Journal of Surgical Oncology 44: 166-170 (1990)
Malignant Fibrous Histiocytoma of Bone REKHA V. KUMAR, MD, GEETASHREE MUKHERJEE, MD, AND M . KRISHNA BHARGAVA, MD From the Department of Pathology, Kidwai Memorial Institute of Oncology, Bangalore, India
Malignant fibrous histiocytoma (MFH) of bone is a malignant primary bone tumour that is being increasingly recognised, as more details emerge in the literature regarding its natural history and precise (although sometimes diverse) histological appearance. When the whole bulk of a malignant bone tumour fits the criteria laid down, the designation of MFH seems appropriate. Seven cases of MFH of bone were encountered from a total of 220 primary malignant bone tumours in our files over a 4-year period. The metaphyses of long bones were the most common sites harbouring the tumour, and a wide age range was represented. Amputation was the treatment of choice in all cases. The relevant literature is reviewed. KEY WORDS:sarcoma, pleomorphism, skeleton
INTRODUCTION Malignant fibrous histiocytoma (MFH) is a pleomorphic sarcoma that most often arises in the soft tissues of the trunk and extremities. It has now been well established that bone may be the primary site of MFH, quite distinct from its involvement by direct extension of a soft tissue tumour or by metastatic spread [Feldman and Norman, 1972; Spanier et al., 1975; Dahlin et al.. 19771. Primary MFH of bone has been fairly extensively reported in the literature [Feldman and Norman, 1972; Spanier et al., 1975; HUVOS,1976; Feldman and Lattes, 1977; Mirra et al., 1977; Michael and Dorfman, 1977; Kahn et al., 1978; McCarthy et al., 19791. A few of the reported cases have been associated with bone infarcts [Spanier et al., 1975; Michael and Dorfman, 1977; Mirra et al., 1977; Galli et al., 1978; McCarthy et al., 19791. Seven cases of primary MFH of bone encountered over a 4-year period (1985-1988) at the Kidwai Memorial Institute of Oncology (KMIO) were studied. The salient microscopic findings that led to the diagnosis after other tumours had been excluded or overruled are discussed, together with a review of the relevant literature. MATERIALS AND METHODS Seven cases of MFH of bone were encountered from a Of 220 primary bone at the KMIO over a 4-year period (1985-1988). Only those 0 1990 Wiley-Liss, Inc.
tumours having a histology permitting classification as MFH according to the criteria outlined for soft-tissue tumours by Soule and Enriquez [ 19721 and Kempson and Kyriakos [ 19721 were studied. Haematoxylin and eosin (H & E) stained paraffin sections were studied in all cases. Wilder’s reticulin stain, Masson’s trichrome stain, Van Gieson, Sudan 111, and Alcian blue stains were available in most cases. Radiographs and clinical data were available for all inpatients. The histologic material from one additional case was referred to our pathology department for consultation. Because the cases were relatively recent, the follow-up period was necessarily short, with the longest follow-up being 3 years.
OBSERVATIONS Clinical Data Six of seven patients were male. The average age was 28; the youngest patient was 14 and the oldest was 58. The most common presenting symptoms were pain and swelling (six of seven cases) and were associated with trauma in two cases. Serum alkaline phosphatase levels were done in four cases and were within normal limits in three cases, whereas the fourth case registered a mildly
Accepted for publication February 4, 1990. Address reprint requests to Dr. R e b a V. Kumar. Lecturer in Pathology, Kidwai Memorial Institute of Oncology. Hosur Road, Bangalore 560 029, India.
Malignant Fibrous Histiocytorna of Bone
Fig. I . Roentgenogram of turnour at upper end of tibia showing a predominantly osteolytic lesion.
elevated value of 66 IU/liter, with normal values between 20 and SO IU/liter. Six lesions occurred in the end of a long bone, affecting the metaphysis, and often the adjacent epiphyseal and diaphyseal regions, three lesions in the proximal tibia, one each in the distal tibia, distal femur, and proximal humerus. One lesion occurred in the mandible. Koentgenograms showed most of the tumours as illdefined osteolytic areas, with endosteal erosion. There was minimal reactive bone formation, with no evidence of tumour calcification (Fig. 1). In two cases, there was an associated pathological fracture. The most common x-ray diagnosis was osteolytic osteosarcoma. One case was interpreted as a benign bone tumour.
Treatment Surgery was the treatment of choice in all cases. Amputation (including limb disarticulation) and hemimandiblectomy were the operative procedures. Two cases received preoperative chemotherapy based on clinical and radiological features suggestive of osteosarcoma. Radiotherapy was not given to any case. The longest follow-up was 3 years; the patient is alive and well. Two of the seven cases developed pulmonary metastases and succumbed 6 and 8 months following surgery.
167
Pathological Features Gross pathology. All lesions where the gross specimen was received (in six of seven cases) were destructive tumours and associated with a pathological fracture in two cases. The tumours in the long bones showed erosion and destruction of the cortex, with extension into the adjacent soft tissues, often with a “pushing” border. Multiple tumour nodules in the soft tissue were also observed. The cut surfaces were variegated. Most of the tumours were grey-white and fleshy, with areas of haemorrhage and necrosis. There was variation in consistency, some tumours being soft and others firm to hard. Microscopic pathology. There was evidence of both fibroblastic and histiocytic differentiation in all tumours. Formation of storiform or cartwheel patterns by spindle cells and collagen production were evidence of fibroblastic differentiation (Fig. 2). The histiocytic element was represented by foam cells, erythrophagocytosis, and giant cell formation in cells having the cytologic characteristics of histiocytes, i.e., folded or lobulated vesicular nuclei, many with prominent, acidophilic nucleoli, and rather abundant cytoplasm. Pleomorphism of the histiocytic element was seen in many cases. The multinucleated giant cells were usually pleomorphic tumour giant cells in areas of histiocytic differentiation (Fig. 3). However, osteoclast-like giant cells and an occasional Langhans giant cell were also observed. In most cases, there was a diffuse intermingling of both fibroblastic and histiocytic elements. Detailed study of 20-25 sections per case was sufficient to identify both elements. No tumour osteoid was seen. In one case, there was a uniform infiltration of inflammatory cells composed of lymphocytes, polymorphonuclear leukocytes, and plasma cells, which in some areas obscured the neoplastic cells (Fig. 4).This variant has a soft tissue counterpart in the inflammatory variant of MFH as described by Kempson and Kyriakos [ 19721. It was noticed that a milder round and polymorphonuclear cellular infiltrate was present even in the other turnours. The malignant nature of these tumours was generally obvious with numerous mitotic figures (often atypical) and hyperchromatic, pleomorphic nuclei. In all instances, the usual criteria for malignancy used for other sarcomas of bone were readily identifiable. No evidencc of lymph node metastasis was provided by this series.
DISCUSSION MFH is a relatively recently described and controversial tumour. It has an uncertain histogenesis. Some authors believe that the cell of origin is the tissue histiocyte [Kempson and Kyriakos, 1972; Ozzello et al., 19631. which has the propensity to assume fibroblastic features
168
Kumar et al.
Fig. 2.
Area of tuinour composed of fairly unifomi fibrocytic type cells growing in a storiform pattern.
H & E,
X
100.
Fig. 3 . Giant cell in area of histiocytic differentiation exhibiting phagocytosed pol ymorphs. H & E. x 1 .ooo.
and to lay down collagen (facultative fibroblasts). Others though one or another of the elements may predominate. have suggested that the cell of origin is a primitive mes- They usually show a fairly severe degree of cellular atypenchymal cell [Weiss and Enzinger, 19781. In its most ism and prominent mitotic activity, including the prescharacteristic form, regardless of the precise histogene- ence of abnormal mitoses [Kahn et al., 19781. sis, MFH is composed of rounded histiocyte-like cells Although the natural history of soft tissue MFH has and spindle-shaped fibroblastic cells arranged in a stori- been established, similar data regarding MFH of bone form or whorled pattern [Weiss and Enzinger, 19781. remains more speculative [Weiner et al., 19831. The tuMost of these tumours thus have a biphasic pattern, al- mours may arise at almost any age (Table I). MFH of
Malignant Fibrous Histiocytorna of Bone
Fig. 4. X 400.
169
Inflammatory variant of MFH showing leukocytic infiltration amidst tunlour cells. H & E,
TABLE 1. Sites of Malignant Fibrous Histiocytoma of Bone Authors Huvos 119761 Spanier [ 19771 Feldnian and Lattcs [ 19771 Dahlin er al. 119771 Kahn et A . 119781 McCarthy et al. [I9791 Present sludy [ 198Xl
Age range Mean age Number of (years) (years) tuinours studied 7-57 18-68
9-79 6-72 16--68
11-69 14-58
28 44 55 39 32.4 34
28
bone has a male predominance, although there are reports that a difference may exist between the age distribution of the sexes, whereby the older paticnts are male and the younger patients female IHuvos, 1976; Weiner et al., 19831. This tumour may occur at any site, although its most common site is around the knee joint (Table I). Apart from thc metaphyses of long bones, the jaw bones [Spector and Ogura, 1974; Webber and Wienke, 1977; Dahlin ct al., 19771 and vertebrae [Newland et al., 19751 may also be the primary sites of MFH. MFH has been known to occur following irradiation IDahlin ct al., 1977; Hardy et al., 19781. Its tendcncy to complicate bone infarcts has alrcady been noted. There is no significant association with trauma. The roentgenographic appearances are usually indicative of an osteolytic malignant process; but many, on occasion, have a deceptivcly benign-appearing margin. Thera are no roentgcnographic features to indicate a specific diagnosis of MFH [Kahn ct al., 19781. The most
18
II 23 35 7 35 7
Most common site
Distal femur Metaphyscs of long bones Mctaphyses of long bones ih4ctdphyses of long bones Proximal tibia Distal femur and proximal tibia Proximal tibia
common x-ray diagnoses are fibrosarcoma. osteolytic ostcosarcoma, and reticulum cell sarcoma. Many dediffercntiated sarcomas of fibrocytic, chondrogenic, or osteogenic origin may contain areas that resemble MFH [Dahlin et al., 19771; therefore, tumours like fibrosarcoma and osteosarcoma have to be considcred in the differential diagnosis of MFH of bone. Maintenance of a herringbone pattern (even if poorly developed), uniform histology among many tissue blocks, and absence or paucity of benign or malignant-appearing multinucleatcd giant cells are compatible with the diagnosis of fibrosarcoma [Pritchard et al., 19741. The presence of lymph node involvement would favour the diagnosis of MFH [Spanier et al., 19751. Osteolytic osteosarcoma may have radiological features very similar to that of MFH of bonc. However, the occurrence of pathological fracture in osteosarcoma is low, whereas its occurrence in MFH is high. Raised serum alkaline phosphatase activity would also favour osteosarcoma. The final separation rests upon the dem-
170
Kumar et al.
onstration of tumour osteoid in histologic sections of osteosarcoma. Malignant giant cell tumour of bone may have to be ruled out in those cases where osteoclast-like giant cells predominate. Malignant lymphoma may also have to be excluded in those tumours that predominantly exhibit the histiocytic element in multiple tissue blocks. MFH of bone is biologically a fully malignant tumour. Not only does it recur locally, but it metastasises via both the bloodstream and lymphatic channels to distant sites such as the lungs, soft tissues, and other bones [Weiner et al., 19831. Pulmonary metastases appear to be the most common [Spanier et al., 1975; Kahn et al., 1978; McCarthy et al., 19791 although metastases to other bones, liver, heart, and adrenals have also been described [Spanier et al., 19751. Lymph node metastasis is also frequent [Feldman and Norman, 1972; Spanier et al., 197.51. Apart from metastatic lesions, multiple primary “fibroxanthosarcomas” of bone have also been described that are distinct clinicopathologically [Chen, 19781. Surgical resection or amputation comprise the mainstay of therapy for MFH of bone. However, surgery may not always control the disease. Several investigators have employed adjuvant radiotherapy with varying degrees of success [Dahlin et al., 1977; Huvos, 1976; Spanier et al., 19751. Weiner et al. [1983] have used adjuvant chemotherapy with good results.
REFERENCES Chen KTK (1978): Multiple Fibroxanthosarcoma of bone. Cancer 42: 770-773. Dahlin DC, Unni KK, Matsuno T (1977): Malignant fibrous histiocytoma of bone. Fact or fancy. Cancer 39:1508-1516. Feldman F, Lattes R (1977): Primary malignant fibrous histiocytoma (fibrous xanthonia) of bone. Skeletal Radio1 1: 145-160. Feldman F. Norman D (1972): lntra- and extraosseous malignant histiocytonia (malignant fibrous xanthoma). Radiology 104:497508.
Galli SJ, Weintraub HP, Proppe KH (1978): Malignant fibrous histiocytoma and pleomorphic sarcoma in association with medullary bone infarcts. Cancer 41:607-619. Hardy TJ, Teisa A, Brown PW, Terz JJ (1978): Postirradiation sarcoma (MFH) of axilla. Cancer 42:118-124. Huvos AG (1976): Primary malignant fibrous histiocytoma of bone. Clinicopathological study of 18 patients. NY State J Med 76: 552-559. Kahn LB, Webber B. Mills E, Anstey L, Heselson NG (1978): Malignant fibrous histiocytoma (malignant fibrous xanthoma; xanthosarcoma) of bone. Cancer 42:640-651. Kernpson RL, Kyriakos M ( 1972): Fibroxanthosarcoma of the soft tissues: A type of malignant fibrous histiocytoma. Cancer 29: 96 1-976. McCarthy EF, Matsuno T, Dorfman HD (1979): Malignant fibrous histiocytoma of bone: A study of 35 cases. Hum Pathol 10: 57-70. Michael RH, Dorfman HD ( 1977): Malignant fibrous histiocytoma associated with bone infarcts. Clin Orthop I18:180-194. Mirra JM, Gold RH, Marafiote R (1977): Malignant (fibrous) histiocytoma arising in association with a bone infarct in sickle cell disease: Coincidence or cause-and-effect’? Cancer 39: 186194. Newland RC, Harrison MA, Wright RG ( 1975): Fibroxanthosarcoma of bone. Pathology 7:203-208. Ozzello L, Stout AP, Murray MR (1963): Cultural characteristics of malignant histiocytomas and fibrous xanthomas. Cancer 16: 331-344. Pritchard DJ, Soule EH, Taylor WF, Ivins JC (1974): Fibrosarconya-A clinicopathologic and statistical study of 199 turnours of the soft tissues of the extremities and trunk. Cancer 33: 888-897. Soule E, Enriquez P (1972): Atypical fibrous histiocytoma, malignant fibrous histiocytoma, malignant histiocytonia epithelioid sarcoma. A comparative study of 65 tumours. Cancer 30:128143. Spanier SS (1977): Malignant fibrous histiocytoma of bone. Orthop Clin North Am 8:947-961. Spanier SS, Enneking WF, Enriquez P (1975): Primary malignant fibrous histiocytoma of bone. Cancer 369084-2098. Spector GF, Ogura JH (1974): Malignant fibrous histiocytoma of the maxilla. A report of an unusual lesion. Arch Otolaryngol 99: 385-387. Webber W, Wienke EC (1977): Malignant fibrous histiocytoma of the mandible. Case report. Plast Recontr Surg 60:629-634. Weiner M, Sedlis M, Johnston AD. Dick HM, Wolff JA (1983): Adjuvant chemotherapy of malignant fibrous histiocytoma of bone. Cancer 5 1:25-29. Weiss SW, Enzinger FM (1978): Malignant fibrous histiocytoma. An analysis of 200 cases. Cancer 41:2250-2266.
Malignant Fibrous Histiocytoma of Bone REKHA V. KUMAR, MD, GEETASHREE MUKHERJEE, MD, AND M . KRISHNA BHARGAVA, MD From the Department of Pathology, Kidwai Memorial Institute of Oncology, Bangalore, India
Malignant fibrous histiocytoma (MFH) of bone is a malignant primary bone tumour that is being increasingly recognised, as more details emerge in the literature regarding its natural history and precise (although sometimes diverse) histological appearance. When the whole bulk of a malignant bone tumour fits the criteria laid down, the designation of MFH seems appropriate. Seven cases of MFH of bone were encountered from a total of 220 primary malignant bone tumours in our files over a 4-year period. The metaphyses of long bones were the most common sites harbouring the tumour, and a wide age range was represented. Amputation was the treatment of choice in all cases. The relevant literature is reviewed. KEY WORDS:sarcoma, pleomorphism, skeleton
INTRODUCTION Malignant fibrous histiocytoma (MFH) is a pleomorphic sarcoma that most often arises in the soft tissues of the trunk and extremities. It has now been well established that bone may be the primary site of MFH, quite distinct from its involvement by direct extension of a soft tissue tumour or by metastatic spread [Feldman and Norman, 1972; Spanier et al., 1975; Dahlin et al.. 19771. Primary MFH of bone has been fairly extensively reported in the literature [Feldman and Norman, 1972; Spanier et al., 1975; HUVOS,1976; Feldman and Lattes, 1977; Mirra et al., 1977; Michael and Dorfman, 1977; Kahn et al., 1978; McCarthy et al., 19791. A few of the reported cases have been associated with bone infarcts [Spanier et al., 1975; Michael and Dorfman, 1977; Mirra et al., 1977; Galli et al., 1978; McCarthy et al., 19791. Seven cases of primary MFH of bone encountered over a 4-year period (1985-1988) at the Kidwai Memorial Institute of Oncology (KMIO) were studied. The salient microscopic findings that led to the diagnosis after other tumours had been excluded or overruled are discussed, together with a review of the relevant literature. MATERIALS AND METHODS Seven cases of MFH of bone were encountered from a Of 220 primary bone at the KMIO over a 4-year period (1985-1988). Only those 0 1990 Wiley-Liss, Inc.
tumours having a histology permitting classification as MFH according to the criteria outlined for soft-tissue tumours by Soule and Enriquez [ 19721 and Kempson and Kyriakos [ 19721 were studied. Haematoxylin and eosin (H & E) stained paraffin sections were studied in all cases. Wilder’s reticulin stain, Masson’s trichrome stain, Van Gieson, Sudan 111, and Alcian blue stains were available in most cases. Radiographs and clinical data were available for all inpatients. The histologic material from one additional case was referred to our pathology department for consultation. Because the cases were relatively recent, the follow-up period was necessarily short, with the longest follow-up being 3 years.
OBSERVATIONS Clinical Data Six of seven patients were male. The average age was 28; the youngest patient was 14 and the oldest was 58. The most common presenting symptoms were pain and swelling (six of seven cases) and were associated with trauma in two cases. Serum alkaline phosphatase levels were done in four cases and were within normal limits in three cases, whereas the fourth case registered a mildly
Accepted for publication February 4, 1990. Address reprint requests to Dr. R e b a V. Kumar. Lecturer in Pathology, Kidwai Memorial Institute of Oncology. Hosur Road, Bangalore 560 029, India.
Malignant Fibrous Histiocytorna of Bone
Fig. I . Roentgenogram of turnour at upper end of tibia showing a predominantly osteolytic lesion.
elevated value of 66 IU/liter, with normal values between 20 and SO IU/liter. Six lesions occurred in the end of a long bone, affecting the metaphysis, and often the adjacent epiphyseal and diaphyseal regions, three lesions in the proximal tibia, one each in the distal tibia, distal femur, and proximal humerus. One lesion occurred in the mandible. Koentgenograms showed most of the tumours as illdefined osteolytic areas, with endosteal erosion. There was minimal reactive bone formation, with no evidence of tumour calcification (Fig. 1). In two cases, there was an associated pathological fracture. The most common x-ray diagnosis was osteolytic osteosarcoma. One case was interpreted as a benign bone tumour.
Treatment Surgery was the treatment of choice in all cases. Amputation (including limb disarticulation) and hemimandiblectomy were the operative procedures. Two cases received preoperative chemotherapy based on clinical and radiological features suggestive of osteosarcoma. Radiotherapy was not given to any case. The longest follow-up was 3 years; the patient is alive and well. Two of the seven cases developed pulmonary metastases and succumbed 6 and 8 months following surgery.
167
Pathological Features Gross pathology. All lesions where the gross specimen was received (in six of seven cases) were destructive tumours and associated with a pathological fracture in two cases. The tumours in the long bones showed erosion and destruction of the cortex, with extension into the adjacent soft tissues, often with a “pushing” border. Multiple tumour nodules in the soft tissue were also observed. The cut surfaces were variegated. Most of the tumours were grey-white and fleshy, with areas of haemorrhage and necrosis. There was variation in consistency, some tumours being soft and others firm to hard. Microscopic pathology. There was evidence of both fibroblastic and histiocytic differentiation in all tumours. Formation of storiform or cartwheel patterns by spindle cells and collagen production were evidence of fibroblastic differentiation (Fig. 2). The histiocytic element was represented by foam cells, erythrophagocytosis, and giant cell formation in cells having the cytologic characteristics of histiocytes, i.e., folded or lobulated vesicular nuclei, many with prominent, acidophilic nucleoli, and rather abundant cytoplasm. Pleomorphism of the histiocytic element was seen in many cases. The multinucleated giant cells were usually pleomorphic tumour giant cells in areas of histiocytic differentiation (Fig. 3). However, osteoclast-like giant cells and an occasional Langhans giant cell were also observed. In most cases, there was a diffuse intermingling of both fibroblastic and histiocytic elements. Detailed study of 20-25 sections per case was sufficient to identify both elements. No tumour osteoid was seen. In one case, there was a uniform infiltration of inflammatory cells composed of lymphocytes, polymorphonuclear leukocytes, and plasma cells, which in some areas obscured the neoplastic cells (Fig. 4).This variant has a soft tissue counterpart in the inflammatory variant of MFH as described by Kempson and Kyriakos [ 19721. It was noticed that a milder round and polymorphonuclear cellular infiltrate was present even in the other turnours. The malignant nature of these tumours was generally obvious with numerous mitotic figures (often atypical) and hyperchromatic, pleomorphic nuclei. In all instances, the usual criteria for malignancy used for other sarcomas of bone were readily identifiable. No evidencc of lymph node metastasis was provided by this series.
DISCUSSION MFH is a relatively recently described and controversial tumour. It has an uncertain histogenesis. Some authors believe that the cell of origin is the tissue histiocyte [Kempson and Kyriakos, 1972; Ozzello et al., 19631. which has the propensity to assume fibroblastic features
168
Kumar et al.
Fig. 2.
Area of tuinour composed of fairly unifomi fibrocytic type cells growing in a storiform pattern.
H & E,
X
100.
Fig. 3 . Giant cell in area of histiocytic differentiation exhibiting phagocytosed pol ymorphs. H & E. x 1 .ooo.
and to lay down collagen (facultative fibroblasts). Others though one or another of the elements may predominate. have suggested that the cell of origin is a primitive mes- They usually show a fairly severe degree of cellular atypenchymal cell [Weiss and Enzinger, 19781. In its most ism and prominent mitotic activity, including the prescharacteristic form, regardless of the precise histogene- ence of abnormal mitoses [Kahn et al., 19781. sis, MFH is composed of rounded histiocyte-like cells Although the natural history of soft tissue MFH has and spindle-shaped fibroblastic cells arranged in a stori- been established, similar data regarding MFH of bone form or whorled pattern [Weiss and Enzinger, 19781. remains more speculative [Weiner et al., 19831. The tuMost of these tumours thus have a biphasic pattern, al- mours may arise at almost any age (Table I). MFH of
Malignant Fibrous Histiocytorna of Bone
Fig. 4. X 400.
169
Inflammatory variant of MFH showing leukocytic infiltration amidst tunlour cells. H & E,
TABLE 1. Sites of Malignant Fibrous Histiocytoma of Bone Authors Huvos 119761 Spanier [ 19771 Feldnian and Lattcs [ 19771 Dahlin er al. 119771 Kahn et A . 119781 McCarthy et al. [I9791 Present sludy [ 198Xl
Age range Mean age Number of (years) (years) tuinours studied 7-57 18-68
9-79 6-72 16--68
11-69 14-58
28 44 55 39 32.4 34
28
bone has a male predominance, although there are reports that a difference may exist between the age distribution of the sexes, whereby the older paticnts are male and the younger patients female IHuvos, 1976; Weiner et al., 19831. This tumour may occur at any site, although its most common site is around the knee joint (Table I). Apart from thc metaphyses of long bones, the jaw bones [Spector and Ogura, 1974; Webber and Wienke, 1977; Dahlin ct al., 19771 and vertebrae [Newland et al., 19751 may also be the primary sites of MFH. MFH has been known to occur following irradiation IDahlin ct al., 1977; Hardy et al., 19781. Its tendcncy to complicate bone infarcts has alrcady been noted. There is no significant association with trauma. The roentgenographic appearances are usually indicative of an osteolytic malignant process; but many, on occasion, have a deceptivcly benign-appearing margin. Thera are no roentgcnographic features to indicate a specific diagnosis of MFH [Kahn ct al., 19781. The most
18
II 23 35 7 35 7
Most common site
Distal femur Metaphyscs of long bones Mctaphyses of long bones ih4ctdphyses of long bones Proximal tibia Distal femur and proximal tibia Proximal tibia
common x-ray diagnoses are fibrosarcoma. osteolytic ostcosarcoma, and reticulum cell sarcoma. Many dediffercntiated sarcomas of fibrocytic, chondrogenic, or osteogenic origin may contain areas that resemble MFH [Dahlin et al., 19771; therefore, tumours like fibrosarcoma and osteosarcoma have to be considcred in the differential diagnosis of MFH of bone. Maintenance of a herringbone pattern (even if poorly developed), uniform histology among many tissue blocks, and absence or paucity of benign or malignant-appearing multinucleatcd giant cells are compatible with the diagnosis of fibrosarcoma [Pritchard et al., 19741. The presence of lymph node involvement would favour the diagnosis of MFH [Spanier et al., 19751. Osteolytic osteosarcoma may have radiological features very similar to that of MFH of bonc. However, the occurrence of pathological fracture in osteosarcoma is low, whereas its occurrence in MFH is high. Raised serum alkaline phosphatase activity would also favour osteosarcoma. The final separation rests upon the dem-
170
Kumar et al.
onstration of tumour osteoid in histologic sections of osteosarcoma. Malignant giant cell tumour of bone may have to be ruled out in those cases where osteoclast-like giant cells predominate. Malignant lymphoma may also have to be excluded in those tumours that predominantly exhibit the histiocytic element in multiple tissue blocks. MFH of bone is biologically a fully malignant tumour. Not only does it recur locally, but it metastasises via both the bloodstream and lymphatic channels to distant sites such as the lungs, soft tissues, and other bones [Weiner et al., 19831. Pulmonary metastases appear to be the most common [Spanier et al., 1975; Kahn et al., 1978; McCarthy et al., 19791 although metastases to other bones, liver, heart, and adrenals have also been described [Spanier et al., 19751. Lymph node metastasis is also frequent [Feldman and Norman, 1972; Spanier et al., 197.51. Apart from metastatic lesions, multiple primary “fibroxanthosarcomas” of bone have also been described that are distinct clinicopathologically [Chen, 19781. Surgical resection or amputation comprise the mainstay of therapy for MFH of bone. However, surgery may not always control the disease. Several investigators have employed adjuvant radiotherapy with varying degrees of success [Dahlin et al., 1977; Huvos, 1976; Spanier et al., 19751. Weiner et al. [1983] have used adjuvant chemotherapy with good results.
REFERENCES Chen KTK (1978): Multiple Fibroxanthosarcoma of bone. Cancer 42: 770-773. Dahlin DC, Unni KK, Matsuno T (1977): Malignant fibrous histiocytoma of bone. Fact or fancy. Cancer 39:1508-1516. Feldman F, Lattes R (1977): Primary malignant fibrous histiocytoma (fibrous xanthonia) of bone. Skeletal Radio1 1: 145-160. Feldman F. Norman D (1972): lntra- and extraosseous malignant histiocytonia (malignant fibrous xanthoma). Radiology 104:497508.
Galli SJ, Weintraub HP, Proppe KH (1978): Malignant fibrous histiocytoma and pleomorphic sarcoma in association with medullary bone infarcts. Cancer 41:607-619. Hardy TJ, Teisa A, Brown PW, Terz JJ (1978): Postirradiation sarcoma (MFH) of axilla. Cancer 42:118-124. Huvos AG (1976): Primary malignant fibrous histiocytoma of bone. Clinicopathological study of 18 patients. NY State J Med 76: 552-559. Kahn LB, Webber B. Mills E, Anstey L, Heselson NG (1978): Malignant fibrous histiocytoma (malignant fibrous xanthoma; xanthosarcoma) of bone. Cancer 42:640-651. Kernpson RL, Kyriakos M ( 1972): Fibroxanthosarcoma of the soft tissues: A type of malignant fibrous histiocytoma. Cancer 29: 96 1-976. McCarthy EF, Matsuno T, Dorfman HD (1979): Malignant fibrous histiocytoma of bone: A study of 35 cases. Hum Pathol 10: 57-70. Michael RH, Dorfman HD ( 1977): Malignant fibrous histiocytoma associated with bone infarcts. Clin Orthop I18:180-194. Mirra JM, Gold RH, Marafiote R (1977): Malignant (fibrous) histiocytoma arising in association with a bone infarct in sickle cell disease: Coincidence or cause-and-effect’? Cancer 39: 186194. Newland RC, Harrison MA, Wright RG ( 1975): Fibroxanthosarcoma of bone. Pathology 7:203-208. Ozzello L, Stout AP, Murray MR (1963): Cultural characteristics of malignant histiocytomas and fibrous xanthomas. Cancer 16: 331-344. Pritchard DJ, Soule EH, Taylor WF, Ivins JC (1974): Fibrosarconya-A clinicopathologic and statistical study of 199 turnours of the soft tissues of the extremities and trunk. Cancer 33: 888-897. Soule E, Enriquez P (1972): Atypical fibrous histiocytoma, malignant fibrous histiocytoma, malignant histiocytonia epithelioid sarcoma. A comparative study of 65 tumours. Cancer 30:128143. Spanier SS (1977): Malignant fibrous histiocytoma of bone. Orthop Clin North Am 8:947-961. Spanier SS, Enneking WF, Enriquez P (1975): Primary malignant fibrous histiocytoma of bone. Cancer 369084-2098. Spector GF, Ogura JH (1974): Malignant fibrous histiocytoma of the maxilla. A report of an unusual lesion. Arch Otolaryngol 99: 385-387. Webber W, Wienke EC (1977): Malignant fibrous histiocytoma of the mandible. Case report. Plast Recontr Surg 60:629-634. Weiner M, Sedlis M, Johnston AD. Dick HM, Wolff JA (1983): Adjuvant chemotherapy of malignant fibrous histiocytoma of bone. Cancer 5 1:25-29. Weiss SW, Enzinger FM (1978): Malignant fibrous histiocytoma. An analysis of 200 cases. Cancer 41:2250-2266.
