Stolnicu Simona, Molnar Claudiu, Barsan Iulia, Boros Monica, Nogales Francisco F, Soslow Robert A
Departments of Pathology (S.S., I.B.) Obstetrics and Gynecology Clinic (C.M.), University of Medicine and Pharmacy, Targu Mures Department of Pathology (M.B.), County Emergency Clinical Hospital, Oradea, Romania Department of Pathology (F.F.N.), San Cecilio University Hospital, Granada, Spain Department of Pathology (R.A.S.), Memorial Sloan Kettering Cancer Center, New York, New York.
Int J Gynecol Pathol. 2016 Mar;35(2):147-52. doi: 10.1097/PGP.0000000000000231.
Mullerian adenosarcomas are uncommon tumors of the female genital tract characterized by a synchronous proliferation of benign glands and sarcomatous stroma. In general, uterine Mullerian adenosarcomas are associated with a low risk of recurrence. The presence of "stromal overgrowth" (SO), historically defined by an estimate of the volume of sarcoma growing independently of epithelium, is associated with deep myometrial invasion, presence of heterologous elements, and poor outcomes. Very rarely, the stromal component can harbor foci resembling ovarian sex cord tumors (FROSCT). The aim of this study was to determine whether the presence of an extensive FROSCT component in Mullerian adenosarcomas has an impact on survival, akin to more typical types of SO. Six patients were included in this study. Age ranged from 39 to 71 yr. Five patients presented with uterine lesions (4 intracavitary, 1 isthmic), and 1 was located in the ovary. Tumors ranging in size from 2.5 to 19 cm were all diagnosed as Stage I. Morphologically, all had prominent FROSCT-like components that comprised 60% to 90% of tumor volume. Immunohistochemically, the FROSCT component was positive for CAM 5.2, vimentin, WT1, CD56, α-inhibin, calretinin, androgen and progesterone receptors, α-actin, and desmin. All patients are alive without disease at 26 to 102 mo. Compared with adenosarcomas with typical forms of SO, FROSCT overgrowth is low grade and not associated with recurrence or metastasis in this small series. Therefore, Mullerian adenosarcoma with extensive FROSCT should not be equated with SO.
苗勒管腺肉瘤是女性生殖道的罕见肿瘤，其特征是良性腺体和肉瘤样间质同步增殖。一般来说，子宫苗勒管腺肉瘤的复发风险较低。“间质过度生长”（SO）的存在，历史上通过独立于上皮生长的肉瘤体积估计来定义，与子宫肌层深层浸润、异源性成分的存在及不良预后相关。非常罕见的是，间质成分可含有类似卵巢性索肿瘤的病灶（FROSCT）。本研究的目的是确定苗勒管腺肉瘤中广泛存在的FROSCT成分是否对生存有影响，类似于更典型的SO类型。本研究纳入了6例患者。年龄范围为39至71岁。5例患者表现为子宫病变（4例为宫腔内病变，1例为峡部病变），1例位于卵巢。肿瘤大小从2.5至19 cm不等，均诊断为I期。形态学上，所有肿瘤均有突出的FROSCT样成分，占肿瘤体积的60%至90%。免疫组化显示，FROSCT成分对CAM 5.2、波形蛋白、WT1、CD56、α-抑制素、钙视网膜蛋白、雄激素和孕激素受体、α-肌动蛋白及结蛋白呈阳性。所有患者在26至102个月时均无病存活。与具有典型SO形式的腺肉瘤相比，在这个小系列中，FROSCT过度生长为低级别，且与复发或转移无关。因此，伴有广泛FROSCT的苗勒管腺肉瘤不应与SO等同。
