Noninvasive carcinoma ex pleomorphic adenoma of the parotid gland: A difficult diagnosis on fine needle aspiration.
ABSTRACT: Carcinoma ex pleomorphic adenoma (CXPA) is a rare epithelial malignancy that arises from a primary or recurrent pleomorphic adenoma (PA). It may be noninvasive (NI) or invasive. NI CXPA is extremely rare. Preoperative diagnosis on fine needle aspiration (FNA) of CXPA may be difficult and poses a diagnostic challenge to clinicians and pathologists. Herein, we describe the FNA findings of a case of NI-CXPA. A 69-year-old woman presented with rapid enlargement of a stable parotid mass of 25 years. Cytologically, malignant cells were focally associated with metachromatic fibromyxoid matrix that was homogeneous and dense with a vague fibrillary quality. There were cell groups, papillary-like clusters and single malignant cells. The nuclei were pleomorphic with irregularly dispersed chromatin, and the cytoplasm was ill-defined and granular. Nucleoli were small to inconspicuous. Mitoses and necrosis were not seen. Cytological features were not specific for any type of salivary gland carcinoma. The FNA diagnosis was primary high-grade adenocarcinoma of the parotid gland, not otherwise specified. Facial nerve-sparing total parotidectomy was performed, which histologically showed PA interspersed with ducts and nests composed of pleomorphic atypical nuclei surrounded by extensive hyalinization. Single cells were also noted. No capsular infiltration was seen in the entirely sampled tumor. Immunohistochemistry for Ki-67 showed a higher proliferation rate in the malignant ducts and p63 positive cells focally surrounded some of the malignant ducts. Histological diagnosis was NI-CXPA. Accurate diagnosis is important for proper surgical management; however, the preoperative diagnosis of NI-CXPA is difficult to make on FNA.
Project description:<h4>Introduction</h4>Pleomorphic adenoma is the most common benign neoplasm of the salivary glands. Extensive lipomatous involvement of the tumor is, however, a very rare finding.<h4>Case report</h4>Herein, a rare case of lipomatous pleomorphic adenoma arising in the parotid gland of a 14-year-old Japanese woman is presented.<h4>Conclusion</h4>This is the sixth case of lipomatous pleomorphic adenoma in the English literature. Recognition of this rare subtype of pleomorphic adenoma is important for clinical diagnosis and management. On CT scan, it may not be detected possibly due to the extensive fatty component.
Project description:Objective:The epidemiology of the salivary glands tumour is not well documented in the Caribbean countries. Therefore, the aim of this study is to determine the local trend of salivary gland tumours with a review of current diagnostic techniques. Design & Methods:Retrospective data was collected from the electronic database at the Pathology department of the San Fernando Teaching Hospital between the periods January 2005 to June 2015. All patients who underwent primary resection of either the parotid, submandibular or minor salivary glands for diagnosed tumour cytologically or suspected tumour were included in this study. The clinical and histopathological data were then collected and analyzed. Results:A total of 85 surgeries were performed for suspected or diagnosed neoplasia, 54 parotidectomies, 26 submandibular gland and 5 minor salivary gland excisions. The benign neoplastic lesions, pleomorphic adenoma and Warthin's tumour, were the most common 53 (62.4%) of all the resections performed, followed by non-neoplastic lesions 25 (29.4%) such as sialadenitis, cysts or normal glands. Malignant neoplasms made up the minority with only 7 cases whereby mucoepidermoid carcinoma was the most common malignant neoplasm found followed by squamous cell carcinoma. Conclusion:Parotid gland remains the most frequent site of salivary gland tumours (80%), with pleomorphic adenoma being the most common benign tumour. Triple assessment is still required to manage these cases adequately with stress on preoperative tissue diagnosis FNAB vs USS guided core biopsy.
Project description:BACKGROUND: Among the salivary gland carcinomas, carcinoma in pleomorphic adenoma has been regarded as a representative carcinoma type which arises secondarily in the background of a pre-existent benign pleomorphic adenoma. It is still poorly understood how and which benign pleomorphic adenoma cells transform into its malignant form, carcinoma ex pleomorphic adenoma. METHODS: We have established five cell systems from a benign pleomorphic adenoma of the parotid gland of a 61-year-old woman. They were characterized by immunofluorescence, classical cytogenetics, p53 gene mutational analysis, fluorescence in-situ hybridization, and histopathological and immunohistochemical examinations of their xenografts, to demonstrate their potency of secondary transformation. RESULTS: We established and characterized five cell systems (designated as SM-AP1 to SM-AP5) from a benign pleomorphic adenoma of the parotid gland. SM-AP1 to SM-AP3 showed polygonal cell shapes while SM-AP4 and SM-AP5 were spindle-shaped. SM-AP1-3 cells were immunopositive for keratin only, indicating their duct-epithelial or squamous cell differentiation, while SM-AP4/5 cells were positive for both keratin and S-100 protein, indicating their myoepithelial cell differentiation. Chromosome analyses showed numeral abnormalities such as 5n ploidies and various kinds of structural abnormalities, such as deletions, translocations, derivatives and isodicentric chromosomes. Among them, der(9)t(9;13)(p13.3;q12.3) was shared by all five of the cell systems. In addition, they all had a common deletion of the last base G of codon 249 (AGG to AG_) of the p53 gene, which resulted in generation of its nonsense gene product. Transplanted cells in nude mice formed subcutaneous tumors, which had histological features of squamous cell carcinoma with apparent keratinizing tendencies. In addition, they had ductal arrangements or plasmacytoid appearances of tumor cells and myxoid or hyaline stromata, indicating some characteristics of pleomorphic adenoma. CONCLUSION: This study demonstrates in vitro that certain cell types from pleomorphic adenoma are able to clone and survive over a long term and develop subcutaneous tumors in nude mice. The histological features of squamous cell carcinoma from the transplanted cell systems in nude mice might suggest a secondary onset of malignancy from a pre-existing benign adenoma.
Project description:The most common benign salivary tumour is a pleomorphic adenoma. Transformation to malignancy, carcinoma ex pleomorphic adenoma (cxpa), occurs in 6% of cases. Management focuses on surgical resection and radiotherapy; however, rare cases require systemic management. We present the case of a 60-year-old woman with a cxpa of the left parotid gland who required systemic therapy for locally recurrent disease. Treatment options were guided by the literature concerning malignant salivary gland tumour and by whole-genome and transcriptome sequencing of the tumour. The patient received multiple systemic agents during the course of her disease, with cyclophosphamide-doxorubicin-cisplatin providing the best control (partial response). Genomeand transcriptome-directed therapy, including sorafenib and vismodegib, were utilized with limited clinical benefit. Malignant transformation in cxpa is a complex process, and therapy directed at a single tumour pathway might not be sufficient to control disease.
Project description:Pleomorphic adenoma, also known as benign mixed tumor, is the most common tumor affecting the parotid gland and can reach massive size; however, intracranial invasion is rare. Recurrence of pleomorphic adenoma after excision is a well-known phenomenon and can present decades after resection of the primary tumor. Here we present the case of a 53-year-old man who presented to our clinic with ear fullness, otalgia, and hearing loss 30 years after undergoing total parotidectomy and external beam radiotherapy for pleomorphic adenoma. Magnetic resonance imaging revealed a massive transcranial tumor invading the mastoid cavity, the dura of the posterior fossa, the fallopian and semicircular canals, the jugular foramen, the lateral infratemporal fossa skull base, the sigmoid and transverse sinuses, and the superior parapharyngeal region. Gross examination and histopathological studies confirmed that the mass was a recurrent pleomorphic adenoma. Here we discuss the features of recurrent pleomorphic adenoma and review the current literature.
Project description:INTRODUCTION:A vast increase in knowledge of numerous aspects of malignant salivary gland tumours has emerged during the last decade and, for several reasons, this has not been the case in benign epithelial salivary gland tumours. We have performed a literature review to investigate whether an accurate histological diagnosis of the 11 different types of benign epithelial salivary gland tumours is correlated to any differences in their clinical behaviour. METHODS:A search was performed for histological classifications, recurrence rates and risks for malignant transformation, treatment modalities, and prognosis of these tumours. The search was performed primarily through PubMed, Google Scholar, and all versions of WHO classifications since 1972, as well as numerous textbooks on salivary gland tumours/head and neck/pathology/oncology. A large number of archival salivary tumours were also reviewed histologically. RESULTS:Pleomorphic adenomas carry a considerable risk (5-15%) for malignant transformation but, albeit to a much lesser degree, so do basal cell adenomas and Warthin tumours, while the other eight types virtually never develop into malignancy. Pleomorphic adenoma has a rather high risk for recurrence while recurrence occurs only occasionally in sialadenoma papilliferum, oncocytoma, canalicular adenoma, myoepithelioma and the membranous type of basal cell adenoma. Papillomas, lymphadenoma, sebaceous adenoma, cystadenoma, basal cell adenoma (solid, trabecular and tubular subtypes) very rarely, if ever, recur. CONCLUSIONS:A correct histopathological diagnosis of these tumours is necessary due to (1) preventing confusion with malignant salivary gland tumours; (2) only one (pleomorphic adenoma) has a considerable risk for malignant transformation, but all four histological types of basal cell adenoma can occasionally develop into malignancy, as does Warthin tumour; (3) sialadenoma papilliferum, oncocytoma, canalicular adenoma, myoepithelioma and Warthin tumour only occasionally recur; while (4) intraductal and inverted papilloma, lymphadenoma, sebaceous adenoma, cystadenoma, basal cell adenoma (apart from the membranous type) virtually never recur. No biomarker was found to be relevant for predicting recurrence or potential malignant development. Guidelines for appropriate treatment strategies are given.
Project description:The objective of this article is to present the first case reported in the literature of metachronous pleomorphic adenoma of bilateral parotid glands and submaxillary gland. The authors report the case of a 27-year-old female with metachronous mixed tumors in her right parotid and submandibular glands. The patient has no history of previous radiotherapy. All three lesions were diagnosed by fine-needle aspiration. The histopathologic evaluation of all three major salivary gland masses demonstrated pleomorphic adenomas, with no occult malignancy observed on serial sections. The presentation of pleomorphic adenomas in the parotids and submandibular glands probably represents three unrelated primary sites of tumor, yet the possibility of metastasis from one gland to the other cannot be excluded.
Project description:Previous studies have shown that extracapsular dissection (ECD) is an alternative approach to superficial parotidectomy (SP) for pleomorphic adenoma parotid tumours, associated with low recurrence rates equal to those following SP, but with significantly reduced morbidity. However, if a malignant tumour masquerades as a clinically benign lump, this approach may be inappropriate. This study addressed this question by analysing the outcome of 821 consecutive patients with parotid tumours treated at one centre over 40 years and with a median 12 (range 5-30) years follow-up. Tumours were classified as 'simple' (discrete, mobile, < 4 cm: n=662) and 'complex' (deep, fixed, facial nerve palsy, > or =4 cm: n=159). Among the 'simple' or clinically benign tumours, 503 patients underwent ECD; 159 patients underwent SP. In all, 32 (5%) clinically benign cases were subsequently revealed as malignant histologies (ECD, 12; SP, 20). For each group, 5- and 10-year cancer-specific survival rates were 100 and 98%, respectively. There were no differences in recurrence rates when subanalysed by surgical groups, but ECD was associated with significantly reduced morbidity (P < 0.001). This study demonstrates that ECD is a viable alternative to superficial parotidectomy for the majority of parotid tumours, associated with reduced morbidity without oncological compromise.
Project description:Thyroid neoplasia is common and requires appropriate clinical workup with imaging and fine-needle aspiration (FNA) biopsy to evaluate for cancer. Yet, up to 20% of thyroid nodule FNA biopsies will be indeterminate in diagnosis based on cytological evaluation. Genomic approaches to characterize the malignant potential of nodules showed initial promise but have provided only modest improvement in diagnosis. Here, we describe a method using metabolic analysis by desorption electrospray ionization mass spectrometry (DESI-MS) imaging for direct analysis and diagnosis of follicular cell-derived neoplasia tissues and FNA biopsies. DESI-MS was used to analyze 178 tissue samples to determine the molecular signatures of normal, benign follicular adenoma (FTA), and malignant follicular carcinoma (FTC) and papillary carcinoma (PTC) thyroid tissues. Statistical classifiers, including benign thyroid versus PTC and benign thyroid versus FTC, were built and validated with 114,125 mass spectra, with accuracy assessed in correlation with clinical pathology. Clinical FNA smears were prospectively collected and analyzed using DESI-MS imaging, and the performance of the statistical classifiers was tested with 69 prospectively collected clinical FNA smears. High performance was achieved for both models when predicting on the FNA test set, which included 24 nodules with indeterminate preoperative cytology, with accuracies of 93% and 89%. Our results strongly suggest that DESI-MS imaging is a valuable technology for identification of malignant potential of thyroid nodules.
Project description:The most common benign salivary gland tumor is pleomorphic adenoma. They can attain giant proportions and weigh several kilograms. They can cause facial disfigurement and, if untreated, could lead malignant transformation. This image depicts a 7.5 kg pleomorphic adenoma of right submandibular gland with malignant change in a 55-year-old male.