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- HYUH Case 1 Diagnosis
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kiseokjang 离线
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* In radiologic evaluation and gross exam, This tumor diffusely involved pleural surface, making diffuse pleural thickening and pleural effusion. There is no definitive parenchymal mass except for directly extended foci from pleural mass.
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*Histologic Findings of this tumor
- Solid sheets or nests of large pleomorphic epithelioid cells
- Small amount of fibrocollagenous stroma
- Slitlike or cystlike spaces, Anastomosing channels
- Cystic spaces lined by papillary or micropapillary tumor projections
- Large round to oval pleomorphic nuclei with prominent nucleoli
- Intracytoplasmic vacuoles or lumen
- High mitotic and apoptotic figures
- Confluent necrosis
*Above histologic findings are highly suggestive of malignant mesothelioma and typical features of malignat mesothelioma. Gross findings are fully supportive. I diagnosed this tumor as malignant mesothelioma at first glance, and performed several immunohistochemical markers for confirmation (cytokeratin, vimentin and calretinin). If these markers are positive, I make a diagnosis of malignant mesothelioma. ^^
*But, tumor cells showed no immunoreactivity for cytokeratin and calretinin. After then, I search out differential diagnosis for this tumor as following.
*Differential Diagnosis
• - Epithelioid angiosarcoma
- Epithelioid hemangioendothelioma
- Epithelioid mesothelioma
- Epithelioid hemangioendothelioma
- Epithelioid mesothelioma
• - Pseudomesotheliomatous carcinoma
• - Metastatic adenocarcinoma
* mesothelioma, carcinoma and metastatic carcinoma were already ruled out, because
cytokeratin and calretinin immunostainig were negative.
*Notably, we have to know that vascular tumors have closely similar histologic features of mesothelioma. The only histologic differential point is intracytoplasmic vaculoe and lumen which is containing degenerative red blood cells, in addition to vasoformative growth pattern. Immunohistochemical study should be required.
*Finally, this tumor showed immunoreactivity for vascular markers (CD34, CD31).
Highly anaplastic, epithelioid tumor cell morphology, necrosis and active mitosis suggest a epithelioid angiosarcoma rather than hemangioendothelioma.
*In addition, I can find basal lamina around the rumor cell and Weibel-Palade bodies in the cytoplasm on electron microscopic examination.
***Primary Angiosarcoma of the Pleura
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- Extremely rare in the pleura
- Extremely rare in the pleura
•- Age: 22~79 yrs (mean: 57 yrs)
•- Male predominance
- Cause: Tuberculous pyothorax, previous irradiation, asbestos exposure
- Cause: Tuberculous pyothorax, previous irradiation, asbestos exposure
••- Clinical presentation: Diffuse pleural thickening and effusion
--> Clinically and radiologically prompting the diagnosis of mesothelioma in most cases
--> Clinically and radiologically prompting the diagnosis of mesothelioma in most cases
•- Regional lymph node metastasis
– --> Angiosarcoma: 5/46 (10.9%) cf. 3.4% of all soft tissue sarcoma
•- Prognosis: Fatal
– --> Patients died few months after diagnosis of angiosarcoma
***Differential Diagnosis
***Differential Diagnosis
- Immunohistochemistry
– - Mesothelial markers (calretinin, hBME, cytokeratin)
– - Epithelial marker (cytokeratin, TTF-1)
• -- All mesotheliomas and adenocarcinomas are strongly positive for cytokeratin
• -- Cytokeratin may be weakly positive in the epithelioid type of angiosarcoma
– - Endothelial cell markers (CD31, CD34, Factor VIII, vWF, Ulex Europaeus)
• -- When mesothelial and epithelial marker is negative or focal, weakly positive
• -- Vascular marker panel should be applied
•
- Electron microscopy
- Electron microscopy
– -- Adenocarcinoma: microvilli
– -- Mesothelioma: microvilli (long, narrow and slender)
– -- Vascular tumor (basal lamina around the cell border, lumen formation, Weibel-Palade body)
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本帖最后由 于 2007-03-14 20:20:00 编辑
 HYUH Case 1最后诊断放射学和大体观察，该肿瘤弥漫浸润胸膜表面，致胸膜弥漫增厚和渗出。肿瘤大体表现为胸膜肿物的延伸性病灶，没有明确的实性团块形成。
组织学呈如下表现：
大而多形性上皮样细胞呈实性片状或巢状排列
少量纤维胶原间质
裂隙状或囊样空腔呈网状交通
囊腔内附乳头和微乳头样肿瘤突起
核大、圆形或卵圆形或多型性；核仁显著
胞浆内空泡，小腔
核分裂象多见；可见凋亡特征
融合坏死灶
以上组织学表现高度提示：该肿瘤具有恶性间皮瘤的特点，可能为恶性间皮瘤。大体观察也支持。我第一印象诊断为恶性间皮瘤，为了证实这点，行cytokeratin, vimentin 及免疫组化。如果这些标记为阳性，那么就可以诊断为恶性间皮瘤。
结果cytokeratin和calretinin均为阴性。此后，又作出以下鉴别诊断：
上皮样血管肉瘤
上皮样血管内皮细胞瘤
上皮样间皮瘤
假间皮瘤样癌
转移性腺癌
因cytokeratin和calretinin均为阴性，间皮瘤、癌及转移性癌均被排除。
众所周知，血管性肿瘤与间皮瘤在组织学特点上很相似。除了做为一种血管形成模式，该例唯一鉴别点是胞浆内空泡，这些空泡包含了蜕变的红细胞。为明确诊断，应进一步免疫组化。结果，血管标记CD34及CD31均为阳性。高度间变、上皮样肿瘤细胞形态、坏死及活跃的核分裂像提示该肿瘤更可能是上皮样血管肉瘤，而非上皮样血管内皮细胞瘤。而且通过电镜观察，在肿瘤细胞周围可见到基底膜，胞浆内可见到Weibel-Palade体。
原发性胸膜血管肉瘤知识点：
胸膜及其少见
年龄：22-79岁（平均57岁）
男性多见
病因：结核性脓胸，放射或石棉接触史
临床表现：弥漫性胸膜增厚及渗出，因而，在多数病例中，临床及放射学都支持间皮瘤的诊断。
区域淋巴结转移：血管肉瘤转移率5/46 (10.9%)，而所有软组织肿瘤总的转移率3.4% 。
预后：很差，患者多于血管肉瘤诊断明确后数月死亡。
诊断要点
免疫组化：
间皮标记物（calretinin, hBME, cytokeratin）及上皮标记物（cytokeratin, TTF-1）：所有间皮瘤及癌cytokeratin标记呈强阳性。Cytokeratin可能在上皮样血管肉瘤中呈弱阳性表达。
内皮标记物(CD31, CD34, Factor VIII, vWF, Ulex Europaeus)：如果间皮或上皮标记呈阴性或局灶弱阳性，则应进行血管标记。
电镜观察：
腺癌：微绒毛结构。
间皮瘤：微绒毛结构（长、窄而细）。 血管肿瘤：基膜围绕细胞周围，胞浆内有小腔管结构，Weibel-Palade小体可见。 |
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