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The gastrointestinal system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Sharon J. White, Francis A. Carey
By far the most frequent salivary gland neoplasm is the pleomorphic adenoma. This shows mixed patterns of differentiation with combinations of epithelial and myoepithelial cells set in a characteristic stroma (Figure 10.15). The tumours are variably encapsulated and, if their excision is incomplete, particularly in the parotid, residual tumour can give rise to multiple recurrences, which are very difficult to treat. A further complication is the possibility of malignant transformation (carcinoma ex pleomorphic adenoma) in longstanding pleomorphic adenoma.
A rare case of mucoepidermoid carcinoma ex pleomorphic adenoma of the lacrimal gland
Published in Orbit, 2023
Nicole J. Topilow, Shanlee M. Stevens, Ying Chen, Umangi Patel, Sander R. Dubovy, Thomas E. Johnson
Carcinoma ex pleomorphic adenoma (CEPA or malignant mixed tumor) is a rare carcinoma that arises from a pre-existing pleomorphic adenoma. CEPAs represent less than 1% of all orbital lesions.6 CEPAs can be classified as non-invasive, minimally invasive (<1.5 mm), or invasive (>1.5 mm) based on the extension of the tumor beyond the capsule. CEPAs can have several histologic subtypes, including adenocarcinoma, adenoid cystic carcinoma, apocrine carcinoma, myoepithelial carcinoma, and mucoepidermoid carcinoma. CEPA with mucoepidermoid carcinoma histologic subtype is exceedingly rare with only one prior case described clinically7 and six other cases mentioned in the literature.1,8,9 In addition to the common signs and symptoms associated with lacrimal gland lesions, CEPA can be associated with pain related to perineural invasion as well as cranial neuropathies due to orbital and intracranial extension.10 Management of lacrimal CEPA is widely debated due to its extremely low incidence and may include globe-sparing resection or orbital exenteration with or without adjuvant radiation and chemotherapy.10 We report a rare case of mucoepidermoid CEPA of the lacrimal gland including its clinical presentation, radiographic and histopathologic findings, management, and outcome. Collection and evaluation of protected patient health information were HIPAA compliant.
Lacrimal gland pleomorphic adenoma with extensive necrosis
Published in Orbit, 2022
Micheal A. O’Rourke, Penelope A. McKelvie, Christopher M. Angel, Alan A. McNab
PLAG1-CTNNB1 fusion was first identified as the key event in the pathogenesis of pleomorphic adenoma by Kas et al. in 1997.17 This finding has been confirmed by multiple groups as the most common molecular event in pleomorphic adenoma and carcinoma ex pleomorphic adenoma. However, detection of PLAG1 translocation requires techniques such as FISH and RT-PCR which may not be available routinely in pathology laboratories. Fusion involving PLAG1 gene causes an overexpression and PLAG1 protein, which can be demonstrated by immunohistochemistry (IHC).18 A recent study by Katabi et al. demonstrated high sensitivity of 96% of PLAG1 IHC for pleomorphic adenoma, but a lower specificity in a cohort of other salivary gland tumours, since it has been detected in myoepithelioma, myoepithelial/epithelial carcinoma, and basal cell adenocarcinomas. In our case, the finding of myxoid stroma and positive PLAG1 immunohistochemistry confirmed the diagnosis of pleomorphic adenoma. Examining specific molecular abnormalities in lacrimal gland neoplasia shows some promise for future treatments but has not identified unique identifying markers for specific tumour types.
An evidence-based analysis of the management of N0 neck in patients with cancer of the parotid gland
Published in Expert Review of Anticancer Therapy, 2019
Jose Guilherme Vartanian, Joao Gonçalves Filho, Luiz Paulo Kowalski, Jatin P. Shah, Carlos Suárez, Alessandra Rinaldo, Remco De Bree, Juan P Rodrigo, Marc Hamoir, Robert P. Takes, Antti A. Mäkitie, Peter Zbären, Simon Andreasen, Vincent Vander Poorten, Alvaro Sanabria, Henrik Hellquist, K. Thomas Robbins, Carsten C. Bödeker, Carl Silver, Alfio Ferlito
Histology of major salivary gland carcinomas plays a major role. Some histological types have shown a greater potential to develop lymph node metastasis than others. The risk of occult metastasis is greater than 50% in undifferentiated carcinomas, squamous cell carcinomas, salivary duct carcinomas, adenocarcinomas not otherwise specified (NOS), and high-grade mucoepidermoid carcinomas. On the other hand, in acinic cell carcinoma, secretory carcinoma, adenoid cystic carcinomas and low-grade mucoepidermoid carcinomas the risk is quite low (2–4%) [1,3,4,8,21,29]. However, in cases of high-grade transformation, mainly reported in adenoid cystic carcinoma, acinic cell carcinoma and secretory carcinoma, the biological behavior is associated with increased tumor aggressiveness, and it tends to carry a higher risk for lymph node metastasis [17,30–33]. In carcinomas, ex pleomorphic adenoma lymph node involvement has been reported to be as high as 52% [21]. It is important to note that carcinoma ex pleomorphic adenoma has usually an aggressive behavior, but the malignant component may also represent a low-grade neoplasm like myoepithelial carcinoma with low neck metastatic potential. However, in many cases the malignant component is difficult to classify and usually is represented by a high-grade component [34].