Mucormycosis of Maxillary Sinus in Immunocompetent Patient Masquerading as Neoplasm: A Case Report
Mucormycosis is a common fungal infection usually noted in immunocompromised individuals like diabetic and AIDS patients, patients receiving systemic corticosteroid therapy, cancer chemotherapy and organ transplant patients. It is very rare to affect healthy individual. Here we report a case of 50 years old immunocompetent female presented with cheek swelling with past history of nasal polypectomy. Clinically and radiologically diagnosis of maxillary carcinoma was made. After maxillary debridement surgery diagnosis of maxillary necrosis with mucormycosis was confirmed.
Keywords: Diabetic; Fungi; immunocompromised; Immunocompetent; Mucormycosis; Necrosis; Neoplasm.
Mucor is a saprophytic fungus of class Phycomycetes (Zygomycetes), order Mucorales, family Mucoraceae.1-5 Mucormycosis is an opportunistic fulminant fungal infection. Mucormycosis was first described by Paultauf in 1885.5 Mucor is found worldwide in decaying vegetations and soil. The spores in the air frequently expose humans. Mucormycosis is one of the most rapidly progressive lethal infection in human being having mortality of 70 to 100%.6 The infection manifests as rhinocerebral, pulmonary, gastrointestinal, cutaneous or disseminated form.1 The predisposing factors are uncontrolled diabetes, renal failure, tuberculosis, organ transplant, long term corticosteroids, immunosuppressive therapy, cirrhosis, burns, protein energy malnutrition, leukemias, lymphomas and AIDS.1 But it can rarely infect healthy individuals and may create a diagnostic as well as therapeutic dilemma for those who are not familiar with mucormycosis.7,8 The purpose of this case report is to draw attention to the clinical presentation and pathogenesis of mucormycosis in a immunocompetent patient.
A 50 years old female patient reported for evaluation of left cheek swelling since five months. There was history of nasal bleeding. Rhinoscopy showed evidence of deviated nasal septum to right and a polyp in the left nostril of size 1x1cm. General examinations showed evidence of swelling over left cheek which was diffuse, soft to firm, non-tender, non-erythematous, no discharge and normal local temperature. There was deviation of angle of mouth of left side (Fig 1a). Oral examination revealed normal lips, gums, teeth, tongue, tonsils, anterior and posterior pillars and palate. There was history of left molar toothache and patient had taken treatment for that. Pain had subsided but swelling had remained. Patient was a known case of hypertension and taking treatment regularly since two years.
Complete blood count showed combined deficiency anemia, normal white blood cell and platelet counts. Patient was negative for HBsAg, HCV, HIV. Fasting and post-prandial sugar were within normal limits. Blood urea, serum electrolytes, liver function test, renal function test were within normal limits. First polypectomy was done. Histopathology showed morphology of inflammatory (allergic) polyp. But swelling still persisted. The computed tomography revealed soft tissue lesion of approximate size 5.6x5.6x5.4cm in left maxillary sinus causing erosion and destruction of bony wall of maxillary sinus, adjacent osteomeatal complex and extending within the ipsilateral orbit, anterior ethmoidal air cells and soft tissues of left cheek (Fig 1b). Thus the diagnosis suggestive of carcinoma of maxilla was made. Caldwell Luc operation was performed and material was sent for histopathological examination. It showed only granulomatous reaction. Further maxillary debridement surgery was done and all material including deeper tissue was sent for histopathological study. Multiple sections were studied but there was no evidence of malignancy. The Hematoxylin and Eosin staining showed necrotic tissue along with non-septate fungal hyphae with right angled branching that resembled mucormycosis (Fig 1c). Periodic Acid Schiff staining revealed PAS positive hyphae (Fig d1). Silver stain also confirmed the diagnosis (Fig d2). Thus histopathologically it was (fungal infection) mucormycosis with maxillary sinus necrosis. Patient was treated accordingly and showed good improvement.
Figure 1: The clinical photograph showing swelling of left cheek and deviation of angle of mouth towards the ipsilateral side (a). The computed tomography shows soft tissue lesion in the left maxillary sinus causing erosion and destruction of bony wall and extending into orbit, anterior ethmoidal sinus and soft tissue of left cheek (b). The photomicrograph of hematoxylin and eosin stained sections shows areas of necrosis along with colonies of aseptate fungus (Inset), long, broad, slender, right angled branching hyphae characteristic of mucormycosis (c). The photomicrograph of periodic acid Schiff stained section shows positive hyphae (d1) and Grocott methenamine silver stained section showing brown-black hyphae (d2) of mucormycosis.
Mucor is a saprophytic fungus of class Phycomycetes (Zygomycetes), order Mucorales, family Mucoraceae. They have broad non-septate hyphae. Branches arise haphazardly often at right angles to parent hyphae.1-4,9 The hyphae and their branches are distributed randomly in the lesion rather than aligned in similar direction as seen with aspergilli.4 These opportunistic pathogen exists in the environment, air, soil, food and animal excreta. Under aerobic condition the temperature required for their growth is 28-300C.10 The incubation period was two to five days and it starts by inhalation or inoculation of spores into the damaged skin, followed by dissemination in immunocompromised host. Mucormycosis rarely affects immunocompetent patients.10 Nose and maxillary sinus is the predominant source of infection of respiratory tract in immunocompetent host.11 If sporangiospores are greater than 10μm it remains localized to upper airways giving rise to sinosal or rhinoform. If less than 10 μm the sporangiospores colonize in the distal alveolar spaces giving rise to pulmonary form of mucormycosis.11
Mucosal and cutaneous epithelium and endothelium acts as a barrier to tissue invasion and angioinvasion. Therefore invasive fungal infection in immunocompetent patient is rare. Mucor infection in such patients may be due to ability of mucor sporangiospores to attack epithelium previously damaged by infection, direct trauma or due to toxins or proteases secreted by sporangiospores which may directly destroy endothelial cells.11
Maxillary necrosis is very rare because of rich vascularity. The infectious etiology of maxillary necrosis may be bacterial, viral or fungal infections like mucormycosis, aspergillosis etc. The mucor invades the blood vessels.1 The hyphae forms thrombi in blood vessels and causes decrease in blood supply leading to necrosis of hard and soft tissue.1,2 In the maxillary antrum the sinus fills with microbial colonies and necrotic tissue with rapidly evolving destruction of the osseous antral wall. Large foci of necrosis can evolve in the palate with osseous destructive oral-antral communication and bony sequestration. Teeth loosen and spontaneously exfoliates.12
The review of literature from 35 different countries showed total 212 immunocompetent patients in the world affected by mucormycosis. India was the most affected with 94 patients followed by USA with 42 patients. India was the most affected country may be due to climatic conditions, socio-economic status, poor hygiene and delay in diagnosis. In India significant proportion of population is below the line of poverty and is therefore malnourished which may predispose them to easily contract an invasive fungal infection. Diabetes Mellitus is a well-known predisposing factor and is more common in India. Many cases of invasive fungal infection in India showed undiagnosed Diabetes Mellitus.11 The most common form was cutaneous with 90 cases, followed by rhino-orbito-cerebral with 81 cases, 18 genito-urinary cases, 10 disseminated cases, seven pulmonary cases, five gastrointestinal and one vascular case.11 However in India it was found that rhino-orbito-cerebral type (44.2%) was the commonest followed by cutaneous (15.5%) and renal (14.0%) involvement in the retrospective analysis for ten years by Chakrabarti et al.13 Equal sex distribution is seen in all forms of mucormycosis except pulmonary which shows male preponderance (Male:Female = 3:1).11
Thus mucormycosis with maxillary sinus necrosis in an immunocompetent patient is very rare. Clinically differential diagnosis for cheek swelling with maxillary sinus necrosis are squamous cell carcinoma of maxillary sinus, malignant salivary gland tumor arising from accessory glands of palate, extranodal NK-T cell lymphoma and wegner’s granulomatosis. It clinically mimics malignancy and creates dilemma in clinicians mind. But histopathological study confirms the diagnosis and helps in proper management of the patient.1
Mucormycosis with maxillary sinus necrosis in a healthy individual is very rare. As this lesion clinically may be misdiagnosed as malignancy, histopathological evaluation plays a crucial role in definitive diagnosis to avoid delay in treatment.
We would like to thank all the staff members from the department of Oral Pathology for their guidance and support.
1.Dr.Jayashri Anand Khiste, Assistant Professor, 2.Dr.Saroj Ashok Bolde, Associate Professor, 3.Dr.Gopal Ambadas Pandit, Professor and Head, 4.Dr.Naveed Abdul Sattar Tamboli, Junior Resident, Department of Pathology, Dr.V.M.Government Medical College, Solapur, Maharashtra.
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Dr. Jayashri Anand Khiste
Dr.V.M.Government Medical College, Solapur – 413003, Maharashtra State, India.
Ph: +91 9422459727
Source of Support: Nil, Conflict of Interest: None Declared.
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