Bruett Carter T, Freedman Paul D, Reich Renee F
New York Presbyterian/Queens, Flushing, Queens, New York, USA.
, 56-31 141st Street, Flushing, NY, USA.
Head Neck Pathol. 2024 Feb 9;18(1):4. doi: 10.1007/s12105-023-01607-0.
Dysgenetic polycystic disease, also known just as polycystic disease, is a very rare developmental abnormality affecting the salivary gland duct system. This entity has been reported in only 21 patients previously, although a careful review suggests only 16 patients have histological evidence of the disease. In previously reported cases, this lesion most commonly presents as either an incidental finding or as a swelling affecting the parotid glands bilaterally, or rarely the submandibular glands bilaterally. This case report details the first time dysgenetic polycystic disease is found affecting the minor salivary glands of the tongue in a 55-year-old male. Histochemical and immunohistochemical stains are presented and include positivity for AE1/AE3 and p63, and negativity for progesterone receptor, androgen receptor, mammaglobin, S100 and BRAF V600E. PAS-D and Congo Red highlight special microamyloid spheroliths structures intraluminally.
发育异常性多囊病，也简称为多囊病，是一种非常罕见的发育异常，影响唾液腺导管系统。此前仅有21例患者有该病例报道，不过经仔细审查发现只有16例患者有该病的组织学证据。在先前报道的病例中，这种病变最常见的表现是偶然发现，或是双侧腮腺肿大，或很少见的双侧下颌下腺肿大。本病例报告详细介绍了首次发现一名55岁男性的舌小唾液腺受发育异常性多囊病影响。文中展示了组织化学和免疫组织化学染色结果，包括AE1/AE3和p63呈阳性，而孕激素受体、雄激素受体、乳腺珠蛋白、S100和BRAF V600E呈阴性。PAS-D和刚果红染色突出显示管腔内特殊的微淀粉样球石结构。
