Colonic schwannoma: A case of unusual presentation and outcome.

IF 0.9 Q4 ONCOLOGY
Rare Tumors Pub Date : 2022-06-24 eCollection Date: 2022-01-01 DOI:10.1177/20363613221110837
Salsabil Nasri, Mohamed Hedi Mraidha, Mehdi Ben Abdelkrim, Sabri Youssef, Fehmi Hamila, Mohamed Amine Elghali
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引用次数: 0

Abstract

Background: Schwannomas are mesenchymal tumors arising from neural sheath cells and whose diagnosis is based on immunohistochemistery. The digestive and especially colonic location of this tumor is rare. Commonly described in elderly patient, their malignancy is unusual.

Case report: We report the case of a 23-year-old girl, with learning disability, operated in emergency for acute peritonitis. Peroperatively, we discovered a peritonitis secondary to a bulky perforated cecal tumor. We performed a right colectomy and an ileostomy. The posterior margin of the resection was macroscopically involved. The diagnosis of colonic schwannoma was confirmed with immunopathological examination of the surgical specimen. Surgical exploration 4 months later and morphological investigations during 2 years showed stability of the tumoral residue in the right iliac fossa. However, we noticed, on CT scanning control 2 years postoperatively, the appearance of a mesenteric recurrence. Exploratory laparotomy confirmed the unresectability of this mesenteric mass and showed the presence of multiple parietal nodules whose biopsies revealed their neurofibromatous nature. The 4 year follow-up of the patient didn't reveal any complication rather than need to right nephrostomy due to ureter compression by the primary tumoral residue.

Discussion: Colonic schwannoma is a rare disease, commonly described in uncomplicated stages. The learning disability of our patient had favorized the absence of declared symptoms and promoted the evolution of the cecal swhannoma until its perforation. This complication was not described before. Local recurrence has been also rarely reported in the literature. Association with neurofibroma may be hereditary in a context of neurofibromatosis or sporadic.

Conclusion: Colonic schwannoma may have polymorphic presentation mimicking malignant tumor in such cases. Not treated in time, it can lead to severe complications, such as tumoral perforation. Surgical resection remains the mainstay treatment. The slow evolutionary genius of schwannoma, even when incompletely resected by necessity, confirms its good prognosis.

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Abstract Image

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结肠神经鞘瘤:一个不寻常的表现和结果。
背景:神经鞘瘤是起源于神经鞘细胞的间充质肿瘤,其诊断基于免疫组织化学。这种肿瘤很少发生在消化道,尤其是结肠。常见于老年患者,恶性肿瘤不常见。病例报告:我们报告一例23岁的女孩,有学习障碍,在急性腹膜炎急诊手术。手术中,我们发现一个腹膜炎继发于一个巨大的盲肠穿孔肿瘤。我们做了右结肠切除术和回肠造口术。切除后缘在宏观上受累。手术标本的免疫病理检查证实了结肠神经鞘瘤的诊断。4个月后手术探查和2年形态学检查显示右侧髂窝肿瘤残留稳定。然而,我们注意到,在术后2年的CT扫描对照中,出现肠系膜复发。剖腹探查证实了这个肠系膜肿块的不可切除性,并显示存在多个肠壁结节,活检显示其神经纤维瘤性质。患者4年随访未发现任何并发症,无需因原发肿瘤残留压迫输尿管而行右肾造口术。讨论:结肠神经鞘瘤是一种罕见的疾病,通常分期简单。本例患者的学习障碍倾向于没有明确的症状,并促进盲肠鱼鞘瘤的发展直至其穿孔。这种并发症以前没有被描述过。文献中也很少报道局部复发。与神经纤维瘤的关联在神经纤维瘤病或散发性的情况下可能是遗传性的。结论:结肠神经鞘瘤可能具有类似恶性肿瘤的多形态表现。如果不及时治疗,它会导致严重的并发症,如肿瘤穿孔。手术切除仍是主要的治疗方法。神经鞘瘤的缓慢进化天赋,即使在必要时不完全切除,也证实了其良好的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Rare Tumors
Rare Tumors ONCOLOGY-
CiteScore
1.50
自引率
0.00%
发文量
15
审稿时长
15 weeks
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