Inter-Sphincteric Resection of Rectum for Ulcerated S-100 Negative Large Granular Cell Tumour

A. Moniruddin, Shakila Jannat, Halima Khatun Doly, Md Masudar Rahman, M. Rouf, Sayeed Bin, Md Sabbir Raihan, Tanvirul Hasan, Sabera Sultana, Rifat Newaz
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Abstract

Non-neural granular cell tumor was first described in 1991 as an unusual primitive, polypoid variant of the conventional GCT (granular cell tumor). To date, this neoplasm remains as a rare entity and the cell of origin is uncertain. While the histological features are similar to the conventional (traditional) GCTs, it represents a distinct entity, as it is negative for S-100 and lacks true nerve sheath differentiation. We report here a case of a 49-year-old male patient with S-100 negative, vimentin positive large benign, ulcerated, bleeding non-neural GCT in the lower rectum. We confirmed the diagnosis histopathologically and immunohistochemically. We treated it by ISRR (Inter-Sphincteric Resection of Rectum) and primary colo-anal anastomosis. We find no other report of non-neural GCT located in the rectum in surgical literature. This is probably the first case of non-neural GCT identified in the rectum. This case report is presented here to document a non-neural GCT located in the rectum for the first time ever reported, and also to highlight the optimum best surgical approach, as we deemed here for this large ulcerated bleeding tumor in the rectum. This rectal non-neural GCT posed here as an unusual differential diagnosis from such more common neoplastic bleeding lesions in the rectum as carcinoma rectum, hemorrhoid, rectal polyp, etc. Here we like to discuss our experience, reviewing in brief the available literature implicating the biological behavior, histopathological and immunohistochemical features, differential diagnosis and therapeutic considerations of neural and non-neural GCTs. KYAMC Journal Volume: 14, No: 03, October 2023: 181-187.
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直肠括约肌间切除术治疗溃疡性 S-100 阴性大颗粒细胞瘤
非神经颗粒细胞瘤于 1991 年首次被描述为传统 GCT(颗粒细胞瘤)的一种不寻常的原始多形性变异。迄今为止,这种肿瘤仍然是一种罕见的肿瘤,其起源细胞尚不确定。虽然其组织学特征与传统的 GCT 相似,但由于其 S-100 阴性且缺乏真正的神经鞘分化,因此是一种独特的肿瘤。我们在此报告了一例 S-100 阴性、波形蛋白阳性的 49 岁男性直肠下部大型良性溃疡性出血非神经性 GCT 患者。我们通过组织病理和免疫组化确诊了该患者。我们采用括约肌间直肠切除术(ISRR)和结肠肛门吻合术进行治疗。在外科文献中,我们没有发现其他关于位于直肠的非神经性 GCT 的报道。这可能是第一例在直肠内发现的非神经性 GCT。本病例报告旨在记录有史以来首次报道的位于直肠的非神经性 GCT,同时强调最佳的手术方法,正如我们在此所认为的治疗直肠大面积溃疡出血肿瘤的最佳方法。这种直肠非神经性 GCT 与直肠癌、痔疮、直肠息肉等更常见的直肠肿瘤性出血病变有不同寻常的鉴别诊断。在此,我们想谈谈自己的经验,简要回顾一下现有文献中有关神经性和非神经性 GCTs 的生物学行为、组织病理学和免疫组化特征、鉴别诊断和治疗注意事项的内容。
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