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Multiple cerebral cryptococcomas in an immunocompetent man: an unlikely diagnosis A 59-year-old man complained of flashing lights and intermittent blurriness in the lower quadrant of his right eye 9 months after cataract surgery, prompting a magnetic resonance imaging (MRI) of the brain, which revealed two separate intracerebral lesions. On neurosurgical review, the patient reported feeling well, with no symptoms of raised intracranial pressure or infection. He was generally fit and well with no medical history and a normal neurological examination. MRI of the brain revealed two large lobulated multiseptal cystic lesions with rim enhancement and peri-lesional oedema located at the inferior aspect of the right temporal lobe and the left occipital lobe (Figs 1,2). Laboratory investigations revealed normal white cell counts. Differential diagnoses included cerebral metastases, multifocal glioma and cerebral abscess. A stereotactic craniotomy for removal of the right temporal lobe lesion was performed. The lesion was carefully exposed, revealing an avascular, pearly, multilobulated lesion. This was removed en bloc without breach of content (Fig. 3). Frozen section identified a cryptococcoma with cultures identifying Cryptococcus gattii. Amphotericin-B and 5-FC IV was commenced. HIV testing was negative. The left occipital lobe lesion was removed 6 days later in the same manner, revealing cryptococcoma. Tissue cultures on this occasion yielded no fungal growth. Recovery from both operations was uneventful. He continued a 4-week course of i.v. antifungal therapy and followed by 12 months of oral antifungal therapy. Follow-up bloods and cultures were clear. MRI of the brain at 12 months was normal.
Of over 30 species of the Cryptococcus fungus, only a small number of species are major pathogens to humans. Central nervous system (CNS) cryptococcal infections often occur in immunocompromised hosts, including patients with AIDS, transplant recipients or other patients receiving immunosuppressive therapies.1 They can also occur in patients with less severe levels of immunosuppression, including pregnancy and diabetes. Cryptococcus neoformans, found in soil contaminated with pigeon droppings, is the main pathogen to cause cryptococcosis in the immunocompromised population. There is a substantial regional variation in the geographical distribution of C. neoformans infections within Australia, with the highest incidence reported from the Northern Territory and Queensland. While it is established that CNS cryptococcosis is common among the immunocompromised, it is rarely found in the immunocompetent. C. gattii is the typical pathogen to infect the immunocompetent host. It is more geographically restricted and is associated with tropical and subtropical climates including Australia and South America2 with eucalyptus trees and decaying hollows in living trees as the major reservoir.1 In Australia, C. gattii infections are more often found in rural or semirural areas on the Australian mainland, broadly corresponding to the distribution of eucalyptus trees with the highest incidence in the Northern Territory.3 Most immunocompetent hosts with cryptococcoma (an accumulation of cryptococcus and inflammatory cells) present with symptoms suggesting intracranial pathology, including headache, vomiting, change in consciousness or mental state, cranial nerve dysfunction or seizures.4 The diagnosis of CNS cryptococcosis has
Fig. 1. T1-weighted magnetic resonance image (axial) with contrast showing the rim-enhancing lesion in the right temporal lobe.
© 2013 Royal Australasian College of Surgeons
ANZ J Surg •• (2013) ••–••
2
Images for surgeons
Fig. 2. T1-weighted magnetic resonance image (sagittal) with contrast showing the left occipital lobe lesion.
CNS cryptococcoma is known but rare among the immunocompetent population. Although most cerebral cryptococcomas present with systemic illness and signs of intracranial pathology, this case demonstrates that they may be clinically silent with few symptoms and signs, even in the presence of multiple cerebral lesions.
References
Fig. 3. The cryptococcoma, resected en bloc.
been reported to take up to 1 year from the onset of symptoms in an immunocompetent host.5 The misdiagnosis rate was 52.9%, rendering tumour a priority for the differential diagnosis. The intensity features of MRI T1- and T2-weighted images with gadolinium of most cases are insufficient to distinguish cryptococcoma from neoplasms.6 Isolated cryptococcomas or enhancing cortical nodules are seen only in a minority of patients, and the most common sites for these lesions are the midbrain and basal ganglia.
1. Li SS, Mody CH. Cryptococcus. Proc. Am. Thorac. Soc. 2009; 7: 186–96. 2. Casadevall A, Perfect J. Cryptococcus neoformas. Washington, DC: ASM Press, 1998. 3. Chen S, Sorrell T, Nimmo G et al. Epidimiology and host and varietydependent characteristics of infection due to Cryptococcus neoformans in Australia and New Zealand. Clin. Infect. Dis. 2000; 31: 499–508. 4. Li Q, You C, Liu Y. Central nervous system cryptococcoma in immunocompetent patients: a short review illustrated by a new case. Acta Neurochir. (Wien) 2010; 152: 129–36. 5. Ecevit IZ, Clancy CJ, Schmalfuss IM, Nguyen MH. The poor prognosis of central nervous system cryptococcosis among non-immunisupressed patients: a call for better disease recognition and evaluation of adjucts to antifungal therapy. Clin. Infect. Dis. 2006; 42: 1443–7. 6. Li Q, You C, Liu Y. Central nervous system cryptococcoma in immunocompetent patients: a short review illustrated by a new case. Acta Neurochir. (Wien) 2010; 152: 129–36.
Vincent Sze Sern King, MBChB Mark Jonathan Winder, MBBS (Hons), FRACS St Vincent’s Hospital, Sydney, New South Wales, Australia doi: 10.1111/ans.12250
© 2013 Royal Australasian College of Surgeons
Multiple cerebral cryptococcomas in an immunocompetent man: an unlikely diagnosis A 59-year-old man complained of flashing lights and intermittent blurriness in the lower quadrant of his right eye 9 months after cataract surgery, prompting a magnetic resonance imaging (MRI) of the brain, which revealed two separate intracerebral lesions. On neurosurgical review, the patient reported feeling well, with no symptoms of raised intracranial pressure or infection. He was generally fit and well with no medical history and a normal neurological examination. MRI of the brain revealed two large lobulated multiseptal cystic lesions with rim enhancement and peri-lesional oedema located at the inferior aspect of the right temporal lobe and the left occipital lobe (Figs 1,2). Laboratory investigations revealed normal white cell counts. Differential diagnoses included cerebral metastases, multifocal glioma and cerebral abscess. A stereotactic craniotomy for removal of the right temporal lobe lesion was performed. The lesion was carefully exposed, revealing an avascular, pearly, multilobulated lesion. This was removed en bloc without breach of content (Fig. 3). Frozen section identified a cryptococcoma with cultures identifying Cryptococcus gattii. Amphotericin-B and 5-FC IV was commenced. HIV testing was negative. The left occipital lobe lesion was removed 6 days later in the same manner, revealing cryptococcoma. Tissue cultures on this occasion yielded no fungal growth. Recovery from both operations was uneventful. He continued a 4-week course of i.v. antifungal therapy and followed by 12 months of oral antifungal therapy. Follow-up bloods and cultures were clear. MRI of the brain at 12 months was normal.
Of over 30 species of the Cryptococcus fungus, only a small number of species are major pathogens to humans. Central nervous system (CNS) cryptococcal infections often occur in immunocompromised hosts, including patients with AIDS, transplant recipients or other patients receiving immunosuppressive therapies.1 They can also occur in patients with less severe levels of immunosuppression, including pregnancy and diabetes. Cryptococcus neoformans, found in soil contaminated with pigeon droppings, is the main pathogen to cause cryptococcosis in the immunocompromised population. There is a substantial regional variation in the geographical distribution of C. neoformans infections within Australia, with the highest incidence reported from the Northern Territory and Queensland. While it is established that CNS cryptococcosis is common among the immunocompromised, it is rarely found in the immunocompetent. C. gattii is the typical pathogen to infect the immunocompetent host. It is more geographically restricted and is associated with tropical and subtropical climates including Australia and South America2 with eucalyptus trees and decaying hollows in living trees as the major reservoir.1 In Australia, C. gattii infections are more often found in rural or semirural areas on the Australian mainland, broadly corresponding to the distribution of eucalyptus trees with the highest incidence in the Northern Territory.3 Most immunocompetent hosts with cryptococcoma (an accumulation of cryptococcus and inflammatory cells) present with symptoms suggesting intracranial pathology, including headache, vomiting, change in consciousness or mental state, cranial nerve dysfunction or seizures.4 The diagnosis of CNS cryptococcosis has
Fig. 1. T1-weighted magnetic resonance image (axial) with contrast showing the rim-enhancing lesion in the right temporal lobe.
© 2013 Royal Australasian College of Surgeons
ANZ J Surg •• (2013) ••–••
2
Images for surgeons
Fig. 2. T1-weighted magnetic resonance image (sagittal) with contrast showing the left occipital lobe lesion.
CNS cryptococcoma is known but rare among the immunocompetent population. Although most cerebral cryptococcomas present with systemic illness and signs of intracranial pathology, this case demonstrates that they may be clinically silent with few symptoms and signs, even in the presence of multiple cerebral lesions.
References
Fig. 3. The cryptococcoma, resected en bloc.
been reported to take up to 1 year from the onset of symptoms in an immunocompetent host.5 The misdiagnosis rate was 52.9%, rendering tumour a priority for the differential diagnosis. The intensity features of MRI T1- and T2-weighted images with gadolinium of most cases are insufficient to distinguish cryptococcoma from neoplasms.6 Isolated cryptococcomas or enhancing cortical nodules are seen only in a minority of patients, and the most common sites for these lesions are the midbrain and basal ganglia.
1. Li SS, Mody CH. Cryptococcus. Proc. Am. Thorac. Soc. 2009; 7: 186–96. 2. Casadevall A, Perfect J. Cryptococcus neoformas. Washington, DC: ASM Press, 1998. 3. Chen S, Sorrell T, Nimmo G et al. Epidimiology and host and varietydependent characteristics of infection due to Cryptococcus neoformans in Australia and New Zealand. Clin. Infect. Dis. 2000; 31: 499–508. 4. Li Q, You C, Liu Y. Central nervous system cryptococcoma in immunocompetent patients: a short review illustrated by a new case. Acta Neurochir. (Wien) 2010; 152: 129–36. 5. Ecevit IZ, Clancy CJ, Schmalfuss IM, Nguyen MH. The poor prognosis of central nervous system cryptococcosis among non-immunisupressed patients: a call for better disease recognition and evaluation of adjucts to antifungal therapy. Clin. Infect. Dis. 2006; 42: 1443–7. 6. Li Q, You C, Liu Y. Central nervous system cryptococcoma in immunocompetent patients: a short review illustrated by a new case. Acta Neurochir. (Wien) 2010; 152: 129–36.
Vincent Sze Sern King, MBChB Mark Jonathan Winder, MBBS (Hons), FRACS St Vincent’s Hospital, Sydney, New South Wales, Australia doi: 10.1111/ans.12250
© 2013 Royal Australasian College of Surgeons
