Rabha Punam, Wisely Jerryes Pious, Jain Ashutosh, Raina Vinod, Ahlawat Sunita, Das Sumanta
Department of Medical Oncology, Fortis Memorial Research Institute, Gurugram, India.
Department of Pathology, Agilus Diagnostics Ltd, Fortis Memorial Research Institute, Gurugram, India.
Int J Surg Case Rep. 2025 Jul;132:111450. doi: 10.1016/j.ijscr.2025.111450. Epub 2025 May 19.
Lung cancer is the leading cause of cancer-related mortality worldwide, with small cell lung carcinoma (SCLC) accounting for significant morbidity and mortality. While SCLC typically arises from neuroendocrine cells in the central lung, primary pleural small cell carcinoma is exceedingly rare. Its atypical presentation poses diagnostic challenges, emphasizing the need for early recognition and accurate diagnosis to optimize outcomes.
A 70-year-old male presented with progressive shortness of breath and significant weight loss. Imaging revealed massive pleural effusion. Pleural fluid analysis via pleurocentesis, including cytological evaluation and cell block analysis, revealed features consistent with small cell neuroendocrine carcinoma. Immunohistochemical studies confirmed the diagnosis, with tumor cells positive for pancytokeratin, TTF1, synaptophysin, and INSM1, and negative for Napsin-A, p40, WT1, calretinin, and chromogranin. A Ki-67 proliferation index of >90 % indicated a highly proliferative tumor. Chemotherapy with Carboplatin and Etoposide was initiated, leading to a partial response as evidenced by follow-up PET-CT imaging.
Primary pleural small cell carcinoma is an exceptionally rare and aggressive variant of SCLC. Accurate diagnosis relies on cytological and immunohistochemical analyses to differentiate it from other pleural malignancies, particularly mesothelioma. The high Ki-67 index indicates its aggressive nature. Management follows conventional SCLC protocols, with systemic chemotherapy as the primary treatment modality. The patient's partial response highlights the potential for disease control with prompt intervention.
This case highlights the rarity and aggressive nature of primary pleural small cell carcinoma. Advanced diagnostic techniques and timely treatment are critical.
肺癌是全球癌症相关死亡的主要原因,其中小细胞肺癌(SCLC)导致了显著的发病率和死亡率。虽然SCLC通常起源于肺中央的神经内分泌细胞,但原发性胸膜小细胞癌极为罕见。其非典型表现带来了诊断挑战,强调了早期识别和准确诊断以优化治疗结果的必要性。
一名70岁男性出现进行性气短和显著体重减轻。影像学检查显示大量胸腔积液。通过胸腔穿刺进行的胸水分析,包括细胞学评估和细胞块分析,发现了与小细胞神经内分泌癌一致的特征。免疫组织化学研究证实了诊断,肿瘤细胞全细胞角蛋白、甲状腺转录因子1、突触素和胰岛1号锌指蛋白呈阳性,而Napsin-A、p40、WT1、钙视网膜蛋白和嗜铬粒蛋白呈阴性。Ki-67增殖指数>90%表明肿瘤具有高度增殖性。开始使用卡铂和依托泊苷进行化疗,后续PET-CT成像显示出现部分缓解。
原发性胸膜小细胞癌是SCLC一种极其罕见且侵袭性强的变体。准确诊断依赖于细胞学和免疫组织化学分析,以将其与其他胸膜恶性肿瘤,特别是间皮瘤相鉴别。高Ki-67指数表明其侵袭性。治疗遵循传统的SCLC方案,以全身化疗作为主要治疗方式。患者的部分缓解突出了及时干预控制疾病的潜力。
本病例突出了原发性胸膜小细胞癌的罕见性和侵袭性。先进的诊断技术和及时治疗至关重要。