PDF(Pubmed)
Abstract：
BACKGROUND: Alveolar soft part sarcoma is a rare tumour of soft tissues, mostly localized in muscles or deep soft tissues of the extremities. In rare occasions, this tumour develops in deep tissues of the abdomen or pelvis.
METHODS: In this case report, we described the case of a 46 year old man who developed a primary splenic alveolar soft part sarcoma. The tumour displayed typical morphological alveolar aspect, as well as immunohistochemical profile notably TFE3 nuclear staining. Detection of ASPSCR1 Exon 7::TFE3 Exon 6 fusion transcript in molecular biology and TFE3 rearrangement in FISH confirmed the diagnosis.
CONCLUSIONS: We described the first case of primary splenic alveolar soft part sarcoma, which questions once again the cell of origin of this rare tumour.
METHODS: In this case report, we described the case of a 46 year old man who developed a primary splenic alveolar soft part sarcoma. The tumour displayed typical morphological alveolar aspect, as well as immunohistochemical profile notably TFE3 nuclear staining. Detection of ASPSCR1 Exon 7::TFE3 Exon 6 fusion transcript in molecular biology and TFE3 rearrangement in FISH confirmed the diagnosis.
CONCLUSIONS: We described the first case of primary splenic alveolar soft part sarcoma, which questions once again the cell of origin of this rare tumour.
摘要：
背景：肺泡软组织肉瘤是一种罕见的软组织肿瘤，大多位于四肢的肌肉或深层软组织。在极少数情况下，这种肿瘤在腹部或骨盆的深层组织中发展。
方法：在本案例报告中，我们描述了一例46岁的男性，他发展为原发性脾肺泡软组织肉瘤。肿瘤表现出典型的形态肺泡方面，以及免疫组织化学谱，特别是TFE3核染色。分子生物学中ASPSCR1外显子7::TFE3外显子6融合转录本的检测和FISH中TFE3重排的检测证实了诊断。
结论：我们描述了第一例原发性脾肺泡软组织肉瘤，这再次质疑这种罕见肿瘤的起源细胞。
方法：在本案例报告中，我们描述了一例46岁的男性，他发展为原发性脾肺泡软组织肉瘤。肿瘤表现出典型的形态肺泡方面，以及免疫组织化学谱，特别是TFE3核染色。分子生物学中ASPSCR1外显子7::TFE3外显子6融合转录本的检测和FISH中TFE3重排的检测证实了诊断。
结论：我们描述了第一例原发性脾肺泡软组织肉瘤，这再次质疑这种罕见肿瘤的起源细胞。
