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PubMed1997

Benign pleomorphic adenoma of the larynx: report of a case and review and analysis of 20 additional cases in the literature.

Among the benign pleomorphic adenomas of the minor salivary glands, those that develop in the larynx are very rare. One such case of benign pleomorphic adenoma of the larynx in an adult female is described. The patient was treated with epiglottectomy with a lateral pharyngotomy approach. Review of the literature showed an additional 20 well-described cases of benign pleomorphic adenoma of the larynx. These patients usually present with hoarseness, dyspnea or dysphagia, or any combination of these depending on the site of origin and the size of the tumor. Occasionally the tumor may be discovered incidentally. The tumor can occur in any part of the larynx with the epiglottis being the most common site. Clinically they are indistinguishable from other benign laryngeal neoplasms, as surface mucosal lining remains intact. Histopathological examination is used to make the definitive diagnosis, but rarely, misinterpretation may occur. Conservative surgery provides a lasting cure.

Adenoma, Pleomorphic↗

PubMedDate not supplied

[Phenotypic characterization of cells and structure of the extracellular matrix in pleomorphic adenoma of the salivary glands].

Phenotypic characteristics of cells and extracellular matrix (ECM) of 14 salivary gland pleomorphic adenomas were studied by indirect immunofluorescence. All cells in the epithelial structures expressed cytokeratin 8, the majority of cells produced vimentin, laminin and IV type collagen. Cells of myxoid areas expressed vimentin: type IV collagen was observed in the form of fibrillar structures. Fibrous areas were characterized by vimentin and cytokeratin 8 expression; types I, III, IV collagens and fibronectin with ED-A sequence were present in ECM. Terms "epithelial structures", "epithelial" and "myoepithelial" cells in relation to pleomorphic adenoma may be used only relatively as it is mainly represented by cells with features of both epithelial and mesenchymal phenotype. Characteristics of ECM synthesized in various structures of pleomorphic adenoma may serve an additional criterion of cell transformation.

Adenoma, Pleomorphic↗

PubMed1997

[Pleomorphic adenoma as a parapharyngeal tumor. The role of the CT in differential diagnosis].

We described 2 cases of pleomorphic adenoma as parapharyngeal masses, one of the deep lobule of the parotid gland and the other one in palatine accessory salivary glands. After computed tomography (CAT) we made the anatomopathological diagnosis of pleomorphic adenoma with fine needle aspiration. We discuss about the role of CAT in diagnosis of pleomorphic adenoma occupying the parapharyngeal space.

Adenoma, Pleomorphic↗

PubMedDate not supplied

Pleomorphic adenoma of the trachea: a case report.

Pleomorphic adenoma is the most common form of major salivary gland tumors,but it is exceedingly rare in the trachea. We present a case of pleomorphic adenoma of the trachea in an 80-years-old man. Chest three-dimensional CT demonstrated a bosselated mass with a lobulated surface arising from the membranous portion of the upper trachea. Transbronchoscopic biopsy from the mass showed a biphasic histologic appearance produced by a mixture of glandular structure and hyaline-like stroma. The pathological diagnosis was pleomorphic adenoma.

Adenoma, Pleomorphic↗

PubMed1998

[A case of very late malignant degeneration of pleomorphic adenoma].

Incomplete excision of a pleomorphic adenoma exposes to a high risk of recurrence and tumor spread, making secondary surgery more difficult or a malignant transformation with a poor vital prognosis likely. Three histological types of pleomorphic adenomas can be observed when the tumor undergoes a transformation, namely the carcinoma ex-pleomorphic adenoma, the true malignant mixed tumor and the benign metastasizing mixed tumour.

Adenocarcinoma↗

PubMed1987

Malignant transformation of presumed pleomorphic adenoma of lacrimal gland after 60 years.

A woman was diagnosed as having a benign lacrimal gland mass at age 23 years and no treatment was advised. About 60 years later, the previously stationary proptosis began to increase, and biopsy specimens demonstrated a malignant mixed tumor (pleomorphic adenocarcinoma) of the lacrimal gland. The patient was subsequently treated with orbital exenteration. From the clinical history and histopathologic findings, it appears that the patient harbored a long-standing pleomorphic adenoma (benign mixed tumor) that gave rise to a pleomorphic adenocarcinoma after remaining stationary for 60 years.

Adenoma↗

PubMed1977

Pleomorphic adenoma of the epiglottis: report of a case.

We describe the clinical and pathologic aspects of an unusual case of pleomorphic adenoma of the epiglottis. A 69-year-old man had impaired speech and a "lumpy sensation" in the throat. Following clinical evaluation and a diagnostic biopsy, the tumor was totally excised with excellent results. Pleomorphic adenoma of the larynx is most uncommon. To our knowledge, no report describing the clinical and pathologic features of this entity in the epiglottis or larynx has been previously reported. This is the only example of an epiglottic pleomorphic adenoma among 391 cases seen at Presbyterian-University Hospital and the Eye and Ear Hospital of Pittsburgh during a 21-year period.

Adenoma↗

PubMed1981

Pleomorphic lipoma: a benign tumor simulating liposarcoma. A clinicopathologic analysis of 48 cases.

A clinicopathologic study of 48 cases of pleomorphic lipoma from the files of the Armed Forces Institute of Pathology reveals that this tumor occurs principally in males (83%) in the fifth to seventh decades (mean 57 years) and shows a predilection for the posterior neck, shoulder, and back. Typically, the lesion appears as a painless, circumscribed subcutaneous mass that, on gross examination, resembles an ordinary lipoma. However, microscopically, in contrast to the uniform appearance of the mature adipose tissue cells of an ordinary lipoma, this neoplasm is characterized by an intimate admixture of variably-sized fat cells, and bizarre, pleomorphic, multinucleated giant cells. Many of the latter cells show a distinctive floret-like arrangement of the nuclei and are associated with interlacing bundles of dense birefringent collagen. Despite this pleomorphic picture, which not infrequently leads to a misdiagnosis of liposarcoma, follow-up data obtained for 34 patients (median follow-up period of three years) establish the invariably benign clinical behavior of this unusual tumor.

Adult↗

PubMed1992

Pleomorphic granular cell astrocytoma of the pineal gland.

BACKGROUND: Primary neoplasms of the pineal gland are uncommon. Two patients with unusual primary pineal tumors that had similar distinctive histologic features are reported. METHODS: The surgically resected neoplastic pineal tissue from these patients were examined by light microscopy, immunohistochemistry, and electron microscopy and correlated with the patients' clinical course. RESULTS: These pineal tumors consisted of a mixture of spindle-shaped cells with fibrillated cell processes and many large lipidized and/or granular pleomorphic cells, some of which were multinucleated. These two tumors superficially resembled pleomorphic xanthoastrocytoma and granular cell tumors of the central nervous system. The pleomorphic tumor cells expressed glial fibrillary acidic protein and some also produced retinal S-antigen, a marker for retinal photoreceptor cells. Long-term follow-up (8 years) on one of these patients suggested a relatively "benign" clinical course. CONCLUSIONS: It is possible that this newly described tumor may be a distinct subset of pineal gland neoplasias with a favorable biologic behavior despite the histologic features that would suggest otherwise.

Adult↗

PubMed1994

Primary intracranial pleomorphic angioleiomyoma--a new morphologic variant. An immunohistochemical and electron microscopic study.

BACKGROUND: Angioleiomyomas usually are benign subcutaneous neoplasms that occur most often in extremities of middle-aged individuals. Very few cases have been described in other locations; none along the neuroaxis. An intracranial example of angioleiomyoma displaying unusual morphologic features not seen in the typical peripheral variants of this tumor is described. METHODS: The tumor was studied with conventional histology, immunohistochemistry with morphometric calculation of proliferation index, immunoelectron microscopy, and DNA flow cytometry. RESULTS: The tumor was composed of large epithelioid and pleomorphic cells filled with intermediate filaments positive for desmin and vimentin. Scattered cells also expressed myosin and muscle-specific actin. Smooth muscle cell differentiation was confirmed by ultrastructural demonstration of subplasmalemmal dense bodies, attachment plaques, and discontinuous basal lamina. The proliferation index with proliferating cell nuclear antigen (PCNA) monoclonal antibody was 75.78%, whereas it was only 4.22% with Ki67 monoclonal antibodies adopted to paraffin material (MIB-1). CONCLUSION: The tumor represents a unique morphologic variant of a pleomorphic angioleiomyoma. Cellular pleomorphism and a strong reaction for PCNA in numerous cells suggested that the lesion was malignant. However, the absence of mitotic figures, a small number of Ki-67-positive cells, a diploic DNA pattern, and a low proliferation index in flow cytometry all supported the concept that this neoplasm represented an unusual histologic variant of benign angiogenic leiomyoma. Encapsulation and demarcation of the surgical specimen and the survival of the patient for more than 4 years without recurrence after resection support this interpretation.

Adult↗

PubMed2003

Myoid differentiation and prognosis in adult pleomorphic sarcomas of the extremity: an analysis of 92 cases.

BACKGROUND: The results of a recent study demonstrated an association between myoid differentiation and an adverse prognosis in adult patients with pleomorphic sarcoma, as determined by 5-year metastasis-free survival rates. METHODS: To confirm the importance of muscle differentiation on prognosis in a well controlled clinical context, 92 samples from patients with pleomorphic sarcoma of the extremity from a single institution were immunostained with 4 monoclonal antibodies believed to be correlated with myoid differentiation: alpha-smooth muscle actin, muscle-specific actin, desmin, and myoglobin. RESULTS: Forty-two cases were positive for at least 1 muscle marker and 50 cases were uniformly negative. Between the two groups, there was no significant difference in tumor size, tumor extent, or patient age found; however, histologic grade was significantly higher (P = 0.038) in the myoid tumors. The 5-year survival differed significantly between patients with myoid tumors (35%) and those without myoid tumors (65%) (P = 0.0054). Myoid differentiation remained an adverse prognostic indicator after adjusting for clinically significant factors (i.e., histologic grade, tumor size, tumor extent, and patient age) (P = 0.01) (hazard ratio, 2.39; 95% confidence interval, 1.24-4.63). Furthermore, there was an inverse relation found between the number of myoid markers present and survival (P = 0.004). CONCLUSIONS: Myoid differentiation was found to be an independent indicator of adverse prognosis in adult patients with pleomorphic spindle cell sarcoma of the extremity.

Actins↗

PubMed2004

Fine-needle aspiration in liposarcoma: cytohistologic correlative study including well-differentiated, myxoid, and pleomorphic variants.

We have reviewed cytopathology and the corresponding histopathology material of 86 liposarcomas (55 patients) seen at Institut Curie. The liposarcomas (LS) were well differentiated in 14 cases (9 pure, 2 dedifferentiated, 3 sclerosing), 64 myxoid, and 8 pleomorphic. Twenty-four tumors were primary, 34 recurrent, and 28 secondary. Smears in LS were composed in different proportions of round, spindle cells, lipoblasts, and myxoid and vascular arborizing structures. Pure well-differentiated LS were frequently composed of lipoblasts, and round or spindle cells were occasionally seen. Dedifferentiated and sclerosing liposarcomas were composed of spindle or round cells, but lipoblasts were also occasionally present. Myxoid or vascular arborizing structures were absent. Myxoid LS (including round and spindle cell LS) frequently showed a myxoid background and less frequently vascular arborizing structures. Tumor cells were round or spindle. Lipoblasts were also seen. Pleomorphic LS were composed of an admixture of all cellular and stromal elements. Well-differentiated LS should be distinguished from hibernoma and spindle cell lipoma, and myxoid LS from myxoma, myxoid chondrosarcoma, chordoma, myxoid leiomyosarcoma, and myxoid malignant fibrous histiocytoma. The demonstration of the specific translocation t(12;16)(q13;p11) of myxoid LS is very helpful to establish the diagnosis. Pleomorphic LS should be differentiated from other high-grade sarcomas, whenever possible.

Adolescent↗

PubMed2005

Fine-needle aspiration cytology of pleomorphic hyalinized angiectatic tumor: A case report.

Pleomorphic hyalinized angiectatic tumor (PHAT) of soft parts is a neoplasm characterized by spindle and pleomorphic cells associated with an angiectatic vasculature. We describe the cytological findings of a fine-needle aspiration biopsy (FNAB) from the right medial knee of a 45-yr-old woman. The aspirate material was entirely submitted in Cytolit solution. The specimen was moderately cellular and was comprised of spindle cells in a background of fibrinous material. The cells varied from small, bland spindle cells with a fine chromatin pattern and inconspicuous nucleoli to larger pleomorphic cells with coarser chromatin and occasional intranuclear inclusions. Most of the cells were arranged singly with sporadic small cluster formation with indistinct cell borders. Rare mononuclear inflammatory cells morphologically compatible with mast cells were identified. The differential diagnosis include solitary fibrous tumor (SFT) and ancient schwannoma, which also shows fibrous-like material and spindle cells that may have intranuclear inclusions.

Biopsy, Fine-Needle↗

PubMedDate not supplied

Differential diagnosis of the pleomorphic aspiration biopsy sample of nonepithelial lesions.

The major problem confronting the cytopathologist in interpretation of the pleomorphic aspiration biopsy sample (ABS) of nonepithelial lesions is to provide a reasonable differential diagnosis, since the presence of cellular pleomorphism alone does not always denote malignancy. One must be fully cognizant of a vast number of pathologic processes to make a correct interpretation. Some indication of the extent of the problem and the potential diagnostic pitfalls are outlined in selection of nonneoplastic processes and benign and malignant tumors of mesenchymal origin presenting as pleomorphic ABSs.

Adipose Tissue↗

PubMed2006

CHCHD7-PLAG1 and TCEA1-PLAG1 gene fusions resulting from cryptic, intrachromosomal 8q rearrangements in pleomorphic salivary gland adenomas.

Pleomorphic salivary gland adenomas are characterized by recurrent chromosome rearrangements of 8q12, leading to activation of the PLAG1 oncogene. Here we demonstrate that CHCHD7-PLAG1 is a novel and recurrent gene fusion generated by a cytogenetically cryptic rearrangement in pleomorphic adenomas. CHCHD7 is a newly identified member of a multifamily of proteins containing a conserved (coiled coil 1)-(helix 1)-(coiled coil 2)-(helix 2) domain. Northern blot analysis revealed that the gene is ubiquitously expressed. Its biological function is unknown and the gene has hitherto not been associated with neoplasia. CHCHD7 and PLAG1 are located head-to-head about 500 bp apart in 8q12. Molecular analyses of 27 tumors revealed CHCHD7-PLAG1 fusions in three tumors, two of which had t(6;8) and t(8;15) translocations as the sole anomalies and one a normal karyotype. FISH analyses of interphase nuclei and nuclear chromatin fibers of a fourth adenoma with a normal karyotype revealed that a second fusion partner gene, TCEA1, located about 2 Mb centromeric to PLAG1, also is fused to PLAG1 as a result of a cryptic 8q rearrangement. The breakpoints in both fusions occur in the 5'-noncoding regions of the genes, leading to activation of PLAG1 by promoter swapping/substitution. Western blot and immunohistochemical analyses demonstrated that the PLAG1 protein was overexpressed in epithelial, myoepithelial, and mesenchymal-like tumor cells in tumors with both fusions. Our findings further emphasize the significance of PLAG1 activation in pleomorphic adenomas and demonstrate that the gene is more frequently activated than previously anticipated.

Adenoma↗

PubMed1991

Malignant peripheral nerve sheath tumour with annulate lamellae mimicking pleomorphic malignant fibrous histiocytoma.

The nature and nosologic status of malignant fibrous histiocytoma (MFH), or at least its most common pleomorphic variant, are a matter of controversy and it is possible that this entity represents the shared morphologic phenotype of a range of other dedifferentiated neoplasms. An unusual case of malignant peripheral nerve sheath tumour which very closely mimicked pleomorphic MFH is presented. Its true nature was only disclosed by ultrastructural examination. A further interesting and previously unreported feature in tumours of this type was the presence of annulate lamellae in many of the tumour cells. This case adds weight to the assertion that the light microscopic features of pleomorphic MFH may not be specific.

Aged↗

PubMed2005

Pleomorphic lobular carcinoma of the breast: role of comprehensive molecular pathology in characterization of an entity.

Immunohistochemical analysis of E-cadherin has changed the way lobular neoplasia is perceived. It has helped to classify difficult cases of carcinoma in situ with indeterminate features and led to the identification of new variants of lobular carcinoma. Pleomorphic lobular carcinoma (PLC) and pleomorphic lobular carcinoma in situ (PLCIS), recently described variants of invasive and in situ classic lobular carcinoma, are reported to be associated with more aggressive clinical behaviour. Although PLC/PLCIS show morphological features of classic lobular neoplasia and lack E-cadherin expression, it is still unclear whether these lesions evolve through the same genetic pathway as lobular carcinomas or are high-grade ductal neoplasms that have lost E-cadherin. Here we have analysed a case of extensive PLCIS and invasive PLC associated with areas of E-cadherin-negative carcinoma in situ with indeterminate features, using immunohistochemistry, chromogenic in situ hybridization, high-resolution comparative genomic hybridization (CGH) and array-based CGH. We observed that all lesions lacked E-cadherin and beta-catenin and showed gain of 1q and loss of 16q, features that are typical of lobular carcinomas but are not seen in high-grade ductal lesions. In addition, amplifications of c-myc and HER2 were detected in the pleomorphic components, which may account for the high-grade features in this case and the reported aggressive clinical behaviour of these lesions. Taken together, these data suggest that at least some PLCs may evolve from the same precursor or through the same genetic pathway as classic lobular carcinomas.

Adult↗

PubMed1987

Epithelial properties of pleomorphic xanthoastrocytomas determined in ultrastructural and immunohistochemical studies.

Three cases of pleomorphic xanthoastrocytoma (PXA), one of which showed anaplastic evolution, are described. In all three the PXA tumors were well circumscribed and could be totally removed. Light-microscopically, pleomorphic tumor cells clustered gregariously and often formed alveolar structures. Electron microscopy revealed various epithelial properties, such as junctions and interdigitations between apposing tumor cells, and prominent basal laminae surrounding tumor nests. The circumscribed growth of PXA, as contrasted with an infiltrative growth of usual astrocytoma, can be attributed to the cellular cohesion based on the epithelial properties of the tumor cells. In the third patient, tumor recurred 6 months postoperatively. Although the recurrent tumor retained the alveolar structures, pleomorphism and various degenerative features of the tumor cells diminished with advance in the proliferative activities.

Adult↗