|Year : 2016 | Volume
| Issue : 1 | Page : 79-81
Detrimental effect of mandibular mucormycosis burlesquing as osteosarcoma
Kiran Alam, Shagufta Qadri, Feroz Alam, Shakeba Quadri
Department of Pathology, Jawaharlal Nehru medical College (JNMC), Aligarh Muslim University (AMU), Aligarh, Uttar Pradesh, India
|Date of Web Publication||21-Jan-2016|
Department of Pathology, Jawaharlal Nehru Medical College (JNMC), Aligarh Muslim University (AMU), Aligarh - 202 002, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Mucormycosis is an acute, opportunistic infection caused by a saprophytic fungi typically inhabiting the soil or decaying organic matter. It is an invasive and potentially fatal infection caused by fungi of the order Mucorales, which commonly affects structures in the head and neck including air sinuses, orbits, and the brain. We describe our clinical experience of a case of mandibular mucormycosis, developing in a 45-year-old male who had a family history of diabetes. He presented with complaints of pain and foul-smelling discharge from a nonhealing, ulcerative wound at the mandibular alveolus, encompassing the area around the left canine and 1st premolar. The x-ray revealed osteolytic bony lesion with massive destruction of the mandibular ramus. A clinical impression of osteosarcoma was deduced. Surgical curettage was performed; however, the histopathological examination and subsequent treatment with periodic acid-Schiff stain aided in achieving a conclusive diagnosis of mandibular mucormycosis.
Keywords: Immunocompromised, mandible, mucormycosis, osteosarcoma
|How to cite this article:|
Alam K, Qadri S, Alam F, Quadri S. Detrimental effect of mandibular mucormycosis burlesquing as osteosarcoma. Muller J Med Sci Res 2016;7:79-81
|How to cite this URL:|
Alam K, Qadri S, Alam F, Quadri S. Detrimental effect of mandibular mucormycosis burlesquing as osteosarcoma. Muller J Med Sci Res [serial online] 2016 [cited 2020 Jun 1];7:79-81. Available from: http://www.mjmsr.net/text.asp?2016/7/1/79/174665
| Introduction|| |
Mucormycosis, also called zygomycosis, is a fulminant fungal infection that was first described by Paultauf in 1885.  It often occurs in debilitated and immunocompromised individuals or in those suffering from diabetes mellitus. Mucormycosis can manifest either as an acute and rapidly advancing disease or can even progress to a devastatingly fatal disease.
Though microbiology, clinical types and the pathology of mucormycosis are well-established but the rarity of the disease leads to difficult and delayed diagnosis, resulting in a poor prognosis. We hereby report this case to draw attention toward the clinical presentation of mucormycosis and as a reminder to clinicians to emphasize the possibility of opportunistic fungal infection in the differential diagnosis, whenever a case of prolonged nonhealing ulcer or wound is encountered.
| Case Report|| |
A 45-year-old Indian male presented with complaints of pain and foul-smelling discharge from a nonhealing, ulcerative wound in his lower jaw. On local examination, multiple lesions were found to be located on the mandibular alveolus, the larger ulcerated encompassing area around the left canine and 1st premolar. He had a strong family history of diabetes. On general clinical examination, the patient had a poor built and nutrition, was febrile (100˚F) but well-oriented. His hematological and biochemical profiles included: Total leukocyte count (TLC) of 11,000/mm 3 , hemoglobin of 10 g/dL, platelet count of 324,000/mm 3 , sodium of 138 mmol/L, potassium of 3.5 mmol/L, fasting and post prandial blood glucose levels of 225 mg/dL and 375 mg/dL, respectively, glycated hemoglobin (HbA1C) of 10.4%, and blood urea and serum creatinine of 58 mg/dL and 2 mg/dL, respectively. He was detected to be diabetic on the basis of raised blood glucose levels and HbA1C. Orthopantomograph (OPG) revealed multiple osteolytic bony lesions, with massive cortical destruction of the left mandibular ramus extending up to the body [Figure 1]. A clinical impression of osteosarcoma was suspected. Surgical curettage was performed under antibiotic coverage to reach an exact diagnosis and the tissue was sent for histopathological examination. The gross specimen comprised multiple brownish fragments of necrotic and hemorrhagic tissues with some bony chips. The entire tissue was processed and examined. On light microscopy, hematoxylin and eosin-stained sections revealed necrotic bones with ragged borders, surrounded by dense neutrophilic infiltrates, enmeshed with broad aseptate fungal hyphae [Figure 2] and spores, highlighted by subsequent treatment with periodic acid-Schiff stain [Figure 3]. The sections were thoroughly examined but evidence of malignancy was completely absent. Fungal culture was also positive for mucormycetes. Thus, combining the clinical presentation, OPG findings, histopathological results, and culture results, a conclusive diagnosis of mandibular mucormycosis was derived. Antifungal therapy was started immediately after confirmation of report, which included amphotericin B, 40 mg twice a day [bis die (BD)] intravenously for 4 weeks, in combination with cefoperazone and sulbactum 1.2 g intravenously BD for 10 days, and metronidazole 100 mL intravenously thrice daily. His diabetes was controlled with daily insulin. He was considerably stable after 1 week's therapy. The dressings were regularly changed and the wound was irrigated with hydrogen peroxide; the wound was healthy, healing day by day but the postoperative mandibular defect persisted. His recovery was smooth and he was discharged from the hospital after 1 month's stay. He was asked to revisit every week and later on once in 1 month for 6 months during which he remained asymptomatic and is doing well at present.
|Figure 1: Orthopantomograph (OPG) revealing multiple osteolytic bony lesions, with massive cortical destruction of the left mandibular ramus extending up to the body|
Click here to view
|Figure 2: Section showing the necrotic bone and neutrophilic infi ltrates with enmeshed ribbon-like aseptate hyphae (H and E ×100)|
Click here to view
| Discussion|| |
Mucormycosis amalgamates a variety of infections caused by zygomycetes, a class of fungi that produces branching ribbon-like hyphae and reproduce sexually by the formation of zygospores. Humans are affected by three genera of documented pathogens - Rhizopus, Absidia, and Mucor. These fungi belongs to the family Mucorales of class zygomycetes.  Mucorales is a subtype of zygomycetes, which produces a discrete pattern of clinical infection. The fungi are normally avirulent; they become pathogenic only when the host resistance is exceptionally low. Mucosal ulceration or postextraction wound in the mouth can act as a port of entry for mucormycosis in the maxillary or mandibular region, particularly in immunocompromised hosts. Invasive mucormycosis is characterized by the incursion of blood vessels by the organisms that leads to evolution of thrombus formation, causing rapid tissue necrosis.
It helps in determining fungal invasion into the tissue and vessel, along with the demonstration of a vivid host response against the fungus. Histopathology can also provide rapid presumptive diagnosis of the fungus while waiting for fungal culture results, or it may provide the only available material when there is no culture growth. Histopathology is even mandatory in circumstances demanding to distinguish whether an organism recovered in culture represents contamination, colonization, or true infection. Necrosis and invasion of tissue and vessels are important histopathologic characteristics that can help making this distinction.
Mucormycosis is a devastating disease menacing lives, for which medical management alone is not effective as the extensive vascular thrombosis leads to poor drug delivery to the infection site.  Therefore, initial medical management of mucormycosis should aim to treat the infection aggressively, along with elimination of the underlying predisposing factor , and incorporation of auxiliary surgical management should be early in the course of therapy. Surgery should include debridement of all infected tissues even if it compels radical resection. The ultimate outcome of mucormycosis solely depends on the prognosis of the underlying disease and the host immunity to reverse the predisposing condition.  Though amphotericin B remains an important drug treatment for mucormycosis, recent studies have proved the therapeutic efficacy of posaconazole, an extended-spectrum triazole agent against zygomycetes, that would represent a better option for patients with serious invasive fungal disease.  The overall mortality rate is expected to be 50% although higher survival rates, reaching up to 80-85% have been currently reported. 
| Conclusions|| |
- Health practitioners should be familiar with the signs and symptoms of the disease and should maintain a high level of suspicion in patients with diabetes.
- Correction of underlying predisposing factors and early diagnosis coupled with an aggressive multimodality treatment including surgical debridement remains the cornerstone therapy.
- Histopathologic examination remains an indispensable tool in defining the diagnostic significance of positive culture isolates.
Financial Support and Sponsorship
Conflicts of Interest
There are no conflicts of interest.
| References|| |
Paulltauf A. Mycosis mucorina. Virchowa Arch (A) 1885;102:543-9.
BakathirAA. Mucormycosis of the jaw after dental extractions: Two case reports.Sultan Qaboos Univ Med J2006;6:77-82.
Pandey A, Bansal V, Asthana AK, Trivedi V, Madan M, Das A. Maxillary osteomyelitis by mucormycosis: Report of four cases.Int J Infect Dis 2011;15:e66-9.
Torres HA, Hachem RY,Chemaly RF,Kontoyiannis DP,Raad II. Posaconazole: A broad-spectrum triazole antifungal. Lancet Infect Dis 2005;5:775-85.
Oswal NP, Gadre PK, Sathe P, Gadre KS.Mucormycosis of mandible with unfavorable outcome.Case Rep Dent 2012;2012:257940.
Sugar A. Agents of mucormycosis is and related species. In: Mandell GL,Bennett JE, Dolin R, editors. Mandell Douglas and Bennett′s Principles and Practice of Infectious Diseases.6 th
ed. Vol. 2. Philadelphia, Pennsylvania,USA: Elsevier, Churchill Livingstone; 2005. p. 2973-84.
González-Ramos MM,Bertrán-Pasarell J,Guiot H,Soto R,Santana J,Amador R,et al
. Conde clinical experience with posaconazole in patients with invasive mucormycosis: A case series. P R Health Sci J 2008;27:328-32.
Auluck A. Maxillary necrosis by mucormycosis: A case report and literaturereview. Med Oral Pathol Oral Cir Bucal 2007;12:E360-4.
[Figure 1], [Figure 2], [Figure 3]