Midfacial granuloma syndrome or an inflammatory non‐specific disease? A case report

Torre, Valerio; Bucolo, Sebastiano; Galletti, Bruno; Cavallari, Vittorio

doi:10.1034/j.1600-0714.2001.300310.x

Cited by 6 publications

(6 citation statements)

References 3 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…With a variety of descriptive names such as "nonhealing granuloma, " "granuloma gangraenescens, " "malignant granuloma, " "idiopathic midline destructive disease, " and "lethal midline granuloma" (5)(6)(7)(8), it is now known as the "extra nodule natural killer NK/T cell lymphoma, nasal type (ENKTL-NT), " because natural killer cells,although on a lesser extent T cells, are often detected along with cytotoxic granule associated proteins (9)(10)(11). However, diagnosis and treatment of LMG has remained challenging; in some cases, the condition of patients worsened due to anti-cancer therapy (2,(12)(13)(14)(15)(16) in the early or later stages (17), or achieved resolution with antifungal therapy alone or in combination with other medication (18)(19)(20)(21), as what can also be obtained with rhino-orbital-cerebral mucormycosis (ROCM) (22).…”

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.

View full text Add to dashboard Cite

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.

show abstract

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.

View full text Add to dashboard Cite

show abstract

“…The clinical signs and the various paraclinical examinations cannot yet clearly establish the pathogenesis of upper airway granulomatosis. 4,5 The literature presents a great diversity of terminology to name this pathology, in addition there is also a clinical similarity between midfacial granulomatosis and other destructive midfacial lesions. 6 Midfacial granulomatosis, described as enigmatic by some authors, has several names: Robert Woods in 1921, "malignant granuloma of the nose", Stewart in 1933, "progressive lethal granulomatous ulceration of the nose", Tsokos in 1980, "idiopathic destructive disease of the midline.…”

Section: Discussionmentioning

confidence: 99%

Mediofacial malignant granuloma: a case report and review of the literature

Aliou,

Ibrahima,

Sayon

et al. 2023

JOENTR

View full text Add to dashboard Cite

We report a case of malignant midfacial granuloma or idiopathic midfacial granulomatosis in a 50-year-old male farmer with no previous history. He was admitted to the department in an emergency context. The history was reportedly 18 months old. He presented a symptomatology made of nasal obstruction, fever, headache and rhinorrhea with an altered general state. He consulted several health facilities. The evolution was marked by the aggravation of the symptomatology. The examination at admission noted an alteration of the general state. Examination of the ENT sphere revealed ulcerative-necrotic lesions with a destructive nasal origin, with bone sequestration, midface edema, and extension to the sinuses and hard palate. The CT scan revealed bony and cartilaginous lysis, irregular thickening of the nasal pyramid, continuity of the nasal septum, and isodensity of the maxillary, ethmoidal, and left sphenoidal sinus walls without total filling. Deep biopsies were performed under general anesthesia. Histology was consistent with a granulomatous reaction with progressive tissue destruction. Mycobacterium tuberculosis was absent. Medical treatment was initiated. The evolution was marked by the extension of the lesions, malnutrition and the appearance of generalized oedema. The patient died on the 45th day of his hospitalization in a septic shock situation. Conclusion: Mediofacial granulomatosis is a difficult lesion to diagnose and manage. It requires careful histological exploration in order to differentiate it from other mediofacial destructive lesions. The evolution is most often towards extension and lethal complications.

show abstract

“…The predominant risk factor for ROCM is uncontrolled diabetes mellitus, particularly for those patients suffering from diabetic ketoacidosis (1,16,19,39,40,44,46,68,(81)(82)(83)(84)(85)(86)(87)(88)(89), followed by leukemia and other hematologic malignancies (39,40,54,71,82). Other predisposing factors are malignancy, organ transplant, chronic organ failure, steroid use, burns, immune deficiency, AIDS, addiction to drugs (90,91), and latent COVID-19 infection (92).…”

Section: Risk Factorsmentioning

confidence: 99%

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

Lun

2022

Front. Med.

View full text Add to dashboard Cite

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.

show abstract

Midfacial granuloma syndrome or an inflammatory non‐specific disease? A case report

Cited by 6 publications

References 3 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

Mediofacial malignant granuloma: a case report and review of the literature

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

Contact Info

Product

Resources

About