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Pathologic Quiz Case: Residents' Page
Pathologic Quiz Case: Residents' Page
Pathologic Diagnosis
Gliosarcoma Containing Malignant Fibrohistiocytic,
Osseous, and Chondroid Elements
Gliosarcoma is a biphasic neoplasm that contains both
malignant glial and mesenchymal elements. In most lesions, the malignant glial component is glioblastoma multiforme (GBM), although an oligodendroglial component
has occasionally been described. These tumors are rare,
representing between 1.8% and 8% of malignant glial neoplasms.1,2
Feigin and Gross3 described 3 patients with tumors
composed of mixed glial and sarcomatous elements. These
authors postulated that the sarcomatous component arose
from neoplastic transformation of vascular endothelium
within a primary GBM. Many studies utilizing immunohistochemical and ultrastructural markers for vascular origin have both supported and refuted this hypothesis.
Still, these tumors are often referred to as Feigin tumors.
Later investigators classified mixed glial and sarcomatous
neoplasms according to their presumed primary component. In 1979, Lalitha and Rubinstein4 suggested the term
sarcoglioma for malignant mesenchymal brain tumors containing secondary glial elements. They described lesions with a central meningioma or sarcoma surrounded
by gliomatous elements ranging from reactive astrocytosis
to GBM. Based on the tumor arrangement, the authors
proposed that the sarcoma induced the development of
glioma.
A temporal location is classically reported for gliosarcomas, and compiled data support this observation (Table). As in most gliomas, gliosarcomas are more common
in males than females. The majority occur in the aged, as
is the case for most high-grade glial neoplasms. In contrast, sarcogliomas tend to occur in children or young
adults. Gliosarcomas have shown a predilection to arise
in patients having undergone previous cerebral radiation
therapy. The mean survival of patients with gliosarcoma
is similar to that of patients with GBM, being less than 12
months. In contrast to GBM, there is a propensity for
gliosarcoma to disseminate and produce extracranial metastases.6
Neuroimaging reveals a hypodense enhancing mass
that is superficially located within the cerebral parenchyma and often associated with the leptomeninges and dura.
Peritumoral edema is common, and calcification and cystification are sometimes evident. Grossly, gliosarcomas appear firm, discrete, and lobulated. Frequently, there is
meningeal attachment, and the macroscopic appearance
may suggest meningioma. The cut surface is often variegated with firm gritty areas alternating with softened yellow areas. Microscopically, the tumor possesses fascicles
of sarcoma interspersed with GBM in a marbled arrangement. In advanced lesions, neoplastic glial cells may be
only sparsely distributed within the sarcoma. The spindle
cells of sarcomatous areas typically have cytologic features
of malignancy, including pleomorphic nuclei with vesicular or clumped chromatin and prominent nucleoli. Mitotic figures are present, although they may not be abundant. Although fibrosarcoma is the most frequently reported stromal malignancy,7 other patterns resembling
malignant fibrous histiocytoma, rhabdomyosarcoma, os-
Percent
Age,
,40
8
13
4059
27
43
.60
28
44
Sex
Male
35
56
Female
28
44
Location*
Frontal
18
21
Temporal
30
36
Parietal
26
31
Occipital
7
8
Other
3
4
* Tumors located in overlapping lobes are scored in all areas which
apply.