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.
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http://dx.doi.org/10.3390/medicina59061140 | DOI Listing |
J Liq Biopsy
December 2024
Dr. Nasser Ibrahim Al-Rashid Orbital Vision Research Center, Bascom Palmer Eye Institute, Miller School of Medicine, University of Miami, Miami, FL 33136, USA.
Adenoid cystic carcinoma (ACC) is a rare and lethal malignancy that originates in secretory glands of the head and neck. A prominent molecular feature of ACC is the overexpression of the proto-oncogene MYB. ACC has a poor long-term survival due to its high propensity for recurrence and protracted metastasis.
View Article and Find Full Text PDFJ Exp Clin Cancer Res
January 2025
Department of Medicine, Section of Hematology and Oncology, University of Chicago, Chicago, IL, USA.
Background: Adenoid cystic carcinoma (ACC) is a rare glandular malignancy, commonly originating in salivary glands of the head and neck. Given its protracted growth, ACC is usually diagnosed in advanced stage. Treatment of ACC is limited to surgery and/or adjuvant radiotherapy, which often fails to prevent disease recurrence, and no FDA-approved targeted therapies are currently available.
View Article and Find Full Text PDFJ Clin Endocrinol Metab
January 2025
Department of Pediatrics, Ribeirao Preto Medical School - University of Sao Paulo, Ribeirao Preto, Brazil.
Background: Adrenocortical cancer (ACC) is rare and aggressive, with YAP1 overexpression associated with poor outcomes in pediatric patients. In this study, we investigated the mechanisms by which YAP1 drives ACC progression and explored it as a potential target therapy.
Methods: YAP1 expression and methylation in ACC were analyzed from pediatric and adult cohorts.
Clin J Gastroenterol
January 2025
Division of Endoscopy, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi-Cho, Sunto-Gun, Shizuoka, 411-8777, Japan.
Anal canal cancer (ACC) is a rare yet noteworthy malignancy that is strongly associated with high-risk human papillomaviruses (HPVs). This case report highlights the diagnostic utility of endocytoscopy (EC) in distinguishing high-grade squamous intraepithelial lesions (HSILs) from low-grade lesions (LSILs) in a 57-year-old male presenting with hematochezia. Traditional magnifying endoscopy was inconclusive; however, EC provided detailed visualization of cellular and vascular changes, facilitating a diagnosis of HPV-associated HSIL or carcinoma in situ.
View Article and Find Full Text PDFCureus
December 2024
Oncology: Radiation Oncology, Tawam Hospital, Al Ain, ARE.
Background Adenoid cystic carcinoma (ACC) is a rare malignancy characterized by slow progression, local recurrence, and distant metastases. This study aims to evaluate the demographic patterns, clinical presentations, outcomes, and survival trends of patients with ACC. Methods A retrospective analysis of 14 patients diagnosed with ACC from 2010 to 2024 at a tertiary cancer center in the United Arab Emirates was conducted.
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