Neuroradiology Case of the Month

December 2002

Jose A. Echeverri, MD, Manoj Ketkar, MD, Akio Hiwatashi, MD, Ahmed Abdelhalim, MD, Ramon de Guzman, MD, and P-L Westesson, MD, PhD, DDS

Clinical Presentation: A 35-year-old female presented with sudden onset of headaches, left arm numbness, dysarthria, and left facial numbness and tingling. She has a history of a stage IIA nodular sclerosing Hodgkin’s disease with bulky mediastinal lymphadenopathy, diagnosed when she was 20, for which she received chemotherapy and mantle radiation therapy.

Radiological Findings:

Figure 1: Precontrast Head CT: There is a well demarcated hypodense extra-axial mass overlying the right frontal lobe.
Figure 2A: Contrast-enhanced Head CT: The mass is extra-axial, enhances diffusely and has a broad dural base. There is edema of the adjacent parenchyma.	Figure 2B: Coronal reformation.

Figure 3: Head MRI

Figure 3A: Sagittal T1WI: The mass is hypointense.	Figure 3B: Coronal FLAIR: The mass is hyperintense and is surrounded by edema.
Figure 3C: Axial DWI: There is restricted diffusion in the periphery of this lesion. ADC was hyperintense, consistent with a T2 shine through effect.	Figure 3D: Axial T2WI: There is a well defined extra-axial hyperintense lesion, surrounded by hyperintense edema of the adjacent parenchyma.
Figure 3E: Axial T1WI: The mass is hypointense to the brain parenchyma.	Figure 3F: Post IV Gadolinium Axial MT: The lesion enhances diffusely.

Diagnosis: Malignant Fibrous Histiocytoma of the Meninges

Discussion: Intracranial malignant fibrous histiocytoma (MFH) is a rare tumor. It is composed of proliferating histiocytes, fibroblasts, multinucleated giant cells, and foamy lipid-filled xanthomatous cells characteristically arranged in a storiform or pinwheel pattern [1,2]. Most intracranial MFH are of meningeal origin, and more rarely they can be intracerebral. The latter are thought to originate from perivascular mesenchymal cells or perivascular pial sheath [2]. Even more rarely, they can originate from the cranial bone and extend into the subdural space [2].
   It lacks characteristic CT or MRI features. It is usually seen as a heterogeneous mass that may enhance, may contain areas of hemorrhage and may have large cystic components. It may resemble intracranial metastasis. Cases resembling a meningioma, as ours, have been described [1, 3]. Peritumoral hemorrhage can be seen and is thought to be due to infiltration of tumor along vessels [4].
    MFH has been described as originating after therapeutic irradiation within the irradiated field [5].
    Differential diagnosis includes meningiomas, dural sarcomas [1] and dural based metastasis. Radical resection of primary intracranial MFH is very difficult [1].

References:

1. Ozhan S, Tali ET, Isik S, et al. Haematoma-like primary intracranial malignant fibrous histiocytoma in a 5-year-old girl. Neuroradiology 1999; 41: 523-525.
2. Ogino A, Ochi M, Hayashi K, et al. MRI of intracranial meningeal malignant fibrous histiocytoma. Neuroradiology 1996; 38: 785-787.
3. Tsutsumi M, Kawano T, Kawaguchi T et al. Intracranial meningeal malignant fibrous histiocytoma mimicking parasagital meningioma-case report. Neurol Med Chir 2001; 41(2): 90-93.
4. Matsuzaki K, Fujimoto N, Kohyama Y et al. No Shinkei Geka 1996; 24 (6): 557-562. (article in Japanese).
5. Amendola BE, Amendola MA, McClatchey KD. Radiation-induced malignant fibrous histiocytoma: a report of five cases including two occurring post whole brain irradiation. Cancer Invest 1985; 3(6): 507-13.