Boustani Jihane, Kim Stefano, Lescut Nicolas, Lakkis Zaher, de Billy Marjolaine, Arbez-Gindre Francine, Jary Marine, Borg Christophe, Bosset Jean-François
Department of Radiotherapy, University Hospital of Besançon, Besançon, France.
Department of Medical Oncology, University Hospital of Besançon, Besançon, France.
Am J Case Rep. 2015 Aug 31;16:581-5. doi: 10.12659/AJCR.893830.
Rectal linitis plastica (RLP) is a rare disease with poor outcome. It is often accompanied by a delayed histopathological diagnosis, primarily due to submucosal disease. A concentric ring pattern or "target sign" on T2-weighted magnetic resonance imaging (MRI) has been proposed as being characteristic for early suspicion. Even though RLP is more aggressive and has poorer survival than other rectal adenocarcinomas, no specific treatment is recommended. In this case report of 3 patients, we challenge the sensitivity of the characteristic radiological pattern, and we review the existing data for a treatment strategy.
One patient presented classic clinical characteristics of RLP with young age and advanced stage at diagnosis, with chemo-refractory disease and rapid fatal evolution. Biopsies confirmed the RLP with the presence of signet-ring cells (SRC) in a strong desmoplastic stromal reaction. However, the characteristic concentric ring pattern was absent. Instead, he had a large vegetative lesion with important tumor infiltration in mesorectum and pelvic organs, with major lymph node involvement. The 2 other patients presented resectable locally advanced disease with characteristic concentric ring pattern. No clinical and radiological responses were observed to neo-adjuvant chemoradiotherapy (CRT), including 1 patient with non-resectable disease at surgery and another with upstaged disease at pathological specimen after resection. However, data suggest 2 types of RLP: about half of patients are extremely sensitive to CRT with pathological complete response, and the other half are highly resistant with no response to CRT. Current data are insufficient to distinguish between these 2 populations.
The absence of a concentric ring pattern should not eliminate the suspicion of RLP, especially in young patients with aggressive clinical presentation. There are probably 2 types of RLP in terms of chemoradiosensitivity, and neoadjuvant CRT could delay the curative-intent surgery in refractory patients. Future molecular analysis of the tumor and its environment are required to characterize the 2 different forms of RLP to develop more personalized treatment strategies.
直肠皮革胃（RLP）是一种罕见疾病，预后较差。其组织病理学诊断常被延迟，主要原因是黏膜下病变。T2加权磁共振成像（MRI）上的同心圆模式或“靶征”被认为是早期怀疑该病的特征。尽管RLP比其他直肠腺癌更具侵袭性且生存率更低，但目前尚无推荐的特异性治疗方法。在这份关于3例患者的病例报告中，我们对特征性放射学模式的敏感性提出质疑，并回顾现有数据以寻找治疗策略。
1例患者表现出RLP的典型临床特征，诊断时年龄较轻且处于晚期，患有化疗难治性疾病且病情迅速进展至死亡。活检证实为RLP，存在印戒细胞（SRC），伴有强烈的促纤维增生性间质反应。然而，其不存在特征性的同心圆模式。相反，他有一个巨大的赘生性病变，直肠系膜和盆腔器官有大量肿瘤浸润，伴有主要淋巴结受累。另外2例患者表现为可切除的局部晚期疾病，具有特征性的同心圆模式。新辅助放化疗（CRT）后未观察到临床和放射学反应，其中1例患者手术时为不可切除疾病，另1例患者切除术后病理标本显示疾病分期上升。然而，数据提示存在2种类型的RLP：约一半患者对CRT极度敏感，可获得病理完全缓解，另一半则高度耐药，对CRT无反应。目前的数据不足以区分这2类人群。
同心圆模式的缺失不应排除RLP的怀疑，尤其是在临床表现具有侵袭性的年轻患者中。就放化疗敏感性而言，可能存在2种类型的RLP，新辅助CRT可能会延迟难治性患者的根治性手术。未来需要对肿瘤及其环境进行分子分析，以明确RLP的2种不同形式，从而制定更具个性化的治疗策略。
