Adenoid Cystic Carcinoma of Esophagus with Lung Metastasis: Case Report.

Adenoid cystic carcinoma (ACC) is a malignant tumor originating in the salivary glands. It most commonly affects the salivary and lacrimal glands, with less frequent occurrences in the esophagus. Esophageal ACC (EACC) typically manifests in the middle or lower parts of the esophagus, with exceedingly rare instances in the upper part. Lung metastasis in EACC is uncommon, and understanding its clinical features and treatment strategies remains challenging. In this study, we present a case of ACC originating in the upper esophagus with lung metastasis. The patient, a middle-aged female, was admitted to the Department of Respiratory and Critical Care Medicine due to an esophageal mass discovered during physical examination that had been present for 4.5 years, along with a newly identified pulmonary nodule for 2 weeks. An X-ray barium meal revealed the presence of a benign esophageal cervical mass. Gastroscopy revealed elevated lesions below the esophageal inlet, and a pathological biopsy confirmed the diagnosis of EACC. The aim of this case report is to enhance understanding of this rare condition and improve clinicians' awareness of the disease. By providing details of the patient's diagnosis, clinical presentation, imaging features and pathological features, we aim to improve diagnostic accuracy and clinical management of similar cases in the future.

Carcinosarcoma of the parotid gland: a case report and review of the literature.

BACKGROUND: Carcinosarcoma of the parotid gland is an extremely rare malignancy comprising of 0.04-0.16% of all salivary gland tumors. This is the first case of an adenoid cystic carcinoma with chondrosarcoma to the best of our knowledge. They consist of distinct carcinomatous and sarcomatous components and may arise de novo or from a preexisting pleomorphic adenoma. CASE PRESENTATION: Herein we present a case of an 80-year-old white female who presented with progressively increasing left facial swelling over 6 weeks. Magnetic Resonance Imagining revealed a mass (3.4 cm) in the parotid gland with a predominant cystic/necrotic component. The cytology was atypical (Milan3) and a total parotidectomy and selective lymph node dissection was done. The resection showed extensive necrosis with high grade sarcomatous (chondrosarcoma) areas. The epithelial component was adenoid cystic carcinoma with perineural invasion. The patient is currently undergoing radiotherapy of the tumor bed and skull base due to propensity of perineural invasion of the adenoid cystic component. The most common carcinomas in carcinosarcomas of salivary glands are adenocarcinoma and squamous cell carcinoma. CONCLUSION: Carcinosarcoma is a high-grade aggressive lesion with a poor prognosis and should be treated aggressively. More studies are needed to understand the origin of these tumors.

Treatment outcomes of single-fraction stereotactic radiosurgery for adenoid cystic carcinoma: a case series of 55 patients.

PURPOSE: This study aimed to analyze the treatment outcomes of single-fraction stereotactic radiosurgery (SRS) for adenoid cystic carcinoma patients. METHODS: Retrospective analysis was conducted for 55 patients with 66 lesions. SRS intentions were categorized as definitive, adjuvant, salvage, and palliative. Tumor control was defined as local (within 50% isodose line), marginal (outside 50% isodose line), and distant (metastasis outside head/neck). RESULTS: The median age was 60 years (range 21-85), with 53% males. Tumor origin was head/neck for 88% and trachea/lung for 12%. 61% were recurrent lesions. Median interval from diagnosis to SRS was 14 months. Preceding surgery was performed in 30%. SRS was administered as definitive (30 lesions), adjuvant (13), salvage (19), and palliative (4). SRS was used as a boost to external beam radiation therapy (EBRT) in 39%. Concurrent chemotherapy was administered in 26%. 5-, 10-, and 15-year local control rates were 60%, 33%, and 27%, respectively; local/marginal control rates were 29%, 13%, and 10%. For recurrent lesions treated with SRS without EBRT, 5-year local control rate was 14%, and local/marginal control rate was 5%. For recurrent lesions treated with SRS and EBRT, 5-year local control rate was 100%, and local/marginal control rate was 40%. The rate of distant failure after SRS was 40%. Older age and distant metastasis before SRS were negative factors for overall survival. CONCLUSION: SRS provided a high rate of local tumor control, but marginal failure was frequent. Integrating SRS with added EBRT exhibits potential for enhancing local and local/marginal tumor control, particularly in recurrent cases.

Solid basal adenoid cystic carcinoma of the breast: A case report and literature review.

RATIONALE: Adenoid cystic carcinoma (AdCC) is a rare malignancy of the breast with a low Ki-67 index and good prognosis. Owing to the rarity of breast AdCC, the misdiagnosis rate is as high as 50%, and there is no consensus or recognized guidelines for the treatment of this disease. Therefore, it is necessary to conduct a detailed clinical and pathological analysis in combination with a literature review to improve our understanding, diagnosis, and treatment of the disease. METHODS: A 68-year-old woman sought medical attention due to a recently increasing mass in the breast. The left breast mass was 1.3 cm × 1 cm in size. We analyzed the morphology, immunohistochemistry, and molecular characteristics of the tumor removed by surgery, and reviewed relevant literature. DIAGNOSES: Solid basal AdCC of the breast. INTERVENTIONS: We performed biopsy, immunohistochemistry and molecular testing on surgical resection specimens. OUTCOMES: Combining morphological and immunohistochemical features, it is consistent with solid basal AdCC of the breast, and Fish detected MYB gene break. LESSONS: Due to the high misdiagnosis rate of AdCC, accurate histopathological diagnosis is particularly important. At present, breast conserving surgery and local tumor resection are mainly used for the treatment of breast AdCC, and postoperative adjuvant radiotherapy is feasible.

Rare but clinically important salivary gland-type tumor of the lung: A review.

Salivary gland-type tumor (SGT) of the lung, which arises from the bronchial glands of the tracheobronchial tree, was first recognized in the 1950s. SGT represents less than 1% of all lung tumors and is generally reported to have a good prognosis. Mucoepidermoid carcinoma (MEC) and adenoid cystic carcinoma (ACC) are the two most common subtypes, comprising more than 90% of all SGTs. The reported 5-year survival rate of patients with SGT is 63.4%. Because this type of tumor develops in major bronchi, patients with SGT commonly present with symptoms of bronchial obstruction, including dyspnea, shortness of breath, wheezing, and coughing; thus, the tumor is usually identified at an early stage. Most patients are treated by lobectomy and pneumonectomy, but bronchoplasty or tracheoplasty is often needed to preserve respiratory function. Lymphadenectomy in the surgical resection of SGT is recommended, given that clinical benefit from lymphadenectomy has been reported in patients with MEC. For advanced tumors, appropriate therapy should be considered according to the subtype because of the varying clinicopathologic features. MEC, but not ACC, is less likely to be treated with radiation therapy because of its low response rate. Although previous researchers have learned much from studying SGT over the years, the diagnosis and treatment of SGT remains a complex and challenging problem for thoracic surgeons. In this article, we review the diagnosis, prognosis, and treatment (surgery, chemotherapy, and radiotherapy) of SGT, mainly focusing on MEC and ACC. We also summarize reports of adjuvant and definitive radiation therapy for ACC in the literature.

Apoptotic Marker Expression of Resected Lacrimal Gland Adenoid Cystic Carcinoma Tumor Margins After Intra-arterial Chemotherapy and Globe-Sparing Excision.

PURPOSE: Lacrimal gland adenoid cystic carcinoma (LGACC) is a rare orbital malignancy with devastating lethality. Neoadjuvant intra-arterial chemotherapy (IACC) has demonstrated cytoreductive effects on LGACC macroscopically, but limited studies have examined cellular and molecular determinants of the cytoreductive effect. This post hoc study assessed apoptotic marker expression on excised tumor specimens after neoadjuvant IACC and globe-sparing resection, emphasizing the examination of tumor margins. METHODS: This retrospective study identified LGACC specimens resected in a globe-sparing technique after neoadjuvant IACC by reviewing the Florida Lions Ocular Pathology database at Bascom Palmer Eye Institute. Histopathology slides of the specimens were re-examined to confirm the diagnosis and identify the tumor margin. Immunofluorescent staining was performed for apoptotic markers, including P53, cleaved caspase-3, cleaved PARP-1, and terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL). Positive expression was determined by comparison to the negative control. RESULTS: Tumor specimens from 5 patients met inclusion criteria. All 5 cases were positive at the center and the margin for TUNEL, p53, and cleaved caspase-3. One case did not show positive expression of cleaved PARP-1 at the margin but was positive for the other apoptotic markers. CONCLUSIONS: This post hoc study demonstrated positive staining for multiple apoptotic markers in post-IACC tumor specimens at the tumor center and margin. Apoptotic marker expression along the margins of post-treatment specimens is important, as it may offer surrogate information to speculate on the state of residual cancer cells adjacent to the excision margin inadvertently remaining in the orbit.

Post-operative radiotherapy in adenoid cystic carcinoma of salivary glands versus surgery alone: what is the evidence about survival and local control? A systematic review and meta-analysis.

BACKGROUND: Adenoid cystic carcinoma of the salivary glands is a relatively rare malignancy characterized by slow growth and a poor prognosis, and effective treatments remain challenging to identify. This systematic review, following the PRISMA guidelines, aimed to analyze the potential benefits of post-operative radiotherapy in terms of local control of recurrences and survival advantages when compared with surgery alone in patients with adenoid cystic carcinoma. METHODS: A comprehensive systematic review was conducted by searching the MEDLINE, Cochrane, EMBASE, and OVID databases from January 1999 to July 2022. The goal was to identify articles comparing surgery alone with surgery plus postoperative radiotherapy for adenoid cystic carcinoma of the salivary glands. Downs and Black Checklist was used to assess the methodological quality and risk of bias of each included study. The data analysis was performed using Review Manager version 5.4.1. RESULTS: This review included 8 studies comprising a total of 3103 patients, divided based on the analyzed outcomes. The pooled odds ratio for overall survival at 5 years was 0.87 (95% confidence interval 0.43-1.76, p = 0.70), and at 10 years was 1.23 (95% confidence interval 0.69-2.16, p = 0.48). In both cases, no statistically significant differences were observed. However, the pooled odds ratio for local control at 5 years was 3.37 (95% confidence interval 1.35-8.42, p = 0.009), providing strong support for the use of post-operative radiation. CONCLUSIONS: The findings from the meta-analysis suggest that post-operative radiotherapy significantly improves local control in patients with adenoid cystic carcinoma. However, there was no statistically significant increase in survival at 5 and 10 years. It is essential to note that the quality of the studies included in this meta-analysis ranged from fair to poor. To better clarify the indications for post-operative radiotherapy, future high-quality research is needed, particularly with improved stratification of patient groups. Additionally, it is important to recognize that achieving local control in adenoid cystic carcinoma is crucial for enhancing the overall quality of life for patients. We acknowledge that this review was not registered in the PROSPERO database, and the data pooling was conducted using a random effects model.

Adenoid cystic carcinoma of the thymus gland.

BACKGROUND: Thymic carcinomas are rare and aggressive tumours. They constitute a heterogeneous group of tumours with various histological patterns and subtypes resembling epithelial tumours arising from other organs. CASE PRESENTATION: We hereby represent a case of primary thymic carcinoma with adenoid cystic carcinoma-like features (TCACC) which is an extremely rare variant of thymic adenocarcinoma. To date and to the best of our knowledge, there are nine reported cases in literature and ours is the tenth. Our case was treated surgically but the implementation of adjuvant chemoradiotherapy has been reported in few of the published cases. CONCLUSIONS: TCACC constitutes a rare entity of thymic adenocarcinoma with limited available literature. The current data is derived from few case reports and case series. The histological overlap of these tumours and primary ACC of salivary glands poses a diagnostic challenge. Radiological investigations, immunohistochemical phenotyping and genetic analysis are crucial in establishing the diagnosis.

Locally Advanced Adenoid Cystic Carcinoma of the Breast-A Case Report with a Review of the Literature.

Breast cancer (BC) is a heterogeneous disease distinguished by four main subtypes based on the expression of estrogen, progesterone receptors, and human epidermal growth factor-2 on the cancer cells. Triple-negative breast cancer (TNBC) consists of approximately 10-20% of all BCs and is characterized by a poor prognosis. Adenoid cystic carcinoma (ACC) of the breast is a rare, special type of TNBC with low metastatic potential and usually favorable prognosis. There are no established recommendations concerning systemic therapy in advanced ACC. We present a case of a 70-year-old woman with locally advanced ACC with progression after radical mastectomy, and review the literature concerning the treatment of metastatic disease focused on systemic therapy.

Successful surgery with preservation of laryngeal function in a patient with collision primary squamous cell carcinoma and adenoid cystic carcinoma in the hypopharynx and synchronous esophageal carcinoma: A case report.

BACKGROUND: The definition of "collision tumor" is the coexistence of two histologically and morphologically distinct tumors within the same anatomical area without histological admixture. Collision tumors featuring primary squamous cell and adenoid cystic carcinomas of the hypopharynx, combined with synchronous esophageal carcinoma, are very rare. METHODS: We describe a patient with a collision tumor of the hypopharynx and synchronous esophageal carcinoma who underwent partial laryngectomy, with preservation of laryngeal function, and radical esophageal resection featuring esophageal reconstruction using a gastric tube. Surgery was successful. RESULTS: Postoperative radiotherapy was recommended after surgery; the patient exhibited no recurrence or distant metastasis to the 17-month follow-up. CONCLUSION: To the best of our knowledge, this is the first report of collision of primary squamous cell carcinoma and adenoid cystic carcinoma in the hypopharynx and synchronous esophageal carcinoma. We performed appropriate surgery and prescribed postoperative radiotherapy. This preserved laryngeal function.

Outcomes and predictors of survival for tracheal cancer.

INTRODUCTION: Primary tracheal cancer is uncommon, with poor survival. While surgical resection is the mainstay of therapy, the role of chemotherapy and radiation is poorly defined. We aimed to study the impact of treatment modalities on survival. METHODS: Patients with primary tracheal cancer were identified from the National Cancer Database over 12 years, 2004-2015. Patient characteristics, tumor characteristics, treatment modalities, and survival were recorded. Factors associated with survival were assessed using Cox Regression. RESULTS: Of the 1726 patients identified, 59% were male, 83% White race, 62% had a comorbidity index of zero, median age 64 years, median tumor size 2.7 cm, and median survival was 28.5 months (89 months for patients undergoing surgical resection). Cox Regression for all patients found adenoid cystic carcinoma (ACC) (p < 0.001), radiation (p < 0.001), and surgical resection (p < 0.001) to be associated with improved survival, while increasing age (p < 0.001) decreased survival. For patients receiving resection, ACC (p < 0.001) was associated with improved survival, while increasing age (p < 0.001) and positive margins (p = 0.002) were associated with worse survival. For R0 resections, ACC (p < 0.001) was associated with improved survival, while increasing age (p < 0.001) decreased survival, with chemotherapy and radiation having no impact. For R1/2 resections, ACC (p < 0.001) and radiation (p < 0.001) were associated with improved survival, while increasing age (p < 0.001) decreased survival, with chemotherapy having no impact on survival. CONCLUSIONS: Primary tracheal cancer is highly lethal, with surgical resection leading to the best chance of survival. For patients undergoing resection, radiation provided survival benefits for R1/2 but not R0, while chemotherapy did not impact survival regardless of margin status.

Subglottotracheal Adenoid Cystic Carcinoma in a 16-Year-Old Female-A Case Report.

Cystic adenoid carcinoma (ACC) is a rare malignant epithelial tumor arising from exocrine glands and accounts for only 1% of head and neck cancers. ACCs are common in the fifth and sixth decades of life, predominantly in women, and characterized by slow progression, local aggression, recurrence, and high metastasis. Subglottotracheal ACC is a rare tumor in the pediatric population, with only a few cases reported in the literature. We present a case of a 16-year-old female who was diagnosed with ACC in the subglottic and tracheal region. The patient presented with respiratory failure but without a history of dysphonia, dyspnea, stridor, or dysphagia. The diagnosis was confirmed by a biopsy, and subsequent imaging studies showed a large tumor involving the subglottic and tracheal region. The therapeutic management of this patient has been challenging due to the rarity of this tumor in the pediatric population and the potential long-term complications associated with tumor recurrence and psychological impact. This case highlights the diagnostic and therapeutic challenges in the management of subglottotracheal ACC in children and the importance of a multidisciplinary approach to optimize patient outcomes.

The clinical significance for primary tumor surgery in metastatic head and neck adenoid cystic carcinoma.

PURPOSE: This study aimed to determine the prognostic significance and optimal candidates for primary tumor surgery (PTS) among patients with metastatic head and neck adenoid cystic carcinoma (HNACC). METHODS: The data were retrieved from Surveillance, Epidemiology, and End Results (SEER) database. Patients with metastatic HNACC at the initial diagnosis were included in this study. Univariate survival analysis was performed using the Kaplan-Meier method, and the difference in survival curves between PTS and non-PTS groups was estimated using the log-rank test. Multivariate analysis was performed to evaluate the independent prognostic factors associated with overall survival (OS) and cancer-specific survival (CSS). RESULTS: Overall, 155 patients were eligible, of whom 93 underwent palliative PTS. Patients with lung metastasis alone were more likely to undergo PTS. PTS was associated with significantly improved OS and CSS compared with non-PTS. In the multivariate model, patients who underwent PTS had better OS than those who did not undergo PTS; however, no improvement was observed in the CSS. Subgroup analyses further revealed that patients aged < 60 years with T3-4 or N0 classification might benefit from PTS. CONCLUSION: PTS significantly improved the OS of patients with metastatic HNACC. PTS had a favorable prognostic impact on highly selected patients, namely, those aged < 60 years with T3-4 and N0 classification, which could be adopted in future clinical practice.

Optimal surgical procedure for treating early-stage adenoid cystic carcinoma of the breast.

To explore the superiority of breast conservation surgery (BCS) to mastectomy in treating early-stage adenoid cystic carcinoma of the breast (BACC). Patients with surgically treated stage I/II BACC were enrolled between 2000 and 2019 in the SEER database; they were divided into the BCS and mastectomy groups. Overall survival (OS) and disease-specific survival (DSS) were compared between the two groups, and Cox hazard regression models were used to determine the independent predictors. Of the 583 patients in the study, 386 were included in the BCS group. The 10-year OS rates for the BCS and mastectomy groups were 78% (95% CI: 74-82%) and 76% (95% CI: 70-82%), respectively, but the difference was not statistically significant (p = 0.968). The 10-year DSS rates for the BCS and mastectomy groups were 95% (95% CI: 93-97%) and 89% (95% CI: 85-93%), respectively, and the difference was statistically significant (p = 0.002). Pathological examination of regional lymph nodes and adjuvant treatment were not associated with improved OS or DSS, but age, disease grade, and lymph node metastasis were independent prognostic factors. For stage I/II BACC, BCS can achieve more satisfactory 10-year OS and DSS than mastectomy.