52 patients were reviewed with a median age of 59 years (range 23-89 years),
details of which are summarised in table 1.
The thigh (37%) was the commonest primary STS location,
myxofibrosarcoma (Fig 2 and 3) and spindle sarcoma were the commonest histological subtypes (14% both groups respectively; table 2).
Tumour site was available for all tumour types with the thigh the commonest overall (37%).
Tumour grade was available for 32 patients (60%),
of these,
68% (21/32) had grade 3 tumours (table 3). 30 involved the right side of the patient,
21 the left,
a single lesion arose from the pelvis.
Lymph node involvement was divided into regional and distant disease with regional nodal infiltration far more common at 10% (5/52) as summarised in table 4. Distant nodal disease was far less frequent (2/52,
4%).
25% (13/52) had metastatic disease,
predominantly involving the lung (92%,
11/12).
70% (9/13) patients with metastatic disease where grading was available,
had grade 3 tumours,
the remaining four were uncharacterised (table 5a).
Additionally,
there were at least four patients with distant metastatic disease which were at multiple sites as summarised by table 5b.
Further comparisons were made relating metastatic disease as demonstrated in table 6-8.
Firstly,
a comparison was made between the primary STS anatomical site of origin and the location of distant metastatic disease (table 6).
It was noted that 70% (9/13) were from a lower limb origin (pelvis,
thigh,
knee and lower leg),
a small fraction 31% (4/13) were from the upper limb with one unusual site including the thumb (the remainder originated from the arm and elbow).
Primary STS histology and the location of distant metastatic disease was also analysed (table 7).
All but one distant metastatic focus was in the lung,
92% (12/13).
The remaining metastatic focus was in an iliac node (1/12).
The most common histology was a myxofibrosarcoma (Figure 2 and 3) 25% (3/12),
followed by MPNST (Figure 4 and 5) 16.6% (2/12).
Primary STS grade,
histology and metastatic disease were assessed (table 8).
Myxofibrosarcoma 25% (3/12) and leiomyosarcoma 16.6% (2/12) were all grade 3 presenting with indeterminate nodules in the lungs.
50%,
(26/52) patients had deep primary lesions (table 9),
42%,
(22/52) superficial primary lesions (table 10),
8%,
(4/52) had involvement of the deep and superficial compartments (table 11).
Table 9 describes patients with deep primary lesions that had skin involvement in 4% (1/26) of cases from a Pleomorphic sarcoma (Figure 6),
muscle involvement (Figure 7) in 46 % (12/26),
bone involvement in 8% (2/26) and distant metastatic disease in 23% (6/26).
Patients with superficial primary lesions (table 10) had skin involvement (Figure 8) in 46% (10/22) of cases,
muscle involvement in 23% (5/22),
bone involvement in 5% (1/22) and metastatic disease in 32% (7/22).
Patients with an STS involving superficial and deep compartments had skin involvement in 25% (1/4) of cases,
muscle (Figure 9) involvement in 50% (2/4) and bone (Figure 10) involvement in 50% (2/4).
There were no distant metastases.
Table 1: Demographics
Total
|
n = 52
|
Gender
|
M
|
F
|
22
|
30
|
Age
|
59
|
Range
|
23-89 (median 56)
|
Table 2: Histological subtypes
Myxofibrosarcoma
|
7
|
Myxofibrosarcoma
|
7
|
Spindle cell sarcoma
|
7
|
Spindle cell sarcoma
|
7
|
MPNST
|
5
|
MPNST
|
5
|
Leiomyosarcoma
|
4
|
Leiomyosarcoma
|
4
|
Myxoid liposarcoma
|
4
|
Myxoid liposarcoma
|
4
|
Pleomorphic sarcoma
|
3
|
Pleomorphic sarcoma
|
3
|
ALT
|
2
|
ALT
|
2
|
Cutaneous leiomyosarcoma
|
2
|
Cutaneous leiomyosarcoma
|
2
|
Pleomorphic liposarcoma
|
1
|
Pleomorphic liposarcoma
|
1
|
ALT sclerosing fibrous type
|
1
|
ALT sclerosing fibrous type
|
1
|
ALT sclerosing tumour
|
1
|
ALT sclerosing tumour
|
1
|
Clear cell sarcoma
|
1
|
Clear cell sarcoma
|
1
|
Desmoid-type fibromatosis
|
1
|
Desmoid-type fibromatosis
|
1
|
Epithelioid haemangioendothelioma
|
1
|
Epithelioid haemangioendothelioma
|
1
|
Epithelioid MPNST
|
1
|
Epithelioid MPNST
|
1
|
Fibrosarcoma
|
1
|
Fibrosarcoma
|
1
|
Table 3: Primary STS site
Thigh
|
19
|
Lower leg
|
9
|
Arm
|
3
|
Foot
|
3
|
Chest wall
|
3
|
Knee
|
2
|
Elbow
|
2
|
Hip
|
2
|
Flank
|
1
|
Ankle
|
1
|
Breast/Chest wall
|
1
|
Pelvis
|
1
|
Popliteal fossa and gluteal region
|
1
|
Proximal thigh/gluteal area
|
1
|
Shoulder
|
1
|
Thumb
|
1
|
Toe
|
1
|
Table 4: Lymph node involvement
Involved lymph nodes
|
|
Yes
|
4
|
No
|
48
|
Regional lymph node metastases
|
|
Yes
|
5 (two multinodular)
|
No
|
47
|
Distant lymph nodes or other metastases
|
|
Yes
|
2 (iliac and axilla + breast lesion)
|
No
|
44
|
N/A: no imaging
|
6
|
Table 5a: Location of distant metastatic disease
Location of distant metastatic disease
|
n = 13
|
Indeterminate lung nodules
|
11
|
Lung metastases
|
1
|
Iliac node
|
1
|
Table 5b: Location of distant metastatic disease at multiple sites
Location of distant metastatic disease at multiple sites
|
n = 4
|
Indeterminate lung nodules + breast/axilla
|
1
|
Indeterminate lung nodules + subpleural mass
|
1
|
Indeterminate lung nodules + neurofibroma
|
1
|
Indeterminate lung nodules + Mediastinal,
supraclavicular and axillary nodes
|
1
|
Table 6: Primary STS location and distant metastatic disease
Anatomic location of primary STS
|
Location distant metastasis
|
Arm
|
Indeterminate lung nodules
|
Arm
|
Indeterminate lung nodules
|
Elbow
|
Indeterminate lung nodules
|
Knee
|
Indeterminate lung nodules
|
Knee
|
Indeterminate lung nodules
|
Lower leg
|
Indeterminate lung nodules
|
Lower leg
|
Lung metastases
|
Lower leg
|
Indeterminate lung nodules
|
Pelvis
|
Indeterminate lung nodules
|
Thigh
|
Iliac node
|
Thigh
|
Indeterminate lung nodules
|
Thigh
|
Indeterminate lung nodules
|
Thumb
|
Indeterminate lung nodules
|
Table 7: Primary STS histology and metastatic site
Histologic type of STS
|
Location distant metastases
|
Epithelioid haemangioendothelioma
|
Iliac node
|
Leiomyosarcoma
|
Indeterminate lung nodules
|
Leiomyosarcoma
|
Indeterminate lung nodules
|
Malignant myoepithilioma
|
Indeterminate lung nodules
|
Mellanocytic tumour
|
Indeterminate lung nodules
|
MPNST
|
Lung metastases
|
MPNST
|
Indeterminate lung nodules
|
Myxofibrosarcoma
|
Indeterminate lung nodules
|
Myxofibrosarcoma
|
Indeterminate lung nodules
|
Myxofibrosarcoma
|
Indeterminate lung nodules
|
Myxoid liposarcoma
|
Indeterminate lung nodules
|
Pleomorphic sarcoma
|
Indeterminate lung nodules
|
Spindle cell sarcoma
|
Indeterminate lung nodules
|
Table 8: Grade,
Histology and Distant metastatic disease comparison
GRADE
|
Histologic type of STT
|
Location distant metastases
|
3
|
Pleomorphic sarcoma
|
Indeterminate lung nodules
|
3
|
Myxofibrosarcoma
|
Indeterminate lung nodules
|
3
|
Leiomyosarcoma
|
Indeterminate lung nodules
|
3
|
Leiomyosarcoma
|
Indeterminate lung nodules
|
3
|
Myxofibrosarcoma
|
Indeterminate lung nodules
|
3
|
Mellanocytic tumour
|
Indeterminate lung nodules
|
3
|
Myxofibrosarcoma
|
Indeterminate lung nodules
|
3
|
MPNST
|
Lung metastases
|
3
|
Spindle cell sarcoma
|
Indeterminate lung nodules
|
U
|
Epithelioid haemangioendothelioma
|
Iliac node
|
U
|
Malignant myoepithilioma
|
Indeterminate lung nodules
|
U
|
MPNST
|
Indeterminate lung nodules
|
U
|
Myxoid liposarcoma
|
Indeterminate lung nodules
|
Table 9: Deep STS,
skin,
bone and muscle involvement
Histologic type of STS
(n = 26/52)
|
Skin infiltration
|
Bone infiltration
|
Muscle infiltration
|
Distant metastases
|
ALT x 2
|
|
|
|
|
ALT sclerosing tumour
|
|
|
Y
|
|
Desmoid-type fibromatosis
|
|
|
Y
|
|
Epithelioid haemangioendothelioma
|
|
|
Y
|
Iliac node
|
Epithelioid MPNST
|
|
|
|
|
Fibrosarcoma
|
|
|
|
|
Leiomyosarcoma
|
|
|
Y
|
Indeterminate lung nodule and RT subpleural mass
|
Leiomyosarcoma
|
|
Y
|
Y
|
|
Malignant myoepithilioma
|
|
Y
|
|
Indeterminate lung nodules
|
MPNST x 3
|
|
|
Y (2/3)
|
First case: Indeterminate lung nodule/mediastinal neurofibroma + Second case: indeterminate nodule
|
Myxofibrosarcoma x 3
|
|
|
Y (1/3)
|
Lung metastases
|
Myxoid liposarcoma x 3
|
|
|
|
|
PEComa
|
|
|
Y
|
|
Pleomorphic sarcoma
|
Y
|
|
Y
|
|
Rhabdomyosarcoma
|
|
|
Y
|
|
Solitary fibrous tumour
|
|
|
|
|
Spindle cell sarcoma x 2
|
|
|
Y (1/2)
|
|
Table 10: Superficial STS,
skin,
bone and muscle involvement
Histology STS (n = 22)
|
Skin infiltration
|
Bone infiltration
|
Muscle infiltration
|
Distant metastases
|
ALT sclerosing fibrous type
|
|
|
|
|
Cutaneous leiomyosarcoma x 2
|
Y
|
|
|
|
Leiomyosarcoma x 2
|
Y (single case)
|
|
Y – (single case)
|
Indeterminate lung nodules
|
Melanocytic tumour
|
|
|
Y
|
Indeterminate lung nodules
|
MPNST
|
Y
|
|
|
|
Multifocal cutaneous angiosarcoma
|
|
|
|
Indeterminate lung nodules
|
Myxofibrosarcoma x 4
|
Y (two cases)
|
|
Y (single case)
|
Indeterminate lung nodules
|
Myxoid liposarcoma
|
|
|
|
Multiple lung nodules
|
Myxoid/round cell liposarcoma
|
|
|
|
|
Pleomorphic liposarcoma
|
|
|
|
|
Pleomorphic sarcoma x 2
|
Y (single case)
|
|
Y (single case)
|
Indeterminate lung nodules
|
Spindle cell sarcoma x 4
|
Y
|
|
Y
|
Indeterminate lung nodules
|
Spindle cell sarcoma
|
Y
|
Y
|
|
|
Spindle cell sarcoma
|
|
|
|
|
Spindle cell sarcoma
|
Y
|
|
|
|
Unusual spindle cell tumour
|
Y
|
|
|
|
Table 11: Primary STS and superficial and deep infiltration
Histologic type STS (n = 4)
|
Super/Deep
|
Skin infiltration
|
Bone infiltration
|
Muscle infiltration
|
Clear cell sarcoma
|
S>Deep
|
|
Y
|
|
Myxoid spindle cell sarcoma
|
S>Deep
|
|
|
|
Sclerosing Epithelioid sarcoma
|
S and D
|
|
|
Y
|
Spindle cell sarcoma
|
S>Deep
|
Y
|
Y
|
Y
|