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.

Metastatic salivary gland neoplasms to pleural effusion: diagnostic challenges and prognostic significance in a series with 9 patients.

INTRODUCTION: Pleural effusions can present a diagnostic challenge as they are not always caused by malignancy in patients with a history of typical visceral primaries. MATERIAL AND METHODS: At 2 major academic medical centers, we have identified several cases in which salivary gland neoplasms metastasized to pleural effusions in patients who have been aggressively managed with various treatment modalities including chemotherapy, radiation, and/or surgical excision. RESULTS: Herein, we present a range of primary salivary gland tumors that metastasized to serous effusions and characterize their cytomorphology, immunoprofiles, and clinical courses. Our case series shows that many tumor types metastasize to pleural effusions and they present unique diagnostic challenges in each case. We found that metastasis of a salivary gland neoplasm to a pleural effusion is a late-stage event and is often associated with poor prognosis. CONCLUSIONS: This series serves as a resource to demonstrate the cytomorphologic and immunohistochemical features of malignant pleural effusions due to salivary gland neoplasms and draws attention to poor prognosis in cases of salivary duct carcinoma, mucoepidermoid carcinoma and adenoid cystic carcinoma.

Critical tracheal stenosis from adenoid cystic carcinoma during pregnancy: Case report.

Malignancy during pregnancy complicates approximately 0.1% of patients. Primary tumors of the trachea comprise only 0.2% of respiratory system malignancies. Adenoid cystic carcinoma (ACC) is an adenocarcinoma that can originate from the seromucinous submucosal glands of the trachea and cause airway obstruction. Here we present the collaborative operative management of a Cesarean section delivery for a patient with critical airway obstruction secondary to ACC.

Metaplastic spiradenocarcinoma: Report of two cases with sarcomatous differentiation.

Spiradenocarcinoma (SC) is a very rare malignant skin adnexal tumor with sweat gland differentiation that develops from a pre-existing spiradenoma, cylindroma, or hybrid tumor called spiradenocylindroma, or arises de novo. We present two exceptionally rare SC cases showing sarcomatous differentiation; we also discuss the clinicopathologic features of SC, as well as its differential diagnoses and available therapeutic modalities. Given the aggressive behavior of SC, rapid diagnosis and complete removal of the tumor with tumor-free margins is mandatory. Owing to the marked morphological heterogeneity of individual SC cases, dermatopathologists must be familiar with the different possible histopathologic manifestations of this neoplasm.

Rehabilitation Therapy for Vocal Fold Paralysis Caused by Lung Cancer: A Case Report.

OBJECTIVE: Unilateral vocal fold paralysis (UVFP) can be caused by iatrogenic injury or tumor-induced damage to the recurrent laryngeal nerve. Studies of comprehensive rehabilitation therapies for patients suffering from severe UVFP are limited. The purpose of this case report is to describe an improvement in complete aphonia after comprehensive rehabilitation therapies in a patient with severe UVFP due to a lung tumor. METHODS: An 81-year-old woman with a history of bronchial adenoma had complete aphonia due to compression of the left recurrent laryngeal nerve by the tumor. Dynamic fibrolaryngoscope revealed paralysis of the left vocal fold. The patient was treated with interferential current therapy, vocal training, and kinesiology taping. Indicators of voice recovery were scored according to the grade, roughness, breathiness, asthenia, strain scale, and the voice handicap index. RESULTS: After 10 days of comprehensive rehabilitation treatment, the patient recovered from complete aphonia to normal communication. The hoarseness and breathiness of patient were significantly improved. In addition, the grade, roughness, breathiness, asthenia, strain, and the voice handicap index scores changed from severe to mild or absent. CONCLUSION: This case provided a novel comprehensive treatment for a patient with UVFP, which was safe, cost-effective, and easy to implement in clinic.

[Covered airway stent loaded with (125)I seeds for tracheal adenoid cystic carcinoma: a clinical observation of 8 cases].

Objective: To explore the efficacy and safety of the covered airway stent loaded with (125)I seeds for the treatment of tracheal adenoid cystic carcinoma (TACC). Methods: We retrospectively reviewed the clinical data from 8 patients with TACC who had received placement of the covered stent loaded with (125)I seeds between December 2014 and July 2017 in the endoscopic center of the Second Affiliated Hospital of Xiamen Medical College. We compared the difference in the dyspnea index, the diameter of the airway lumen, and the lesion surrounding the airway wall before and after treatment. The complications were also recorded during follow-up. Results: Eight patients underwent successful placement of a total of 11 radioactive stents (2 straight-type stents, 2 L-shape stents, and 7 Y-shape stents, all loaded a total of 243 radioactive particles). Displacement of stents took place within 2 weeks in 2 patients, who were managed with re-stenting and fixation. No further displacement occurred during follow-up. The median time to stent removal was 2.9(interquartile range: 2.3,3.0) months. After stent placement, the dyspnea index was significantly decreased compared with pre-treatment level (mean: 0.1 vs. 3.4, t=8.881, P<0.001). Bronchoscopic re-assessment showed that the residual tumor within the airway was detected in only one patient and that the tumor completely disappeared in the remaining 7 patients. Treatment with stents loaded with radioactive particles yielded smooth and pale airway mucosa with formation of partial scar formation. Chest computed tomography re-assessment demonstrated significantly larger luminal diameter than that before treatment (mean: 13.1 mm vs. 3.3 mm, t=-7.839, P<0.001). The airway wall thickness was notably reduced after treatment (mean: 4.3 mm vs. 14.4 mm, t=7.620, P<0.001). The lesions surrounding the airway wall completely disappeared in 7 patients and decreased for more than 50% in a single patient. The median duration of follow-up was 28.0(interquartile range: 24.8,31.5) months. Recurrence of tumor was documented in a single case within 2 years. Six patients did not experience recurrence within the 2-year follow-up period. No death or severe complications were recorded during follow-up. Conclusion: The (125)I radioactive stent is effective for dilating the stenotic airway and ameliorating the symptoms, and thus might be an effective and safe method for the treatment of TACC. Further studies that explore the efficacy of stents loaded with (125)I particles are needed.

Tonic pupil caused by adenoid cystic carcinoma versus postradiation changes to the ciliary ganglion.

A tonic pupil, without other features of an oculomotor neuropathy, is due to a lesion in the ciliary ganglion or short ciliary nerves. Here, we present a case of a tonic pupil in a woman with radiation-treated adenoid cystic carcinoma of the nasopharynx with perineural spread and skull base involvement. This a rare case of a tonic pupil caused by direct invasion of the ciliary ganglion or postradiation effects.

Primary Adenoid Cystic Carcinoma of the Upper Anterior Mediastinum Mimicking a Thyroid Tumor: A Case Report and Review of Literature.

Primary adenoid cystic carcinoma (ACC) of the upper anterior mediastinum mimicking a thyroid tumor has rarely been seen in clinical practice and lacks a standard of care therapy. Here, we report a 47-year old female patient with an ACC originated from the upper anterior mediastinum presenting as a thyroid gland tumor. The patient received gross surgical resection of the tumor and underwent post-surgical chemotherapy and radiotherapy. The patient was free from local recurrence 3-years following initial treatment, but developed multiple lung metastases. She remains under clinical observation without discomfort and is still followed as an outpatient. Here, we also summarized recent reports of similar cases with hope to provide some experience for future clinical practice.

Long-term outcomes and clinicogenomic correlates in recurrent, metastatic adenoid cystic carcinoma.

BACKGROUND: Little is known about the long-term impact of local and systemic therapies for recurrent/metastatic (R/M) adenoid cystic carcinoma (ACC), or the clinical significance of molecular alterations. METHODS: We identified 72 R/M cases among 123 ACC patients from our institution. We report long-term outcomes, predictors of recurrence and survival, and the impact of sequential cancer-directed therapy among R/M patients. We integrate genomic data for 36 sequenced ACC patients. RESULTS: Median overall survival (OS) from initial diagnosis was 35.1 ys (95%CI: 25.8-37.3) for R/M ACC patients. 10-y OS among R/M patients was 84.7%, worse for patients with extra-pulmonary metastatic disease (p = 0.02). Only initial disease stage predicted recurrence (OR 1.69, p = 0.03). Longer time to first R/M treatment predicted improved survival (p < 0.01); those treated ≤ 3 years from their R/M diagnosis had poor outcomes (p = 0.01). R/M patients who received systemic therapy vs. active surveillance had similar survival (p = 0.35). Molecular findings predicted outcomes: 10-y OS: 100% MYB, 53.3% PI3K, 32.1% NOTCH1 and others, p = 0.03. PI3K mutations predicted a longer disease-free interval (p = 0.04). CONCLUSIONS: Underlying disease biology remains the strongest predictor of outcomes in R/M ACC. Shorter time to R/M therapy predicts poor outcomes. Molecular alterations are prognostic, and PI3K mutations identify an intermediate-risk ACC subgroup.

Resection of Septal Adenoid Cystic Carcinoma and Primary Reconstruction of the Surgical Defect via Open Rhinoplasty.

Adenoid cystic carcinoma is one of the most common minor salivary gland malignancies of the head and neck region. However, adenoid cystic carcinoma of the nasal septum is extremely rare. The authors herein report a case of a septal adenoid cystic carcinoma in a 68-year-old man who complained of nasal bleeding and nasal obstruction for several months. Diagnostic nasal endoscopy revealed a protruding mass arising from the anterosuperior part of the nasal septum. The tumor was removed with a safety margin using the open rhinoplasty approach and primary reconstruction of the surgical defect was performed using septal cartilage. Histopathology indicated an adenoid cystic carcinoma with cribriform pattern. Two years postoperatively, there was no evidence of recurrence, the functional and cosmetic results were good, and the patient was satisfied with the treatment outcome.

Low recurrence of lung adenoid cystic carcinoma with radiotherapy and resection.

BACKGROUND: Adenoid cystic carcinoma is a rare cause of thoracic malignancy, and the prognosis may depend on the extent of surgical resection and adjuvant radiotherapy. Complete resection has low rates of local recurrence but is complicated by the involvement of central airways. Adjuvant radiotherapy is frequently recommended but unproven. METHODS: We describe the technicalities of radical resection and adjuvant radiotherapy using the primary endpoint of local recurrence and secondary endpoints of locoregional (mediastinal) recurrence and distant metastasis. Resections were classed as microscopically and macroscopically clear (R0) or only macroscopically clear (R1). RESULTS: Twelve patients (eight males) diagnosed between 1999 and 2016, with an average age of 44 ± 12 years, were included. Six of these were operable (operative group), and six had non-resectable lesions (radiotherapy group). In the operative group, three had tracheal disease and three had bronchial disease. Tracheal lesions underwent excision with tracheal anastomosis (all R1 resections). Main bronchial lesions underwent complete excision via pneumonectomy (two R0 and one R1 resections). All these patients received 50-60 Gray of adjuvant radiotherapy. At an average follow-up of 6.1 ± 4.3 years, no patient had local recurrence, two had locoregional recurrence and four had distant metastasis. The radiotherapy group received 60-70 Gray as definitive therapy, and at an average follow-up of 5.4 ± 4.2 years, three had locoregional recurrence and four had distant metastasis. CONCLUSION: Our case series consolidates evidence that early radical resection and radiotherapy is associated with a low risk of local recurrence in patients with thoracic adenoid cystic carcinoma.

Phase II Study of Lenvatinib in Patients With Progressive, Recurrent or Metastatic Adenoid Cystic Carcinoma.

PURPOSE: Recurrent or metastatic adenoid cystic carcinoma (R/M ACC) is a malignant neoplasm of predominantly salivary gland origin for which effective therapies are lacking. We conducted a phase II trial evaluating the multitargeted tyrosine kinase inhibitor lenvatinib in patients with R/M ACC. PATIENTS AND METHODS: This study was conducted with a two-stage minimax design. Patients with histologically confirmed R/M ACC of any primary site with radiographic and/or symptomatic progression were eligible. Any prior therapy was allowed except previous lenvatinib. Patients received lenvatinib 24 mg orally per day. The primary end point was overall response rate. Secondary end points were progression-free survival and safety. An exploratory analysis of how MYB expression and genomic alterations relate to outcomes was conducted. RESULTS: Thirty-three patients were enrolled; 32 were evaluable for the primary end point. Five patients (15.6%) had a confirmed partial response, 24 patients (75%) had stable disease, two patients (6.3%) discontinued treatment as a result of toxicity before the first scan, and one patient (3.1%) had progression of disease as best response. Median progression-free survival time was 17.5 months (95% CI, 7.2 months to not reached), although only eight progression events were observed. Patients otherwise were removed for toxicity (n = 5), as a result of withdrawal of consent (n = 9), or at the treating physician's discretion (n = 6). Twenty-three patients required at least one dose modification, and 18 of 32 patients discontinued lenvatinib for drug-related issues. The most common grade 3 or 4 adverse events were hypertension (n = 9; 28.1%) and oral pain (n = 3; 9.4%). Three grade 4 adverse events were observed (myocardial infarction, n = 1; posterior reversible encephalopathy syndrome, n = 1; and intracranial hemorrhage, n = 1). CONCLUSION: This trial met the prespecified overall response rate primary end point, demonstrating antitumor activity with lenvatinib in R/M ACC patients. Toxicity was comparable to previous studies, requiring monitoring and management.

Adenoid cystic carcinoma of the hard palate presenting as ipsilateral sixth nerve palsy.

Adenoid cystic carcinoma (ACC) is an uncommon malignant neoplasm composed of basaloid epithelial and myoepithelial cells. The palate is the most commonly involved intraoral site for ACC. Here, we document the case of an advanced ACC arising from the hard palate that presented with right-sided sixth nerve palsy in a 75-year-old male with no other systemic illnesses. ACC of the head and neck involving the cavernous sinus and presenting as isolated sixth nerve palsy is exceedingly rare. In the absence of vasculopathic or ischemic risk factors, regardless of the age of the patient; neuroimaging should be performed in cases of isolated nontraumatic sixth nerve palsy.

Adenoid Cystic Carcinoma of the Maxillary Sinus with Isolated Trigeminal Anesthesia.

Adenoid cystic carcinoma (ACC) is a rare malignant secretory gland tumor. It is characterized by slow growth, long clinical course, local recurrences, and distant metastases. In the sinonasal tract, it most commonly arises in the maxillary sinus. It often presents at an advanced stage with perineural spread (PNS). Our patient presented with left-sided facial numbness without other symptoms. The numbness was localized to the left cheek, left side of nose, and left upper lip. Magnetic resonance imaging (MRI) of the brain revealed an enhancing lesion involving the left maxillary sinus with orbital invasion and posterior extension into the cavernous sinus. Transnasal endoscopic exploration with tissue removal revealed ACC. 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) scan revealed no evidence of distant metastases. Presentation of sinonasal ACC (SNACC) is variable depending on the involved structures. Characteristic PNS with ACC may cause neuropathic symptoms. This case displays a unique presentation of an advanced ACC of the maxillary sinus manifesting as isolated unilateral trigeminal anesthesia without sinonasal symptoms. The patient also failed to demonstrate any ocular or oculomotor symptoms despite extensive involvement of the orbit and surrounding structures. This case highlights the importance of recognizing ACC due to its association with late symptomatic manifestations. It also reinforces the need for clinical diligence with the workup of new onset neuropathic symptoms in the maxillary distribution of the trigeminal nerve.