Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects.
Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects.
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DOI：
10.1186/1750-1172-9-71
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发表时间：
2014-05-05
影响因子：
3.7
通讯作者：
Morris DL
中科院分区：
文献类型：
--
作者：
Amini A;Masoumi-Moghaddam S;Ehteda A;Morris DL
腹膜假粘液瘤（PMP，ORPHA26790）是一种临床综合征，其特征是腹部和骨盆中粘液性肿瘤和粘液性腹水的进行性播散。 PMP 是一种罕见疾病，估计发病率为百万分之一至二。临床上，PMP通常表现为多种非特异性体征和症状，包括腹痛腹胀、腹水甚至肠梗阻。也可在对腹盆腔内脏进行手术或非手术检查时偶然诊断出来。 PMP 是一种起源于阑尾原发性粘液性肿瘤的肿瘤性疾病，具有独特的腹膜扩散模式。计算机断层扫描和组织病理学是最可靠的诊断方式。该疾病的鉴别诊断包括继发性腹膜癌和一些罕见的腹膜疾病。粘蛋白和粘蛋白分泌肿瘤的最佳消除包括专业中心提供的当前 PMP 护理标准，如内脏切除术和腹膜切除术联合腹膜内化疗。据报道，这种多学科方法的中位生存率为 16.3 年，中位无进展生存率为 8.2 年，10 年和 15 年生存率分别为 63% 和 59%。尽管 PMP 是一种惰性、温和的肿瘤，但它是一种使人衰弱的疾病，严重影响生活质量。它往往在晚期才被诊断出来，并且在治疗后经常复发。然而，在研究中被忽视的 PMP 仍然是一个具有挑战性的、神秘的实体。 PMP综合征的临床病理学特征及其并发症与分泌性粘蛋白聚集物和粘蛋白分泌植入物的多灶性分布密切相关。因此，需要新的策略来促进宏观和微观消除粘蛋白及其作为疾病关键成分的来源。在这方面，已发现MUC2、MUC5AC和MUC5B是与PMP相关的分泌性粘蛋白。粘蛋白靶向疗法的开发可能是本文讨论的未来研究的一个有前途的途径。
Pseudomyxoma peritonei (PMP, ORPHA26790) is a clinical syndrome characterized by progressive dissemination of mucinous tumors and mucinous ascites in the abdomen and pelvis. PMP is a rare disease with an estimated incidence of 1–2 out of a million. Clinically, PMP usually presents with a variety of unspecific signs and symptoms, including abdominal pain and distention, ascites or even bowel obstruction. It is also diagnosed incidentally at surgical or non-surgical investigations of the abdominopelvic viscera. PMP is a neoplastic disease originating from a primary mucinous tumor of the appendix with a distinctive pattern of the peritoneal spread. Computed tomography and histopathology are the most reliable diagnostic modalities. The differential diagnosis of the disease includes secondary peritoneal carcinomatoses and some rare peritoneal conditions. Optimal elimination of mucin and the mucin-secreting tumor comprises the current standard of care for PMP offered in specialized centers as visceral resections and peritonectomy combined with intraperitoneal chemotherapy. This multidisciplinary approach has reportedly provided a median survival rate of 16.3 years, a median progression-free survival rate of 8.2 years and 10- and 15-year survival rates of 63% and 59%, respectively. Despite its indolent, bland nature as a neoplasm, PMP is a debilitating condition that severely impacts quality of life. It tends to be diagnosed at advanced stages and frequently recurs after treatment. Being ignored in research, however, PMP remains a challenging, enigmatic entity. Clinicopathological features of the PMP syndrome and its morbid complications closely correspond with the multifocal distribution of the secreted mucin collections and mucin-secreting implants. Novel strategies are thus required to facilitate macroscopic, as well as microscopic, elimination of mucin and its source as the key components of the disease. In this regard, MUC2, MUC5AC and MUC5B have been found as the secreted mucins of relevance in PMP. Development of mucin-targeted therapies could be a promising avenue for future research which is addressed in this article.
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DOI：
10.1200/edbook_am.2013.33.221
发表时间：
2013-01-01
期刊：
American Society of Clinical Oncology educational book. American Society of Clinical Oncology. Annual Meeting
影响因子：
--
作者：
Buell-Gutbrod, Rebecca;Gwin, Katja
通讯作者：
Gwin, Katja
影响因子：
3.7
作者：
Baratti, Dario;Kusamura, Shigeki;Deraco, Marcello
通讯作者：
Deraco, Marcello
影响因子：
3.4
作者：
Alexander-Sefre, F;Chandrakumaran, K;Moran, B
通讯作者：
Moran, B
影响因子：
2.8
作者：
Chua, Terence C.;Liauw, Winston;Morris, David L.
通讯作者：
Morris, David L.
影响因子：
4
作者：
Amini A;Ehteda A;Masoumi Moghaddam S;Akhter J;Pillai K;Morris DL
通讯作者：
Morris DL