肾上腺皮质癌、肝肾透明细胞癌、乳腺富脂质癌等。

肺部肿瘤，似位于被膜下，呈分叶样多灶性分布，与周边肺泡组织分界清晰；灶状区域为较细纤维血管分割的上皮样实性细胞团（部分区域也象实性膨胀的乳头样结构）或为少部分区域的腺泡样结构。细胞学为胞浆空亮核较小温和的透明性上皮样细胞（为主）或见少量散在嗜双色上皮样细胞。

患者肾脏、肾上腺、肝脏、卵巢、子宫、头颈部临床和CT、B超检查均未找到可疑肿瘤。

问题：

1、是否为肺的原发肿瘤？支气管肺泡癌的特殊亚型？

2、现在所见仍然不能除外肺转移性肿瘤，是否有早些时候的手术史呢？

非常好的诊断思路，请您能否从上述各部位透明细胞肿瘤的形态特点鉴别一下？谢谢！

看到Dr.XLJin的问题，感到很难回答的全面正确，渴望老师在后面的时间更好的总结。

总体感觉透明细胞肿瘤的概念和肿瘤细胞的透明细胞改变都是很宽大和广泛的概念，涉及的内容非常多，自己大脑有些转动不灵的感觉。结合网上的搜索和记忆，无章法的写写，不好意思啊。

1、先把问题复杂化：

上皮性透明细胞样恶性肿瘤

头颈部上皮来源

Salivary clear cell adenocarcinoma

Thyroid clear cell adenocarcinoma

泌尿系统

Renal clear cell carcinoma

Bladder clear cell adenocarcinoma

女性生殖系统及乳腺

Ovarian clear cell adenocarcinoma

Cervix clear cell adenocarcinoma

Endometrium clear cell adenocarcinoma

glycogen-rich clear cell carcinoma of breast

男性生殖系统

Prostate clear cell adenocarcinoma

呼吸系统

Lung clear cell carcinoma

mucoepidermoid carcinoma

消化系统

Pancreas clear cell adenocarcinoma

Liver clear cell carcinoma

Colone clear cell adenocarcinoma
肾上腺

Clear cell adrenal cortical carcinoma.

皮肤附件

Sweat gland clear cell carcinoma

sebaceous carcinoma

上皮性的透明细胞样良性肿瘤

以上除特殊部位如肾脏的良性透明细胞样肿瘤。

肌上皮来源的良性与恶性透明细胞样肿瘤

间叶来源的透明细胞良性与恶性肿瘤。如脂肪、软骨、平滑肌、血管、来源不明的等

淋巴造血系统。

神经及神经外胚叶肿瘤。恶黑，神经鞘来源

生殖细胞来源。

以上的可能肿瘤，共性的是以细胞学的透明胞浆细胞为主体或部分肿瘤细胞胞浆透明。个性的是不同肿瘤的细胞核特征、异形性、核分裂、核仁等有所不同也有相同（有待学习）；胞核的位置和和核浆的比例有不同（有待学习）；细胞的排列方式，主体细胞构架和组织结构，与间质或血管的关系等有不同。

2、再把问题具体化（这可能是Dr.XLJin老师的问题要点）：

结合本例的肿瘤分叶样多灶性分布，与周边肺泡组织分界清晰；灶状区域为较细纤维血管分割的上皮样实性细胞团（部分区域也象实性膨胀的乳头样结构）或为少部分区域的腺泡样结构。细胞学为胞浆空亮核较小温和的透明性上皮样细胞（为主）或见少量散在嗜双色上皮样细胞。

实际上形态学还是倾向于上皮来源的透明细胞样恶性肿瘤。所以主要与以下的几个透明细胞样的肿瘤鉴别。

A 透明细胞肾细胞癌：Many tumors demonstrate a predominant compact alveolar architecture （腺泡状结构为主）(Fig 5). Tubular or cystic areas are commonly associated with the alveolar pattern. Focal papillarity is not rare, but a predominantly papillary architecture is almost never associated with clear cell RCC; such tumors are most likely chromophil RCC（可见乳头样结构但不是主体）. Most clear cell RCC contains numerous capillaries and thin-walled blood vessels in the supporting stroma（薄壁血管和毛细血管丰富） — a helpful diagnostic feature that is usually retained when the tumor metastasizes. The cytoplasm of many tumor cells is rich in lipids and glycogen（胞浆透明充满脂质和糖原）, which dissolve during processing and provide the characteristic clear cytoplasm. The cytoplasm of adrenal cortical cells from the zona fasciculata, although similar, is foamy.（与束状带肾上腺皮质细胞相似但后者是泡沫样）

Fig 5. — Clear cell RCC. Tumor cells with abundant clear cytoplasm and an alveolar pattern of growth (hematoxylin-eosin, x 600).

本例与典型的透明细胞肾细胞癌很相似。

2、肾上腺皮质腺癌

Adrenocortical Carcinoma

摘自阿克曼病理学八版：镜下，这些肿瘤的分化范围很广。一些肿瘤细胞与正常肾上腺皮质细胞非常相似，细胞浆以小的合有脂质的空泡为特征，某些空泡使位于中部的细胞核形成凹

陷，类似于皮脂腺细胞和脂肪母细胞。这些细胞有时也被称作海绵状细胞或透明细胞。（此段位描述正常肾上腺皮质细胞特点），而另外一些肿瘤细胞则完全未分化，可见具有丰富的嗜酸性胞浆和奇异深染细胞核的巨细胞及多核巨细胞。在某种情况下，可以见到大量的中性粗细胞，其中绝大多数在间质中，另外一些在肿瘤巨细胞的脑浆中。一种肿瘤变型，具有癌和肉瘤样成分的双向形态特征．称为‘癌肉瘤‘。也见过肾上腺皮质癌的嗜酸细胞变型，以及肿瘤伴有腺鳞分化的报告。

在文献上看到有透明细胞型的肾上腺皮质腺癌，但是没有找到原文。

本例与高分化的肾上腺皮质腺癌有相似性，但少见乳头样结构。

3、肝细胞透明细胞癌

Microscopically, all cases of HCC-CC showed moderate-to-marked cytoplasmic accumulation of

glycogen and/or macro- and microvesicular intracytoplasmic fat droplets that dissolved during processing,leaving behind a “clear” cytoplasm. The tumor growth patterns ranged from sheets of cells

to trabeculae with or without pseudoglands, or a combination of patterns (Fig. 1).

和本例有相似性。

4、卵巢透明细胞癌

Growth patterns:

tubular–cystic ，papillary；solid sheet；Stroma-rich variants:clear cell adenocarcinofibroma，cystadenocarcinofibroma。

Tumor cells:

Large ，nuclei: some protrude into lumina, resulting in hobnail configuration ；cytoplasm:clear，often contains:glycogen，mucin，fat，may be PAS-positive diastase-resistant hyaline globules，may appear oxyphilic rather than clear。

和本例不是很相符。

5、子宫内膜透明细胞腺癌

clear cell adenocarcinoma of endometrium：癌细胞呈实性细胞团、大小不等的囊泡状结构，乳头状结构，细胞学胞浆空亮，边界清晰，胞核异型性明显，可见鞋钉样癌细胞，胞核周围内陷。

与本例不是很相符。

6、甲状腺透明细胞癌。

Clear cell carcinoma of the thyroid is an uncommon tumour which can have a follicular, papillary or mixed The clear cytoplasm is most likely due to different etiologic factors: (a) glycogen in some cases6 or (b) distended empty mitochondria in others. The clear cell thyroid tumours with a follicular or mixed pattern are thought to behave like a follicular carcinoma.’

7、胰腺透明细胞癌

The histologic features of clear cell carcinoma of the pancreas have varied in the previous reports. Kanai et al. (7) first reported on a case of clear cell carcinoma and the tumor was predominantly composed of a clear cell component（透明细胞成分）. The tumor in that case had a small amount of extracellular mucin formation （细胞外粘液形成）and the neoplastic cells seemed to have scant intercellular adhesiveness; this histologic configuration appears to be similar to that of signet-ring cell carcinoma（类似印戒细胞癌）. The tumor in the case of Luttges et al. (8) was predominantlycomposed of a clear cell component and it had one area of a papillotubular component（具有乳头导管结构）. Jamali et al. (9) reported a case of adenosquamous carcinoma of the pancreas with both clear cell and rhabdoid components（横纹肌样成分）, and this was similar to our case. Rhabdoid cells have been recognized in a wide range of epithelial and mesenchymal tumors. The rhabdoid phenotype is characterized by a large round to polygonal cell with eccentric nuclei, prominent nucleoli and abundant deeply acidophilic/eosinophilic, dot-like, hyaline filamentous cytoplasmic inclusions. The rhabdoid phenotype represents a common clonal dedifferentiated end point in malignant tumors of varying histogenesis (12). It has been recently suggested that rhabdoid changes may be a type of degeneration or a preliminary stage before apoptosis or cell necrosis。

与本例有一定的区别。

8、乳腺

Microscopic examination showed an invasive adenocarcinoma of the breast, characterized by average-sized cells,with well-defined borders and polygonal, rather than rounded contours. The neoplastic cells formed a matrix of solid, lobular, acinar and rarely papillary areas, with a fine vascular network in between (Figure 2). Foci of linear,trabecular and tubular growth patterns were visible. A few ducts with an intraductal (in situ) carcinoma of solid type were also noticed. The cytoplasm was clear and eccentrically placed and hyperchromatic nuclei with a low mitotic number (2 mitoses per 10HPF) were detected. Cells with mildly eosinophilic cytoplasma, nuclear pleomorphism and  higher mitotic number were also seen.

有相似性与本例。