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.

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.

[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.

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.

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.

[Adenoid cystic carcinoma of the larynx: a case report]. / Nabliudenie adenokistoznogo raka gortani.

Adenoid cystic carcinoma is one of the rare pathological conditions affecting the larynx. It is known to develop from the glandula elements present in this organ. The authors report a clinical observation of adenoid cystic carcinoma in the form of an exophytic tumour of the pale pink colour having a smooth surface and the well-pronounced vascular patterns at the base. The neoplasm is localized in the arytenoid and retroarytenoid cartilage regions. Being of 3.5-4 cm in size, the tumour causes the narrowing of the larynx in its posterior portions and restricts its mobility. Bearing in mind the considerable extension of the neoplastic process, we undertook laryngectomy including the resection of the orolaryngopharynx and the cervical portion of the oesophagus, the subtotal resection of the thyroid gland and the simultaneous reconstruction of the orolaryngopharynx.

Double stenting with silicone and metallic stents for malignant airway stenosis.

For severe malignant airway stenosis, there are several types of commercially available airway stents, and each has its own advantages and disadvantages. We herein describe the safety and efficacy of combination stenting with silicone and metallic stents for patients with extended malignant airway stenosis. Seven patients with malignant airway stenosis were treated via combination stenting with a silicone stent and a metallic stent for extended airway stenosis from the central to peripheral airways. Five patients were diagnosed with advanced esophageal cancer, two of whom had tracheoesophageal fistulas. One patient had adenoid cystic carcinoma, and another had mediastinal tumor. There were no specific complications related to the double stenting. Combination stenting with silicone and metallic stents proved to be a safe option for patients with severe, extended, and complicated malignant airway stenosis.

[Tracheal Resection and Primary Anastomosis for Adenoid Cystic Carcinoma Using an Extracorporeal Membrane Oxygenation].

We report a case of tracheal resection and primary anastomosis for adenoid cystic carcinoma using an extracorporeal membrane oxygenation (ECMO). A 45-year-old female was referred to our hospital because of a tracheal tumor that occupied most of the tracheal lumen. In case of airway obstruction by the tracheal tumor during anesthesia and operation, we decided to use ECMO before induction of general anesthesia. Under secure respiratory control using ECMO, tracheal resection and primary anastomosis was performed. Since histopathological examination revealed microscopically positive results at the surgical margin, postoperative adjuvant radiation therapy( 60 Gy/30 Fr) was conducted. Although a tracheal tumor is a relatively rare neoplasm, careful planning and a treatment strategy are necessary with special emphasis on the location and size of tumor. In this case, ECMO made a substantial contribution to secure respiratory control during surgery.

Primary adenoid cystic carcinoma of the skin metastatic to the lymph nodes: immunohistochemical study of a new case and literature review.

Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare adnexal skin tumor first described in 1975, of which merely 62 cases have so far been studied in detail and reported in the English literature. PCACC is usually regarded as apocrine in origin/differentiation, but its precise histogenesis is still not well known. PCACC has in most cases a rather indolent course but can produce local recurrences and, more rarely, regional (lymph node) and distant (pulmonary) metastases. We report herein a Greek woman with a long-standing PCACC that grew slowly over several years and produced metastasis in the regional lymph nodes, highlighting the potentially aggressive course of this tumor. The primary and metastatic tumors were studied immunohistochemically and proved to express several (sweat gland-related) antigens (such as keratin 7, epithelial membrane antigen, CD10, and CD117) but neither hormonal receptors nor p63 or Gross Cystic disease Fluid Protein 15. The salient clinicopathologic features of this rare cutaneous adnexal tumor are reviewed.

Clinical manifestation and management of primary malignant tumors of the cervical trachea.

To explore clinical manifestation and therapies of primary malignant tumors of the cervical trachea, we retrospectively reviewed 31 patients with primary cervical tracheal malignant tumors diagnosed in the last 15 years by means of clinical manifestation, fiberoptic endoscopy, CT scanning and histopathological examinations. All of them were hospitalized and treated at the Second Xiangya Hospital, Central South University. Of them, 4 underwent emergent tracheotomy under local anesthesia, 9 were inserted with a laryngeal mask airway, 18 underwent tumorectomy under general anesthesia with endotracheal intubation, and of them 11 had tracheotomy during surgery. Of those 31 patients, tracheal malignant tumors in 9 cases were resected via laryngeal and retrograde tracheal incisions under endoscope; the tumors in 13 cases were excised via sleeve trachea resection and end-to-end anastomosis; those in 8 were removed by tracheofissure, and the tumor in 1 case was not excised surgically. Among the 30 resected patients, 20 patients received both radiotherapy and chemotherapy; 6 received radiotherapy only, and 4 did not receive any adjuvant therapies. During follow-up between 2 and 11 years, among 31 patients, there was no recurrence in 24 cases. Among the 7 deceased patients, 1 displayed multiple tracheal chondrosarcoma, 4 displayed adenoid cystic carcinoma, and 2 displayed squamous cell carcinoma. Emergency lower tracheotomy is necessary only when patients with tracheal, malignant tumors are in a critical condition. Sleeve trachea resection is the optimal therapy for tracheal malignant tumors. However, in the treatment of tracheal malignant tumors adjacent to the larynx or the involved trachea is over 6 cm in length, other surgeries shall be performed. Postoperative adjuvant radiotherapy and chemotherapy can achieve the same therapeutic effect as sleeve trachea resection.

[A 75-year-old female patient with pleural effusion and gastric metastases of a poorly differentiated carcinoma]. / 75-jährige Patientin mit Pleuraerguss und gastralen Metastasen eines wenig differenzierten Karzinoms.

A 75-year-old woman was found to have left-sided pleural effusion and endoscopy revealed the rare entity of adenoid cystic carcinoma metastases in the gastric mucosa. Approximately 20% of patients with this carcinoma suffer from distant metastases. For the initial staging detection of adenoid cystic carcinoma metastasis with positron emission tomography (PET) or PET computed tomography (CT) is recommended. The recurrent t(6;9)(q22-23;p23-24) translocation that results in a fusion of the two transcription factor genes MYB and NFIB is detectable in half of the cases. As in our case molecular pathology can confirm the correct diagnosis and identification of the localization of the primary tumor.

Dysphagia due to adenoid cystic carcinoma of the base of the tongue.

OBJECTIVES: Although oropharyngeal neoplasia can often lead to dysphagia, salivary gland tumors rarely grow within the tongue base. We present the case of a 75-year-old man with adenoid cystic carcinoma of the base of the tongue causing profound dysphagia and weight loss, and provide a current literature review and update on the management of these rare tumors. METHODS: We present a case report and a literature review. RESULTS: Physical examination performed at the initial visit revealed a firm right base-of-tongue mass with no palpable lymphadenopathy. Flexible fiberoptic laryngoscopy confirmed a large submucosal mass at the right base of the tongue that obscured the right vallecula. Histopathologic analysis of the operative biopsy specimens revealed the classic features of adenoid cystic carcinoma. Treatment included radical pharyngotomy with wide local excision and primary closure, followed by postoperative radiation treatment. CONCLUSIONS: We demonstrate the clinical examination findings and histopathologic characteristics of this disease, and review the literature for clinical treatment recommendations for this rare cause of dysphagia.

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.

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.

Cervical adenoid basal carcinoma associated with invasive squamous cell carcinoma: a report of rare co-existence and review of literature.

Cervical adenoid basal carcinoma (ABC) rarely can harbor associated malignancies like adenoid cystic carcinoma or squamous cell carcinoma (SCC), which express markedly different prognosis from a pure ABC, making an appropriate biopsy essential to provide a clear diagnosis and therapeutic plan. We report a 64-year-old asymptomatic lady with an abnormal cervical cytology, who underwent a conization to reveal an ABC with overlying microinvasive SCC. Doubtful resection margins led us to perform radical hysterectomy with lymph node dissection. Subsequent pathological examination showed a true invasive SCC co-existing with ABC, with invasion of the parametrium. Unlike the indolent course of many pure ABC patients, the prognosis of 11 previously reported co-existing invasive SCC with ABC patients appears to depend on the SCC component. Our case reiterates the importance of adequate biopsy with careful interpretation to cover the possibility of a co-existent malignancy. Besides, it presents an argument in favor of radical surgery for the primary treatment of suspicious associated malignancy, and supports adjuvant treatment according to the unfavorable extent of the co-existent invasive carcinoma.

[Adenoid cystic carcinoma of the scalp]. / Carcinome adénoïde kystique du cuir chevelu.

BACKGROUND: Few cases of adenoid cystic carcinoma of the scalp have been described in the literature. Herein, we report a new case illustrating the difficulties of diagnosis and treatment of this rare form of tumour. CASE REPORT: A 49-year-old man consulted for a large ulcerated scalp tumour. The biopsy sample exhibited an aspect of cystic adenoid carcinoma. Bone invasion had occurred. Extremely wide surgical excision was carried out, extending down to the dura mater, with covering by means of a frontal flap with left temporal pedicle. Since the edges of the resection were also tumoural, postoperative radiotherapy at 56Gy was given. Twelve months later, the patient was still in remission. DISCUSSION: The standard treatment for adenoid cystic carcinoma is complete surgical excision but this approach may be complicated by difficulties relating to the degree of tumour spread or to the anatomical site. Postoperative radiotherapy appears to improve local control, as illustrated by our case.