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1/72. Diagnostic and therapeutic approach to sialoblastoma: report of a case.

    The natural history of a rare parotid tumor, the sialoblastoma (embryoma) is reported. It is a blastematous neoplasm said to recapitulate the epithelial differentiation of a gland at various stages of development. The tumor grew in a period of 5 years (from shortly after birth until it was excised) to a firm asymptomatic mass measuring 5 cm in greatest diameter. With a diagnosis of adenoid cystic carcinoma (a common misdiagnosis), the patient was referred to the reporting institution, where the diagnosis was revised. Five months later, completion parotidectomy for presumed recurrence failed to demonstrate residual tumor. One year later, the patient appears to be free of disease. The authors propose that sialoblastomas should be regarded neither as benign nor malignant, but as one single disease with local infiltrative potential. Based on this concept, sialoblastomas can be treated with early conservative surgery alone, provided that free margins are obtained.
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2/72. HCG induced hyperthyreosis in germ cell cancer.

    Human germ cell tumors have the unique capacity for totipotential differentiation. AFP (the product of normal yolk sac) and HCG (produced by trophoblastic tissues) are frequently produced by germ cell tumors. The a-subunit of the glycoprotein HCG is identical to that of several pituitary glycoprotein hormones (e.g. TSH, LH, FSH), whereas the b-subunit of HCG, TSH, LH and FSH is homologous but distinct in the terminal amino acid sequence suggesting that HCG is part of a superfamily of gestational hormones. However, the role of TSH within this hormone superfamily is still not yet established. A 24-year old patient was admitted to our clinic because of a widespread recurrence of a germ cell tumor (stage IIIC, Lugano classification). The routine hematologic and blood chemical tests were normal, yet, an elevated HCG was found. In addition, increased levels of the thyroid hormones FT3 and FT4 were seen, although, this was not associated with clinical symptoms of a hyperthyreosis. There was no history of hyperthyreosis and thyroidal autoantibody screening revealed normal titers. An ultrasound examination of the thyroid gland showed no abnormalities and no iodine exposure had occurred during the last months. To mobilize peripheral stem cells (PBSC) he was initially treated with paclitaxel (175 mg/m2) and ifosfamide (8.000 mg/m2)) followed by apheresis of PBSC. The patient was then entered in our phase-II-study for relapsing germ cell carcinomas using a high-dose chemotherapy regime (paclitaxel 175 mg/m2, ifosfamide 9.000 mg/m2, carboplatin 900 mg/m2, etoposide 900 mg/m2) with subsequent retransfusion of collected stem cells. Due to cranial metastases an cranial irradiation was also performed. After three courses of this protocol an excellent partial remission of the tumor lesions was achieved and the HCG value dramatically decreased. Due to elevated thyroidal hormones, the patient was initially treated with thiamazole (20 mg) resulting in decrease of the thyroidal hormones. Thus, the thiamazole dose was reduced to 5 mg and then omitted. The decrease of the thyroidal hormones FT3 and FT4 strongly correlated with the reduction of HCG values (r2 0.91 and 0.77, p < 0.0008). To date there is only slight evidence that enhanced HCG levels may cause, at least in part, a hyperthyreosis (e.g. gestational hyperthyreosis), however, the underlying biochemical mechanism still remains unclear. In this case report we have demonstrated a clear positive correlation between HCG levels and thyroidal hormones in a patient with germ cell tumor suggesting a direct stimulation of hormone producing thyroidal cells by HCG, however, this was not associated with clinical symptoms of hyperthyreosis. Currently, several in vitro studies are underway in our laboratory to further elucidate the biochemical mechanisms of HCG induced hyperthyreosis.
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3/72. Case report: congenital salivary gland analage tumor presenting with neonatal respiratory distress.

    Stridor in the newborn is not an unusual entity. This article describes a rare presentation of neonatal respiratory distress caused by a benign nasopharyngeal salivary gland analage tumor.
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4/72. Pleomorphic adenoma of the human female breast.

    We are presenting an interesting rare benign breast tumor which meets the characteristics of a salivary gland pleomorphic adenoma. The tumor was misdiagnosed during frozen section procedure, because several clusters, mainly composed of myoepithelial cells and surrounded by a chondroid matrix, were mistaken for cancerous blasts. Additionally the clinical and mammographic findings were very suspicious. Although this particular tumor is very infrequent, the pathologist should be aware of the difficulties in the differential diagnosis during frozen section and thus defer his final answer to the paraffin sections.
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5/72. Melanotic neuroectodermal tumour as a predominant component of an immature testicular teratoma. Case report with immunohistochemical investigations.

    A case of melanotic neuroectodermal tumour (MNT), or so-called retinal anlage tumour, as a predominant component of an immature testicular teratoma is presented. The patient was a 17-year-old man who furthermore had a mature mediastinal teratoma. The MNT was composed mainly of two cell types: small immature neuroblast-like cells and large columnar or cuboidal epithelial-like cells with or without melanin granules. The tumour cells were arranged in solid formations, nests, cords, alveolar and pseudoglandular structures with cleft-like or glomeruloid-like spaces. Myogenic differentiation was found in minor foci. immunohistochemistry showed both neuroepithelial and mesenchymal features with positive staining reaction for neuron-specific enolase (NSE), S-100 protein (S-100), melanoma antigen (HMB45), cytokeratin and vimentin. vimentin, desmin and actin were present in the myoid cells. To the best of our knowledge this is the first reported case of MNT originating in the testis. As this tumour component occurred in an immature teratoma, neuroectodermal differentiation of germ cell origin is considered most likely.
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6/72. A case report of mixed tumor arising in the vagina.

    We reported a case of mixed tumor arising in the vagina of a 32-year-old Japanese woman. The tumor was a finger-like polyp protruded from the lower portion of the vagina. The histologic feature of the tumor resembled that of pleomorphic adenoma of the salivary gland, consisting of stromal-type cells embedded in the myxomatous stroma, although there was no cartilagenous component. Immunohistochemical study also suggested resemblance to pleomorphic adenoma of salivary gland, being positively stained with antibodies against cytokeratin, epithelial membrane antigen, vimentin and muscle actin, but negatively with an antibody against S-100 protein. The histogenesis of the tumor is not clear, but it may be originated from myoepithelial cells.
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7/72. Mixed germ cell-sex cord stromal tumor with heterologous structures.

    A mixed germ cell-sex cord stromal tumor developed in the right ovary of a 4-year-old girl. The patient's cells contained sex chromatin and the karyotype was 46,XX. Clinically, she exhibited mild isosexual pseudopuberty. Some of the tumor cells showed differentiation to sertoli cells, whereas others had characteristics of germ cells. The neoplasm was composed of solid cords consisting of a mixture of the two tumor cell types and surrounded by a delicate connective-tissue network. In one small area, the tumor contained heterologous development in the form of glands and cysts lined by columnar mucinous epithelium containing numerous goblet cells and occasional argyrophilic neuroendocrine cells. Normal ovarian tissue was present at the periphery of the tumor. Electron microscopic study confirmed the presence of both Sertoli-like and germ cell-type cells. Immunohistochemical studies demonstrated vimentin positivity in both cell types (with preference for the Sertoli-like cells) and cytokeratin positivity in the Sertoli-like cells. The patient was symptom free 4 years after right oophorectomy, radiotherapy, and chemotherapy.
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8/72. Ceruminous gland tumours: a reappraisal.

    Ceruminous glands should no longer be regarded as purely apocrine glands, but as apoeccrine glands with both apocrine and eccrine modes of secretion. We present two cases of pleomorphic adenoma of ceruminous glands, among the rarest of such tumours. The use of such terms as 'ceruminoma' and 'hidradenoma' should finally be abandoned, and 'ceruminous gland tumour' used instead as a generic term. classification should be based on Wetli's prototype (adenoma, pleomorphic adenoma, adenoid cystic carcinoma and adenocarcinoma), with the addition of benign eccrine cylindroma and syringocystadenoma papilliferum; the inclusion of mucoepidermoid carcinoma should await full validation. Wide local excision is necessary for all tumours, with only follow-up for histologically benign neoplasms. Malignant tumours need early aggressive surgery and radiotherapy. If marginal invasion cannot be assessed histologically, then adenoma and adenocarcinoma cannot be distinguished and we suggest that the tumour be reported as 'of uncertain malignant potential'. Long-term studies are needed to confirm or refute the view that all ceruminous gland tumours are potentially malignant.
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9/72. Immunohistochemical study of pleomorphic adenoma of the nasal septum.

    A rare case of pleomorphic adenoma of the nasal septum is reported. A 48-year-old man complaining of nasal obstruction and nasal bleeding was referred to our hospital for treatment of a left nasal tumor. The tumor, including the nasal septum, was resected by the modified Denker operation and lateral rhinotomy. The tumor was firm, parenchymatous, pedunculated, and 24 x 22 x 14 mm in size. Pathological examination revealed pleomorphic adenoma with slight cellular atypism. Immunohistochemical observations suggested that characterization of this tumor might be similar to pleomorphic adenoma of the parotid gland.
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10/72. pulmonary blastoma--a rare tumour.

    A 57-year-old man was found to have a tumour in the right lower lobe of the lungs, which was not classifiable by biopsy. The tumour could only be partially removed by surgical resection. The diagnosis of a pulmonary blastoma was made from the resected tissue. Clinically, rapid progress occurred with invasion in the mediastinal space and the epigastrium. In spite of radiation therapy, the patient died about 2 1/2 months after surgery of respiratory insufficiency. autopsy confirmed a pulmonary blastoma with extensive infiltration of the mediastinal space and upper abdomen as well as metastases in the regional lymph nodes, pleura, peritoneum, thyroid gland, heart and central nervous system. The present report of a pulmonary blastoma should draw attention to this extremely rare tumour. It should be included in the differential diagnosis, because the survival time can be increased if the correct diagnosis is made very early.
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