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Microglandular adenosis with transition into adenoid cystic carcinoma of the breast.

Microglandular adenosis (MGA) is a well-recognized, if rare and incompletely characterized, entity in which carcinoma is rarely thought to develop. We report 17 cases in which patterns of adenoid cystic carcinoma (ACC) coexisted with MGA. Immunocharacterization with beta-catenin, E-cadherin, cytokeratins (AE1/AE3), epithelial membrane antigen, S-100 protein, smooth muscle actin, and vimentin was also performed. Most cases had areas of invasive ACC characterized by its defining dual-lumen types. Some cases of ACC appeared to have expanded glands intermingled within the MGA, whereas in other cases ACC formed a transition with the characteristic small, gland-like spaces of MGA. MGA and "atypical MGA" stained irregularly and similarly to that seen in myoepithelium with the three markers of myoepithelial cells in breast: S-100 protein, smooth muscle actin, and vimentin. These markers were also positive in the more solid elements of the ACC. Our study suggests that ACC may develop in a background of and in continuity with MGA. Altered myoepithelial cells appear to be the major neoplastic element in both ACC and "atypical MGA." "Atypical MGA" with transition to ACC may show histologic patterns and an immunohistochemical profile similar to that of ACC. These lesions might be best interpreted as ACC in situ. Both MGA and ACC of the breast grow in an expansile and diffusely infiltrative pattern without having significant metastatic capacity. Their unusual interaction with the surrounding stroma may play a role in this benign biologic behavior.

Adult↗

Adenoid cystic carcinoma of the breast. Data from the Connecticut Tumor Registry and a review of the literature.

The case records of the Connecticut Tumor Registry were reviewed from 1952-1982. There were 37 cases of adenoid cystic carcinoma of the breast (ACC) from a total of 40,350 invasive breast tumors. Patient survival, complications, and pathologic sections were reviewed. Only 14 of 27 surgical pathology slides available for review could be confirmed histologically as ACC. All patients were white females with a mean age of 64 years. The tumor remained localized to the breast in all cases. Nine patients had either radical or modified radical mastectomy, four patients had either simple mastectomy or lumpectomy, and one patient refused treatment. There was no evidence of axillary node involvement, metastases, or local recurrence after excision. At the time of follow-up, nine patients were alive and disease free and four died of disease unrelated to their breast cancer. The one patient who died of breast cancer had a radical mastectomy and survived 11.7 years after diagnosis. It is concluded that ACC has a favorable biologic behavior characterized by a prolonged clinical course and good prognosis. Simple mastectomy is all that is required as initial treatment, and a chest x-ray and thorough physical examination looking for local recurrence is all that is needed for follow-up.

Aged↗

Tuberculous granuloma and adenoid cystic carcinoma presenting as a single buccal space mass.

We report an unusual case of a 58-year-old man with a "collision lesion" of the buccal space. Initially diagnosed as tuberculoma, the patient showed only partial response to an extensive course of antibiotics, prompting more vigorous investigation. This revealed the presence of a concomitant malignancy, specifically adenoid cystic carcinoma. Definitive treatment was then achieved by surgical resection and flap reconstruction with postoperative radiation therapy.

Carcinoma, Adenoid Cystic↗

Neurotrophic spread of both neuroblastoma and adenoid cystic carcinoma in the maxillofacial district: common aspects of pre- and postsurgical adjuvant treatment. Multidisciplinary study.

We have studied a common aspect of both adenoid cystic carcinoma (ACC) and neuroblastoma (NB) with the purpose of finding the optimal medical and surgical treatment. This article is based on a case study involving 22 cases of ACC and NB performed at the University of Rome La Sapienza from 1993 to 2001. Of the 22 cases, 17 were diagnosed with ACC and 5 with NB. ACC largely involved the maxillae, having been detected in this location in eight cases. Two of these patients, one affected by ACC and the other affected by NB, received neoadjuvant treatment, whereas 14 were affected by ACC, and 3 with NB received adjuvant treatment. Despite the fact that the two neoplasms have different embryogenic structures, they share common aspects. Both spread invasively through the perineural sheets, and both are responsive to radiotherapy or chemotherapy. The common biologic behavior and the similar response that both show to neoadjuvant radiotherapy or chemotherapy may suggest a way to develop a more effective treatment protocol. The aim of this article is to suggest a line of research along that path.

Adult↗

Adenoid cystic carcinoma of the lacrimal gland: a case report.

A 70-year-old woman was referred to our clinic for evaluation and treatment of a recurring case of adenoid cystic carcinoma of the lacrimal gland. She had been diagnosed with this tumor three years earlier, and a local resection was attempted on two occasions. We encountered this patient on the third recurrence of the tumor, and a thorough work-up revealed extensive local involvement but no metastases. A total exenteration of the right orbit was then performed with aggressive resection of local extension followed by irradiation. Thirteen years later, our patient presented with metastases throughout her scalp. She died shortly thereafter, having survived a total of 16.5 years after the time of her initial treatment and 13 years after exenteration with bony removal of the orbit and irradiation. A review of the literature reveals that this patient had one of the longest reported survival times in the literature from the time of initial treatment. Also noteworthy is the 13 years this patient survived after aggressive surgical resection combined with radiotherapy following two previous incomplete resections.

Aged↗

Primary adenoid cystic carcinoma of the lacrimal sac: report of a case.

A 41-year-old white woman presented with a 1-month history of epiphora and a painless medial canthal mass on the left that was unresponsive to antibiotic treatment. Computerized tomography (CT) and magnetic resonance imaging (MRI) scans delineated a soft tissue mass with bony destruction originating in the area of the left lacrimal sac with extension into the maxillary and ethmoid sinuses and inferomedial orbit. Open biopsy of the mass revealed adenoid cystic carcinoma (ACC). On surgical exploration, the tumor was found to originate from the lacrimal sac wall. Radical surgery with wide excision of surrounding bone and periorbital tissue was performed in light of the histological diagnosis and tumor extension. Orbital exenteration was not performed in order to preserve the patient's left eye. A 6-week course of adjunctive radiotherapy was applied without complication. The patient was clinically and radiologically free of tumor at 1-year follow-up. This case represents only the third time that primary ACC arising from the lacrimal sac has been reported.

Adult↗

Salivary adenoid cystic carcinoma. Features of an intra-abdominal metastasis.

An adenoid cystic carcinoma of salivary gland origin metastasized to the abdomen four years after the initial diagnosis. It showed heavy uptake of Ga-67 citrate and hypervascularity. The clinical and pathologic features are discussed. Gallium scan and arteriography (usually external carotid artery injection) may prove helpful in the early diagnosis and management in the various phases of the disease.

Abdominal Neoplasms↗

Adenoid cystic carcinoma of the trachea.

OBJECTIVE: Primary tracheal tumors are rare, occurring in 0.2 per 100,000 persons per year. Adenoid cystic carcinoma (ACC) is the second most common histologic type of tracheal malignancy. Its clinical behavior is different from the other tracheal neoplasms and thus should be studied separately. STUDY DESIGN/METHODS: Retrospective review of the medical records of six patients with tracheal ACC who were treated at University Hospitals of Cleveland between 1971 and 1996 and literature review. RESULTS/CONCLUSION: Tracheal ACC is an indolent tumor that affects people at any age but has a peak incidence in the fifth decade. There is a nearly equal male-to-female ratio. Almost half of tracheal ACCs occur in the proximal trachea, accounting for the most common presenting symptoms: dyspnea, cough, and hoarseness. Because of the hoarseness, patients are often referred to an otolaryngologist. Complete resection provides the best chance for increased survival. Neutron beam radiotherapy holds promise for adjuvant therapy.

Adult↗

Neural cell adhesion molecule expression in adenoid cystic carcinoma of the head and neck.

OBJECTIVE: To investigate whether there is a correlation between neural cell adhesion molecule (NCAM) expression and perineural spread in patients with adenoid cystic carcinoma of the head and neck (ACCHN). STUDY DESIGN: Retrospective review of medical records and immunohistochemical staining of specimens from 37 patients treated at the University of Arkansas in Little Rock from 1987 to 1997. METHODS: Sections from paraffin-embedded specimens were examined for the presence of NCAM using monoclonal anti-NCAM antibody by avidin-biotin-peroxidase immunohistochemical staining. NCAM staining was scored in each specimen and correlated with the data obtained from patient charts. RESULTS: Twenty-five of 37 specimens (68%) showed histopathological evidence of perineural spread. All 37 specimens (100%) stained positive for NCAM, regardless of perineural spread status. CONCLUSION: Our results suggested that the use of NCAM expression as a predictor of perineural spread is highly unlikely.

Adult↗

Molecular differences in mucoepidermoid carcinoma and adenoid cystic carcinoma of the major salivary glands.

OBJECTIVE/HYPOTHESIS: Mucoepidermoid carcinoma (MEC) and adenoid cystic carcinoma (ACC), the most common malignancies of the major salivary glands, are clinically and pathologically different. To determine whether MEC and ACC have different molecular characteristics, we examined the expression of erbB-2, erbB-3, epidermal growth factor receptor (EGFR), and transforming growth factor-alpha (TGF-alpha), important molecular features in other malignancies. STUDY DESIGN/METHODS: Archival tissue sections of 22 MEC and 6 ACC tumors of the major salivary glands were evaluated immunohistochemically for expression of erbB-2, erbB-3, EGRF, and TGF-alpha. A differential immunostaining score, reflecting the difference in immunostaining between carcinoma and uninvolved salivary gland tissue, was calculated for cytoplasmic and membranous staining. RESULTS: Positive immunostaining for all biomarkers was observed in the cytoplasm and membrane of both tumors. However, expression was higher in MEC than in ACC tumors and was statistically significant for cytoplasmic EGFR (P =.009), TGF-alpha (P =.041), and membranous EGFR (P =.004). A significantly higher percentage of MEC cells also demonstrated positive immunostaining for cytoplasmic erbB-3 (P =.022), EGFR (P =.005), membranous erbB-3 (P =.022), and EGFR (P =.013). The differential immunostaining score was significantly higher for MEC compared with uninvolved alveolar tissue and the membranes of uninvolved ductal tissue. There were no statistically positive differential immunostaining scores for ACC. CONCLUSIONS: There is a clear difference in the molecular phenotypes of MEC and ACC. The lack of statistically significant expression in ACC, when compared with similar uninvolved salivary gland tissue, suggests minimal involvement for these molecular structures in the pathogenesis of ACC. Conversely, erbB-2, erbB-3, EGFR, and TGF-alpha may have a role in the development and progression of MEC. These results have therapeutic implications for MEC of the major salivary glands.

Carcinoma, Adenoid Cystic↗

Expression of intercellular adhesion molecule (ICAM)-1 in adenoid cystic carcinoma of the head and neck.

OBJECTIVES/HYPOTHESIS: Adenoid cystic carcinoma of the head and neck (ACCHN) is characterized by late recurrence and frequent distant metastasis. Tumor attack by cytotoxic T lymphocytes and macrophages is mediated by the interaction of leukocyte function-associated antigen (LFA)-1 on lymphocytes with intercellular adhesion molecule (ICAM)-1 on the tumor surface. Thus, the reduced expression of ICAM-1 on tumor cells could contribute to their escape from host immune surveillance. To investigate the relationship between the clinical features of ACCHN and host immune surveillance, the expression of ICAM-1 and infiltration of T/natural killer (NK) cells and macrophages were immunohistochemically examined. STUDY DESIGN: Retrospective analysis of immunohistochemical tumor characteristics and clinical outcome. METHODS: Immunohistochemical study of ICAM-1, T/NK cells, and macrophages was performed on paraffin sections of 42 patients with ACCHN. The expression of T/NK cells and macrophages was represented by T-cell-restricted antigen (TIA)-1 and CD68 expression, respectively. The expression of these molecules and clinical features were analyzed. RESULTS: Of 42 ACCHN cases, 15, 9, and 15 patients were classified as ICAM-1 high, TIA-1 high, and CD68 high, respectively. The TIA-1 expression scores in ICAM-1-low patients were significantly lower than those in ICAM-1-high patients (1.3 +/- 3.7 vs. 8.3 +/- 12.7, P =.0031). The CD68 expression scores in ICAM-1-low patients were also significantly lower than those in ICAM-1-high patients (9.6 +/- 9.6 vs. 21.1 +/- 17.6, P =.0047). Moreover, ICAM-1-high patients had a significantly better disease-free survival rate (P =.043). CONCLUSIONS: Reduced expression of ICAM-1 may promote immune evasion and metastasis, resulting in poor prognosis in ACCHN.

Adult↗

Role for postoperative radiation therapy in adenoid cystic carcinoma of the head and neck.

OBJECTIVE: Clarify the role for postoperative radiation for adenoid cystic carcinoma (ACC) of the head and neck as it relates to tumor site, T-stage, and surgical margin status. STUDY DESIGN: Retrospective cohort study at an academic tertiary care hospital. METHODS: A review of 129 patients with biopsy-proven ACC was performed. Previous treatment failures and nonoperative candidates were excluded, with 75 patients considered eligible for further study. Patients were grouped according to treatment modality and Kaplan-Meier estimates of overall survival, locoregional control, and distant control were compared using log-rank tests. Patients were also stratified according to tumor site, T-stage, and surgical margin status, and pair-wise comparisons of treatment outcome within each group were performed using Wald tests from Cox proportional hazards models. RESULTS: Twenty-five patients were treated with surgery alone, and 50 were treated with surgery and postoperative radiation. There was no significant difference in outcome between treatment groups when correlated with tumor site (P =.89). However, postoperative radiation was associated with improved overall survival for advanced T-stage (T4) tumors (P =.019) and greater locoregional control for patients with microscopically positive margins (P =.018). There was no demonstrated benefit of postoperative radiation for patients with microscopically negative margins (P =.93). CONCLUSIONS: The findings of this study suggest that advanced T-stage and positive microscopic margins are important factors in determining the necessity for postoperative radiation therapy for ACC of the head and neck and that radiation therapy may not be necessary for patients with early T-stage tumors and negative surgical margins.

Adolescent↗

Peripheral adenoid cystic carcinoma of the lung.

The large peripheral lung mass found in a 63-year-old female veteran proved to be adenoid cystic carcinoma of the lung. These rare primary lung tumors should be distinguished from adenocarcinoma, as the biologic activity and prognosis are much more favorable.

Carcinoma, Adenoid Cystic↗

Chemoradiotherapy for adenoid cystic carcinoma: preliminary results of an organ sparing approach.

OBJECTIVES: A retrospective review of primary chemoradiotherapy (CRT) for adenoid cystic carcinoma (ACC) was performed to determine if CRT might be considered as an alternative to radiotherapy and/or definitive surgery. METHODS: All treatment-naive patients with ACC of the head and neck who were treated with definitive primary chemoradiotherapy using carboplatinum and paclitaxel at Dana-Farber Cancer Institute in 2000 through 2004 were identified. Information on site, stage, presenting symptoms, performance status, treatment, toxicity, and follow up were collected and tabulated for review. RESULTS: Five patients were identified with previously untreated ACC of the head and neck who received primary carboplatinum/paclitaxel CRT for unresectability or organ preservation. Patients had a median age of 41, 4 had primaries in the paranasal sinuses invading the base of skull and 1 had a transglottic laryngeal lesion. All patients completed a course of definitive chemoradiotherapy without treatment break. Grade 3 mucosal reactions developed during CRT in all patients. With a median follow-up of 36 months (range, 20-43) all patients have local regional control; 1 patient developed distant metastases at 7 months and is alive at 20 months. CONCLUSIONS: Preliminary data suggest that carboplatinum/paclitaxel based CRT for ACC provides local regional control and is a potential alternative to surgery or radiotherapy for patients with locally advanced ACC. Carboplatinum/paclitaxel based CRT warrants further study.

Adult↗

Adenoid cystic carcinoma of the major salivary glands treated with surgery and radiation.

OBJECTIVE: To examine patient characteristics, pathologic features, and treatment outcomes of adenoid cystic carcinoma of the major salivary glands. STUDY DESIGN: Retrospective review of patients in an academic medical center. METHOD: Review of medical records regarding demographics, extent of tumor, stage, histologic characteristics, and treatment outcomes of patients treated with surgery and postoperative radiation. RESULTS: Of the 33 patients, 19 (58%) were male, and 14 (42%) were female. The average age of presentation was 49 (range 22-81) years. Of the 29 patients fully staged at the time of diagnosis, 7 (24%) presented at American Joint Committee on Cancer stage I, 9 (31%) at stage II, 4 (14%) at stage III, and 9 (31%) at stage IV. The cribriform histologic subtype was predominant (64%). The majority originated in the parotid gland (21, 64%), with the remaining originating in either the submandibular gland (10, 30%) or the sublingual gland (2, 6%). Local control was 94% at 5 years and 73% at 10 years. Metastatic control was 82% at 5 years and 63% at 10 years. Four patients failed locally and nine failed distally. Overall survival was 85% at 5 years and 69% at 10 years, with a median of 12.9 years. CONCLUSION: Surgical excision with postoperative radiation provides a long period of disease-free survival. Patients were more likely to fail with metastases than with local recurrence.

Adult↗