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Cervico-facial adenoid cystic carcinoma: study of 102 cases. Influence of radiation therapy.

One hundred two patients with cervico-facial adenoid cystic carcinoma were treated with surgery alone, radiotherapy alone or both from 1951 to 1980. All the cases have a 5-year minimum follow-up. The local control rate is 55.5% at 5 years and 37.7% at 10 years. The 5-year local control rate is 44% with surgery alone, 65.8% with radiotherapy alone and 77.8% with post-operative radiotherapy. The difference between surgery alone and radiosurgical association is significant (p less than 0.01). The bone involvement diminished local control rate (32.2%/k 68.8%). The 5-year survival rate of the patients who recurred and have been retreated is 70.5%. The 5-year survival rate of the patients after the appearance of a metastasis is 38.1% and 2 patients have survived more than 10 years. The NED 5-year survival rate is 48.8%. There is no significant difference in the NED 5-year survival rate according to sites or treatments. The crude 5-year survival rate is 70%, 51.4% at 10 years and 32.2% at 15 years. Our study shows that adenoid cystic carcinoma have a peculiar and slow evolution. Surgery with post-operative radiotherapy obtains the best local control. These results and the radiosensibility of these lesions allow us to propose an aggressive treatment for the recurrence and the primary tumor of the directly metastatic patients.

Actuarial Analysis↗

Clinical analysis of the effect of intraarterial cytoreductive chemotherapy in the treatment of lacrimal gland adenoid cystic carcinoma.

PURPOSE: To determine the effect of intraarterial cytoreductive chemotherapy (IACC) as an adjunct to conventional surgery and radiation therapy for lacrimal gland adenoid cystic carcinoma (ACC). DESIGN: A retrospective, comparative, interventional case series. METHODS: setting: Institutional. patient population: Nine consecutive patients with lacrimal gland ACC were treated with IACC, followed by orbital exenteration and chemoradiotherapy. This case series was compared with a series of seven patients treated by conventional local therapies in the same institution. intervention procedure: Clinical records, imaging studies, histologic sections, and archival specimens from all 16 patients were reviewed. Information analyzed included site of disease, histologic characteristics, extent of disease, incidence of locoregional recurrence or distant metastases, and disease-free survival and overall survival time. main outcome measure: The effect of IACC was assessed by the radiographic and histologic response and survival outcome in comparison to a historical cohort of patients managed by conventional local therapies. RESULTS: The difference between the carcinoma cause-specific death rate of the study group versus conventional treatment was significant (P = .029, log rank test). The cumulative 5-year carcinoma cause-specific death rate in the IACC treated group was 16.7% compared with 57.1% in the conventional treatment group. The cumulative 5-year recurrence rate in the IACC treated group was 23.8% compared with 71.4% in the conventional treatment group. CONCLUSIONS: The preliminary data suggest that IACC as an integral component of a multimodal treatment strategy is potentially effective in improving local disease control and overall disease-free survival in lacrimal gland adenoid cystic carcinoma.

Adult↗

Adenoid cystic carcinoma arising in a fibroadenoma.

Adenoid cystic carcinoma (ACC) of the breast is a rare neoplasm, accounting for only 0.1 % of all breast carcinomas. An intriguing factor of ACC in the breast is its good prognosis compared to ACC in other locations, mainly in the minor salivary glands. The incidence of axillary lymph node involvement is also very low compared to that of other breast tumors, and distant metastases are uncommon. We report the case of a 65-year-old woman with a 2-year history of a well-circumscribed breast nodule sonographically thought to be a lymph node which was later excised because of rapid growth. Histologic examination showed an adenoid cystic carcinoma with squamous differentiation originating in a fibroadenoma. A review of the literature reveals no previous report of such a case.

Biomarkers, Tumor↗

Long-term survival after resection of primary adenoid cystic and squamous cell carcinoma of the trachea and carina.

BACKGROUND: Tracheal resection for primary carcinoma may extend survival. We evaluated survival after surgical resection or palliative therapy to identify prognostic factors. METHODS: We conducted a retrospective study of patients diagnosed with primary adenoid cystic carcinoma (ACC) or squamous cell carcinoma (SCC) of the trachea between 1962 and 2002. Laryngotracheal, tracheal, or carinal resection was performed when distant metastasis and invasion of adjacent mediastinal structures were absent and tumor length permitted. Radiotherapy was administered after operation (54 Gy), except in superficial tumors, or as palliation (60 Gy). RESULTS: Of 270 patients with ACC or SCC (135 each), 191 (71%) were resected. Seventy-nine were not resected due to tumor length (67%), regional extent (24%), distant metastasis (7%), or other reasons (2%). Overall operative mortality was 7.3% (14/191) and improved each decade from 21% to 3%. Tumor in airway margins was present in 40% (17/191) of resected patients (ACC 59% versus SCC 18%) and lymph node metastasis in 19.4% (37/191). Overall 5- and 10-year survival in resected ACC was 52% and 29% (unresectable 33% and 10%) and in resected SCC 39% and 18% (unresectable 7.3% and 4.9%). Multivariate analysis of long-term survival found statistically significant associations with complete resection (p < 0.05), negative airway margins (p < 0.05), and adenoid cystic histology (p < 0.001), but not with tumor length, lymph node status, or type of resection. CONCLUSIONS: Locoregional, not distant, disease determines resectability in primary tracheal carcinoma. Resection of trachea or carina is associated with long-term survival superior to palliative therapy, particularly for patients with complete resection, negative airway margins, and ACC.

Carcinoma, Adenoid Cystic↗

Immunology of tonsils and adenoids: everything the ENT surgeon needs to know.

The lymphoid tissue of Waldeyer's ring, and particularly the nasopharyngeal tonsil (adenoids), appears to be functionally comparable to nasal-associated lymphoid tissue in rodents. Antigen-stimulated lymphoid follicles give rise to: (a) clonal B-cell expansion; (b) B-cell receptor affinity maturation; (c) positive selection of B cells according to receptor affinity for antigen; (d) differentiation to B memory cells and plasma cells; and (e) variable induction of the joining (J)-chain gene. B-cell differentiation is also important to promote downstream isotype switching of the immunoglobulin (Ig) heavy chain constant genes. For tonsillar B cells, this process gives mainly rise to IgG and IgA plasma cells, partially associated with J-chain expression. Because the J chain is a key peptide in the polymer structure of secretory IgA, tonsils and adenoids may provide B cells for mucosal effector sites. Thus, several observations suggest that these lymphoid organs generate polymeric IgA (pIgA)-expressing B cells that migrate to the upper airway mucosa, lacrimal glands and salivary glands. Accordingly, the nasal route of vaccination induces secretory IgA-dependent regional mucosal immunity and will also enhance systemic immunity. Although the pIgA-producing capacity of tonsillar B cells is considerably decreased in children with recurrent tonsillitis, a conservative attitude towards adenotonsillectomy appears immunologically desirable, particularly in the young age group.

Adenoidectomy↗

Laminin-1 and SIKVAV a laminin-1-derived peptide, regulate the morphology and protease activity of a human salivary gland adenoid cystic carcinoma cell line.

In a previous paper, we demonstrated that laminin-1 and its derived peptide SIKVAV modulates the morphology of an adenoid cystic carcinoma cell line (CAC2 cells). Light microscopy of CAC2 cells grown in three-dimensional preparations of SIKVAV-enriched laminin-1 showed the presence of pseudocystic spaces. Pseudocysts are hallmarks of adenoid cystic carcinoma in vivo. We hypothesized that these pseudocystic spaces could be due to the protease-inducing/activating role of SIKVAV. Thus, we studied the presence of matrix metalloproteinases (MMPs) in CAC2 cells treated either by laminin-1 or by SIKVAV-enriched laminin-1. Immunohistochemistry and zymography suggested that SIKVAV enhanced the secretion of MMP-2 and MMP-9 in CAC2 cells. We propose that SIKVAV induces pseudocystic formation probably through the secretion of MMPs 2 and 9.

Carcinoma, Adenoid Cystic↗

Expression of TIMP-1 and -2 in different growth patterns of adenoid cystic carcinoma.

Tissue inhibitors of metalloproteinases (TIMPs) are special inhibitors of matrix metalloproteinases. To evaluate their roles in adenoid cystic carcinoma (ACC), we compared TIMP-1 and -2 mRNA and protein expression in different histological pattern of ACC. We obtained carcinoma cells from each of cribriform and tubular pattern of ACCs using by laser microdissection (LM), to determine the mRNAs expression of TIMP-1 and -2 using by real-time quantitative reverse transcription-polymerase chain reaction (RT-PCR), and to confirm expression of them by immunohistochemical staining. Our results showed that mRNA expression of TIMP-1 tended to be decreased in cribriform pattern compared with tubular pattern in four cases, and TIMP-1 significantly decreased in three cases. TIMP-2 also significantly decreased in cribriform than in tubular pattern in three of four cases. Protein expression of TIMP-1 and -2 decreased in the cribriform pattern compared to tubular pattern. These results indicate that there is close relationship between TIMPs and growth patterns of ACC, and TIMP-1 and -2 may play important roles in morphogenesis and biological character of adenoid cystic carcinoma.

Aged↗

Correlation of histopathological variants, cellular DNA content, and clinical outcome in adenoid cystic carcinoma of the salivary glands.

OBJECTIVE: To study the correlation between flow cytometrically measured DNA ploidy with prognostically important histopathologic groups and clinical outcome in patients with adenoid cystic carcinoma of the salivary glands. STUDY DESIGN: 46 tumor specimens were analyzed flow cytometrically for DNA content and assessed for histological grade. Correlations were made between tumor DNA ploidy and histopathological grade, and disease-free and overall survival of these patients. RESULTS: Of the 46 patients, 31 had a cribiform/tubular histologic pattern, and 15 had a solid pattern. 84% of the tumors with cribriform/tubular pattern were DNA diploid, compared with 33% of tumors that were graded solid. This difference proved to be statistically significant (chi(2)11.75, P = 0.0006). Overall and disease-free survival periods were longer for patients with DNA diploid tumors in both groups, 63% vs. 36% and 62% vs 38%, respectively. CONCLUSIONS: Tumor DNA ploidy correlates with prognostically important tumor histopathology as well as overall and disease-free survival in patients with adenoid cystic carcinoma of the salivary gland. EBM RATING: B-3.

Adult↗

Solid variant of mammary "adenoid cystic carcinoma with basaloid features" merging with "small cell carcinoma".

We describe a rare case of a solid variant of a mammary adenoid cystic carcinoma with basaloid features (sbACC) and its coexistence with a "small cell" carcinoma (SCC), identified and confirmed by histological and immunohistochemical observations: the absence of glandular structures and PAS-positive globules, positivity for neuroendocrine markers (NSE, synaptophysin and chromogranin), and negativity for 34betaE12 and SMA actin were the aspects suggesting the presence of SCC. Furthermore, positivity for CD10 was found both in sbACC and in SCC, supporting the hypothesis that the two components share the same histogenetic myoepithelial origin and represent an example of dedifferentiation along neuroendocrine phenotype lines occurring in a multipotential neoplastic stem line, already committed towards a myoepithelial phenotype. To our knowledge, this is the first reported case of a solid basaloid adenoid cystic carcinoma merging with an SCC carcinoma. Furthermore, it is the first study in which CD10 was used to investigate the histogenesis of the two neoplastic components.

Adult↗

p16 tumor suppressor therapy in salivary adenoid cystic carcinoma cell line SACC83.

OBJECTIVE: This study aimed to determine the growth inhibitory effects of transfecting the wild-type p16 gene into human salivary adenoid cystic carcinoma (SACC) cells in vitro. METHODS: p16 cDNA was cloned into pDOR-neo plasmid by recombination technique, and the human eukaryotic expression vector, pDOR-p16 , was constructed. P16 gene was transfected into SACC83 cells using the liposome method, and the expression of the transfected genes was detected by RT-PCR. The efficacy of transfection was determined by MTT-based colorimetric formats. The DNA metabolic rate of transfected cells was measured by 3 H-TdR incorporation. To describe the cell division phase, the cell cycles of the p16 -transfected gene cells and untransfected cells were analyzed by flow cytometry. RESULTS: The growths in vitro of the p16-transfected cells were inhibited significantly. 3H-TdR incorporation showed a decreased DNA metabolic rate of p16-transfected cells, and flow cytometry suggested a significant increase of the cells in the G1 phase and decrease in the S phase. CONCLUSION: The study confirmed the reversal effect of wild-type p16 on malignant phenotype of the salivary adenoid cystic carcinoma and provides valuable data for further clinical trial of gene therapy with p16.

Carcinoma, Adenoid Cystic↗

Adenoid size is related to severity but not the number of episodes of obstructive apnea in children.

OBJECTIVE: The objective of this study was to determine the extent to which adenotonsillar hypertrophy contributes to the severity of obstructive sleep apnea (OSA) in children. STUDY DESIGN: Thirty-three consecutive children who were referred to a sleep disorders center for evaluation of suspected OSA had standard lateral neck roentgenography performed. Adenoid size was determined by measuring the adenoidal-nasopharyngeal (AN) ratio. Tonsil size was quantitated on physical examination. The severity of OSA was determined by full-night polysomnography in the sleep laboratory. RESULTS: All of the patients reported snoring with trouble breathing, apneas, or both problems witnessed by a parent. The patients' respiratory disturbance index ranged from 0 to 95.3 (mean +/- SD 12.5 +/- 9.1). The patients' AN ratio ranged from 0.48 to 0.98 (0.76 +/- 0.14); 30 (91%) of the 33 patients had AN ratios greater than published normal means, and 16 (48%) had AN ratios more than 2 standard deviations above published means. Although the AN ratio and tonsil size did not predict the number of apneas, a significant relationship was seen between the AN ratio and the duration of obstructive apneas (r = 0.48, p < 0.01). Obesity (percent ideal body weight) was the only independent predictor for the number of respiratory events per hour of sleep (r = 0.49, p < 0.01). Percent ideal body weight was also the major predictor of the lowest oxyhemoglobin saturation (r = -0.58, p < 0.0001), but the AN ratio also contributed to the variance in saturation, with a correlation coefficient (r) of 0.69 for the two factors (p < 0.0001). CONCLUSION: Lymphoid hyperplasia affects the severity of apnea more than the number of obstructive apneas. The pathophysiologic characteristics of OSA in children probably involve complex interactions between pharyngeal size and mechanics.

Adenoids↗

Adenoid cystic carcinoma of the airway: thirty-two-year experience.

METHODS: We have reviewed our experience in 38 patients with adenoid cystic carcinoma of the upper airway seen between 1963 and 1995. The mean age was 44.8 years (15 to 80 years) with a male/female ratio of 1:1.1. Thirty-two of the 38 patients were treated by resection and reconstruction (primary anastomosis 28; Marlex mesh prosthesis 4). Twenty-six of the 32 patients undergoing resection received adjuvant radiotherapy. Six patients with unresectable tumors were treated primarily with radiotherapy only. RESULTS: Pathologic examination revealed local invasion beyond the wall of the trachea in all patients. In a majority, microscopic extension was found in submucosal and perineural lymphatics, well beyond the grossly visible or palpable limits of the tumor. Lymphatic metastases were relatively uncommon, occurring in only five of 32 (19%) patients undergoing resection. Metachronous hematogenous metastases occurred in 17 of 38 patients (44%). Thirteen of these 38 patients (33%) had pulmonary metastases. Sixteen of 32 resections were complete and potentially curative. There were two deaths within 30 days of operation. The mean survival in the 14 patients undergoing complete resection was 9.8 years (12 months to 29 years). Sixteen of 32 resections were incomplete (residual tumor at the airway margin on final pathologic examination), with one operative death occurring in this group. The mean survival in the 15 surviving patients was 7.5 years (4 months to 21 years). Six patients were treated with primary radiation only and had a mean survival of 6.2 years (2 months to 14.3 years). In the patients with pulmonary metastases, mean survival was 37 months (4 months to 7 years) from the time of diagnosis of the pulmonary metastasis until their death. CONCLUSION: Adenoid cystic carcinoma of the upper airway is a rare tumor, which is locally invasive and frequently amenable to resection. Although late local recurrence after resection is a feature of this tumor (up to 29 years), excellent long-term palliation is commonly achieved after both complete and incomplete resection. There was a small difference in survival between patients having complete and incomplete resection. Long periods of control can be obtained with radiotherapy alone. The best results, in this series of patients, were obtained by resection. Adjuvant radiotherapy is assumed to favorably influence survival.

Actuarial Analysis↗

Adenoid cystic carcinoma of Cowper's gland.

We report an additional case of primary adenoid cystic carcinoma of Cowper's gland in an otherwise healthy asymptomatic 66-year-old man. Based on a review of similar lesions presenting in the head and neck, our treatment plan entailed pelvic exenteration followed by radiation therapy. Whether adenoid cystic carcinoma of Cowper's gland behaves as aggressively as it does in the head and neck remains unclear. However, in view of the extensive local invasion in our patient and a previously successful outcome, a combined surgical and radiation approach appears to be the most appropriate therapy.

Aged↗

Sore throat, tonsillitis, and adenoiditis.

Diseases of the adenoids and tonsils are a frequent cause for patients to seek medical attention. A thorough understanding of the disease processes reflected in the adenoids and tonsils and their diagnosis and treatment is an important part of routine care delivery. Diagnostic methodologies and timing of surgical intervention are reviewed along with general considerations relating to the differential diagnosis in adult and pediatric patients.

Abscess↗

Adenoid cystic carcinoma of the lacrimal gland: the clinical significance of a basaloid histologic pattern.

The authors reviewed 74 adenoid cystic carcinomas of the lacrimal gland, 54 of which had sufficient follow-up data for analysis of survival. Patients with a basaloid pattern in their tumor had a five-year survival rate of 21 per cent and a median survival of three years, whereas patients whose tumor contained no trace of a basaloid component had a five-year survival rate of 71 per cent and a median survival rate of eight years. Nonparametric statistical analysis revealed that this difference in survival was significant at the 0.0005 level. The authors propose that, in the future, pathologists label all adenoid cystic carcinomas as either "basaloid" or "nonbasaloid" and code each case accordingly.

Adult↗

Obstructive hypertrophic adenoids and tonsils as a cause of infantile failure to thrive: reversed by tonsillectomy and adenoidectomy.

Isolated failure to thrive in an infant caused by chronic hypoventilation due to hypertrophic adenoids and tonsils, has not been previously described. A 9 month-old infant presented with weight loss and mild clinical respiratory signs. Hypoxemia and CO2 retention, however, were documented by transcutaneous gas measurement, and ENT examination revealed enlarged tonsils and adenoids. Adenoidectomy and tonsillectomy at 9 1/2 months of age completely reversed the patient's hypoventilation and growth pattern. While the exact mechanism of failure to thrive secondary to upper airway obstruction in infancy is not clear, we conclude that routine ENT evaluation should be a regular part of the workup of infants under one year who suffer from failure to thrive.

Adenoidectomy↗

Pathological and biochemical study in the adenoid of mucopolysaccharidosis II.

In the patients with mucopolysaccharidosis II (MPS II, Hunter's syndrome), conductive and sensorineural hearing deficits are frequently observed. Two patients with MPS II underwent adenoidectomy and an ear douche, and their conductive hearing loss recovered after the surgery. Pathological examination of the adenoids revealed the infiltration of faintly PAS-positive plasmacytes with perinuclear vacuole and strongly Alcian-blue positive fibrotic area. Biochemical study was performed by chromatoscanning of electropholesis. The amount of glycosaminoglicans (GAG) in the specimens was increased 2.8-fold compared with the normal control. The electropholetic pattern showed an increase of dermatan sulfate (DS), heparan sulfate (HS) and hyaluronic acid (HA) in the adenoids of the patients. The ratio of DS to HS was 1.1-1.2. In the patients of MPS II, the accumulation of GAG occurs in the pharyngeal tonsil and causes conductive hearing impairment. We recommend adenoidectomy for such patients.

Adenoids↗

Primary adenoid cystic carcinoma in the lung: report of two cases and therapeutic considerations.

Primary adenoid cystic carcinoma in the lung is an uncommon disease, which is regarded as a slow growing low-grade malignancy. However, this disease has a high risk of incomplete resection because of its unique histological invasion patterns. The cases of two patients who recently underwent surgery for adenoid cystic carcinoma in the lung are reported. Both patients received postoperative radiotherapy treatment as a follow-up to a histologically incomplete resection. Both patients have survived for 51 months and 7 months, respectively, with no recurrence. Postoperative radiotherapy is an acceptable treatment of choice to control residual lesions and provides long-term survival even in cases of incomplete resection.

Aged↗