Annals of the Royal College of Surgeons of England
(I979) vol 6i
Fibrous histiocytoma: benign and malignant variants Maged S Barsoum Mch FRCS FRCSEd Assistant Professor of Surgery, Kasr el-Aini School of Medicine, Cairo University
Saad S Eissa MD(Path) Mohamed A Mansour Mch Departments of Surgery and Pathology, Kasr el-Aini and Cancer Institute Hospitals, Cairo.
Summary Twenty cases of benign and malignant fibrous histiocytoma are presented. Six were benign, i i malignant fibrous, and 3 pure malignant histiocytomas. These tumours arise from tissue histiocytes and have a wide range of histological appearances. The characteristic histological features are described and the variable clinical picture and different lines of treatment and management are discussed. Introduction Fibrohistiocytic tumours arise from histiocytes in soft tissues. They comprise benign proliferations known as fibrous histiocytoma and malignant tumours (malignant fibrous histiocytoma and pure malignant histiocytoma)1. The diverse neoplastic potential of the tissue histiocyte is responsible for the previously confusing nomenclature of this group of softtissue tumours2.
Material and methods
Benign fibrous histiocytoma was found in 4 male and 2 female patients whose age varied from 30 to 50 years. The lesions were either skin nodules (pigmented in 2 cases) or circumscribed subcutaneous lumps. Their mean size was I.95 cm. The lumps were firm and mobile and the overlying skin was tethered and showed a few dilated veins. All 6 cases were clinically misdiagnosed either as fibrolipoma or mole. Excision and primary skin closure was performed on all patients. No recurrence occurred for up to 3 years. Histologically, two basic cell types were found: spindle-shaped fibroblasts arranged in fascicles and a storiform ('cartwheel') pattern mixed with aggregates of histiocytes. Variable numbers of multinucleated giant cells and occasional iron-laden macrophages were present
(Fig. i). Malignant fibrous histiocytoma was found in i i patients, 7 male and 4 female, ranging in age from I5 to 50 years. The swellings
Soft-tissue tumours from the files of the Kasr el-Aini and Cancer Institute Hospitals during the period I974-76 were reviewed. Sections were recut, stained with haematoxylin and eosin, special connective tissue stain (Masson trichrome), and Gomori silver reticulin stain3. The characteristics of the fibrohistiocytic tumours found are presented, showing their variable clinical presentation, wide range of histological patterns, and different lines of treatment. Results Fibrohistiocytic tumours were found in 20 patients with soft-tissue tumours: benign fibrous histiocytoma in 6, malignant fibrous FIG. i Benign fibrous histiocytoma showing histiocytoma in ii, and malignant histio- storiform pattern; X25, haematoxylin and cytoma in 3. eoszn.
IPo
Maged S Barsoum, Saad S Eissa, and Mohamed A Mansour
were solitary except in one patient with two lumps, one in the neck and one on the back. Their mean size was 8.63 cm. They affected subcutaneous tissue and muscles and in one patient the skin of the nose. The rate of growth was rather slow initially, followed by a rapid increase in size. The lumps were nodular, illdefined, and firm but occasionally frankly cystic, containing blood. Excision was performed in all cases and was followed by irradiation in 5 in which it was felt that the excision was inadequate. Five patients were alive 3 years later-2 after excision alone and 3 after excision and irradiation. Six patients died 4 who developed local recurrence and, in 2 cases, distant metastases after excision alone and 2 who developed distant metastases after excision and irradiation. Histologically, the tumours had displacing rather than infiltrating margins. Numerous fusiform fibroblastic cells with elongated bland or irregular hyperchromatic nuclei were identified. Irregular epithelioid histiocytic cells with abundant cytoplasm and vesicular nuclei were seen. Bizarre giant histiocytes with pale, eosinophilic, foamy cytoplasm and large, irregular, often multiple nuclei with prominent macronucleoli were interspersed throughout (Fig. 2). The mitotic rate was high. Malignant histiocytoma was found in 3 male patients ranging in age from 40 to 49 years. The swellings were irregular, soft, and partly cystic and attained large size (mean I2.5 cm).
*z **-; * o-=, .n.l !'-n
..
".
-
.1.
FIG. 3 Malignant histiocytoma showing epithelioid cells and foamy histiocytes, X4oo, haematoxylin and eosin. Excision was the primary treatment followed by postoperative irradiation. Two patients died within 3 years with local recurrence, accompanied in i case by distant metastases. One patient is alive free from recurrence. Histologically, histiocytic proliferation with minimal fibroblastic elements was seen. The cells had a distinctly epithelioid appearance with many foam cells (Fig. 3).
Discussion Tissue histiocytes can differentiate along two lines-as phagocytic macrophages and/or as fibroblasts forming connective-tissue fibres2. This ability is shown in the wide histological spectrum of fibrohistiocytic tumours, which varies from a predominantly fibrogenic to a pronounced histiocytic appearance depending on whether the histiocytes are exercising their phagocytic potential or behaving as facultative
fibroblasts'. Benign fibrous histiocytoma usually affects adults. The tumours arise from the skin and subcutaneous tissue of the face, neck, trunk, and lower limbs. They are painless, slowly growing, small lumps, firm in consistency and occasionally vascular. To the naked eye the tumours are yellowish in colour with few _' S cystic areas. The basic microscopic feature is the storiform pattern of fibroblasts and abundant histiocytes. FIG. 2 Malignant fibrous histiocytoma showing tumour giant cells, X4oo, haematoxylin Malignant fibrous histiocytoma arises in and eosin. middle age. The male: female ratio is 2 I .
Fibrous histiocytoma: benign and malignant variants They most frequently involve the skin, subcutaneous tissue, or deep structures such as muscles. Occasionally the retroperitoneal tissue4 and viscera such as the kidney and cervix uteri5'0 are involved. The lower limb is the most common site, followed by the upper limb, trunk, retroperitoneal tissue, head, and neck. Deep tumours are usually larger than superficial ones. Metastases usually occur to lymph nodes, lungs, and bones7. Histologically, the less malignant forms are predominently fibroblastic while the more aggressive ones usually contain a less conspicuous fibrous component. Malignant histiocytoma arises from subcutaneous, deep, and retroperitoneal tissues. Males are more commonly affected than females, and children are occasional victims. They usually affect the trunk and extremities and often reach a large size. They are illdefined, irregular in shape, and soft in consistency. Metastases usually involve the soft tissues, brain, lungs, and lymph nodes8. Histologically, the tumours are composed of malignant histiocytes proliferating in a milieu devoid of the plemorphism which is encountered in the malignant fibrous histiocytoma5. The rationale of treatment is to perform wide excision even for the apparently innocent tumours. This aggressive attitude is important
151
if the high recurrence rate is to be minimised. Block dissection of involved lymph nodes should be done and even amputation if bone is infiltrated. Postoperative irradiation is recommended for the malignant varieties. Although moderately radiosensitive, they tend to recur and irradiation cannot be given alone for control of disease. Palliative chemotherapy can be tried.
References I
2
3
4 5 6 7 8
Soule, E H, and Enriquez, P (1972) Cancer, 30, I 28. Mackenzie, D H (1975) In Recent Advances in Pathology, No 9, ed. C V Harrison and K. Weinbren, p i83. Edinburgh, Churchill Livingstone. Lee, C L (i968) Manual of Histopathologic Staining Methods of the Armed Forces Institute of Pathology, 3rd edn, p 72. New York, McGraw Hill Book Co., pp 72. Rosas-Uribe, A, Ring, A M, and Rappaport, H (1970) Cancer, 26, 827. Ozzello, L, Stout, A P, and Murray, M R (I963) Cancer, i6, 33I. Bonfiglio, TA, Patten, S F, and Woodworth, F E (I976) Acta cytologica, 20, 501. Wasserman, T H and Stuard, I D (i974) Cancer, 33, I41. Stout A P, and Lattes, R (I967) In Atlas of Tumour Pathology, 2nd series, vol I, pp 107I15. Washington, DC, Armed Forces Institute of Pathology.
(I979) vol 6i
Fibrous histiocytoma: benign and malignant variants Maged S Barsoum Mch FRCS FRCSEd Assistant Professor of Surgery, Kasr el-Aini School of Medicine, Cairo University
Saad S Eissa MD(Path) Mohamed A Mansour Mch Departments of Surgery and Pathology, Kasr el-Aini and Cancer Institute Hospitals, Cairo.
Summary Twenty cases of benign and malignant fibrous histiocytoma are presented. Six were benign, i i malignant fibrous, and 3 pure malignant histiocytomas. These tumours arise from tissue histiocytes and have a wide range of histological appearances. The characteristic histological features are described and the variable clinical picture and different lines of treatment and management are discussed. Introduction Fibrohistiocytic tumours arise from histiocytes in soft tissues. They comprise benign proliferations known as fibrous histiocytoma and malignant tumours (malignant fibrous histiocytoma and pure malignant histiocytoma)1. The diverse neoplastic potential of the tissue histiocyte is responsible for the previously confusing nomenclature of this group of softtissue tumours2.
Material and methods
Benign fibrous histiocytoma was found in 4 male and 2 female patients whose age varied from 30 to 50 years. The lesions were either skin nodules (pigmented in 2 cases) or circumscribed subcutaneous lumps. Their mean size was I.95 cm. The lumps were firm and mobile and the overlying skin was tethered and showed a few dilated veins. All 6 cases were clinically misdiagnosed either as fibrolipoma or mole. Excision and primary skin closure was performed on all patients. No recurrence occurred for up to 3 years. Histologically, two basic cell types were found: spindle-shaped fibroblasts arranged in fascicles and a storiform ('cartwheel') pattern mixed with aggregates of histiocytes. Variable numbers of multinucleated giant cells and occasional iron-laden macrophages were present
(Fig. i). Malignant fibrous histiocytoma was found in i i patients, 7 male and 4 female, ranging in age from I5 to 50 years. The swellings
Soft-tissue tumours from the files of the Kasr el-Aini and Cancer Institute Hospitals during the period I974-76 were reviewed. Sections were recut, stained with haematoxylin and eosin, special connective tissue stain (Masson trichrome), and Gomori silver reticulin stain3. The characteristics of the fibrohistiocytic tumours found are presented, showing their variable clinical presentation, wide range of histological patterns, and different lines of treatment. Results Fibrohistiocytic tumours were found in 20 patients with soft-tissue tumours: benign fibrous histiocytoma in 6, malignant fibrous FIG. i Benign fibrous histiocytoma showing histiocytoma in ii, and malignant histio- storiform pattern; X25, haematoxylin and cytoma in 3. eoszn.
IPo
Maged S Barsoum, Saad S Eissa, and Mohamed A Mansour
were solitary except in one patient with two lumps, one in the neck and one on the back. Their mean size was 8.63 cm. They affected subcutaneous tissue and muscles and in one patient the skin of the nose. The rate of growth was rather slow initially, followed by a rapid increase in size. The lumps were nodular, illdefined, and firm but occasionally frankly cystic, containing blood. Excision was performed in all cases and was followed by irradiation in 5 in which it was felt that the excision was inadequate. Five patients were alive 3 years later-2 after excision alone and 3 after excision and irradiation. Six patients died 4 who developed local recurrence and, in 2 cases, distant metastases after excision alone and 2 who developed distant metastases after excision and irradiation. Histologically, the tumours had displacing rather than infiltrating margins. Numerous fusiform fibroblastic cells with elongated bland or irregular hyperchromatic nuclei were identified. Irregular epithelioid histiocytic cells with abundant cytoplasm and vesicular nuclei were seen. Bizarre giant histiocytes with pale, eosinophilic, foamy cytoplasm and large, irregular, often multiple nuclei with prominent macronucleoli were interspersed throughout (Fig. 2). The mitotic rate was high. Malignant histiocytoma was found in 3 male patients ranging in age from 40 to 49 years. The swellings were irregular, soft, and partly cystic and attained large size (mean I2.5 cm).
*z **-; * o-=, .n.l !'-n
..
".
-
.1.
FIG. 3 Malignant histiocytoma showing epithelioid cells and foamy histiocytes, X4oo, haematoxylin and eosin. Excision was the primary treatment followed by postoperative irradiation. Two patients died within 3 years with local recurrence, accompanied in i case by distant metastases. One patient is alive free from recurrence. Histologically, histiocytic proliferation with minimal fibroblastic elements was seen. The cells had a distinctly epithelioid appearance with many foam cells (Fig. 3).
Discussion Tissue histiocytes can differentiate along two lines-as phagocytic macrophages and/or as fibroblasts forming connective-tissue fibres2. This ability is shown in the wide histological spectrum of fibrohistiocytic tumours, which varies from a predominantly fibrogenic to a pronounced histiocytic appearance depending on whether the histiocytes are exercising their phagocytic potential or behaving as facultative
fibroblasts'. Benign fibrous histiocytoma usually affects adults. The tumours arise from the skin and subcutaneous tissue of the face, neck, trunk, and lower limbs. They are painless, slowly growing, small lumps, firm in consistency and occasionally vascular. To the naked eye the tumours are yellowish in colour with few _' S cystic areas. The basic microscopic feature is the storiform pattern of fibroblasts and abundant histiocytes. FIG. 2 Malignant fibrous histiocytoma showing tumour giant cells, X4oo, haematoxylin Malignant fibrous histiocytoma arises in and eosin. middle age. The male: female ratio is 2 I .
Fibrous histiocytoma: benign and malignant variants They most frequently involve the skin, subcutaneous tissue, or deep structures such as muscles. Occasionally the retroperitoneal tissue4 and viscera such as the kidney and cervix uteri5'0 are involved. The lower limb is the most common site, followed by the upper limb, trunk, retroperitoneal tissue, head, and neck. Deep tumours are usually larger than superficial ones. Metastases usually occur to lymph nodes, lungs, and bones7. Histologically, the less malignant forms are predominently fibroblastic while the more aggressive ones usually contain a less conspicuous fibrous component. Malignant histiocytoma arises from subcutaneous, deep, and retroperitoneal tissues. Males are more commonly affected than females, and children are occasional victims. They usually affect the trunk and extremities and often reach a large size. They are illdefined, irregular in shape, and soft in consistency. Metastases usually involve the soft tissues, brain, lungs, and lymph nodes8. Histologically, the tumours are composed of malignant histiocytes proliferating in a milieu devoid of the plemorphism which is encountered in the malignant fibrous histiocytoma5. The rationale of treatment is to perform wide excision even for the apparently innocent tumours. This aggressive attitude is important
151
if the high recurrence rate is to be minimised. Block dissection of involved lymph nodes should be done and even amputation if bone is infiltrated. Postoperative irradiation is recommended for the malignant varieties. Although moderately radiosensitive, they tend to recur and irradiation cannot be given alone for control of disease. Palliative chemotherapy can be tried.
References I
2
3
4 5 6 7 8
Soule, E H, and Enriquez, P (1972) Cancer, 30, I 28. Mackenzie, D H (1975) In Recent Advances in Pathology, No 9, ed. C V Harrison and K. Weinbren, p i83. Edinburgh, Churchill Livingstone. Lee, C L (i968) Manual of Histopathologic Staining Methods of the Armed Forces Institute of Pathology, 3rd edn, p 72. New York, McGraw Hill Book Co., pp 72. Rosas-Uribe, A, Ring, A M, and Rappaport, H (1970) Cancer, 26, 827. Ozzello, L, Stout, A P, and Murray, M R (I963) Cancer, i6, 33I. Bonfiglio, TA, Patten, S F, and Woodworth, F E (I976) Acta cytologica, 20, 501. Wasserman, T H and Stuard, I D (i974) Cancer, 33, I41. Stout A P, and Lattes, R (I967) In Atlas of Tumour Pathology, 2nd series, vol I, pp 107I15. Washington, DC, Armed Forces Institute of Pathology.
