PMC8208083_01
"Rhizopus arrhizus associated lethal midline granuloma/rhino-orbital cerebral mycosis." Destruction of the sinus, nose, face, palate, and orbit; defects of the left sided external nose, soft tissues, and the front wall of the maxillary sinus; and yellow pus was seen within the left maxillary sinus. (A,B) Show patient onset before and after debridement with biopsies. (C) Reveals the patches dark discoloration (short arrow), light discoloration (medium arrow), and perforation (long arrow) of the hard palate that represent inflammation, ischemia, and necrosis, respectively. (D) Shows widespread destruction in the left side of the palate (arrow). (E) Reveals pale gray wool-like mycelium grown on the necrotic nose tissue (arrow). (F) Shows complete remission with scars 4 months after antifungal therapy.
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F1
Black eschar covered the left side of the face with an underlying grayish pus (Figure 1A). The anterior wall of the left maxilla was perforated with a green-yellowish pus seen at the bottom of the maxilla (Figure 1B). The hard palate had patches of black, pale discoloration showed perforation (Figure 1C), and soon proceeded with complete destruction (Figure 1D). Pale gray, wool-like mycelium was observed on the necrotic tissue of the nose (Figure 1E).
"Rhizopus arrhizus associated lethal midline granuloma/rhino-orbital cerebral mycosis." Pathologic characteristics of the midline face destruction patient, the fungus and hyperplasia replicated in the mouse models. (A) Shows the pathological characteristics in the granulomatous area with immunohistochemically positive staining of CD2, CD3, CD8, GranzymeB, and Ki-67 from granulomatous tissues (Envision method, original magnification x400) and angioinvasion (H&E, original magnification x400). (B)
Rhizopus arrhizus (PUTH 20111112) isolated from tissue: lanose appearance with grayish color 5 days growth on PDA and thin-walled hyphae (arrows) and conidia (lactophenol cotton blue staining, original magnification x400). (C) Fungal thromboses (long arrow), angiodestruction and angiocentric coagulative necrosis (short arrows, H&E, original magnification x200 and x400, respectively). (D) Atypical cells (short arrows) and hyphae (long arrows) among Onion-skin lesions (H&E staining, original magnification x400). (E) Atypical lymphoid cells (short arrows) and hyphae (long arrows) (H&E staining, original magnification x400 and x1,000, respectively). (F) Ki-67 positive cells and hyphae with immunochemical staining in mouse infected by Rhizopus arrhizus (PUTH 20111112) (Envision method, original magnification x400) hyperplasia with H&E staining (original magnification x400).
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F2
Angioinvasive, angiocentric, and angiodestructive characteristics were typical with "Onion-skin" lesions (Figure 2A). In immunohistochemistry, the atypical lymphoid cells were positive for NK/T cell markers (CD2+, CD3+, CD8+, and individual CD56+), cytotoxic granule associated proteins (GZMB and TIA1), and hyperplasia (Ki67+):typical of ENKTL (Figure 2).