Department of Liver Transplantation & GI Surgery, Fortis Escort Liver and Digestive Institute, FEHI, New Delhi, India.
Department of Gastroenterology and Hepatology, Fortis Escort Liver and Digestive Institute, FEHI, New Delhi, India.
Korean J Gastroenterol. 2023 Jan 25;81(1):40-45. doi: 10.4166/kjg.2022.100.
An undifferentiated carcinoma (UC) of the gall bladder behaves aggressively and has a grave prognosis. Small cell type undifferentiated carcinoma of the gall bladder is a rare variant. This paper reports a case of UC of gall bladder with PAS-positive diastase- resistant eosinophilic hyaline globules present as liver mass (on imaging) in a male patient. The microscopic findings of the liver and gall bladder after a right tri-segmentectomy showed an un-differentiated malignant neoplasm composed of cells with round to oval nuclei, prominent nucleoli, and scanty neoplasm. No definite cell pattern was identified with these neoplastic cells. A section from the gall bladder revealed a tumor arising from the lining epithelium and infiltrating through the muscularis. This tumor was infiltrating the adherent liver tissue directly and forming a mass of undifferentiated malignant cells. The focal area within the tumor mass showed the presence of PAS-positive, diastase-resistant, eosinophilic hyaline globules within the neoplastic cells. The immunohistochemistry test was diffusely positive for perinuclear anti-neutrophil cytoplasmic antibodies and negative for chromogranin, vimentin, Desmin, alpha-fetoprotein, leukocyte common antigen, CD34, and bcl2. When the clinical and radiological data are inconclusive, careful analysis of the histological and immunophenotypic features is needed to make the final diagnosis of UC of the gall bladder. The biological behavior and prognosis of this tumor remain unclear because of its rarity. Further studies will be needed to understand the characteristics of this deadly tumor and to establish an effective therapy for it.
胆囊未分化癌(UC)侵袭性强,预后极差。胆囊小细胞未分化癌是一种罕见的变体。本文报告了 1 例男性患者,其胆囊 UC 伴有 PAS 阳性、耐淀粉酶的嗜酸性透明小体,表现为肝肿块(影像学)。右三叶切除术切除的肝和胆囊的显微镜检查结果显示,一种未分化的恶性肿瘤由具有圆形至椭圆形核、明显核仁、稀少肿瘤的细胞组成。这些肿瘤细胞没有明确的细胞模式。胆囊切片显示肿瘤起源于衬里上皮并浸润肌层。该肿瘤直接浸润附着的肝组织并形成未分化恶性细胞的肿块。肿瘤内的局灶区域内可见 PAS 阳性、耐淀粉酶、嗜酸性透明小体存在于肿瘤细胞内。免疫组织化学试验显示核周抗中性粒细胞细胞质抗体弥漫阳性,而嗜铬粒蛋白、波形蛋白、结蛋白、甲胎蛋白、白细胞共同抗原、CD34 和 bcl2 均为阴性。当临床和影像学数据不确定时,需要仔细分析组织学和免疫表型特征,以做出胆囊 UC 的最终诊断。由于其罕见性,该肿瘤的生物学行为和预后仍不清楚。需要进一步研究以了解这种致命肿瘤的特征,并为其建立有效的治疗方法。