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The radiological appearances of bone lymphoma in AIDS
ClinicalRadiology (1992)
45, 169 171
The Radiological Appearances of Bone Lymphoma in AIDS A. K. BANERJEE,
T. MARSHALL*
a n d B. G . G A Z Z A R D *
Department of Radiology and Medicine, and *AIDS Unit, Westminster Hospital, London We report the radiologieal features of two cases of biopsy proven intraosseous involvement of non-Hodgkin's lymphoma in AIDS patients. B a n e r j e e , A . K . , M a r s h a l l , T . & G a z z a r d ~ B . G . ( 1 9 9 2 ) . Clinical in AIDS
Radiology
45, 169-171_ The Radiological
Over the last decade a markedly increased incidence of non-Hodgkin's lymphoma (NHL) in patients with AIDS has been noted. Although the majority of these patients have extranodal involvement oflymphoma, there are few reports of intraosseous involvement. We report radiological features of two cases of biopsy proven intraosseous involvement of non-Hodgkin's lymphoma_
Ji
'
Fig. 1 - Bone scintigraphy showing increased tracer uptake in the left shoulder and frontal bone.
CASE
Appearances
of Bone Lymphoma
REPORTS
Case 1, A 42-year-old HIV positive m a n presented with a 1 m o n t h history of back pain and bilateral hip and knee discomfort. He had night sweats and had lost 12.5 kg in weight over 3 months. An episode of right knee arthralgia had occurred 6 m o n t h s previously. He was febrile. There was no generalized lymphadenopathy but there were two non-tender soft tissue swellings 3 cm in diameter over his forehead. A small effusion in the right knee joint was aspirated and grew a Pseudomonas species which was treated with Ciprafloxacin. After an initial response the fever returned and bone scintigraphy revealed widespread increased tracer uptake over the frontal bone, the left shoulder, the right femoral head and ankle joints (Fig. 1). Reduced uptake was seen over the fifth lumbar vertebra. Radiographic examination showed lytic areas in the right femoral neck (Fig. 2) and erosive changes on the anterior surface of the fifth lumbar vertebra• A computed tomographic (CT) scan confirmed the erosive changes in the anterior surface of L5 with bony destruction and an adjacent soft tissue mass (Fig. 3). Biopsy of one of the forehead masses revealed a high-grade nonHodgkin's B cell lymphoma. The bone marrow was also involved. The patient was treated with chemotherapy and radiotherapy to the spine and is currently pain free. Case 2. A 65-year-old m a n presented initially with a pain in the left knee following a fall. A pathological fracture was noted in the distal end of the left femur. Bone biopsy revealed no evidence of infection or t u m o u r and a diagnosis of ischaemic necrosis was made (Fig. 4). Three months later he again presented with bone pain over the left knee. General examination was unremarkable. Blood chemistry revealed a marked hypercalcaemia (4.5 mmol/litre). An HIV antibody test was positive• CT scans of the head, thorax and abdomen, and bone marrow aspirate were normal. Bone scintigraphy showed increased tracer uptake over the distal left femur and a C T scan showed diffuse destruction of medullary and cortical bone with breaching of the cortex at several sites and an extensive soft tissue mass (Fig. 5). A pathological fracture was present with the distal fragment being displaced posteriorly and medially. Diffuse swelling of the surrounding soft tissues was noted with obliteration of the soft tissue plain. In addition, low attenuation
Fig. 2 - Pelvic radiograph showing multiple lytic lesions in the right femoral neck. Correspondence to: Dr A. K. Banerjee, 29B Blenheim Gardens, Willesden Green, London NW2.
Fig. 3 CT scan showing permeative changes on the anterolateral surface of the vertebral body and a soft tissue mass.
170
CLINICAL RADIOLOGY areas in the soft tissue were seen, thought to represent liquifying haematoma or tumour necrosis. Biopsy revealed an immunoblastic high grade, non-Hodgkin's lymphoma. The patient was treated with local radiotherapy and a course of chemotherapy but deteriorated and died within 6 weeks of presentation.
DISCUSSION
Fig. 4 Plain radiograph of lower femur showing a large permeative lytic lesion with ill-defined zone of transition with normal bone.
Fig. 5 CT scan of lower end of femur showing destruction of cortical and medullary bone with a soft tissue mass. A pathological fracture with displacement of the distal fragment is present.
The first r e p o r t s o f n o n - H o d g k i n ' s l y m p h o m a ( N H L ) in patients with A I D S a p p e a r e d in 1982 (Ziegler et al., 1982) a n d b y 1990 over 400 cases h a d been r e p o r t e d ( H a s k a l et al., 1990). A I D S - r e l a t e d N H L differs from N H L in the general p o p u l a t i o n as the m a j o r i t y are highgrade B cell types consisting o f small n o n - c l e a v e d o r large i m m u n o b l a s t i c types ( K a p l a n et al., 1989). T h e l y m p h o mas usually present late and have a p o o r prognosis. The m a j o r i t y have e x t r a n o d a l involvement, the c o m m o n sites including the bowel, central nervous system, skin, rectum, liver a n d testes. In a d d i t i o n , the b o n e m a r r o w is t h o u g h t to be involved in a third o f cases ( H a s k a l et al., 1990). H o w e v e r , there are no reports o f the r a d i o l o g i c a l a p p e a r ances o f N H L in b o n e in H I V seropositive patients. P r i m a r y N H L o f b o n e is rare in the general p o p u l a t i o n . Only 1% o f cases o f N H L present with single bone i n v o l v e m e n t which w o u l d be considered a p r i m a r y lymp h o m a o f b o n e a c c o r d i n g to the C o o l e y criteria (Cooley et al., 1950). A n o t h e r 5% o f cases have b o n e involvement in a d d i t i o n to visceral or n o d e involvement. Bone involvement in systemic N H L a n d Hodgkin~s disease in which up to 20% m a y have b o n e involvement (Braunstein, 1980) m a y be r a d i o l o g i c a l l y indistinguishable f r o m o t h e r b o n e t u m o u r s . The lesions in N H L are p r e d o m i n a n t l y osteolytic with a p e r m e a t i v e pattern. H o w e v e r , in 43% o f cases they m a y be sclerotic a n d in 16% o f cases they m a y be m i x e d ( N g a n and Preston, 1975). A long, indistinct t r a n s i t i o n zone m a y be present. A periosteal reaction m a y also be present a l t h o u g h this is m o r e c o m m o n in Ewing's t u r n o u t a n d H o d g k i n ' s disease ( R o d m a n et al., 1982)_ A soft tissue mass often a c c o m p a n i e s the b o n e lesion a n d this is best d e m o n s t r a t e d on C T o r M R I . P a t h o l o g i c a l fracture m a y be one o f the p r e s e n t i n g features in a d d i t i o n to pain, fever and weight loss. Strictly, only the second case t h a t we present fits into the c a t e g o r y o f p r i m a r y N H L o f bone_ The first case d e m o n s t r a t e d s i m u l t a n e o u s systemic a n d b o n e involvement. H o w e v e r , they b o t h exhibit certain features similar to N H L o f b o n e in the general p o p u l a t i o n including a p e r m e a t i v e osteolytic process, a n d an a d j a c e n t soft tissue mass. T h e lesion in Case 2 was aggressive with extensive cortical destruction, a p o o r p r o g n o s t i c factor ( L o d w i c k et al., 1980). O n r a d i o i s o t o p e b o n e scintigraphy, these sites o f l y m p h o m a d e m o n s t r a t e an increased r a d i o n u c l i d e uptake. The r e d u c e d u p t a k e seen over L5, a k n o w n site o f involvement in Case 1, is possibly due to greater osteoclastic activity at this site c o m p a r e d with others. Lytic lesions in the b o n e in patients with A I D S r e m a i n a rare presenting c o m p l i c a t i o n o f the disease. A recent study has described a new A I D S s y n d r o m e o f bacillary a n g i o m a t o s i s p r e s e n t i n g with osteolytic lesions in patients with A I D S ( B a r o n et al., 1990). M o s t o f the lytic lesions in the r e p o r t e d patients were in the distal extremity. The r a d i o l o g i c a l a p p e a r a n c e s r a n g e d f r o m a well defined lytic a r e a to ill-defined regions o f extensive cortical d e s t r u c t i o n a n d a m e d u l l a r y p e r m e a t i o n associated with an aggressive periosteal reaction. A d j a c e n t
RADIOLOGICAL APPEARANCES OF BONE LYMPHOMA 1N AIDS soft tissue m a s s e s m a y also be seen in t h e s e p a t i e n t s . I n c r e a s e d u p t a k e o n b o n e s c i n t i g r a p h y was also n o t e d c o r r e s p o n d i n g w i t h the a r e a s o f lyric d e s t r u c t i o n . A d i a g n o s i s o f the c o n d i t i o n c a n be m a d e f r o m the c u t a neous v a s c u l a r lesions seen in t h e p a t i e n t s w h i c h , a l t h o u g h a p p e a r i n g s i m i l a r to K a p o s i ' s s a r c o m a , c o n t a i n a b a c t e r i u m similar to o r i d e n t i c a l w i t h the r e c e n t l y identified c a t scratch disease bacillus. In a d d i t i o n , destructive lytic lesions m a y be seen in t h e e n d e m i c A f r i c a n type o f K a p o s i ' s s a r c o m a (Bayley, 1988). T o this differential we a d d n o n - H o d g k i n ' s l y m p h o m a o f b o n e a n d r e c o m m e n d a b i o p s y to e n a b l e a d i a g n o s i s to be m a d e a n d appropriate treatment instituted. REFERENCES
Baron, AL, Steinbach, LS, Le Boit, PE, Mills, CM, Gee, JH & Berger, TG (1990). Osteolytic lesions and bacillary angiomatosis in HIV infection: radiologic differentiation from AIDS-related Kaposi's sarcoma. Radiology, 177:77 81. Bayley, AC (1988). Atypical aggressive Kaposi's sarcoma in Africa. In:
171
Gottlieb, GJ & Ackerman, B. eds. Kaposi's sarcoma: A Text and Atlas. pp. 151 170. Lea and Febiger, Philadelphia. Braunstein, EM (1980). Hodgkin disease of bone: radiographic correlation with histologic classification. Radiology, 159:291 304. Cooley, BL, Higinbotham, NL & Groesbeck, HP (1950). Primary reticulum cell sarcoma of bone: summary of 37 cases. Radiology, 55" 641 658. Haskal, ZJ, Lindan, CE & Goodman, PC (1990). Lymphoma in the Immunocompromised patient in imaging the lymphomas. Ed. Libshitz, HI. Radiological Clinics North America, 28: 885-901. Kaplan, LD, Abrams, DI, Feigal, E, McGrath, M, Kahn, J, Neville, Pet aL (1989). AIDS associated non-Hodgkin's lymphoma in San Francisco. Journal c~fAmerican Medical Association, 261" 719-724. Lodwick, GS, Wilson, AJ, Farrell, C, Virtana, P, Smeltzer, FM & Dittrich, F (1980). Estimating rate of growth in bone lesions: observer performance and error. Radiology, 134: 585-590. Ngan, H & Preston, BJ (1975). Non-Hodgkin's lymphoma presenting with osseous lesions, Clinical Radiology, 26" 351 356. Rodman, D, Raymond, AK & Phillips, WC (1982). Case report 201: Primary lymphoma of bone (PLB) left fibula. Skeletal Radiology, 8:235 237. Ziegler, JL, Miner, RC & Rosenbaum, ET (1982). Outbreak of Burkitt's like lymphoma in homosexual men. Lancet, ii, 631-633.
45, 169 171
The Radiological Appearances of Bone Lymphoma in AIDS A. K. BANERJEE,
T. MARSHALL*
a n d B. G . G A Z Z A R D *
Department of Radiology and Medicine, and *AIDS Unit, Westminster Hospital, London We report the radiologieal features of two cases of biopsy proven intraosseous involvement of non-Hodgkin's lymphoma in AIDS patients. B a n e r j e e , A . K . , M a r s h a l l , T . & G a z z a r d ~ B . G . ( 1 9 9 2 ) . Clinical in AIDS
Radiology
45, 169-171_ The Radiological
Over the last decade a markedly increased incidence of non-Hodgkin's lymphoma (NHL) in patients with AIDS has been noted. Although the majority of these patients have extranodal involvement oflymphoma, there are few reports of intraosseous involvement. We report radiological features of two cases of biopsy proven intraosseous involvement of non-Hodgkin's lymphoma_
Ji
'
Fig. 1 - Bone scintigraphy showing increased tracer uptake in the left shoulder and frontal bone.
CASE
Appearances
of Bone Lymphoma
REPORTS
Case 1, A 42-year-old HIV positive m a n presented with a 1 m o n t h history of back pain and bilateral hip and knee discomfort. He had night sweats and had lost 12.5 kg in weight over 3 months. An episode of right knee arthralgia had occurred 6 m o n t h s previously. He was febrile. There was no generalized lymphadenopathy but there were two non-tender soft tissue swellings 3 cm in diameter over his forehead. A small effusion in the right knee joint was aspirated and grew a Pseudomonas species which was treated with Ciprafloxacin. After an initial response the fever returned and bone scintigraphy revealed widespread increased tracer uptake over the frontal bone, the left shoulder, the right femoral head and ankle joints (Fig. 1). Reduced uptake was seen over the fifth lumbar vertebra. Radiographic examination showed lytic areas in the right femoral neck (Fig. 2) and erosive changes on the anterior surface of the fifth lumbar vertebra• A computed tomographic (CT) scan confirmed the erosive changes in the anterior surface of L5 with bony destruction and an adjacent soft tissue mass (Fig. 3). Biopsy of one of the forehead masses revealed a high-grade nonHodgkin's B cell lymphoma. The bone marrow was also involved. The patient was treated with chemotherapy and radiotherapy to the spine and is currently pain free. Case 2. A 65-year-old m a n presented initially with a pain in the left knee following a fall. A pathological fracture was noted in the distal end of the left femur. Bone biopsy revealed no evidence of infection or t u m o u r and a diagnosis of ischaemic necrosis was made (Fig. 4). Three months later he again presented with bone pain over the left knee. General examination was unremarkable. Blood chemistry revealed a marked hypercalcaemia (4.5 mmol/litre). An HIV antibody test was positive• CT scans of the head, thorax and abdomen, and bone marrow aspirate were normal. Bone scintigraphy showed increased tracer uptake over the distal left femur and a C T scan showed diffuse destruction of medullary and cortical bone with breaching of the cortex at several sites and an extensive soft tissue mass (Fig. 5). A pathological fracture was present with the distal fragment being displaced posteriorly and medially. Diffuse swelling of the surrounding soft tissues was noted with obliteration of the soft tissue plain. In addition, low attenuation
Fig. 2 - Pelvic radiograph showing multiple lytic lesions in the right femoral neck. Correspondence to: Dr A. K. Banerjee, 29B Blenheim Gardens, Willesden Green, London NW2.
Fig. 3 CT scan showing permeative changes on the anterolateral surface of the vertebral body and a soft tissue mass.
170
CLINICAL RADIOLOGY areas in the soft tissue were seen, thought to represent liquifying haematoma or tumour necrosis. Biopsy revealed an immunoblastic high grade, non-Hodgkin's lymphoma. The patient was treated with local radiotherapy and a course of chemotherapy but deteriorated and died within 6 weeks of presentation.
DISCUSSION
Fig. 4 Plain radiograph of lower femur showing a large permeative lytic lesion with ill-defined zone of transition with normal bone.
Fig. 5 CT scan of lower end of femur showing destruction of cortical and medullary bone with a soft tissue mass. A pathological fracture with displacement of the distal fragment is present.
The first r e p o r t s o f n o n - H o d g k i n ' s l y m p h o m a ( N H L ) in patients with A I D S a p p e a r e d in 1982 (Ziegler et al., 1982) a n d b y 1990 over 400 cases h a d been r e p o r t e d ( H a s k a l et al., 1990). A I D S - r e l a t e d N H L differs from N H L in the general p o p u l a t i o n as the m a j o r i t y are highgrade B cell types consisting o f small n o n - c l e a v e d o r large i m m u n o b l a s t i c types ( K a p l a n et al., 1989). T h e l y m p h o mas usually present late and have a p o o r prognosis. The m a j o r i t y have e x t r a n o d a l involvement, the c o m m o n sites including the bowel, central nervous system, skin, rectum, liver a n d testes. In a d d i t i o n , the b o n e m a r r o w is t h o u g h t to be involved in a third o f cases ( H a s k a l et al., 1990). H o w e v e r , there are no reports o f the r a d i o l o g i c a l a p p e a r ances o f N H L in b o n e in H I V seropositive patients. P r i m a r y N H L o f b o n e is rare in the general p o p u l a t i o n . Only 1% o f cases o f N H L present with single bone i n v o l v e m e n t which w o u l d be considered a p r i m a r y lymp h o m a o f b o n e a c c o r d i n g to the C o o l e y criteria (Cooley et al., 1950). A n o t h e r 5% o f cases have b o n e involvement in a d d i t i o n to visceral or n o d e involvement. Bone involvement in systemic N H L a n d Hodgkin~s disease in which up to 20% m a y have b o n e involvement (Braunstein, 1980) m a y be r a d i o l o g i c a l l y indistinguishable f r o m o t h e r b o n e t u m o u r s . The lesions in N H L are p r e d o m i n a n t l y osteolytic with a p e r m e a t i v e pattern. H o w e v e r , in 43% o f cases they m a y be sclerotic a n d in 16% o f cases they m a y be m i x e d ( N g a n and Preston, 1975). A long, indistinct t r a n s i t i o n zone m a y be present. A periosteal reaction m a y also be present a l t h o u g h this is m o r e c o m m o n in Ewing's t u r n o u t a n d H o d g k i n ' s disease ( R o d m a n et al., 1982)_ A soft tissue mass often a c c o m p a n i e s the b o n e lesion a n d this is best d e m o n s t r a t e d on C T o r M R I . P a t h o l o g i c a l fracture m a y be one o f the p r e s e n t i n g features in a d d i t i o n to pain, fever and weight loss. Strictly, only the second case t h a t we present fits into the c a t e g o r y o f p r i m a r y N H L o f bone_ The first case d e m o n s t r a t e d s i m u l t a n e o u s systemic a n d b o n e involvement. H o w e v e r , they b o t h exhibit certain features similar to N H L o f b o n e in the general p o p u l a t i o n including a p e r m e a t i v e osteolytic process, a n d an a d j a c e n t soft tissue mass. T h e lesion in Case 2 was aggressive with extensive cortical destruction, a p o o r p r o g n o s t i c factor ( L o d w i c k et al., 1980). O n r a d i o i s o t o p e b o n e scintigraphy, these sites o f l y m p h o m a d e m o n s t r a t e an increased r a d i o n u c l i d e uptake. The r e d u c e d u p t a k e seen over L5, a k n o w n site o f involvement in Case 1, is possibly due to greater osteoclastic activity at this site c o m p a r e d with others. Lytic lesions in the b o n e in patients with A I D S r e m a i n a rare presenting c o m p l i c a t i o n o f the disease. A recent study has described a new A I D S s y n d r o m e o f bacillary a n g i o m a t o s i s p r e s e n t i n g with osteolytic lesions in patients with A I D S ( B a r o n et al., 1990). M o s t o f the lytic lesions in the r e p o r t e d patients were in the distal extremity. The r a d i o l o g i c a l a p p e a r a n c e s r a n g e d f r o m a well defined lytic a r e a to ill-defined regions o f extensive cortical d e s t r u c t i o n a n d a m e d u l l a r y p e r m e a t i o n associated with an aggressive periosteal reaction. A d j a c e n t
RADIOLOGICAL APPEARANCES OF BONE LYMPHOMA 1N AIDS soft tissue m a s s e s m a y also be seen in t h e s e p a t i e n t s . I n c r e a s e d u p t a k e o n b o n e s c i n t i g r a p h y was also n o t e d c o r r e s p o n d i n g w i t h the a r e a s o f lyric d e s t r u c t i o n . A d i a g n o s i s o f the c o n d i t i o n c a n be m a d e f r o m the c u t a neous v a s c u l a r lesions seen in t h e p a t i e n t s w h i c h , a l t h o u g h a p p e a r i n g s i m i l a r to K a p o s i ' s s a r c o m a , c o n t a i n a b a c t e r i u m similar to o r i d e n t i c a l w i t h the r e c e n t l y identified c a t scratch disease bacillus. In a d d i t i o n , destructive lytic lesions m a y be seen in t h e e n d e m i c A f r i c a n type o f K a p o s i ' s s a r c o m a (Bayley, 1988). T o this differential we a d d n o n - H o d g k i n ' s l y m p h o m a o f b o n e a n d r e c o m m e n d a b i o p s y to e n a b l e a d i a g n o s i s to be m a d e a n d appropriate treatment instituted. REFERENCES
Baron, AL, Steinbach, LS, Le Boit, PE, Mills, CM, Gee, JH & Berger, TG (1990). Osteolytic lesions and bacillary angiomatosis in HIV infection: radiologic differentiation from AIDS-related Kaposi's sarcoma. Radiology, 177:77 81. Bayley, AC (1988). Atypical aggressive Kaposi's sarcoma in Africa. In:
171
Gottlieb, GJ & Ackerman, B. eds. Kaposi's sarcoma: A Text and Atlas. pp. 151 170. Lea and Febiger, Philadelphia. Braunstein, EM (1980). Hodgkin disease of bone: radiographic correlation with histologic classification. Radiology, 159:291 304. Cooley, BL, Higinbotham, NL & Groesbeck, HP (1950). Primary reticulum cell sarcoma of bone: summary of 37 cases. Radiology, 55" 641 658. Haskal, ZJ, Lindan, CE & Goodman, PC (1990). Lymphoma in the Immunocompromised patient in imaging the lymphomas. Ed. Libshitz, HI. Radiological Clinics North America, 28: 885-901. Kaplan, LD, Abrams, DI, Feigal, E, McGrath, M, Kahn, J, Neville, Pet aL (1989). AIDS associated non-Hodgkin's lymphoma in San Francisco. Journal c~fAmerican Medical Association, 261" 719-724. Lodwick, GS, Wilson, AJ, Farrell, C, Virtana, P, Smeltzer, FM & Dittrich, F (1980). Estimating rate of growth in bone lesions: observer performance and error. Radiology, 134: 585-590. Ngan, H & Preston, BJ (1975). Non-Hodgkin's lymphoma presenting with osseous lesions, Clinical Radiology, 26" 351 356. Rodman, D, Raymond, AK & Phillips, WC (1982). Case report 201: Primary lymphoma of bone (PLB) left fibula. Skeletal Radiology, 8:235 237. Ziegler, JL, Miner, RC & Rosenbaum, ET (1982). Outbreak of Burkitt's like lymphoma in homosexual men. Lancet, ii, 631-633.