James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, Boston, MA.
Am J Surg Pathol. 2023 Jul 1;47(7):774-784. doi: 10.1097/PAS.0000000000002049. Epub 2023 May 15.
Sclerosis is well-known in sclerosing stromal tumors (SSTs), as its name indicates, but has not been evaluated in other ovarian sex cord-stromal tumors (SCSTs). Its presence in other SCSTs has sporadically caused diagnostic problems in cases we have seen, and this prompted us to review SCSTs with appreciable sclerosis; tumors containing at least 20% sclerosis were included. Seventy cases were identified: 20 thecomas, 20 juvenile granulosa cell tumors (JGCTs), 8 adult granulosa cell tumors (AGCTs), 5 sex cord tumors with annular tubules, 6 retiform Sertoli-Leydig cell tumors (SLCTs; all of the intermediate differentiation), 4 nonretiform SLCTs (3 well-differentiated, 1 of intermediate differentiation with heterologous elements), 4 Sertoli cell tumors, and 3 microcystic stromal tumors (MSTs). Paucicellular sclerotic zones comprised 20% to 95% of the tumors and when conspicuous often obscured diagnostic features. Thirty-one tumors (10 thecomas, 19 JGCTs, 1 AGCT, and 1 MST) showed sclerotic zones focally enveloping nodules of tumor cells, imparting a pseudolobular appearance, and sclerosis often occurred within lobules as well. Ten of these (5 thecomas and 5 JGCTs) also had prominent staghorn blood vessels, generating a low-power appearance focally similar to SST. In 17 tumors, the sclerosis resulted in "compression" of the tumor cells into cords and/or solid tubules. Correct diagnosis in these cases is dependent on careful examination of the cellular zones of the neoplasms, but awareness of the extent of sclerosis that may be seen in diverse SCSTs may be crucial in suggesting the correct diagnosis particularly when the material is limited as in the intraoperative setting. Our findings highlight for the first time the occurrence and character of sclerosis in sex cord tumors other than SSTs and fibromas. Sclerosis is seen in descending proportion of the tumor types as follows: retiform SLCTs, thecomas, MSTs, JGCTs, sex cord tumors with annular tubules, Sertoli cell tumors, AGCTs, and nonretiform SLCTs. Its character can vary somewhat, having particular features in the sex cord tumor with annular tubules (hyaline material within tubules often coalescing and extending beyond the nests to form confluent aggregates) and retiform SLCTs (common in papillary cores).
硬化在硬化性间质肿瘤（SST）中是众所周知的，如其名称所示，但在其他卵巢性索-间质肿瘤（SCST）中尚未进行评估。在我们所见的病例中，其在其他 SCST 中的存在偶尔会导致诊断问题，这促使我们复习具有明显硬化的 SCST；包含至少 20%硬化的肿瘤被纳入研究。共确定了 70 例病例：20 例纤维瘤，20 例幼年颗粒细胞瘤（JGCT），8 例成人颗粒细胞瘤（AGCT），5 例含有环状小管的性索肿瘤，6 例 rete 型 Sertoli-Leydig 细胞瘤（SLCT；均为中分化），4 例非 rete 型 SLCT（3 例高分化，1 例中分化伴异源成分），4 例 Sertoli 细胞瘤和 3 例微囊性基质瘤（MST）。少细胞性硬化区占肿瘤的 20%至 95%，当显著时，常使诊断特征变得模糊。31 例肿瘤（10 例纤维瘤，19 例 JGCT，1 例 AGCT 和 1 例 MST）显示硬化区局灶性包裹肿瘤细胞结节，呈假小叶外观，小叶内也常发生硬化。其中 10 例（5 例纤维瘤和 5 例 JGCT）也有明显的鹿角状血管，在低倍镜下产生类似 SST 的局灶性外观。在 17 例肿瘤中，硬化导致肿瘤细胞呈条索状和/或实性小管状“压缩”。在这些病例中，正确诊断取决于对肿瘤细胞区的仔细检查，但在术中有限的标本情况下，认识到在不同的 SCST 中可能出现的硬化程度可能对提示正确诊断至关重要。我们的研究结果首次强调了除 SST 和纤维瘤以外的性索肿瘤中硬化的发生和特征。硬化在肿瘤类型中的比例依次下降：rete 型 SLCT、纤维瘤、MST、JGCT、含有环状小管的性索肿瘤、Sertoli 细胞瘤、AGCT 和非 rete 型 SLCT。其特征可能有所不同，在含有环状小管的性索肿瘤中具有特定特征（小管内的透明物质常融合并延伸超出巢，形成融合的聚集物）和 rete 型 SLCT（常见于乳头状核）。
