Kawashima A, Goldman S M, Fishman E K, Kuhlman J E, Onitsuka H, Fukuya T, Masuda K
Russell E. Morgan Department of Radiology and Radiological Science, Johns Hopkins Medical Institutions, Baltimore, MD 21205.
AJR Am J Roentgenol. 1994 Feb;162(2):339-42. doi: 10.2214/ajr.162.2.8310922.
A retrospective study of abdominal CT scans of patients with proved intraabdominal desmoid tumors was done to determine if any objective characteristics exist to differentiate desmoids related to Gardner's syndrome from isolated desmoids. Because the desmoid tumors of Gardner's syndrome can predate the diagnosis of Gardner's syndrome, it would be helpful to know which patients with desmoids need careful follow-up studies as well as initial workup for Gardner's syndrome and all its ramifications. Also, it would be important to differentiate benign from malignant desmoids associated with Gardner's syndrome. It was hoped that the location, enhancement characteristics, and/or the presence or absence of infiltration might be of value. We were interested in noting if, over time, the growth characteristics of desmoids found in Gardner's syndrome were different from those of isolated desmoids.
We reviewed 101 abdominal CT scans obtained in 23 patients during a 13-year period. Forty desmoid tumors were intraabdominal, including 30 lesions associated with Gardner's syndrome in 13 patients and 10 desmoids of the idiopathic form in 10 patients. These tumors were studied to define location; whether they were single or multiple; and whether they had any specific CT characteristics regarding margins, attenuation numbers, or contrast enhancement.
Desmoid tumors associated with Gardner's syndrome were more likely to be multiple (38%, five of 13 patients) and to involve the mesentery (60%, 18 of 30 tumors) and the abdominal wall (40%, 12 of 30 tumors), whereas isolated desmoid tumors were singular (all 10 patients) and were located in the retroperitoneum (six cases), pelvis (three), and anterior wall (one). Desmoids related to Gardner's syndrome also tended to be smaller (mean diameter, 4.8 cm) than idiopathic desmoids (mean diameter, 13.8 cm). No differentiating CT characteristics regarding margins, attenuation numbers, or response to contrast material were ascertained. Ten new lesions (seven intraabdominal, three mesenteric) developed in three patients with Gardner's syndrome, whereas no new intraabdominal lesions developed in patients with idiopathic desmoids. Follow-up data on 16 surgically resected desmoids in nine patients (seven with Gardner's syndrome and two with isolated desmoids) revealed seven local recurrences (two in the two patients with isolated desmoids and five in two patients with Gardner's syndrome).
No CT characteristics, such as attenuation values, margins, and response to the contrast material, were found that would enable differentiation between isolated intraabdominal desmoids and those associated with Gardner's disease. Desmoid tumors associated with Gardner's syndrome tend to occur in the mesentery and abdominal wall, whereas isolated desmoids involve the retroperitoneum and pelvis. When studying CT scans obtained over time, new lesions were noted to develop in a few of the patients with Gardner's syndrome (three of 13), whereas no new lesions were found in patients with isolated desmoids.
对已确诊的腹内硬纤维瘤患者的腹部CT扫描进行回顾性研究，以确定是否存在任何客观特征来区分与加德纳综合征相关的硬纤维瘤和孤立性硬纤维瘤。由于加德纳综合征的硬纤维瘤可能在加德纳综合征诊断之前出现，因此了解哪些硬纤维瘤患者需要进行仔细的随访研究以及对加德纳综合征及其所有后果进行初步检查将是有帮助的。此外，区分与加德纳综合征相关的良性和恶性硬纤维瘤也很重要。希望肿瘤的位置、强化特征和/或有无浸润可能具有价值。我们感兴趣的是，随着时间的推移，加德纳综合征中发现的硬纤维瘤的生长特征是否与孤立性硬纤维瘤不同。
我们回顾了13年间23例患者的101份腹部CT扫描。40个硬纤维瘤位于腹内，其中13例患者中有30个病变与加德纳综合征相关，10例患者中有10个特发性硬纤维瘤。对这些肿瘤进行研究以确定其位置；是单发还是多发；以及它们在边缘、衰减值或对比增强方面是否有任何特定的CT特征。
与加德纳综合征相关的硬纤维瘤更可能是多发的（38%，13例患者中有5例），并累及肠系膜（60%，30个肿瘤中有18个）和腹壁（40%，30个肿瘤中有12个），而孤立性硬纤维瘤是单发的（所有10例患者），位于腹膜后（6例）、骨盆（3例）和前壁（1例）。与加德纳综合征相关的硬纤维瘤也往往比特发性硬纤维瘤小（平均直径4.8 cm）（平均直径13.8 cm）。未确定关于边缘、衰减值或对造影剂反应的鉴别CT特征。3例加德纳综合征患者出现了10个新病变（7个腹内，3个肠系膜），而特发性硬纤维瘤患者未出现新的腹内病变。对9例患者（7例加德纳综合征患者和2例孤立性硬纤维瘤患者）中16个手术切除的硬纤维瘤的随访数据显示有7例局部复发（2例孤立性硬纤维瘤患者中有2例，2例加德纳综合征患者中有5例）。
未发现能够区分孤立性腹内硬纤维瘤和与加德纳病相关的硬纤维瘤的CT特征，如衰减值、边缘和对造影剂的反应。与加德纳综合征相关的硬纤维瘤倾向于发生在肠系膜和腹壁，而孤立性硬纤维瘤累及腹膜后和骨盆。在研究随时间获得的CT扫描时，注意到少数加德纳综合征患者（13例中有3例）出现了新病变，而孤立性硬纤维瘤患者未发现新病变。
