关键词: anaplastic carcinoma case report mucinous cystic neoplasms mural nodules ovarian tumour sarcomatoid differentiation

来  源:   DOI:10.3332/ecancer.2023.1557   PDF(Pubmed)

Abstract:
Ovarian mucinous cystic tumours with mural nodules are rare tumours of the ovary that are often missed out during diagnosis. They are classified under the ovarian mucinous surface epithelial-stromal tumours. These mural nodules can be sarcoma-like (benign), anaplastic carcinoma, sarcomas, or mixed malignant (carcinosarcoma). However, very few cases of anaplastic malignant mural nodules have been reported. Here, we present a case of a borderline ovarian mucinous cystadenoma with anaplastic mural nodule that has sarcomatoid differentiation, in a 39-year-old woman who presented with a 1-year history of progressive abdominal swelling and pain. There were intraoperative findings of huge right ovarian cystic tumour with omental and umbilical deposits. Differential diagnosis of possible germ cell tumours, vascular tumours, melanoma, sarcoma and sarcoma-like nodules were ruled out with routine histology (Haematoxylin & Eosin), histochemical (reticulin) and immunohistochemical stains (CK AE1/3+, CD30+, AFP-, HCG-, EMA-, S100 protein-, CD31-, and CD34-) and the final diagnosis of a mural nodule of anaplastic carcinoma with sarcomatoid differentiation in a borderline ovarian mucinous cystadenoma established. Unfortunately, due to the aggressive nature of the tumour and disease progression, the patient passed on a few months after the surgery. This rare tumour, especially the ones with anaplastic carcinoma or mixed tumours, usually has an aggressive clinical course with most patients presenting late when the disease is advanced with poor clinical outcomes as is seen with the index patient. A high index of suspicion of this tumour with early detection and a multidisciplinary approach to its management is advised.
摘要:
带有壁结节的卵巢粘液性囊性肿瘤是罕见的卵巢肿瘤,在诊断过程中经常被遗漏。它们被分类为卵巢粘液性表面上皮基质肿瘤。这些壁结节可以是肉瘤样(良性),间变性癌,肉瘤,或混合恶性(癌肉瘤)。然而,很少报道过间变性恶性壁结节。这里,我们介绍了一例交界性卵巢粘液性囊腺瘤,并伴有肉瘤样分化的间变性壁结节,在一名39岁女性中,她有1年的进行性腹部肿胀和疼痛病史。术中发现巨大的右卵巢囊性肿瘤伴网膜和脐沉积。可能的生殖细胞肿瘤的鉴别诊断,血管肿瘤,黑色素瘤,常规组织学检查排除肉瘤和肉瘤样结节(苏木精和伊红),组织化学(网织蛋白)和免疫组织化学染色(CKAE1/3+,CD30+,AFP-,HCG-,EMA-,S100蛋白-,CD31-,和CD34-),并最终诊断出在交界性卵巢粘液性囊腺瘤中具有肉瘤样分化的间变性癌的壁结节。不幸的是,由于肿瘤和疾病进展的侵袭性,病人在手术后几个月就去世了。这个罕见的肿瘤,尤其是患有间变性癌或混合肿瘤的患者,通常具有积极的临床过程,大多数患者在疾病进展时出现晚期,临床结果较差,如索引患者所见。建议对这种肿瘤进行高度怀疑,并进行早期发现和多学科管理。
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