Salivary gland tumors in Jordan: a retrospective study of 221 patients.
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AIM: To evaluate the types and clinical outcome of salivary gland tumors in Jordan. METHODS: Hospital records of 221 patients (85 women and 136 men) with salivary gland tumors, diagnosed from January 1988 to December 1997 were reviewed. The patients were analyzed according to sex, age, histopathological type and site of the tumor. Survival curves for patients with malignant tumors were constructed using Kaplan-Meier's method. RESULTS: Of the total 221 salivary gland tumors, 155 (70.2%) were parotid tumors, 42 (19%) minor salivary gland tumors, 23 (10.4%) submandibular gland tumors, and a single (0.4%) sublingual gland tumor. Most of the tumors (151, or 68.4%) were classified as benign and 70 (31.6%) were malignant. Men to women ratio was 1.6:1, and the age of the patients ranged from 2 to 81 years. The overall 5 and 10 year-survival rates for the 70 malignant tumors were 67% and 53%, respectively, for all tumor stages. Mucoepidermoid carcinoma had the best, and squamous cell carcinoma the worst 10-year survival rate. Patients treated with surgery and subsequent radiation therapy had better survival rates than those treated with surgery or radiation therapy alone. CONCLUSION: The principal site for salivary gland tumors in Jordan population was the parotid, and the pleo- morphic adenoma the most common pathological finding. Tumor characteristics and survival data for the Jordanian population are comparable to those from western countries. (+info)
Clinically benign parotid tumours: local dissection as an alternative to superficial parotidectomy in selected cases.
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In a personal series of 162 tumours, 101 were pleomorphic adenomas 28 of which were removed by elective local extra capsular dissection and 73 by a conventional nerve dissection. There were no recurrences in either group after a mean follow-up 10.3 years, range 3-21 years for local dissection and 8.3 years, range 3-22 years for nerve dissection. Frey's syndrome did not occur after local dissection but was present in 25% of patients after a nerve dissection. Of the 162 parotid lumps, 17 proved to be a carcinoma but only one was deemed suitable for a local removal, a low grade muco epidermoid carcinoma of the accessory lobe and no recurrence has occurred after 8 years. In benign disease, local dissection gives similar results to conventional nerve dissection with less morbidity and confirms that tumour recurrence cannot be ascribed to any properties of the tumour but lies in the hands of the surgeon and depends on the care with which the tumour is removed. (+info)
PLAG1, the main translocation target in pleomorphic adenoma of the salivary glands, is a positive regulator of IGF-II.
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PLAG1, a novel developmentally regulated C2H2 zinc finger gene, is consistently rearranged and overexpressed in pleomorphic adenomas of the salivary glands with 8q12 translocations. In this report, we show that PLAG1 is a nuclear protein that binds DNA in a specific manner. The consensus PLAG1 binding site is a bipartite element containing a core sequence, GRGGC, and a G-cluster, RGGK, separated by seven random nucleotides. DNA binding is mediated mainly via three of the seven zinc fingers, with fingers 6 and 7 interacting with the core and finger 3 with the G-cluster. In transient transactivation assays, PLAG1 specifically activates transcription from its consensus DNA binding site, indicating that PLAG1 is a genuine transcription factor. Potential PLAG1 binding sites were found in the promoter 3 of the human insulin-like growth factor II (IGF-II) gene. We show that PLAG1 binds IGF-II promoter 3 and stimulates its activity. Moreover, IGF-II transcripts derived from the P3 promoter are highly expressed in salivary gland adenomas overexpressing PLAG1. In contrast, they are not detectable in adenomas without abnormal PLAG1 expression nor in normal salivary gland tissue. This indicates a perfect correlation between PLAG1 and IGF-II expression. All of these results strongly suggest that IGF-II is one of the PLAG1 target genes, providing us with the first clue for understanding the role of PLAG1 in salivary gland tumor development. (+info)
Cartilage-specific matrix protein chondromodulin-I is associated with chondroid formation in salivary pleomorphic adenomas: immunohistochemical analysis.
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Chondromodulin-I (ChM-I) is a novel cartilage-specific matrix protein. In the growth plates of the long bones, ChM-I was shown to be expressed in mature to upper hypertrophic chondrocytes, and to be deposited in the cartilage matrix. As ChM-I strongly inhibits angiogenesis, cartilage is avascular. Also, ChM-I has bifunctional activity against chondrocyte proliferation. On the other hand, pleomorphic adenomas of the salivary glands frequently have chondroid elements. To elucidate the relationship between chondroid formation and hypovascularity in salivary pleomorphic adenomas, we immunohistochemically examined the expression and localization of ChM-I in 35 cases of this tumor. ChM-I was immunolocalized to the lacunae in the chondroid elements of pleomorphic adenomas (100%). Type II collagen and aggrecan were immunolocalized throughout the matrix around lacuna cells of the chondroid element (100%, 91.7%), and ChM-I was infrequently immunolocalized to the spindle-shaped myoepithelial cells in the myxoid element (37.5%). Fibroblast growth factor-2 was strongly immunolocalized to the lacuna cells in the chondroid element (100%), among the neoplastic myoepithelial cells in the myxoid elements (96.9%), and on the basement membranes around the solid nests of neoplastic myoepithelial cells (71.4%). Although CD34 is a marker of endothelial cells, CD34 was expressed in the endothelial cells in only a few areas around the epithelial elements and in the fibrous element of pleomorphic adenomas. No signals for CD34 were observed in chondroid elements in pleomorphic adenomas (P < 0.001), but a few signals were seen in the myxoid elements (P < 0.05). These findings suggested that lacuna cells and neoplastic myoepithelial cells expressed ChM-I, and that this molecule may play an important role in hypovascularity and chondroid differentiation in pleomorphic adenoma. In conclusion, pleomorphic adenoma expressed ChM-I, which is involved in hypovascularity and chondroid formation in this type of tumor. (+info)
Pulmonary epithelial-myoepithelial tumor of unproven malignant potential: report of a case and review of the literature.
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Epithelial-myoepithelial tumors of the lung are rare neoplasms whose biological behavior and clinical course still remain to be defined. A case of epithelial-myoepithelial tumor of the lung arising from bronchial mucosa-submucosa and occurring as a polypoid lesion of the upper left bronchus in a 47-year-old man is reported. The tumor did not infiltrate the cartilaginous wall of the bronchus and showed a biphasic histological appearance with a double layering of epithelial and myoepithelial cells. Myoepithelial spindle cells with eosinophilic cytoplasm were also observed. Mitotic figures were very rare and necrosis absent. Immunohistochemical study for epithelial and muscular markers confirmed the presence of a double-cell component in the tumor, namely epithelial and myoepithelial. The patient is alive and well, with no evidence of recurrent or metastatic disease 6 months after surgery. On the basis of the present case and the six previously reported cases, we suggest using the noncommittal term pulmonary epithelial-myoepithelial tumor of unproven malignant potential (PEMTUMP) for this type of neoplasm. In addition, we first introduce p63 as a novel marker for highlighting the myoepithelial cells of the respiratory tract and speculate on the role of these cells in the development of this unusual tumor. (+info)
High-dose-rate endobronchial brachytherapy in endobronchial metastatic malignant chondroid syringoma.
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A 65-year-old man with malignant chondroid syringoma (MCS) was found to have pulmonary metastases in the form of multiple pulmonary nodules 4 years after wide excision and adjuvant radiotherapy of a primary abdominal wall tumor. Atelectasis of the lingula due to obstructive endobronchial metastasis, resistant to combination chemotherapy, led us to perform high-dose rate (HDR) endobronchial brachytherapy for the first time in this rare tumor with a favorable response. This case emphasizes the role of HDR brachytherapy as a palliative procedure in endobronchial tumors not responding to other treatment modalities, even those considered to be radioresistant. (+info)
Translocation, deletion/amplification, and expression of HMGIC and MDM2 in a carcinoma ex pleomorphic adenoma.
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Carcinoma ex pleomorphic adenoma (CexPA) is a carcinoma developing within a pre-existing benign pleomorphic adenoma (PA). Here we describe the identification and characterization of a series of genetic events leading to translocation, deletion/amplification, and overexpression of the HMGIC and MDM2 genes in a CexPA at an early stage of development. The tumor had a pseudodiploid stemline karyotype with a del(5)(q22-23q32-33) and a t(10;12)(p15;q14-15). In addition, there were several sidelines with double minute chromosomes (dmin) or homogeneously staining regions (hsr). Fluorescence in situ hybridization (FISH) mapping revealed that the 12q14-15 breakpoint was located centromeric to HMGIC and that the entire gene was juxtaposed to the der(10) chromosome. Detailed analysis of cells with dmin and hsr revealed that HMGIC and MDM2 were deleted from the der(10) and that the dmin and hsr were strongly positive for both genes. Southern blot analysis confirmed that both HMGIC and MDM2 were amplified and that no gross rearrangements of the genes had occurred. Immunostaining revealed that the HMGIC protein was highly overexpressed particularly in the large polymorphic cells within the carcinomatous part of the tumor. These findings suggest that amplification and overexpression of HMGIC and possibly MDM2 might be important genetic events that may contribute to malignant transformation of benign PA. (+info)
Intraosseous pleomorphic adenoma: case report and review of the literature.
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Pleomorphic adenoma is the most common neoplasm of the salivary glands, affecting mainly the parotid gland. The preferential intraoral site of this tumor is the palate. A case of a 31-year-old woman with an intraosseous pleomorphic adenoma located in the maxilla (left paramedian region), showing an approximate evolution of one year is reported. The present intraosseous case represents a rare location, with the tumor probably originating from glandular epithelial remnants captured during embryogenesis. In a review of the literature of 142 cases of intragnathic localization (24% in the maxilla) are identified. A slight predominance of women was observed (56%), with 55% of the patients being affected during the 5th to 7th decade of life. The tumors were malignant in 94% of the cases, with special predominance of mucoepidermoid carcinoma (65%). Intraosseous pleomorphic adenomas are rare, with the present patient being the 6th case reported in the literature and the second found in the maxilla. Mean age of the 5 previously reported cases was 58.8 years. (+info)