Bone and soft tissue malignancies account for a small portion of brain metastases. In this review, we characterize their incidence, treatments, and prognosis. Most of the data in the literature is based on case reports and small case series. Less than 5% of brain metastases are from bone and soft tissue sarcomas, occurring most commonly in Ewing’s sarcoma, malignant fibrous tumors, and osteosarcoma. Mean interval from initial cancer diagnosis to brain metastasis is in the range of 20–30 months, with most being detected before 24 months (osteosarcoma, Ewing sarcoma, chordoma, angiosarcoma, and rhabdomyosarcoma), some at 24–36 months (malignant fibrous tumors, malignant peripheral nerve sheath tumors, and alveolar soft part sarcoma), and a few after 36 months (chondrosarcoma and liposarcoma). Overall mean survival ranges between 7 and 16 months, with the majority surviving < 12 months (Ewing’s sarcoma, liposarcoma, malignant fibrous tumors, malignant peripheral nerve sheath tumors, angiosarcoma and chordomas). Management is heterogeneous involving surgery, radiosurgery, radiotherapy, and chemotherapy. While a survival advantage may exist for those given aggressive treatment involving surgical resection, such patients tended to have a favorable preoperative performance status and minimal systemic disease.
It is estimated that up to 30% of patients with cancer will develop brain involvement [
Accounting for 0.8% of all cancers, musculoskeletal bone and soft tissue sarcomas make up a small portion of patients with brain metastases [
The purpose of this review is to present the current literature on brain metastasis (BM) from bone and soft tissue cancers, with an emphasis on musculoskeletal sarcomas and those most commonly metastasizing to the brain. We describe the incidence, diagnostic strategies, treatment paradigms, and prognostic outcomes as well as relevant background information.
A search of the published literature was conducted for patients with brain metastasis from musculoskeletal sarcomas. The national library of medicine search engine, PubMed, was utilized for the literature search. For each of the sarcomas, the search terms “brain” and “intracranial” were combined with the tumor’s name: “osteosarcoma,” “Ewing’s sarcoma,” “chondrosarcoma,” “chordoma,” “malignant fibrous tumor,” “malignant fibrous histiocytoma,” “fibrosarcoma,” “liposarcoma,” “alveolar soft part sarcoma,” “rhabdomyosarcoma,” “malignant peripheral nerve sheath tumor,” “MPNST,” or “angiosarcoma.” Relevant articles describing case reports or clinical studies were selected, and the reference lists from these articles were also inspected for other relevant articles. Each of the resultant articles were examined closely and reported in this review. Cases in which there was contiguous extension of a primary tumor into intracranial structures (i.e., a skull tumor) as opposed to frank metastasis from a distant site were excluded. Only publications in English, peer-reviewed journals were included.
The primary malignant bone tumors discussed are the most commonly reported to metastasize to the brain: osteosarcoma (osteogenic sarcoma), Ewing’s sarcoma, chondrosarcoma, chordoma, and fibroblastic/fibrohistiocytic tumors.
After plasmacytoma (33%), osteosarcoma (20%) is the most frequently occurring primary malignant bone tumor [
Table
Comparison of published reports detailing characteristics of patients with brain metastases from primary osteosarcoma.
Case author | Age, sex | Primary site | Primary treatment | IB Mo | Metastatic site | Metastatic treatment | OS Mo | Special aspects |
---|---|---|---|---|---|---|---|---|
Rabah et al., 2013 [ |
10 F | Right humerus | (None) | 0a | Right frontal lobe | Chemo, WBRT | 14 | Cerebral metastasis on presentation |
|
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Onodera et al., 2012 [ |
14 F | Left femur | Surgery, chemo | 12 | Right parietal lobe | SR | NR | No active pulmonary metastasis |
|
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Chou et al., 2011 [ |
|
NR | NR | NR | Single cerebrum (3); multiple (2) | SR + RT | D | |
|
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Flannery et al., 2010 [ |
|
Pelvis, femur, humerus | Chemo | NR | 1 single, 2 multiple | GKSRS | 36–84 | Gamma knife radiosurgery as therapy |
|
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Salvati et al., 2010, 1998 [ |
|
Femur, tibia | NR | 0–110 | Frontal (5), multiple (3), parietal, temporal | SR + WBRT | NR | |
|
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Niazi et al., 2009 [ |
16 M | Right femur | (None) | 0a | Left cerebellum | SR | 6 days | Posterior fossa hemorrhage on presentation |
|
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Kebudi et al., 2005 [ |
|
NR | NR | 3–27 | Single (3), |
None (2); |
1–6 | Literature review included in original study |
|
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Yonemoto et al., 2003 [ |
14 F | Right femur | SR + chemo | 26 | Tumor infiltration outside the bone cortex | SR + WBRT | Alive at 72 | N/a |
|
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Paulino et al., 2003 [ |
|
Femur (2), humerus, tibia, scapula | NR | 10–48 | Frontal (3), occipital (1), multiple (1) | SR, chemo, WBRT, None (2) | 1–10 | |
|
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Weil et al., 2005 [ |
26 M | Right tibia | Chemo + SR | 36 | Skull and brain metastasis | SR | 4 | Patient refused chemo or RT for brain met treatment |
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Bouffet et al., 1997 [ |
|
NR | NR | 12–15 | Supratentorial (2) | None |
2–4 | |
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Wroński et al., 1995 [ |
|
Femur (3), maxilla humerus | NR | 18–63 | Temporal, parietal/occipital (3), posterior fossa | SR | 2.4–13 | WBRT was given to select patients (unspecified) |
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Chang et al., 1994 [ |
20 M | Bilateral femur | (None) | 0a | Right frontal lobe | SR + RT | 5 | Early metastasis, multifocal |
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Marina et al., 1993 [ |
3 M | Left humerus | SR, chemo | 4 | Temporal and left parietal | Chemo | Alive at 108 | Additional review of 13 patients |
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Wexler et al., 1993 [ |
10 F | Right femur | SR, chemo | 51 | Left parietal and occipital | SR, WBRT | Alive at 120 | N/a |
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Niedeggen et al., 1990 [ |
7 M | Left femur | SR, chemo | 76 | Right parietal lobe | SR, RT | Alive at 13 | Histological findings included |
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Baram et al., 1988 [ |
|
NR | Chemo ± SR | 2–46 | Frontal (2), multiple (1), NR (2) | Chemo ± SR | D | Incidence, clinical, and radiological findings and management |
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Lewis, 1988 [ |
15 F | Right humerus | Local radiotherapy | 0c | Left frontal lobe, left corpus callosum | Chemo | 0 | Brain lesions discovered upon autopsy |
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Ozarda et al., 1983 [ |
23 M | Right femur | NR | 8 | Right occipital lobe | Chemo + RT | NR | Bone scintigraphy usage |
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Danziger et al., 1979 [ |
|
Right femur (3) | SR, chemo, RT | 6–24 | Temporal/occipital, frontoparietal, parietal | Chemo + RT |
D (2), NR |
IB: interval to brain metastasis (mo); OS: overall survival in months; M: male; F: female; chemo: chemotherapy; SR: surgical resection; GKSRS: Gamma knife stereotactic radiosurgery. WBRT: whole brain radiotherapy; RT: radiotherapy; D: death prior to treatment; NR: not reported; abrain metastasis at initial presentation; balive at last follow-up without recurrence; cbrain metastasis at autopsy.
Yonemoto et al. [
As a commonly diagnosed musculoskeletal cancer in children and young adults, osteosarcoma, when metastatic, mainly spreads to the lungs and other bones and rarely to the brain. As such, it is difficult to form consensus guidelines on treatment once BM occurs. As many of these lesions are solitary, surgical excision has been the standard of care, with chemotherapy and RT for palliative measures.
Ewing’s sarcoma is commonly seen in young adults and has a slight male predominance [
Approximately 40 cases of ESBM have been described in the literature (Table
Comparison of published reports detailing characteristics of patients with brain metastases from Ewing’s sarcoma.
Case author | Age, sex | Primary site | Primary treatment | IB Mo | Metastatic site | Metastatic treatment | OS Mo | Special aspects |
---|---|---|---|---|---|---|---|---|
Baid et al., 1992 [ |
8 M | Left thigh | RT |
1 | Right sellar and parasellar region | WBRT | NR | No response of local disease to chemotherapy |
Mineura et al., 1989 [ |
5 F | Axilla | SR |
5 | Right occipital region | SR, WBRT, |
0 | Suppression of blood flow, metabolism of grey matter adjacent to BM |
Capitini et al., 2009 [ |
26 M | Left lateral femur | RT |
3 | Left parietal and right occipital | SR, WBRT, GKSRS | 10 | Developed GVHD after allogeneic hematopoietic stem cell transplant |
Simpson et al., 1989 [ |
21 M | Right 6th and 7th rib | SR |
24 | Left parietal cortex | SR, WBRT | 2b | Initial sarcoma misdiagnosed as costochondritis, |
Parasuraman et al., 1999 [ |
|
Pelvis (4), humerus, scapula, tibia, vertebrae (2) | RT |
12–54 | Parietal (4), frontal (5), temporal | RT, S, chemotherapy | 3 | Pediatric case series |
Turgut et al., 1994 [ |
22 M | Sacroiliac joint | SR chemotherapy | 24 | Temporal lobe, parietal | RT | 8 | No active lesion within primary, BM occurred 24 months s/p treatment |
Kies and Kennedy, 1978 [ |
|
NR | RT, chemo | NR | Multiple | WBRT | 3 | Retrospective analysis of 134 patients with Ewing sarcoma. Found 3 BM |
Olivi et al., 1991 [ |
30 F | Rib | SR. RT, chemo | 48 | Right posterior frontal | WBRT, S, E, Df |
19b | Pancytopenias, and |
Chou et al., 2011 [ |
|
NR | RT, chemo | NR | Multiple | Palliative | NR | Respiratory failure, neuropenian, pneumonia |
Salvati et al., 2010, 1998 [ |
|
Femur (2), sacrum, tibia, ulna | RT, chemo | 1–13 | Multiple (2), occipital, frontal, parietal | WBRT/unspecified | NR | Retrospective analysis of 35 cases of supratentorial brain metastases |
Paulino et al., 2003 [ |
|
Scapula (2), rib, femur, humerus, pelvis | RT, chemo | 0–8 | Multiple (2), frontal, parietal, temporal (2) | WBRT + chemo (5), palliative | 1.5–9 | 1 death due to BM |
Ogose et al., 1999 [ |
|
Thigh, buttock | RT, chemo | 3-4 | Right temporal, occipital | Conservative | 1–6 | |
Bindal et al., 1994 [ |
|
Tibia, femur, thigh, chest wall | NR | 29–115 | Multiple (3), frontal | S (3),WBRT | NR | |
Flannery et al., 2010 [ |
33 F | Pelvis | RT, chemo | 96 | Multiple | S,WBRT,GKSRS | 24 | |
Bouffet et al., 1997 [ |
17 M | NR | RT, chemo | 11 | Multiple | RT | 2 | Patient expired secondary to BM |
IB: interval to brain metastasis (mo); OS: overall survival in months; M: male; F: female; chemo: chemotherapy; SR: surgical resection; GKSRS: Gamma knife stereotactic radiosurgery. GVHD, graft versus host disease; ICH: intracerebral hemorrhage on presentation; WBRT: whole brain radiotherapy; RT: radiotherapy; D: death; NED: no evidence of disease; NR: not reported; NF: associated with neurofibromatosis type-1; abrain metastasis at initial presentation; balive at last follow-up without recurrence.
Overall, the appearance of BM signifies a poor prognostic outcome in patients with ES. The current increased frequency of CNS metastases has been linked to prolonged survival and alteration of host response following chemotherapy [
Chondrosarcoma is a malignancy of mesenchyme that is a common primary bone tumor subsequent to osteosarcoma in frequency [
Table
Comparison of published reports on patients with brain metastases from chondrosarcoma and chordoma.
Case author | Age, sex | IB yr. | BM treatment | OS Mo |
---|---|---|---|---|
Chondrosarcoma | ||||
Flannery et al., 2010 [ |
14 M, 56 M | 0.5–1.3 | GKSRS | 18–21 |
Francés-Muñoz et al., 2012 [ |
53 F | 10 | Chemo for lung | NR |
Kawaguchi et al., 2012 [ |
54 M | 0.7 | Chemo | 52 |
Jallu et al., 1992 [ |
54 F | 0.7 | SR | NR |
Konishi et al., 1994 [ |
72 F | 2 | SR | 2 |
Reyaz and Ashraf, 2006 [ |
34 F | 7 | SR | NR |
Talerman, 1967 [ |
26 F | — | — | — |
Templeton et al., 1985 [ |
15 M | 34 | D | NR |
Waga et al., 1972 [ |
32 M | 12 | SR | NR |
Tsutsumi et al., 2010 [ |
60 M | 4.4 | Chemo, GKSRS | 10+ |
Fox et al., 1968 [ |
51 F | — | D | 11 |
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Chordoma | ||||
Kamel et al., 2005 [ |
12 M | 32 | SR | 32 (alive) |
Anderson and Meyers, 1968 [ |
69 M | 24 | SR (gross total) | 36 (alive) |
Al-Adra et al., 2011 [ |
29 M | 16 | SR, WBRT | ? |
Morris and Rabinovitch, 1947 [ |
41 M | 2.5 | WBRT | 2.5 |
Fichardt and De Villiers, 1974 [ |
59 M | 36 | SR | 42 |
Higinbotham et al. 1967 [ |
58 F | ? | None (autopsy finding) | 72 |
Mesgarzadeh et al., 2008 [ |
27 F | 18 | None | 24 |
Chemo: chemotherapy; D: death prior to treatment; F: female; GKSRS: Gamma knife stereotactic radiosurgery; IB: interval between diagnosis of brain metastasis from chondrosarcoma in years (yr); M: male; NR: not reported; OS: overall survival in months; Radio: radiotherapy; SR: surgical resection; WBRT: whole brain radiotherapy; adiagnosed postmortem; bbrain metastasis diagnosed first; +: survival reported for at least the stated amount.
Treatment with chemo- and radiotherapy has resulted in poor results, postulated due to the resistant extracellular matrix, low mitotic index, and sparse vascularization of cells [
Though chondrosarcoma accounts for 40% of primary bone cancers in adults [
Chordomas are rare, malignant tumors, which arise from the embryonic notochord [
Inherently, chordomas destroy and replace bone in which they develop [
Metastasis from chordomas depends on many factors such as histology, mitotic activity, and treatment regimens [
Previously designated under “malignant fibrous tumors,” these are rare malignancies characterized by pleomorphic, high-grade tumor cells with histiocytic, fibroblastic, and myofibroblastic features [
Although these tumors can originate from either bone or soft tissue, defining the source is difficult [
As with other sarcomas, pulmonary metastasis is most common, with its detection increasing the risk of future BM [
Comparison of published reports detailing characteristics of patients with brain metastases from fibroblastic and fibrohistiocytic tumors.
Case author | Age, sex | Tumor type; primary site | Primary treatment | IB Mo | Metastatic site | Metastatic treatment | OS Mo | Special aspects/ |
---|---|---|---|---|---|---|---|---|
Wu et al., 2012 [ |
32 F | MFH; maxillary sinus | SR (radical); RT | 24 | Right parietal | SR; WBRT | 25 |
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Graber et al., 2011 [ |
56 M | MFH; aorta | RT; chemo | 0a | Multiple | Biopsy | ? | Cystic brain lesion; mimics neurocysticercosis |
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Louis et al., 2007 [ |
38 M | MFH; mandible | SR; RT | 12 | Extensive, unspecifieda | None | 12 | MFH associated w/a Marjolin’s ulcer |
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Kousar et al., 2009 [ |
20 F | AFS; mandible | SR; RT | 6 | Multiple | None | 15 |
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Hoshi et al., 2008 [ |
45 M | MFH; forearm | SR; RT; chemo | 48 | Multiple, parietal, and occipital | Chemo, WBRT | 60 | Previous lung/bone metastasis at 3 yrs; SC metastasis at 5 yrs |
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Erguvan-Önal et al., 2004 [ |
45 F | FS; thigh | SR; RT | 2 | Right frontal | SR | 7 | Epitheloid transformation; |
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Rogers and Whelan, 2000 [ |
|
MFH; humerus, femur, knee, pelvis | Chemo; RT | 4–148 | Multiple (2), single (2) | RT (2), none (2) | 7–153 | Previous systemic metastasis in all |
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Ogose et al., 1999 [ |
|
Thigh (3), back | NR | 0–10 | Frontal (3), parietal, temporal | None (2), SR (2) | 0.5–16 |
|
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Salvati et al., 2010 [ |
|
MFH; heart, heart | RT, chemo | 19–21 | Frontal | SR | 9–11 |
|
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I. A. Auer and R. N. Auer, 1998 [ |
43 M | DFS; abdomen | NR | 72 | Tempro-parietal | SR | 144 | Brain recurrence at 144; prior skin, lung metastasis |
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Kim et al., 1997 [ |
18 F | MFS; heart | SR, RT, chemo | 24 | Occipital, cerebellum | SR, WBRT | 50 |
|
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Jeffery and Ford, 1995 [ |
67 F | FS; abdominal wall | SR | 60 | Right parietal lobe | None | 61 | ICH; previous bone metastasis; |
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Onoda et al., 1990 [ |
45 M | DFS; upper arm | SR | 76 | Multiple | none | 84 | Lungs, skin |
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Wroński et al., 1995 [ |
|
MFH; scalp, thigh, pul.a. | NR | 3–27 | Occipital, parietal, posterior fossa | SR | 10.2b–65b | Lungs |
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Bindal et al., 1994 [ |
|
MFH; trunk, heart, shoulder | 12–2218 | Right parietal, multiple (2) | SR, WBRT | 2.3b–11.8b | Prev lungs | |
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Lewis, 1988 [ |
36–53, M | DFS; clavicular; MFH, scapular | SR; RT, chemo | 36–312 | Multiple | SR; nonec | 313, LTF |
|
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Takamiya et al., 1986 [ |
43 M | FS; pectoralis major | SR | 1 | Right frontoparietal | SR, RT, WBRT | Alive at 13 |
|
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Zucker et al., 1978 [ |
42 F | FS; liver? | None | 24 | Pons | none | 24 |
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Dal Canto and Valsamis, 1973 [ |
60 F | FS; renal capsule | None | 0 | Pons | None | 0 |
|
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Gercovich et al., 1975 [ |
|
FS; lip, retroperitoneal | Chemo | 3–55 | Frontal, parietal | NR | NR |
|
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Ho, 1979 [ |
70 F | FS; thigh | SR (partial) | 12 | Leptomeninges, cortical | Nonec | NR |
|
AFS: ameloblastic fibrosarcoma; FS: fibrosarcoma; DFS: dermatofibrosarcoma; IB: interval to brain metastasis (mo); OS: overall survival in months; M: male; F: female; chemo: chemotherapy; GKSRS: Gamma knife stereotactic radiosurgery; MFS: myxofibrosarcoma; ICH: intracerebral hemorrhage on presentation; SR: surgical resection; WBRT: whole brain radiotherapy; RT: radiotherapy; D: death prior to treatment; LTF: lost to follow-up; MFH: malignant fibrous histiocytoma (undifferentiated pleomorphic sarcoma); NR: not reported.
aBrain metastasis diagnosed at presentation; bsurvival after craniotomy; cbrain metastasis reported at autopsy.
Periodic imaging of the brain, the primary site, and the metastatic sites has been suggested to prolong life expectancy, though it remains controversial [
The primary malignant soft tissue tumors (STS) discussed are also the most likely to metastasize to the brain: liposarcoma, rhabdomyosarcoma, malignant peripheral nerve sheath tumor, angiosarcoma, and alveolar soft part sarcoma.
Liposarcomas comprise about 10–20% of all STS [
Espat et al. [
Comparison of published reports detailing characteristics of patients with brain metastases from liposarcoma and rhabdomyosarcoma.
Case author | Age, sex | IB yr. | BM treatment | OS Mo |
---|---|---|---|---|
Rhabdomyosarcoma | ||||
Ahola et al., 1998 [ |
13 M | 0.7 | SR | 14 |
Andersen-Ranberg and Helmer-Hansen, 1987 [ |
19 M | 0.5 | NR | 6+ |
Flannery et al., 2010 [ |
18 F | 5.0 | GKSRS, WBRT | 60 |
Ho, 1979 [ |
79 M | — | D | — |
Kebudi et al., 2005 [ |
11 M | 0 | RT, Chemo | 3 |
Kleinert et al., 1985 [ |
14 F | D | 48 | |
Noda et al., 1995 [ |
2 M | 0.5 | Chemo, RT | 32+ |
Ogose et al., 1999 [ |
7 F, 16 M | NR, 0.2 | Chemo, RT | 4, 4 |
Osawa et al., 2011 [ |
|
1.1–12.2 | SR, RT, Chemo | 21–207 |
Paulino et al., 2003 [ |
|
NR | WBRT, Chemo | 2–63 |
Rodriguez-Galindo et al., 2001 [ |
|
0.1–1.1 | NR | 1.5–24 |
Salvati et al., 2010 [ |
|
0.2–0.3 | SR | 12.8 |
|
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Liposarcoma | ||||
Arepally et al., 1996 [ |
56 M | 312 | SR, WBRT | 324 |
Bailey et al., 2001 [ |
54 F | 108 | SR, WBRT | Alive at 114 |
Can et al., 1993 [ |
22 M | 0 | none | |
Ferguson et al., 2006 [ |
48 F | 276 | RT | 278 |
Fitzpatrick et al., 1999 [ |
74 F | 24 | SR | Alive at 30 |
Haft et al., 1988 [ |
52 F | 216 | SR (total), chemo | 228 |
Kumar and Teasdale, 2000 [ |
73 F | 12 | SR | Alive at 12 |
Salvati et al., 2010 [ |
48 M | NR | SR | NR |
Utsunomiya et al., 1999 [ |
44 M | 72 | SR (subtotal) | 77 |
IB: Interval to brain metastasis (mo); OS: overall survival in months; M: male; F: female; chemo: chemotherapy; SR: surgical resection; GKSRS: Gamma knife stereotactic radiosurgery; ICH: intracerebral hemorrhage on presentation; WBRT: whole brain radiotherapy; RT: radiotherapy; D: death prior to treatment; NR: not reported; UK: unknown; asurvival after craniotomy.
As the lifespan of patients diagnosed with liposarcoma extends, the natural history of the disease leads to CNS metastases, which occurs in a large part after 10 years following original diagnosis [
Rhabdomyosarcoma (RMS) is a malignancy of striated muscle and one of the common STS in those under 20 years [
Vezeridis et al. [
Like other sarcomas, multimodality treatment is instigated for those with BM from RMS [
RMS is a common STS affecting young patients, and it has been known to metastasize to different organs and tissues. Patients with a history of metastasis have a significant risk of future BM [
A number of entities are synonymous with malignant peripheral nerve sheath tumors (MPNST) including neurofibrosarcoma, neurogenic sarcoma, and malignant schwannoma [
Comparison of published reports detailing characteristics of patients with brain metastases from malignant peripheral nerve sheath tumor (MPNST) and angiosarcoma.
Case author | Age, sex | IB yr. | BM treatment | OS Mo |
---|---|---|---|---|
Malignant peripheral nerve sheath tumors | ||||
Xu et al., 2012 [ |
8 M | 14 | (None) | 16 |
Flannery et al., 2010 [ |
34 F | 36 | SR, WBRT, GKSRS | 48 |
Tilgner et al., 2007 [ |
60 M | 0a | SR, WBRT | Alive at 14 |
Park et al., 2007 [ |
21 M | 0a | SR, WBRT, Chemo | 16 |
van Eck and Horstmann, 2006 [ |
83 M | 108 | SR, GKSRS | 122 |
Matyja et al., 2004 [ |
33 M | ~56 | SR | ~60 |
Yone et al., 2004 [ |
4 M | 7 | WBRT | 21 |
Oishi et al., 2000 [ |
48 M | 61 | SR (en bloc) | NR |
Maschke et al., 1999 [ |
17 F | 0a | SR | NR |
Probst-Cousin et al., 1997 [ |
19 F | 156 | None | 157 |
Haisa et al., 1996 [ |
58 F | 180 | SR (en bloc) | 181 |
Fenzi et al., 1995 [ |
45 F | 5 | None | 18 |
Seppälä and Haltia, 1993 [ |
13 M | 2 | None | 2 |
D'Angelo et al., 1991 [ |
68 F | 24 | SR (en bloc) | 36 |
Valdueza et al., 1991 [ |
47 M | ~13 | None | 18 |
Cras et al., 1990 [ |
46 F | 0 | None | 0 |
Hirose et al., 1989 [ |
53 M | 15 | SR (en bloc), chemo | Alive at 20b |
Hasegawa et al., 1984 [ |
51 M | 2 | Biopsies | 3 |
Macaulay, 1978 [ |
18 M | 2 | None | 2 |
White Jr., 1971 [ |
20 M | NR | SR, radiation | 7 |
D’Agostino et al., 1963 [ |
22 F | NR | NR | 68 |
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Angiosarcoma | ||||
Liassides et al., 2004 [ |
24 F | 3 | Palliative | 6 |
Eguchi et al., 2002 [ |
64 M | 19 | Palliative | 0 |
Chami et al., 1994 [ |
59 M | NR | Palliative | NR |
Vaquero et al., 1990 [ |
30 M | 4 | SR | 6 |
Kuratsu et al., 1991 [ |
17 M | — | SR | 12 |
Haft et al., 1988 [ |
31 F | — | WBRT | NR |
Akutsu et al., 2004 [ |
53 M | 2 | SR | 6 |
Ellegala et al., 2002 [ |
76 M | 24 | SR | 2 |
Søndenaa et al., 1993 [ |
73 F | 3 | Palliative | 3 |
Gallo et al., 2001 [ |
33 M | 12 | SR | 0 |
Hassane et al., 2010 [ |
48 M | 3 | Palliative | 0 |
Simpson et al., 1989 [ |
21 M | 24 | SR, WBRT | 26b |
Angrish et al., 1979 [ |
38 M | 3 | Palliative | 0 |
Plotnik et al., 2008 [ |
61 F | 60 | SR | NR |
Chaudhuri et al., 1980 [ |
31 F | 72 | SR, WBRT | 2 |
Macaulay, 1978 [ |
18 M | 2 | Palliative | 0 |
IB: interval to brain metastasis (mo); OS: overall survival in months; M: male; F: female; chemo: chemotherapy; SR: surgical resection; GKSRS: Gamma knife stereotactic radiosurgery; ICH: intracerebral hemorrhage on presentation; WBRT: whole brain radiotherapy; RT: radiotherapy; D: death prior to treatment; NR: not reported; NF: associated with neurofibromatosis type-1; abrain metastasis at initial presentation.; balive at last follow-up without recurrence.
According to Table
Aggressive SR followed by WBRT is the basis of treatment for BM. However, because of the limited pool of patients, there are cases surviving for many months without treatment and others dying over a short period after aggressive intervention [
MPNST is a very aggressive cancer associated with high recurrence and metastatic potential. Prognosis is generally poor and worse with metastasis [
Angiosarcoma is an exceedingly rare malignancy, which arises from endothelial cells of the vasculature. The least common among vascular tumors, angiosarcoma has a prevalence of <1% of all sarcomas [
Current understanding of the metastatic behavior of angiosarcomas is limited. Of reported cases with BM, the often involved primary site is the heart [
Overall, the prognosis of angiosarcoma is grim. Typical 5-year survival is 12%, with metastases and reoccurrence typically occurring within the first 2 years [
Alveolar soft part sarcomas (ASPS) account for a small percentage of STS at roughly 1% [
Portera et al. [
The literature is abundant with reports of BM from ASPS. Table
Summary of cases of alveolar soft part sarcoma with brain metastases as reported in previous published series.
Case Author |
|
Age, sex | Primary site | Primary treatment | IB Mo |
|
Metastatic treatment* | OS* (Mo) | Special aspects |
---|---|---|---|---|---|---|---|---|---|
Daigeler et al., 2008 [ |
11 | 19–24, M (7) | Thigh (2), lower leg (2), thoracic wall (2), upper arm (2), forearm (1), Foot (1) | SR; chemo with RT | 78 months (5–156) | 3/11 |
SR, RT | 73% of patients are still alive at follow-up with no evidence of disease | Localized disease, complete resection. And adjuvant radiation within 2 years had favorable outcome |
|
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Kayton et al., 2006 [ |
20 | 16.5; 6–24, |
Thigh (8), trunk (6), Retroperito. (2), scalp, neck, forearm, calf | SR, chemo, RT | 36 months | 2/20 | SR | Follow-up: patients were alive after mean 22 years (4–32 years) | Tumors, 5 cm were associated with longer progression-free survival |
|
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Park et al., 1999 [ |
6 | 24.5 (17–35) |
Femur (3), fibula (2), ilium | SR, chemo, RT | 10–12 months | 2/6 | N/A | Follow-up range of patients with no evidence of disease (7 mo.–8 yrs.) | ASPS arising in bone |
|
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Portera et al., 2001 [ |
74 | 26 (3–68) |
Extremities (60%), trunk (20%), head and neck (12%), Retroper. (8%) | SR; RT; chemo | 5–24 months | 14/74 | Chemo | Median was 41 months for patients w/o metastasis and 40 months with metastasis | |
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Bindal et al., 1994 [ |
2 | 40–48 |
Thigh, rectum | SR | 1–15 months | 2/2 | SR | Still alive after 24.7 and 16.4 months | |
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Flannery et al., 2010 [ |
2 | 42.4 (14–74) | Leg, gluteal | Chemo and SR | 30–48 months | 2/2 | SR | Survived from 5 to 7 years after surgery with no evidence of disease; 31–36 months after metastasis | |
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Salvati et al., 2010 [ |
3 | 35 (19–61); males | Thigh (100%) | SR | 24–58 months | 3/3 | Alive from 15 to 24 months after SR with no evidence of disease | ||
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Wroński et al., 1995 [ |
2 | 7–14 |
Thigh, tongue | SR | 23 months | 2/2 | SR | Alive 2.7–23 months SR with no evidence of disease | |
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Chou et al., 2011 [ |
4 | 25 (17–33); M | NR | SR and RT | 14.53 months | 4/4 | SR and RT | 3 dead; 1 still alive |
Neurosurgical resection can be favorable and may increase long-term survival [
Malignant histiocytomas (undifferentiated pleomorphic sarcomas) and fibrosarcomas originating from the soft tissues are covered under the
Previous landmark studies on the treatment of brain metastases have shown efficacy in surgical resection and postoperative WBRT [
Generally, BM from these cancers is very infrequent, most commonly occurring in Ewing’s sarcoma, fibroblastic/fibrohistiocytic tumors, and osteosarcoma. Pediatric patients are more likely to have RMS. Though ASPS commonly metastasizes to the brain, it is a much rare cancer and thus accounts for a lesser proportion. Many of the larger studies suggest a slight preponderance in males (52–60%) [
Comprehensive neurological evaluation and imaging should be performed on development of suspicious neurological signs or symptoms. Clinical presentation of BM is characterized by rapid onset of neurological symptoms, such as paresthesias, visual field defects, and headache. Generally, routine neuroimaging studies without these indications are not warranted because of the rarity of BM and because they almost always result in signs and symptoms if they are present though it has been suggested to screen patients with osteosarcoma and MFT with extensive disease, especially if there is a short disease free interval [
The extent of systemic disease and Karnofsky Performance Scale (KPS), as well as sarcoma histotype are important factors in determining patient outcomes. The presurgical KPS gives an overall clinical picture, and a score above 60–70 has been cited as a good prognostic indicator associated with higher median survival [
While SR has been suggested to lead to improved outcomes [
Brain metastasis in bone and soft tissue cancers usually occurs late in the malignancy. As most of the data in the literature is from case reports and case series utilizing heterogeneous treatments, it is difficult to discern the best therapeutic strategy. Prolonged survival and an adequate quality of life are achievable in a small, select population of patients. While such a survival advantage may exist for those given an aggressive treatment course involving surgical resection, it should be reserved for those with a favorable preoperative performance status and minimal systemic disease. Future large prospective studies can help give more insight on this uncommon, yet growing group of brain metastases. Overall, the occurrence of BM in patients with bone and soft tissue cancers is a poor prognostic sign that suggests late stage disease.
The authors declare that there is no conflict of interests regarding the publication of this paper.