原发性腹膜恶性肿瘤是例外。其中,透明细胞癌极为罕见，自1990年以来，文献中只有13例。我们报告了一名48岁的白人妇女在阿利坎特大学总医院接受治疗的病例。在过去的七个月中，由于进行性腹痛，她进行了咨询，初步诊断为肾输尿管绞痛。腹部和骨盆的超声和计算机断层扫描显示25×15厘米，明确的囊性病变伴乳头状突起，位于腹部的中央。放射学报告建议将原发性卵巢肿瘤与腹膜植入物作为首选。该患者接受了剖腹探查术，显示膀胱腹膜内有大的囊性肿块，牢牢地附着在肠系膜上。整个腹部肿瘤被完全切除,并进行了全子宫切除术，双侧附件卵巢切除术和结肠下网膜切除术。最终的组织学研究显示，位于膀胱腹膜的原发性腹膜透明细胞癌的新病例，牢牢地附着在肠系膜上。严重的,它是完整的，多囊性，乳头状生长累及部分内壁。微观上,它显示肾小管囊性和乳头状模式，具有高度不典型的肿瘤细胞。经过广泛的免疫组织化学分析，最相关的发现是ARID1A丢失,通过显示ARID1A缺失的分子分析得到证实.患者接受卡铂和紫杉醇方案的全身化疗（5~4个周期）。第8个月后的患者随访显示，腹膜植入物主要位于右diaphragm肌，经组织学证实为复发。她刚刚接受了另外六个周期的卡铂和紫杉醇化疗。在这个不常见的位置识别原发性腹膜透明细胞癌，排除卵巢转移,代表了一个诊断挑战.
Primary peritoneal malignant tumors are exceptional. Among them, clear cell carcinoma is extremely rare, being only thirteen cases previously reported in the literature since 1990. We report a
case of a 48-year-old Caucasian woman who was treated at the University General Hospital of Alicante. She consulted because of progressive abdominal pain over the last seven months, with the initial diagnosis of renal-ureteral colic. Ultrasound and computed tomography of the abdomen and pelvis revealed a 25 × 15 cm, well-defined cystic lesion with papillary projections, centrally located in the abdomen. The radiology report suggested a primary ovarian tumor versus peritoneal implant as the first option. The patient underwent an exploratory laparotomy showing a large cystic mass located in the urinary bladder peritoneum, firmly attached to the mesentery. The entire abdominal tumor was completely excised, and total hysterectomy with bilateral salpingo-oophorectomy and infra-colical omentectomy were performed. The final histological study revealed a new
case of primary peritoneal clear cell carcinoma located in the urinary bladder peritoneum, firmly attached to the mesentery. Grossly, it was well-circumscribed and multicystic with papillary growth involving part of the inner wall. Microscopically, it showed tubulocystic and papillary patterns with highly atypical tumor cells. After an extensive immunohistochemical analysis, the most relevant finding was an ARID1A loss that was corroborated by molecular analysis showing an ARID1A deletion. The patient received systemic chemotherapy with carboplatin and paclitaxel protocol (Å ~ 4 cycles). Patient follow-up after the eighth month showed peritoneal implants predominantly in the right diaphragmatic cupule that were histologically confirmed as recurrence. She has just received another six cycles of chemotherapy with carboplatin and paclitaxel. Recognition of primary peritoneal clear cell carcinoma in this uncommon location, and exclude metastasis from the ovary, represents a diagnostic challenge.
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