| Objective:To investigate whether the expression of MiTF and TFE3 in perivascular epithelial-like cell tumors(PEComa)and acinar soft tissue sarcoma(ASPS)is related to the biological behavior of the tumor.Treatment offers new ideas.Methods:A total of 59 PEComa and 34 ASPS paraffin specimens and clinical information were collected.Immunohistochemistry(Elivision~TMM Plus/HRP method)was used to detect the expression of MiTF and TFE3 in PEComa and ASPS,respectively.Retrospectively analyze and summarize the clinicopathological features and immunophenotypes,and conduct clinical follow-up and prognostic analysis.Results:In this study,59 cases of PEComa(28 cases of kidney,26 cases of liver,5 cases of uterus),18 cases of male and 41 cases of female,with a median age of 50 years.There were 34 ASPS cases,14 males and 20 females,with a median age of 27 years.Gross:Renal PEComa:The median diameter of the tumor is 6.0 cm.The cut surface is gray-red,gray-yellow,and slightly hard in texture.Liver PEComa:The median diameter of the tumor is 6.5cm,the boundary is clear,the capsule appears to be solid,the cut surface is solid,gray-yellow,and the texture is slightly hard.Uterine PEComa:Tumors have a median diameter of 5.0 cm,all of which are wall-to-wall,with a gray cut surface,fish-like shape,slightly soft texture,and occasional necrosis.ASPS:The median diameter of the tumor is 5.5cm,the border is clear,some are pseudocapsules,nodular,2 of them show invasive growth,the cut surface is grayish gray,the texture is slightly soft,and some are visible Bleeding and necrosis.Microscopic examination:PEComa:Under low magnification,the boundary between tumor tissue and normal tissue is still clear,and most of them are composed of proliferating epithelial cells and spindle cells,which are arranged in a nested trabecular or nested sheet shape;in most cases,visible Tumor cells are arranged radially around thick-walled blood vessels;Under high magnification,the tumor cells are polygonal,the cytoplasm is translucent,and there are uneven eosinophilic granules;the nucleus is oval,the nucleoli is obvious,the chromatin is loose,there may be irregularities,and the mitotic image is rare;the interstitial can be seen Feathery collagen fibers,few with bleeding and necrosis.ASPS:Under low magnification,tumor cells are divided into multiple nodules by fibrous tissue.The tumor cells are arranged in acinar or nested slices,and the sinusoids are abundant.At high magnification,tumor cells are epithelial or polygonal,with atypical heteromorphism.The cytoplasm is eosinophilic,partially translucent,the nucleus is oval,the chromatin is loose,the nucleoli is obvious,and mitotic figures are rare.PAS special staining,purple red rod-shaped crystals can be seen in the cytoplasm of tumor cells.Immunohistochemistry:In PEComa,tumor cells mostly expressed melanocyte markers HMB45,Melan-A and smooth muscle markers SMA,but MiTF and TFE3 were mostly negative.In this study,only one case of MiTF and TFE3 were positive in renal PEComa;two cases were MiTF positive in uterine PEComa,and three cases were TFE3 positive;most Ki-67 proliferation indexes were<5%.In ASPS,tumor cells mostly expressed MiTF and TFE3,MyoD1 was often cytoplasmic positive,and Ki-67 proliferation index was mostly<20%.Follow-up:PEComa(21-77 months),only 2 cases of lymph node metastasis occurred in uterine PEComa,and the other patients had a good prognosis without metastasis and recurrence.In ASPS(14 to 70 months),7 cases recurred and 8 cases metastasized,4of which were lung metastases,and the rest had a better prognosis.Conclusions:(1)The expression of MiTF and TFE3 in PEComa and ASPS was positively correlated with tumor metastasis or recurrence.The stronger the expression,the worse the prognosis of patients.(2)MiTF and TFE3 are expressed differently in different parts of PEComa.Compared with kidney and liver,the positive rate of MiTF and TFE3 in PEComa of the uterus is higher.(3)MiTF gene family members have different expressions in PEComa and ASPS,and TFE3 has a higher positive rate than MiTF. |
PDF Full Text Request