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Computed tomography and radiotherapy in giant hemangioma with thrombocytopenia
Computerized Radio/. Vol. 7, No. 5, pp. 319-322, in the U.S.A. All rights reserved
1983 Copyright
Printed
0
0730-4862/83 $3.00 + 0.00 1983 Pergamon Press Ltd
COMPUTED TOMOGRAPHY AND RADIOTHERAPY IN GIANT HEMANGIOMA WITH THROMBOCYTOPENIA KAMLA Medical
College
of Georgia, (Received
Department 18 January
of Radiation
case of giant hemangioma of the right infant is presented. The role of computed tomography in planning radiation therapy is discussed. Computed
Oncology,
1982; in reoisedform
Abstract-A
Hemangioma
SHAH Augusta.
25 October
GA 30912. U.S.A.
1982)
thigh and thrombocytopenia in a 5-month old male (CT) in assessing the extent of the lesion and its value
tomography
INTRODUCTION
Capillary and cavernous hemangioma in infants and children are usually present at birth or soon afterwards. Almost 90% regress and disappear over a period of 2 to 3 years and need no treatment. Irradiation is used for lesions that affect vital areas such as airways, gastrointestinal tract, or vertebral bodies and for lesions that are causing symptoms. Usually, only a low dose of radiation is necessary. It may be given with radium, yttrium, superficial X-rays, electron beam, or cobalt irradiation, depending on the site and size of the tumor. The risk of damage to normal structures, and the potential for a malignant tumor to develop years later secondary to the irradiation, make it important that only high-risk hemangioma be treated with radiation and that irradiation treatment be planned with great care and accuracy. Computerized tomography is extremely useful in assessing the extent of the disease and planning radiation ports so as to obviate or minimize radiation to vital structures, as illustrated in the following case. CASE
REPORT
The patient was admitted to the hospitals of The University of Texas Medical Branch at Galveston at the age of 7 weeks, with what appeared to be an infected hemangioma on the right thigh. At birth, he had been observed to have a swollen right thigh, with an area of bluish discoloration about 5 x 5 cm. He was examined by several physicians, including a pediatrician and a dermatologist. The parents were told that if the swelling did not resolve spontaneously, further evaluation would be necessary. The leg began to get more swollen and red 3 days before admission. He was brought to the emergency room with a temperature of lOO”F, but continued to eat well and not have any other problems. On examination, the thigh had an area of pronounced redness, induration and warmth, extending from the knee to about 2 cm below the inguinal area on the anterior aspect, with an area of bluish discoloration above the knee as shown in Figs 1 and 2. The circumference of the right thigh was 23.5 cm and multiple petechiae were evident. The knee was maintained at about 80” of flexion. Laboratory studies yielded the following findings: hemoglobin 9.5 gm, WBC 12,800. and platelet count 12,000. Roentgenograms of the right leg revealed soft tissue density with no evidence that the bone was involved. The initial diagnosis was of an infected, cavernous hemangioma, and disseminated intravascular coagulation. The infant was given antibiotics and steroids for 20 days with no improvement in the lesion. When the treatment regimen was changed to different antibiotics the lesion began to improve. After the erythema and swelling decreased, the patient was discharged. The patient was readmitted at the age of 5 months because the hemangioma was increasing in size and had been chronically infected. He also had persistent thrombocytopenia and increased clotting abnormalities, secondary to the consumption of blood factors by the hemangioma. The diameter of 319
320
KAMLA SHAH
Fig
1. Gross
appearance
of hemangioma
of right thigh.
the thigh had increased to 36 cm. He was referred for radiotherapy evaluation. A computed tomography (CT) scan (Fig. 3) was performed to determine the extent and depth of the disease and to aid in planning radiotherapy. CT scan revealed the extent of the hemangioma, the absence of bony involvement, and the presence of a large, ill-defined, soft tissue mass of the right thigh, located essentially lateral to the femur. The mass extended 2 cm above the greater trochanter at the top and above the knee at the lower margin. The patient was treated on the cobalt machine, and received 300 rad daily (given dose-d,,, at 0.5 cm) for three days to a total of 900 rad. The lesion was treated with a single anterior angled port, 17 x 12 cm in size (Fig. 4). The response of the lesion to treatment was good. The swelling regressed and the infection cleared. Laboratory data collected throughout his hospitalization revealed persistent thrombocytopenia, with platelet counts as low as 1000. Following radiation therapy, the platelet count improved gradually. Within 3 months of completion of treatment, the platelet count was normal, and it has remained normal since. In the follow-up clinic 2 years after completion of treatment, he has remained well and has continued to grow. The leg does show some fibrosis and a slight germ valgum deformity, but the patient now leads a full and active life (Fig. 5). The association of thrombocytopenia with hemangioma is rare [l]. The treatment of hemangiomas has been predominantly by either irradiation or surgical excision, but surgical excision has not been always possible. The problems of operating on small children with large vascular lesions in the presence of hemorrhagic tendencies have often prevented surgical treatment. Corticosteroids usually have little beneficial effect.
DISCUSSION Irradiation seems to be the safest and the best modality of treatment for giant hemangiomas, and if dosage and depth of treatment are adequate, it is likely to be quickly effective. Computed
Fig, 2. Comparison
of size between
hemangioma
and normal
leg.
Giant
hemangioma
with thrombocytopenia
(4
321
09
CC) Fig. 3. Hemangioma of the right thigh. (A) A computed tomogram through the pelvis and upper margin of the lesion demonstrates its size (thickness) and its independence of the adjacenl skeletal and muscular structures. (B) A more caudad tomogram through the proximal thigh (leg externally rotated) shows the lesion to be larger at this level and depicts its infiltration throughout the lateral compartment of the thigh. such that the muscle planes, seen so well in the normal contralateral thigh, are obliterated. (C) An image through the proximal leg and the lower extent of the lesion demonstrates involvement of the subcutaneous tissue. the muscles, and their intervening tissue planes (Fig. 3C). (Courtesy of C. J. Fagan. M.D.).
tomography, as shown in this patient, is exceptionally useful in planning radiation therapy. It helps in defining the extent of the disease. In this patient the swelling extended from the inguinal area to below the knee with a great deal of inflammation and infection. These findings made it difficult to assess the extent of the tumor clinically. The CT helped define the top and bottom extent of the lesion and we were able to avoid irradiating the hip and knee joints and so prevent contracture of these joints and also avoid radiation injury to the growing end of the bone. The CT also showed that most of the lesion was lateral to the femur and we were able to direct the beam and reduce the dose to the femur. The CT revealed that the lesion did not extend to the medial aspect of the thigh and by not irradiating the entire circumference of the thigh we were able to prevent soft tissue
Fig. 4. Isodose
distribution
of “‘Cobalt
to hemangioma
of leg using CT scan for planning
322
KAMLA
Fig. 5. Appearance
SHAH
of leg two years after treatment.
fibrosis and contracture. Also, CT revealed the lesion to be extremely bulky and defined the depth (10cm). This was useful in selecting the optimum energy for treatment. Superficial X-ray and electron beam would have been inadequate since only the superficial part of the lesion would have been treated. Selection of the cobalt beam permitted treatment to be more penetrating and to deliver a more homogeneous dose distribution. Since most hemangiomas occur in infants and children, computerized tomography is a valuable, nontraumatic procedure. Also, quite often, because of the vascular nature of the lesion, it is almost impossible to obtain a biopsy. CT helps plan radiotherapy and minimize radiation damage to normal structures.
SUMMARY A case of giant hemangioma of the right thigh and thrombocytopenia presented. The value of CT in assessing the extent of the disease and discussed.
in a 5-month old infant planning radiation ports
REFERENCES I. V. Duncan.
K. E. Halnan.
Giant
About the Author-KAMLA
Haemangioma
with thrombocytopenia.
Clin. Radial.
15, 224--231 (1964).
SHAH is currently Professor and Director of Radiation Oncology at the Medical College of Georgia, Augusta. Dr Shah received a M.D. degree from the Bombay University. She trained in Internal Medicine and subsequently in Radiation Oncology in London, England. Dr Shah is board certified in Radiation Oncology. She was Director of Residency Training Program at Queen’s University, Ontario (1973-77) and at University of Texas Medical Branch, Galveston (1978-82). Dr Shah is a member of numerous societies, including AMA, ACR, RSNA, ASTR and the Radium Society.
is is
1983 Copyright
Printed
0
0730-4862/83 $3.00 + 0.00 1983 Pergamon Press Ltd
COMPUTED TOMOGRAPHY AND RADIOTHERAPY IN GIANT HEMANGIOMA WITH THROMBOCYTOPENIA KAMLA Medical
College
of Georgia, (Received
Department 18 January
of Radiation
case of giant hemangioma of the right infant is presented. The role of computed tomography in planning radiation therapy is discussed. Computed
Oncology,
1982; in reoisedform
Abstract-A
Hemangioma
SHAH Augusta.
25 October
GA 30912. U.S.A.
1982)
thigh and thrombocytopenia in a 5-month old male (CT) in assessing the extent of the lesion and its value
tomography
INTRODUCTION
Capillary and cavernous hemangioma in infants and children are usually present at birth or soon afterwards. Almost 90% regress and disappear over a period of 2 to 3 years and need no treatment. Irradiation is used for lesions that affect vital areas such as airways, gastrointestinal tract, or vertebral bodies and for lesions that are causing symptoms. Usually, only a low dose of radiation is necessary. It may be given with radium, yttrium, superficial X-rays, electron beam, or cobalt irradiation, depending on the site and size of the tumor. The risk of damage to normal structures, and the potential for a malignant tumor to develop years later secondary to the irradiation, make it important that only high-risk hemangioma be treated with radiation and that irradiation treatment be planned with great care and accuracy. Computerized tomography is extremely useful in assessing the extent of the disease and planning radiation ports so as to obviate or minimize radiation to vital structures, as illustrated in the following case. CASE
REPORT
The patient was admitted to the hospitals of The University of Texas Medical Branch at Galveston at the age of 7 weeks, with what appeared to be an infected hemangioma on the right thigh. At birth, he had been observed to have a swollen right thigh, with an area of bluish discoloration about 5 x 5 cm. He was examined by several physicians, including a pediatrician and a dermatologist. The parents were told that if the swelling did not resolve spontaneously, further evaluation would be necessary. The leg began to get more swollen and red 3 days before admission. He was brought to the emergency room with a temperature of lOO”F, but continued to eat well and not have any other problems. On examination, the thigh had an area of pronounced redness, induration and warmth, extending from the knee to about 2 cm below the inguinal area on the anterior aspect, with an area of bluish discoloration above the knee as shown in Figs 1 and 2. The circumference of the right thigh was 23.5 cm and multiple petechiae were evident. The knee was maintained at about 80” of flexion. Laboratory studies yielded the following findings: hemoglobin 9.5 gm, WBC 12,800. and platelet count 12,000. Roentgenograms of the right leg revealed soft tissue density with no evidence that the bone was involved. The initial diagnosis was of an infected, cavernous hemangioma, and disseminated intravascular coagulation. The infant was given antibiotics and steroids for 20 days with no improvement in the lesion. When the treatment regimen was changed to different antibiotics the lesion began to improve. After the erythema and swelling decreased, the patient was discharged. The patient was readmitted at the age of 5 months because the hemangioma was increasing in size and had been chronically infected. He also had persistent thrombocytopenia and increased clotting abnormalities, secondary to the consumption of blood factors by the hemangioma. The diameter of 319
320
KAMLA SHAH
Fig
1. Gross
appearance
of hemangioma
of right thigh.
the thigh had increased to 36 cm. He was referred for radiotherapy evaluation. A computed tomography (CT) scan (Fig. 3) was performed to determine the extent and depth of the disease and to aid in planning radiotherapy. CT scan revealed the extent of the hemangioma, the absence of bony involvement, and the presence of a large, ill-defined, soft tissue mass of the right thigh, located essentially lateral to the femur. The mass extended 2 cm above the greater trochanter at the top and above the knee at the lower margin. The patient was treated on the cobalt machine, and received 300 rad daily (given dose-d,,, at 0.5 cm) for three days to a total of 900 rad. The lesion was treated with a single anterior angled port, 17 x 12 cm in size (Fig. 4). The response of the lesion to treatment was good. The swelling regressed and the infection cleared. Laboratory data collected throughout his hospitalization revealed persistent thrombocytopenia, with platelet counts as low as 1000. Following radiation therapy, the platelet count improved gradually. Within 3 months of completion of treatment, the platelet count was normal, and it has remained normal since. In the follow-up clinic 2 years after completion of treatment, he has remained well and has continued to grow. The leg does show some fibrosis and a slight germ valgum deformity, but the patient now leads a full and active life (Fig. 5). The association of thrombocytopenia with hemangioma is rare [l]. The treatment of hemangiomas has been predominantly by either irradiation or surgical excision, but surgical excision has not been always possible. The problems of operating on small children with large vascular lesions in the presence of hemorrhagic tendencies have often prevented surgical treatment. Corticosteroids usually have little beneficial effect.
DISCUSSION Irradiation seems to be the safest and the best modality of treatment for giant hemangiomas, and if dosage and depth of treatment are adequate, it is likely to be quickly effective. Computed
Fig, 2. Comparison
of size between
hemangioma
and normal
leg.
Giant
hemangioma
with thrombocytopenia
(4
321
09
CC) Fig. 3. Hemangioma of the right thigh. (A) A computed tomogram through the pelvis and upper margin of the lesion demonstrates its size (thickness) and its independence of the adjacenl skeletal and muscular structures. (B) A more caudad tomogram through the proximal thigh (leg externally rotated) shows the lesion to be larger at this level and depicts its infiltration throughout the lateral compartment of the thigh. such that the muscle planes, seen so well in the normal contralateral thigh, are obliterated. (C) An image through the proximal leg and the lower extent of the lesion demonstrates involvement of the subcutaneous tissue. the muscles, and their intervening tissue planes (Fig. 3C). (Courtesy of C. J. Fagan. M.D.).
tomography, as shown in this patient, is exceptionally useful in planning radiation therapy. It helps in defining the extent of the disease. In this patient the swelling extended from the inguinal area to below the knee with a great deal of inflammation and infection. These findings made it difficult to assess the extent of the tumor clinically. The CT helped define the top and bottom extent of the lesion and we were able to avoid irradiating the hip and knee joints and so prevent contracture of these joints and also avoid radiation injury to the growing end of the bone. The CT also showed that most of the lesion was lateral to the femur and we were able to direct the beam and reduce the dose to the femur. The CT revealed that the lesion did not extend to the medial aspect of the thigh and by not irradiating the entire circumference of the thigh we were able to prevent soft tissue
Fig. 4. Isodose
distribution
of “‘Cobalt
to hemangioma
of leg using CT scan for planning
322
KAMLA
Fig. 5. Appearance
SHAH
of leg two years after treatment.
fibrosis and contracture. Also, CT revealed the lesion to be extremely bulky and defined the depth (10cm). This was useful in selecting the optimum energy for treatment. Superficial X-ray and electron beam would have been inadequate since only the superficial part of the lesion would have been treated. Selection of the cobalt beam permitted treatment to be more penetrating and to deliver a more homogeneous dose distribution. Since most hemangiomas occur in infants and children, computerized tomography is a valuable, nontraumatic procedure. Also, quite often, because of the vascular nature of the lesion, it is almost impossible to obtain a biopsy. CT helps plan radiotherapy and minimize radiation damage to normal structures.
SUMMARY A case of giant hemangioma of the right thigh and thrombocytopenia presented. The value of CT in assessing the extent of the disease and discussed.
in a 5-month old infant planning radiation ports
REFERENCES I. V. Duncan.
K. E. Halnan.
Giant
About the Author-KAMLA
Haemangioma
with thrombocytopenia.
Clin. Radial.
15, 224--231 (1964).
SHAH is currently Professor and Director of Radiation Oncology at the Medical College of Georgia, Augusta. Dr Shah received a M.D. degree from the Bombay University. She trained in Internal Medicine and subsequently in Radiation Oncology in London, England. Dr Shah is board certified in Radiation Oncology. She was Director of Residency Training Program at Queen’s University, Ontario (1973-77) and at University of Texas Medical Branch, Galveston (1978-82). Dr Shah is a member of numerous societies, including AMA, ACR, RSNA, ASTR and the Radium Society.
is is