Touloukian R J, Maharaj A, Ghoussoub R, Reyes-Mugica Miguel
Section of Pediatric Surgery, Yale University School of Medicine, New Haven, CT, USA
Section of Surgical Pathology, Yale University School of Medicine New Haven, CT, USA
J Pediatr Surg. 1997 Feb;32(2):272-4. doi: 10.1016/s0022-3468(97)90193-7.
The presence of squamous epithelium in the cyst wall of children with large splenic cysts has generally been thought to require complete resection of the cyst lining to prevent recurrence. The risks of major bleeding or possible total splenectomy necessitates a simplified procedure to preserve splenic function without cyst recurrence.
Six patients treated for splenic cyst over a 10-year period underwent partial splenic decapsulation retaining the hilar portion of the spleen. The patient group, including five girls, ranged in age from 6 to 18 years (mean, 13 years). Presenting findings were splenomegaly (n = 4), progressive cyst enlargement (n = 4), left upper quadrant pain (n = 2), ultrasound for unrelated condition (n = 2), and prior history of trauma (n = 1). Cyst location varied from peripheral to deep cortical and ranged in size from 5 to 20 cm containing from 200 to 1,700 mL of sterile straw-colored to dark brown-colored liquid.
Palpable splenomegaly resolved in all patients with progressive decrease in size of the splenic remnant, and return of vascularity to normal was confirmed by Doppler ultrasound. Each patient remains asymptomatic without recurrence of the splenic cyst from 1 to 10 years after surgery. Squamous epithelial lining cells found in sheets and clusters were seen within the cyst lining of all patients. Immunohistochemistry of the paraffin-embedded tissue showed that these cells expressed keratin, epithelial membrane antigen (EMA), carcino-embryonic antigen (CEA), but were negative for BerEP4 (conventionally positive in cells of epithelial origin).
The authors conclude that splenic decapsulation is an effective treatment for splenic epithelial cysts, which both preserves splenic function and prevents recurrence despite retention of hilar cyst lining. Epithelial metaplasia of the mesothelial-mesodermal undifferentiated cells from exposure to an unidentified irritant may explain the histological presence of squamous epithelium within the cyst wall.
一般认为，巨大脾囊肿患儿的囊肿壁中存在鳞状上皮，这需要完整切除囊肿内衬以防止复发。大出血或可能进行全脾切除术的风险使得有必要采用一种简化手术来保留脾功能且不复发囊肿。
在10年期间接受脾囊肿治疗的6例患者接受了保留脾门部分的部分脾被膜剥除术。患者组包括5名女孩，年龄在6至18岁之间（平均13岁）。呈现的症状有脾肿大（4例）、囊肿进行性增大（4例）、左上腹疼痛（2例）、因无关病症行超声检查（2例）以及既往有外伤史（1例）。囊肿位置从周边到深部皮质不等，大小从5至20厘米，囊内含有200至1700毫升无菌稻草色至深褐色液体。
所有患者可触及的脾肿大均消失，脾残余体积逐渐减小，多普勒超声证实血管恢复正常。每位患者术后1至10年均无症状，脾囊肿未复发。在所有患者的囊肿内衬中均可见成片和成群的鳞状上皮衬里细胞。石蜡包埋组织的免疫组织化学显示，这些细胞表达角蛋白、上皮膜抗原（EMA）、癌胚抗原（CEA），但对BerEP4呈阴性（通常上皮来源细胞呈阳性）。
作者得出结论，脾被膜剥除术是治疗脾上皮囊肿的有效方法，尽管保留了囊肿壁的脾门部分内衬，但该方法既能保留脾功能又能防止复发。间皮 - 中胚层未分化细胞因接触不明刺激物而发生上皮化生，这可能解释了囊肿壁内鳞状上皮的组织学存在。
