1991, The British Journal of Radiology, 64, 894-897
Sclerosing haemangiomatosis: a case report and review of the literature By Ibrahim Fikry Abdelwahab, M D Department of Radiology of The Mount Sinai Medical Center, CUNY, New York, USA
{Received December 1990)
Keywords: Haemangiomatosis, Sclerotic bone deposits, Benign skeletal tumours
Abstract. A case of sclerotic haemangiomatosis of bone without accompanying lytic lesions is presented.
Haemangiomatosis of bone, though rare, is a well established entity (Ritchie & Zeier, 1956; Wallis et al, 1964; Cutierrez & Spjut, 1972; Feldman, 1978; Wilner, 1981). Presentation with purely sclerotic lesions, without accompanying lytic lesions, is extremely rare. The lack of familiarity and awareness of such a presentation can lead to late diagnosis and confusion with metastatic bone cancer, lymphoma, carcinoid and other unrelated diseases.
Computed tomography (CT) of the abdomen with contrast medium enhancement revealed an enlarged spleen with increased attenuation in its superior part, for which the diagnosis of haemangioma of the spleen was entertained (Fig. 2). Histopathology of an open biopsy from a large sclerotic lesion in the right iliac wing revealed extensive sclerosis of the cancellous bone with capillary dilatation consistent with the primary histological diagnosis, 11 years earlier, of capillary haemangioma.
Case report
A 36-year-old white male was admitted for investigation of low back pain radiating to the left leg. Twelve years earlier, he suffered, in a fight, contusions to the upper torso and pain in the lower back. Four months later, because of his progressive back pain, he was hospitalized. Physical examination at that time was unremarkable. A routine radiograph of the pelvis showed a sclerotic lesion in the left iliac bone, which was then biopsied. Histopathology revealed a capillary haemangioma. Post-operatively, the patient developed infection of the left sacroiliac joint with gram-negative coliform bacteria and underwent fusion of the left sacroiliac joint 18 months later because of recurrent back and left flank pain. In our hospital a skeletal survey revealed diffuse, sharply defined, densely sclerotic lesions varying in size from a few millimetres to a few centimetres, involving both scapulae, ribs, thoracolumbar spine, pelvis and proximal femora (Fig. 1). Laboratory studies were within normal limits. Chest radiographs, barium meal and enema and intravenous pyelography were all unremarkable in a search for neoplasia. Radionuclide scanning with technetium 99m diphosphonate did not reveal any focal bony abnormalities. Radionuclide scanning of the liver and spleen with technetium 99m sulphur colloid revealed increased uptake of the radioisotope in the superior part of the slightly enlarged spleen. Address for correspondence: Box 1234, Department of Radiology, Mount Sinai Medical Center, 1 Gustave L. Levy Place, New York, NY 10029-6574, USA. 894
Discussion Wallis et al (1964) proposed a classification for diffuse skeletal haemangiomatosis involving multiple sites. They reviewed 26 cases including two of their own. They classified these 26 cases into two groups. Group 1, without visceral involvement, included nine cases, and Group 2, with visceral involvement, included 17 cases. They then subdivided Group 2 into (a) histologically benign (13 out of 17 cases) and (b) histologically malignant (four out of 17 cases). Radiographic examination of these cases revealed a combination of lytic and sclerotic lesions, multiple expanding cysts and generalized osseous demineralization with vertebral collapse. Lytic lesions were present in all cases and sclerotic lesions in only 50%, while expansion of bone was seen in one-third of Group 1 and half of Group 2 cases. Increasing sclerosis in some areas, with lysis in others, often occurred in the same patient over a period of years. They concluded that progressive sclerosis predominated in older patients and in those followed over a long period of time. However, neither they nor others could explain the propensity for vascular tumours to cause bone destruction in some cases and bone production in others. They and other authors noticed that diffuse haemangiomatosis limited to the skeleton appears to be a benign condition in spite of its widespread involvement (Waldron & Zeller, 1969; Schajowicz et al, 1978). The skeletal distribution of the lesions in their series varied but almost always involved the spine, ribs and pelvis. The skull, shoulders and long bones were frequently involved. The British Journal of Radiology, October 1991
Sclerosing haemangiomatosis
Figure 1. (a) Lateral view of the thoracic spine, (b) lateral view of the lumbar spine, and (c) frontal view of the pelvis showing dense, sharply denned sclerosis.
Waldron and Zeller (1969) followed a case of diffuse skeletal haemangiomatosis with visceral involvement for 16 years and noticed that the lesions which were lytic and sclerotic involving the ribs, spine and pelvis did not Vol. 64, No. 766
change. Spjut and Lindrom (1962) followed another case for 32 years, and found that some lesions stabilized while others regressed as the patient aged. The observation of the authors was supported by Schajowcz et al 895
/. F. Abdelwahab
lesions of the spine and pelvis. The later radiographs showed predominantly sclerotic lesions though small areas of osteolysis were noted. Our case is unusual in that it presented with purely sclerotic lesions without accompanying lytic lesions. There was no expansion of the involved bone. The skull and the bones distal to the elbows and knees were not involved. As in similar reports, the only visceral involvement besides the bone was the spleen (Ritchie & Zeier, 1956; Sammis et al, 1971; Dadash-Zadeh & Schwartz, 1976). In spite of many reports indicating either stabilization or regression of the lesions as the patient ages, we noticed in this case that not only had the sclerotic lesions markedly increased in size (some tripled in size in less than 10 years), but also that new sclerotic lesions had appeared (Fig. 3). Only a single other report of a case that presented with purely sclerotic lesions involving the right parietal bone and sacrum has been found (Raden & Mantz, 1955). Figure 2. Computed tomographic scan of the liver and spleen after contrast medium enhancement shows increased attenuation in the superior part of the enlarged spleen consistent with haemangioma. Note the multiple sclerotic densities in the cancellous and cortical bone of the lower thoracic vertebrae.
(1978). Two of three cases of skeletal haemangiomatosis followed by them showed spontaneous regression. Feldman (1978) mentioned that diffuse skeletal haemangiomatosis may become sclerotic following surgical procedures such as curettage, or even spontaneously, and that de novo sclerotic haemangiomatosis can occur. The case presented in her book was that of a middle-aged man who was followed for 26 years with visceral involvement and documented lytic and sclerotic
References CUTIERREZ, R. M. & SPJUT, H. J., 1972. Skeletal angiomatosis.
Report of three cases and review of the literature. Clinical Orthopaedics, 85, 82-96. DADASH-ZADEH, M. & SCHWARTZ, A. D., 1976. Skeletal and
splenic hemangiomatosis with consumption coagulopathy: response to splenectomy. Pediatrics, 57, 803-807. FELDMAN, F., 1978. Radiology /pathology and Immunology of Bones and Joints. A Review of Current Concepts (Appleton/ Century/Crofts, New York), pp. 7-22. RADEN, P. A. & MANTZ, JR, F. A., 1955. Benign systemic
hemangiomatosis without cutaneous involvement: confusing diagnostic problem. Radiology, 64, 252-257.
a
RITCHIE, G. & ZEIER, F. G., 1956. Hemangiomatosis of the
skeleton and the spleen. Journal of Bone & Joint Surgery, 38A, 115-121.
(a) (b) Figure 3. (a) Frontal radiograph of the pelvis showing fusion of the left sacroiliac joint and a big defect in the left iliac bone (both surgical), (b) Frontal radiograph of the pelvis taken 8 years later, which reveals that the sclerotic densities have increased in size. The lesion in the outer part of the right iliac bone, the site of the second biopsy, had tripled in size (arrows) and new sclerotic lesions had appeared in the pubic bones and proximal femora. The British Journal of Radiology, October 1991
Sclerosing haemangiomatosis SAMMIS JR, A. F., WEITZMAN, S. & ARCAMANO, J. P., 1971.
WALDRON, R. L. & ZELLER, J. A.,
1969. Diffuse
skeletal
Hemangiolymphangioma of spleen and bone. New York State Journal of Medicine, 14, 1762-1764.
hemangiomatosis with visceral involvement. Journal of the Canadian Association of Radiologists, 20, 119-123.
SCHAJOWICZ, F., AIELLO, C. L., FRANCONE, M. V. & GIANNINI,
WALLIS, L. A., ASCH, T. & MAISEL, B., 1964. Diffuse skeletal
R. E., 1978. Cystic angiomatosis (hamartous haemolyphangomatosis) of bone. A clinico-pathological study of three cases. Journal of Bone & Joint Surgery, 60B, 100-106. SPJUT, H. J. & LINDROM, A., 1962. Skeletal angiomatosis. Report of two cases. Ada Pathologica et Microbiologica Scandinavica, 55, 49-58.
hemangiomatosis: report of two cases and review of the literature. American Journal of Medicine, 37, 545-563. WILNER, D., 1981. Radiology of Bone Tumors and Allied Disorders (Saunders, Philadelphia), pp. 708-723.
Vol. 64, No. 766
897
Sclerosing haemangiomatosis: a case report and review of the literature By Ibrahim Fikry Abdelwahab, M D Department of Radiology of The Mount Sinai Medical Center, CUNY, New York, USA
{Received December 1990)
Keywords: Haemangiomatosis, Sclerotic bone deposits, Benign skeletal tumours
Abstract. A case of sclerotic haemangiomatosis of bone without accompanying lytic lesions is presented.
Haemangiomatosis of bone, though rare, is a well established entity (Ritchie & Zeier, 1956; Wallis et al, 1964; Cutierrez & Spjut, 1972; Feldman, 1978; Wilner, 1981). Presentation with purely sclerotic lesions, without accompanying lytic lesions, is extremely rare. The lack of familiarity and awareness of such a presentation can lead to late diagnosis and confusion with metastatic bone cancer, lymphoma, carcinoid and other unrelated diseases.
Computed tomography (CT) of the abdomen with contrast medium enhancement revealed an enlarged spleen with increased attenuation in its superior part, for which the diagnosis of haemangioma of the spleen was entertained (Fig. 2). Histopathology of an open biopsy from a large sclerotic lesion in the right iliac wing revealed extensive sclerosis of the cancellous bone with capillary dilatation consistent with the primary histological diagnosis, 11 years earlier, of capillary haemangioma.
Case report
A 36-year-old white male was admitted for investigation of low back pain radiating to the left leg. Twelve years earlier, he suffered, in a fight, contusions to the upper torso and pain in the lower back. Four months later, because of his progressive back pain, he was hospitalized. Physical examination at that time was unremarkable. A routine radiograph of the pelvis showed a sclerotic lesion in the left iliac bone, which was then biopsied. Histopathology revealed a capillary haemangioma. Post-operatively, the patient developed infection of the left sacroiliac joint with gram-negative coliform bacteria and underwent fusion of the left sacroiliac joint 18 months later because of recurrent back and left flank pain. In our hospital a skeletal survey revealed diffuse, sharply defined, densely sclerotic lesions varying in size from a few millimetres to a few centimetres, involving both scapulae, ribs, thoracolumbar spine, pelvis and proximal femora (Fig. 1). Laboratory studies were within normal limits. Chest radiographs, barium meal and enema and intravenous pyelography were all unremarkable in a search for neoplasia. Radionuclide scanning with technetium 99m diphosphonate did not reveal any focal bony abnormalities. Radionuclide scanning of the liver and spleen with technetium 99m sulphur colloid revealed increased uptake of the radioisotope in the superior part of the slightly enlarged spleen. Address for correspondence: Box 1234, Department of Radiology, Mount Sinai Medical Center, 1 Gustave L. Levy Place, New York, NY 10029-6574, USA. 894
Discussion Wallis et al (1964) proposed a classification for diffuse skeletal haemangiomatosis involving multiple sites. They reviewed 26 cases including two of their own. They classified these 26 cases into two groups. Group 1, without visceral involvement, included nine cases, and Group 2, with visceral involvement, included 17 cases. They then subdivided Group 2 into (a) histologically benign (13 out of 17 cases) and (b) histologically malignant (four out of 17 cases). Radiographic examination of these cases revealed a combination of lytic and sclerotic lesions, multiple expanding cysts and generalized osseous demineralization with vertebral collapse. Lytic lesions were present in all cases and sclerotic lesions in only 50%, while expansion of bone was seen in one-third of Group 1 and half of Group 2 cases. Increasing sclerosis in some areas, with lysis in others, often occurred in the same patient over a period of years. They concluded that progressive sclerosis predominated in older patients and in those followed over a long period of time. However, neither they nor others could explain the propensity for vascular tumours to cause bone destruction in some cases and bone production in others. They and other authors noticed that diffuse haemangiomatosis limited to the skeleton appears to be a benign condition in spite of its widespread involvement (Waldron & Zeller, 1969; Schajowicz et al, 1978). The skeletal distribution of the lesions in their series varied but almost always involved the spine, ribs and pelvis. The skull, shoulders and long bones were frequently involved. The British Journal of Radiology, October 1991
Sclerosing haemangiomatosis
Figure 1. (a) Lateral view of the thoracic spine, (b) lateral view of the lumbar spine, and (c) frontal view of the pelvis showing dense, sharply denned sclerosis.
Waldron and Zeller (1969) followed a case of diffuse skeletal haemangiomatosis with visceral involvement for 16 years and noticed that the lesions which were lytic and sclerotic involving the ribs, spine and pelvis did not Vol. 64, No. 766
change. Spjut and Lindrom (1962) followed another case for 32 years, and found that some lesions stabilized while others regressed as the patient aged. The observation of the authors was supported by Schajowcz et al 895
/. F. Abdelwahab
lesions of the spine and pelvis. The later radiographs showed predominantly sclerotic lesions though small areas of osteolysis were noted. Our case is unusual in that it presented with purely sclerotic lesions without accompanying lytic lesions. There was no expansion of the involved bone. The skull and the bones distal to the elbows and knees were not involved. As in similar reports, the only visceral involvement besides the bone was the spleen (Ritchie & Zeier, 1956; Sammis et al, 1971; Dadash-Zadeh & Schwartz, 1976). In spite of many reports indicating either stabilization or regression of the lesions as the patient ages, we noticed in this case that not only had the sclerotic lesions markedly increased in size (some tripled in size in less than 10 years), but also that new sclerotic lesions had appeared (Fig. 3). Only a single other report of a case that presented with purely sclerotic lesions involving the right parietal bone and sacrum has been found (Raden & Mantz, 1955). Figure 2. Computed tomographic scan of the liver and spleen after contrast medium enhancement shows increased attenuation in the superior part of the enlarged spleen consistent with haemangioma. Note the multiple sclerotic densities in the cancellous and cortical bone of the lower thoracic vertebrae.
(1978). Two of three cases of skeletal haemangiomatosis followed by them showed spontaneous regression. Feldman (1978) mentioned that diffuse skeletal haemangiomatosis may become sclerotic following surgical procedures such as curettage, or even spontaneously, and that de novo sclerotic haemangiomatosis can occur. The case presented in her book was that of a middle-aged man who was followed for 26 years with visceral involvement and documented lytic and sclerotic
References CUTIERREZ, R. M. & SPJUT, H. J., 1972. Skeletal angiomatosis.
Report of three cases and review of the literature. Clinical Orthopaedics, 85, 82-96. DADASH-ZADEH, M. & SCHWARTZ, A. D., 1976. Skeletal and
splenic hemangiomatosis with consumption coagulopathy: response to splenectomy. Pediatrics, 57, 803-807. FELDMAN, F., 1978. Radiology /pathology and Immunology of Bones and Joints. A Review of Current Concepts (Appleton/ Century/Crofts, New York), pp. 7-22. RADEN, P. A. & MANTZ, JR, F. A., 1955. Benign systemic
hemangiomatosis without cutaneous involvement: confusing diagnostic problem. Radiology, 64, 252-257.
a
RITCHIE, G. & ZEIER, F. G., 1956. Hemangiomatosis of the
skeleton and the spleen. Journal of Bone & Joint Surgery, 38A, 115-121.
(a) (b) Figure 3. (a) Frontal radiograph of the pelvis showing fusion of the left sacroiliac joint and a big defect in the left iliac bone (both surgical), (b) Frontal radiograph of the pelvis taken 8 years later, which reveals that the sclerotic densities have increased in size. The lesion in the outer part of the right iliac bone, the site of the second biopsy, had tripled in size (arrows) and new sclerotic lesions had appeared in the pubic bones and proximal femora. The British Journal of Radiology, October 1991
Sclerosing haemangiomatosis SAMMIS JR, A. F., WEITZMAN, S. & ARCAMANO, J. P., 1971.
WALDRON, R. L. & ZELLER, J. A.,
1969. Diffuse
skeletal
Hemangiolymphangioma of spleen and bone. New York State Journal of Medicine, 14, 1762-1764.
hemangiomatosis with visceral involvement. Journal of the Canadian Association of Radiologists, 20, 119-123.
SCHAJOWICZ, F., AIELLO, C. L., FRANCONE, M. V. & GIANNINI,
WALLIS, L. A., ASCH, T. & MAISEL, B., 1964. Diffuse skeletal
R. E., 1978. Cystic angiomatosis (hamartous haemolyphangomatosis) of bone. A clinico-pathological study of three cases. Journal of Bone & Joint Surgery, 60B, 100-106. SPJUT, H. J. & LINDROM, A., 1962. Skeletal angiomatosis. Report of two cases. Ada Pathologica et Microbiologica Scandinavica, 55, 49-58.
hemangiomatosis: report of two cases and review of the literature. American Journal of Medicine, 37, 545-563. WILNER, D., 1981. Radiology of Bone Tumors and Allied Disorders (Saunders, Philadelphia), pp. 708-723.
Vol. 64, No. 766
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