case_images: PMC8018373_01
case_images: MRI presentation of the tumor. MRI revealed a solid mass involved the nasal cavity and sinuses, destroying bone tissue.
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case_images: T1 and long T2 soft tissue signal shadows in the right nasal cavity, which were significantly and nonuniformly enhanced on contrast-enhanced imaging. The lesions invaded the sphenoid sinus and the right ethmoid sinus upward, invading the posterior nostril, the right orbital lateral wall, and the right maxillary sinus (Figure 1).
case_images: (A) The cells are arranged in microcystic or reticular pattern a in a myxoid stroma. (B) The tumor displays a papillary pattern in focal area. (C) The characteristic endodermal sinuses or perivascular formations (Schiller-Duval bodies) are also present in the tumor. (D) Eosinophilic basement membrane material is frequently observed inside and outside the microcysts. (E) The numerous round hyaline globules can be seen inside the cells. (F) The microcysts are lined by cuboidal or polygonal cells with lightly stained cytoplasm and mild to moderate atypia.
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case_images: The lining cells demonstrated cuboidal or polygonal shapes, a lightly stained cytoplasm, and mild-to-moderate atypia (Figure 2).
case_images: (A) The tumor cells are strongly positive for CK. (B) GPC-3 is strongly expressed in cytoplasm. (C) CDX-2 is diffusely expressed in the nucleus. (D) SALL4 is diffusely expressed expression in the nucleus. (E) Focal expression of AFP is present in tumor cells. (F) Ki-67 proliferative index is approximately 60%.
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case_images: The Ki-67 proliferative index was approximately 60% (Figure 3).
case_images: (A) All tumors exhibited complete loss of SMARCB1 immunostaining, with stromal and inflammatory cells serving as an internal positive control. (B) Fish detection also showed a homogeneous deletion of SMARCB1.
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case_images: Additional immunohistochemistry revealed absence of INI-1 expression (Figure 4A). FISH using a SMACRB1 missing probe (LBP, China) showed that 1R2G accounted for 3% and 2G accounted for 75% of all cells, indicating the deficiency of SMACRB1 (Figure 4B).