Glumov V Ia, Kuznetsov A A
Arkh Patol. 1987;49(5):70-2.
An observation of multiple primary tumors of various histogenesis and a rare combination (uterine body carcinoma, mammary fibroadenoma, bronchial carcinoid, jejunum carcinoma) developing in a female patient at various periods of her life is described. The autosome-dominant type of the transfer proves the possibility of a genetic predisposition of the tumor development (the patient's mother and brother also had tumors). This observation demonstrates the variety of tumor combinations and tumor topography in cases of genetically predetermined blastomogenic process. This may represent a basis for distinguishing separate variants of Fraumeni syndrome.
描述了一名女性患者在其生命不同时期发生的多种不同组织发生的原发性肿瘤以及一种罕见组合(子宫体癌、乳腺纤维腺瘤、支气管类癌、空肠癌)。常染色体显性遗传类型的传递证明了肿瘤发生存在遗传易感性的可能性(患者的母亲和兄弟也患有肿瘤)。该观察结果表明,在基因预先决定的致瘤过程中,肿瘤组合和肿瘤部位具有多样性。这可能是区分弗劳梅尼综合征不同变体的基础。