Invasive sphenoid sinus aspergillosis mimicking a cerebello-pontine angle mass lesion


Invasive sphenoid sinus aspergillosis is a rare clinical entity in immunocompetent patients. It is often misdiagnosed because of its diverse clinical presentation and lack of suspicion. Timely diagnosis and early treatment of such cases may improve the outcome of this potentially fatal condition. We hereby present a rare occurrence of invasive sphenoid sinus aspergillosis in an immunocompetent host, presenting as a cerebello-pontine angle mass lesion.

Keywords: Aspergillosis, cerebello-pontine angle, paranasal sinuses, sphenoid sinus aspergillosis

How to cite this article:
Chaudhary SC, Sawlani KK, Malhotra HS, Yathish B E, Verma SK. Invasive sphenoid sinus aspergillosis mimicking a cerebello-pontine angle mass lesion. Ann Trop Med Public Health 2014;7:234-7
How to cite this URL:
Chaudhary SC, Sawlani KK, Malhotra HS, Yathish B E, Verma SK. Invasive sphenoid sinus aspergillosis mimicking a cerebello-pontine angle mass lesion. Ann Trop Med Public Health [serial online] 2014 [cited 2020 Sep 22];7:234-7. Available from:

Aspergillosis is the most common fungal infection of the paranasal sinuses and is mostly seen in immunocompromised patients. [1] The central location of the sphenoid sinus in the skull base provides multiple pathways for intracranial spread to critical regions of the central nervous system including access to the cerebral vasculature. The inflammatory affliction of the sphenoid sinus most frequently manifests as headache (50-98%) and retro orbital pain; however, a few reports of invasive sphenoid aspergillosis with uncommon presentations exist. [2] A case with painless loss of vision, ptosis and ophthalmoplegia of the right eye has been described, along with a presentation akin to a pituitary mass. [3],[4] We hereby report a case presenting clinically as a cerebello-pontine angle mass in an immunocompetent gentleman who was later diagnosed as having invasive sphenoid sinus aspergillosis on the basis of radiology and histopathological findings. To the best of our knowledge, this is probably the first case report of invasive sphenoid sinus aspergillosis mimicking a cerebello-pontine angle mass lesion.

Case Report

A 20-year-old gentleman was referred to our university, a tertiary care facility, in a state of deep coma (E 1 M 1 V 1 ; impaired oculocephalic response) after having sustained multiple episodes of generalized tonic-clonic seizures. Past history and previous clinical notes (of consultations done elsewhere) were suggestive of symptoms of headache in the fronto-temporal region, numbness involving the right side of the face, redness of the right eye, impaired hearing from the right ear, and double vision for 2-3 months. Clinical examination had shown involvement of both the motor as well as the sensory components of all the three divisions of right trigeminal nerve, right abducens palsy, and right sensorineural deafness. There was no history suggestive of diabetes mellitus or any other immunocompromised state.


Investigations revealed hemoglobin −12.8 g/dL, total leukocyte count-11,610/mm 3 with polymorphs −85%, lymphocytes −15% and platelets −119,000/mm 3 . The fasting blood sugar was 98.2 mg/dL while the postprandial sugar was measured at 124.8 mg/dL; other biochemical parameters were as follows: blood urea −47.3 mg/dL, serum creatinine −0.9 mg/dL, serum sodium −135 mEq/L, serum potassium −4 mEq/L, serum calcium (total −9.6 and ionic −5.2 mg/dL). HIV I and II was tested negative by ELISA on two occasions. Magnetic resonance imaging (MRI) of the brain revealed a homogeneously enhancing mass filling the sphenoid sinuses and extending posteriorly into the right cerebello-pontine angle cistern, traversing the right parasellar region and the Meckle’s cave. Anterolateral extension could be traced to the right inferior orbital fissure and the right medial temporal lobe. The well-defined homogenously enhancing extra-axial mass (54 mm × 51 mm × 36 mm) in the right cerebello-pontine angle cistern showed mass effect in the form of compression of the lower midbrain and the right middle cerebellar peduncle [Figure 1]a-f and [Figure 2]a-d. Cerebrospinal fluid (CSF) examination showed a total leucocyte count of 410/mm 3 (polymorphs −60%, lymphocytes −40%), protein −90 mg/dL, and sugar −46 mg/dL (corresponding blood sugar −102.4 mg/dL); India ink preparation, cryptococcal latex antigen test and polymerase chain reaction for mycobacterium tuberculosis were negative.

Figure 1: Axial magnetic resonance imaging of the brain depicts a homogeneous lesion involving the sphenoid sinus, and extending posteriorly into the right cerebello-pontine angle cistern, and anterolaterally to the right inferior orbital fi ssure and the right medial temporal lobe. The lesion (arrow heads) is iso-to hypointense on T1-weighted (a and b) and hypointense on fl uid attenuation inversion recovery (c and d) and T2-weighted images (e and f), with suggestion of mass effect. Erosion of the supero-lateral wall of the sphenoid sinus (arrow heads) is evident in the bonewindow images of computed tomography of the paranasal sinuses (g and h)

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Figure 2: T1-weighted gadolinium contrast images of the brain show a homogeneously enhancing mass lesion with a better delineated extent (arrow heads), as compared to the noncontrast sequences, on the axial (a and b) and sagittal (c and d) images

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In view of the clinical picture and radiology, an otorhinolaryngologist’s opinion was sought, and computed tomography (CT) of the paranasal sinuses was done to assess the extension of the lesion through the bone. CT showed a left maxillary polyp, right maxillary sinusitis, and sphenoid sinusitis with erosion of supero-lateral wall of right sphenoid sinus with extension into the region of orbital apex and epidural space overlying right temporal lobe [Figure 1]g and h. Endoscopic biopsy of the lesion from the sphenoid sinus showed periodic acid-Schiff positive uniform branching septate hyphae suggestive of Aspergillus species.


Patient was initially managed conservatively with intravenous (IV) anti-epileptics (levetiracetam 750 mg 12 hourly), empirical antibiotics and fluids; after the biopsy report, injectable voriconazole was started at 6 mg/kg body weight 12 hourly (day 1), followed by 4 mg/kg body weight 12 hourly.

Outcome and follow-up

Barring an initial period of improvement lasting about a day, the patient had a persistently downhill course despite specific therapy. He developed refractory status epilepticus, leading to aspiration pneumonia and subsequently respiratory failure. He was intubated and put on mechanical ventilation, but he succumbed to his illness on the 4 th day of admission.

Aspergillosis is the most common fungal infection of the paranasal sinuses and is mostly seen in immunocompromised patients. The primary host immunodeficiencies that are responsible for the increased risk of invasive aspergillosis (IA) are neutropenia and corticosteroid-induced immunosuppression. Neutrophil and/or phagocyte dysfunction is also an important risk factor, as evidenced by aspergillosis in chronic granulomatous disease, advanced HIV infection, and relapsed leukemia. In neutropenic patients, IA is characterized by thrombosis and hemorrhage from rapid and extensive hyphal growth. The lack of inflammatory infiltrates, despite the production of tumor necrosis factor alpha, results in low levels of inflammation. A variety of nonneutropenic patients, most commonly those on corticosteroid therapy are susceptible to IA, although the pathology of the disease is quite different. IA in these patients is nonangioinvasive, characterized by limited fungal development with pyogranulomatous infiltrates, tissue necrosis, and excessive inflammation. Aspergillus fumigatus is the most common etiological agent of human aspergillosis. Despite the breadth of studies of Aspergillus pathogenesis, there are few well-defined factors that contribute to A. fumigatus-related IA. Perhaps most important are the basic biological characteristics of this species. Small, easily airborne conidia access the lower respiratory tract, and A. fumigatus grows well at 37°C, with germination rates exceeding those of other species. In addition to these traits, A. fumigatus adapts to environmental conditions imposed within the mammalian lung. In immunosuppressed individuals at risk for IA, conidia colonize injured lung tissue or lung epithelia, evade macrophage killing, and degrade surrounding tissue to obtain or synthesize nutrients necessary for growth. Depending on the underlying host immune status, A. fumigatus may grow uncontrolled and disseminate (neutropenia) or be controlled by neutrophils and result in excessive inflammation (steroid-induced immunosuppression). [5]

In the recent past, fungal diseases of the nose and paranasal sinuses have been reported with increasing frequency. Aspergillus species are by far the most common invaders of the paranasal sinuses and the maxillary antrum represents the site most commonly infected. Two main categories of fungal sinusitis presently recognized are invasive and noninvasive. The invasive form, also called mucosal or penetrating sinusitis, is further classified into two subtypes, the chronic (or: Indolent) and the acute (or: Fulminant) fungal sinusitis. The latter is mainly found in immunocompromised neutropenic individuals and requires rapid diagnosis and aggressive treatment, representing a medical emergency. In contrast, the noninvasive or extramucosal form is benign in nature, and also consists of two distinct subtypes: Mycetoma and allergic fungal sinusitis, previously mentioned as allergic Aspergillus sinusitis. Except for the fulminant variant, the other types are encountered in apparently healthy individuals. The relationship of the sphenoid sinus to important adjacent structures accounts for the neurological symptoms and signs observed in the cases of sphenoid infection. Diagnosis is confirmed by surgery and histopathology, the latter revealing characteristic uniform branching septate hyphae. Differential diagnosis includes bacterial infections, granulomatous inflammations and generally, all sorts of sinus occupying masses – such as polyps, mucocele and pyocele, aneurysms, primary sphenoid tumors (e.g., squamous cell carcinoma), secondary sphenoid invaders (craniopharyngioma, nasopharyngeal angiofibroma, et cetera) and metastatic tumors. It is commonly accepted that prolonged antibiotic therapy – for any reason may alter the normal flora of the sinuses, predisposing to fungal growth. [6]

The cerebello-pontine angle cistern is a CSF filled triangular space bounded by the petrous temporal bone laterally, with the pons and the cerebellum (plus cerebellar peduncle) forming its medial limits. The usual lesions, which occur at this level are neuromas (acoustic or trigeminal) or meningiomas, but invasive sphenoid sinus aspergillosis presenting clinically as cerebello-pontine angle mass lesion does not figure in the literature. The presence of headache and the rapidity of symptom evolution is atypical of the commonly observed slow growing lesions at the cerebello-pontine angle viz., neuromas, and meningiomas. Another aspect is the origin and the extent of involvement in our case, which points toward an alternate diagnosis, either an inflammatory or a rapidly growing malignant lesion. A delay in the diagnosis and treatment in such cases may lead to life threatening complications like skull base osteomyelitis, meningitis, meningoencephalitis, brain abscess, cavernous sinus thrombosis, infarcts, and rarely, fungal aneurysms. [7],[8],[9]

The presence of focal neurological deficits and a rapid march of events should have prompted an earlier imaging, in this case. MRI with gadolinium contrast should be obtained in all the patients suspected of similar parenchymal afflictions in order to avoid delays in diagnosis and initiating treatment. The utility of a contrast-enhanced CT lies in demonstrating the breach in osseous anatomical boundaries, if any, which may aid the treating surgeon in planning the approach to the involved site and the level of intervention required. Thus, one should have a high index of suspicion and consider sphenoid sinus aspergillosis in appropriate patients presenting clinically as a cerebello-pontine angle mass lesion to facilitate early management and avert potential mortality.

In situations in which moderate or high risk is predicted (e.g., after induction therapy for acute myeloid leukemia), the need for antifungal prophylaxis for superficial and systemic candidiasis and for IA is generally accepted. Fluconazole is commonly used in these situations but has no activity against Aspergillus spp. Itraconazole capsules are ineffective, and itraconazole solution offers only modest efficacy. Posaconazole solution is more effective. Some data support the use of IV micafungin. No prophylactic regimen is completely successful. [10]

Learning points

  • Invasive sphenoid sinus aspergillosis is rare in immunocompetent hosts and has a diverse clinical presentation.
  • High index of suspicion should be kept in patients where involvement of the paranasal sinuses is occurring with suggestion of invasiveness, either intra-axial or extra-axial.
  • A rapid course and presence of focal neurological deficits should prompt a contrast neuroimaging, preferably MRI, to assess the site, nature and extent of the lesion.
  • Early diagnosis and treatment are the keys to prevent life-threatening complications of IA.
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Denning DW. Aspergillosis. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson L, Loscalzo J, editors. Harrison’s Principles of Internal Medicine. 18 th ed. New York: McGraw-Hill, McGraw-Hill Medical Publishing Division; 2012. p. 1655-60.

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1755-6783.151779


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