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      A Rare Nasopharyngeal Adenoid Cystic Carcinoma: Case Report and Literature Review

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          Abstract

          Patient: Male, 56-year-old

          Final Diagnosis: Adenoid cystic carcinoma

          Symptoms: Head and neck mass • headache • nasal congestion • nasal mass

          Clinical Procedure: —

          Specialty: Oncology • Otolaryngology • Palliative medicine

          Objective:

          Unusual clinical course

          Background:

          Adenoid cystic carcinoma (ACC) is a malignant tumor that involves the salivary glands and is rarely seen in the nasopharynx. It is characterized by pain, a high rate of recurrence, and poor survival rate and poor prognosis as it tends to invade the perineurium. Due to the ambiguity of patient presentations, most patients present at a late stage, at which point metastasis has already occurred.

          Case Report:

          The patient in this case report was an otherwise healthy 56-year-old male smoker who presented to the clinic in March 2022 complaining of nasal obstruction, mouth breathing, recurrent epistaxis, headache, and facial pain. He reported significant weight loss in the past 6 months. The nasal scope showed nasopharyngeal fullness and biopsy-confirmed ACC exhibiting tubular, solid, and focal cribriform growth patterns. Further work-up showed an advanced form of nasopharyngeal ACC with intracranial extension and lung metastasis. The patient received concurrent radiotherapy and chemotherapy. After 6 months, the patient reported an improvement in the pain, and repeated images showed regression of the tumor size.

          Conclusions:

          ACC is a malignant disease that is rarely seen in the nasopharynx. Currently, the most commonly agreed upon management for resectable ACCs in the literature is through surgical resection with adjuvant radiotherapy. However, most of the reported cases received radiation and chemotherapy due to the difficulty and risk of radical surgical excision of nasopharyngeal malignant tumors. Patients should be followed up to rule out any local recurrence or distant metastasis.

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          Most cited references41

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          Histologic grading of adenoid cystic carcinoma of the salivary glands.

          Seventy-nine patients with adenoid cystic carcinoma arising in salivary glands were studied to determine whether a correlation existed between the morphologic features of the tumor and the prognosis. Three histologic grades were established: Grade I, tumors with tubular and cribriform areas but without solid components; Grade II, cribriform tumors that were either pure or mixed with less than 30% of solid areas; and Grade III, tumors with a predominantly solid pattern. Cumulative survival rates at 15 years were 39%, 26%, and 5%, for Grades I, II, and III, respectively. Grade III tumors were larger, recurred frequently, and killed the patients within 4 years. Grade I lesions were smaller, were amenable to complete surgical excision, and had a protracted clinical course. Grade II tumors lay between the other two forms both clinically and pathologically. Other important prognostic features of the adenoid cystic carcinoma were its primary site, its presence or absence at surgical margins, and the anatomic structures it involved.
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            Recurrent fusion of MYB and NFIB transcription factor genes in carcinomas of the breast and head and neck.

            The transcription factor gene MYB was identified recently as an oncogene that is rearranged/duplicated in some human leukemias. Here we describe a new mechanism of activation of MYB in human cancer involving gene fusion. We show that the t(6;9)(q22-23;p23-24) translocation in adenoid cystic carcinomas (ACC) of the breast and head and neck consistently results in fusions encoding chimeric transcripts predominantly consisting of MYB exon 14 linked to the last coding exon(s) of NFIB. The minimal common part of MYB deleted as the result of fusion was exon 15 including the 3'-UTR, which contains several highly conserved target sites for miR-15a/16 and miR-150 microRNAs. These microRNAs recently were shown to regulate MYB expression negatively. We suggest that deletion of these target sites may disrupt repression of MYB leading to overexpression of MYB-NFIB transcripts and protein and to activation of critical MYB targets, including genes associated with apoptosis, cell cycle control, cell growth/angiogenesis, and cell adhesion. Forced overexpression of miR-15a/16 and miR-150 in primary fusion-positive ACC cells did not significantly alter the expression of MYB as compared with leukemic cells with MYB activation/duplication. Our data indicate that the MYB-NFIB fusion is a hallmark of ACC and that deregulation of the expression of MYB and its target genes is a key oncogenic event in the pathogenesis of ACC. Our findings also suggest that the gain-of-function activity resulting from the MYB-NFIB fusion is a candidate therapeutic target.
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              The influence of positive margins and nerve invasion in adenoid cystic carcinoma of the head and neck treated with surgery and radiation.

              Surgery is the primary treatment for adenoid cystic carcinomas arising from major and minor salivary glands of the head and neck. However, local recurrence is frequent because of the infiltrative growth pattern and perineural spread associated with these tumors. At UTMDACC, we have had a longstanding policy of using postoperative radiotherapy to reduce the risk of local recurrence and to avoid the need for radical surgery; this 30-year retrospective study analyzes the results of this combined modality approach. Between 1962 and 1991, 198 patients ages 13-82 years, with adenoid cystic carcinomas of the head and neck, received postoperative radiotherapy for known or suspected microscopic residual disease following surgery. Distribution of primary sites was: parotid: 30 patients; submandibular/sublingual: 41 patients; lacrimal: 5 patients; and minor salivary glands: 122 patients. Eighty-three patients (42%) had microscopic positive margins and an additional 55 (28%) had close ( or = 56 Gy, respectively (p = 0.006). Actuarial 5-, 10-, and 15-year freedom from relapse rates were 68%, 52%, and 45%, respectively. Base of skull and neck failures were uncommon with or without elective treatment, developing in 2 and 3% of patients, respectively. Distant metastases were the most common type of disease recurrence, developing in 74 patients (37%) of whom 62 (31%) were disease-free at the primary site. Excellent local control rates were obtained in this population using surgery and postoperative radiotherapy and we recommend this combined approach for most patients with adenoid cystic carcinomas of the head and neck. Perineural invasion was an adverse prognostic factor only when a major (named) nerve was involved. Microscopic positive margins was also an adverse prognostic factor, but even when present, local control was achieved in over 80% of our patients. We recommend a dose of 60 Gy to the tumor bed, supplemented to 66 Gy for patients with positive margins. Despite effective local therapy, one-third of patients fail systemically, and good treatment to address this problem is lacking.
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                Author and article information

                Journal
                Am J Case Rep
                Am J Case Rep
                amjcaserep
                The American Journal of Case Reports
                International Scientific Literature, Inc.
                1941-5923
                2023
                01 April 2023
                : 24
                : e938858-1-e938858-6
                Affiliations
                [1 ]College of Medicine, King Saud University, Riyadh, Saudi Arabia
                [2 ]Department of Otolaryngology – Head & Neck Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia
                [3 ]Department of Surgery, Unit of Otolaryngology – Head & Neck Surgery, Security Forces Hospital Program, Riyadh, Saudi Arabia
                Author notes
                Corresponding Author: Ahmed Alhussien, e-mail: AlhussienAh@ 123456gmail.com

                Authors’ Contribution:

                [A]

                Study Design

                [B]

                Data Collection

                [C]

                Statistical Analysis

                [D]

                Data Interpretation

                [E]

                Manuscript Preparation

                [F]

                Literature Search

                [G]

                Funds Collection

                Financial support: None declared

                Conflict of interest: None declared

                Author information
                https://orcid.org/0000-0003-3281-8467
                https://orcid.org/0000-0003-4754-0293
                Article
                938858
                10.12659/AJCR.938858
                10075003
                37002593
                5dd006a1-ce34-488f-89c5-f8c3c8511d65
                © Am J Case Rep, 2023

                This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International ( CC BY-NC-ND 4.0)

                History
                : 04 November 2022
                : 16 February 2023
                : 24 February 2023
                Categories
                Articles

                carcinoma, adenoid cystic,nasopharyngeal neoplasms,salivary gland neoplasms,salivary glands, minor

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