1979, British Journal of Radiology, 52, 916-917 Case reports REFERENCES
the ureter. The Journal of Urology, 175,651-653.
BANNER, M. P. and POLLACK, H. M., 1979. Fibrous ureteral
SODERDAHL, D. W. and SCHUSTER, S. R., 1969. Benign
polyps. Radiology, 130, 73—76.
ureteral polyp in the newborn. Journal of the American Medical Association, 207, 1714-171 5.
CRUM, P. M., SAYEGH, E. S., SACHER, E. C. and WESTCOTT,
J. W., 1969. Benign ureteral polyps. The Journal of Urology, 102, 678-682.
STUPPLER, S. A. and KANDZARI, S. J., 1975. Fibroepithelial
polyps of ureter—a benign ureteral tumor. Urology, 5, 553-558.
DAVIDES, K. C. and KING, L. M., 1976. Fibrous polyps of
Intradural extramedullary aspergilloma complicating chronic lymphatic leukaemia By D. P. E. Kingsley, F.R.C.S., F.R.C.R., Elizabeth White, M.R.C.P., F.R.C.R., Annabella Marks, B.Sc, M.R.C.P., and Ann Coxon, M.R.C.P. Departments of Radiodiagnosis, Radiotherapy and Neurology, St. Bartholomew's Hospital, London EC1 {Received January 1979 and in revised form May 1979) Intradural extramedullary masses causing compression of the spinal cord consist almost exclusively of benign tumours, the majority being neurofibromata and meningiomas (Epstein, 1976), while lipomata (Caram et al., 1957), dermoids, epidermoids and teratomas (McCarty et al., 1959) are occasionally encountered. Less commonly compression may be due to malignant tumours, usually metastases from primary brain tumours (Sagerman et al., 1965) or more rarely from sites outside the nervous system. Spinal cord compression in the lymphomas and leukaemias is usually due to an extradural deposit with or without bony involvement (Verity, 1968; Mullins et al., 1971). Since these lesions are radiosensitive and urgent treatment is often indicated, radiotherapy may be undertaken without histological evidence of the nature of the lesion. Inflammatory lesions causing cord compression are not uncommon but are almost exclusively extradural and are often associated with adjacent bony involvement (Browder and Myers, 1937). Intradural inflammatory lesions are rare and usually cause arachnoiditis. Since no example of an intradural extramedullary inflammatory mass complicating leukaemia has been reported we would like to document this case because of its therapeutic implications.
During the spring and summer of 1978 transient pulmonary infiltrates were noted on chest radiographs, but sputum culture for aspergillosis proved negative at that time. In September, 1978 she was admitted with a two week history of generalized malaise, pyrexia, cough, pain between the scapulae, a progressive thoracic paraparesis and urinary retention. The CSF showed 100 polymorphs and 427 lymphocytes per millilitre. Radiographs of the spine and chest at that time were normal. Myelography was undertaken using metrizamide. There was irregularity of the theca anteriorly, between D7 and D9 with a number of separate intradural extramedullary defects at that level (Fig. 1A). There were also discrete nodules on the surface of the cord throughout its length (Fig. 1B). There was no evidence of bony involvement or para-vertebral soft tissue mass. Computed tomography of the spine undertaken at the same time was unhelpful. A decompressive thoracic laminectomy was undertaken and the presence of an intradural mass was confirmed on the right side at D7 extending anteriorly, infiltrating and involving the cord and its adjacent roots. In addition, 4 cm proximally there were three circular white deposits attached to nerve roots. There was no extradural component and no sign of meningeal inflammation. Histology demonstrated fungal mycelia consisting predominantly of hyphae with some yeast-like elements, later confirmed as aspergillus fumigatus. She was treated with amphotericin B intravenously and intrathecally and flucytosine orally, but after some initial improvement her paraplegia became worse. A further myelogram two weeks later demonstrated complete obstruction at D5 while contrast introduced through a cisternal puncture demonstrated obstruction at the lower cervical level. She was given further amphotericin B both intrathecally and intravenously as well as through an Ommaya reservoir, but gradually deteriorated and died approximately one month after admission. Post-mortem examination was refused.
CASE HISTORY
A 52-year-old, married housewife had a five year history of chronic lymphatic leukaemia, treated with corticosteroids (5-10 mg daily) and chlorambucil. During this period she had remained in the chronic phase of the disease with a raised white cell count and hepatosplenomegaly and a slight anaemia. IgA was reduced but IgG was at the lower limits of normal. In May, 1977 she developed transient numbness of the right side of the face with severe ataxia and vertigo suggesting a brain-stem lesion. Cerebro-spinal fluid at that time demonstrated an excess of lymphocytes. She made a gradual but incomplete recovery without treatment.
DISCUSSION
Opportunistic infections are becoming more frequent in patients with malignant disease due to deficient immunological competence as a result of drug therapy or irradiation (Young et al., 1970; O'Connell et al., 1973; Ingwer, et al., 1978). In most cases involvement of the central nervous system is part of a systemic dissemination of the fungus and
916
VOL. 52, No. 623
Case reports rare. Intrathecal fungal infection is extremely difficult to diagnose without positive histology since the mycelia are almost invariably undetectable in the cerebro-spinal fluid and the value of other more specific investigation may be limited because of cortico-steroid treatment. Although these patients frequently require urgent treatment, the demonstration of such a lesion therefore requires histological confirmation before radiotherapy is undertaken. ACKNOWLEDGMENT
The authors wish to thank Dr. George Marsh of the North Middlesex Hospital and Dr. Geoffrey Gawler of St. Bartholomew's Hospital, for permission to publish this case report and Miss Wendy Pidgeon for typing the manuscript. REFERENCES BROWDER, J. and MYERS, R., 1937. Infections of the spinal
epidural space; an aspect of vertebral osteomyelitis. American Journal of Surgery, 37, 4-26. CARAM, P. C , SCARCELLA, G. and CARTON, C. A., 1957.
Intradural lipomas of the spinal cord with particular emphasis on the "intramedullary" lipomas. Journal of Neurosurgery, 14, 28-42. DIAMOND, H. D., WILLIAMS, H. M. and CRAVER, L. F.,
1960. The pathogenesis and management of neurological complications of malignant lymphomas and leukaemia. Ada Unto Internationalis Contra Cancrum, 16, 813-840. EPSTEIN, B. S., 1976. The spine. A Radiological Text and Atlas. 4th ed., p. 718 (Lea and Febiger, Philadelphia). INGWER, I., MCLEISH, K. R., TIGHT, R. R. and WHITE, A. A
C , 1978. Aspergillus fumigatus epidural abscess in a renal transplant recipient. Archives of Internal Medicine, 138, 1 53-154.
B
1. Water-soluble contrast myelogram in the mid-dorsal region. (A) AP view demonstrating multiple intradural extramedullary masses. (B) AP view of the cauda equina demonstrating small nodules attached to nerve roots. FIG.
MCCARTY, C. S., LEAVENS, M. E., LOVE, J. G. and KERNOHAN,
J. W., 1959. Dermoid and epidermoid tumours in the central nervous system of adults. Surgery, Gynaecology and Obstetrics, 705, 191-198. MUKOYAMA, M., GIMPLE, K. and POSER, C. M., 1969.
even then intracranial infection is far more common than involvement of the spinal cord (Mukoyama et al, 1969; Seres, et al, 1972). Neurological complications involving the spinal cord are uncommon in the lymphomas and even rarer in leukaemia. Diamond et al. (1960), in a large series covering 30 years up to 1956, found a 2.7% incidence of spinal cord involvement in 3725 patients with lymphoma, but only 0.7% in 1864 patients with leukaemia. The vast majority of these were extradural masses and no comment was made about intradural deposits. Other authors report an incidence of 3 to 5% of spinal cord involvement in Hodgkin's disease (Thies et al., 1961; Murphy and Bilge, 1964; Newall 1965). Involvement of the neuraxis by aspergillosis occurs almost exclusively as a terminal manifestation of the disease and few patients survive, ours being no exception. An intradural extramedullary mass in a patient with diminished immunological competence raises the possibility of an opportunistic infection, since such an appearance due to metastatic disease is
Aspergillosis of the central nervous system. Report of a brain abscess and review of the literature. Neurology, {Minneapolis), 19, 967-974. MULLINS, G. M., FLYNN, J. P. G., E L MAHDI, A. M., MCQUEEN, J. D. and OWENS, A. H., 1971. Malignant
lymphoma of the spinal epidural space. Annals of Internal Medicine, 74,416-423. MURPHY, W. T. and BILGE, N., 1964. Compression of the
spinal cord in patients with malignant lymphomas. Radiology, 82, 495-501. NEWALL, J., 1965. The management of Hodgkin's disease. Clinical Radiology, 76,40-50. O'CONNELL, C. J., CHERRY, A. V. and ZOLL, I. G., 1973.
Osteomyelitis of the cervical spine—Candida guillermondii. Annals of Internal Medicine, 79, 748. SAGERMAN, R. H. BAGASHAW, M. A. and HANBERRY, J., 1965.
Considerations in the treatment Radiology, 84, 401-408.
of ependymoma.
SERES, S. L., ONO, H. and BENNER, E. J., 1972. Aspergillosis
presenting as spinal cord compression. Journal of Neurosurgery, 36, 221-224. THIES, H., KIEFER, H. and NOETZEL, H., 1961. Neurological
complications of Hodgkin's disease. Deutsche Med Wochenschr., 86, 1908-1917. VERITY, G. L., 1968. Neurological manifestations and complications of lymphoma. Neurological Clinics of North America, 6, 97-109. YOUNG, R. C , BENNET, J. E., VOGEL, C. L., CARBONNE, P. P.
917
and DEVITA, V. T., 1970. Aspergillosis, the spectrum of the disease in 98 patients. Medicine (Baltimore), 49, 147-173.
the ureter. The Journal of Urology, 175,651-653.
BANNER, M. P. and POLLACK, H. M., 1979. Fibrous ureteral
SODERDAHL, D. W. and SCHUSTER, S. R., 1969. Benign
polyps. Radiology, 130, 73—76.
ureteral polyp in the newborn. Journal of the American Medical Association, 207, 1714-171 5.
CRUM, P. M., SAYEGH, E. S., SACHER, E. C. and WESTCOTT,
J. W., 1969. Benign ureteral polyps. The Journal of Urology, 102, 678-682.
STUPPLER, S. A. and KANDZARI, S. J., 1975. Fibroepithelial
polyps of ureter—a benign ureteral tumor. Urology, 5, 553-558.
DAVIDES, K. C. and KING, L. M., 1976. Fibrous polyps of
Intradural extramedullary aspergilloma complicating chronic lymphatic leukaemia By D. P. E. Kingsley, F.R.C.S., F.R.C.R., Elizabeth White, M.R.C.P., F.R.C.R., Annabella Marks, B.Sc, M.R.C.P., and Ann Coxon, M.R.C.P. Departments of Radiodiagnosis, Radiotherapy and Neurology, St. Bartholomew's Hospital, London EC1 {Received January 1979 and in revised form May 1979) Intradural extramedullary masses causing compression of the spinal cord consist almost exclusively of benign tumours, the majority being neurofibromata and meningiomas (Epstein, 1976), while lipomata (Caram et al., 1957), dermoids, epidermoids and teratomas (McCarty et al., 1959) are occasionally encountered. Less commonly compression may be due to malignant tumours, usually metastases from primary brain tumours (Sagerman et al., 1965) or more rarely from sites outside the nervous system. Spinal cord compression in the lymphomas and leukaemias is usually due to an extradural deposit with or without bony involvement (Verity, 1968; Mullins et al., 1971). Since these lesions are radiosensitive and urgent treatment is often indicated, radiotherapy may be undertaken without histological evidence of the nature of the lesion. Inflammatory lesions causing cord compression are not uncommon but are almost exclusively extradural and are often associated with adjacent bony involvement (Browder and Myers, 1937). Intradural inflammatory lesions are rare and usually cause arachnoiditis. Since no example of an intradural extramedullary inflammatory mass complicating leukaemia has been reported we would like to document this case because of its therapeutic implications.
During the spring and summer of 1978 transient pulmonary infiltrates were noted on chest radiographs, but sputum culture for aspergillosis proved negative at that time. In September, 1978 she was admitted with a two week history of generalized malaise, pyrexia, cough, pain between the scapulae, a progressive thoracic paraparesis and urinary retention. The CSF showed 100 polymorphs and 427 lymphocytes per millilitre. Radiographs of the spine and chest at that time were normal. Myelography was undertaken using metrizamide. There was irregularity of the theca anteriorly, between D7 and D9 with a number of separate intradural extramedullary defects at that level (Fig. 1A). There were also discrete nodules on the surface of the cord throughout its length (Fig. 1B). There was no evidence of bony involvement or para-vertebral soft tissue mass. Computed tomography of the spine undertaken at the same time was unhelpful. A decompressive thoracic laminectomy was undertaken and the presence of an intradural mass was confirmed on the right side at D7 extending anteriorly, infiltrating and involving the cord and its adjacent roots. In addition, 4 cm proximally there were three circular white deposits attached to nerve roots. There was no extradural component and no sign of meningeal inflammation. Histology demonstrated fungal mycelia consisting predominantly of hyphae with some yeast-like elements, later confirmed as aspergillus fumigatus. She was treated with amphotericin B intravenously and intrathecally and flucytosine orally, but after some initial improvement her paraplegia became worse. A further myelogram two weeks later demonstrated complete obstruction at D5 while contrast introduced through a cisternal puncture demonstrated obstruction at the lower cervical level. She was given further amphotericin B both intrathecally and intravenously as well as through an Ommaya reservoir, but gradually deteriorated and died approximately one month after admission. Post-mortem examination was refused.
CASE HISTORY
A 52-year-old, married housewife had a five year history of chronic lymphatic leukaemia, treated with corticosteroids (5-10 mg daily) and chlorambucil. During this period she had remained in the chronic phase of the disease with a raised white cell count and hepatosplenomegaly and a slight anaemia. IgA was reduced but IgG was at the lower limits of normal. In May, 1977 she developed transient numbness of the right side of the face with severe ataxia and vertigo suggesting a brain-stem lesion. Cerebro-spinal fluid at that time demonstrated an excess of lymphocytes. She made a gradual but incomplete recovery without treatment.
DISCUSSION
Opportunistic infections are becoming more frequent in patients with malignant disease due to deficient immunological competence as a result of drug therapy or irradiation (Young et al., 1970; O'Connell et al., 1973; Ingwer, et al., 1978). In most cases involvement of the central nervous system is part of a systemic dissemination of the fungus and
916
VOL. 52, No. 623
Case reports rare. Intrathecal fungal infection is extremely difficult to diagnose without positive histology since the mycelia are almost invariably undetectable in the cerebro-spinal fluid and the value of other more specific investigation may be limited because of cortico-steroid treatment. Although these patients frequently require urgent treatment, the demonstration of such a lesion therefore requires histological confirmation before radiotherapy is undertaken. ACKNOWLEDGMENT
The authors wish to thank Dr. George Marsh of the North Middlesex Hospital and Dr. Geoffrey Gawler of St. Bartholomew's Hospital, for permission to publish this case report and Miss Wendy Pidgeon for typing the manuscript. REFERENCES BROWDER, J. and MYERS, R., 1937. Infections of the spinal
epidural space; an aspect of vertebral osteomyelitis. American Journal of Surgery, 37, 4-26. CARAM, P. C , SCARCELLA, G. and CARTON, C. A., 1957.
Intradural lipomas of the spinal cord with particular emphasis on the "intramedullary" lipomas. Journal of Neurosurgery, 14, 28-42. DIAMOND, H. D., WILLIAMS, H. M. and CRAVER, L. F.,
1960. The pathogenesis and management of neurological complications of malignant lymphomas and leukaemia. Ada Unto Internationalis Contra Cancrum, 16, 813-840. EPSTEIN, B. S., 1976. The spine. A Radiological Text and Atlas. 4th ed., p. 718 (Lea and Febiger, Philadelphia). INGWER, I., MCLEISH, K. R., TIGHT, R. R. and WHITE, A. A
C , 1978. Aspergillus fumigatus epidural abscess in a renal transplant recipient. Archives of Internal Medicine, 138, 1 53-154.
B
1. Water-soluble contrast myelogram in the mid-dorsal region. (A) AP view demonstrating multiple intradural extramedullary masses. (B) AP view of the cauda equina demonstrating small nodules attached to nerve roots. FIG.
MCCARTY, C. S., LEAVENS, M. E., LOVE, J. G. and KERNOHAN,
J. W., 1959. Dermoid and epidermoid tumours in the central nervous system of adults. Surgery, Gynaecology and Obstetrics, 705, 191-198. MUKOYAMA, M., GIMPLE, K. and POSER, C. M., 1969.
even then intracranial infection is far more common than involvement of the spinal cord (Mukoyama et al, 1969; Seres, et al, 1972). Neurological complications involving the spinal cord are uncommon in the lymphomas and even rarer in leukaemia. Diamond et al. (1960), in a large series covering 30 years up to 1956, found a 2.7% incidence of spinal cord involvement in 3725 patients with lymphoma, but only 0.7% in 1864 patients with leukaemia. The vast majority of these were extradural masses and no comment was made about intradural deposits. Other authors report an incidence of 3 to 5% of spinal cord involvement in Hodgkin's disease (Thies et al., 1961; Murphy and Bilge, 1964; Newall 1965). Involvement of the neuraxis by aspergillosis occurs almost exclusively as a terminal manifestation of the disease and few patients survive, ours being no exception. An intradural extramedullary mass in a patient with diminished immunological competence raises the possibility of an opportunistic infection, since such an appearance due to metastatic disease is
Aspergillosis of the central nervous system. Report of a brain abscess and review of the literature. Neurology, {Minneapolis), 19, 967-974. MULLINS, G. M., FLYNN, J. P. G., E L MAHDI, A. M., MCQUEEN, J. D. and OWENS, A. H., 1971. Malignant
lymphoma of the spinal epidural space. Annals of Internal Medicine, 74,416-423. MURPHY, W. T. and BILGE, N., 1964. Compression of the
spinal cord in patients with malignant lymphomas. Radiology, 82, 495-501. NEWALL, J., 1965. The management of Hodgkin's disease. Clinical Radiology, 76,40-50. O'CONNELL, C. J., CHERRY, A. V. and ZOLL, I. G., 1973.
Osteomyelitis of the cervical spine—Candida guillermondii. Annals of Internal Medicine, 79, 748. SAGERMAN, R. H. BAGASHAW, M. A. and HANBERRY, J., 1965.
Considerations in the treatment Radiology, 84, 401-408.
of ependymoma.
SERES, S. L., ONO, H. and BENNER, E. J., 1972. Aspergillosis
presenting as spinal cord compression. Journal of Neurosurgery, 36, 221-224. THIES, H., KIEFER, H. and NOETZEL, H., 1961. Neurological
complications of Hodgkin's disease. Deutsche Med Wochenschr., 86, 1908-1917. VERITY, G. L., 1968. Neurological manifestations and complications of lymphoma. Neurological Clinics of North America, 6, 97-109. YOUNG, R. C , BENNET, J. E., VOGEL, C. L., CARBONNE, P. P.
917
and DEVITA, V. T., 1970. Aspergillosis, the spectrum of the disease in 98 patients. Medicine (Baltimore), 49, 147-173.
