发生于腹膜的恶性间皮瘤。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {上皮性间皮瘤分为弥漫型、高分化乳头型、合胞体型。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {比较少见的类型为肉瘤样型包括促纤维增生型。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {

年龄和性别弥漫性间皮瘤发生的平均年龄为50岁，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m高分化乳头型间皮瘤的平均年龄为58岁。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {

发病率和死亡率与发生于女性Müllerian系统的良性及恶性肿瘤比较，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m腹膜原发性肿瘤罕见。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {临床上，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m良性和低度恶性的多囊性间皮瘤及高分化乳头型间皮瘤比恶性弥漫性间皮瘤常见。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {而且，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m卵巢外原发性或继发性浆液性癌也比恶性弥漫性间皮瘤常见。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {

病因学临床上，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m一部分高分化乳头型、弥漫型、合胞体型间皮瘤与接触石棉有关。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {

最常见的临床表现为腹水和腹痛。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {

典型者为多结节性，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m结节直径＜1.5 cm。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {肉眼上，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m与相对常见的癌的腹膜播散（carcinomatosis）及卵巢外腹膜原发性癌难以鉴别。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {

高分化乳头型间皮瘤和弥漫性恶性间皮瘤是最常见的组织学类型。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {典型者由一致性的瘤细胞组成，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m瘤细胞胞质丰富、嗜酸性。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {上皮样间皮瘤的另一种变异型为合胞体型，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m与异位的旺盛的蜕膜样反应相似。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {腹膜也可发生肉瘤样型包括促纤维增生型间皮瘤，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m但远较发生于胸膜的少见。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {

所有高分化乳头型间皮瘤都有明显的灶性的、分化好的乳头状结构，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m或管状乳头状结构，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m由一致性单层细胞组成乳头或管状结构。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {瘤细胞为良性的立方状或扁平的间皮细胞，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m核分裂罕见。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {偶尔，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m细胞可出现轻度非典型性。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {常见大量纤维组织内不规则的腺样结构，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m可能易与灶性恶性间皮瘤或腺癌混淆。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {部分病例出现砂砾体。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {

〖鉴别诊断〗

恶性间皮瘤最重要的特征是瘤组织浸润脂肪组织和脏器的管壁。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {但有时在小的活检标本中很难判定浸润。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {在腹膜比较常见的良性细胞陷入肉芽组织或脂肪内，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m可能误诊为恶性间皮瘤的浸润灶。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {

肉眼和组织学上，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m腹膜恶性弥漫性间皮瘤可能表现为癌的生长方式，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m因此，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m可能与腹膜原发性浆液性乳头状肿瘤混淆。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {在这种情况下，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m免疫组织化学检测瘤细胞钙网蛋白和Ber-EP4的表达很有帮助，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m而间皮细胞的其他标记物敏感性很低。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {高分化乳头状间皮瘤无复层的乳头状结构、细胞的非典型性及核分裂，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m而这些特点常为浆液性腺癌的特点。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {此外，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m根据是否有腹腔手术病史或其他可引起腹膜增生性变化的病史，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m可将高分化乳头状间皮瘤与间皮增生相鉴别。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {

典型的弥漫性上皮性间皮瘤为高度侵袭性。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {然而，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m与胸膜间皮瘤不同，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m有相当一部分病例临床呈相对惰性。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {无法划分预后好与不好的形态学特征，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m与弥漫型间皮瘤比较，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m高分化乳头型间皮瘤常为局限性，. [+ y) `; c' Z4 B0 C' _, ]+ R* f2 B8 U+ m预后相对较好。+ j2 {; H6 Z, O- X* v6 C2 x; ]4 `4 {