Skeletal Radiol (1992) 21 : 474-477
Skeletal Radiology
Case report 755 Ronald A. Bloom, M.D. 1, Nili Peylan-Ramu, M.D. 2, and Elimelech Okon, M.D. 3 Departments of ~ Radiology, 2 Paediatric Oncology, 3 Pathology, Hadassah University Hospital, Jerusalem, Israel
Address reprint requests to: Dr. R.A. Bloom, Hadassah University Hospital, Ein Kerem, Jerusalem, Israel
9 1992 International Skeletal Society
Fig. 1. Radiograph of the left foot demonstrating an expanding lytic lesion in the talus with a faintly sclerotic margin Fig. 2. Histological section of a biopsy of the talus. A tumor of uniform, small, noncleaved lymphoid polygonal cells is present H & E
R.A. Bloom et al. : Case report 755
475
Cfinical information
Fig. 3. CT of the orbits. A soft-tissue mass in the lateral aspect of the right orbit is seen
A 5-year-old Arab boy presented to another hospital with a 6-month history of pain and swelling of the left foot. A radiograph of the foot demonstrated a lytic lesion in the talus with an apparant sclerotic margin (Fig. 1). A provisional diagnosis o f osteomyelitis was made and the child started empirically on a course of antibiotics. Because the child did not improve, an open biopsy of the lesion of the talus was performed. The biopsy material was interpreted as Ewing tumor of bone (Fig. 2). The
patient was transferred to the Hadassah University Hospital for further evaluation and treatment. The positive clinical findings at this time were a mild right periorbital swelling and a hard, tender, fixed mass on the dorsum of the right foot. The radiograph of the chest and the chest, abdominal, and pelvic computed tomography (CT) scans were normal. The blood count, blood chemical studies and bone marrow aspirate were all normal. Orbital CT showed a soft-tissue mass in the lower lateral aspect of the right orbit (Fig. 3).
476
R.A. Bloom et al. : Case report 755
Diagnosis: Burkitt's lymphoma of the talus A biopsy of the orbital soft-tissue mass was performed. Histological sections of the tumor using H & E staining showed a high grade malignant lymphoma of the small, non cleaved cell type. The findings were considered consistent with a Burkitt's type of lymphoma (Fig. 4). Immunoperoxidase staining with monoclonal L26 antibody stain showed positive cytoplasmic staining of the lymphoid cells. Macrophages with "tingible bodies" showed no staining with this dye. Reexamination of slides from the bone biopsy of the lesion in the talus showed the same malignant lymphoma. Restaining by immunoperoxidase with the L26 antibody showed a similar staining of the lymphoid cells. The child was treated with chemotherapeutic agents including vincristine, cyclophosphamide, Adriamycin, prednisone, ara-C, and hydrocortisone. The patient developed a central nervous system relapse some months later. An Ommaya reservoir was placed in the lateral ventricle of the brain, and methotrexate was delivered via that route. Craniospinal radiotherapy was later administered. The child responded well and is currently on maintenance chemotherapy.
Discussion Burkitt described the disease that now bears his name as a sarcoma involving the jaws of African children [2]. It is a clinically and histologically distinct neoplasm arising from B lymphocytes. The disorder has a rapid doubling time but responds dramatically to combination chemotherapy [I 1]. Although endemic in tropical Africa, it is now recognised worldwide. The occurrence of Burkitt's lymphoma in endomic locations and its association with Epstein-Barr virus has evoked much interest in a viral aetiology. Clinical findings in patients with this disorder from nonendemic areas of Africa and in other parts of the world differ from those in endemic areas. Thus, the predilection for tumors of the jaw
Fig. 4. Histological section of the orbital biopsy shows a high-grade malignant lymphoma of diffuse, small-cell type with a prominent "starry sky" appearance. Note the jigsaw puzzle arrangement of the lymphoid cells characteristics of Burkitt's lymphoma. H & E
in patients in endemic areas is not a common finding in nonendemic areas, where there is a higher incidence of abdominal disease and peripheral glandular involvement [5]. In the endemic areas of Africa, over 50% of children with Burkitt's disease present with a bony tumour arising from the mandible, maxilla, or both. Involvement of other skeletal sites is much less common. In a series of 106 children with Burkitt's tumor from East Africa, only 3 presented with a peripheral bone tumour, all involving the femur. In a further 5 cases, subsidiary lesions were also noted in the femur. Deposits in other bones were uncommonly demonstrated but included involvement of the tibia, frontal bone, and pelvis [3]. In a series of 110 patients from Ghana, only 2 patients had peripheral bone tumors, whose individual sites were unspecified [8]. A higher percentage of peripheral bone involvement was recorded in a series reported from Nigeria. Six of 51 cases had peripheral bone involvement, but the bony sites were not specified [9]. The most comprehensive study of peripheral bone involvement in the endemic areas of Africa is by Fowles et al. [7]. Of 290 Ugandan children and adolescents with primary Burkitt's lymphoma, 11
[4%] had lesions in the long bones or pelvis. The bones involved were the pubis, ilium, humerus, femur, tibia, and ulna. Burkitt's lymphoma in nonendemic areas is rare, and few series have been reported. In these nonendemic cases, jaw tumors are much less common, most cases presenting with abdominal tumors, pleural effusions, or peripheral lymph node involvement. Among the largest of such series was that of 40 American patients reported by Dunnick et al. [6]. Four cases had bone involvement. Two of these had mandibular involvement as well as other bony tumors. The other bones involved in these four cases were cranial vault, humerus, tibia, and ribs. No evidence of bone involvement was found in a further series of 12 American cases [1]. This must be contrasted with the 20 American patients reported by Cohen et al. [5], of which 7 had an involvement of the bones of the jaw and one of whom also had a tibial lesion. Another patient had a vertebral lesion. The pattern of skeletal involvement in tumors of the jaw bones and long bones is very similar. The disease is essentially intramedullary and multifocal, and the deposits generally present as small, multiple, osteolytic
R.A. Bloom et al. : Case report 755 lesions affecting the medullary cavity and tending to be streaky in the shaft and permeating or having multiple, small, radiolucent lesions that are poorly defined or punched out in the metaphyses. The margins of the lesions are ill-defined, with no sclerotic reaction around them. In the later stages of the lesion, the affected bone may become enlarged and expanded. Cortical erosion with subsequent breaching of the cortex may occur. In such cases, a classical " o n i o n skin" or " s u n b u r s t " periosteal reaction may be seen [7, 10]. In summary the appearances in the present case were distinctly unusual in that a well-defined lytic lesion with a faint sclerotic margin was present in the talus (Fig. 1). This may have occurred because the lesion developed in the cancellous bone of the talus. Periorbital development fol-
477 lowed. No previous description of the involvement of a bone in the hand or foot has been found in the literature. However, Cockshott mentioned that tarsal and carpal foci may be seen late in the course of the disease [4].
References 1. Alford BA, Coccia PF, L'Heureux PP (1977) Roentgenographic features of American Burkitt's lymphoma. Radiology 124:763 2. Burkitt D (1958) A sarcoma involving the jaws in African children. Br J Surg 46:218 3. Burkitt D, O'Conor GT (1961) Malignant lymphoma in African children. A clinical syndrome. Cancer 14:258 4. Cockshott PW (1965) Radiological aspects of Burkitt's lymphoma. Br J Radiol 38 : 172
5. Cohen MH, Bennett JM, Berard CV, Zeigler JL, Vogel CL, Sheagren JN, Carbone PP (1969) Burkitt's tumor in the United States, Cancer 23 : 1259 6. Dunnick NR, Reaman GH, Head GL, Shawker TH, Zeigler JL (1979) Radiographic manifestations of Burkitt's lymphoma in American patients. AJR 132:1 7. Fowles JV, Olweny CLM, KatongoleMbidde E, Lakanga-Ndawular A, Ower R (1983) Burkitt's lymphoma in the appendicular skeleton. J Bone Joint Surg [Br] 65:464 8. Nkrumah FK, Perkins IV (t976) Burkitt's lymphoma: a clinical study of 110 patients. Cancer 37: 671 9. Nzeh DA (1987) Importance of the jaw radiograph in diagnosis of Burkitt's lymphoma. Clin Radiol 38:519 10. Whittaker LR (1969) The radiological appearance of Burkitt's tumour involving bone. Australas Radiol 13:307 11. Ziegler JL (1977) Treatment results of 54 American patients with Burkitt's lymphoma as similar to the American experience, N Engl J Med 297:75
Skeletal Radiology
Case report 755 Ronald A. Bloom, M.D. 1, Nili Peylan-Ramu, M.D. 2, and Elimelech Okon, M.D. 3 Departments of ~ Radiology, 2 Paediatric Oncology, 3 Pathology, Hadassah University Hospital, Jerusalem, Israel
Address reprint requests to: Dr. R.A. Bloom, Hadassah University Hospital, Ein Kerem, Jerusalem, Israel
9 1992 International Skeletal Society
Fig. 1. Radiograph of the left foot demonstrating an expanding lytic lesion in the talus with a faintly sclerotic margin Fig. 2. Histological section of a biopsy of the talus. A tumor of uniform, small, noncleaved lymphoid polygonal cells is present H & E
R.A. Bloom et al. : Case report 755
475
Cfinical information
Fig. 3. CT of the orbits. A soft-tissue mass in the lateral aspect of the right orbit is seen
A 5-year-old Arab boy presented to another hospital with a 6-month history of pain and swelling of the left foot. A radiograph of the foot demonstrated a lytic lesion in the talus with an apparant sclerotic margin (Fig. 1). A provisional diagnosis o f osteomyelitis was made and the child started empirically on a course of antibiotics. Because the child did not improve, an open biopsy of the lesion of the talus was performed. The biopsy material was interpreted as Ewing tumor of bone (Fig. 2). The
patient was transferred to the Hadassah University Hospital for further evaluation and treatment. The positive clinical findings at this time were a mild right periorbital swelling and a hard, tender, fixed mass on the dorsum of the right foot. The radiograph of the chest and the chest, abdominal, and pelvic computed tomography (CT) scans were normal. The blood count, blood chemical studies and bone marrow aspirate were all normal. Orbital CT showed a soft-tissue mass in the lower lateral aspect of the right orbit (Fig. 3).
476
R.A. Bloom et al. : Case report 755
Diagnosis: Burkitt's lymphoma of the talus A biopsy of the orbital soft-tissue mass was performed. Histological sections of the tumor using H & E staining showed a high grade malignant lymphoma of the small, non cleaved cell type. The findings were considered consistent with a Burkitt's type of lymphoma (Fig. 4). Immunoperoxidase staining with monoclonal L26 antibody stain showed positive cytoplasmic staining of the lymphoid cells. Macrophages with "tingible bodies" showed no staining with this dye. Reexamination of slides from the bone biopsy of the lesion in the talus showed the same malignant lymphoma. Restaining by immunoperoxidase with the L26 antibody showed a similar staining of the lymphoid cells. The child was treated with chemotherapeutic agents including vincristine, cyclophosphamide, Adriamycin, prednisone, ara-C, and hydrocortisone. The patient developed a central nervous system relapse some months later. An Ommaya reservoir was placed in the lateral ventricle of the brain, and methotrexate was delivered via that route. Craniospinal radiotherapy was later administered. The child responded well and is currently on maintenance chemotherapy.
Discussion Burkitt described the disease that now bears his name as a sarcoma involving the jaws of African children [2]. It is a clinically and histologically distinct neoplasm arising from B lymphocytes. The disorder has a rapid doubling time but responds dramatically to combination chemotherapy [I 1]. Although endemic in tropical Africa, it is now recognised worldwide. The occurrence of Burkitt's lymphoma in endomic locations and its association with Epstein-Barr virus has evoked much interest in a viral aetiology. Clinical findings in patients with this disorder from nonendemic areas of Africa and in other parts of the world differ from those in endemic areas. Thus, the predilection for tumors of the jaw
Fig. 4. Histological section of the orbital biopsy shows a high-grade malignant lymphoma of diffuse, small-cell type with a prominent "starry sky" appearance. Note the jigsaw puzzle arrangement of the lymphoid cells characteristics of Burkitt's lymphoma. H & E
in patients in endemic areas is not a common finding in nonendemic areas, where there is a higher incidence of abdominal disease and peripheral glandular involvement [5]. In the endemic areas of Africa, over 50% of children with Burkitt's disease present with a bony tumour arising from the mandible, maxilla, or both. Involvement of other skeletal sites is much less common. In a series of 106 children with Burkitt's tumor from East Africa, only 3 presented with a peripheral bone tumour, all involving the femur. In a further 5 cases, subsidiary lesions were also noted in the femur. Deposits in other bones were uncommonly demonstrated but included involvement of the tibia, frontal bone, and pelvis [3]. In a series of 110 patients from Ghana, only 2 patients had peripheral bone tumors, whose individual sites were unspecified [8]. A higher percentage of peripheral bone involvement was recorded in a series reported from Nigeria. Six of 51 cases had peripheral bone involvement, but the bony sites were not specified [9]. The most comprehensive study of peripheral bone involvement in the endemic areas of Africa is by Fowles et al. [7]. Of 290 Ugandan children and adolescents with primary Burkitt's lymphoma, 11
[4%] had lesions in the long bones or pelvis. The bones involved were the pubis, ilium, humerus, femur, tibia, and ulna. Burkitt's lymphoma in nonendemic areas is rare, and few series have been reported. In these nonendemic cases, jaw tumors are much less common, most cases presenting with abdominal tumors, pleural effusions, or peripheral lymph node involvement. Among the largest of such series was that of 40 American patients reported by Dunnick et al. [6]. Four cases had bone involvement. Two of these had mandibular involvement as well as other bony tumors. The other bones involved in these four cases were cranial vault, humerus, tibia, and ribs. No evidence of bone involvement was found in a further series of 12 American cases [1]. This must be contrasted with the 20 American patients reported by Cohen et al. [5], of which 7 had an involvement of the bones of the jaw and one of whom also had a tibial lesion. Another patient had a vertebral lesion. The pattern of skeletal involvement in tumors of the jaw bones and long bones is very similar. The disease is essentially intramedullary and multifocal, and the deposits generally present as small, multiple, osteolytic
R.A. Bloom et al. : Case report 755 lesions affecting the medullary cavity and tending to be streaky in the shaft and permeating or having multiple, small, radiolucent lesions that are poorly defined or punched out in the metaphyses. The margins of the lesions are ill-defined, with no sclerotic reaction around them. In the later stages of the lesion, the affected bone may become enlarged and expanded. Cortical erosion with subsequent breaching of the cortex may occur. In such cases, a classical " o n i o n skin" or " s u n b u r s t " periosteal reaction may be seen [7, 10]. In summary the appearances in the present case were distinctly unusual in that a well-defined lytic lesion with a faint sclerotic margin was present in the talus (Fig. 1). This may have occurred because the lesion developed in the cancellous bone of the talus. Periorbital development fol-
477 lowed. No previous description of the involvement of a bone in the hand or foot has been found in the literature. However, Cockshott mentioned that tarsal and carpal foci may be seen late in the course of the disease [4].
References 1. Alford BA, Coccia PF, L'Heureux PP (1977) Roentgenographic features of American Burkitt's lymphoma. Radiology 124:763 2. Burkitt D (1958) A sarcoma involving the jaws in African children. Br J Surg 46:218 3. Burkitt D, O'Conor GT (1961) Malignant lymphoma in African children. A clinical syndrome. Cancer 14:258 4. Cockshott PW (1965) Radiological aspects of Burkitt's lymphoma. Br J Radiol 38 : 172
5. Cohen MH, Bennett JM, Berard CV, Zeigler JL, Vogel CL, Sheagren JN, Carbone PP (1969) Burkitt's tumor in the United States, Cancer 23 : 1259 6. Dunnick NR, Reaman GH, Head GL, Shawker TH, Zeigler JL (1979) Radiographic manifestations of Burkitt's lymphoma in American patients. AJR 132:1 7. Fowles JV, Olweny CLM, KatongoleMbidde E, Lakanga-Ndawular A, Ower R (1983) Burkitt's lymphoma in the appendicular skeleton. J Bone Joint Surg [Br] 65:464 8. Nkrumah FK, Perkins IV (t976) Burkitt's lymphoma: a clinical study of 110 patients. Cancer 37: 671 9. Nzeh DA (1987) Importance of the jaw radiograph in diagnosis of Burkitt's lymphoma. Clin Radiol 38:519 10. Whittaker LR (1969) The radiological appearance of Burkitt's tumour involving bone. Australas Radiol 13:307 11. Ziegler JL (1977) Treatment results of 54 American patients with Burkitt's lymphoma as similar to the American experience, N Engl J Med 297:75
