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 »  Abstract
 »  Introduction
 »  Discussion
 »  Case No. 1
 »  Case No. 2
 »  References

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Year : 2004  |  Volume : 52  |  Issue : 3  |  Page : 387-390

Calvarial malignant fibrous histiocytoma

Departments of Neurosurgery, PGIMER, Chandigarh, India

Date of Acceptance19-Oct-2002

Correspondence Address:
Department of Neurosurgery, PGIMER, Chandigarh - 160012, India
[email protected]

 » Abstract 

Primary malignant fibrous histiocytoma (MFH) of the central nervous system (CNS) is uncommon. We report cases of two young patients of MFH arising from the cranial meninges and involving the adjacent skull and scalp. There was infiltration of the brain in one case. Both the lesions were excised and primary scalp repair was performed.

How to cite this article:
Chhabra R, Gupta S K, Manjunath Prasad K S, Gupta D, Vasishta R K, Sharma R K, Khosla V K. Calvarial malignant fibrous histiocytoma. Neurol India 2004;52:387-90

How to cite this URL:
Chhabra R, Gupta S K, Manjunath Prasad K S, Gupta D, Vasishta R K, Sharma R K, Khosla V K. Calvarial malignant fibrous histiocytoma. Neurol India [serial online] 2004 [cited 2022 Aug 18];52:387-90. Available from: https://www.neurologyindia.com/text.asp?2004/52/3/387/12768

 » Introduction Top

Malignant fibrous histiocytoma (MFH) is a common soft tissue tumor in adults occurring in the 5th to 7th decades of life and accounts for 10.5% to 21.6% of all soft tissue malignant neoplasms.[1] The primary incidence involving the central nervous system (CNS) was described by Gonzalez-Vitale et al[2] in 1976. Since then there have been only few sporadic case reports of MFH involving the CNS.[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14]
We report two young patients with malignant fibrous histiocytoma presenting as rapidly growing skull tumors.

 » Case No. 1 Top

A 25-year-old female presented with a swelling over the left parieto-occipital area for the past five months. The swelling showed ulcerative changes two months prior to admission in hospital. There was intermittent bleeding and purulent discharge from the swelling. There was local pain and mild fever with occasional vomiting. There was no history of trauma.
On examination the patient was anemic and had no neurological deficit. There was a 15cm x 20cm globular mass in the left parieto-occipital region with area of ulceration and necrosis of the overlying scalp. The swelling was firm, non-tender, involving the scalp and the underlying bone. It had well-defined margins and was fixed to the bone. Skull roentgenogram revealed a soft tissue mass in the occipital area with bony destruction in the left parieto-occipital region [Figure - 1]. CT scan showed a mixed density mass with bony destruction. The heterogeneously enhancing mass showed involvement of the left occipital cortex [Figure - 2]. Fine needle aspiration cytology (FNAC) was suggestive of a giant cell tumor.
At surgery, the fungating scalp tumor was excised with a rim of healthy bone all around including the intracranial extension and along with the involved dura. The tumor was very vascular, and was seen infiltrating the left occipital lobe. Growth of the tumor into the superior sagittal sinus, left transverse sinus and torcula was seen at surgery. The tumor infiltrating into the occipital lobe was excised but the extension into the venous sinuses was not removed. The defect created by the dural excision was patched with fascia lata. Skin cover for a 10 x 15-cm scalp defect was provided by a rotation flap, based on the superficial temporal artery. The secondary de fect in the scalp was covered by a split thickness skin graft. Cranioplasty was not performed due to the presence of areas of ulceration and infection. Microscopic examination showed the tumor to be poorly encapsulated. There was a prominent plump spindle cell stroma with focal storiform pattern. Marked nuclear pleomorphism and atypia with a few multinucleated giant cells were seen. Areas of necrosis were present. There were no lipoblasts or cells with striations. Inflammatory cells such as lymphocytes and neutrophils were present focally. The combination of pleomorphism and spindled stroma with focal storiform pattern suggested a diagnosis of malignant fibrous histiocytoma. Postoperative radiotherapy was advised but the patient refused further treatment and did not follow up.

 » Case No. 2 Top

A 22-year-old male was admitted with history of a rapidly growing swelling in left temporal region for the past two months. On examination the swelling was situated in the left temporal region just above and anterior to the pinna. It was a 7x5-cm globular, firm and mildly tender mass, fixed to the underlying bone, with well-defined margins. A contrast CT scan demonstrated an enhancing mass in the left temporal region with underlying bone destruction [Figure - 3]. At surgery, the temporal bone was eroded and destroyed but the cortical surface of the brain was intact. The mass was excised along with the middle fossa dura, which was involved. Repair was done using a fascia lata graft. Postoperatively, the patient had a CSF leak, which was managed by a lumbar drain. Postoperative CT showed gross total excision [Figure - 4]. He was referred for radiotherapy. He had a local recurrence 1 month later and was advised chemotherapy but refused treatment. Histological examination showed a pleomorphic tumor composed of short fascicles of spindle cells and plumper histocytic cells [Figure - 5] & [Figure - 6]. Immunostaining for skeletal muscle actin was negative, ruling out a rhabdomyosarcoma. There were no lipoblasts. Other features similar to the previ ous case were present. No syncytial pattern or focal whirling was noted to suggest meningeal origin. Epithelial membrane antigen (EMA) was negative.

 » Discussion Top

Malignant fibrous histiocytoma, in extracranial locations, is the most common soft tissue sarcoma in adults,[8],[16] the most common sites being the retroperitoneum and the deep soft tissue of the extremities.
Primary MFH of the CNS is uncommon. In a review in 1998, Akimoto et al reported one case and described 17 previously reported cases.[15] In both our cases there was no evidence of a primary tumor elsewhere. MFH elsewhere with brain secondaries usually occurs in old age while primary brain MFH occurs in relatively younger patients.[15],[16] Cerebral MFH usually involves the parietal, frontal or temporal lobes, whereas in one of our patients the occipital lobe was involved. The lesion in this patient possibly had a meningeal origin with secondary involvement of the overlying bone and scalp and infiltration of the underlying brain.
Immunohistochemistry has provided evidence that these tumors are not derived from cells of monocytic or macrophage lineage but rather from fibroblasts.[17] In case no. 2 we had performed an immunostain for skeletal muscle actin which was negative, excluding a rhabdomyosarcoma.
Plain roentgenograms in MFH involving bone show a poorly circumscribed lytic lesion with areas of calcification while tomograms reveal subtle permeative radiolucent changes extending beyond the poorly demarcated central area of radiolucency. Angiography shows an extensive neovascular response while isotope studies reveal increased uptake beyond the tumor margin. The differential diagnosis includes cystic gliomas, glioblastoma, meningioma, abscess, metastasis and tuberculomas.[8] MRI findings of an intracranial MFH secondary to an extracranial primary lesion have been described.[18] Here, a cystic lesion with a ring-shaped tumor rim of decreasing signal intensity in T2-weighted image was seen. MRI is superior to CT in demonstrating tumor extent and edema.
The treatment for MFH has been described as a combination of radical excision, radiotherapy and chemotherapy. However, MFH of the brain has always been a gloomy prospect with a relentless course leading to death within the 1st year after surgery.[1] Radical removal in case No.1 would have been extremely formidable due to the involvement of more than one venous sinus converging onto torcular Herophili. The hazards associated with interruption or diversion of venous sinus flow and reconstruction of the torcular Herophili in an attempt to achieve complete resection of a peritorcular malignant tumor seem unwarranted.[19] 

 » References Top

1.Weiss SW. Malignant fibrous histiocytoma. A reaffirmation. Am J Surg Pathol 1992;6:773-84.  Back to cited text no. 1    
2.Gonzalez-Vitale JC, Slavin RE, McQueen JD. Radiation induced intracranial malignant fibrous histiocytoma. Cancer 1976;37:2960-3.  Back to cited text no. 2    
3.Castillo M, Davis PC, Takie YD, et al. Intracranial cystic malignant fibrous histiocytoma in a child: Sonographic and CT findings. Pediatr Radiol 1990;20:194-5.  Back to cited text no. 3    
4.Grant JW, Gallagher PL. Pleomorphic xanthoastrocytoma. Immunohistochemical methods for differentiation from fibrous histiocytomas with similar morphology. Am J Surg pathol 1986;10:336-41.  Back to cited text no. 4    
5.Kalyanaraman UP, Taraska JJ, Fierer JA, et al. Malignant fibrous histiocytoma of the meninges. Histological, ultrastructural and immunocytochemical studies. J Neurosurg 1981;55:957-62.  Back to cited text no. 5    
6.Kepes JJ "Xanthomatous" lesions of the central nervous system: Definition, classification and some recent observations, In Zimmerman HM, editor: Progress in Neuropathology, Vol. 4. New York. Raven Press 1989. p. 179-213.  Back to cited text no. 6    
7.Schrader B, Holland BR, Friedrichsen C. Rare case of primary malignant fibrous histiocytoma of the brain. Neuroradiol 1989;31:177-9.  Back to cited text no. 7    
8.Sima AAF, Ross RT, Hang G, Rozdilsky B, Riocee M. Malignant intracranial fibrous histiocytomas. Histologic, ultrastructural and immunohistochemical studies of two cases. Can J Neurol Sci 1986;13:138-45.  Back to cited text no. 8    
9.Simpson RH, Phillips H, Miller P, et al. Intracerebral malignant fibrous histiocytoma: A light and electron microscope study with immunohistochemistry. Clin Neuropathol 1986;5:185-9.  Back to cited text no. 9    
10.Swamy KS, Shankar SK, Asha I, et al. Malignant fibrous histiocytoma arising from the meninge of the posterior fossa. Surg Neurol 1986;25:18-24.  Back to cited text no. 10    
11.Nakayama K, Nenoto Y, Inow Y, et al. Malignant fibrous histocytoma of the temporal bone with endocranial extension. Am J Neuroradiol 1997;18:331-4.  Back to cited text no. 11    
12.Yoshida D, Harashima K, Node Y, et al. Malignant fibrous histocytoma in the parietal bone. Neurol Med Chir (Tokyo) 1998;38:359-62.  Back to cited text no. 12    
13.Akimoto J, Takeda Y, Hasue M, et al. Primary meningeal malignant fibrous histiocytoma with cerebrospinal dissemination and pulmonary metastasis. Acta Neurochir (Wien) 1998;140:1191-6.  Back to cited text no. 13    
14.Tsutsumi M, Kawano T, Kawaguchi T, et al. Intracranial meningeal malignant fibrous histiocytoma mimicking parasagittal meningioma - case report. Neuro Med Chir (Tokyo) 2001;41:90-3.  Back to cited text no. 14    
15.Meister P. Malignant fibrous histiocytoma. History, histology and histogenesis. Path Res Pract 1988;183:1-7.  Back to cited text no. 15    
16.Espana P, Chang P, Wiemik PH. Increased incidence of brain metastases in sarcoma patients. Cancer 1980;45:377.  Back to cited text no. 16    
17.Wood GS, Beckstead JG, Turner RR, et al. Malignant fibrous histiocytoma turnover cells resemble fibroblasts. Am J Surg Pathol 1986;10:233.  Back to cited text no. 17    
18.Enjoji M, Hashimoto H, Tsuneyoshi M, et al. Malignant fibrous histiocytoma. Acta Pathol 1980;30:727.  Back to cited text no. 18    
19.Harsh IV GR, Wilson CB. Peritorcular meningiomas. In Wilkins RH, Rengachary SS, editor: Neurosurgery Updated New York McGraw Hill 1990, p. 428-33.  Back to cited text no. 19    


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