Nasri Salsabil, Hedi Mraidha Mohamed, Abdelkrim Mehdi Ben, Youssef Sabri, Hamila Fehmi, Amine Elghali Mohamed
Department of Visceral Surgery, Farhat Hached University Hospital, Sousse, Tunisia.
Rare Tumors. 2022 Jun 24;14:20363613221110837. doi: 10.1177/20363613221110837. eCollection 2022.
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.
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.
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.
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.
施万细胞瘤是起源于神经鞘细胞的间叶性肿瘤，其诊断基于免疫组织化学。这种肿瘤在消化道尤其是结肠的定位很少见。通常在老年患者中描述，其恶性情况并不常见。
我们报告一名23岁有学习障碍的女孩的病例，她因急性腹膜炎接受急诊手术。术中，我们发现腹膜炎继发于一个巨大的穿孔性盲肠肿瘤。我们进行了右半结肠切除术和回肠造口术。切除的后缘肉眼可见受累。手术标本的免疫病理检查证实了结肠施万细胞瘤的诊断。4个月后的手术探查以及2年的形态学检查显示右髂窝肿瘤残余稳定。然而，我们在术后2年的CT扫描复查中注意到肠系膜复发。剖腹探查证实该肠系膜肿块无法切除，并显示存在多个壁层结节，其活检显示为神经纤维瘤性质。对该患者的4年随访未发现任何并发症，仅因原发性肿瘤残余压迫输尿管而需要进行右肾造瘘。
结肠施万细胞瘤是一种罕见疾病，通常在未出现并发症的阶段被描述。我们患者的学习障碍导致未出现明显症状，并促使盲肠施万细胞瘤发展直至穿孔。这种并发症以前未被描述过。局部复发在文献中也很少报道。与神经纤维瘤的关联在神经纤维瘤病背景下可能是遗传性的，也可能是散发性的。
结肠施万细胞瘤在这种情况下可能有模仿恶性肿瘤的多形性表现。如果不及时治疗，它可能导致严重并发症，如肿瘤穿孔。手术切除仍然是主要的治疗方法。施万细胞瘤的缓慢演变特性，即使在必要时未完全切除，也证实了其良好的预后。
