Intracranial hemorrhage as the initial presentation of gliosarcoma in a young woman
A 23-year-old female with no significant medical history developed a headache. On the evening of the following day, the headache was accompanied by nausea and vomiting. Her mother discovered her having a seizure the following morning and brought her immediately to the ED. She had another seizure and vomited upon arrival to the ED, and shortly thereafter, she was sent for a noncontrast head CT. An MRI was acquired 2 days later. Several days after these imaging studies, the patient underwent surgery.
The noncontrast head CT demonstrates a large, roughly spherical, acute hematoma in the left medial frontal lobe, near the falx cerebri, with associated vasogenic edema (Figure 1). The frontal lobe cortex immediately anterior and medial to the hematoma appears somewhat enlarged. MRI again demonstrates the acute hematoma and vasogenic edema (Figure 2). Best seen on the T2W images, there is abnormal signal intensity and mild expansion of the frontal lobe cortex medial and anterior to the hematoma, corresponding to the findings on the CT scan. On post-contrast images, there is a component of abnormal enhancement along the medial aspect of the hematoma. The histology slides show two distinct components of the abnormal tissue (Figure 3), one of glial origin, the other sarcomatous.
Photos courtesy of M. Ghesani
There is a wide range in the reported frequency of acute intracranial hemorrhage as the initial presentation of cerebral neoplasm, between 9% and 42%. In the absence of trauma history, there are some radiologic and clinical features of acute intracranial hemorrhage that may raise suspicion of an underlying lesion such as a neoplasm or vascular malformation.
The degree of vasogenic edema surrounding an acute hematoma with underlying neoplasm may be more pronounced, at initial imaging, than in the setting of bland hemorrhage. Bland hemorrhage is commonly seen in the setting of hypertension and typically located in the basal ganglia region. Lobar hemorrhages such as those seen in the setting of amyloid angiopathy usually affect elderly patients. Hemorrhage may present acutely in the setting of venous sinus thrombosis and associated venous infarct. This patient is relatively young, and the hemorrhage is in an uncommon location. Despite proximity of the hematoma to the falx, there is no radiologic evidence of superior sagittal sinus thrombosis. Nor are there any abnormal flow voids to suggest an arteriovenous malformation. Most importantly, there is abnormal enhancing tissue within the medial aspect of the frontal lobe cortex, adjacent to the hematoma on the post-contrast MRI, suspicious for a high-grade glial tumor. The mass was resected, and the histology of the medial frontal lobe mass was consistent with gliosarcoma.
The specific imaging characteristics of gliosarcoma may include a heterogeneously enhancing mass. There may be dural invasion, depending on the location of the tumor. On CT, gliosarcoma can appear as a large necrotic mass with heterogeneous contrast enhancement mimicking glioblastoma. Another variant can appear as a hyperdense lesion with well-defined margins and homogeneous enhancement. There are few detailed descriptions of the MRI appearance of gliosarcoma, as many of the case series were published before the widespread use of MRI. However, foci of hemorrhage and necrosis have been described in gliosarcoma, as well as marked peritumoral edema and heterogeneous appearance on T1 and T2 weighted images.
Gliosarcoma is a rare glioblastoma variant that features glial and mesenchymal components, accounting for approximately 2% of all glioblastoma. WHO describes gliosarcoma as a well-circumscribed lesion with clearly identifiable biphasic glial and metaplastic mesenchymal components. Gliosarcoma has been described as being most common in the temporal lobe. Other small series cite the frontal lobe as the most common location. Gliosarcoma is almost never infratentorial in location. The histiogenesis of gliosarcoma is uncertain, and there are several theories in the literature for the origins of the sarcomatous component.
Gliosarcoma presents most commonly between the 4th through 7th decades with a male-to-female predominance of 2:1. Presenting symptoms are similar to those of any space-occupying lesion and include aphasia, headache, seizures and cognitive decline. Similar to glioblastoma multiforme, gliosarcoma has a poor prognosis, due mainly to the high frequency of local recurrence. If untreated, the prognosis is approximately 4 months. There is a slightly better prognosis with treatment, approximately 6 to 10 months. Compared with glioblastoma, extracranial metastases from gliosarcoma occur more frequently and are often found to have only the sarcomatous components. Metastases to cervical lymph nodes, spleen, skin, adrenal glands, kidneys, oral mucosa, bone marrow, skull, ribs and the spine have all been reported.
Munir Ghesani, MD, is an attending radiologist at St. Luke’s-Roosevelt Hospital Center, and Beth Israel Medical Center and a HemOnc Today section editor. He is an associate clinical professor of radiology at Columbia University College of Physicians and Surgeons.
Lauren Moomjian, MD, is a radiology resident at St Luke’s-Roosevelt Hospital Center, New York.
Daniel Meltzer, MD is an assistant attending physician in radiology at St Luke-Roosevelt Hospital Center and Beth Israel Medical Center and Assistant Professor of Clinical Radiology at Albert Einstein College of Medicine, New York.
For more information:
- Han SJ. J Neurooncol. 2010;96:313-320.
- Koul R. J HK Coll Radiology. 2008;11:116-121.
- Meis JM. Cancer. 1991;67:2342-2348.
- Pakos EE. J Neurooncol. 2005;74:301-304.
- Schrader B. Acta Neurochir (Wien). 2000;142:979-985.