Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0677930 (primary tumor)
20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Malignant mixed mesodermal tumor is a rare tumor of the ovary and its histogenesis is controversial. We report the case of an ovarian tumor that seemed to be a pure carcinoma and recurred as a carcinosarcoma, and suggest a possible histogenesis for this kind of tumor. The patient was a 62-year-old Japanese woman. The primary tumor was confined to the right ovary and was a histologically poorly differentiated endometrioid adenocarcinoma with focal squamous differentiation. The tumor recurred as peritoneal dissemination 9 months later showing a histological appearance of carcinosarcoma of heterologous type. The recurrent tumor also contained intermingled foci of similar histology as the primary tumor. The carcinomatous component of the recurrent tumor showed more obvious differentiation to adenocarcinoma with increased expression of epithelial markers compared to the primary tumor. Epithelial membrane antigen was positive also in a few cells of the sarcomatous component, which implies that this tumor had features of metaplastic carcinoma. The DNA ploidy pattern of the primary ovarian tumor was diploid, while an additional aneuploid subpopulation appeared in the recurrent tumor. These findings suggest the possible histogenesis of carcinosarcoma of the ovary as progression and clonal evolution of endometrioid adenocarcinoma.
...
PMID:Ovarian carcinoma recurring as carcinosarcoma. 1142 97

Metaplastic carcinoma of the breast, a neoplasm with both epithelial and mesenchymal elements, represents less than 1 per cent of all breast cancer. We reviewed the records of all patients diagnosed with localized metaplastic breast cancer from 1991 to 2003 at our institution. We identified 21 patients. Mean primary tumor size was 4.62 cm. Eight patients (38%) had axillary node involvement at presentation. All the tumors were high grade. Only two (10%) of the tumors were hormone receptor positive. Seventeen (81%) of the patients received adjuvant chemotherapy, and 12 (57%) of the patients received radiation. Ten (29%) patients suffered a local recurrence. With a mean follow-up of 46 months, the 5-year disease-free and overall survival was 42 per cent (95% CI: 20% to 65%) and 71 per cent (95% CI: 46% to 96%), respectively. Stage-specific overall survival was 100 per cent, 83 per cent, and 53 per cent for stages I, II, and III, respectively. By multivariate analysis, there was no impact on recurrence or survival with regard to size, age, menopausal status, nodal status, histologic subtype, adjuvant therapy, or extent of surgery. Metaplastic breast cancer is a unique neoplasm that tends to present at an advanced stage and has a propensity for local recurrence. When stratified by stage, however, survival appears similar to that of adenocarcinoma of the breast, and these tumors should be treated as such.
...
PMID:Metaplastic breast cancer: clinical features and outcomes. 1646 6

Carcinosarcoma of the breast, often referred to as metaplastic carcinoma of the breast, is a rare malignancy with two distinct cell lines described as a breast carcinoma of ductal type with a sarcoma-like component. Clinically, carcinosarcoma of the breast is an aggressive breast cancer. The prognosis for carcinosarcoma of the breast is less favorable compared to more common types of breast cancer such as infiltrating ductal or lobular carcinoma. Currently, the evaluation of breast carcinoma includes hormone receptor analysis of the tumor tissue, with those positive for estrogen or progesterone responding better to both hormonal and chemotherapy.Trastuzumab (Herceptin(R)) is available as an adjunct treatment for tumors which over-express the HER2/neu gene. Typically, metaplastic carcinomas of the breast do not express the estrogen or progesterone receptors and do not over-express the HER2/neu oncogene. As a result of this "triple negative" phenotype, such tumors tend to be more aggressive and are unlikely to respond to targeted therapy with Herceptin. The epidermal growth factor receptor HER-1/EGFR protein is expressed in the majority of metaplastic carcinomas and thus may serve as a potential therapeutic target for EGFR inhibitors such as gefitinib and cetuximab. The two cases we describe exemplify the aggressive nature of carcinosarcoma of the breast and support the findings that this tumor type does not express the common receptors found in other breast carcinomas. These case reports also emphasize the need for investigating the role for blockade of the HER-1/EGFR receptor with targeted therapies when found to be over-expressed in the primary tumor.
...
PMID:Carcinosarcoma of the breast: two case reports and review of the literature. 1912 25

Breast cancer with cartilaginous and/or osseous metaplasia is a type of metaplastic carcinomas and is a rare disease. We report the case of a 49 year-old female who underwent right mastectomy for a large breast tumor. Histological examinations revealed a mixed tumor with both stromal and epithelial elements;the stroma showed poor differentiated spindle-shape and multiform cells with a massive osseous matrix, and atypical epithelial cells, which mainly existed on the surface of the cysts, showed nucleic atypia. The tumor was diagnosed as a malignant phyllodes tumor with osteosarcomatous differentiation;it was not identified as a metaplastic carcinoma because of the lack of proof of a cancerous component. Two years after a mastectomy, swelling of the axillary lymph nodes was found and a biopsy was performed. Histological findings for the lymph node indicated a metastasis of the invasive ductal carcinoma. The primary tumor was re-examined and was considered to be the origin of the lymph nodal metastasis. Lymph nodal metastasis of cancer proved that the primary tumor had cancerous potential, and the pathological diagnosis was altered to a breast cancer with cartilaginous and/or osseous metaplasia.
...
PMID:Breast cancer with cartilaginous and/or osseous metaplasia diagnosed by lymph nodal metastasis: a case report. 2003 93

Metaplastic breast carcinoma is an uncommon tumor that develops from conventional ductal mammary carcinoma, usually consisting of squamous and/or spindle cell components and/or mesenchymal elements. Although several morphological subtypes of metaplastic breast carcinoma are known, sebaceous metaplasia has not yet been described in this context. The authors report a case of an 84-year-old woman with a huge, ulcerated primary tumor in her left breast that had already been present for 10 years. Pulmonary and bone metastases and a tumor nodule in the contralateral breast were also detected at the time of admission. Left simple mastectomy was performed. Histological examination of the tumor revealed metaplastic carcinoma consisting of ductal carcinoma, which immunohistochemically exhibited a triple-negative immunoprofile, along with dominant areas of squamous and sebaceous differentiation. Adjuvant chemotherapy was subsequently given with partial regression of the systemic metastases. Seven months after surgery and diagnosis, a new, rapidly growing, large soft tissue metastatic tumor appeared in the intramuscular compartment of the patient's right thigh, which was removed and histologically examined. Morphologically this metastatic tumor showed ductal adenocarcinoma along with areas of sebaceous differentiation and, in addition, osteochondroid metaplasia. Immunohistochemically, unlike the primary, this tumor expressed HER-2. The case is presented because of its rarity, and sebaceous differentiation is also proposed as a novel type of metaplasia in this context, expanding the spectrum of the histological patterns of metaplastic breast carcinoma. The literature concerning breast sebaceous lesions is reviewed, and the hypothetical biological mechanisms responsible for the tumor pathogenesis in this case are discussed.
...
PMID:Metaplastic carcinoma of the breast with dominant squamous and sebaceous differentiation in the primary tumor and osteochondroid metaplasia in a distant metastasis: report of a case with review of sebaceous differentiation in breast tumors. 2186 68

The patient was a 58-year-oldpostmenopausal woman. Vacuum assistedbiopsy of the left breast tumor revealedinvasive ductal carcinoma. Immunohistochemical examination was negative for estrogen receptor(ER), negative for progesterone receptor(PgR), andshowedan HER2 score of 3+. FDG-PET/CT revealedmultiple metastases to the left supracravicular and axillary lymph nodes and lungs. She was diagnosed with HER2-positive T3N3M1, Stage IV breast cancer. A 2-year regimen of chemotherapy with trastuzumab andvinorelbine achieveda complete response with regardto the metastatic sites; however, the size of the primary tumor increasedd espite the chemotherapy, andsurgical resection of the left breast with axillary lymph node dissection was performed for local control. Pathological examination of the surgical specimen revealed metaplastic carcinoma with sarcoma component surrounded by non-invasive ductal carcinoma. No component of invasive ductal carcinoma was found. Immunohistochemically, metaplastic carcinoma was negative for ER, negative for PgR, andrevealedan HER2 score of 0. There was discordance of HER2 status between pre- andpost -chemotherapy. The patient receivedno further chemotherapy following surgery andhas been without disease progression for 6 years. We suggest there is heterogeneity, that is, the metastatic sites andthe partial primary tumor were HER2-positive invasive ductal carcinoma and the remainder of the primary tumor was triple negative metaplastic carcinoma. As a result, the patient was able to discontinue chemotherapy with higher quality of life.
...
PMID:[A Case of Stage IV Metaplastic Breast Cancer in Which Primary Tumor Was Removed after Treatment with Anti-HER2 Systemic Therapy]. 2936 39