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Diagnostic Radiology
Malignant Giant Cell Tumor of the Soft Tissue: Angiographic Manifestations 1 Issa Yaghmal, M.D. Two cases of malignant giant cell tumor originating within the soft tissues of the thigh are reported. In both instances, angiography demonstrated hypervascular masses with arterial and venous abnormalities indicative of an aggressive malignancy. The histopathologic appearance of the lesions was similar to that of osseous giant cell tumors. The clinical and radiographic features of this rare entity are briefly reviewed. INDEX TERMS: Neoplasms, blood supply. Neoplasms, giant cell • Soft tissues, neoplasms • Thigh, neoplasms
Radiology 120:329-331, August 1976
•
•
HE extra-osseous occurrence of giant cell tumor is
CASE II. A 54-year-old man was referred for further evaluation of a large, soft-tissue mass in the lateral aspect of the left thigh. The lesion had tripled in size since the patient had become aware of it 10 months earlier. During the past four months, he had experienced pain at the site of the lesion while walking, and during palpation, and had lost 6 kg in weight. Physical examination disclosed a large mass within the soft tissues of the thigh. It appeared adherent to the adjacent femur. There was also enqorqement of the superficial veins over the lesion, and the left inguinal lymph nodes were palpable. Radiographs of the left thigh did not show evidence for destruction of the femur or calcification in the soft tissues. A needle biopsy contained mostly necrotic tissue. Left femoral angiography was then performed which showed a hypervascular mass measuring 7 X 15 cm displacing the superficial femoral artery medially. The vascular supply of the lesion was derived from branches of deep and superficial femoral arteries. Within the mass was an avascular zone suggestive of necrosis or a hematoma. There was early visualization of the femoral veins proximal to the tumor (Fig. 2, B and C). An open biopsy of the lesion was performed in the vascular portion of the lesion and multiple specimens were obtained. Histologic examination demonstrated the presence of a giant cell tumor of soft tissue composed of multinucleated giant cells, mononuclear histiocytes and fibrocytes. On the patient's request, only a local excision of the tumor was carried out and supplemented by radiotherapy. The postoperative course was uneventful. No evidence for recurrence has been found over six months of follow-up.
T
rare. The pathologic features of these soft-tissue tumors resemble those of giant cell tumors of the bone but their clinical course is usually more progressive and often fatal. Approximately 50 cases have b~en recorded in the literature, sometimes under designations such as malignant giant cell tumor of tendon sheath (1), extraskeletal osteolytic osteosarcoma (3), or giant cell fascial sarcoma (6). In this report, the angiographic findings in 2 patients with giant cell tumor of the soft tissue will be described and pertinent clinical and pathologic features of the malignancy will be reviewed. CASE REPORTS CASE I. A 62-year-old woman was admitted for evaluation of a tender soft-tissue mass in the right thigh, which had been noticed by the patient six months earlier. More recently, however, the lesion had become larger and painful on palpation. No history of trauma was present. Physical examination revealed a well-circumscribed, palpable, tender mass in the posteromedial aspect of the right thigh. The lesion appeared deeply located within the soft tissues, and mobile with respect to the femur. The regional lymph nodes were not palpable. Plain radiographs of the right thigh demonstrated an oval-shaped, soft-tissue mass demarcated from the surrounding muscles. There were no soft-tissue calcifications. The femur appeared intact. A femoral angiogram showed a hypervascular tumor measuring 6-8 cm 1n diameter. The lumen of the superficial femoral artery appeared narrowed in the segment adjacent to the lesion. An intense tumor stain occurred in the capillary phase. There was early opacification of numerous veins around the tumor resulting in filling of the superficial femoral vein (Fig. 1, A and B). The femoral vein was completely obstructed. Microscopic evaluation of biopsy specimens revealed the presence of a tumor primarily consisting of multinucleated giant cells and mononuclear histiocytes. These were arranged in a multinodular pattern, with some areas of intraluminal extension of the cells into the adjacent veins (Fig. 1, C). The pathologic diagnosis was malignant giant cell tumor of soft tissue. Subsequently disarticulation of the right leg was performed. However, the patient died six months later due to metastatic spread of the tumor to the lungs, liver, and the left thigh.
DISCUSSION
Approximately 50 previously reported cases of giant cell tumor of the soft tissues were noted in patients ranging in age from 9 to 87 years (4). Including the present 2 cases, the average age at the time of diagnosis is 57 years, with the majority of tumors found during the fifth or sixth decade of life. Fifty-seven per cent of patients have been male and 43 % female. The tumor usually presents as a painful and rapidly growing mass. The usual sites of involvement are the thighs for deep-seated lesions and the leg for the superficial ones. Less commonly, the lesions are located in the abdominal wall, shoulders, arms, and face. Compared to the superficial lesions, the deep-seated tumors have a less favorable prognosis, probably because of delayed recognition.
1 From the Department of Radiology, Medical College of Virginia, Virginia Commonwealth University, Richmond, Va. Accepted for publication in November 1975. shan
329
330
ISSA Y AGHMAI
Fig. 1. CASE I. A. arterial and B, venous phase reveal encasement of the femoral artery , a hypervascular mass with numerous abnormal neovascularities. early visualization of the femoral vein in the early arterial phase (arrow), uneven tumor stain, large collateral veins. and complete obstruction of the femoral vein. C. Same case. Photomicrograph reveals numerous multinucleated giant cells.
On plain radiographs, the deep tumor presents as a soft-tissue mass usually well demarcated from the adjacent structures although displacing the intermuscular adipose tissue. No radiographic evidence for ossification or calcification within the tumor was found in our 2 patients or in 27 previous cases for which the information was given. Only a few of the reported cases showed evidence of periosteal reaction or cortical invasion of the adjacent bones (4). The striking angiographic findings noted in our 2 cases have seldom been emphasized in the literature (6). In both, the tumor presented as a rather well-circumscribed hypervascular mass. The femoral artery segment adjacent to the tumor was encased in one pa-
August 1976
tient (Fig. 1, A) and merely displaced by the tumor in the other. In both instances, there was early opacification of the draining veins, probably due to arteriovenous shunting within the tumor (Fig. 1, A and B). The veins may demonstrate evidence for invasion of the wall or obstruction of the lumen (Fig. 1, B and Fig. 2, e). It should be noted that the giant cell tumors of the . soft tissue occur in two varieties: small, superficial and large, deep tumors . The former lesions arise from the superficial fascia and subcutis. They may occur in the trunk, face, leg, and knee. Only two superficial lesions were seen in the thigh, which is usually involved by deep large tumors. The superficial tumors are generally 3-5 cm in diameter, well-circumscribed and usually soft to firm in consistency. Deep-seated tumors arise from deep fascia, skeletal muscles and tendons. The thigh and leg are the most frequent site of involvement. These lesions range in size from 4.5 to 30 cm in diameter, with an average of 12.2 cm. The tumors are usually soft to firm in consistency and appear pseudo-encapsulated. The cut surface is usually white to tan and shows areas of necrosis and hematoma. Histologically, the tumor consists of multinucleated and pleomorphic giant cells, mononuclear histiocytes and fibroblasts. These cellular elements are usually arranged in a multinodular pattern which may contain areas of necrosis and hemorrhage. A preoperative diagnosis may be suggested on the basis of the radiographic features. On plain films, the tumor usually does not show any calcification, or ossification and rarely involves the adjacent bone. However, definite criteria of malignancy can be shown on angiography. The anqioqraphlc features of these giant cell tumors include demonstration of neovascularity, displacement and encasement of the adjacent arterial branches, obstruction of larger adjacent veins, and rapid shunting with early filling of other draining veins. These features of malignancy are similar to those observed with other malignant soft-tissue tumors such as fibrosarcoma, liposarcoma, or rhabdomyosarcoma. However, the findings do permit differentiation from benign hypervascular soft-tissue lesions such as neurofibroma, hemangioma, or abscess. Furthermore, the tumor stain in the capillary phase can demonstrate areas of avascularity due to hemorrhage or necrosis, and provide a guide for selection of an optimal site for biopsy. Although the number of cases of deep-seated malignant giant cell tumors is not sufficient to indicate the most optimal mode of treatment, it appears that the prognosis is not favorable in most cases despite amputation and/or radiation therapy. However, the prognosis of superficial lesions is favorable after surgical excision.
REFERENCES 1. Ackerman LV: 1953. P 697
Surgical Pathology. St. Louis, C. V. Mosby,
Vol. 120
MALIGNANT GIANT CELL TUMOR OF THE SOFT TISSUE
331
Diagnostic Radiology
Fig. 2. CASE II. A. Plain film of the left thigh reveals a soft-tissue mass with no evidence of calcification, ossification or bony abnormalities. Band C. Arterial and venous phase reveal a large mass measuring 12 X 7 em with numerous abnormal vessels, encasement of second and third degree arteries, tumor stain and large abnormal veins compatible with malignancy. Area of less vascularity in the lower part of the mass resulted from hematoma. Note branches of superficial and deep femoral arteries supplying the tumor.
2. Christopher F: Giant cell sarcoma apparently arising from the aponeurosis of the forearm. JAMA 87:167-168, 17 Jul 1926 3. Fine G, Stout AP: Osteogenic sarcoma of the extraskeletal soft tissues. Cancer 9:1027-1043, Sep-Oct 1956 4. Guccion JG, Enzinger FM: Malignant giant cell tumor of soft parts . An analysis of 32 cases. Cancer 29:1518-1529, Jun 1972 5. Hirsch EF: Radial inclusions of giant cells. Arch Pathol 5: 665-682, Nov 1935
6. Martel WM, Abell MR: Radiologic evaluation of soft tissue tumors. A retrospective study. Cancer 32:352-366, August 1973
Department of Radiology MCV Station Box 728 Richmond, Va. 23298
Diagnostic Radiology
Malignant Giant Cell Tumor of the Soft Tissue: Angiographic Manifestations 1 Issa Yaghmal, M.D. Two cases of malignant giant cell tumor originating within the soft tissues of the thigh are reported. In both instances, angiography demonstrated hypervascular masses with arterial and venous abnormalities indicative of an aggressive malignancy. The histopathologic appearance of the lesions was similar to that of osseous giant cell tumors. The clinical and radiographic features of this rare entity are briefly reviewed. INDEX TERMS: Neoplasms, blood supply. Neoplasms, giant cell • Soft tissues, neoplasms • Thigh, neoplasms
Radiology 120:329-331, August 1976
•
•
HE extra-osseous occurrence of giant cell tumor is
CASE II. A 54-year-old man was referred for further evaluation of a large, soft-tissue mass in the lateral aspect of the left thigh. The lesion had tripled in size since the patient had become aware of it 10 months earlier. During the past four months, he had experienced pain at the site of the lesion while walking, and during palpation, and had lost 6 kg in weight. Physical examination disclosed a large mass within the soft tissues of the thigh. It appeared adherent to the adjacent femur. There was also enqorqement of the superficial veins over the lesion, and the left inguinal lymph nodes were palpable. Radiographs of the left thigh did not show evidence for destruction of the femur or calcification in the soft tissues. A needle biopsy contained mostly necrotic tissue. Left femoral angiography was then performed which showed a hypervascular mass measuring 7 X 15 cm displacing the superficial femoral artery medially. The vascular supply of the lesion was derived from branches of deep and superficial femoral arteries. Within the mass was an avascular zone suggestive of necrosis or a hematoma. There was early visualization of the femoral veins proximal to the tumor (Fig. 2, B and C). An open biopsy of the lesion was performed in the vascular portion of the lesion and multiple specimens were obtained. Histologic examination demonstrated the presence of a giant cell tumor of soft tissue composed of multinucleated giant cells, mononuclear histiocytes and fibrocytes. On the patient's request, only a local excision of the tumor was carried out and supplemented by radiotherapy. The postoperative course was uneventful. No evidence for recurrence has been found over six months of follow-up.
T
rare. The pathologic features of these soft-tissue tumors resemble those of giant cell tumors of the bone but their clinical course is usually more progressive and often fatal. Approximately 50 cases have b~en recorded in the literature, sometimes under designations such as malignant giant cell tumor of tendon sheath (1), extraskeletal osteolytic osteosarcoma (3), or giant cell fascial sarcoma (6). In this report, the angiographic findings in 2 patients with giant cell tumor of the soft tissue will be described and pertinent clinical and pathologic features of the malignancy will be reviewed. CASE REPORTS CASE I. A 62-year-old woman was admitted for evaluation of a tender soft-tissue mass in the right thigh, which had been noticed by the patient six months earlier. More recently, however, the lesion had become larger and painful on palpation. No history of trauma was present. Physical examination revealed a well-circumscribed, palpable, tender mass in the posteromedial aspect of the right thigh. The lesion appeared deeply located within the soft tissues, and mobile with respect to the femur. The regional lymph nodes were not palpable. Plain radiographs of the right thigh demonstrated an oval-shaped, soft-tissue mass demarcated from the surrounding muscles. There were no soft-tissue calcifications. The femur appeared intact. A femoral angiogram showed a hypervascular tumor measuring 6-8 cm 1n diameter. The lumen of the superficial femoral artery appeared narrowed in the segment adjacent to the lesion. An intense tumor stain occurred in the capillary phase. There was early opacification of numerous veins around the tumor resulting in filling of the superficial femoral vein (Fig. 1, A and B). The femoral vein was completely obstructed. Microscopic evaluation of biopsy specimens revealed the presence of a tumor primarily consisting of multinucleated giant cells and mononuclear histiocytes. These were arranged in a multinodular pattern, with some areas of intraluminal extension of the cells into the adjacent veins (Fig. 1, C). The pathologic diagnosis was malignant giant cell tumor of soft tissue. Subsequently disarticulation of the right leg was performed. However, the patient died six months later due to metastatic spread of the tumor to the lungs, liver, and the left thigh.
DISCUSSION
Approximately 50 previously reported cases of giant cell tumor of the soft tissues were noted in patients ranging in age from 9 to 87 years (4). Including the present 2 cases, the average age at the time of diagnosis is 57 years, with the majority of tumors found during the fifth or sixth decade of life. Fifty-seven per cent of patients have been male and 43 % female. The tumor usually presents as a painful and rapidly growing mass. The usual sites of involvement are the thighs for deep-seated lesions and the leg for the superficial ones. Less commonly, the lesions are located in the abdominal wall, shoulders, arms, and face. Compared to the superficial lesions, the deep-seated tumors have a less favorable prognosis, probably because of delayed recognition.
1 From the Department of Radiology, Medical College of Virginia, Virginia Commonwealth University, Richmond, Va. Accepted for publication in November 1975. shan
329
330
ISSA Y AGHMAI
Fig. 1. CASE I. A. arterial and B, venous phase reveal encasement of the femoral artery , a hypervascular mass with numerous abnormal neovascularities. early visualization of the femoral vein in the early arterial phase (arrow), uneven tumor stain, large collateral veins. and complete obstruction of the femoral vein. C. Same case. Photomicrograph reveals numerous multinucleated giant cells.
On plain radiographs, the deep tumor presents as a soft-tissue mass usually well demarcated from the adjacent structures although displacing the intermuscular adipose tissue. No radiographic evidence for ossification or calcification within the tumor was found in our 2 patients or in 27 previous cases for which the information was given. Only a few of the reported cases showed evidence of periosteal reaction or cortical invasion of the adjacent bones (4). The striking angiographic findings noted in our 2 cases have seldom been emphasized in the literature (6). In both, the tumor presented as a rather well-circumscribed hypervascular mass. The femoral artery segment adjacent to the tumor was encased in one pa-
August 1976
tient (Fig. 1, A) and merely displaced by the tumor in the other. In both instances, there was early opacification of the draining veins, probably due to arteriovenous shunting within the tumor (Fig. 1, A and B). The veins may demonstrate evidence for invasion of the wall or obstruction of the lumen (Fig. 1, B and Fig. 2, e). It should be noted that the giant cell tumors of the . soft tissue occur in two varieties: small, superficial and large, deep tumors . The former lesions arise from the superficial fascia and subcutis. They may occur in the trunk, face, leg, and knee. Only two superficial lesions were seen in the thigh, which is usually involved by deep large tumors. The superficial tumors are generally 3-5 cm in diameter, well-circumscribed and usually soft to firm in consistency. Deep-seated tumors arise from deep fascia, skeletal muscles and tendons. The thigh and leg are the most frequent site of involvement. These lesions range in size from 4.5 to 30 cm in diameter, with an average of 12.2 cm. The tumors are usually soft to firm in consistency and appear pseudo-encapsulated. The cut surface is usually white to tan and shows areas of necrosis and hematoma. Histologically, the tumor consists of multinucleated and pleomorphic giant cells, mononuclear histiocytes and fibroblasts. These cellular elements are usually arranged in a multinodular pattern which may contain areas of necrosis and hemorrhage. A preoperative diagnosis may be suggested on the basis of the radiographic features. On plain films, the tumor usually does not show any calcification, or ossification and rarely involves the adjacent bone. However, definite criteria of malignancy can be shown on angiography. The anqioqraphlc features of these giant cell tumors include demonstration of neovascularity, displacement and encasement of the adjacent arterial branches, obstruction of larger adjacent veins, and rapid shunting with early filling of other draining veins. These features of malignancy are similar to those observed with other malignant soft-tissue tumors such as fibrosarcoma, liposarcoma, or rhabdomyosarcoma. However, the findings do permit differentiation from benign hypervascular soft-tissue lesions such as neurofibroma, hemangioma, or abscess. Furthermore, the tumor stain in the capillary phase can demonstrate areas of avascularity due to hemorrhage or necrosis, and provide a guide for selection of an optimal site for biopsy. Although the number of cases of deep-seated malignant giant cell tumors is not sufficient to indicate the most optimal mode of treatment, it appears that the prognosis is not favorable in most cases despite amputation and/or radiation therapy. However, the prognosis of superficial lesions is favorable after surgical excision.
REFERENCES 1. Ackerman LV: 1953. P 697
Surgical Pathology. St. Louis, C. V. Mosby,
Vol. 120
MALIGNANT GIANT CELL TUMOR OF THE SOFT TISSUE
331
Diagnostic Radiology
Fig. 2. CASE II. A. Plain film of the left thigh reveals a soft-tissue mass with no evidence of calcification, ossification or bony abnormalities. Band C. Arterial and venous phase reveal a large mass measuring 12 X 7 em with numerous abnormal vessels, encasement of second and third degree arteries, tumor stain and large abnormal veins compatible with malignancy. Area of less vascularity in the lower part of the mass resulted from hematoma. Note branches of superficial and deep femoral arteries supplying the tumor.
2. Christopher F: Giant cell sarcoma apparently arising from the aponeurosis of the forearm. JAMA 87:167-168, 17 Jul 1926 3. Fine G, Stout AP: Osteogenic sarcoma of the extraskeletal soft tissues. Cancer 9:1027-1043, Sep-Oct 1956 4. Guccion JG, Enzinger FM: Malignant giant cell tumor of soft parts . An analysis of 32 cases. Cancer 29:1518-1529, Jun 1972 5. Hirsch EF: Radial inclusions of giant cells. Arch Pathol 5: 665-682, Nov 1935
6. Martel WM, Abell MR: Radiologic evaluation of soft tissue tumors. A retrospective study. Cancer 32:352-366, August 1973
Department of Radiology MCV Station Box 728 Richmond, Va. 23298
