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31/98. Bilateral synchronous submandibular lumps in a patient with gastric carcinoma.

    This case report deals with a patient who was readmitted with a bilateral submandibular swelling after having received primary surgery due to gastric adenocarcinoma 6 months before. After bilateral submandibulectomy both glands were diagnosed histopathologically as metastasis of adenocarcinoma. This is the rare case of a submandibular gland metastasis and the first case of a bilateral synchronous submandibular gland metastasis from gastric carcinoma.
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ranking = 1
keywords = carcinoma, adenocarcinoma
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32/98. Long-term treatment in infantile choriocarcinoma.

    The long-term treatment of a 5 month old boy with precocious puberty secondary to the production of chorionic gonadotropin (hCG) by a choriocarcinoma is described. Of 13 cases of infantile choriocarcinoma reported in the literature, none were successfully treated. The present study describes a partially successful outcome with embolization of a hepatic tumor, irradiation of lung and right submandibular tumors, chemotherapy with methotrexate (MTX), actinomycin D (ACD), cyclophosphamide (CPA) and etoposide (VP16), and splenectomy and hepatic lobectomy. Subsequently, the residual hepatic tumors were treated with high dose melphalan (HDM) followed by reinfusion of unpurged autologous marrow.
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ranking = 0.74573530144931
keywords = carcinoma
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33/98. Oncocytic carcinoma in the submandibular gland: report of a case based on anti-mitochondrial immunohistochemical observations.

    Oncocytic carcinoma arising in the submandibular gland is a very rare tumor that has only previously been reported in nine cases. This paper describes an additional case of oncocytic carcinoma in the right submandibular gland. The patient was a 55-year-old Japanese male who was presented with a 3 cm right submandibular mass. Both radical resection of the tumor and right supraomohyoid neck dissection were performed. Histologically, the tumor cells exhibited an abundant eosinophilic cytoplasm, which appeared to be finely granular, and invaded the surrounding tissues. In addition, perineural invasion was also observed. Electron microscopy demonstrated the presence of an abundance mitochondria in the cytoplasm of the tumor cells. However, since the fine structure of cytoplasm was destroyed by fixation and embedding of the tissue for the light microscopy, the cytoplasm of the tumor could not be observed clearly. Immunohistochemically, anti-mitochondrial antibody positivity was evident in the cytoplasm of the tumor cells. Consequently, we diagnosed the mass as oncocytic carcinoma. Usually, electron microscopy is necessary to diagnose oncocytic lesions, but the paraffin-embedded tissue was not suitable for electron microscopy. immunohistochemistry using an anti-mitochondrial antibody was found to be useful and helpful for the diagnosis of oncocytic lesions.
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ranking = 0.87002451835753
keywords = carcinoma
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34/98. Large-cell undifferentiated carcinoma of the submandibular gland.

    Large-cell undifferentiated carcinoma (LCUC) arising in the submandibular gland is so rare that there have been only a few reported cases. We encountered a 65-year-old Japanese male, whose left submandibular gland was enlarged due to LCUC. Exenteration of the left submandibular gland together with selective neck dissection was performed, followed by postoperative radiotherapy. Current literature concerning the histopathological and clinical features of this neoplasm was reviewed.
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ranking = 0.62144608454109
keywords = carcinoma
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35/98. Deregulation of the hedgehog signalling pathway: a possible role for the PTCH and SUFU genes in human rhabdomyoma and rhabdomyosarcoma development.

    The naevoid basal cell carcinoma syndrome (NBCCS) is caused by mutations in the hedgehog receptor PTCH gene. It is characterized by developmental defects and a predisposition to the development of certain tumours, such as basal cell carcinoma, medulloblastoma and meningioma, and potentially fetal rhabdomyomas and embryonal rhabdomyosarcomas. This study aimed to analyse PTCH status in an NBCCS patient with fetal rhabdomyoma and to investigate whether deregulation of hedgehog signalling, as shown by altered expression of hedgehog pathway components and/or genetic imbalances, is a general finding in sporadic rhabdomyomas and rhabdomyosarcomas. The NBCCS patient had a novel PTCH germ-line mutation, 1370insT, and developed a fetal rhabdomyoma that harboured a 30 bp in-frame deletion in the second allele resulting in homozygous inactivation of PTCH. Sporadic rhabdomyomas and rhabdomyosarcomas showed overexpression of PTCH (43/43) and GLI1 (41/43) mRNA, as determined by in situ hybridization, indicating ongoing active hedgehog signalling. Immunohistochemical staining revealed a subgroup of fetal rhabdomyomas and embryonal rhabdomyosarcomas (12/34) lacking PTCH immunoreactivity. Four of nine informative fetal rhabdomyomas and embryonal rhabdomyosarcomas showed loss of heterozygosity (LOH) in the PTCH region with two of these (one fetal rhabdomyoma and one embryonal rhabdomyosarcoma) also showing LOH in the SUFU region. These findings suggest that haploinsufficiency for the two tumour suppressor genes PTCH and SUFU, which are both active in the same signalling pathway, may be important for tumour development. Based on our results we propose that the pathogenesis of rhabdomyoblastic tumours, particularly fetal rhabdomyomas and embryonal rhabdomyosarcomas, involves deregulation of the hedgehog signalling pathway.
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ranking = 0.24857843381644
keywords = carcinoma
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36/98. The sclerosing variant of adenoid cystic carcinoma: a previously unrecognized neoplasm of major salivary glands.

    We report 2 cases of a previously unrecognized sclerosing variant of adenoid cystic carcinoma (ACC) of major salivary glands. One of the tumors arose in the parotid and the other in the submaxillary gland of young adult patients. The tumors were composed predominantly of varying-sized large sclerotic and hypocellular nodules containing myoepithelial cells and pseudovascular spaces, most likely the result of artifactual retraction. In moderately cellular nodules, there were numerous small globules or spherules surrounded by myoepithelial cells similar to those of collagenous or mucinous spherulosis. Focal cribriform areas and ductal structures lined by epithelial cells were also identified. Both tumors showed perineural invasion. Electron microscopy revealed that both large nodules and small globules or spherules were composed of excessive amounts of basement membrane and thick-banded collagen fibers. The myoepithelial cells showed immunoreactivity for smooth muscle actin (SMA), S100 protein, and vimentin. collagen IV showed variable reactivity in both the large nodules and small spherules. More cases of the sclerosing variant of ACC and long-term follow-up of the patients are needed to determine the biologic behavior of this unusual but distinctive variant of ACC.
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ranking = 0.62144608454109
keywords = carcinoma
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37/98. Malignant salivary gland tumors: squamous cell carcinoma of the submandibular gland in a child.

    PURPOSE: Malignant neoplasms of the salivary glands are extremely unusual in the pediatric age group. methods: We report an 11-year-old boy who presented with a mass in the left submandibular region and a second mass in the jugularfacial venous angle. RESULTS: Histologic evaluation determined that this was a squamous cell carcinoma of the submandibular gland with metastasis to a cervical node. review of the literature was undertaken to identify the rate of malignant salivary gland tumors in children. Malignant salivary gland tumors are extremely rare in children. When malignancy does occur, nearly 90% are present in the parotid gland. The submandibular gland is effected in 7.7% of cases. The most common malignancy in salivary glands of children is the mucoepidermoid carcinoma. Squamous cell carcinoma occurs in less than 2% of cases. CONCLUSIONS: Salivary gland tumors are very rare in children in contrast to adults. The proportion of malignant tumors in children is higher than that observed in adults; however, it is not possible to draw conclusions concerning treatment from the few case reports that exist. A higher rate of local recurrence and cervical lymph node metastasis may be expected in children.
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ranking = 0.87002451835753
keywords = carcinoma
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38/98. Central adenoid cystic carcinoma of the mandible: case report and literature review of 16 cases.

    Central intraosseous adenoid cystic carcinoma (ACC) of the mandible, formerly known as cylindroma, is a rare neoplasm with only 16 cases reported in the literature. We describe the diagnosis, etiology, and treatment of a central ACC located in the mandibular premolar region. We also review the literature. This case illustrates 2 key facts regarding the diagnosis and etiology of ACC. First, central salivary gland tumors should be considered in the differential diagnosis of cystic lytic lesions in the mandible. Second, even though the origin of this type of tumor is still unknown, the presence of ectopic tissue anterior to the submandibular gland in the submandibular area indicates that this tumor might be made up of ectopic embryogenic inclusions.
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ranking = 0.62144608454109
keywords = carcinoma
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39/98. Hepatic metastases in adenoid cystic carcinoma of the submandibular gland.

    We present a case of adenoid cystic carcinoma of the submandibular gland resulting in metastases to the liver, nine years following initial presentation.
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ranking = 0.62144608454109
keywords = carcinoma
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40/98. A case report of coexistence of a sialolith and an adenoid cystic carcinoma in the submandibular gland.

    The occurrence of sialoliths in the submandibular gland is 80% due to the specific anatomy of both the gland and its duct. The diagnosis is rather easy because of the obvious clinical signs of the entity. Imaging studies are always necessary in order to treat the patient as effectively as possible. The stones do not tend to occur within the gland as frequently as in the respective duct. The coexistence of sialoliths and malignant tumors is extremely rare. A 70-year-old woman with intraparenchymal stone was operated in our ENT department. In addition to the sialolith the pathological examination revealed the existence of an adenoid cystic carcinoma (ACC), that extended to the neighboring skeletal muscle. This is the reason why we believe it would be useful to report this case of a large stone (14 mm in diameter) located in the submandibular gland coexisting with ACC. This case report is a very good example illustrating that all available means should be used prior to reaching a conclusion and making a health professional decision.
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ranking = 0.62144608454109
keywords = carcinoma
(Clic here for more details about this article)
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