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Adenoid cystic carcinoma of the head and neck.

Adenoid cystic carcinoma (ACC) is a rare malignant tumour believed to arise from the epithelial cells of mucous-secreting glands. It is a slow-growing but aggressive tumour with a propensity for perineural invasion. A 10 year review of 45 patients (19 males and 26 females with a median age of 56 years) treated for ACC at a single institution between 1989 and 1999 was performed to analyse factors involved in treatment failure, local control, treatment-related morbidity and mortality. The data collected were treated for survival curves according to the Kaplan-Meier method and the log-rank test was used to assess the statistical significance of the various groups. The overall survivals at 3 years and 5 years were 71% and 65%, respectively, while the disease-free survivals at 3 years and 5 years were 73% and 63% respectively. Out of the 45 patients, only six had local recurrences, yielding an 87% locoregional freedom from relapse; 16 patients (35.6%) developed distant metastases, with the lung (8/16) being the commonest site. Patients treated for tumours of the nose and paranasal sinuses experienced more morbidity than those with tumours at other sites. Positive margins, perineural invasion and solid histology of ACC were associated with increased morbidity and treatment failure. Patients treated with combined therapy did better than those who underwent a single treatment modality.

Adolescent↗

Adenoid cystic carcinoma of the head and neck--a 20 years experience.

In this retrospective study we give a clinical review of our experience with adenoid cystic carcinoma (ACC) and compare our results with those reported in the literature. Between 1981 and 2000, 74 patients with this disease were treated at our department. Complete resection was reached in 45 cases. Fourteen patients received postoperative radiation. Local control rates at 5, 10 and 15 years were 64%, 56% and 52% with a mean local control time of 11.1 years. Tumour size (P<or= 0.001), perineural invasion (P<or= 0.001) and margin status (P<or= 0.001) showed significant influence on local control. Statistically, we were not able to demonstrate a positive effect for postoperative radiation. Distant control rates at 5, 10 and 15 years were 72%, 62% and 59% with a mean distant control time of 14.4 years. Distant failure was diagnosed in lungs and bones and occurred independently of local treatment outcome. Overall survival rates at 5, 10 and 15 years were 71%, 54% and 37% with a mean overall survival of 11.2 years. Recurrence-free survival rates at 5, 10 and 15 years were 57%, 45% and 37% with a mean recurrence-free survival of 9.1 years. The survival rates at 5, 10 and 15 years after diagnosis of tumour progression were 35%, 15% and 0% with a mean survival time of 4.7 years. Our results provide insights to the unusual nature of ACC, which may be useful for making clinical decisions. However, prospective randomized multicentric studies are needed to define the optimal treatment for ACC with special regard to adjuvant treatment modalities.

Adolescent↗

Primary adenoid cystic carcinoma of the esophagus: a case report.

We report on the case of a 61-year-old woman with a pedunculated esophageal tumor that had a central ulceration. Histology of the resected tumor revealed adenoid cystic carcinoma which was localized entirely in the submucosal layer. The tumor was composed predominantly of solid nests of tumor cells, with some areas showing a cribriform pattern.

Carcinoma, Adenoid Cystic↗

[Local excision of an adenoid cystic carcinoma of the lacrimal gland in a 12-year-old girl: case report (author's transl)].

The authors report on a case of an adenoid cystic carcinoma of the lacrimal gland in a 12-year-old girl. This is probably the earliest manifestation of that tumor so far reported. The tumor was locally excised. The eye was left in place. Despite the high mortality rate of this tumor in the literature the child is in good health and without evidence of metastases or local recurrence two years after surgery.

Carcinoma, Adenoid Cystic↗

[Adenoid-cystic carcinoma (cylindroma) of the trachea. An analysis of nine cases (author's transl)].

Nine patients with an adenoid-cystic carcinoma of the trachea are describes. It is a rare tumour which grows relatively slowly; it has a tendency for early invasion of neighbouring tissues, but late distant metastases. A correct diagnosis can be made by the radiologist. The trachea should be visible on every film of the thorax and should be inspected. In particular, a diagnosis of therapy-resistant asthma should direct the attention of the radiologist to the trachea.

Adult↗

The role of radiotherapy for patients with adenoid cystic carcinoma of the salivary gland.

OBJECTIVE: Adenoid cystic carcinoma (ACC) is a rare malignant tumor occurring in the salivary gland that has some characteristic features, including slow growth, extremely diffuse invasion, and a high incidence of distant metastasis. The indication of radiotherapy (RT) for ACC is controversial. The aim of this study was to examine the usefulness of RT for patients with ACC. STUDY DESIGN: The study group was composed of 17 patients (18 lesions) with ACC of the salivary gland who underwent RT. They were divided into 3 groups: (1) those who underwent RT alone for a primary tumor, (2) those who underwent RT alone for a recurrent tumor after surgery, and (3) those who underwent postoperative RT because of a histologically positive surgical margin. The clinical course of each patient was examined retrospectively. RESULTS: Of the 11 patients undergoing RT alone for a primary or recurrent tumor, 5 showed complete remission of the tumor, and 3 were free from local recurrence for a long period. Seven patients who underwent postoperative RT seemed to show better local control than did those who did not undergo postoperative RT, although there were no significant differences. CONCLUSIONS: We find RT to be an effective treatment procedure, especially for those who had an inoperable, advanced tumor or who had distant metastasis. Postoperative RT was recommended for those who had a histologically positive surgical margin.

Carcinoma, Adenoid Cystic↗

C-kit expression in the salivary gland neoplasms adenoid cystic carcinoma, polymorphous low-grade adenocarcinoma, and monomorphic adenoma.

OBJECTIVE: Differentiating between adenoid cystic carcinomas (ACCs), polymorphous low-grade adenocarcinomas (PLGAs), and the monomorphic adenomas (including canalicular adenomas, trabecular adenomas, and basal cell adenomas) can present a diagnostic challenge, especially when examining tissue obtained from small incisional or fragmented biopsies. Recent studies have revealed that overexpression of the tyrosine kinase receptor protein c-kit occurs in a narrow subset of malignant neoplasms, including gastrointestinal stromal tumors, myeloid leukemias, seminomas, and ACCs. C-kit reportedly is not expressed in PLGAs. We compared the expression of the c-kit antigen in the malignant salivary gland neoplasms ACC and PLGA with its expression in salivary gland monomorphic adenoma (including canalicular adenoma and basal cell adenoma). STUDY DESIGN: Formalin-fixed paraffin-embedded sections of 49 salivary gland neoplasms (17 monomorphic adenomas, 17 PLGAs, and 15 ACCs) accessioned between 1989 and 2002 were retrieved from the files of the Department of Pathology, Long Island Jewish Medical Center, and were stained with an anti-c-kit polyclonal antibody. RESULTS: C-kit reactivity was uniformly positive in the cytoplasm of luminal neoplastic cells in ACCs (15/15, 100%). Positive reactivity was also identified in the majority of PLGAs (16/17, 94%), with at least 25% of the tumor cells being positive. Similar reactivity was seen in monomorphic adenomas (16/17, 94%). CONCLUSIONS: In contrast to previous reports, we find that c-kit expression was not restricted to ACC but was expressed in all 3 tumor types evaluated (ACC, PLGA, and monomorphic adenoma). Therefore, c-kit does not appear to be a useful marker for distinguishing between either ACC and PLGA in equivocal cases, or in benign and malignant salivary gland neoplasms.

Adenocarcinoma↗

Prognostic factors of head and neck adenoid cystic carcinoma: quantitative morphological analysis of 19 cases.

This quantitative study was prompted by concerns regarding the clinical and histopathologic correlation of adenoid cystic carcinoma (ACC). We reviewed the clinical and histopathologic data of 19 cases of ACC in the head and neck region treated during the last 25 years. An image analyzing system was employed for quantitative analysis. Each specimen was observed microscopically at 100 x magnification, by two of the authors, the area of pure cellular component in five randomly selected fields was measured and the averaged cellular ratio per field was calculated as a percentage and then correlated with the behavior of the tumor. The cellular ratio was 66% for the 8 patients who died of disease and 47% (at 3-year follow-up; n = 9) and 58% (at 10-year follow-up; n = 4) for the 11 patients who survived without evidence of disease. For all of the patients who developed distant metastasis the cellular ratio was > 60%. The results of this quantitative study did correlate, to some extent, with the patients' clinical behavior. Although non-quantitative clinical and histological criteria of ACC have been reported to correlate with clinical behavior, it is important to thoroughly understand these criteria and also to combine multiple criteria in order to manage head and neck ACC.

Adult↗

p53 oncoprotein accumulation in adenoid cystic carcinoma of parotid and palatine salivary glands.

Previous studies have suggested that alterations of the p53 gene are the most common genetic abnormality in human cancer. The aims of the present study were to evaluate p53 protein (p53P) immunostaining in adenoid cystic carcinoma (ACC) of the salivary gland and to correlate the expression with patient survival. A total of 27 cases of ACC in the parotid gland (n = 12) and the minor palatine glands (n = 15) were studied, with ten cases each of normal parotid and palatine glands as non-neoplastic controls. Staining was performed with mouse monoclonal antibody DO-7 against p53 (Dako, USA) using the ABC method. Stained nuclei irrespective of intensity or frequency were considered as positive. The frequency of positive nuclei was evaluated as the p53P index (p53PI), the percentage of the total nuclei in the reference epithelium. Clinical survival data were available for patients for periods up to 156 months. Our data showed that no normal tissues showed immunoreactivity with p53P in their nuclei. Thirteen of 15 (87%) cases of palatal and two of 12 (17%) cases of parotid neoplasms stained with p53P and the p53PI ranged from 0.01 to 10%. The number of p53P positive tumors was significantly higher in palatal than in parotid neoplasms, suggesting that palatal ACCs may be more aggressive in comparison with parotid ACCs. Our data also showed that the number of p53P positive tumors was significantly increased in patients who died of tumors than in patients with no evidence of disease at the end of the follow-up period between 60 to 156 months. These results suggest that p53P may be involved in the development of salivary gland ACCs and that p53P analysis may be a useful indicator of poor prognosis.

Adolescent↗

Late recurrence of adenoid cystic carcinoma of the prostate.

A 44-year-old patient underwent radical prostatectomy and adjuvant radiotherapy for adenoid cystic carcinoma of the prostate. After 7 years and 3 months he recurred locally and was treated with external beam irradiation. The current follow up comprises 9 years and 5 months at which the patient is asymptomatic with stable disease. Lymph node or distant metastases did never occur and the prostate specific antigen level was always within the normal range.

Adult↗

Surface expression of intercellular adhesion molecule 1 on epithelial cells in the human adenoid.

Human rhinoviruses enter the host by way of the nose and conjunctiva. Intercellular adhesion molecule 1 (ICAM-1) is the cellular receptor for the majority of rhinoviruses. ICAM-1 expression on the luminal surface of epithelial cells in the upper airway may be an important determinant of virus localization in the airway. Eighteen adenoids and 5 nasopharyngeal biopsies were evaluated by immunohistochemistry for surface expression of ICAM-1. Heavy immunoreactivity of ICAM-1 was found on the surface of a small number of single nonciliated cells in the lymphoepithelium. Squamous epithelial cells showed minimal to no staining, and ciliated epithelium had positive ICAM-1 staining of the basal cells but not on the ciliated border. The localization of ICAM-1 expression to specific, limited areas of the surface epithelium of the nasopharynx may have important implications in the pathogenesis of rhinovirus infections, especially initiation of the host response to rhinovirus.

Adenoids↗

Reduced E-cadherin expression correlates with unfavorable prognosis in adenoid cystic carcinoma of salivary glands of the oral cavity.

Reduced expression of E-cadherin (E-cad), a transmembrane glycoprotein, is associated with loss of differentiation, acquisition of an invasive phenotype, and an unfavorable prognosis in carcinomas from several sites. We used immunohistochemistry to study the expression of E-cad in 50 adenoid cystic carcinomas (ACCs) in salivary glands to evaluate correlations with clinicopathologic parameters and patient survival. Absent or low E-cad expression was observed more frequently in solid than in cribriform or tubular carcinomas. E-cad expression also was significantly correlated with histologic grade and the growth pattern. In addition, ACCs showing low or absent E-cad expression were more frequently larger than 4 cm in diameter, and distant metastases developed more frequently. Reduced expression correlated with shorter disease-free intervals and actuarial survival rates. Univariate and multivariate analysis identified tumor stage and E-cad expression as the only 2 parameters predictive of the disease-free interval. E-cad expression and tumor stage, grade, and type of growth were significant prognostic factors for survival in univariate analysis, while tumor stage, type of growth, and E-cad expression were the only significant covariates in multivariate analysis. These findings indicate that the loss of E-cad has an important role in the natural history of ACC, as it is associated with loss of differentiation and development of metastases. We also provide evidence that E-cad expression is an independent indicator of clinical aggressiveness in patients with ACC, together with clinical stage and type of growth at the periphery of the tumor.

Adult↗

New prognostic criterion in adenoid cystic carcinoma of salivary gland origin.

Adenoid cystic carcinoma (ACC) of salivary gland origin shows histologic patterns (tubular, cribriform, solid) that have been correlated with the clinical course of the disease. Recognition of tubular pattern is generally easy. Discrimination between cribriform and solid types leaves some margin for the pathologist's subjective interpretation because it is essentially based on the amount of gland-like spaces, containing mucinous or hyalinized material, which are present in neoplastic nests. To overcome this problem and with the aim of identifying a more objective basis for prognostic evaluation of cribriform and solid ACC, the authors counted the number of gland-like spaces per square millimeter of tumor, excluding supporting stroma and small areas with the tubular pattern, in a group of tumors similar with regard to therapy, stage of disease, and microscopic evidence of tumor-free surgical margins. According to the authors' data, the number of gland-like spaces per square millimeter of tumor appears to be a reliable and objective measure for prognostic evaluation of cribriform and solid pattern ACC; in particular, the greater the number of gland-like spaces per square millimeter of tumor, the longer the survival of the patient.

Carcinoma, Adenoid Cystic↗

Adenoid cystic carcinoma. A clinicopathologic study with flow cytometric analysis.

Forty-five patients with adenoid cystic carcinoma (ACC) of salivary glands were retrospectively studied to determine the prognostic use of DNA ploidy analysis compared with clinicopathologic features. Nuclear suspensions were prepared from 37 of these tumors by the Hedley technique on paraffin-embedded material. The DNA content was analyzed by flow cytometry after propidium iodide staining. Thirty-five tumors were diploid and 2 were tetraploid. Mean survival was 117 and 52 months for the diploid and tetraploid patients, respectively. The median S-phase fraction (Spf) of the 35 diploid tumors was 4.45%. Of the 17 diploid patients who died of disease, there were 11 tumors with high Spf (greater than 4.45%) and 6 tumors with a low Spf (P less than 0.05 chi-square test). The presence of more than 30% of a solid pattern in the tumor was strongly associated with poor median survival in Kaplan-Meier survival curve analysis (P less than 0.05 log rank test). Grade, stage, recurrence, and metastases were also found to be important prognostic factors. Because few tumors were nondiploid, these results suggest that S-phase fraction analysis may be a more useful prognostic indicator than ploidy classification.

Actuarial Analysis↗

Concentrations of cefpodoxime in plasma, adenoid, and tonsillar tissue after repeated administrations of cefpodoxime proxetil in children.

Cefpodoxime proxetil was administered to 36 children undergoing tonsillectomy, adenoidectomy or both. It was very well tolerated. The detectable tissue concentrations of cefpodoxime were moderate but remained constant (approximately 0.05 mg/kg) 3, 6, and 12 h after the last dose of the drug, while the respective plasma concentrations were declining. This suggests the possibility of twice-daily administration. However, 30% of children did not have quantifiable concentrations in the tonsil and more than half the adenoids did not have quantifiable levels. Whether a higher dosage would lead to higher and more satisfactory tissue concentrations is a matter for further investigation.

Adenoids↗

Epirubicin in patients with advanced or recurrent adenoid cystic carcinoma of the head and neck: a phase II study of the EORTC Head and Neck Cancer Cooperative Group.

BACKGROUND: Because of the rarity of salivary gland cancer, little is known about the single-agent activity of most anticancer agents in the different histologic types of these cancers. PATIENTS AND METHODS: Twenty patients with advanced or recurrent adenoid cystic carcinoma of the head and neck were treated with epirubicin on a low-dose weekly schedule (30 mg/m2/week) for eight weeks and, in instances of no response, a high-dose three-weekly schedule (> or = 90 mg/m2). Thirteen patients had locoregional disease (12 recurrences), seven of them also had distant metastases and seven had distant metastases only. All had documented progressive disease, and 17 were symptomatic. RESULTS: Two objective responses of 7.5 and 20 months were observed in patients with locally recurrent disease, and ten patients showed disease stabilization. The median time to progression was 16 weeks (range 2-250 weeks) and the median survival 67 weeks (range 13-272+ weeks), with three patients still alive more than five years after the initiation of chemotherapy. Contrary to the rather modest objective response rate, symptomatic improvement occurred more frequently (29.4%) and was evident within 8 weeks of treatment. CONCLUSIONS: This type of chemotherapy should be reserved for patients with rapidly progressive disease or those with symptomatic disease.

Adult↗

A comparative immunohistochemical study of adenoid cystic carcinoma of the skin and salivary glands.

We performed an immunohistochemical study that compared a primary adenoid cystic carcinoma (ACC) of the skin with two salivary gland ACC. All three tumors stained positively and in identical fashion for epithelial membrane antigen (EMA), carcinoembryonic antigen (CEA), broad-spectrum keratins, and low-molecular-weight keratins. Both EMA and CEA were localized to the luminal surfaces and the secreted contents of the tubular structures and the ductlike structures of the cribriform formations. The staining reactions for both types of keratin were more intense in the cells lining the tubular structures and the ductlike structures of the cribriform formations. One of the two salivary ACCs stained positively for S-100 protein; the other was positive for vimentin. The cutaneous ACC was negative for both antigens. Leu-7 antigen was not detected in either type of ACC. These results show that primary cutaneous ACC and salivary ACC have similar immunohistochemical staining patterns for a number of antigens. We believe this similarity is due to the fact that these antigens are shared by the sweat glands and salivary glands, which are considered to be the respective sites of origin for these two types of tumors.

Adult↗

Dedifferentiation in adenoid cystic carcinoma of salivary gland: an uncommon complication associated with an accelerated clinical course.

Adenoid cystic carcinoma (ACC) of the salivary gland is generally an indolent tumor that pursues a protracted clinical course with recurrences and late metastasis. The authors report three cases of ACC with dedifferentiation to high-grade malignant neoplasms. One patient developed dedifferentiated ACC ab initio, with extensive local disease and multiple lymph nodes metastases at first presentation, requiring mutilating surgery. Two patients had dedifferentiated ACC 4 and 10 years, respectively, following excision of the initial uncomplicated ACC; both patients died within 1.5 years after recurrence. Histologically, the dedifferentiated component appeared as a distinct population of anaplastic cells with more abundant cytoplasm, irregular-shaped tumor islands infiltrating a desmoplastic stroma, and total loss of bicellular differentiation characteristic of ACC. The immunophenotypic profile was altered in comparison with the ACC, such as acquisition of strong staining for S100 protein and lack of a myoepithelial component in the two cases that were interpreted as being poorly differentiated adenocarcinoma. One case was a sarcomatoid neoplasm with focal myoepithelial features. Overexpression of p53 protein was demonstrated in the dedifferentiated component in one case, and overexpression of cyclin D1 was seen in two cases. The dedifferentiated component had a higher Ki67 index than did the ACC. To the authors' best knowledge, this report represents the first documentation of dedifferentiation as a form of tumor progression in ACC, which is associated with a sinister clinical outcome.

Adult↗