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Year : 2013  |  Volume : 61  |  Issue : 5  |  Page : 526--528

Cerebral pheohyphomycosis: Report of a rare case with review of literature

Shantha Ravisankar1, R Vimal Chander2,  
1 Department of Pathology, Institute of Neurology, Madras Medical College, Chennai, Tamil Nadu, India
2 Department of Pathology, Saveetha Medical College, Thandalam, Chennai, Tamil Nadu, India

Correspondence Address:
Shantha Ravisankar
Department of Pathology, Institute of Neurology, Madras Medical College, Chennai, Tamil Nadu

How to cite this article:
Ravisankar S, Chander R V. Cerebral pheohyphomycosis: Report of a rare case with review of literature.Neurol India 2013;61:526-528

How to cite this URL:
Ravisankar S, Chander R V. Cerebral pheohyphomycosis: Report of a rare case with review of literature. Neurol India [serial online] 2013 [cited 2022 Jun 29 ];61:526-528
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Pheohyphomycosis is a group of fungal infections characterized by presence of septate pigmented hyphae in the tissues and includes cutaneous, subcutaneous and systemic infections. [1] Central nervous system pheohyphomycosis is very rare and presents with unusual features and is associated with poor prognosis without appropriate treatment. [2]

A 65-year-old male patient presented with fever and later developed left sided hemiparesis of 1 month duration. Contrast computed tomography (CT) and magnetic resonance imaging showed a ring enhancing lesion in the right frontal lobe [Figure 1]. Excision of the mass was performed. Gross examination showed multiple gray white soft-tissue fragments measuring 1 cc in aggregate. Microscopic examination showed portions of reactive glial tissue with central area of necrosis and dense infiltrate of neutrophils, lymphocytes and plasma cells with scattered foreign body type of giant cells and fungal hyphae with pigmented walls. There were also focal micro abscesses composed of septate, thin mycelial filaments and spores with brown pigmentation surrounded by chronic inflammatory infiltrates [Figure 2] and [Figure 3]. Periodic acid Schiff stain showed PAS positive fungal hyphae-slender, septate hyphae with conidia and spores [Figure 4]a. The fungal elements were also positive on Masson Fontana staining indicating the presence of melanin [Figure 4]b. A final impression of cerebral pheohyphomycosis was given. The postoperative course was uneventful, itraconazole was continued and he was discharged after 2 weeks. He is doing well and attends the outpatient department regularly.{Figure 1}{Figure 2}{Figure 3}{Figure 4}

Pheohyphomycosis is the term used for infections caused by dematiaceous fungi with characteristic presence of melanin pigment in their cell walls. Clinically, phaeohyphomycosis has been classified according to the anatomical site involved as (1) cutaneous (2) subcutaneous (3) paranasal sinus and (4) cerebral types. [1] Cerebral pheohyphomycosis is a very rare, but the most serious form of disease, usually caused by Cladophialophora bantiana. [2] Less common causes include Ramichloridium mackenzei, Ochroconis gallopavum, Thielavia subthermophila and Fonsecaea species. [3] The portal of entry is not known, though inhalation of spores into the lung and colonization and subsequent hematogenous spread has been suggested. [2]

Life-threatening fungal infections are often associated with severe immunocompromised states. However, primary cerebral pheohyphomycosis appears to be an exception to this rule, since it is increasingly recognized as a cause of serious disease in patients with or without immunodeficiency and patients are often immunocompetent with no known underlying diseases. [2] Presence of melanin in these fungi provides a protective advantage in evading host defenses and may be responsible for its pathogenic potential. [4]

Symptoms and signs are largely nonspecific mostly as a result of pressure effect leading to headache, papilledema and hemiparesis. It clinically presents usually as brain abscess, rarely as chronic meningitis, encephalitis, myelitis or significant cerebrospinal fluid eosinophilia and very rarely as an intraventricular mass. [5] CT typically show an irregular variably contrast enhancing mass with surrounding hypodense edema, mimicking high grade gliomas or metastatic lesions in brain. [6] On histopathological examination, they usually have a characteristic appearance of irregularly swollen hyphae with yeast-like structures. Masson-Fontana stain for melanin is used to confirm the presence of dematiaceous hyphae. Prognosis is very poor without treatment. There is no standard therapy for these often fatal infections, and few studies reported that a combination of amphotericin B, 5-fluorouracil and itraconazole was associated with improved survival. [7]


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