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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histomorphologic features and routine endocrine immunohistochemical (IHC) markers do not differentiate neuroendocrine tumors (NETs) in relation to their location, making it difficult to establish the site of origin of a metastatic neoplasm. Site-specific markers would be useful, particularly when examining small biopsies. CDX-2 and thyroid transcription factor-1 (TTF-1) are transcription factors that have been recently proposed as IHC markers of intestinal and pulmonary adenocarcinomas, respectively. However, their expression in NETs has not been widely studied. The objective of this study is to evaluate the expression of TTF-1 and CDX-2 in NETs and their potential usefulness in distinguishing gastrointestinal and pulmonary NETs from other sites. We performed an IHC study on formalin-fixed, paraffin-embedded sections from 155 primary NETs, including 60 pulmonary, 60 gastrointestinal, 30 pancreatic, and 5 NETs from other sites. In addition, we evaluated 13 metastatic NETs, including 11 cases of gastrointestinal and 2 of pulmonary origin. In this study, CDX-2 was expressed in 28/60 (47%) of gastrointestinal NETs with the following results: 11/11 (100%) appendiceal, 12/14 (86%) small intestinal, 3/4 (75%) colonic, 2/11 (18%) rectal, and 0/20 (0%) gastric. TTF-1 was expressed in pulmonary carcinoid tumors in 13/30 (43%) and in 27/30 (90%) pulmonary small cell carcinomas. NETs of other origins (pancreas, skin, ovary, and thymus) were negative for both TTF-1 and CDX-2. Metastatic neuroendocrine neoplasms of intestinal origin were positive for CDX-2 and negative for TTF-1. In conclusion, CDX-2 expression is highly specific in identifying NETs of intestinal origin and TTF-1 expression is helpful in identifying NETs of pulmonary origin, which can be quite useful in the diagnosis of metastatic NETs of unknown origin.
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PMID:Diagnostic value of CDX-2 and TTF-1 expressions in separating metastatic neuroendocrine neoplasms of unknown origin. 1809 83

Extramammary Paget's disease (EMPD) is a rare malignant neoplasm with a predilection for the apocrine-rich anogenital skin and less commonly for the axilla. The tumor rarely occurs in non-apocrine bearing regions where it is referred to as ectopic EMPD. Here, we report a case of a patient that presented with a poorly circumscribed, erythematous plaque with patchy alopecia on the scalp. Histology showed pagetoid infiltration of the epidermis by atypical single and nested cells with abundant amphophilic cytoplasm with nuclear hyperchromasia, extending along the adnexae. Immunohistochemistry showed that the tumor cells stained positively for mucicarmine, periodic acid schiff, cytokeratin-7, polyclonal carcinoembryonic antigen, epithelial membrane antigen, gross cystic disease fluid protein, androgen receptor and Her-2-neu; and negatively for S-100, HMB-45, CDX-2, thyroid transcription factor-1, estrogen receptor and progesterone receptor, thus, establishing the diagnosis of ectopic EMPD. Subsequent workup showed no underlying malignancy. To our knowledge, we report the second case, and the first in the English literature, of an ectopic EMPD involving the scalp without any associated malignancy.
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PMID:Extramammary Paget's disease presenting as alopecia neoplastica. 1958 7

Previous studies have indicated that resistin-like molecule beta (RELM beta), an intestinal goblet cell-specific protein, is markedly increased in the intestinal tumors of min mice and over-expressed in a human colon cancer cell line. We hypothesized that RELM beta might be enhanced in human colon cancer. The aim of this study was to examine the clinical importance of RELM beta expression in colon cancer patients and to correlate its expression with various clinicopathological parameters, upstream regulatory molecule expression, tumor proliferative capacity, and patients' survival. Of the 80 colon cancer patients studied, 65 (81.25%) tested positive for RELM beta, mainly in the cytoplasm of colon mucosa. Contrasting sharply with the strongly RELM beta-positive tumors, normal colon mucous membrane was negative or weakly positive. RELM beta positivity in colon cancer was correlated with histological grade of differentiation and lymph node metastasis, but not with age, gender, tumor location and size, tumor infiltration, Dukes' stage, liver metastasis, and venous invasion. RELM beta expression was significantly correlated with the expression of transcription factor CDX-2 (P < 0.01) but not with that of proliferative index Ki-67 (P > 0.05). The mean postoperative survival time (2.76 years) of RELM beta-positive patients was significantly longer than that (1.26 years) of RELM beta-negative patients (P = 0.032). These findings support evidence of the enhanced RELM beta expression in colon cancer patients and suggest that further investigation is warranted to explore the role of RELM beta in colon cancer.
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PMID:Enhanced expression of resistin-like molecule beta in human colon cancer and its clinical significance. 1859 73

Bronchogenic cyst (BC), a rare developmental abnormality of the primitive foregut and usually found above the diaphragm, can occur in the retroperitoneum and other unusual locations where distinction from metastatic teratoma is essential. We studied 22 BCs and 34 teratomas metastatic to the retroperitoneum in men. Of 22 BCs, 17 (77%) showed well or moderate architectural differentiation toward normal tracheobronchial structures, whereas none of the teratomas did. In addition, 20 (91%) of the BCs had respiratory-type epithelium and none had enteric-type epithelium. Immature elements, atypia, and tumor necrosis were absent in BCs. All BCs expressed cytokeratin (CK) 7, and none expressed CDX-2 in immunohistochemical analysis. Teratomas had mixed enteric and respiratory-type epithelium. The majority of the glands were positive for CK7 (33 [97%]), CK20 (34 [100%]), CDX-2 (34 [100%]), and thyroid transcription factor (TTF)-1 (17 [50%]). Coexpression of CDX-2 and TTF-1 was found in 11 teratomas (32%) but none of the BCs. BCs can be reliably distinguished from teratomas based on the gross, histologic, and immunohistochemical features.
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PMID:The distinction of bronchogenic cyst from metastatic testicular teratoma: a light microscopic and immunohistochemical study. 1862 97

Ovarian mucinous neoplasm (OMN) is traditionally classified as either intestinal type or endocervical-like subtypes. The 2 subtypes represent different clinicopathologic characteristics. The immunophenotype of the 2 subtypes has not been adequately investigated. In this study, we investigated 14 intestinal type OMNs (borderline and adenocarcinoma) and 12 endocervical-like OMNs (borderline and adenocarcinoma) for their expression of PDX-1, CDX-2, CA-125, CK7, CK20, WT-1, D2-40, and TTF-1. We also included 15 colorectal adenocarcinomas metastatic in the ovary, as they may occasionally mimic OMN. The intestinal type OMNs were positive for PDX-1 (100%), CK7 (100%), CK20 (100%), CDX-2 (29%), whereas were negative for CA-125. The endocervical-like OMNs were positive for CA-125 (100%) and CK7 (100%), whereas were negative for CK20, PDX-1, and CDX-2. Metastatic colorectal adenocarcinomas were positive for CK20 (100%), CDX-2 (100%), and PDX-1 (33%), whereas were negative for CA-125 and CK7. All of the intestinal type and endocervical-like OMNs as well as metastatic colorectal adenocarcinomas were negative for WT-1, D2-40, and TTF-1. Our results demonstrated that the intestinal type and endocervical-like OMNs are immunophenotypically distinct entities. The 2 subtypes can be separated from metastatic colorectal adenocarcinoma by the different immunohistochemical profile of PDX-1, CA-125, CK7, CK20, and CDX-2. In the work-up of mucinous adenocarcinoma in the ovary or abdominal cavity, caution should be exercised in interpreting the possible primary site on the basis of the immunohistochemical profiles.
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PMID:Intestinal type and endocervical-like ovarian mucinous neoplasms are immunophenotypically distinct entities. 1866 37

Signet-ring adenocarcinoma is an aggressive form of primary bladder adenocarcinoma that has been associated with poor outcomes. The utility of immunohistochemical markers in tumors with signet-ring morphology may vary from more typical adenocarcinomas arising at the same location, although this has not been examined in bladder adenocarcinoma. We examined a series of bladder adenocarcinomas to determine the impact of signet-ring cell features on clinical outcomes and immunohistochemical findings. We identified 25 patients with bladder adenocarcinoma, ranging in age from 28 to 78 years (mean, 57 years) and with a male-female ratio of 18:7. Six cases were urachal in origin. Signet-ring cells occurred in 19 of 25 bladder adenocarcinomas (76%) and ranged from 5% to 100% of tumor volume, with most tumors demonstrating more than 60% signet-ring cell differentiation (15/19), when present. Regional lymph node metastases were present in 8 of 19 patients (42%) who underwent cystectomy. The percentage of tumor containing signet-ring cells was significantly associated with the presence of adverse pathologic features (defined as unresectable primary tumor or regional lymph node metastasis, P = .013) and decreased overall survival (P = .034), and the latter remained significant in multivariable analysis after adjusting for positive soft tissue margins (P = .026). A comparison between immunohistochemical markers frequently used to analyze bladder adenocarcinoma demonstrated decreased expression of several markers in signet-ring (n = 9) versus colonic-type (n = 8) morphology, including CDX-2, beta-catenin, and E-cadherin, although these results did not reach statistical significance. In summary, the extent of signet-ring differentiation in bladder adenocarcinoma is associated with worsened survival and higher stage disease; the utility of immunohistochemical analysis in foci consisting of predominant signet-ring cells may be limited, although further studies that address this finding are needed.
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PMID:Clinicopathologic features and utility of immunohistochemical markers in signet-ring cell adenocarcinoma of the bladder. 1878 86

Mucinous cystic neoplasms (MCNs) of the pancreas, ovary and lung have a similar histologic appearance. We investigated if immunohistochemical (IHC) studies could help in separating these neoplasms. Twenty-six ovarian MCNs (invasive carcinoma and borderline tumor), 12 pancreatic MCNs (invasive carcinoma, and with moderate or high-grade dysplasia), and 3 pulmonary MCNs (only invasive carcinoma) were retrieved. Our study demonstrated that pancreatic MCNs are positive for CDX-2 (67%), PDX-1 (100%), CK7 (83%) and CK20 (100%), while are negative for CA-125. The IHC profile of ovarian intestinal type MCN is similar to that of pancreatic MCNs, except for lower frequency of CDX-2 expression (29% vs. 67%). Ovarian endocervical like MCNs are positive for CA-125 (100%) and CK7 (100%), while are negative for CDX-2, PDX-1 and CK20. Pulmonary MCNs are positive for CDX-2 (100%), CK7 (100%) and CK20 (100%), while are negative for PDX-1 and CA-125. All tumors are negative for TTF-1, D2-40 and WT-1. We concluded that an IHC panel of CDX-2, PDX-1, CA-125, and CK20 is useful in separating MCNs of the pancreas, ovary and lung.
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PMID:Distinctive immunohistochemical profile of mucinous cystic neoplasms of pancreas, ovary and lung. 1901 47

Carcinoid tumors of the ovary are rare neoplasms that may be primary or metastatic. Clinicopathologic features such as unilaterality and early stage favor a primary ovarian neoplasm but in the absence of other teratomatous elements it may be difficult or impossible to determine whether an ovarian carcinoid is primary or metastatic. CDX-2 is a marker of intestinal differentiation that has been proposed as a marker of midgut origin for metastatic carcinoids. Its expression has not been tested in ovarian carcinoids. Additional markers of potential help in defining the origin of a carcinoid include cytokeratin (CK) 20, CK7, and thyroid transcription factor (TTF-1), none of which have been studied in ovarian carcinoids. We evaluated the diagnostic utility of CDX-2, CK20, CK7, and TTF-1 as well as conventional clinicopathologic features in determining the site of origin in 26 ovarian carcinoids (16 primary and 10 metastatic from midgut). Non-neoplastic premenopausal ovaries (n=10) served as controls. All primary ovarian carcinoids were unilateral whereas only 3/10 metastatic carcinoids were unilateral. Multinodular growth occurred in 6/10 metastatic carcinoids but not in any primary carcinoid. The average size of primary ovarian carcinoids was 3.4 cm (range: 0.2-13.5 cm) versus 10.2 cm for metastatic carcinoids (range: 4-32 cm). Of the primary ovarian carcinoids, 12/16 were 3 cm or smaller whereas all metastatic carcinoids were 4 cm or larger. Teratomatous elements were present in association with 10/16 primary ovarian carcinoids, whereas none were present in any metastatic carcinoid. The primary ovarian carcinoid types were insular (n=6), trabecular (n=3), strumal (n=6, of which 5 were trabecular pattern and 1 was insular pattern) or mucinous (n=1). CDX-2 was not expressed in any cells in normal ovaries. Among primary ovarian neoplasms, there was diffuse nuclear CDX-2 expression in 4/6 insular, 0/3 trabecular, 1/6 strumal (1/1 insular pattern and 0/5 trabecular pattern strumal carcinoids), and 1/1 mucinous carcinoids. All metastatic carcinoids, except for two of mucinous type, were insular. CDX-2 was diffusely and strongly expressed in all 8 metastatic insular carcinoids and in both metastatic mucinous carcinoids. None of the metastases was trabecular in type but 12 primary hindgut or foregut trabecular carcinoids were evaluated and all were negative for CDX-2. None of the ovarian carcinoids expressed TTF-1, CK7, or CK20, except for the primary and metastatic mucinous carcinoids, all of which were CK20-positive. These results demonstrate that CDX-2 cannot be used to determine if a carcinoid is primary in the ovary or metastatic from the intestine as insular and mucinous types of either origin express this marker. Trabecular carcinoids of either origin lack CDX-2 expression. CK20, CK7, or TTF-1 do not have diagnostic utility in this context. Conventional clinicopathologic features (unilaterality, lack of multinodular growth, early stage, presence of teratomatous elements, and size 3 cm or smaller) are the most helpful findings in suggesting a primary origin for an ovarian carcinoid tumor.
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PMID:Primary ovarian carcinoid tumors may express CDX-2: a potential pitfall in distinction from metastatic intestinal carcinoid tumors involving the ovary. 1904 9

Pulmonary adenocarcinoma with intestinal differentiation is rare and typically expresses proteins common to lung primaries. We report a case in a 51-year-old woman with a solitary 3.3-cm mass in the left lower lobe. Additional clinical investigation, including positron emission tomography scan with fluorine 18-labeled fluorodeoxyglucose, colonoscopy, and capsule endoscopy of her small bowel, revealed no evidence of tumor elsewhere. She underwent left lower lobectomy with mediastinal lymphadenectomy. Histologic examination revealed tall columnar cells without goblet cell differentiation arranged in a cribriform and acinar pattern with extensive central necrosis. Metastatic carcinoma was present in multiple hilar lymph nodes. Mediastinal lymph nodes were negative. Immunohistochemical stains demonstrated diffuse positivity for cytokeratin (CK) 20 and CDX-2 in neoplastic cells with negative staining for CK7 and thyroid transcription factor-1. CK7 expression has been documented in all 14 cases previously reported. This is the first description of pulmonary adenocarcinoma with intestinal differentiation with histopathologic and immunophenotypic findings indistinguishable from metastatic colorectal adenocarcinoma.
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PMID:Primary pulmonary adenocarcinoma with intestinal differentiation mimicking metastatic colorectal carcinoma: case report and review of literature. 1909 76

Merkel cell carcinoma (MCC) is an uncommon and aggressive primary neuroendocrine malignancy of the skin. Frequent local recurrences and disseminations to regional lymph nodes and distant organs are characteristic. MCC within the lymph nodes in the absence of a primary site is rare and has only been reported sporadically. We report a case of MCC presenting as a painless mass in the left inguinal area for 5 months in a 57-year-old man. The histopathology of the excised lesion revealed a poorly differentiated basophilic small cell tumor. The tumor cells were positive for cytokeratin 20 and CD56, negative for cytokeratin 7, thyroid transcription factor-1 and CDX-2. These immunohistochemical findings were consistent with the diagnosis of a metastatic MCC. Despite extensive clinical and radiological investigation, we failed to identify the origin of the tumor. Our case may represent a lymph node metastasis from an occult or regressed skin primary, but we cannot preclude the possibility of a primary nodal tumor.
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PMID:Merkel cell carcinoma of the inguinal lymph node with an unknown primary site. 1933 94


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