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Synchronous mucoepidermoid carcinoma of tongue and pleomorphic adenoma of submandibular gland.

Presentation with synchronous salivary gland tumors is rare, with the most typical combination being Warthin's tumor and pleomorphic adenoma of the parotid gland. Involvement of minor salivary glands in such occurrences is extremely uncommon. We report a case of simultaneous mucoepidermoid carcinoma of the tongue and pleomorphic adenoma of the submandibular gland in a 40-year-old woman. The submandibular mass was initially considered to represent regional lymph node tumor metastasis but later was shown to be an intraglandular primary neoplasm.

Adenoma, Pleomorphic↗

Acinar phenotypes in salivary pleomorphic adenoma: unusual differentiation or disordered functional activity?

To explain the occurrence in salivary pleomorphic adenoma of structures with an arrangement and appearance of tumour cells resembling acini, two tumours showing such structures and, for comparison, a tumour showing goblet cells were examined with the use of histochemistry and immunocytochemistry for constituents of the salivary secretory process. One tumour consisted mainly of slightly granular cells with an acinar arrangement, which contained neutral and carboxylated glycoproteins, -SH groups and cytoplasmic epithelial membrane antigen. The second tumour showed a minor component of structures resembling mucous acini, which contained neutral and carboxylated glycoproteins, -SS- groups and fucoglycoconjugates. The goblet cells of the third tumour contained sulphated glycoproteins and were associated with cystic lumina. Acinar phenotypes in salivary pleomorphic adenoma could reflect either an unusual line of differentiation or luminal cells with increased synthesis and/or retention of variably mature glycoproteins different from those of goblet cells. Disordered secretion and externalization of glycoproteins are possible factors influencing phenotypes in this tumour.

Adenoma, Pleomorphic↗

MRI of intranasal pleomorphic adenoma.

A pleomorphic adenoma in the nasal cavity constitutes a rarity. A 48-year-old woman presented with intranasal pleomorphic adenoma revealed by unilateral continuous obstruction and occasional epistaxis. Nasal endoscopy showed a mucosa-covered and opalescent polypoidal mass arising from the left nasal septum. Unlike cases affecting the major salivary gland, magnetic resonance image (MRI) revealed that the mass showed a low signal intensity on T1-weighted image and a heterogeneous, intermediate signal intensity on T2-weighted image. MRI findings can aid in diagnosis and help exclude the possibility of other neoplasms that occur at this site.

Adenoma, Pleomorphic↗

Endoscopic resection of pleomorphic adenoma arising in the nasopharynx.

Pleomorphic adenoma is the commonest benign tumor of the salivary glands. It has rarely been reported arising in the nasopharynx. A pleomorphic adenoma originating from the left lateral wall of the nasopharynx was found in a 61-year-old female who presented with nasal obstruction and intermittent epistaxis. It was successfully removed by combined transnasal and transoral surgery under endoscopic visualization. Endoscope-guided surgery may be helpful for the complete resection of benign nasopharyngeal tumors whilst minimizing injury to the Eustachian tube openings or velopharynx.

Adenoma, Pleomorphic↗

The frequency and distribution of mast cells in pleomorphic adenomas of salivary glands.

AIM: To investigate whether the frequency and distribution of mast cells (MCs) in pleomorphic adenomas (PAs) of major and minor salivary glands justifies the suggestion that there exists an association between MCs and mucoid stromal changes in PAs. METHODS: The material consisted of 22 cases of pleomorphic adenoma (eight arising in major and 14 in minor salivary glands) and a control group represented by five cases of monomorphic adenoma (MA). Representative 3-microm thick, paraffin-embedded sections were stained with H&E and Azur A. Computer-aided image analysis was performed in order to evaluate the relative surface area occupied by epithelial and connective tissue components, as well as the absolute number of MCs. RESULTS: According to our findings, PAs from minor salivary glands contain significantly greater numbers of mast cells compared with tumours from major glands. Additionally, the distribution of MCs within the stromal connective tissue appeared not to be random. CONCLUSION: It is possible that differences in the pattern of connective tissue might influence the actual concentration of MCs and that these differences are responsible for the observed variations between major and minor gland PAs.

Adenoma, Pleomorphic↗

Pleomorphic adenoma of the lacrimal gland in an 18-year-old girl irradiated for bilateral retinoblastoma.

PURPOSE: To report a case of pleomorphic adenoma of the lacrimal gland following irradiation for bilateral retinoblastoma. MATERIALS AND METHODS: Case report. A 4-month-old girl, with bilateral retinoblastoma, underwent enucleation of the right eye, systemic chemotherapy and bilateral external beam irradiation with a lateral field. The right anophthalmic socket (because of infiltration of the optic nerve) and the left eye (for relapse) were irradiated;. 17 years later, she developed a mass in the superotemporal quadrant of the left orbit. The mass was completely excised. RESULTS: Pleomorphic adenoma is rare in children and teenagers; it usually presents as a painless, slow growing mass in healthy adults. In this case, it developed as a second primary tumor after irradiation for retinoblastoma.

Adenoma, Pleomorphic↗

Eighteen-year survival of a patient with malignant pleomorphic xanthoastrocytoma associated with von Recklinghausen neurofibromatosis.

The authors report the first adult case of pleomorphic xanthoastrocytoma associated with neurofibromatosis-1. A 40-year-old man with history of neurofibromatosis-1 underwent total removal of a right temporo-parietal superficial glioma followed by radiation therapy. Eighteen years after surgery, the patient remained well with no tumour recurrence, which prompted review of the surgical material. The diagnosis of pleomorphic xanthoastrocytoma was then made though the tumour exhibited malignant features histologically, with marked necrosis. This extremely good clinical outcome suggest that some astrocytoma tumours in patients with neurofibromatosis might be associated with a favourable prognosis.

Adenoma, Pleomorphic↗

Basal cell (monomorphic) and minimally pleomorphic adenomas of the salivary glands. Distinction from the solid (anaplastic) type of adenoid cystic carcinoma in fine-needle aspiration.

Cytologic features of the cell-stroma interface are useful in distinguishing between monomorphic adenomas of the basal cell type and adenoid cystic carcinoma. In basal cell adenomas, the collagenous stroma interdigitates with adjacent cells, whereas in adenoid cystic carcinoma, the two are separated by a sharp smooth border. Furthermore, the stroma of basal cell adenomas can contain rare spindle cells or capillaries, but the cylinders of adenoid cystic carcinoma are acellular. The authors review their experience with five cases of basal cell adenoma, and three cases that were designated "minimally pleomorphic adenomas." The latter group showed the small blue cell pattern of basal cell adenoma at the time of fine-needle aspiration, and histology revealed only small foci of typical pleomorphic adenoma. With the exception of one cystic case, the cell-stroma interface of basal cell adenoma was observed in all eight cases. These cases are contrasted with three adenoid cystic carcinomas with extensive solid (anaplastic) areas. All showed the small blue cell pattern and cell-stroma interface features of basal cell adenoma. Neither showed the smooth-bordered cylinders of adenoid cystic carcinoma. Two of these three were incorrectly interpreted as benign at the time of fine-needle aspiration. The authors suggest that the stroma aspirated from solid adenoid cystic carcinoma represents desmoplastic tumor stroma that mimics the pattern of basal cell adenoma in smear material. Distinction between basal cell adenoma and the solid type of adenoid cystic carcinoma at the time of fine-needle aspiration remains a very difficult problem.

Adenoma↗

PDGFRA amplification could be a poor prognostic factor of advanced undifferentiated pleomorphic sarcoma in a comprehensive genomic profiling cohort.

BACKGROUND: Undifferentiated pleomorphic sarcoma (UPS) is the most common pleomorphic sarcoma, and its genomic landscape has been analysed, albeit in small numbers. This study aimed to clarify the relationship between gene variants and the prognosis of patients with advanced UPS. METHODS: This retrospective cohort study was conducted to analyse the data of patients with advanced UPS using a registry of the Center for Cancer Genomics and Advanced Therapeutics (C-CAT) database up to Oct 2025 in Japan, analysed using comprehensive genomic profiling assay. RESULTS: A total of 233 patients with advanced UPS were identified in the C-CAT database; 151 men (64.8%), median age: 60.2&#xa0;years. TP53 variant (55%) was the most frequent event and the rate of Platelet-derived growth factor receptor alpha (PDGFRA) and KDR amplification were 9% and 6%, respectively. PDGFRA amplification co-occurred with KDR amplification (P&#xa0;<&#xa0;0.001). Survival from the initiation of chemotherapy was analysed by adjusting for length bias inherent in the database using the Kaplan-Meier estimator, an established method of adjustment. Patients with PDGFRA amplification (11 patients) had a worse prognosis than those without PDGFRA amplification [hazard ratio 2.9, 95% confidence interval 1.2-6.9 (P&#xa0;=&#xa0;0.02)]. TP53 alterations (P&#xa0;=&#xa0;0.24) were not associated with prognosis. In addition, treatment time with pazopanib with PDGFRA amplification [4 patients, 2.3&#xa0;months (1.2-11.2&#xa0;months)] was not different with those without PDGFRA amplification [28 patients, 3.8&#xa0;months (0.9&#xa0;months-not reached)] (P&#xa0;=&#xa0;0.52). CONCLUSIONS: For patients with advanced UPS, PDGFRA amplification was a poor prognostic factor and is not related to the efficacy of pazopanib treatment.

PDGFRA amplification↗

Fast neutron radiation for recurrent pleomorphic adenomas of the parotid gland.

Between 1984 and 1989, six patients with recurrent pleomorphic adenomas of the parotid gland were treated with fast neutron radiotherapy. All of the patients in this series had one or more unfavorable prognostic features, which would predict suboptimal local tumor control rates with the utilization of standard surgical and adjuvant photon/electron radiation therapies. These prognostic features included multiply recurrent disease in three patients, known gross postoperative residual disease in one patient, extensive unresectable disease in one patient, and multiply recurrent disease plus gross postoperative residual disease in one patient. Patients had an average of three surgeries for recurrent adenomas before their neutron irradiation. The median period from the original diagnosis to the time of neutron treatment was 11 years, with a range of 1.3-32 years. With a median follow-up of 52 months, an overall local control rate of 100% was achieved. Only one grade III (European Organization for Research and Treatment of Cancer-Radiation Therapy Oncology Group scale) late complication occurred secondary to the neutron irradiation. Similar to their malignant counterparts, benign salivary gland neoplasms appear to display excellent response and control rates to treatment with fast neutron irradiation. Neutron irradiation of pleomorphic adenomas should be considered an appropriate therapeutic approach in situations, such as recurrent disease and postoperative gross residual disease, where one may be concerned about potential local failures with the use of standard surgical and radiotherapeutic modalities. Neutron radiation also carries a low risk of facial nerve damage, a consideration that may argue for limiting the extent of surgical resection of recurrent disease.

Adenoma, Pleomorphic↗

Histogenesis of benign pleomorphic adenoma (mixed tumor) of the major salivary glands. An ultrastructural and immunohistochemical study.

Twenty-two benign pleomorphic adenomas of the major salivary glands were studied by transmission electron microscopy and immunohistochemical techniques (three cases) in order to characterize the cell types comprising the epithelial and so-called mesenchymal regions of the tumors. Light- and electron-microscopic studies showed the tumors to consist of variable mixtures of neoplastic ductular epithelial cells, rare acinar cells, and metaplastic myoepithelial cells. Many of the loosely organized "stromal cells" contained structures indicative of their myoepithelial origin, e.g., perinuclear tonofilaments, ectoplasmic actin microfilaments, and remnants of basement membrane. Polyclonal antikeratin antisera strongly stained ductular epithelial and myoepithelial cells, squamoid cell nests, and periductular myoepithelial cells, whereas myxoid and chondroid cells were less intensely stained. Monoclonal cytokeratin antibody AE1 stained only the ductular epithelial cells in both the normal glands and tumors. In contrast, S-100 protein, which is present only in scattered acinar cells and myoepithelial cells in the normal parotid gland, was found in the ductular and periductular myoepithelial cells, isolated myxoid cells, and chondroid and cartilagenous cells in the tumors. Actin was found in all the cell types of the tumor but staining was strongest in the ducts. Double immunofluorescence staining for cytokeratin and vimentin revealed coexpression of both types of intermediate filaments in occasional normal acinar and intercalated duct myoepithelial cells, and in some cells in the myxoid and chondroid regions of the tumors. In the tumors, vimentin was present in occasional periductular myoepithelial cells, stellate myxoid cells, and especially in chondroid cells and chondrocytes. Our findings indicate that benign pleomorphic adenomas of the major salivary glands are pure epithelial cell tumors. The histologic complexity of these neoplasms is due to the ability of the neoplastic ductular myoepithelial cell to modulate its morphologic appearance and intermediate filament composition, and to produce large amounts of matrix substances. We further postulate that these tumors arise from neoplastically transformed intercalated ducts.

Actins↗

Differential diagnosis between adenoid cystic carcinoma and pleomorphic adenoma of the minor salivary glands of palate.

Tumors arising from minor salivary glands of the palate may exhibit an overlap of clinical and biologic features that may produce diagnostic and therapeutic dilemmas. Surgical treatment can be very different, depending on the dimensions and malignant or benign nature of the tumors, and therefore should be planned on the basis of an accurate differential diagnosis. A retrospective analysis in 24 patients with pleomorphic adenoma and adenoid cystic carcinoma of minor salivary glands of the palate was performed to investigate the accuracy of fine needle aspiration cytology (FNAC) and biopsy with histology in the preoperative diagnosis. Preoperative diagnoses obtained with FNAC and biopsy were compared with findings of the definitive histopathologic examination performed on the resected mass. Correspondence between the preoperative diagnoses determined by FNACs and the definitive histopathologic results was observed in 22 of 24 cases, whereas a complete equivalence was found with regards to histology. In the analysis, FNAC was associated with 91.6% accuracy and an error rate of 8.4% in the diagnosis of pleomorphic adenoma and adenoid cystic carcinoma of the palate. From the results of the analysis, histologic examination is still the most accurate diagnostic tool in such tumors. FNAC can be considered in tumors of the head and neck regions that are difficult to reach by means of a common biopsy.

Adenoma, Pleomorphic↗

Pleomorphic adenoma with extensive lipometaplasia: report of three cases.

We report a series of three cases of pleomorphic adenoma with extensive lipometaplasia, a recently described subtype of pleomorphic adenoma of salivary gland origin. Two patients were female and one male, ranging in age from 30 to 45 years. Two occurred in the minor salivary glands of the lip and palate, respectively, and one in the parotid. Typical histologic findings are presented. In addition, one case consists of a proliferation of spindle cells with an interesting combination of mature adipose tissue, hyaline cartilage, and bone in the absence of ductal structures. The differential diagnosis, as it pertains to other fat-containing tumors (such as lipoadenoma, spindle cell lipoma, interstitial lipomatosis, and benign mesenchymoma), is discussed. It is likely that the ability of myoepithelial cells to undergo various metaplasias is the cause of the unusual histologic appearances of this tumor.

Adenoma↗

Giant pleomorphic adenoma of soft palate leading to obstruction of the nasopharyngeal port.

Pleomorphic adenoma is the most common tumor of minor salivary glands, but it is rarely located in the soft palate. It is generally a small mass at the time of diagnosis, but if it is neglected it can rarely become larger, leading to obstructive symptoms such as snoring, hypernasal speech, and dysphagia. Surgical removal of the mass is curative. In this paper, we present a case of an unusually large pleomorphic adenoma of the soft palate leading to snoring and hypernasal speech. The defect that resulted from the excision of the mass is covered with a uvular flap.

Adenoma, Pleomorphic↗

Pleomorphic adenoma of the lip.

A case of pleomorphic adenoma in the upper lip is reported. A 61-year-old male had a tumor at the left side of the upper lip for 8 years. The tumor was surgically excised without recurrence. Histopathologically, the tumor was a typical pleomorphic adenoma with keratinization and glandular differentiation of tumor cells and fibroblastic, mucinous, hyalinized, and cartilagenous stroma.

Adenoma, Pleomorphic↗

Ultrastructural analysis of salivary gland pleomorphic adenoma, with particular reference to myoepithelial cells.

Previous ultrastructural studies of pleomorphic adenoma have presented conflicting results with regard to the role of myoepithelial cells in the histogenesis of this tumour. In the present study specimens of ten major salivary gland pleomorphic adenomas were examined ultrastructurally and a number of cell types identified. The material was subjected to quantification using the stereological method of point counting. The results showed a wide spectrum of differentiation within these tumours in which typical myoepithelial cells were rarely encountered even in situations where they are reported to occur in routine histological preparations. Cells with some myoepithelial features were more numerous but duct cells accounted for the majority of tumour cells. The ultrastructural findings correlated well with previously reported immunocytochemical data and further support certain ideas about salivary gland tumour histogenesis.

Adenoma, Pleomorphic↗

Non-invasive (intracapsular) carcinoma ex pleomorphic adenoma: recognition of focal carcinoma by HER-2/neu and MIB1 immunohistochemistry.

AIMS: Non-invasive carcinoma ex pleomorphic adenoma is defined as a carcinoma arising within the boundaries of a pleomorphic adenoma (PA), but which fails to display invasion beyond the capsule of host PA. Alternative names are intracapsular, in situ, or focal carcinoma. The true nature of non-invasive carcinoma ex-PA is still controversial; for example, it is not clear whether it represents early but genuine carcinomatous changes with the genetic make-up of malignant cells, or simply cytological, possibly metaplastic or 'bizarre' changes in PA. Strong overexpression and amplification of HER-2/neu protein has recently been demonstrated in invasive carcinoma ex-PA. In addition, data from breast cancer studies suggest that amplification of HER-2/neu and overexpression of its gene product is mainly involved in the initiation of breast oncogenesis. We sought to establish whether this method could help to demonstrate that what is described as non-invasive carcinoma ex-PA is really a genuine malignancy, albeit in an early phase. METHODS AND RESULTS: Eleven cases of non-invasive carcinoma (in situ) ex-PA were studied for HER-2/neu status using immunohistochemistry and fluorescent in-situ hybridization (FISH). Cells of focal non-invasive carcinoma ex-PA were strongly positive for HER-2/neu protein, while the cells of the maternal PA were always negative. Two cases of low-grade non-invasive myoepithelial carcinoma ex-PA were negative. In four cases out of a total of six tumours studied by FISH, we detected amplification of HER-2/neu gene signals in tumour cells of focal, non-invasive, carcinoma. CONCLUSIONS: The current data suggest that non-invasive carcinoma ex PA is a genuine carcinoma within a PA. However, the presence of cyto-nuclear atypia is not sufficient to make a definite diagnosis of malignant change, which requires a combination of morphology and immunohistochemistry.

Adenoma, Pleomorphic↗