PDF(Pubmed)
Abstract：
Desmoid-type fibromatosis (DF) is a rare subtype of soft tissue sarcomas that most commonly occurs in the anterior abdominal wall. When occurring in the retroperitoneum, DF is usually part of familial syndromes while only rarely sporadic. This makes it imperative to report any instance of experience with DF and the oncological outcomes of the different approaches to management. We report two cases of sporadic and severe DF occurring in the retroperitoneum at our institution.
UNASSIGNED: The first case is a male that presented with urinary obstruction symptoms and underwent surgical resection of the tumor that extended into the left kidney. The second case is a female with a history of recurrent desmoid tumors of the thigh and was incidentally diagnosed with retroperitoneal DF on imaging. She underwent tumor resection and radiotherapy; however, the tumor recurred with urinary obstruction symptoms that required another surgical resection. Histopathological characteristics and radiological imaging of both cases are described below.
UNASSIGNED: Desmoid tumors often recur, thus significantly influencing the quality of life which is reflected in one of our cases. Surgery remains a mainstay treatment, and both cases presented in this report required surgical resection of the tumors as symptomatic and curative measures.
UNASSIGNED: Retroperitoneal DF is a rare entity, and our cases add to the scarce literature available on the topic, which may well contribute to the formulation of practice-changing recommendations and guidelines focused on this rare variant of DF.
UNASSIGNED: The first case is a male that presented with urinary obstruction symptoms and underwent surgical resection of the tumor that extended into the left kidney. The second case is a female with a history of recurrent desmoid tumors of the thigh and was incidentally diagnosed with retroperitoneal DF on imaging. She underwent tumor resection and radiotherapy; however, the tumor recurred with urinary obstruction symptoms that required another surgical resection. Histopathological characteristics and radiological imaging of both cases are described below.
UNASSIGNED: Desmoid tumors often recur, thus significantly influencing the quality of life which is reflected in one of our cases. Surgery remains a mainstay treatment, and both cases presented in this report required surgical resection of the tumors as symptomatic and curative measures.
UNASSIGNED: Retroperitoneal DF is a rare entity, and our cases add to the scarce literature available on the topic, which may well contribute to the formulation of practice-changing recommendations and guidelines focused on this rare variant of DF.
摘要：
纤维样型纤维瘤病（DF）是一种罕见的软组织肉瘤亚型，最常见于前腹壁。当发生在腹膜后，DF通常是家族综合征的一部分，但很少偶发性。这使得必须报告DF的任何经验实例以及不同管理方法的肿瘤学结果。我们报告了两例在我们机构的腹膜后发生的散发性和严重的DF。
■第一个病例是一名男性，表现为尿路梗阻症状，并接受了延伸到左肾的肿瘤的手术切除。第二例是一名女性，有大腿复发性纤维瘤的病史，并在影像学上偶然诊断为腹膜后DF。她接受了肿瘤切除和放疗；然而，肿瘤复发并伴有尿路梗阻症状，需要再次手术切除。两种情况的组织病理学特征和放射学成像如下所述。
■纤维瘤经常复发，从而显著影响生活质量，这反映在我们的一个案例中。手术仍然是主要的治疗手段,本报告中提出的这两个病例都需要手术切除肿瘤作为对症和治愈措施。
■腹膜后DF是一种罕见的实体，我们的案例增加了关于这个主题的稀缺文献，这很可能有助于制定针对这种罕见的DF变体的实践改变建议和指南。
■第一个病例是一名男性，表现为尿路梗阻症状，并接受了延伸到左肾的肿瘤的手术切除。第二例是一名女性，有大腿复发性纤维瘤的病史，并在影像学上偶然诊断为腹膜后DF。她接受了肿瘤切除和放疗；然而，肿瘤复发并伴有尿路梗阻症状，需要再次手术切除。两种情况的组织病理学特征和放射学成像如下所述。
■纤维瘤经常复发，从而显著影响生活质量，这反映在我们的一个案例中。手术仍然是主要的治疗手段,本报告中提出的这两个病例都需要手术切除肿瘤作为对症和治愈措施。
■腹膜后DF是一种罕见的实体，我们的案例增加了关于这个主题的稀缺文献，这很可能有助于制定针对这种罕见的DF变体的实践改变建议和指南。
