Departments of Pathology (Y-SH, C-HW), Neurosurgery (M-DT), and Radiology (Y-YW), Chang Gung Medical College and Chang Gung Memorial Hospital, Taipei, Taiwan, Republic of China Neurosurgery 31; 567-570, 1992 ABSTRACT: A 50-YEAR-OLD woman developed an intracerebral malignant fibrous histiocytoma at the site of a previously clipped aneurysm in the right temporal lobe. This tumor rarely originates within the brain. Review of the literature suggests that postoperative and/or postirradiation effects may contribute to the growth of this particular tumor. KEY WORDS: Intracerebral; Malignant fibrous histiocytoma Malignant fibrous histiocytoma (MFH), also referred to as malignant fibrous xanthoma (15,19) or fibroxanthosarcoma (11,18), is a predominantly pleomorphic sarcoma. It was first described about 20 years ago (19,25). The classification refers to a "malignant tumor consisting of partly pleomorphic, atypical histiocyte- and fibroblast-like cells, with storiform pattern" (World Health Organization) (5,24). Today, MFH is considered the most common soft tissue sarcoma of old age. It accounts for 10.5 to 21.6% of all malignant neoplasia of soft tissue (4,29), arising preferentially in the retroperitoneum or the extremities. MFH is particularly common in the fifth to seventh decades of life (4,28) but can be observed in all age groups, with a predominance in men (16,28). Since its early description by O'Brien and Stout (19), it has remained a controversial entity because of its uncertain histogenesis. Although it has been considered by some investigators to be of histiocytic origin (10,11,20), others have suggested an origin from a primitive mesenchymal cell (6). Irrespective of its precise histogenesis, the tumor contains both fibroblast-like and histiocyte-like cells in varying proportions, accounting in part for its classic form. MFH is composed of spindled (fibroblast-like) and rounded (histiocyte-like) cells arranged in a storiform pattern and accompanied by pleomorphic giant cells and inflammatory cells. Primary MFH involving the central nervous system is a rare occurrence (3,7,8,25-27), with most cases originating from the dura or the leptomeninges. In 1986, Sima et al. (25) reported for the first time a case of intracerebral MFH. Our study reports the case of a patient with an intracerebral MFH that developed at the site of an aneurysm that was clipped 5 months earlier. We review the
CASE REPORT A 50-year-old woman was admitted to Chang Gung Memorial Hospital, Taipei, Taiwan, for evaluation of a right temporal lobe mass, which was seen by computed tomographic scan during the investigation of headache and left motor weakness. The patient had had right pericallosal artery and posterior communicating artery aneurysms, which were clipped 5 and 3 months before admission by interhemispheric and right pterional approaches, respectively. On physical examination, the patient was afebrile, alert, and active. Her pupils were equal and reactive with full visual fields to confrontation. Cranial nerve examination revealed a mild left facial palsy and left deviation of the tongue. The left leg was weak with muscle power at Grade 4. Positive Romberg test and difficulty with tandem gait were the only other findings. Chest and kidney ureter bladder x-ray studies and laboratory data including a complete blood count examination did not reveal any abnormalities. A computed tomographic scan of the head revealed a right temporal mass measuring 4.5 cm in largest dimension, with central and peripheral contrast enhancement (Fig. 1). There was associated surrounding edema with right-to-left shift of the midline structures. Cerebral angiogram revealed no significant tumor stain but showed a local mass effect. The radiographic findings suggested possible hematoma, low-grade astrocytoma, or metastatic disease. Operation A right temporoparietal craniotomy was performed on June 19, 1991. Through the corticotomy at the superior temporal gyrus, the main tumor was intraparenchymatous and was identified by its reddish color. The tumor was intraparenchymatous, but with a clear demarcation from the surrounding brain parenchyma. The central part of the tumor contained necrotic tissue and old liquefying hematoma. After effective central debulking, the tumor could be removed totally. The capsule of the tumor adhered to the temporal dura but was easily separated from it. Effective coagulation at the site of adhesion to the dura was achieved. Postoperative course The patient ran an uneventful postoperative course. She recovered from increased intracranial pressure and left motor weakness satisfactorily. Pathological findings Grossly, the tumor was well circumscribed and dark brown in color with irregular granulations and extensively liquefied hemorrhagic necrotic areas. Frozen section of the intraoperative biopsy showed a very cellular malignant-appearing tumor containing two types of giant cells, osteoclast-like and pleomorphic giant cells, mixed in with a few foamy histiocytes. There were large areas of hemorrhage and necrosis. The diagnosis of giant-cell-type MFH was
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AUTHOR(S): Ho, Yat-Sen, M.D.; Wei, ChienHwa, M.D.; Tsai, Ming-Dar, M.D.; Wai, YauYau, M.D.
literature, with special emphasis on histogenesis and the possibility of a traumatic etiology.
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Neurosurgery 1992-98 September 1992, Volume 31, Number 3 567 Intracerebral Malignant Fibrous Histiocytoma: Case Report and Review of the Literature Case Report
ACKNOWLEDGMENTS
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
DISCUSSION MFH is a pleomorphic tumor that, on light microscopic study, is composed of variable mixtures of bizarre fibroblasts, histiocytes, multinucleated giant cells, and foamy lipid-filled xanthomatous cells arranged in a storiform (pinwheel or cartwheel) pattern with a fibrous or myxoid stroma. Other terms for MFH include xanthosarcoma, fibroxanthosarcoma, and malignant xanthoma. An MFH arising intracerebrally is rare. Only five cases of cerebral MFH have been reported in the literature. These are listed in Table 1 (3,21-23,25,26). Central nervous system MFHs tend to present as large masses at the time of diagnosis and most commonly involve the parietal, frontal, or temporal lobes (3). Our case is of interest because of the possibility of a traumatic etiology, a controversial concept. Strict criteria are necessary to establish a causal relation. The following criteria have been suggested by several investigators (13,17,30): 1) adequacy of the trauma, 2) identical location of the trauma and the tumor, 3) adequate latent period between the trauma and the appearance of the tumor (usually several years), 4) histological confirmation of the tumor, 5) local
symptoms during the latent period, and 6) no known central nervous system illness before the traumatic event. Our case satisfies all of the above-mentioned criteria, except the adequate latent period between the trauma and the appearance of the tumor. A latency period of 10 months after trauma has been reported (2). Central nervous system MFH is an uncommon tumor and the most controversial aspect of this tumor is its histogenesis. In discussing the cell of origin, Lam and Colah (15) suggested a derivation from the mesenchymal stem cells of the dura. Simpson et al. (26) stated that MFH within both the meninges and the brain is still of pia-arachnoidal or meningeal origin. Sima et al. (25) thought that the tumor in their first case originated from the meninges and the tumor in their second case (an intracerebral MFH) was derived from perivascular mesenchymal cells. Iwasaki et al. (9) suggested that it is possible for MFH to develop within the brain from deep blood vessel walls without originating from the meninges. Earlier studies suggested a histiocytic derivation (1, 10-12,20) . More recently, origin from undifferentiated multipotent mesenchymal cells has been proposed (6, 14,18,28) . The present light and electron microscopic findings demonstrating fibroblasts, histiocytes, xanthomatous cells, and multinucleated cells of the Touton type, as well as small undifferentiated cells qualifying as stem cell forms as described by Fu et al. (6) , give further support to the contention that this lesion arose from a primitive mesenchymal cell. In our case, although we cannot entirely rule out meningeal origin of the tumor, we think it was of intraparenchymatous origin because it easily separated from the adhered dura. The immunohistochemical staining included GFAP, vimentin, lysozyme, AAT, S-100, and α-1antichymotrypsin. The results were negative for GFAP and S-100, excluding the possibility of an astroglial tumor and neurilemoma, and positive for vimentin, lysozyme, and AAT. This indicated that the tumor is of mesenchymal cell origin with differentiation of the cells along both fibroblastic and histiocytic lines. The electron microscopic studies, furthermore, confirmed that at least two cell types were present: one resembled a fibroblast, containing organized lamellae of rough endoplasmic reticulum; a second cell resembled a histiocyte and contained numerous lysosomes and phagocytic vacuoles. Based on the recent demonstration of small numbers of undifferentiated mesenchymal cells in ultrastructural studies and tissue culture (6,29), we agree with those who consider MFH a primitive mesenchymal tumor showing partial fibroblastic and histiocytic differentiation. So far, the suggested treatment for MFH is gross surgical removal, followed by radiotherapy and/or chemotherapy (29). Prognosis, however, remains grave in spite of adjuvant therapy. The value and utility of the data from larger series of patients with MFH of the soft tissues for cases with primarily intracerebral MFH is not known and needs further investigation.
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established on the basis of these features. Paraffin sections were stained with hematoxylin and eosin and Gomori's trichrome stain for collagen. Selected blocks were stained with monoclonal mouse antisera to glial fibrillary acidic protein (GFAP), vimentin, S100, α-1-antitryp-sin (AAT), α-1-antichymotrypsin, and lysozyme (all from Boehringer Mann-heim GmbH, Germany). Tissue for electron microscopy was fixed in glutaraldehyde, postfixed in osmium tetroxide, dehydrated in graded alcohols, and embedded in Epon. Sections for ultrastructural were examined with the Jeol-1200 CX electron microscope (Jeol LTD, Tokyo, Japan). The appearance of the tumor in paraffin sections was the same as in the frozen section. The tumor was composed of pleomorphic foamy histiocytes with a prominence of mono- and multinuclear (osteoclastlike) giant cells (Fig. 2). Fibroblast-like tumor cells were interspersed randomly throughout the lesion. The overall mitotic rate was high. In several foci, there was necrosis and hemorrhage. The tumor was relatively well circumscribed with distinct margins against the adjacent brain which, in turn, showed reactive gliosis. Dural invasion was also noted. Immunohistochemically, the tumor cells showed faint and scattered positivity for AAT, α-1antichymotrypsin, lysozyme, and vimentin (Fig. 3), but there was no staining for GFAP. Electron microscopy revealed that the tumor cells had many lysosomes and phagosomes and a few lipid droplets: features of histiocytic cells or activated monocytes (Fig. 4). The scattered more spindle-shaped cells had features of fibroblastic differentiation including ample rough endoplasmic reticulum and free polyribosomes (data not shown). There were no characteristics to suggest meningothelial differentiation such as desmosomes or interdigitating cell processes. The diagnosis of malignant fibrous histiocytoma, giant-cell type, was made.
13. 14.
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Alguacil-Garcia A, Unni KK, Goellner JR: Malignant fibrous histiocytoma, an ultrastructural study of six cases. Am J Clin Pathol 69:121-129, 1978. Bader H, Spohner F, Gerlitzky W, Meyer D: Malignant fibroses Histiozytom nach perikondylärer Oberschenkelfraktur. Dtsch Med Wochenschr 16:336-339, 1981. Berry AD, Reintjes SL, Kepes JJ: Intracranial malignant fibrous histiocytoma with abscesslike tumor necrosis. J Neurosurg 69:780-784, 1988. Enjoji M, Hasimoto H, Tsuneyoshi M, Iwasaki H: Malignant fibrous histiocytoma. Acta Pathol 30:727-741, 1980. Enzinger FM, Lattes R, Torloni H: Histological typing of soft tissue tumors, in International Histological Classification of Tumors, No. 3. Geneva, World Health Organization, 1969. Fu YS, Gabbiani G, Kaye GI, Latters R: Malignant soft tissue tumors of probable histiocyte origin (malignant fibrous histiocytomas): General considerations and electron microscopic and tissue culture studies. Cancer 35:176-198, 1975. Grant JW, Gallagher PJ: Pleomorphic xanthoastrocytoma. Immuno- histochemical methods for differentiation from fibrous histiocytomas with similar morphology. Am J Surg Pathol 10:336-341, 1986. Helle TL, Hanbery JW, Becker DH: Meningeal malignant fibrous histiocytoma arising from a thoracolumbar myelomeningocele: Case report. J Neurosurg 58:185-189, 1986. Iwazaki H, Isayama T, Johzaki H: Malignant fibrous histiocytoma. Evidence of perivascular mesenchymal cell origin, immunocytochemical studies with monoclonal anti-MFH antibodies. Am J Pathol 128:528-537, 1987. Kauffman SL, Stout AP: Histiocytic tumors (fibrous xanthoma and histiocytoma) in children. Cancer 14:469-482, 1961. Kempson RL, Kyriakos M: Fibroxanthosarcoma of the soft tissue. A type
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Received, October 18, 1991. Accepted, March 20, 1992. Reprint requests: Yat-Sen Ho, M.D., Department of Pathology, Chang Gung Memorial Hospital, 199 Tung Hwa North Road, Taipei, Taiwan, 10591 Republic of China.
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of malignant fibrous histiocytoma. Cancer 29:961-976, 1972. Kobak MW, Perlow S: Xanthomatous giant cell tumors arising in soft tissue. Report on an instance of malignant growth. Arch Surg 59:909- 916, 1949. Kramer G: Hirntumor nach trauma: Literaturubersicht und Begutachtungs-Problematik. AKT Neurol 9:112-120, 1982. Lagace R, Delage C, Siemayer TA: Myxoid variant of malignant fibrous histiocytoma. Ultrastructural observation. Cancer 43:526534, 1979. Lam RMY, Colah SA: Atypical fibrous histiocytoma with myxoid stroma. A rare lesion arising from dura mater of the brain. Cancer 43:237-245, 1979. Limacher J, Delage C, Lagace R: Malignant fibrous histiocytoma. Am J Surg Pathol 2:265274, 1987. Mendel K: Der Umfall in der Aetiologie der Nervenkrankheiten. Mschr Psych Neurol 22:158-173, 1907. Merkow LP, Frich JC, Sliekin M, Kyreages CG, Pardo M: Ultrastructure of a fibroxanthosarcoma (malignant fibroxanthoma) Cancer 28:372-383, 1971. O'Brien JE, Stout AP: Malignant fibrous xanthomas. Cancer 17:1445-1548, 1964. Ozzello L, Stout AP, Murray MR: Cultural characteristics of malignant histiocytomas and fibrous xanthomas. Cancer 16:331-344, 1963. Paulus W, Peiffer J, Grote E: Intracerebral malignant fibrous histiocytoma at site of a previously excised low grade glioma. Acta Neurochir (Wien) 99:161-165, 1989. Paulus W, Slowik F, Jellinger K: Primary intracranial sarcomas: Histopathological features of 19 cases. Histopathology 18:395402, 1991. Roosen N, Cras P, Paquier P, Martin JJ: Primary thalamic malignant fibrous histiocytoma of the dominant hemisphere causing severe neuropsychological symptoms. Clin Neuropathol 8:16-21, 1989. Schrader B, Holland BR, Friedrichsen C: Rare case of primary malignant fibrous histiocytoma of the brain. Neuroradiology 31:177-179, 1989. Sima AAF, Ross RT, Hoag G, Rozdilsky B, Diocee M: Malignant intracranial fibrous histiocytomas. Histological, ultrastructural and immunohistochemical studies of two cases. Can J Neurol Sci 13:138-145, 1986. Simpson RHW, Phillips JI, Miller P, Hagen D, Anderson JEM: Intracerebral malignant fibrous histiocytoma: A light and electron microscopic study with immunohistochemistry. Clin Neuropathol 5:185-189, 1986. Swamy KSN, Shankar SK, Asha T: Malignant fibrous histiocytoma arising from the meninges of the posterior fossa. Surg Neurol 25:18-24, 1986.
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
The authors thank our visiting Professor Dr. Jason L. Starr, Medical Oncologist and Professor, University of Southern California, for his valuable comments. This study was supported in part by National Science Council Research Grant NSC 81-0412-B18232 from the Republic of China.
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John J. Kepes Kansas City, Kansas REFERENCES: (1,2) 1. 2.
Enzinger FM: Malignant fibrous histiocytoma 20 years after Stout. Am J Surg Pathol [Suppl] 10:43-53, 1986. Fletcher CDM: Pleomorphic malignant fibrous histiocytoma: Fact or fiction? A critical reappraisal based on 159 tumors diagnosed as pleomorphic sarcoma. Am J Surg Pathol 16:213-228, 1992.
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COMMENT Malignant fibrous histiocytomas are presently regarded as the most common soft tissue sarcomas of adulthood (1). Naturally, the brain having relatively little connective tissue, this tumor is rarely seen within the cerebrum; it is more likely to originate from the meninges, when intracranial. Vascular adventitia within the brain parenchyma, however, may serve as site of origin for this and other connective tissue neoplasms. Some of these patients develop a "postradiation sarcoma," for example, after irradiation of a pituitary adenoma. In the sarcomatous component of gliosarcomas, or Feigin tumors, the pattern of a malignant fibrous histiocytoma may at times be recognized. Vascular adventitial cells should probably be credited (or blamed) for the development of an intracerebral malignant fibrous histiocytoma in the patient of Dr. Ho and co-workers, also. It is interesting that recently it has been proposed that this tumor may represent a histogenetically heterogeneous group: with appropriate immunochemical staining some eventually could be proven to represent leiomyosarcomas, rhabdomyosarcomas, extraskeletal osteosarcomas, and others, even including poorly differentiated spindle cell epithelial neoplasms (2). The authors' case, however, appears as "genuine" histologically as possible, but neither α-1-antitrypsin nor α-1-antichymo-trypsin positivity in tumor cells should be quoted as being proof for histiocytic origin, because these stains have been shown to be largely nonspecific, positive as they often are in astrocytomas, glioblastomas, and even carcinomas.
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28.
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Figure 2. Two types of large cells, osteoclast-like and pleomorphic giant cells, are visible (×66).
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Figure 1. Contrast-enhanced computed tomographic scan showing a space-occupying lesion with some perifocal hypodensity of the right temporal lobe.
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Figure 4. Electron microscopy shows a giant cell with dense heterochromic nuclei and abundant lysosomes in the cytoplasm (×5000).
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Figure 3. By immunohistochemical study, both types of giant cells are positive with AAT (×132).
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Table 1. Clinical and Pathological Findings in Six Cases of Primary Intracerebral Malignant Fibrous Histiocytomaa
CASE REPORT A 50-year-old woman was admitted to Chang Gung Memorial Hospital, Taipei, Taiwan, for evaluation of a right temporal lobe mass, which was seen by computed tomographic scan during the investigation of headache and left motor weakness. The patient had had right pericallosal artery and posterior communicating artery aneurysms, which were clipped 5 and 3 months before admission by interhemispheric and right pterional approaches, respectively. On physical examination, the patient was afebrile, alert, and active. Her pupils were equal and reactive with full visual fields to confrontation. Cranial nerve examination revealed a mild left facial palsy and left deviation of the tongue. The left leg was weak with muscle power at Grade 4. Positive Romberg test and difficulty with tandem gait were the only other findings. Chest and kidney ureter bladder x-ray studies and laboratory data including a complete blood count examination did not reveal any abnormalities. A computed tomographic scan of the head revealed a right temporal mass measuring 4.5 cm in largest dimension, with central and peripheral contrast enhancement (Fig. 1). There was associated surrounding edema with right-to-left shift of the midline structures. Cerebral angiogram revealed no significant tumor stain but showed a local mass effect. The radiographic findings suggested possible hematoma, low-grade astrocytoma, or metastatic disease. Operation A right temporoparietal craniotomy was performed on June 19, 1991. Through the corticotomy at the superior temporal gyrus, the main tumor was intraparenchymatous and was identified by its reddish color. The tumor was intraparenchymatous, but with a clear demarcation from the surrounding brain parenchyma. The central part of the tumor contained necrotic tissue and old liquefying hematoma. After effective central debulking, the tumor could be removed totally. The capsule of the tumor adhered to the temporal dura but was easily separated from it. Effective coagulation at the site of adhesion to the dura was achieved. Postoperative course The patient ran an uneventful postoperative course. She recovered from increased intracranial pressure and left motor weakness satisfactorily. Pathological findings Grossly, the tumor was well circumscribed and dark brown in color with irregular granulations and extensively liquefied hemorrhagic necrotic areas. Frozen section of the intraoperative biopsy showed a very cellular malignant-appearing tumor containing two types of giant cells, osteoclast-like and pleomorphic giant cells, mixed in with a few foamy histiocytes. There were large areas of hemorrhage and necrosis. The diagnosis of giant-cell-type MFH was
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AUTHOR(S): Ho, Yat-Sen, M.D.; Wei, ChienHwa, M.D.; Tsai, Ming-Dar, M.D.; Wai, YauYau, M.D.
literature, with special emphasis on histogenesis and the possibility of a traumatic etiology.
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Neurosurgery 1992-98 September 1992, Volume 31, Number 3 567 Intracerebral Malignant Fibrous Histiocytoma: Case Report and Review of the Literature Case Report
ACKNOWLEDGMENTS
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
DISCUSSION MFH is a pleomorphic tumor that, on light microscopic study, is composed of variable mixtures of bizarre fibroblasts, histiocytes, multinucleated giant cells, and foamy lipid-filled xanthomatous cells arranged in a storiform (pinwheel or cartwheel) pattern with a fibrous or myxoid stroma. Other terms for MFH include xanthosarcoma, fibroxanthosarcoma, and malignant xanthoma. An MFH arising intracerebrally is rare. Only five cases of cerebral MFH have been reported in the literature. These are listed in Table 1 (3,21-23,25,26). Central nervous system MFHs tend to present as large masses at the time of diagnosis and most commonly involve the parietal, frontal, or temporal lobes (3). Our case is of interest because of the possibility of a traumatic etiology, a controversial concept. Strict criteria are necessary to establish a causal relation. The following criteria have been suggested by several investigators (13,17,30): 1) adequacy of the trauma, 2) identical location of the trauma and the tumor, 3) adequate latent period between the trauma and the appearance of the tumor (usually several years), 4) histological confirmation of the tumor, 5) local
symptoms during the latent period, and 6) no known central nervous system illness before the traumatic event. Our case satisfies all of the above-mentioned criteria, except the adequate latent period between the trauma and the appearance of the tumor. A latency period of 10 months after trauma has been reported (2). Central nervous system MFH is an uncommon tumor and the most controversial aspect of this tumor is its histogenesis. In discussing the cell of origin, Lam and Colah (15) suggested a derivation from the mesenchymal stem cells of the dura. Simpson et al. (26) stated that MFH within both the meninges and the brain is still of pia-arachnoidal or meningeal origin. Sima et al. (25) thought that the tumor in their first case originated from the meninges and the tumor in their second case (an intracerebral MFH) was derived from perivascular mesenchymal cells. Iwasaki et al. (9) suggested that it is possible for MFH to develop within the brain from deep blood vessel walls without originating from the meninges. Earlier studies suggested a histiocytic derivation (1, 10-12,20) . More recently, origin from undifferentiated multipotent mesenchymal cells has been proposed (6, 14,18,28) . The present light and electron microscopic findings demonstrating fibroblasts, histiocytes, xanthomatous cells, and multinucleated cells of the Touton type, as well as small undifferentiated cells qualifying as stem cell forms as described by Fu et al. (6) , give further support to the contention that this lesion arose from a primitive mesenchymal cell. In our case, although we cannot entirely rule out meningeal origin of the tumor, we think it was of intraparenchymatous origin because it easily separated from the adhered dura. The immunohistochemical staining included GFAP, vimentin, lysozyme, AAT, S-100, and α-1antichymotrypsin. The results were negative for GFAP and S-100, excluding the possibility of an astroglial tumor and neurilemoma, and positive for vimentin, lysozyme, and AAT. This indicated that the tumor is of mesenchymal cell origin with differentiation of the cells along both fibroblastic and histiocytic lines. The electron microscopic studies, furthermore, confirmed that at least two cell types were present: one resembled a fibroblast, containing organized lamellae of rough endoplasmic reticulum; a second cell resembled a histiocyte and contained numerous lysosomes and phagocytic vacuoles. Based on the recent demonstration of small numbers of undifferentiated mesenchymal cells in ultrastructural studies and tissue culture (6,29), we agree with those who consider MFH a primitive mesenchymal tumor showing partial fibroblastic and histiocytic differentiation. So far, the suggested treatment for MFH is gross surgical removal, followed by radiotherapy and/or chemotherapy (29). Prognosis, however, remains grave in spite of adjuvant therapy. The value and utility of the data from larger series of patients with MFH of the soft tissues for cases with primarily intracerebral MFH is not known and needs further investigation.
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established on the basis of these features. Paraffin sections were stained with hematoxylin and eosin and Gomori's trichrome stain for collagen. Selected blocks were stained with monoclonal mouse antisera to glial fibrillary acidic protein (GFAP), vimentin, S100, α-1-antitryp-sin (AAT), α-1-antichymotrypsin, and lysozyme (all from Boehringer Mann-heim GmbH, Germany). Tissue for electron microscopy was fixed in glutaraldehyde, postfixed in osmium tetroxide, dehydrated in graded alcohols, and embedded in Epon. Sections for ultrastructural were examined with the Jeol-1200 CX electron microscope (Jeol LTD, Tokyo, Japan). The appearance of the tumor in paraffin sections was the same as in the frozen section. The tumor was composed of pleomorphic foamy histiocytes with a prominence of mono- and multinuclear (osteoclastlike) giant cells (Fig. 2). Fibroblast-like tumor cells were interspersed randomly throughout the lesion. The overall mitotic rate was high. In several foci, there was necrosis and hemorrhage. The tumor was relatively well circumscribed with distinct margins against the adjacent brain which, in turn, showed reactive gliosis. Dural invasion was also noted. Immunohistochemically, the tumor cells showed faint and scattered positivity for AAT, α-1antichymotrypsin, lysozyme, and vimentin (Fig. 3), but there was no staining for GFAP. Electron microscopy revealed that the tumor cells had many lysosomes and phagosomes and a few lipid droplets: features of histiocytic cells or activated monocytes (Fig. 4). The scattered more spindle-shaped cells had features of fibroblastic differentiation including ample rough endoplasmic reticulum and free polyribosomes (data not shown). There were no characteristics to suggest meningothelial differentiation such as desmosomes or interdigitating cell processes. The diagnosis of malignant fibrous histiocytoma, giant-cell type, was made.
13. 14.
15.
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Alguacil-Garcia A, Unni KK, Goellner JR: Malignant fibrous histiocytoma, an ultrastructural study of six cases. Am J Clin Pathol 69:121-129, 1978. Bader H, Spohner F, Gerlitzky W, Meyer D: Malignant fibroses Histiozytom nach perikondylärer Oberschenkelfraktur. Dtsch Med Wochenschr 16:336-339, 1981. Berry AD, Reintjes SL, Kepes JJ: Intracranial malignant fibrous histiocytoma with abscesslike tumor necrosis. J Neurosurg 69:780-784, 1988. Enjoji M, Hasimoto H, Tsuneyoshi M, Iwasaki H: Malignant fibrous histiocytoma. Acta Pathol 30:727-741, 1980. Enzinger FM, Lattes R, Torloni H: Histological typing of soft tissue tumors, in International Histological Classification of Tumors, No. 3. Geneva, World Health Organization, 1969. Fu YS, Gabbiani G, Kaye GI, Latters R: Malignant soft tissue tumors of probable histiocyte origin (malignant fibrous histiocytomas): General considerations and electron microscopic and tissue culture studies. Cancer 35:176-198, 1975. Grant JW, Gallagher PJ: Pleomorphic xanthoastrocytoma. Immuno- histochemical methods for differentiation from fibrous histiocytomas with similar morphology. Am J Surg Pathol 10:336-341, 1986. Helle TL, Hanbery JW, Becker DH: Meningeal malignant fibrous histiocytoma arising from a thoracolumbar myelomeningocele: Case report. J Neurosurg 58:185-189, 1986. Iwazaki H, Isayama T, Johzaki H: Malignant fibrous histiocytoma. Evidence of perivascular mesenchymal cell origin, immunocytochemical studies with monoclonal anti-MFH antibodies. Am J Pathol 128:528-537, 1987. Kauffman SL, Stout AP: Histiocytic tumors (fibrous xanthoma and histiocytoma) in children. Cancer 14:469-482, 1961. Kempson RL, Kyriakos M: Fibroxanthosarcoma of the soft tissue. A type
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Received, October 18, 1991. Accepted, March 20, 1992. Reprint requests: Yat-Sen Ho, M.D., Department of Pathology, Chang Gung Memorial Hospital, 199 Tung Hwa North Road, Taipei, Taiwan, 10591 Republic of China.
12.
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The authors thank our visiting Professor Dr. Jason L. Starr, Medical Oncologist and Professor, University of Southern California, for his valuable comments. This study was supported in part by National Science Council Research Grant NSC 81-0412-B18232 from the Republic of China.
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COMMENT Malignant fibrous histiocytomas are presently regarded as the most common soft tissue sarcomas of adulthood (1). Naturally, the brain having relatively little connective tissue, this tumor is rarely seen within the cerebrum; it is more likely to originate from the meninges, when intracranial. Vascular adventitia within the brain parenchyma, however, may serve as site of origin for this and other connective tissue neoplasms. Some of these patients develop a "postradiation sarcoma," for example, after irradiation of a pituitary adenoma. In the sarcomatous component of gliosarcomas, or Feigin tumors, the pattern of a malignant fibrous histiocytoma may at times be recognized. Vascular adventitial cells should probably be credited (or blamed) for the development of an intracerebral malignant fibrous histiocytoma in the patient of Dr. Ho and co-workers, also. It is interesting that recently it has been proposed that this tumor may represent a histogenetically heterogeneous group: with appropriate immunochemical staining some eventually could be proven to represent leiomyosarcomas, rhabdomyosarcomas, extraskeletal osteosarcomas, and others, even including poorly differentiated spindle cell epithelial neoplasms (2). The authors' case, however, appears as "genuine" histologically as possible, but neither α-1-antitrypsin nor α-1-antichymo-trypsin positivity in tumor cells should be quoted as being proof for histiocytic origin, because these stains have been shown to be largely nonspecific, positive as they often are in astrocytomas, glioblastomas, and even carcinomas.
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Figure 2. Two types of large cells, osteoclast-like and pleomorphic giant cells, are visible (×66).
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Figure 1. Contrast-enhanced computed tomographic scan showing a space-occupying lesion with some perifocal hypodensity of the right temporal lobe.
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Figure 4. Electron microscopy shows a giant cell with dense heterochromic nuclei and abundant lysosomes in the cytoplasm (×5000).
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Figure 3. By immunohistochemical study, both types of giant cells are positive with AAT (×132).
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Table 1. Clinical and Pathological Findings in Six Cases of Primary Intracerebral Malignant Fibrous Histiocytomaa
