. 2). Adjacent fat planes were obliterated, but there was no evidence of bone destruction. The clinical suspicion of a malignant tumor was confirmed by an incisional biopsy which showed the features of a leiomyosarcoma. A left mid-thigh amputation was therefore performed. Three months later he developed a swelling in the left inguinal region which was excised. Histology showed similar appearances to the tumor from behind the knee. Lung metastases became evident on further chest films. The patient died 10 months after his initial operation. A postmortem examination was declined.
PATHOLOGY T h e a m p u t a t i o n s p e c i m e n w a s d i s s e c t e d (Fig. 3). T h e r e w a s a I0 x 7 x 6 c m m a s s c e n t e r e d in t h e popliteal fossa around the artery, displacing the tibial n e r v e . T h e r e w a s a s e c o n d n o d u l e m e a s u r i n g 1.5 x 1 x l c m s i t u a t e d on t h e p o p l i t e a l a r t e r y 7 c m p r o x i m a l to the l a r g e r l e s i o n . T h e a r t e r y w a s infiltrated and occluded by the tumor. The popliteal
From the Departments of General Surgel~, and Orthopaedic Surgery, Freeman Hospital, Newcastle upon Tyne, and the Department of Pathology, Royal Victoria Infirmary, Newcastle upon Tvne, England. Reprint requests: Mr. P. J. Briggs, Department of Orthopaedic Surgery, F'reeman Hospital, Newcastle upon Tyne, NE7 7DN, England. 365
366
ANNALS OF V ASCU LAR SURGERY
POPLITEA L A R TER Y LEIOM YOSARCOMA
spindle cell sarcoma with the cells arranged in curving bundles (Fig. 4a). The tumor was intimately associated with the arterial smooth muscle (Fig. 4b). There was little collagen and the tumor reacted positively with antibodies to actin and smooth muscle myosin. Electron microscopy showed poor cellto-cell junctions, dilated rough endoplasmic reticulum, and focal areas of filamentous material in the cytoplasm. All these features confirmed the diagnosis of a high-grade leiomyosarcoma.
DISCUSSION
Fig. 1. Arteriogram showing occlusion of left superficial femoral artery at level of adductor canal.
vein was compressed and distorted by the tumor but was not directly invaded by it. The main vein, although narrowed, was not occluded, but some of its tributaries were occluded by thrombus, Microscopy showed a high grade pleomorphic
Fig. 2. CT scan of knees showing homogeneous mass occupying left popliteal fossa.
Leiomyosarcomata arising from large arteries are rare. Eight cases were reviewed by Kevorkian and Cento [I], but three were considered to be of doubtful validity. Including our patient, a further eight have been described [2-6], giving a total of 13 definite cases. It is the least common variety of vascular leiomyosarcoma; those arising from the large veins are five times as common, and those from the pulmonary artery twice as common [I]. The age range of the reported cases is 37 to 77 years. There were eight men and five women. Their clinical details are summarized in Table I. Arterial occlusion was frequently seen among patients; four had claudication. Another four complained of abdominal pains mimicking visceral disease. However, only when a mass became evident clinically, as in our case or at laparotomy, was the true diagnosis made. Four other patients had a swelling, and another was discovered incidentally on a chest film. Six patients died after periods ranging from three weeks to 14 years after the diagnosis had been made. Where recorded, the length of history was longer than 18 months. All had extensive local spread and two were considered to be inoperable when seen initially. The other four patients in this group developed local recurrence or metastatic disease at times between three months and seven years postoperatively. Seven patients were alive at the time of reporting, four apparently tumor-free six months to 14 years postresection. Three had evidence of metastatic disease. The length of history was relatively shorter, although Varela-Duran and associates [5] described a patient with symptoms for two years prior to diagnosis. This patient developed local recurrence of a grade II tumor which was treated by further excision and radiotherapy. The patient remains well five years later. Local spread which involved structures such as muscle, vein, or bowel occurred in seven of the patients. Blood-borne spread occurred to the lungs in five patients, and to bone in two. It is interesting to note that lymph node spread occurred in our patient, an unusual occurrence in leiomyosarcoma
POPLITEAL ARTERY LEIOMYOSARCOMA
VOLUME 4 N o 4 - 1990
TA -
TUMOUR A
367
S -- S C I A T I C N E R V E
T B -- T U M O U R B
T -
TIBIAL NERVE
P -- P O P L I T E A L A R T E R Y
C -
COMMON PERONEAL NERVE
Fig. 3. D i s s e c t e d a m p u t a t i o n s p e c i m e n s h o w i n g two t u m o r s (TA and TB) arising from popliteal artery (P). Sciatic nerve (S), tibial nerve (T), a n d c o m m o n peroneal (C).
not previously described in those arising from arteries. Since this tumor arises in large arteries, intraarterial metastatic deposits seem more likely to occur distally. However, this occurrence has not been described. In our patient two separate tumors were in fact present in the popliteal artery, but the larger tumor was distally placed and seemed unlikely to be a metastasis from the proximal tumor. Both tumors could of course have been metastatic, but investigations did not reveal evidence this to be so. Delay in diagnosis would seem to be a factor in the high mortality rate associated with this condition. Considering its rarity the delay is perhaps not surprising. In our patient, the diagnosis was not considered until the tumor became palpable. Arteriography had shown no evidence of tumor circula-
tion, a typical feature of soft tissue leiomyosarcomata of the extremities [7]. The development of duplex ultrasound scanning as a technique for the investigation of peripheral vascular disease may detect such unusual causes of claudication earlier. These tumors, where recorded, appear to be histologically aggressive, usually grade II or III. Histological grade is an important prognostic indicator in arterial [5] and other soft tissue leiomyosarcomata [8]. Neither the site of origin of the tumor nor its mode of presentation appear to be related to patient survival. Surgical resection offers the best chance of cure. Although the number of reported cases is small, patient survival appears comparable with the 32% five-year survival of patients with other soft tissue leiomyosarcomata [8]. Earlier diagnosis may improve this.
1913 1925
1967
1968
1973
1976
1977
1977
1977
1979
1979
1986
1989
Ferrarini [1] Ajello [1]
Sadlinski [1]
Hopkins [1]
Kevorkian [1 ] Patient 2
Birkenstock [2]
Stringer [3] Patient 1
Stringer [3] Patient 3
Treiman's [3] patient
Hernandez [4]
Varela-Duran
Gutman [6]
Our patient
[5]
Date
Author
70
55
68
42
77
49
37
75
71
55
60
66 70
Age
M
F
F
M
M
M
F
M
M
M
F
F
Sex M
Common lilac Popliteal
Axillary
Aorta
Popliteal
Inferior mesenteric
Common iliac Internal mammary
Femoral
Common lilac
Femoral
Femoral Femoral
Arterial site
Claudication
Claudication
Swelling + cubital tunnel syndrome
Abdominal pains
Claudication
Asymptomatic shadow on Chest X-ray Abdominal pains
Abdominal pains
Swelling
Claudication
Swelling
Swelling
Symptoms
18 months
9 months
2 years
Several years
5 months
2 years
3 years
2 years
Length of history
Lymph node Lung
spine
Lung.&
Local
Bowel, ureter + vessels
Vein
Muscle Lung
Ureter
Lung
Femur
Spread
TABLE I.--Summary of reported cases
Amputation
Resection radiation therapy + chemotherapy at 7 years Resection and radiation therapy Radiation + chemotherapy Resection, further excision, radiation therapy at 10 months Endarterectomy
Resection
Excision Further excision, radiation therapy at 11 months Resection
Laparotomy and biopsy
Resection
Treatment
Ill
II
II
I
III
II or III
Histological grade
Alive and well at 1 year postop Died at 10 months postop
Died at 2 years postop Alive and well at 6 years postop
Died at 7 months postop
Died at 14 years postop
Alive and well 6 mos postop Alive and well at 14 years postop
Aortic thrombosis Died 16 months after primary treatment
Metastases to femur 10 months postresection Alive at 1 year after treatment Died 3 weeks postlaparotomy
Outcome
>
