Mucormycosis is a rare, opportunistic fungal infection led by fungal agents within the order Mucorales1; occurs almost solely in immunocompromised hosts, such as patients with diabetes mellitus (which is a major predisposing factor), burns, malnutrition, leukemia, lymphoma, septicemia, renal disease, and following long-term treatment with steroids and antibiotics; and is associated with high mortality rates1–3.
Nevertheless, 19% of patients have no underlying condition at the time of infection. Patients with human immunodeficiency virus (HIV) infection represent only 2% of those with zygomycosis4. Based on autopsy data, the prevalence is 1-5 cases per 10,000 individuals with hematological neoplasms, which is far less prevalent than invasive Candida or Aspergillus infections4.
A 23-year-old woman presented with a 1-month history of diffuse abdominal pain, which was predominantly epigastric, in addition to abdominal distension, vomiting, and fever. The patient reported normal feeding. Also, refers previous tuberculosis, with complete treatment; no other previous diseases; and no alcoholism, smoking, or illicit drug use. Physical examination showed a tender abdomen, painful to superficial and deep palpation, with no other notable signs. A complementary examination revealed the following:
- Hemoglobin: 11.0g/dL
- Hematocrit: 30.9%
- Platelets: 336,000/mm3
- Creatinine: 0.6mg/dL
- Urea: 26mg/dL
- Leukocytes: 3,695/mm3
- C-reactive protein: 11.25mg/dL
- Sodium: 141mEq/L
- Potassium: 3.7mEq/L
- Serology for HIV: negative
In the following investigation, contrast-enhanced radiography revealed a hypotonic stomach with excess of fluid retention and difficulty in emptying (Figure 1). Computed tomography (CT) with oral contrast showed diffuse gastric distension associated with diffuse parietal thickening, and heterogeneous impregnation by contrast, identifying the gastric antrum lumen containing liquid contents (Figure 1 and Figure 2). The stomach exhibited inferior displacement of the intestine and transverse colon loops (Figure 2).
Upper gastrointestinal endoscopy revealed enanthematic gastritis of slight intensity. Based on the anatomopathological evaluation, the gastric segment measured 25.0×12.0×6.0cm and was smooth, serous, gleaming, and brownish in color. The mucosa was brownish in color with pre-clearing and a firm consistency, with thickened regions up to 5.0cm. The biopsy demonstrated chronic granulomatous and suppurative diffuse gastritis, associated with marked fibrosis of the gastric wall, presenting voluminous zygomycosis hyphae (Figure 3).
The patient underwent total gastrectomy, with lymphadenectomy and esophagoenteroanastomosis, and treatment containing amphotericin B. Her clinical evolution during 6 weeks of treatment was positive, and she was discharged after being oriented to the outpatient follow-up. The patient received 2.8 grams of amphotericin B throughout the treatment period.
The most frequent forms of mucormycosis presentation are sinus (39%), pulmonary (24%), cutaneous (19%), cerebral (9%), gastrointestinal (7%), disseminated (3%), and kidney (2%)1,4. Among the several forms, gastrointestinal mucormycosis is rare, and the manifestations vary from the colonization of peptic ulcers to infiltrative disease with vascular invasion and dissemination2.
In gastrointestinal involvement, the most frequently compromised organ is the stomach (58%), followed by the colon (32%), small intestine, and esophagus1–3,5. The incidence is increasing, and the diagnosis carries a significant mortality rate of up to 85% due to perforation and massive bleeding1,2,4.
The symptomatology of gastrointestinal mucormycosis varies from fever, nausea, non-specific abdominal pain, and vomiting to hematemesis, melena, hematochezia, or gastrointestinal perforation1. The diagnosis is frequently confirmed histopathologically based on biopsy of the suspected area during surgery or endoscopy1. CT can be instrumental in identifying multi-organ involvement.6 The diagnosis of gastrointestinal mucormycosis can be established based on endoscopic biopsy of the lesions, which show characteristic hyphae6.
Successful management of mucormycosis includes aggressive metabolic support, antifungal therapy with amphotericin B or posaconazole, and surgical debridement of all necrosis-involved tissue2,3; the length of treatment is individualized, but is often 4-6 weeks long. There remains a need to achieve resolution of symptoms and confirm radiologic findings and negative cultures of the affected site6.