Zenezan Dina, Yin Jiejing, Dioufa Nikolina, Firde Kassaye, Mollaee Mehri, Akhtar Israh
Department of Pathology and Laboratory Medicine, Temple University Hospital Philadelphia, PA, USA.
Int J Clin Exp Pathol. 2024 Jun 15;17(6):182-188. doi: 10.62347/KLWT4935. eCollection 2024.
Pulmonary Sclerosing Pneumocytoma (PSP) represents a rare benign tumor that exhibits a predisposition towards females. Often asymptomatic, its identification usually occurs incidentally through imaging modalities. Histologically, PSP demonstrates features consistent with pneumocytic differentiation and possesses a dual-cell population. However, in rare instances it may demonstrate pleural invasion or lymph node metastasis. Diagnosing PSP through small biopsy or frozen section presents considerable challenges attributed to its heterogeneous growth patterns and striking similarity to well-differentiated pulmonary adenocarcinoma. We report a case of PSP in a 57-year-old female smoker, presenting as a slow-growing 2.5 cm mass that recently exhibited enlargement, as noted on computed tomography (CT) scan. The recommendation for excising the mass prompted the patient to undergo a right robotic-assisted thoracoscopic procedure, which entailed wedge resection of the right lower lobe and an intraoperative consultation. A completion right lower lobectomy was performed, accompanied by lymph node dissection, following a frozen section diagnosis indicating at least adenocarcinoma in situ. The permanent section revealed bland cuboidal cells lining papillary and sclerotic areas, with occasional atypical features such as prominent nucleoli and scattered mitotic figures. Adjacent foci of atypical adenomatous hyperplasia (AAH) were noted. Immunohistochemical (IHC) staining revealed positive Napsin A, keratin AE1/3, and CK7 in surface cells but not in round cells. Both EMA and TTF1 immunostains highlighted surface cells and scattered round cells. Elastic stain highlighted visceral pleural involvement. The combined morphology and immunoprofile supported the diagnosis of PSP. This case underscores the critical importance of accurately diagnosing slow-growing pulmonary nodules, which are increasingly detected by the widespread use of imaging for various medical conditions.
肺硬化性肺细胞瘤(PSP)是一种罕见的良性肿瘤,好发于女性。通常无症状,多通过影像学检查偶然发现。组织学上,PSP表现出与肺细胞分化一致的特征,并具有双细胞群。然而,在罕见情况下,它可能表现出胸膜侵犯或淋巴结转移。通过小活检或冰冻切片诊断PSP具有相当大的挑战性,因为其生长模式异质性且与高分化肺腺癌极为相似。我们报告一例57岁女性吸烟者的PSP病例,表现为一个缓慢生长的2.5 cm肿块,计算机断层扫描(CT)显示该肿块近期增大。切除肿块的建议促使患者接受了右机器人辅助胸腔镜手术,包括右下叶楔形切除术及术中会诊。冰冻切片诊断提示至少为原位腺癌后,进行了完整的右下叶切除术及淋巴结清扫。永久切片显示乳头状和硬化区域内衬有温和的立方细胞,偶尔有非典型特征,如明显的核仁和散在的有丝分裂象。可见相邻的非典型腺瘤样增生(AAH)灶。免疫组化(IHC)染色显示表面细胞中Napsin A、角蛋白AE1/3和CK7呈阳性,而圆形细胞中为阴性。EMA和TTF1免疫染色均突出显示表面细胞和散在的圆形细胞。弹性染色突出显示脏层胸膜受累。综合形态学和免疫表型支持PSP的诊断。该病例强调了准确诊断缓慢生长的肺结节的至关重要性,随着各种医疗状况影像学检查的广泛应用,此类结节越来越多地被检测到。