Kurian Kathreena M, Al-Nafussi A
Pathology Department, University of Edinburgh, 37 Liberton Brae, Edinburgh EH16 6AG, UK.
Histopathology. 2002 Jan;40(1):58-64. doi: 10.1046/j.1365-2559.2002.01319.x.
To analyse the clinical and pathological features with long-term follow-up of a series of 12 cases of sarcomatoid carcinoma of the breast. methods and results: The cases were selected from the surgical files of the Department of Pathology, University of Edinburgh, between 1977 and 1988. The following clinical parameters were recorded: the age of the patients, size of tumour, presence or absence of lymph node or distant metastases, and patient survival. Pathological assessment included: the type of epithelial and mesenchymal components, the proportion of monophasic to biphasic tumours and the presence of adjacent in-situ carcinoma/atypical epithelial proliferation. The mean age of the patients was 61 years with a median of 64 and range 46-82 years. The mean size of the tumour was 52 mm (range 22-100 mm). None of the patients had distant metastasis at presentation and only one case had local lymph node metastasis which had a carcinomatous appearance. Five women were still alive after a minimum 12-year follow-up period. Four patients died of their disease (three with lung metastasis only and one with lung and bone metastases), one died of carcinoma of the cervix and two patients were lost to follow-up. Pathologically, four cases (33.3%) had no or almost undetectable epithelial structures by light microscopy, i.e. "monophasic sarcomatoid carcinoma". The remaining cases revealed varying proportions of both epithelial and mesenchymal elements, i.e. "biphasic sarcomatoid carcinoma". Of the epithelial component, six (50%) tumours had predominantly carcinoma of no special type, one lobular and one tubular carcinoma. The mesenchymal component was fibromatosis/nodular fasciitis-like, malignant fibrous histiocytoma-like (MFH), osteosarcoma-like and fibrosarcoma-like in five (42%), four (33%), two (17%) and one (8%) tumours, respectively. In 3/4 monophasic tumours, the mesenchymal component was of a low-grade fibromatosis/nodular fasciitis type. In 6/12 (50%) of the cases there was associated in-situ atypical epithelial proliferation (five ductal carcinoma in situ (DCIS) and one atypical ductal hyperplasia).
From this small series it appears that sarcomatoid carcinoma is an uncommon tumour, which is large in size and tends to lack local or distant metastasis at presentation. Pathologists should be alert to the presence of the bland monophasic sarcomatoid carcinoma which has a pure mesenchymal appearance on light microscopy, but epithelial components demonstrated by cytokeratin immunohistochemistry. These showed metastases on long-term follow-up, similar to other histological patterns of sarcomatoid carcinoma.
对12例乳腺肉瘤样癌患者进行长期随访,分析其临床和病理特征。方法与结果:病例选自1977年至1988年爱丁堡大学病理科的手术档案。记录以下临床参数:患者年龄、肿瘤大小、有无淋巴结或远处转移以及患者生存情况。病理评估包括:上皮和间充质成分的类型、单相与双相肿瘤的比例以及相邻原位癌/非典型上皮增生的存在情况。患者的平均年龄为61岁,中位数为64岁,范围为46 - 82岁。肿瘤的平均大小为52毫米(范围为22 - 100毫米)。所有患者初诊时均无远处转移,仅有1例有局部淋巴结转移,表现为癌性外观。经过至少12年的随访期后,5名女性仍然存活。4例患者死于疾病(3例仅有肺转移,1例有肺和骨转移),1例死于宫颈癌,2例失访。病理上,4例(33.3%)在光学显微镜下无或几乎检测不到上皮结构,即“单相肉瘤样癌”。其余病例显示上皮和间充质成分比例各异,即“双相肉瘤样癌”。在上皮成分中,6例(50%)肿瘤主要为非特殊类型癌,1例为小叶癌,1例为管状癌。间充质成分在5例(42%)、4例(33%)、2例(17%)和1例(8%)肿瘤中分别为纤维瘤病/结节性筋膜炎样、恶性纤维组织细胞瘤样(MFH)、骨肉瘤样和纤维肉瘤样。在3/4的单相肿瘤中,间充质成分为低级别纤维瘤病/结节性筋膜炎类型。在6/12(50%)的病例中存在相关的原位非典型上皮增生(5例导管原位癌(DCIS)和1例非典型导管增生)。结论:从这个小系列病例来看,肉瘤样癌是一种罕见肿瘤,体积较大,初诊时往往缺乏局部或远处转移。病理学家应警惕平淡的单相肉瘤样癌的存在,其在光学显微镜下具有纯间充质外观,但细胞角蛋白免疫组化显示有上皮成分。这些病例在长期随访中出现转移,与肉瘤样癌的其他组织学模式相似。