Clinical Radiology (1992) 45, 284-285
Case Report: Granulocytic Sarcoma (Chloroma) Presenting as a Cerebellopontine Angle Mass C. S. R O M A N I U K
D e p a r t m e n t o)c Radiology, St J a m e s ' s University Hospital, Leeds
Granulocytic sarcomas (chloromas) are rare, solid extramedullary tumours composed of granulocyte precursors occurring in association with granulocytic leukaemia. A cerebellopontine angle granulocytic sarcoma in a 4-year-old child presenting with unilateral facial palsy is reported. The computed tomographic (CT) findings are described, with a review of the literature, and the significance of early diagnosis is discussed. Romaniuk, C.S. (1992). Clinical Radiology 45, 284 285. Case Report: G r a n u l o c y t i c S a r c o m a ( C h l o r o m a ) Presenting as a Cerebellopontine Angle Mass
et al., 1973); increased incidence in m y e l o g e n o u s leukae-
CASE REPORT A 4-year-old girl presented with a 10 month history of recurrent cough, lethargy and ear infections. Right facial weakness had been noted for 2 months. The child was pale, with widespread bruising and a right lower motor neurone facial nerve palsy. Her haemoglobin was 4 gm/dl with a white cellcount of 24 x 109/litrewith peripheral blast cells and a platelet count of 24 × 109/litre. Computed tomography (CT) showed a hyperdense mass 2 cm in diameter in the right cerebellopontine angle cistern, arising from the internal auditory meatus and compressing the cerebellum. Marked enhancement of the mass occurred (Fig. 1). Exammation of the cerebrospinal fluid (CSF) and bone marrow demonstrated blast cells and a diagnosis of acute granulocytic leukaemia was made. Systemic and intrathecal chemotherapy resulted in clearance of blast cells from the CSF and resolution of the facial palsy after 3 weeks. A bone marrow transplant was performed and the patient remains in complete remission 13 months after diagnosis.
DISCUSSION G r a n u l o c y t i c sarcomas (chloromas) are rare, solid extramedullary t u m o u r s o f g r a n u l o c y t e precursors occurring in 3.1-6.8% (Muss a n d Moloney, 1973; Liu et al., 1973) of cases of granulocytic leukaemia. G r a n u l o c y t i c sarcoma was first described in the lacrimal gland by Burns (1811). The term c h l o r o m a was first coined by K i n g (1853), because the lesions often display a greenish colour, as a result of myeloperoxidase in the t u m o u r cells. Dock (! 893) recognized the association with leukaemia. G r a n u l o c y t i c sarcoma usually arises in patients with overt leukaemia, but m a y occur when the b o n e m a r r o w is in remission. It m a y rarely be the first m a n i f e s t a t i o n of disease (Krause, 1979): leukaemia is then usually manifest within 2-8 m o n t h s (Krause, 1979), b u t the delay can be as long as 26 m o n t h s (Seo et al_, 1977). The initially solitary lesion m a y be erroneously diagnosed as l y m p h o m a or eosinophilic g r a n u l o m a on histological e x a m i n a t i o n using r o u t i n e stains ( M c C a r t y et al., 1980; W i e r n i k and Serpick, 1970). The incidence of granulocytic s a r c o m a is similar in acute a n d chronic granulocytic leukaemia (Liu
Correspondence to: Dr C. S. Romaniuk, Department of Radiology, St. James's University Hospital, Leeds LS9 7TF.
mia is partly a t t r i b u t a b l e to longer d u r a t i o n of remissions (Muss a n d M o l o n e y , 1973). The d e v e l o p m e n t of granulocytic sarcoma in patients with l e u k a e m i a implies a p o o r prognosis, often heralding a blastic crisis ( N i e m a n et al., 1981). There appears to be no sex p r e d o m i n a n c e b u t 40% of cases are seen in patients u n d e r 15 years of age (Liu et al., 1973). G r a n u l o c y t i c sarcomas are most c o m m o n l y f o u n d in association with b o n e a n d nervous tissue, particularly the orbit a n d epidural space; the b r a i n is involved in up to 22% o f cases (Liu et al., 1973). The lesions can, however, occur at any site (Lee et al., 1991), are often multiple, a n d a p p r o x i m a t e l y half are a s y m p t o m a t i c (Liu et al., 1973). The m o s t c o m m o n presenting c o m p l a i n t s are local pain, a lump, paraplegia, paresis a n d e x o p h t h a l m o s (Liu et al., 1973). Central nervous system ieukaemia first appears in the walls o f superficial a r a c h n o i d veins, with s u b s e q u e n t diffuse i n v o l v e m e n t of the a r a c h n o i d mater. I n v a s i o n of neural tissue follows d i s r u p t i o n of the n o r m a l pial m e m b r a n e (Price a n d J o h n s o n , 1973). I n t r a c r a n i a l granulocytic s a r c o m a has a relatively consistent C T a p p e a r a n c e with u n i f o r m e n h a n c e m e n t of a mass which is iso- or hyperdense with respect to n o r m a l b r a i n before contrast m e d i u m ( W e n d l i n g et al_, 1979; Sowers et al., 1979). A d j a c e n t oedema m a y be present. A cerebellopontine angle granulocytic s a r c o m a could therfore be confused with a n acoustic n e u r o m a , a m e n i n g i o m a , or the m u c h rarer l y m p h o m a or metastasis, particularly if it antedates systemic leukaemia. C h l o r o m a t o u s masses m a y be cystic with peripheral e n h a n c e m e n t ( W a n g et al., 1987), a n d can mimic abscesses (Vinters a n d Gilbert, 1982). They can cause acute hydrocephalus ( F r u a f f et al., 1988), as they tend to occur in the posterior fossa. I n v a s i o n o f the b r a i n is u n u s u a l (Liu et al_, 1973; Sowers et al., 1979). A l t h o u g h it was a b n o r m a l in the present case, the C S F m a y be negative for blast cells, especially if the mass causes obstructive h y d r o c e p h a l u s ( W e n d l i n g et al., 1979). N o case o f cerebellopontine angle granulocytic sarc o m a w o u l d appear to have been described in association with granulocytic leukaemia. F a m i l i a r i t y with the C T appearances is i m p o r t a n t , as a childhood p r e s e n t a t i o n o f leukaemia a n d as a form of CNS relapse, which m a y mimic a c o m p l i c a t i o n of therapy, such as a n abscess or h a e m o r r h a g e a n d cause hydrocephalus.
GRANULOCYTIC SARCOMA
(a)
285
(e) Fig. 1 (a) Non-contrast CT showing a hyperdense mass in the right cerebellopontine angle cistern at the level of the internal auditory meatus. (b) Enhancement of the mass after intravenous contrast medium. (c) At the same level, shows the internal auditory meatus.
(b) REFERENCES
Burns, A (181 I). Observation of the Surgical Anatomy of the Head and Neck. pp. 364 366. Thomas Royce, Edinburgh. Dock, G (1893). Chloroma and its relationship to leukaemia. American Journal of Medical Science, 106, 152 157. Fruaff, AA, Barasch, ES & Rosenthal, A (1988). Solitary myeloblastoma presenting as acute hydrocephalus: review of literature, implications for therapy. Paediatric Radiology, 18, 369 372.
King, A (1853). A case of chloroma. Monthly Journal of the Medical Society (London and Edinburgh), 19, 97. Krause, JR (1979). Granulocytic leukemia preceding acute leukemia. A report of 6 cases. Cancer, 44, 1017 1021. Lee, M J, Grogan, L, Meehan, S & Breatnach, E (1991). Case report: pleural granulocytic sarcoma: CT characteristics. Clinical Radiology, 43, 57 59. Liu, PI, Ishimaru, T, McGregor, DH, Okada, H & Steer, A (1973). Autopsy study of granulocytic sarcoma (chloroma) in patients with myelogenous leukemia. Hiroshima-Nagasaki 1949 1969. Cancer, 31,948 955. McCarty, KS Jr, Wortman, J, Daly, J, Rundles, RW, Hanker, JS (1980). Chloroma (granulocytie sarcoma) without evidence of leukaemia: facilitated light microscopic diagnosis. Blood, 56, 104-108. Muss, HB & Moloney, WC (1973). Chloroma and other myeloblastic tumours. Blood, 42, 721 728. Nieman, RS, Barcos, M, Berard, C, Bonner, H, Mann, R, Rydell, RE et al. (1981). Granulocytic sarcoma: a clinicopathologic study of 61 biopsied cases. Cancer, 48, 1426 1437. Price, RA & Johnson, WW (1973). The central nervous system in childhood leukemia. 1. The arachnoid. Cancer, 31,520 533. Seo, IS, Hull, MT & Pak, HY (1977). Granulocytic sarcoma of cervix as a primary manifestation. Case without overt leukemic features for 26 months. Cancer, 40, 3030-3037. Sowers, JJ, Moody, DM, Naidich, TP, Ball, MR, Laster, DW & Leeds, NE (1979). Radiographic features of granulocytic sarcoma (chloroma). Computer Assisted Tomography, 3, 226 233. Vinters, HV & Gilbert, JJ (1982). Multifocal chloromas of the brain. Surgical Neurology, 17, 47-51. Wang, AM, Lin, JCT, Power, TC, Haykal, HA & Zamani, AA (1987). Chloroma of cerebellum, tentorium and occipital bone in acute myelogenous leukemia. Neuroradiology, 29, 590. Wendling, LR, Cromwell, LD & Latchaw, RE (1979). Computed tomography of intracerebral Ieukaemia masses. American Journal of Roentgenology, 132, 217 220. Wiernik, PH & Serpick, AA (1970). Granulocytic sarcoma (ehloroma). Blood, 35, 361-369.
Case Report: Granulocytic Sarcoma (Chloroma) Presenting as a Cerebellopontine Angle Mass C. S. R O M A N I U K
D e p a r t m e n t o)c Radiology, St J a m e s ' s University Hospital, Leeds
Granulocytic sarcomas (chloromas) are rare, solid extramedullary tumours composed of granulocyte precursors occurring in association with granulocytic leukaemia. A cerebellopontine angle granulocytic sarcoma in a 4-year-old child presenting with unilateral facial palsy is reported. The computed tomographic (CT) findings are described, with a review of the literature, and the significance of early diagnosis is discussed. Romaniuk, C.S. (1992). Clinical Radiology 45, 284 285. Case Report: G r a n u l o c y t i c S a r c o m a ( C h l o r o m a ) Presenting as a Cerebellopontine Angle Mass
et al., 1973); increased incidence in m y e l o g e n o u s leukae-
CASE REPORT A 4-year-old girl presented with a 10 month history of recurrent cough, lethargy and ear infections. Right facial weakness had been noted for 2 months. The child was pale, with widespread bruising and a right lower motor neurone facial nerve palsy. Her haemoglobin was 4 gm/dl with a white cellcount of 24 x 109/litrewith peripheral blast cells and a platelet count of 24 × 109/litre. Computed tomography (CT) showed a hyperdense mass 2 cm in diameter in the right cerebellopontine angle cistern, arising from the internal auditory meatus and compressing the cerebellum. Marked enhancement of the mass occurred (Fig. 1). Exammation of the cerebrospinal fluid (CSF) and bone marrow demonstrated blast cells and a diagnosis of acute granulocytic leukaemia was made. Systemic and intrathecal chemotherapy resulted in clearance of blast cells from the CSF and resolution of the facial palsy after 3 weeks. A bone marrow transplant was performed and the patient remains in complete remission 13 months after diagnosis.
DISCUSSION G r a n u l o c y t i c sarcomas (chloromas) are rare, solid extramedullary t u m o u r s o f g r a n u l o c y t e precursors occurring in 3.1-6.8% (Muss a n d Moloney, 1973; Liu et al., 1973) of cases of granulocytic leukaemia. G r a n u l o c y t i c sarcoma was first described in the lacrimal gland by Burns (1811). The term c h l o r o m a was first coined by K i n g (1853), because the lesions often display a greenish colour, as a result of myeloperoxidase in the t u m o u r cells. Dock (! 893) recognized the association with leukaemia. G r a n u l o c y t i c sarcoma usually arises in patients with overt leukaemia, but m a y occur when the b o n e m a r r o w is in remission. It m a y rarely be the first m a n i f e s t a t i o n of disease (Krause, 1979): leukaemia is then usually manifest within 2-8 m o n t h s (Krause, 1979), b u t the delay can be as long as 26 m o n t h s (Seo et al_, 1977). The initially solitary lesion m a y be erroneously diagnosed as l y m p h o m a or eosinophilic g r a n u l o m a on histological e x a m i n a t i o n using r o u t i n e stains ( M c C a r t y et al., 1980; W i e r n i k and Serpick, 1970). The incidence of granulocytic s a r c o m a is similar in acute a n d chronic granulocytic leukaemia (Liu
Correspondence to: Dr C. S. Romaniuk, Department of Radiology, St. James's University Hospital, Leeds LS9 7TF.
mia is partly a t t r i b u t a b l e to longer d u r a t i o n of remissions (Muss a n d M o l o n e y , 1973). The d e v e l o p m e n t of granulocytic sarcoma in patients with l e u k a e m i a implies a p o o r prognosis, often heralding a blastic crisis ( N i e m a n et al., 1981). There appears to be no sex p r e d o m i n a n c e b u t 40% of cases are seen in patients u n d e r 15 years of age (Liu et al., 1973). G r a n u l o c y t i c sarcomas are most c o m m o n l y f o u n d in association with b o n e a n d nervous tissue, particularly the orbit a n d epidural space; the b r a i n is involved in up to 22% o f cases (Liu et al., 1973). The lesions can, however, occur at any site (Lee et al., 1991), are often multiple, a n d a p p r o x i m a t e l y half are a s y m p t o m a t i c (Liu et al., 1973). The m o s t c o m m o n presenting c o m p l a i n t s are local pain, a lump, paraplegia, paresis a n d e x o p h t h a l m o s (Liu et al., 1973). Central nervous system ieukaemia first appears in the walls o f superficial a r a c h n o i d veins, with s u b s e q u e n t diffuse i n v o l v e m e n t of the a r a c h n o i d mater. I n v a s i o n of neural tissue follows d i s r u p t i o n of the n o r m a l pial m e m b r a n e (Price a n d J o h n s o n , 1973). I n t r a c r a n i a l granulocytic s a r c o m a has a relatively consistent C T a p p e a r a n c e with u n i f o r m e n h a n c e m e n t of a mass which is iso- or hyperdense with respect to n o r m a l b r a i n before contrast m e d i u m ( W e n d l i n g et al_, 1979; Sowers et al., 1979). A d j a c e n t oedema m a y be present. A cerebellopontine angle granulocytic s a r c o m a could therfore be confused with a n acoustic n e u r o m a , a m e n i n g i o m a , or the m u c h rarer l y m p h o m a or metastasis, particularly if it antedates systemic leukaemia. C h l o r o m a t o u s masses m a y be cystic with peripheral e n h a n c e m e n t ( W a n g et al., 1987), a n d can mimic abscesses (Vinters a n d Gilbert, 1982). They can cause acute hydrocephalus ( F r u a f f et al., 1988), as they tend to occur in the posterior fossa. I n v a s i o n o f the b r a i n is u n u s u a l (Liu et al_, 1973; Sowers et al., 1979). A l t h o u g h it was a b n o r m a l in the present case, the C S F m a y be negative for blast cells, especially if the mass causes obstructive h y d r o c e p h a l u s ( W e n d l i n g et al., 1979). N o case o f cerebellopontine angle granulocytic sarc o m a w o u l d appear to have been described in association with granulocytic leukaemia. F a m i l i a r i t y with the C T appearances is i m p o r t a n t , as a childhood p r e s e n t a t i o n o f leukaemia a n d as a form of CNS relapse, which m a y mimic a c o m p l i c a t i o n of therapy, such as a n abscess or h a e m o r r h a g e a n d cause hydrocephalus.
GRANULOCYTIC SARCOMA
(a)
285
(e) Fig. 1 (a) Non-contrast CT showing a hyperdense mass in the right cerebellopontine angle cistern at the level of the internal auditory meatus. (b) Enhancement of the mass after intravenous contrast medium. (c) At the same level, shows the internal auditory meatus.
(b) REFERENCES
Burns, A (181 I). Observation of the Surgical Anatomy of the Head and Neck. pp. 364 366. Thomas Royce, Edinburgh. Dock, G (1893). Chloroma and its relationship to leukaemia. American Journal of Medical Science, 106, 152 157. Fruaff, AA, Barasch, ES & Rosenthal, A (1988). Solitary myeloblastoma presenting as acute hydrocephalus: review of literature, implications for therapy. Paediatric Radiology, 18, 369 372.
King, A (1853). A case of chloroma. Monthly Journal of the Medical Society (London and Edinburgh), 19, 97. Krause, JR (1979). Granulocytic leukemia preceding acute leukemia. A report of 6 cases. Cancer, 44, 1017 1021. Lee, M J, Grogan, L, Meehan, S & Breatnach, E (1991). Case report: pleural granulocytic sarcoma: CT characteristics. Clinical Radiology, 43, 57 59. Liu, PI, Ishimaru, T, McGregor, DH, Okada, H & Steer, A (1973). Autopsy study of granulocytic sarcoma (chloroma) in patients with myelogenous leukemia. Hiroshima-Nagasaki 1949 1969. Cancer, 31,948 955. McCarty, KS Jr, Wortman, J, Daly, J, Rundles, RW, Hanker, JS (1980). Chloroma (granulocytie sarcoma) without evidence of leukaemia: facilitated light microscopic diagnosis. Blood, 56, 104-108. Muss, HB & Moloney, WC (1973). Chloroma and other myeloblastic tumours. Blood, 42, 721 728. Nieman, RS, Barcos, M, Berard, C, Bonner, H, Mann, R, Rydell, RE et al. (1981). Granulocytic sarcoma: a clinicopathologic study of 61 biopsied cases. Cancer, 48, 1426 1437. Price, RA & Johnson, WW (1973). The central nervous system in childhood leukemia. 1. The arachnoid. Cancer, 31,520 533. Seo, IS, Hull, MT & Pak, HY (1977). Granulocytic sarcoma of cervix as a primary manifestation. Case without overt leukemic features for 26 months. Cancer, 40, 3030-3037. Sowers, JJ, Moody, DM, Naidich, TP, Ball, MR, Laster, DW & Leeds, NE (1979). Radiographic features of granulocytic sarcoma (chloroma). Computer Assisted Tomography, 3, 226 233. Vinters, HV & Gilbert, JJ (1982). Multifocal chloromas of the brain. Surgical Neurology, 17, 47-51. Wang, AM, Lin, JCT, Power, TC, Haykal, HA & Zamani, AA (1987). Chloroma of cerebellum, tentorium and occipital bone in acute myelogenous leukemia. Neuroradiology, 29, 590. Wendling, LR, Cromwell, LD & Latchaw, RE (1979). Computed tomography of intracerebral Ieukaemia masses. American Journal of Roentgenology, 132, 217 220. Wiernik, PH & Serpick, AA (1970). Granulocytic sarcoma (ehloroma). Blood, 35, 361-369.
