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

A 43-year-old woman who had a vulvar mass associated with mild discomfort was found to have a rare primary vulvar adenocarcinoma of probable cloacal origin. The tumor was contiguous with the surface epithelium of the vulva and was a well to moderately differentiated adenocarcinoma of colonic type. Stains of the neoplastic cells were positive for both acid and neutral mucin, and periodic acid-Schiff (PAS) was positive after diastase reaction. The neoplastic cells were strongly positive for carcinoembryonic antigen, broad spectrum cytokeratin, and p-53 antigen. Clinical evaluation failed to show any primary tumor in colon, lung, or breast. The patient was disease free 18 months after operation.
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PMID:Primary vulvar adenocarcinoma of cloacogenic origin. 1153 Nov 87

We undertook a detailed histologic study to identify specific morphologic features that may aid in distinguishing prostatic adenocarcinoma with lung metastases (PALM) from other pulmonary tumors with similar histologic features. In 16 cases, we found 3 predominant architectural patterns: microacinar (n = 10), tubulopapillary (ductal; n = 4), and carcinoid-like (n = 2). Characteristic features of PALM included small acinar and/or cribriform growth, frequent lymphangitic permeation, lack of stromal response, uniform round nuclei with prominent nucleoli, intraluminal blue mucin, and prominent cell borders. By immunohistochemical staining, prostate-specific antigen and prostate-specific acid phosphatase were present in 13 of 14 and 13 of 13 cases, respectively. Metastatic prostatic duct adenocarcinoma exhibited morphologic features similar to metastatic colonic adenocarcinoma. Two cases had a carcinoid-like appearance with nested or solid architecture, parachromatin clearing, and prominent nucleoli, but lacked the finely stippled chromatin pattern of carcinoid tumors. Several features that may result in misinterpretation or lack of association of the neoplasm in the lung with a prostatic primary include lung metastasis preceding the detection of a prostatic primary tumor, solitary pulmonary nodule, tubulopapillary (ductal) or carcinoid-like pattern, scant material in which histologic features of metastatic prostate carcinoma are not fully appreciated, and frequent necrosis. Attention to specific discriminating histologic features, supported by immunohistochemical staining, may be useful in the differential diagnosis, which is therapeutically and prognostically critical.
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PMID:The morphologic spectrum of metastatic prostatic adenocarcinoma to the lung: special emphasis on histologic features overlapping with other pulmonary neoplasms. 1193 29

Mucinous adenocarcinoma is characterized by large pools of extracellular mucin. The tumor has been reported in the nasal cavity, paranasal sinus, breast, colon, stomach, prostate, skin, and lung. Mucinous adenocarcinoma also arises as a primary tumor of the major salivary glands. However, its occurrence is exceedingly rare and has only recently been recognized. Only 9 cases in the major salivary glands have been reported. We present an additional case of MAC in the maxilla that was considered to have developed from a palatal minor salivary gland.
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PMID:Mucinous adenocarcinoma of probable minor salivary gland origin. 1246

Clear-cell (CRCC), papillary (PRCC), and chromophobe (CHRCC) renal-cell carcinoma (RCC) are the three most frequent subtypes of RCC. The rate and distribution of their metastatic lesions have not been well studied in cytopathological materials. Sixty-two fine-needle aspiration biopsy cases of metastatic RCC were studied and correlated with surgical pathology of RCCs with and without metastasis. Special stains for glycogen and immunostaining for cytokeratins, vimentin epithelial membrane antigen (EMA), and carcinoembryonic antigens, and electron microscopic studies were performed. Fifty-nine cases of CRCC and three of PRCC subtypes were retrieved from the cytopathology files at the Ottawa Hospital in a period of 10 years. Of these cases, 10 metastatic CRCC and one metastatic PRCC were diagnosed prior to the diagnosis of the primary tumor. CHRCC and sarcomatoid RCC were not represented in cytopathological specimens. CRCC displayed characteristic filmy cytoplasm and nuclei with prominent nucleoli. PRCC was characterized by dense cytoplasm, large nuclei with prominent nucleoli, and papillary architectures. In addition, all RCCs were characterized by the presence of glycogen and the absence of mucin by using histochemical techniques and electron microscopic studies and positive reactivity for cytokeratins (CK) and vimentin (VIM). In the same period, there were a total of 380 patients with RCC divided into 310 CRCCs, 55 PRCCs, and 15 CHRCCs associated with metastases in 142, 9, and 1 case, respectively. CRCC is by far the most common subtype found in metastases sampled in cytopathology. PRCC, CHRCC, and sarcomatoid RCC were underrepresented. Awareness of this propensity of RCC and the characteristic cytopathological, histochemical, immunohistochemical, and ultrastructural features are helpful in the diagnosis of metastatic RCC.
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PMID:Distribution of subtypes of metastatic renal-cell carcinoma: correlating findings of fine-needle aspiration biopsy and surgical pathology. 1256 Oct 22

The gross and routine microscopic features of 25 stage I primary mucinous ovarian carcinomas without clinical evidence of recurrence and 25 mucinous carcinomas metastatic to the ovaries were compared. Findings that were frequent in the latter and strongly favored a metastasis were: 1) bilaterality, 2) microscopic surface involvement by epithelial cells (surface implants), and 3) an infiltrative pattern of stromal invasion. Findings that were less frequent but present exclusively or almost exclusively in metastatic carcinomas were: 1) a nodular invasive pattern, 2) ovarian hilar involvement, 3) single cell invasion, 4) signet-ring cells, 5) vascular invasion, and 6) microscopic surface mucin. Findings that were frequent in, and strongly favored, primary ovarian carcinoma were: 1) an "expansile" pattern of invasion and 2) a complex papillary pattern. Findings that were less frequent but also favored a primary tumor were: 1) size >10 cm, 2) a smooth external surface, 3) benign-appearing and borderline-appearing areas, 4) microscopic cystic glands, and 5) necrotic luminal debris. Findings that did not distinguish the tumors were: 1) a cystic gross appearance, 2) gross solid, papillary, necrotic, or hemorrhagic areas, 3) nature of cyst contents (mucinous vs nonmucinous), 4) stromal mucin (pseudomyxoma ovarii), 5) cribriform, villous, or solid growth patterns, 6) focal area resembling typical colonic carcinoma, 7) goblet cells, or 8) tumor grade. Primary and metastatic mucinous ovarian carcinomas can be distinguished from each other in the great majority of cases based solely on their conventional histopathologic findings. Careful gross evaluation is also important with special attention paid to the external surface of the ovarian tumor(s) to detect abnormalities that have the features of surface implants on microscopic evaluation.
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PMID:The distinction between primary and metastatic mucinous carcinomas of the ovary: gross and histologic findings in 50 cases. 1260 84

Pancreatic mucinous cystic tumors (MCT) are proliferations of mucin-producing epithelia supported by an ovarian-like stroma. They are classified into adenomas (MCA), borderline (MCB) and noninvasive or invasive carcinomas (MCC). The molecular mechanisms underlying their clinical behavior are poorly understood, partly due to the lack of cellular models. We report the establishment of MCC1, the first cell line from a pancreatic MCT, deriving from the highly dysplastic cell component of a noninvasive MCC. MCC1 has mutations in codon 12 of K-RAS (GGT>GAT), codon 58 of P16 (CGA>TGA) and codon 132 of P53 (AAG>AGG). The FHIT and DPC4 genes are unaltered. Immunohistochemistry shows abnormal expression of MUC1 and p53, loss of p16 and retention of Fhit and Dpc4 in both the cell line and the highly dysplastic cells of the primary lesion. The morphological and molecular features of MCC1 and its corresponding primary tumor are consistent with a model for non-invasive MCC, where K-RAS, P16, P53 and MUC1 alterations are pre-invasive changes associated with progression of malignancy of MCT from adenoma to carcinoma. MCC1 is sensitive to 5-fluorouracil, representing the first assessment of drug sensitivity for MCC. Finally, MCC1 is a suitable model for preclinical studies, as it grows in immunodeficient mice.
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PMID:Mucinous cystic carcinoma of the pancreas: a unique cell line and xenograft model of a preinvasive lesion. 1568 69

Solid papillary carcinomas (SPCs) are uncommon tumors composed of circumscribed large cellular nodules separated by bands of dense fibrosis. The aim of this study was to further elucidate the characteristics of SPC, the types and significance of invasive carcinomas associated with these tumors, and the long-term clinical outcome. Fifty-eight SPCs were analyzed (mean follow-up, 9.4 years). Cases were divided into three groups: 1) SPC only (32.7%), 2) SPC with extravasated mucin (8.6%), and 3) SPC with invasive components (58.7%) consisting of neuroendocrine-like (29.5%), colloid (23.5%), ductal not otherwise specified (14.5%), lobular (3%), tubular (3%), or mixed (26.5%). The mean age was 72 years. All were estrogen receptor positive and 86% were histologic grade 1. The total size of the tumor measured 0.3 to 15 cm. In the group with invasive carcinoma, the size of invasion was 0.1 to 4 cm. Axillary nodes were involved in 13% of the cases (6 of 46); all of these had an invasive component in the primary tumor. Local recurrence was seen in 5 patients, all from the group with invasive carcinoma. Overall, 11.7% died of their tumor, 1 to 4 years after diagnosis (mean, 2.3 years); none of them belongs to the group of noninvasive SPC. Five of the 6 patients who died of tumor had invasive components. The sixth patient who died with "metastatic signet-ring cell carcinoma" at 10 years was in the group of patients with SPC with extravasated mucin where the SPC lesion had prominent signet-ring cell features. In conclusion, SPCs are heterogeneous lesions that arise in older women and have an indolent behavior. Lymph node and distant metastases are uncommon and generally limited to cases with (conventional) invasive components.
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PMID:Clinicopathologic analysis of solid papillary carcinoma of the breast and associated invasive carcinomas. 1662 97

CASE 1: A 64-year-old, otherwise healthy woman was referred to the surgery clinic for a presumed umbilical hernia. On physical examination, a cutaneous nodule was noted on the umbilical region and the patient was referred to the dermatology clinic. The patient was reexamined and an erythematous nodule was observed in the umbilicus measuring 2.5 cm in diameter. The patient denied pain, change in bowel habits, or weight loss. There were no other abdominal masses, no sign of ascites, and no regional lymphadenopathy. A skin biopsy from the nodule showed mucinous adenocarcinoma. Immunohistochemical staining was positive for carcinoembryonic antigen, and negative for cytokeratin (CK)7 and CK20. These results were consistent with a Sister Mary Joseph's nodule and led to the diagnosis of an occult colon carcinoma. The patient had no risk factors for colorectal carcinoma. The patient underwent surgery in another hospital, and died 3 months after the initial diagnosis of Sister Mary Joseph's nodule. CASE 2: A 73-year-old woman was referred to the dermatology clinic for evaluation of a painful, ulcerated, 3-cm lesion in the umbilicus (Figure 1). She was otherwise asymptomatic. A skin biopsy showed neoplastic glandular cells infiltrating among collagen bundles (Figure 2). Stainings for mucin and for CK7 were positive, while staining for CK20 was negative. An abdominopelvic CT scan demonstrated a 3.5-cm space-occupying lesion in the liver. Results of gastroscopy, colonoscopy, chest computed tomographic (CT) scan, and mammography were normal. Serum levels of the tumor-associated protein CA125 were elevated to 164 units, while those of CA 19-9 and carcinoembryonic antigen were within normal range. A gynecologic examination and a transvaginal ultrasound were normal. The patient had no personal or family history of any malignancy or any risk factors for developing a carcinoma. The patient was scheduled for a palliative resection of the umbilical nodule, combined with a laparoscopic inspection in search of the undetected primary tumor. She refused surgery and was lost to follow-up. She died 4 months after the initial diagnosis of umbilical metastasis. CASE 3: A 51-year-old man was aware of a silent mass in his umbilicus for 2 years without seeking medical advice. Following 2 weeks of increasing pain in this area, he was referred to the emergency room for a suspected incarcerated umbilical hernia. Surgery revealed a mass attached to the fascia and peritoneal fat. The mass was removed and diagnosed as a poorly differentiated adenocarcinoma, staining positively for carcinoembryonic antigen, and negatively for CK20, CK7, prostate-specific antigen, and prostatic acid phosphatase. Both gastroscopy and colonoscopy failed to detect the primary tumor. An abdominopelvic CT scan was normal, but a CT scan of the chest disclosed a nodule measuring 2.5 x 1.5 cm in the lower lobe of the right lung. On bronchoscopy, it was found to be an invasive adenocarcinoma, consistent with a primary tumor of the lung. The patient was a heavy smoker (45 pack-years). The patient received 4 cycles of combined chemotherapy with carboplatine and gemcitabine, with no improvement. A month later, the patient complained of abdominal pain. Following demonstration of intra-abdominal spread of disease by CT scan, a second line chemotherapy was instituted with paclitaxel. A month later the patient's condition deteriorated and he complained of cough, sweating, and pain along the right leg. A bone scan revealed bone metastases in the right femur and left tibia. Two weeks later he was admitted to the hospital with intestinal obstruction and underwent laparotomy. He had massive intra-abdominal spread of cancer and ascites. Only a palliative colostomy was performed. The patient died 3 weeks later, 9 months after the diagnosis of adenocarcinoma of the lung. The clinical data on the three patients are summarized in Table I.
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PMID:Sister Mary Joseph's nodule as a presenting sign of internal malignancy. 1695 43

The presence of mucin-containing signet-ring cells in an ovarian neoplasm is considered strong evidence for a metastatic rather than primary tumor. The clinicopathologic features of 2 ovarian adenofibromatous neoplasms with mucin-containing signet-ring cells (AFN-SRs), proven by the long-term follow-up of 10 and 15 years to be primary tumors, are presented as exceptions to this general guideline. Both of these tumors were initially strongly favored to be metastatic adenocarcinoma by expert consultants in gynecologic pathology, which underscores the diagnostic difficulty that these rare and unusual neoplasms can pose. Although long-term follow-up of AFN-SRs is still necessary to exclude the possibility of metastases, the following constellation of features can correctly suggest a primary neoplasm rather than a Krukenberg-like metastatic process at the time of presentation: (i) the appearance of fibromatous stroma as an integral part, as opposed to reactive part, of the neoplasm, (ii) a semiorganized rather than completely haphazard pattern to the placement of the epithelial elements, with a circumscribed noninfiltrative interface with ovarian parenchyma, (iii) the absence of angiolymphatic invasion, (iv) tumor unilaterality, and (v) the lack of a known primary source. The virtual absence of literature on these low-grade malignancies, coupled with the occurrence of 2 such tumors within a 7-year period at a single community hospital, suggests that many AFN-SRs continue to be misdiagnosed as metastatic adenocarcinoma.
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PMID:Primary ovarian adenofibromatous neoplasms with mucin-containing signet-ring cells: a report of 2 cases. 1741 84

Recognition of an ovarian tumor as a metastasis from an undiagnosed primary gastrointestinal tract carcinoma can be difficult when specific symptoms referable to the primary tumor are lacking and the tumor simulates a primary ovarian neoplasm grossly and microscopically. Ovarian metastases of colorectal adenocarcinomas, in particular, continue to pose diagnostic challenges both clinically and pathologically. Clinicopathologic features of 20 cases of ovarian metastases from undiagnosed colorectal adenocarcinomas (U-CRAs) were compared with those of 22 cases having metastases from known colorectal adenocarcinomas (K-CRAs). Women with ovarian metastases from U-CRAs were significantly younger (mean age, 48 years; median, 47 years) than those with ovarian metastases from K-CRAs (mean, 61 years; median, 63 years) (P = 0.002), presented with clinical findings related to the ovarian metastases, often had elevated CA-125 levels, and lacked specific symptoms due to the colorectal carcinomas, which were diagnosed only at the time of intraoperative evaluation of the ovarian tumors. Mean/median ovarian tumor sizes (12.8/13.0 cm for U-CRAs; 14.1/15.8 cm for K-CRAs) and frequencies of bilaterality (45% for U-CRAs and 36% for K-CRAs) were not significantly different for the 2 groups; frequencies of clinically unilateral tumors of more than 10 cm were similar in both groups (30% for U-CRAs and 45% for KCRAs). Other features more commonly observed in ovarian metastases from U-CRAs included mucinous differentiation, extracellular mucin production, and some degree of cytokeratin 7 expression; endometrioid-like differentiation was more common in metastases from K-CRA, but a garland pattern of necrosis and the presence of a confluent glandular, rather than infiltrative, pattern of invasion were similarly common in both groups. In cases having ovarian metastases from U-CRA, the younger age of the women, uniform presentation as pelvic masses without symptoms referable to the bowel, elevated CA-125 levels, occasional presentation as a large clinically unilateral tumor, frequent mucinous differentiation, and frequent coexpression of cytokeratin 7 are features that can contribute to misclassification of these metastases as primary ovarian neoplasms.
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PMID:Women with undiagnosed colorectal adenocarcinomas presenting with ovarian metastases: clinicopathologic features and comparison with women having known colorectal adenocarcinomas and ovarian involvement. 1831 25


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