Carcinoma Ex Pleomorphic Adenoma of the Uvula - Case Report

Introduction: Carcinoma Ex Pleomorphic Adenoma (CEPA) results from the malignant transformation of a benign tumor of the Salivary Glands (SG), the Pleomorphic Adenoma (PA). PA is considered the most common salivary tumor with a 5% risk of malignant transformation and its excision is recommended. CEPA is a rare tumor, corresponding to 3.6% of all salivary tumors and 11.6% of all GS carcinomas. About 18% of CEPAs affect minor SG, with the palate being the most common location. The present work serves to describe a case of a CEPA of the Uvula Minor SG (UMSG). Case Report: We present a case report of a 57-year-old patient, with no relevant medical history, referred to the ENT consultation due to the appearance and progressive growth of a painless uvula lesion. The objective ENT examination showed a 15 mm ulcerative-vegetating lesion with apparent origin on the posterior face of the uvula. The lesion was biopsied and histopathological examination identified the presence of a neoplasm of the minor SG, probably NOS adenocarcinoma. The patient underwent Computed Tomography (CT) scan that showed an irregularity of the uvula, with no signs of invasion of the remaining soft palate, without other significant pharyngo-laryngeal changes. The patient underwent partial pharyngectomy and bilateral selective cervical ganglion dissection, and the histopathology of the surgical specimen confirmed that it was an invasive CEPA, the malignant component of the tumor corresponding to a NOS adenocarcinoma of the UMSG. The patient has been followed up in the ENT consultation, with no signs so far of loco-regional recurrence. Discussion/Conclusion: In the presented case, the patient probably developed an undiagnosed PA that had become malignant over time. Given that it is a poor prognosis neoplasm, it’s essential that the ENT specialists are aware of this disease, in order to facilitate and anticipate the diagnosis and treatment as much as possible.

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Malignant transformation of the salivary gland pleomorphic adenoma in myoepithelial carcinoma – the report of two cases

Polski Przegląd Otorynolaryngologiczny ◽

10.5604/01.3001.0013.0617 ◽

2019 ◽

Vol 8 (1) ◽

pp. 52-55

Author(s):

Paulina Szabelska ◽

Anna Rzepakowska ◽

Benedykt Szczepankiewicz ◽

Elżbieta Niemczyk ◽

Ewa Osuch-Wójcikiewicz ◽

...

Keyword(s):

Salivary Gland ◽

Surgical Treatment ◽

Salivary Glands ◽

Pleomorphic Adenoma ◽

Malignant Transformation ◽

Benign Tumor ◽

Complete Recovery ◽

Surrounding Tissue ◽

Carcinoma Ex Pleomorphic Adenoma ◽

Myoepithelial Carcinoma

A multiform adenoma is the most commonly diagnosed benign tumor of the salivary glands. In the majority of patients, surgical resection of the tumor with the adequate surrounding tissue of salivary gland allows for complete recovery. A small percentage of the cases is a recurring pleomorphic adenoma. Even more rarely the diagnosis of carcinoma ex pleomorphic adenoma is made. The study presents two clinical cases of the malignant transformation of pleomorphic adenoma into the myoepithelial carcinoma. The surgical treatment and additional radiotherapy were performed in both cases.

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Squamous Cell Carcinoma Ex Pleomorphic Adenoma of the Parotid Gland A Case Report with Clinicopathological Review

Journal of Medicine ◽

10.3329/jom.v16i2.25442 ◽

2015 ◽

Vol 16 (2) ◽

pp. 118-121 ◽

Cited By ~ 1

Author(s):

SM Khodeza Nahar Begum ◽

M Alamgir Chowdhury ◽

Afroza Suraya Mojumder ◽

Omid Khan

Keyword(s):

Squamous Cell Carcinoma ◽

Case Report ◽

Parotid Gland ◽

Cell Carcinoma ◽

Pleomorphic Adenoma ◽

Malignant Transformation ◽

Squamous Cell ◽

Social Contact ◽

Carcinoma Ex Pleomorphic Adenoma ◽

Metastatic Focus

Pleomorphic adenoma, though essentially benign can undergo malignant transformation. This is the case report of a 72 year old male with a giant tumor of parotid gland measuring 20.0X18.0X16.5cm & weighing 7 kg. Pleomorphic adenoma can rarely transform into carcinoma ex pleomorphic adenoma over time as it enlarges; moreover, histologically, squamous cell carcinoma (SCC) is exceedingly rare. Patient developed the tumor for a period over 20 years & it caused withdrawal from social contact. The tumor gradually enlarged without any pain or other complication except for rapid growth & pain a year before. Postoperative facial function and local control of tumor was achieved. Despite Of having a cancer with significant extracapsular invasion, our patient did not present any metastatic focus. Besides morphological patterns, authors need to categorize invasive carcinoma ex pleomorphic adenoma according to its potentiality to metastasize. Various parameters which can predict malignant transformation in pleomorphic adenoma is also yet to be explored.J MEDICINE July 2015; 16 (2) : 118-121

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Pleomorphic adenoma of the nasal cavity – a case report

Polski Przegląd Otorynolaryngologiczny ◽

10.5604/01.3001.0014.0945 ◽

2020 ◽

Vol 9 (2) ◽

pp. 1-5

Author(s):

Anna Frączek ◽

Julia Szczygielska ◽

Daniel Majszyk ◽

Kazimierz Niemczyk

Keyword(s):

Case Report ◽

Local Recurrence ◽

Nasal Cavity ◽

Pleomorphic Adenoma ◽

Potential Risk ◽

Benign Tumor ◽

Histopathological Examination ◽

Lateral Nasal Wall ◽

Adenoid Cystic

Introduction: Pleomorphic adenoma is a benign tumor which occurs most commonly in the major salivary glands. It is very rare in the nasal cavity and may be misdiagnosed because of its uncharacteristic clinical and histopathological manifestation. Case report: A case of a 42-year-old patient with pleomorphic adenoma on the lateral nasal wall has been presented. The tumor was accidentally found during FESS and septorhinoplasty. Initially, based on the histopathological examination of the obtained sample, there was a suspicion of adenoid cystic carcinoma. Later, the neoplasm was completely removed by the open septorhinoplasty and histopathological re-examination showed features of pleomorphic adenoma. Long term followup was necessary due to the potential risk of local recurrence.

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Myoepithelial Carcinoma ex Pleomorphic Adenoma: Rare Case Report with Clinicopathologic and Immunohistochemical Features

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqab191.184 ◽

2021 ◽

Vol 156 (Supplement_1) ◽

pp. S86-S87

Author(s):

M Khazaeli ◽

Z Kamaluddin ◽

M Taherian

Keyword(s):

Case Report ◽

Parotid Gland ◽

Pleomorphic Adenoma ◽

Nasal Passage ◽

Carcinoma Ex Pleomorphic Adenoma ◽

Sublingual Gland ◽

Myoepithelial Carcinoma ◽

Minor Salivary Glands ◽

Myoepithelial Differentiation ◽

The Right

Abstract Introduction/Objective Myoepithelial carcinoma is identified by nearly exclusive myoepithelial differentiation and evidence of malignancy. It may arise de novo or in preexisting benign tumors including pleomorphic adenoma and benign myoepithelioma. It occurs most commonly in the parotid gland followed by submandibular glands, minor salivary glands, and occasionally in the sublingual gland. Nasopharyngeal origin has been rarely reported. Methods/Case Report An afebrile 19-year-old female presented to the emergency department with persistent facial pressure, otalgia (right side greater than left side), rhinorrhea, and several episodes of epistaxis. One month before, she has been treated with Amoxicillin for three days with no relief, where Augmentin started for her for five days. About four days prior presentation, she was placed on Prednisone and Cefdinir. Over the next several days, she began having throat pain with difficulty swallowing. Routine blood work revealed a leukocytosis of 14.2 with normal differentiation. CT of the neck showed a large soft tissue mass centered at the right nasopharynx. Flexible nasal endoscopy performed bilaterally to reveal the nasopharynx is entirely obstructed with a lobulated mass filling the nasopharynx. On the right side, it extends into the posterior nasal passage filling the sphenoethmoid recess and the posterior floor of the nasal passage. Outpatient biopsy from nasopharynx mass is also performed. Results (if a Case Study enter NA) Mass biopsy reveals a mucinous and chondromyxoid background with mixed epithelial and myoepithelial differentiation. The is squamous metaplasia of myoepithelial cells and prominent mitotic activity and apoptotic activity. Immunohistochemistry was positive for CK5/6, calponin, BCL2, SMA, BerEp4, Sox10, and a proliferative index up to 40%. Based on this information Myoepithelial Carcinoma ex-pleomorphic adenoma of the nasopharynx is the diagnosis. Conclusion Carcinoma ex pleomorphic adenoma is usually a high-grade malignancy. It occurs most commonly in the parotid gland, followed by submandibular glands, minor salivary glands, and occasionally in the sublingual gland. Our case is one of the few cases of myoepithelial carcinoma arising in nasopharyngeal pleomorphic adenoma.

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Analysis of Amplified Genes in Samples of Pleomorphic Adenoma and Carcinoma Ex Pleomorphic Adenoma by CGH-Array Technique

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqz113.034 ◽

2019 ◽

Vol 152 (Supplement_1) ◽

pp. S51-S51

Author(s):

Erika Egal ◽

Welligton Sabino ◽

João Scarini ◽

Reydson Souza ◽

Albina Altemani ◽

...

Keyword(s):

Pleomorphic Adenoma ◽

Malignant Transformation ◽

Genetic Disorders ◽

Benign Lesion ◽

Carcinoma Ex Pleomorphic Adenoma ◽

Low Grade ◽

Cgh Array ◽

Microsoft Excel ◽

Histopathological Subtype ◽

Common Target

Abstract Introduction Pleomorphic adenoma (PA) is a benign lesion of the salivary glands that can suffer malignant transformation to carcinoma ex adenoma pleomorphic (CXPA). The pathogenesis of CXPA has been attributed to the accumulation of genetic disorders in preexisting PAs. However, there is no confirmation whether there is a common target gene involved in all histopathological subtypes or the decisive factors for malignant transformation in a histopathological subtype are specific. Objectives To further analyze genes found in PA and CXPA using the CGH-array technique. The genes found were analyzed using the InteractiVenn virtual tool (http://www.interactivenn.net/) and grouped into a Microsoft Excel worksheet. Results: Of the 460 genes amplified in the studied samples, 287 (62.4%) were related only to CXPA, whereas 144 (31.3%) were related to residual PA. Twenty-nine (6.3%) of these genes were common between residual PA and CXPA. Regarding the degree of invasion of CXPA, there was an increase in the number of genes amplified as the degree of invasion and aggression increased: 8 genes related to intracapsular CXPA, 65 to minimally invasive CXPA, and 373 to weakly invasive. Moreover, when comparing residual AP and intracapsular CXPA, two genes were common to these groups: ERRB2 and GRB7. As for the histological subtype, the high-grade samples had more amplifications (320 amplified genes) than the low-grade ones (129 genes). Three of these genes were common among residual PAs and CXPA: HMGA2, RPSAP52, and LOC100129940. As for the replicates, MYNC, ERBB2, BRIP1, and HMGA2 were the most repeated amplified genes in the residual PAs. HMGA2, ERRBB2, CDK12, RPSAP52, LOC100129940, and LOC100507250 were the genes with the most replicates in CXPA. Conclusion HMGA2, ERRB2, and RPSAP52 may play a key role in PA carcinogenesis, whereas GRB7, CDK12, MYNC, and BRIP1 appear to act as coadjutants.

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