Liu Xu, Liu Jiao, Chen Lu, Yang Chunrong, Hu Yuchang, Liu Yufei
Institute of Pathology, China Three Gorges University, Yichang, China.
Department of Pathology, Yichang Central People's Hospital, Yichang, China.
Heliyon. 2024 Jul 23;10(15):e35075. doi: 10.1016/j.heliyon.2024.e35075. eCollection 2024 Aug 15.
Most ovarian tumors exhibit a pure histological characteristic. Nevertheless, a combination of tumors with the same histogenetic origin but different histologic subtypes is relatively common. Additionally, co-occurrence of tumors with different histogenetic origins is very rare. Typically, these mixed tumors include mixed epithelial tumors, mixed epithelial-stromal tumors, mixed germ cell-sex cord-stromal tumors, and mixed germ cell tumors. However, mixed epithelial-sex cord stromal-lymphohematopoietic system tumors are rare. Currently, clinicians have limited knowledge of this type of tumor, and the epidemiology, diagnosis, and treatment of this disease are yet to be established.
We report a case of a 73-year-old woman with abdominal distension and pain for three months. Imaging evaluation revealed a large pelvic mass, with ultrasound suggesting a benign ovarian cyst along with leiomyoma. Furthermore, computed tomography (CT) and magnetic resonance imaging (MRI) revealed a malignant tumor. Blood tests showed significant increases in CA125 and CA199 levels. The patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. During the surgery, a large multinodular cystic solid mass was observed in the right ovary, and the maximum nodular diameter was 14.2 cm. The solid areas of the mass appeared gray-white and taupe, whereas the cystic areas contained clear liquid with smooth walls 0.2 cm thick and no intracystic solid areas. The left ovary had solitary nodules, the largest being 4 cm in diameter. Microscopic examination of the right ovary revealed three different cell types. The first type of cell area was analogous, round, fusiform, and staggered mixed cells with unclear boundaries and rare nucleolus or mitosis. The second type of cell area was the cystic dilatation area. The cyst wall was covered with a single layer of flat epithelium, rich eosinophilic cytoplasm, uniform nuclear chromatin, and no papillary structures. The third type was a diffuse lymphoid region with uniform medium-sized cells, rough nuclear chromatin and evident nucleoli and mitosis. The morphology of the left ovarian cell was single, which was consistent with the first type of cell area in the right ovary. Immunohistochemistry of the right ovary indicated that the first region expressed vimentin, inhibin-α, calretinin, SF-1, WT-1 and CD56, with Ki-67 at 5 %, and no CKpan expression. The second region expressed CKpan, with Ki-67 at 1 %. The third region expressed CD20, Pax-5, Bcl-6, Bcl-2, MUM1, CD45, and C-myc, with Ki-67 at 70 %, and positive IGH clonal gene rearrangement. Lastly, the pathological diagnosis was a mixed ovarian tumor in the right ovary, comprising thecoma-fibroma, serous cystadenoma, diffuse large B-cell lymphoma, and a thecoma-fibroma in the left ovary. A follow-up examination of the patient after 15 months showed no mass or lymph node enlargement in other parts of the body, and no recurrence or metastasis was observed.
We present a case of a postmenopausal woman with a rare combination of thecoma-fibroma, serous cystadenoma and diffuse large B-cell lymphoma in the ovary. To the best of our knowledge, this is the first reported case of such a combination. Typical pathological morphology and immunohistochemistry are crucial for the diagnosis of this disease. Owing to the limited knowledge of the disease, its pathogenesis and tissue origin are unknown. Clinicians should be careful about such patients. We believe this case report may provide some novel insights into the diagnosis and therapy of patients with this type of tumor.
大多数卵巢肿瘤具有单一的组织学特征。然而,具有相同组织发生学起源但不同组织学亚型的肿瘤组合相对常见。此外,具有不同组织发生学起源的肿瘤同时出现则非常罕见。通常,这些混合性肿瘤包括混合性上皮肿瘤、混合性上皮 - 间质肿瘤、混合性生殖细胞 - 性索 - 间质肿瘤以及混合性生殖细胞肿瘤。然而,混合性上皮 - 性索间质 - 淋巴造血系统肿瘤较为罕见。目前,临床医生对这类肿瘤的了解有限,该疾病的流行病学、诊断和治疗方法尚未确立。
我们报告一例73岁女性,腹胀、腹痛3个月。影像学评估显示盆腔有一巨大肿块,超声提示为良性卵巢囊肿合并平滑肌瘤。此外,计算机断层扫描(CT)和磁共振成像(MRI)显示为恶性肿瘤。血液检查显示CA125和CA199水平显著升高。患者接受了全腹子宫切除术及双侧输卵管卵巢切除术。手术中,右侧卵巢可见一个巨大的多结节囊实性肿块,最大结节直径为14.2 cm。肿块的实性区域呈灰白色和灰褐色,而囊性区域含有清亮液体,囊壁光滑,厚0.2 cm,无囊内实性区域。左侧卵巢有孤立结节,最大直径为4 cm。对右侧卵巢进行显微镜检查发现三种不同类型的细胞。第一种细胞区域为类似圆形、梭形且交错混合的细胞,边界不清,核仁或有丝分裂少见。第二种细胞区域为囊性扩张区域。囊壁覆盖单层扁平上皮,细胞质嗜酸性丰富,核染色质均匀,无乳头状结构。第三种是弥漫性淋巴样区域,细胞大小均匀,核染色质粗糙,核仁明显且有丝分裂活跃。左侧卵巢细胞形态单一,与右侧卵巢的第一种细胞区域一致。右侧卵巢的免疫组织化学检查表明,第一个区域表达波形蛋白、抑制素-α、钙视网膜蛋白、SF-1、WT-1和CD56,Ki-67为5%,无细胞角蛋白泛抗体(CKpan)表达。第二个区域表达CKpan,Ki-67为1%。第三个区域表达CD20、Pax-5、Bcl-6、Bcl-2、MUM1、CD45和C-myc,Ki-67为70%,免疫球蛋白重链(IGH)克隆基因重排阳性。最后,病理诊断为右侧卵巢混合性肿瘤,包括纤维瘤 - 卵泡膜瘤、浆液性囊腺瘤、弥漫性大B细胞淋巴瘤,左侧卵巢为纤维瘤 - 卵泡膜瘤。患者术后15个月的随访检查显示身体其他部位无肿块或淋巴结肿大,未观察到复发或转移。
我们报告了一例绝经后女性卵巢出现罕见的纤维瘤 - 卵泡膜瘤、浆液性囊腺瘤和弥漫性大B细胞淋巴瘤组合的病例。据我们所知,这是首例报道的此类组合病例。典型的病理形态和免疫组织化学对该病的诊断至关重要。由于对该疾病了解有限,其发病机制和组织起源尚不清楚。临床医生应对此类患者予以关注。我们相信本病例报告可能为这类肿瘤患者的诊断和治疗提供一些新的见解。