Lahat Guy, Nachmany Ido, Itzkowitz Eran, Abu-Abeid Subchi, Barazovsky Eli, Merimsky Offer, Klauzner Joseph
Department of Surgery, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
Isr Med Assoc J. 2009 Jul;11(7):398-402.
Sporadic abdominal desmoid tumors are rare and data on these tumors as a distinct disease entity are lacking. Previous abdominal surgery, trauma, pregnancy and estrogen intake are considered risk factors. Although desmoids are benign, invasion and a high recurrence rate are common.
To evaluate outcomes of surgery for this rare disease.
Since 1995, 16 patients with pathologically confirmed desmoid tumor were operated on in our center. All familial adenomatous polyposis patients were excluded. A retrospective analysis of data was performed.
Of the 16 patients 12 (75%) were females. Mean age was 40.5 years (range 24-70). Thirteen patients were symptomatic and 3 were incidentally diagnosed. All patients presented with an isolated mass; 7 (50%) originated in the abdominal wall, 6 (37.5%) were retroperitoneal and 3 were (18.8%) mesenteric. All tumors except one were completely excised. Morbidity was low with no mortality. One patient was reoperated due to involved margins. None of the patients had recurrence within a median follow-up of 64 months (range 5-143).
The perception of sporadic abdominal desmoids as tumors with a high recurrence rate (20-70%) is probably incorrect. Adequate surgery with wide margins leads to a very low recurrence rate; cure is a legitimate goal.
散发性腹壁硬纤维瘤罕见,且缺乏将这些肿瘤作为一种独特疾病实体的数据。既往腹部手术、创伤、妊娠和雌激素摄入被认为是危险因素。尽管硬纤维瘤是良性的,但侵袭性和高复发率很常见。
评估这种罕见疾病的手术效果。
自1995年以来,我们中心对16例经病理证实的硬纤维瘤患者进行了手术。所有家族性腺瘤性息肉病患者均被排除。对数据进行回顾性分析。
16例患者中12例(75%)为女性。平均年龄为40.5岁(范围24 - 70岁)。13例患者有症状,3例为偶然诊断。所有患者均表现为孤立性肿块;7例(50%)起源于腹壁,6例(37.5%)为腹膜后,3例(18.8%)为肠系膜。除1例肿瘤外,所有肿瘤均被完整切除。发病率低,无死亡病例。1例患者因切缘受累接受了再次手术。在中位随访64个月(范围5 - 143个月)内,无患者复发。
认为散发性腹壁硬纤维瘤具有高复发率(20% - 70%)的观点可能是错误的。进行足够切缘的手术可导致极低的复发率;治愈是一个合理的目标。