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| 名称： | |
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- 左上腹肿块
| 姓 名： | ××× | 性别： | 女 | 年龄： | 44岁 |
| 标本名称： | 左上腹肿块 | ||||
| 简要病史： | 发现左上腹肿块一周。术中见左肾下极巨大肿块25*20* | ||||
| 肉眼检查： | 不整形组织一块18*14* | ||||
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标签：腹膜后 低度恶性神经鞘膜瘤 脂肪肉瘤
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本帖最后由 于 2006-11-12 23:17:00 编辑
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×参考诊断
上海肿瘤医院会诊：低度恶性神经鞘膜瘤
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A retroperitoneal tumor of such large size and histology (spindled cells) has to raise the possibility of dedifferentiated liposarcoma. I urge that any "fat" attached to the tumor be submitted for microscopic examination to detect any feature for a pre-existing and residual low grade liposarcoma. Retroperitoneal liposarcomas are not rare, and are the most common large retroperitoneal tumors in adults. Most of them are low grade or well differentiated liposarcoma (rather than the myxoid or pleomorphic variants of liposarcomas). Microscopically, retroperitoneal well differentiated liposarcomas appear identical to the well circumscribed atypical lipomatous tumors (atypical lipomas) of neck, shoulder, upper back and other regions in extremities. However, retroperitoneal well-differentiated liposarcomas are slow-growing, very large, not encapsulated and very difficult to resect completely due to infiltrative borders.
A minority of retroperitoneal liposarcomas may, either at the time of initial diagnosis or at the time of local recurrence, contain nodule(s) of non-lipogenic sarcomatous elements. Most of these non-lipogenic elements appear like fibrosarcoma or MFH, and often display features of high grade sarcoma. Sometimes, the sarcomatous elements are that of a low grade fibrosarcoma (consisting exclusively of spindled cells). Very rarely, features of high grade osteosarcoma, leiomyosarcoma, chondrosarcoma, rhabdomyosarcoma and malignant peripheral nerve sheath tumor are found. These tumors are known as dedifferentiated liposarcomas. They are presumed to evolve from the pre-existing well differentiated liposarcomas. Unlike well differentiated liposarcomas, dedifferentiated liposarcomas of the retroperitoneum have definite metastatic potential (in 1~18% of cases). No matter what kinds of sarcomatous elements are found in a retroperitoneal dedifferentiated liposarcoma, its clinical behavior is not the same as high grade fibrosarcomas, leiomyosarcomas, osteosarcomas, chondrosarcomas, rhabdomyosarcomas, or MPNST. Dedifferentiated liposarcomas are not unique to the retroperitoneum. Another common location for it is the inguinal area and spermatic cord.
聞道有先後,術業有專攻
| 以下是引用mjma 在2006-11-13 1:56:00的发言： A retroperitoneal tumor of such large size and histology (spindled cells) has to raise the possibility of dedifferentiated liposarcoma. I urge that any "fat" attached to the tumor be submitted for microscopic examination to detect any feature for a pre-existing and residual low grade liposarcoma. Retroperitoneal liposarcomas are not rare, and are the most common large retroperitoneal tumors in adults. Most of them are low grade or well differentiated liposarcoma (rather than the myxoid or pleomorphic variants of liposarcomas). Microscopically, retroperitoneal well differentiated liposarcomas appear identical to the well circumscribed atypical lipomatous tumors (atypical lipomas) of neck, shoulder, upper back and other regions in extremities. However, retroperitoneal well-differentiated liposarcomas are slow-growing, very large, not encapsulated and very difficult to resect completely due to infiltrative borders. |
腹膜后肿瘤,具有如此体积和组织学形态(梭形细胞)让我们不得不考虑去分化脂肪肉瘤的可能性。我强烈建议将任何与该肿瘤相连的脂肪组织送做镜检以发现已有和残留的低级别脂肪肉瘤的特征。腹膜后脂肪肉瘤并不罕见，是成人腹膜后肿瘤中的一个最常见类型。大部分都属于低级别或分化良好型脂肪肉瘤（而非粘液型或多形性脂肪肉瘤）。显微镜下，腹膜后分化良好型脂肪肉瘤在形态上与发生在颈、肩、上背和四肢其他部位的境界清楚的非典型脂肪瘤相似。但是腹膜后分化良好型脂肪肉瘤生长缓慢，体积可以很大，无包膜，由于边界不清很难切除干净。
少数腹膜后脂肪肉瘤可以在初次诊断或局部复发时发现局灶区非脂肪肉瘤的肉瘤成分。大部分非脂肪肉瘤成分镜下很像纤维肉瘤或恶性纤维组织细胞瘤，并且通常显示的是高级别肉瘤的特征。罕见的情况下可以找见高级别骨肉瘤、平滑肌肉瘤、软骨肉瘤、横纹肌肉瘤和恶性外周神经鞘膜瘤的特征。这样的肿瘤被称为去分化的脂肪肉瘤。它们被认为是在已有的分化良好型脂肪肉瘤的基础上发展而来的。与分化良好型脂肪肉瘤不同，腹膜后的去分化脂肪肉瘤具有一定的转移倾向（占病例数的1－18％）。无论在腹膜后去分化型脂肪肉瘤中发现何种类型的其他肉瘤成分，它的临床行为都与高级别纤维肉瘤、平滑肌肉瘤、骨肉瘤、软骨肉瘤、或恶性外周神经鞘膜瘤不同。腹膜后并不是去分化脂肪肉瘤发生的唯一部位。其他常见部位包括腹股沟区和精索。
非常同意 马教授的观点，此例从细胞排列、形态以及间质特点来看，都需要首先考虑去分化脂肪肉瘤的可能。免疫组化在区分去分化脂肪肉瘤与外周神经肿瘤方面好像并没有太大帮助，多取材找相对典型区域可能帮助更大。最近有文献报道MDM－2以及CDK-4在去分化脂肪肉瘤阳性率较高，我们用的结果也还可以。就此例形态来讲主要是去分化脂肪肉瘤、MFH、MPNST之间的鉴别，但有文献报道腹膜后MFH多数可能都是去分化脂肪肉瘤，如果此结论可信的话，这例主要是去分化脂肪肉瘤和MPNST之间的鉴别了。
建议再多取材找找有无相对典型一些的区域。再追问一下以往有无手术史，如果有的话可以复查一下原切片。
- the more we discuss, the more we learn from each other !!
