- Department of Neurology, Hospital de Clínicas de Porto Alegre, Rua Ramiro Barcelos 2350, 90035-903, Porto Alegre, RS, Brazil
- Graduate Program in Medicine: Surgical Sciences, School of Medicine, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos 2400, 90035-003, Porto Alegre, RS, Brazil
- Department of Pathology, Hospital de Clínicas de Porto Alegre, Rua Ramiro Barcelos 2350, 90035-903, Porto Alegre, RS, Brazil
Gustavo R. Isolan
Department of Neurology, Hospital de Clínicas de Porto Alegre, Rua Ramiro Barcelos 2350, 90035-903, Porto Alegre, RS, Brazil
Graduate Program in Medicine: Surgical Sciences, School of Medicine, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos 2400, 90035-003, Porto Alegre, RS, Brazil
DOI:10.4103/2152-7806.140656Copyright: © 2014 Isolan GR. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Isolan GR, Vieira DM, Hehn F, Ápio C. M. Antunes. Paracoccidioidomycosis simulating brain tumor. Surg Neurol Int 12-Sep-2014;5:134
How to cite this URL: Isolan GR, Vieira DM, Hehn F, Ápio C. M. Antunes. Paracoccidioidomycosis simulating brain tumor. Surg Neurol Int 12-Sep-2014;5:134. Available from: http://sni.wpengine.com/surgicalint_articles/paracoccidioidomycosis-simulating-brain-tumor/
Background:Paracoccidioidomycosis (PCM) is a systemic granulomatous disease caused by Paracoccidioides brasiliensis. Involvement of the central nervous system (CNS) occurs in about 10% of cases.
Case Description:A 57-year-old white man presented with the complaint of headache and an episode of focal seizure 1 month earlier. Magnetic resonance imaging (MRI) revealed a ring-enhancing lesion in the right parietal lobe with peri-lesional vasogenic edema suggestive of a primary neoplasm. The patient underwent craniotomy and the intraoperative finding was a yellowish, hard lesion with thick content and yellow inside. Anatomo-pathological findings were pathognomonic of PCM: large, thick-walled, spherical yeast cells with multiple peripheral buds. The patient tested negative for human immunodeficiency virus (HIV). Encephalitis and meningitis were ruled out by cerebrospinal fluid analysis. Culture confirmed the diagnosis of PCM and the patient was treated with amphotericin B. The patient responded well to treatment with resolution of the headache and clinical improvement, despite a bitemporal hemianopia. He was clinically stable and then discharged in good general condition.
Conclusions:Radiographic findings of PCM with CNS involvement may suggest neoplasia, making diagnosis difficult. In endemic areas, the diagnosis of PCM should be promptly considered when a ring-enhancing mass associated with peri-lesional edema is observed on MRI.
Keywords: Brain neoplasms, central nervous system, magnetic resonance imaging, paracoccidioidomycosis
Paracoccidioidomycosis (PCM) is a systemic granulomatous disease caused by a dimorphic fungus known as Paracoccidioides brasiliensis.[
A 57-year-old white man presented to the neurosurgery outpatient clinic at a tertiary care hospital located in the city of Porto Alegre, southern Brazil, with the complaint of headache. The patient reported an episode of focal seizure that had occurred 1 month earlier. Neurological examination revealed no abnormalities, except for left hemianopia on confrontation visual field test.
Brain MRI showed an expansive, relatively well-defined mass in the right parietal lobe [Figure
Preoperative chest X-ray film revealed multiple bilateral micro-nodules, with emphysema bubbles in the apices. The patient underwent craniotomy and the intraoperative finding was a yellowish, hard lesion with good cleavage plane, thick content, and yellow inside. There was a hypothesis of brain abscess. The patient tested negative for human immunodeficiency virus (HIV) antibodies.
On anatomo-pathological examination, the findings were pathognomonic of PCM: large, thick-walled, spherical yeast cells with multiple peripheral buds, with the capsule wall thickness giving the impression of a double capsule, although it was a single capsule [Figure
Histological sections showing (a) the edge of brain parenchyma with reactive infiltrate of lymphocytes and giant cells with central necrosis gliosis (H and E, ×100) and (b) presence of characteristic helm-shaped yeasts compatible with paracoccidioidomycosis (Gomori methenamine silver stain, 600×)
Serum and cerebrospinal fluid (CSF) specimens were negative for cryptococcal antigen by the cryptococcal latex agglutination test (Crypto-LA; Wampole Laboratories, Cranbury, NJ, USA). Gram-stained CSF contained 4 leukocytes/mm3, 8 mg/dl of glucose, and 4 mg/dl of total protein. Four days later, gram-stained CSF contained 2 leukocytes/mm3, 46 mg/dl of glucose, and 33 mg/dl of total protein. No microorganisms were detected on Gram stain of the CSF. Fibrotic bronchoscopy (FOB) performed 1 day later revealed bleeding at the carina and pus in the right upper lobe (RUL). Analysis of cells obtained from RUL broncho-alveolar lavage (BAL) fluid showed a negative smear for acid-fast bacilli (AFB), negative Gram stain, and presence of Strongyloides stercoralis larvae and yeast cells compatible with P. brasiliensis.
Abdominal computed tomography (CT) scan showed a nodule in the prostate and a left adrenal nodule. Chest CT scan showed cavitated apical lesions and diffuse micro-nodular infiltrates in the mid-upper lobe with some areas of atelectasis and ground-glass pattern.
In the immediate postoperative period, the patient had worsening of proprioception and impairment in left-side motor coordination, which improved later. Headache improved with surgery.
Culture of the lesional material confirmed the diagnosis of disseminated PCM (including a right adrenal nodule and lung injury) and the patient was treated with liposomal amphotericin B.
The patient responded well to amphotericin B therapy with resolution of the headache and clinical improvement. Echocardiography showed no abnormal findings. Thyroid ultrasound examination revealed a simple cyst (0.36 cm in diameter) at the junction of the isthmus and the right lobe and another simple cyst (1.31 cm in diameter) located in the lower pole of the left lobe. There were no solid nodules. Visual field testing (perimetry) performed by an ophthalmologist showed bitemporal hemianopia. The patient was clinically stable and was then discharged in good general condition.
PCM may have a broad spectrum of speed of disease spread, form, and aggressiveness, ranging from acute to chronic and from self-limited to a fatal disease.[
Involvement of the CNS is more common in the chronic form of PCM, occurring in the progressive phase of the disease, often after the third decade of life.[
The diagnosis of PCM with CNS involvement is difficult and clinical suspicion may help achieve the correct diagnosis by ordering proper diagnostic imaging studies. The most common symptoms of neurological involvement are intracranial hypertension, cranial nerve deficits, motor deficits, gait disturbances, altered consciousness, hemiparesis, headache, ataxia, and seizures.[
The regimen of choice for PCM with CNS involvement is preferably a combination of oral fluconazole and trimethoprim–sulfamethoxazole for long periods, ranging from 12 to 84 months.[
MRI with intravenous contrast injection often reveals the lesion. However, because the radiological presentation of PCM may be suggestive of neoplasia, patients are commonly referred for cancer treatment and tumor resection, thus delaying diagnosis and definitive treatment. Therefore, in the presence of neuroimaging findings that are characteristic of PCM (ring-enhancing lesion), culture should be considered to confirm the diagnosis of PCM. Whenever possible, stereotactic biopsy should be performed as a less-invasive way to obtain the tissue samples needed for histopathology.[
In conclusion, PCM should be promptly considered in the differential diagnosis of brain tumors in endemic areas when a ring-enhancing mass associated with peri-lesional edema is observed on MRI. Treatment should be introduced as early as possible, with amphotericin B and intravenous sulfamethoxazole for severe manifestations of the disease.
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