Mucormycosis or extranodal natural killer/T cell lymphoma, similar symptoms but different diagnosis

Zhang, Y.; Wang, T.; Liu, G.-L.; Li, J.; Gao, Sujun; Wan, Ling

doi:10.1016/j.mycmed.2016.04.005

Cited by 3 publications

(8 citation statements)

References 18 publications

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“…In another paper, two ROCM cases were reported with NK/T cells and angiocentric characteristics. Diagnosis for one patient was changed from ENKTL-NT to ROCM as fungal mycelium was seen in the tissue, while the diagnosis for the other was from ROCM to NK/T cell lymphoma because the NK/T cells were seen in the tissue ( 27 ). These cases suggest the ambiguity in understanding the role of fungi in the development of midline ulcers ( 27 , 28 ).…”

Section: Introductionmentioning

confidence: 99%

Case Report: Rhizopus arrhizus Rhino-Orbital-Cerebral Mycosis and Lethal Midline Granuloma: Another Fungal Etiological Agent

Lun

et al. 2021

Front. Med.

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Objective: Both rhino-orbital-cerebral mycosis and lethal midline granuloma (LMG) may result in midline destruction. LMG has now been generally considered as a natural killer/T cell lymphoma, nasal type (ENKTL-NT) with an association of EBV. Fungi have been detected from the diseased tissues now and then but are often considered as lymphoma-associated infections. We previously reported an ENKTL-NT case with Mucor irregularis, which played a causal role in the disease and was involved in the overexpression of Ki67 and CD56 in the mouse experiment. The present study describes a chronic Rhizopus arrhizus infection with immunological parameters that are closely similar to LMG. We aim to explore the relationship of another Mucorales fungus, R. arrhizus, and LMG in a patient and in mice.Methods: Case study and mouse infection modules were designed for our observation. A 35-year-old man with midline face ulcers which was clinically suspected as LMG was selected. Biopsy specimens were sent for lymphoma diagnosis and microbiological detection. The isolated fungus was tested in an ICR mouse model for mycological and histological analyses.Results: Five tissue samples yielded Rhizopus arrhizus. In the pathology, characteristic inflammation, necrosis, and granulation with thin-walled hyphae are observed. Immunohistochemistry showed NK/T cell infiltration (CD3+, CD8+, TIA1+, GZMB+, PRF+, individual CD56+) with hyperplasia (Ki67+) and angioinvasion. The patient recovered completely with amphotericin B. In the murine experiment, R. arrhizus caused angioinvasion with NK/T cell infiltration (CD3+, CD56+, TIA1+, GZMB +, PRF+) with proliferation (Ki67+) and was re-isolated from the infected host.Conclusions: We here describe a mid-face destruction patient, which was diagnosed by the top pathologists in China according to the current criteria of NK/T cell lymphoma, with a negative result for EBV and positive result for R. arrhizus. With a then developed mouse experiment, the R. arrhizus in the diseased lesions was responsible for the NK/T cell infiltration (CD3+, CD8+, CD56+, TIA1+, GZMB+, PRF+), proliferation (Ki67+), and angioinvasion, suggesting another fungal etiological agent for LMG, which could be eradicated with amphotericin B.Limitations: The sample size is not sufficient for statistical analysis. However, our findings are suggestive for the role fungus plays in LMG.

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Section: Introductionmentioning

confidence: 99%

Case Report: Rhizopus arrhizus Rhino-Orbital-Cerebral Mycosis and Lethal Midline Granuloma: Another Fungal Etiological Agent

Lun

et al. 2021

Front. Med.

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“…Interestingly, Mucoralean fungi are frequently seen or isolated in ENKTL-NT cases (2,16,21,22,77,78). In some patients, the detection of NK cells or/and T cells in the tissue led to the change of diagnosis from ROCM to ENKTL-NT (1,25), or from ENKTL-NT to ROCM when fungal elements were observed (21), or ENKTL-NT patients died after chemotherapy due to the detection of Mucorales fungal infection on a post-mortem of the nasal cavity (23).…”

Section: Fungi and Enktl-ntmentioning

confidence: 99%

“…Diagnosis for one patient was from ENKTL-NT to ROCM, as fungal mycelium was seen and recovered from tissue, while the diagnosis for the other was from ROCM to NK/T-cell lymphoma because the NK/T cells were seen in the tissue. All these findings suggest an ambiguous understanding of the role of fungi in the development of ENKTL-NT (16,21).…”

Section: Introductionmentioning

confidence: 98%

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Rhino-Orbital-Cerebral Mycosis and Extranodal Natural Killer or/and T-Cell Lymphoma, Nasal Type

Lun

2022

Front. Med.

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BackgroundExtranodal natural killer/T-cell lymphoma, nasal type is a syndrome of middle face destruction with an association to Epstein-Barr virus. Fungi have been recovered from the diseased tissue now and then but were often seen as a lymphoma-associated secondary infection. However, there are ENKTL-NT cases with the recoveries of fungi and complete recovery with antifungal therapy, which are quite similar to rhino-orbital-cerebral mycosis (ROCM) that often confuses the physicians.MethodsWe searched Medline for English-language manuscripts limited to “human” and “case reports,” “letters,” “reviews,” and “clinical conferences” from 1966 to 2022. We used MeSH terms “lymphoma, extranodal nk-t-cell” [MeSH Terms] or “lethal midline granuloma” [MeSH Terms], in combination with MeSH terms “microbiology” [subheading] or “microbiology” [all fields] or “fungi” [all fields] or “fungi” [MeSH Terms] for ENKTL-NT with infections. We used MeSH terms “Mycoses” in combination with “Nose” [Mesh] OR “Orbital Diseases” [Mesh] for rhino-orbital-cerebral fungal infections.ResultsWe appraised 149 included articles and extracted references related to ENKTL-NT and/or ROCM. Themes and subcategories were subsequently derived. Our findings revealed that ROCM and ENKTL-NT are characterized by progressive and destructive ulcers in the midline face or rhino-orbital structures. ROCM is mainly caused by fungi in the order of Mucorales, and ENKTL-NT is usually associated with Epstein-Barr virus and sometimes fungi. Radiologically, both are characterized by non-specific features of sinusitis, soft tissue infection, and necrosis. Pathologically, ROCM and ENKTL-NT share the same characteristics of inflammation, necrosis, and granuloma. ROCM is characterized by the detection of fungi in tissue, while ENKTL-NT is typically positive for NK/T-cell markers and cytotoxic granule-associated proteins, proliferation, and vascular damage of angioinvasion, which could be incited by Mucor irregularis and Rhizopus arrhizus in patients and mice.ConclusionENKTL-NT and ROCM share many similarities in clinical presentations, radiology, and histopathology, and might have the same etiology. This may explain why the two diseases are tangled together in the reported cases, and suggests the role that the fungi may play in the development of these ENKTL-NT/ROCM diseases. The reason why ENKTL-NT and ROCM are sometimes confused is that the main pathogens of ROCM, Mucor irregularis and Rhizopus arrhizus, are the fungal causative agents of ENKTL-NT.

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“…The survival rate ranges between 35% and 85% depending on the severity of symptoms and the local extension. Different case series show a high local recurrence rate of 50% (5) and metastasis in only 13% of patients in skin, lungs and gastrointestinal tract, which represents the order of frequency and worst prognosis (6,7). A correct diagnosis is important to define the type of treatment, which may include surgical procedures, chemotherapy or radiotherapy (8).…”

Section: Introductionmentioning

confidence: 99%

Accurate Diagnose and management of advanced nasal type extranodal NK/T cell lymphoma. A case Report.

Moreno¹,

Parra-Charris²,

Ángel-Obando³

et al. 2017

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Mucormycosis or extranodal natural killer/T cell lymphoma, similar symptoms but different diagnosis

Cited by 3 publications

References 18 publications

Case Report: Rhizopus arrhizus Rhino-Orbital-Cerebral Mycosis and Lethal Midline Granuloma: Another Fungal Etiological Agent

Case Report: Rhizopus arrhizus Rhino-Orbital-Cerebral Mycosis and Lethal Midline Granuloma: Another Fungal Etiological Agent

Rhino-Orbital-Cerebral Mycosis and Extranodal Natural Killer or/and T-Cell Lymphoma, Nasal Type

Accurate Diagnose and management of advanced nasal type extranodal NK/T cell lymphoma. A case Report.

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