SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
We present a case of adenoid cystic carcinoma of the submandibular gland resulting in metastases to the liver, nine years following initial presentation.
Primary cutaneous adenoid cystic carcinoma (ACC) is a rare tumor, with less than 50 cases reported to date. We report an additional case of primary cutaneous ACC arising on the scalp of a 57-year-old woman. The clinical presentation, histologic findings, and subsequent treatment are described. A brief review of the literature is provided.
A 79-year-old male was admitted for macroscopic hematuria and right back pain. A mass shadow in the right lower lung fields had been present for three years previously with unconfirmed diagnosis. Excretory urogram, ultrasonogram and computed tomographic scan revealed bilateral renal tumors. Selective right renal arteriogram demonstrated a slightly hypervascular mass in the upper pole of the right kidney. Chemotherapy was not effective. Slow but definite malignant progression developed and the patient died two and a half years later. Autopsy finally revealed the adenoid cystic carcinoma of the right lung with bilateral renal metastases. The renal metastasis of this tumor is the first case in Japan to our knowledge.
The occurrence of sialoliths in the submandibular gland is 80% due to the specific anatomy of both the gland and its duct. The diagnosis is rather easy because of the obvious clinical signs of the entity. Imaging studies are always necessary in order to treat the patient as effectively as possible. The stones do not tend to occur within the gland as frequently as in the respective duct. The coexistence of sialoliths and malignant tumors is extremely rare. A 70-year-old woman with intraparenchymal stone was operated in our ENT department. In addition to the sialolith the pathological examination revealed the existence of an adenoid cystic carcinoma (ACC), that extended to the neighboring skeletal muscle. This is the reason why we believe it would be useful to report this case of a large stone (14 mm in diameter) located in the submandibular gland coexisting with ACC. This case report is a very good example illustrating that all available means should be used prior to reaching a conclusion and making a health professional decision.
Cystic adenoid carcinoma or cylindroma of the breast is a rare tumour, and until new only 140 cases have been described in the literature. The clinical criteria are not very specific: tumour in a woman of 50 years of age, with slow growth, unilateral, well delimited, firm, peri-areolar, painless and not adherent to the skin nor the deep tissues. The diagnosis is made by histological examination which shows the presence of pseudo-cysts encased in cellular masses heaped up, composing epithelium and myo-epithelial elements. These are sometimes visible with optic microscopy, and if necessary confirmed by electron microscopy and using immunohistochemical techniques. It is now realised that the myo-epithelial cell plays a role in the histogenesis of the cylindroma. The outcome is usually good after simple surgical removal which has to be sufficient to avoid local recurrences. All the same there have been rare cases of metastases in the literature which means that these cases should be followed up carefully.
The infratemporal fossa approach to the skull base is an established method of treating neoplasms of this region. The type C variation has proved an effective means of removing adenoid cystic carcinoma invading the infratemporal fossa and skull base from the nasopharynx. A classification of these tumors is proposed, the surgical technique is described, and the results are given in this article.
OBJECTIVE: To investigate anticancer effect of hydroxycamptothecine and theprubicine in the treatment of established human adenoid cystic carcinoma (ACC) lung metastasis of nude mice and to determine the best therapy sequence of the two drugs. METHODS: The model of ACC lung metastasis (60 animals) was established by injecting the ACC-M cells into the nude mice from their tail veins. Animals were divided into six groups: v acuity control group, HCPT group, THP group, HCPT and THP group 1, HCPT and THP group 2, HCPT and THP group 3 (No.1 - No.6). Different treatment sequence was performed on these groups. The lung samples were collected and observed after HE staining. The area of metastases and lung tissue were analyzed by the images analysis system. RESULTS: The size of the metastatic lesion in the treatment groups were smaller than that in the control group (P < 0.01). The treatment effects between the group with simultaneous administration of HCPT and THP and the group with single drug administration were not different (P > 0.05). No.5 and No.6 groups showed better effect of treatment than the other treatment groups (P < 0.05). CONCLUSIONS: There are two recommended sequences: (1) HCPT should be used after THP; (2) THP is used 48 hours, After HCPT is administered. Because using two drugs together will decrease the anticancer effect, HCPT and THP should not be used simultaneously.
OBJECTIVE: To establish a predicting model of survival rates and to evaluate the weighted contributions of each key prognostic factor of the patients with salivary adenoid cystic carcinoma (SACC). METHODS: One hundred and eighteen follow-up cases with SACC were analyzed for the survival study with retrospective cohort method. Ten possible clinical and pathologic factors were selected. A multivariate analysis was performed by Cox proportional hazard model and prognostic index (PI) was calculated. According to the PI, all cases were divided into three risk subgroups respectively: lower, intermediate and higher risk subgroups. Ten-year survival rate and median survival time were calculated and the predicting models of survival rates were established. RESULTS: The significant prognostic factors influencing the survival rate were age at diagnosis, clinical presentation, TNM clinical stage, treatment, surgical margins (P < 0.05). The predicting formula was PI = 0.031X(2) + 0.665X(5) + 0.420X(6)-0.576X(7) + 0.999X(10). According to the value of PI, the prognosis of the patients was significantly different among the three subgroups (P < 0.05). In the three risk subgroups, 10-year survival rates were 83.56%, 31.45% and 11.20% respectively, the median survival time was 18 years, 7 years and 4 years respectively. CONCLUSIONS: The established predicting model of survival rates can predict the prognosis of the patients with SACC.
OBJECTIVE: To investigate the influence of LY294002 on protein kinase B expression in salivary adenoid cystic carcinoma cells (SACC). METHODS: Using Western-blot and RT-PCR detected the protein and mRNA level of protein kinase B (PKB) both in cell line, absent and present LY294002. The data were analysed by t-test. RESULTS: The expression and transcription level of PKB (Ser(473)) in SACC-83 was lower than in SACC-LM (P < 0.05). The expression of PKB (Ser(473)) in SACC cell lines with LY294002 was lower than in SACC cell lines without LY294002 (P < 0.01). The transcription level of PKB showed no difference in SACC cell lines with and without LY294002 (P > 0.05). CONCLUSIONS: In vitro, the protein expression and transcription level of PKB (Ser(473)) is higher in SACC-LM cell line than in SACC-83 cell line. In vitro, LY294002 can inhibit protein expression of PKB (Ser(473)) in SACC-83 cells and SACC-LM cells.
The aims of this study were to investigate the expression levels of beta-catenin, Pin1 and cyclin D1 in salivary adenoid cystic carcinomas (SACC ) and to evaluate its clinical importance, furthermore, to elucidate whether beta-catenin expression was aberrant in SACC and whether Pin1 was involved in aberrant beta-catenin and cyclin D1 expression. The expression of Pin1, beta-catenin and cyclin D1 were examined in the specimens of 65 patients with SACC by immunohistochemistry, protein and mRNA expressions were detected by western blotting and RT-PCR in four SACC cell lines. Pin1 was overexpressed in 51 cases of SACC (78%), and high levels of Pin1 expression correlated with cyclin D1 positive expression (p = 0.02). Fourteen (22%) cases showed positive immunoreactivity for beta-catenin protein in the nuclear/cytoplasmic fraction in tumor tissues, which was defined as cytoplasm/nucleus staining, among which quite evident nuclear expression of beta-catenin was detected in six cases (9%), while cyclin D1 positive expression was detected in 41 cases of SACC (63%). Reduced membranous expression of beta-catenin was detected in the cases with metastasis (11/14). Theses results suggest that Pin1 and Wnt signalling pathway are activated in SACC and may play a pivotal role in SACC carcinogenesis and metastasis.
Laryngeal or hypopharyngeal localization of adenoid cystic carcinoma is rare due to the poor distribution of the accesory salivary glands in this area. We report one case of such neoplasm on that area, with its diagnosis confirmed histologically. The required a total laryngectomy with later reoperations because of hemorrhage and pharyngeal fistula. One year after the surgery and in spite of postoperative radiotherapy multiple pulmonary metastasis were detected causing the death of the patient. We review the literature of this tumor whose prognosis is much worse than epidermoid or scamous carcinoma.
We present an 82-year-old male patient who presented with complaints of gradually an increasing ulcero-proliferative lesion with persistent mucinous discharge in the left breast. Left side-modified radical mastectomy was done. This was histopathologically diagnosed as an adenoid cystic carcinoma of the left breast. Periodic acid schiff (PAS) staining confirmed the diagnosis. Three of the five axillary lymph nodes excised were positive for malignancy. Although the patient was advised to have postoperative radiotherapy, he did not comply. After 2 years, the patient again presented with local recurrence of the disease. Wide excision of the recurrent malignant nodules over the anterior chest wall was done, and the defect was covered primarily with split thickness skin grafting. Postoperative radiation was given. For the past 9 months, the patient has maintained a regular follow-up on an outpatient basis. He does not have any evidence of recurrence of the tumor--neither locally nor distant metastasis.
PURPOSE: To determine the effect of intra-arterial cytoreductive chemotherapy (IACC) as an adjunct of a multimodality protocol for the treatment of lacrimal gland adenoid cystic carcinoma (ACC). METHODS: This was a retrospective, comparative, consecutive case series. 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. 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, local-regional recurrence or distant metastases, and disease-free survival time. Gene analysis was performed on microdissected tissue samples. Mutational allelotyping targeting nine genomic loci using 15 polymorphic microsatellite markers situated in proximity to known tumor suppressor genes serve as markers for the presence of gene deletion. The effect of IACC was assessed by the radiographic response and survival outcome in comparison to a historical cohort of patients managed by conventional local therapies. A fractional mutation index was used to compare the acquired mutational load between different tumors having nonidentical patterns of microsatellite informativeness. RESULTS: The carcinoma cause-specific death rates between the two treatment groups was significant (P = .029, log-rank test). The cumulative 5-year carcinoma cause-specific death rate was 16.7% in the IACC-treated group compared with 57.1% in the conventional treatment group. 1p36 was the single most common site affected by allelic loss for microsatellite markers in this series. 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 ACC. Allelic loss for microsatellite markers at 1p36 may be a common and an early event in ACC formation and progression.
OBJECTIVE: To investigate the role of DNA methyltransferases (DNMT) in carcinogenesis and development of salivary adenoid cystic carcinoma (ACC) by detection the expression of DNMT in ACC and to assess its correlation to methylation of tumor suppressor genes and biological behavior of ACC. METHODS: The protein expression of DNMT1 and DNMT3b were examined by immunohistochemistry in paraffin-embedded tissues of ACC from 60 patients. The association between the positive staining of DNMT1 and DNMT3b microscopically and the CpG islands methylation status of some tumor suppressor genes, tumor grade and stage was analysed by Fisher exact test. RESULTS: The number of DNMT1 positive cells was much more than that of DNMT3b (P < 0.01). The positive expression of DNMT1 was increased concomitantly to the amounts of methylation of tumor suppressor genes occurred in ACC and was significantly correlated to the methylation status of RASSF1A (P < 0.01). A difference was found between the more and less methylation of tumor suppressor genes group (P < 0.05) The increased positive expression of DNMT1 correlated to the tumor grade (P < 0.05) and stage (P < 0.01). It was much more expressed in the lower grade or more advanced stage cases. The correlation between the expression of DNMT3b and the methylation of tumor suppressor genes, tumor grade and stage was not found. CONCLUSIONS: DNMT1 may play an important role in the maintenance of methylation of tumor suppressor genes in ACC and was related correlated to the methylation of RASSF1A, tumor grade and stage, which implied that DNMT1 may contribute to the carcinogenesis and development of ACC and was likely to be a molecular marker for evaluating the prognosis of patients with ACC.
OBJECTIVE: To observe the expression of Schwann cell marker GFAP and myoepithelial cell marker alpha-SMA in salivary adenoid cystic carcinoma (ACC), and to evaluate the relationship of GFAP, alpha-SMA and perineural invasion in ACC. METHODS: Immunohistochemical SABC method, double-label immunofluorescence and CLSM were used to detect the expression of GFAP and alpha-SMA proteins in salivary ACC tissue samples. RESULTS: In salivary ACC tissue samples, both GFAP and alpha-SMA proteins were positive, which were coexpressed in cytoplasm of the same onco-myoepithelial cells. CONCLUSIONS: There may be Schwann cell differentiation in onco-myoepithelial cell of salivary ACC, and it may be the pathological base of perineural invasion in salivary ACC.
We report the clinical case of an adenoid cystic carcinoma on the base of the tongue, a rare malignant tumor of slow-growing but aggressive with a propepnsity for perineural invasion. Treatment modalities more common include surgery and radiotherapy but locorregional recurrences are frequent and relatively early in the first years. A review of the literature of this pathology is performed.
OBJECTIVE: To investigate the expression of Pin1, beta-catenin and cyclin D1 in salivary adenoid cystic carcinomas (SACC) and to evaluate the role of beta-catenin and Pin1 in SACC carcinogenesis. METHODS: The expressions of Pin1, beta-catenin and cyclin D1 were examined in the specimens of 65 patients with SACC by immunohistochemistry, and Pin1 protein and mRNA expressions detected by Western blot and RT-PCR in four SACC cell lines. RESULTS: Pin1 was overexpressed in 51 cases of ACC (78%), and 41 (63%) cases showed positive immunoreactivity for cyclin D1 protein in the nuclear fraction in tumor tissues. Fourteen (22%) cases showed positive immunoreactivity for beta-catenin protein in the nuclear/cytoplasmic fraction in tumor tissues, 6 of which exhibited quite evident expression of beta-catenin in nucleolus. The expression of membranous beta-catenin was down-regulated in most of the patients with lymph node metastasis (11/14). CONCLUSIONS: The results suggest that Pin1 and beta-catenin signalling pathway were activated in SACC and might play a pivotal role in SACC carcinogenesis and metastasis.
Adenoid cystic carcinoma (ACC) is a generally slow-growing but highly malignant salivary gland neoplasm with remarkable capacities for local invasion and lung metastasis. The precise characteristics of ACC are not fully understood because there was no suitable animal model. We have successfully established a new human tumor line (ACCI) derived from ACC of the oral floor, which showed a cribriform pattern histologically and serially transplantable into nude mice. This tumor developed spontaneous metastasis to the neck at the second passage level, and the histological feature changed from ACC to undifferentiated carcinoma (ACCIM). ACCIM caused spontaneous metastasis to the lung at high incidence when transplanted subcutaneously in nude mice. In this study, we examined the characteristics of this interesting human ACC metastatic line. Tumor fragments were subcutaneously transplanted into nude mice and tumor growth was measured at 1-week intervals. Histological and immunohistochemical examinations were performed. As a result, the tumor growth rate of ACCIM increased as compared to that of ACCI, and the PCNA labeling index was elevated. Furthermore, ACCIM produced multiple metastases to lymph nodes and lungs 5 months after transplantation, and all mice died within 6 months. These multiple metastases were also confirmed in orthotopic transplantation to the tongue. RT-PCR analysis revealed that ACCIM expressed human beta-actin, indicating its human origin. From these findings, ACCIM transplanted into nude mice would provide a useful model for investigating the biological behaviour of ACC.