Angiomatosis of the Metacarpal Skeleton--.l. Btanco Tuhon and F. Perez Gonzalez
ANGIOMATOSIS OF THE METACARPAL SKELETON J. B L A N C O TUlqON and F. P E R E Z G O N Z A L E Z , Madrid SUMMARY We present a case of a tumonr of the right hand. After carrying out a biopsy it was diagnosed as skeletal angiomatosis which, considering the marked osteolysis present, falls within the description of Gorham's disease. The three bones concerned in the turnout were excised and replaced with a bone graft, and the metacarpophalangeal joints preserved with Swanson prostheses. INTRODUCTION The vascular tumours of bone, Haemangioma and Lymphangioma, are of very rare appearance, and of these the most common locations are the vertebral bodies and the bones of the skull. That is why the observation of haemangiomas in other bones of the skeleton besides those quoted is very infrequent. Bucy and Capp (1930) found only fifteen cases up to 1930, and we ourselves in our review of the literature have found only twenty-five cases (Spjut 1962, and Ackerman 1962). Skeletal angiomatosis, also called Angiomatosis or Lymphangiomatosis cystica (Jacobs and Kimmelstiel 1953) consists in the appearance of multicentric vascular tumours with the presence of blood and lymphatic vessels within the bones. On X-rays one appreciates the existence of lacunae, similar to angioma of bone, but multicentric. On section macroscopically, a series of irregular cavities appear surrounded by sclerotic bone. Microscopically, there are formations of lymphatic origin, and foci of vascular growth. In no case has malignant change been described. Some cases become stationary, but others produce a slow and progressive growth including occasionally the development of sclerosis. The entity known as massive osteolysis or phantom disease of bone, or disappearing bone was described by Gorham (1954) and cited later by other authors like Johnson and McClure (1958), Hambach (1958) and Halliday (1964). It is a rare and destructive disease of bone, which histologically is a haemangioma or lymphangioma or even a combination of both. It takes a slow course, insidious and progressive until osteolysis exists, so intense as to lead to complete absorption of the bone, and because of the characteristic histological appearance of angioma or lymphangioma is regarded by the above authors as a variety of skeletal angiomatosis. CASE REPORT The case which is the object of the report was of a man of twenty-four years of age who began three years before to notice a small lump on the back of the right hand. The tumour grew slowly and progressively. He was studied in another centre, where an inconclusive biopsy was carried out. J. Blanco Tufion, M.D., Oruro, 14. Madrid 16, Spain. From The Ciudad Sanitaria "La Paz", Madrid, Dept. of Plastic Surgery. Head, Prof. Dr. F. Enriquez de Salamanca. 88
The Hand--Vol. 9
No. I
1977
Angiomatosis o[ the Metacarpal Skeleton--J. Bl~anco Tu~on and F. Perez Gonzalez
Fig. 1.
Clinical aspect and X-rays of the tumour before operation.
At our first examination he had a hard turnout with stable skin (Fig. 1). In the first web space there was a scar of the biopsy. Flexion and extension were practically normal, and there was a distinct ulnar deviation of the fingers. The X-ray showed the tumour affecting the second, third and fourth metacarpals, which were completely destroyed with the presence of lacunae of varying sizes. The diagnoses of chondroma, enchondroma, chondrosarcoma and angioma of bone were considered possible. An intra-operative biopsy was performed which demonstrated the benign nature of the turnout. Through a median dorsal incision the second, third and fourth metacarpals were extirpated and also the distal row of the carpus. The articular surfaces of the proximal row were freshened, and the defect filled with a bone graft taken from the iliac crest, and fixed with Kirschner wires. The metacarpophalangeal joints of the index, middle and ring fingers were replaced with Swanson prostheses, to maintain the mobility of the fingers. (Figs. 2a and b).
Fig. 2a. X-ray after operation, and 2b Swanson's prostheses embedded in the osseous graft, and reconstituting the metacarpophalangeal joints. The H a n d - - V o l . 9
No. 1
1977
89
A ngiomatosis of the Metacarpal Skeleton--J. Bl.anco Tu~on and F. Perez Gonzalez
PATHOLOGICAL EXAMINATION
The piece extirpated measured 12 x 8 x 6 centimetres, corresponding to the three metacarpals whose hardness was appreciably diminished. Microscopically, multiple sections showed very thin osseous trabeculae which have even in some zones disappeared, the medullary space being occupied by lax connective tissue, with abundant thin walled vascular formations with a single layered endothelial lining. Some of these were dilated, and in some places there was dense infiltration with lymphocytes, histiocytes and eosinophils (Fig. 3). Intermixed with this tissue, there were cellular nests, constituted by endothelial cells, which delimited small vascular windows, without any cytological signs of malignancy. The process was interpreted as a skeletal angiomatosis with marked osseous resorption of the bones affected. The differential diagnosis from haemangioendothelioma was based on the absence of any cytological signs of malignancy, the presence of ample zones of a hamartomatous aspect, well differentiated, as well as the differentation of totally mature vascular tissue in the periphery of the nests.
CONCLUSION
The appearance of a multicentric bony tumour with loci of angiomatosis and lymphangiomatosis and proliferation of endothelial vascular foci leads us to the diagnosis of skeletal angiomatosis. The great osteolysis which may even produce the disappearance of abundant osseous trabeculae leads us to the conclusion that this is a case of the entity described by Gorham as massive osteolysis. With the surgical technique adopted since the case was not a malignant tumour, we were able to preserve the important function of a man's right hand.
Fig. 3. 90
Histology shows proliferation of vascular spaces with thin endothelial walls, and the presence of osseous trabeculae partially reabsorbed and surrounded by vascular spaces. The H a n d - - V o l . 9
No. I
1977
A ngiomatosis of the Metacarpal Skeleton--J. B[anco Tu~on and F. Perez Gonzalez REFERENCES
ACKERMAN, L. V., SPJUT, H. J. Tumors of Bone and Cartilage. Armed Forces Institute of Pathology Fascicule 4, 235, Washington, 1962. BUCY, P. and CAPP, C. S. (1930) Primary Hemangioma of Bone with Special Reference to Roentgenologic Diagnosis. American Journal of Roentgenology. 23: 1-7. GORHAM, L. W., WRIGHT, A. W., SHULTZ, H. H. and MAXON, F. C. Jr. (1954) Disappearing Bones. A Rare form of Massive Osteolysis. Report of Two Cases, One with Autopsy Findings. American Journal of Medicine, 17: 674-682. HALLIDAY, D. R., DAHLIN, D. C., PUGH, D. G. and YOUNG, H. H. (1964) Massive Osteolysis and Angiomatosis. Radiology, 82: 637-644. HAMBACH, R., PUJMAN, J. and MALY, V. (1958) Massive Osteolysis Due to Hemangiomatosis. Report of a Case of Gorham's Disease with Autopsy. Radiology, 71" 43--47. JACOBS, J. E. and KIMMELSTIEL, P. (1953) Cystic Angiomatosis Of The Skeletal System. Journal of Bone and Joint Surgery, 35-A: 409-420, 464. JOHNSON, P. M. and McCLURE, J. G. (1958) Observations on Massive Osteolysis. A Review of the Literature and Report of a Case. Radiology, 7l: 28-42. SPJUT, H. J., LINDBOM, A. (1962) Skeletal Angiomatosis. Report of Two Cases. Acta Pathologica et Microbiologica Scandinavica, 55: 49-58.
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1977
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ANGIOMATOSIS OF THE METACARPAL SKELETON J. B L A N C O TUlqON and F. P E R E Z G O N Z A L E Z , Madrid SUMMARY We present a case of a tumonr of the right hand. After carrying out a biopsy it was diagnosed as skeletal angiomatosis which, considering the marked osteolysis present, falls within the description of Gorham's disease. The three bones concerned in the turnout were excised and replaced with a bone graft, and the metacarpophalangeal joints preserved with Swanson prostheses. INTRODUCTION The vascular tumours of bone, Haemangioma and Lymphangioma, are of very rare appearance, and of these the most common locations are the vertebral bodies and the bones of the skull. That is why the observation of haemangiomas in other bones of the skeleton besides those quoted is very infrequent. Bucy and Capp (1930) found only fifteen cases up to 1930, and we ourselves in our review of the literature have found only twenty-five cases (Spjut 1962, and Ackerman 1962). Skeletal angiomatosis, also called Angiomatosis or Lymphangiomatosis cystica (Jacobs and Kimmelstiel 1953) consists in the appearance of multicentric vascular tumours with the presence of blood and lymphatic vessels within the bones. On X-rays one appreciates the existence of lacunae, similar to angioma of bone, but multicentric. On section macroscopically, a series of irregular cavities appear surrounded by sclerotic bone. Microscopically, there are formations of lymphatic origin, and foci of vascular growth. In no case has malignant change been described. Some cases become stationary, but others produce a slow and progressive growth including occasionally the development of sclerosis. The entity known as massive osteolysis or phantom disease of bone, or disappearing bone was described by Gorham (1954) and cited later by other authors like Johnson and McClure (1958), Hambach (1958) and Halliday (1964). It is a rare and destructive disease of bone, which histologically is a haemangioma or lymphangioma or even a combination of both. It takes a slow course, insidious and progressive until osteolysis exists, so intense as to lead to complete absorption of the bone, and because of the characteristic histological appearance of angioma or lymphangioma is regarded by the above authors as a variety of skeletal angiomatosis. CASE REPORT The case which is the object of the report was of a man of twenty-four years of age who began three years before to notice a small lump on the back of the right hand. The tumour grew slowly and progressively. He was studied in another centre, where an inconclusive biopsy was carried out. J. Blanco Tufion, M.D., Oruro, 14. Madrid 16, Spain. From The Ciudad Sanitaria "La Paz", Madrid, Dept. of Plastic Surgery. Head, Prof. Dr. F. Enriquez de Salamanca. 88
The Hand--Vol. 9
No. I
1977
Angiomatosis o[ the Metacarpal Skeleton--J. Bl~anco Tu~on and F. Perez Gonzalez
Fig. 1.
Clinical aspect and X-rays of the tumour before operation.
At our first examination he had a hard turnout with stable skin (Fig. 1). In the first web space there was a scar of the biopsy. Flexion and extension were practically normal, and there was a distinct ulnar deviation of the fingers. The X-ray showed the tumour affecting the second, third and fourth metacarpals, which were completely destroyed with the presence of lacunae of varying sizes. The diagnoses of chondroma, enchondroma, chondrosarcoma and angioma of bone were considered possible. An intra-operative biopsy was performed which demonstrated the benign nature of the turnout. Through a median dorsal incision the second, third and fourth metacarpals were extirpated and also the distal row of the carpus. The articular surfaces of the proximal row were freshened, and the defect filled with a bone graft taken from the iliac crest, and fixed with Kirschner wires. The metacarpophalangeal joints of the index, middle and ring fingers were replaced with Swanson prostheses, to maintain the mobility of the fingers. (Figs. 2a and b).
Fig. 2a. X-ray after operation, and 2b Swanson's prostheses embedded in the osseous graft, and reconstituting the metacarpophalangeal joints. The H a n d - - V o l . 9
No. 1
1977
89
A ngiomatosis of the Metacarpal Skeleton--J. Bl.anco Tu~on and F. Perez Gonzalez
PATHOLOGICAL EXAMINATION
The piece extirpated measured 12 x 8 x 6 centimetres, corresponding to the three metacarpals whose hardness was appreciably diminished. Microscopically, multiple sections showed very thin osseous trabeculae which have even in some zones disappeared, the medullary space being occupied by lax connective tissue, with abundant thin walled vascular formations with a single layered endothelial lining. Some of these were dilated, and in some places there was dense infiltration with lymphocytes, histiocytes and eosinophils (Fig. 3). Intermixed with this tissue, there were cellular nests, constituted by endothelial cells, which delimited small vascular windows, without any cytological signs of malignancy. The process was interpreted as a skeletal angiomatosis with marked osseous resorption of the bones affected. The differential diagnosis from haemangioendothelioma was based on the absence of any cytological signs of malignancy, the presence of ample zones of a hamartomatous aspect, well differentiated, as well as the differentation of totally mature vascular tissue in the periphery of the nests.
CONCLUSION
The appearance of a multicentric bony tumour with loci of angiomatosis and lymphangiomatosis and proliferation of endothelial vascular foci leads us to the diagnosis of skeletal angiomatosis. The great osteolysis which may even produce the disappearance of abundant osseous trabeculae leads us to the conclusion that this is a case of the entity described by Gorham as massive osteolysis. With the surgical technique adopted since the case was not a malignant tumour, we were able to preserve the important function of a man's right hand.
Fig. 3. 90
Histology shows proliferation of vascular spaces with thin endothelial walls, and the presence of osseous trabeculae partially reabsorbed and surrounded by vascular spaces. The H a n d - - V o l . 9
No. I
1977
A ngiomatosis of the Metacarpal Skeleton--J. B[anco Tu~on and F. Perez Gonzalez REFERENCES
ACKERMAN, L. V., SPJUT, H. J. Tumors of Bone and Cartilage. Armed Forces Institute of Pathology Fascicule 4, 235, Washington, 1962. BUCY, P. and CAPP, C. S. (1930) Primary Hemangioma of Bone with Special Reference to Roentgenologic Diagnosis. American Journal of Roentgenology. 23: 1-7. GORHAM, L. W., WRIGHT, A. W., SHULTZ, H. H. and MAXON, F. C. Jr. (1954) Disappearing Bones. A Rare form of Massive Osteolysis. Report of Two Cases, One with Autopsy Findings. American Journal of Medicine, 17: 674-682. HALLIDAY, D. R., DAHLIN, D. C., PUGH, D. G. and YOUNG, H. H. (1964) Massive Osteolysis and Angiomatosis. Radiology, 82: 637-644. HAMBACH, R., PUJMAN, J. and MALY, V. (1958) Massive Osteolysis Due to Hemangiomatosis. Report of a Case of Gorham's Disease with Autopsy. Radiology, 71" 43--47. JACOBS, J. E. and KIMMELSTIEL, P. (1953) Cystic Angiomatosis Of The Skeletal System. Journal of Bone and Joint Surgery, 35-A: 409-420, 464. JOHNSON, P. M. and McCLURE, J. G. (1958) Observations on Massive Osteolysis. A Review of the Literature and Report of a Case. Radiology, 7l: 28-42. SPJUT, H. J., LINDBOM, A. (1962) Skeletal Angiomatosis. Report of Two Cases. Acta Pathologica et Microbiologica Scandinavica, 55: 49-58.
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