Adenoid cystic carcinoma

The adenoid cystic carcinoma is a rare tumor in the region of the head and neck, it's the common malignant tumor of the salivary glands. Its location in the nasal cavity is exceptional. We report in our study a case of adenoid cystic carcinoma of the nasal cavity of a 68-year-old patient who presents nasal symptoms. The CTscan shows a tissue process in the right nasal cavity, the endoscopy showed a process in the right nasal cavity extending to the lower cornet. The histology confirmed the adenoid cystic carcinoma. The surgical treatment consisted on the excision of the entire tumor followed by radiation therapy.

Epidermal growth factor receptor (EGFR) is highly expressed in adenoid cystic carcinoma (ACC). The efficacy and toxicity of cetuximab with concomitant platinum-based chemoradio- or chemotherapy in patients with locally advanced or metastatic ACC, respectively, was evaluated. Eligible patients (9 with locally advanced tumour and 12 with metastases) had positive tumour EGFR expression. The cetuximab loading dose (400 mg m⁻²) was followed by 250 mg m⁻² per week. Locally advanced tumours were irradiated (mean dose 65 Gy) and treated with concomitant cisplatin (75 mg m⁻², intravenously). Patients with metastases received concomitant cisplatin and 5-fluorouracil (4 × 1000 mg m⁻²). For patients with locally advanced disease (median follow-up: 52 months), the median progression-free survival (PFS) was 64 months and the 2-year overall survival (OS) rate was 100%. For patients with metastases (median follow-up: 72 months), the median PFS and OS were 13 and 24 months, respectively. In both gro...

Adeline Salim, and Stephen Gordon, Liverpool School of Tropical Medicine, UK; Tapiwa Bwakura, Department of Medicine, Harare, Zimbabwe; and Narcisius Dzvanga, Department of Medicine, Bulawayo, Zimbabwe. Correspondence to: Dr Stephen Gordon, Liverpool School of T ropical Medicine, Pembroke Place, Liverpool L3 5QA, UK. Email: sbgordon@liverpool.ac.uk

To compare the long-term outcomes in patients with salivary gland adenoid cystic carcinoma (SGACC) treated with post-operative chemoradiotherapy (POCRT) versus post-operative radiotherapy (PORT). We retrospectively reviewed the records of 91 SGACC patients treated with surgery followed by PORT (n = 58) or POCRT (n = 33) between 2000 and 2013. Treatment outcomes between groups were compared using propensity score matching (1:1 nearest neighbor). The median radiation dose was 66 Gy, and patients were followed up for a median of 71 months. Cisplatin-based concurrent regimens were the most commonly used chemotherapy schedules. In the entire study cohort, patients undergoing POCRT showed a trend toward higher locoregional control (LRC) rates than those treated with PORT alone at both 5 and 8 years (97 and 97 % versus 84 and 79 %, respectively; P = .066). Distant metastases were the most common form of treatment failure and occurred in 31 (34 %) patients (PORT, n = 17; POCRT, n = 14). Aft...

Minor salivary glands are found in the sino-nasal cavities, oropharynx, larynx and trachea with the majority being found in the oral cavity. 80% or more of minor salivary gland tumors are malignant and they tend to have a great variation in presentation and histology. We sought out to compare the presentation of various minor salivary gland tumors (MSGT's) in the Indian population as compared to that reported in different races and regions. We report a clinical case series in which 17 patients with biopsy proven minor salivary gland tumors both benign and malignant at various intraoral sites viz the palate, cheek, retromolar trigone and floor of the mouth were operated upon and followed up over a span of 10 years from 2001 to 2011. 15 patients were treated timely with complete removal of the lesions by wide local excision and as confirmed by histopathology; one patient with adenoid cystic carcinoma delayed in seeking treatment and expired within a month and another with salivary...

Wstep. Metodą referencyjną w diagnostyce obrzekow limfatycznych jest limfoscyntygrafia. Oceny czynnościowej ukladu chlonnego dokonuje sie na podstawie sprawności odplywu znacznika z miejsca podania, czasu pojawienia sie radiofarmaceutyku w wezlach chlonnych, stopnia jego wychwytu w regionalnych wezlach chlonnych oraz obecności patologicznych drog odplywu (wsteczny odplyw skorny). W piśmiennictwie podaje sie, ze istnieje mozliwośc wykorzystania w limfoscyntygrafii poliklonalnej immunoglobuliny G (IgG) znakowanej izotopem technetu ( 99m Tc). Material i metody. Badaniem objeto 17 pacjentow z podejrzeniem obrzeku pochodzenia limfatycznego (11 konczyn gornych i 6 konczyn dolnych). Kazdemu pacjentowi podawano podskornie 0,1 ml kompleksu 99m Tc-IgG (o aktywności ok. 50 MBq) w drugą przestrzen miedzypalcową obu rąk lub stop. Akwizycje dynamiczną z uzyciem gamma kamery Nucline AP rozpoczynano w momencie podania znacznika do obu konczyn. Aby przyspieszyc odplyw znacznika z miejsca podania, pa...

Introduction The incidence of salivary gland tumors is influenced by geographical and racial factors resulting in diverse histology.  While salivary gland tumors account for a low proportion of head and neck cancers, most malignant tumors of the salivary gland are located in the parotid gland. The goals of this study are to describe the clinicopathological behavior of malignant parotid tumors and explore oncological outcomes related to survival in our Pakistani tertiary care cancer hospital. Methods We conducted a retrospective analysis of 209 patients diagnosed with malignant parotid tumors from 2004 to 2016. Data such as demographics, age, gender, histology, grade, clinical and pathological stage, surgical treatment types and adjuvant modalities used were analyzed using SPSS software version 20. We used Kaplan Meier curves to analyze survival data. Results The median patient age at diagnosis was 40 years, and the ratio of men to women was 1.2:1. Mucoepidermoid carcinoma was the mo...

Laryngeal adenoid cystic carcinoma (LACC) is a sporadic neoplasm, especially if supraglottic. The COVID-19 pandemic worsened the presenting stage of many cancers and impacted their prognosis negatively. Here, a case of a patient with adenoid cystic carcinoma (ACC) with delayed diagnosis and a rapid deterioration with distant metastasis due to the COVID-19 pandemic is illustrated. Next, we present a literature review of this rare glottic ACC. The COVID-19 pandemic worsened the stage of presentation of many cancers and adversely affected their prognosis. The present case had a rapidly lethal course, undoubtedly due to the diagnosis delay caused by the COVID-19 pandemic, which impacted the prognosis of this rare glottic ACC. Strict follow-up is recommended for any suspicious clinical findings, as an early diagnosis will improve the disease prognosis, and to consider the influence of the COVID-19 pandemic, especially on the timing of common diagnostic and therapeutic procedures for onco...

BackgroundSalivary gland tumours (SGT) are a relatively rare group of neoplasms with a wide range of histopathological appearance and clinical features. To date, most of the epidemiological studies on salivary gland tumours are limited for a variety of reason including being out of date, extrapolated from either a single centre or country studies, or investigating either major or minor glands only.MethodsThis study aimed to mitigate these shortcomings by analysing epidemiological data including demographic, anatomical location and histological diagnoses of SGT from multiple centres across the world. The analysed data included age, gender, location and histological diagnosis from fifteen centres covering the majority of the world health organisation (WHO) geographical regions between 2006 and 2019.ResultsA total of 5798 cases were analysed including 65% benign and 35% malignant tumours. A slight female predilection (54%) and peak incidence between the fourth and seventh decade for bo...

Adenoid cystic carcinoma (AdCC) is among the most common malignant tumors of the salivary glands. It is characterized by a prolonged clinical course, with frequent local recurrences, late onset of metastases and fatal outcome. High-grade transformation (HGT) is an uncommon phenomenon among salivary carcinomas and is associated with increased tumor aggressiveness. In AdCC with high-grade transformation (AdCC-HGT), the clinical course deviates from the natural history of AdCC. It tends to be accelerated, with a high propensity for lymph node metastasis. In order to shed light on this rare event and, in particular, on treatment implications, we undertook this review: searching for all published cases of AdCC-HGT. We conclude that it is mandatory to perform elective neck dissection in patients with AdCC-HGT, due to the high risk of lymph node metastases associated with transformation.

Cylindroma is a benign adnexal tumor, which occurs as solitary dermal nodules on the scalp and forehead. Cylindroma of the breast is a rare lesion. Fine-needle aspiration is often the first line of investigation. In this communication, we illustrate the cytomorphological feature of cylindroma in a 61-year-old female patient who presented with a breast lump. Fine-needle aspiration cytology in this case reveals the islands of tumor cells formed a "jig-saw" pattern, along with the bland, basaloid cells associated with globular, extracellular material. These features are typical of cylindroma, but differential diagnosis of adenoidcystic carcinoma should always be kept in mind as the management of both tumors are different.

Breast cancer is the most common cancer among women in developed countries. The prevalence of the disease is increasing in the world. Its annual incidence among Iranian women is about 7000 cases. RAP1A, a tumor suppressor gene, is located at 1p13.3 and plays an important role in the cellular adhesion pathway and is involved in the pathogenesis of breast cancer. The DOCK4 gene, which is located at 7q31.1, specifically activates RAP1A gene. In the present study, DNA samples from 64 cases of sporadic breast tumors (referred to Mehrad Hospital in Tehran) were screened using PCR-SSCP method and the number of observed variations compared with the control group (100 normal women). Mutation detection for coding exons of RAP1A gene and the 500 bp upstream of transcription initiation site as promoters of both DOCK4 and RAP1A were carried out and compared with the control group. The promoter region of DOCK4 showed a heterozygous mutation with G>A transition at nucleotide -303 in a fibroaden...

Adenoid cystic carcinoma is a malignant tumour rarely found in trachea, and even less so in our sub-region. In addition to a slow clinical course, this histology has a predilection for perineural invasion and a tendency for local and distant recurrence. Most patients present with dyspnea and symptoms often mimic those of asthma or chronic bronchitis. Although surgical resection is the mainstay of treatment, tumour size and location, comorbidities and patient reluctance may prevent surgery. Furthermore, the recognized chemoresistance of this histological type means that radiotherapy, whether definitive or adjuvant, plays a major role in its management. We report the case of a 30-year-old patient treated in our department with definitive chemoradiotherapy.

BackgroundOptimal treatment and prognostic factors affecting long‐term survival in patients with sinonasal adenoid cystic carcinoma (ACC) have yet to be clearly defined.MethodsWe conducted a retrospective review of patients treated with curative intent from 1980‐2015 at MD Anderson Cancer Center.ResultsOne hundred sixty patients met inclusion criteria, including 8 who were treated with radiotherapy alone. Median follow‐up time was 55 months. The 5‐year overall survival (OS) and disease‐free survival (DFS) rates were 67.0% and 49.0%, respectively. The 10‐year OS and DFS rates were 44.8% and 25.4%, respectively. Factors that portended for poor survival on multivariate analysis were recurrent disease, any solid type histology, epicenter in the sinus cavity, the presence of facial symptoms, or the original disease not treated with surgery. There was no association between surgical margin status or nodal status and survival.ConclusionIn this large cohort of patients with sinonasal ACC wi...

External auditory canal, like other epithelialized surfaces is predisposed to malignancies such as Squamous cell carcinomas (SCC), melanomas and adenocarcinomas. In this background, malignancies like adenoid cystic carcinoma (ACC) are a rare occurrence and need to be thoroughly evaluated both locally and for distant extension. The malignancy needs to be addressed with an aggressive approach surgically with adequate marginal clearance. The role of radiation is debatable considering the outcomes in the limited data. Here we present the case of a 35-year-old female who came in with otalgia and otorrhea associated with a mass in the external auditory canal. After biopsy and imaging, a diagnosis of adenoid cystic carcinoma was made and the patient was managed accordingly.