Cases reported "Adenoma"

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1/59. Aggressive giant pituitary adenoma presenting as a nasopharyngeal mass: magnetic resonance imaging and pathologic findings.

    We report a giant pituitary adenoma with aggressive histologic features that prominently invaded the nasopharynx. magnetic resonance imaging (MRI) demonstrated a large heterogeneous nodular mass that was hypointense to isointense on T1-weighted images and mixed hypointense, isointense, and hyperintense on T2-weighted images. The mass measured 7.5 x 5 x 7 cm, extending from the nasopharynx posteriorly through the clivus, and superiorly through the paranasal sinuses, and sellar-suprasellar region. After contrast administration, heterogeneous nodular enhancement was noted. A nasopharyngeal neoplasm extending into the sella was suspected because voice change and nasal speech long preceded the patient's visual symptoms. A biopsy disclosed an aggressive, infiltrating, hemorrhagic tumor, which was diagnosed as a non-secreting pituitary macroadenoma. This report indicates that pituitary adenomas may grow invasively to tremendously large sizes resulting in their initial presentation as nasopharyngeal masses.
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2/59. Unusual choristoma of the parotid gland in a girl. A possible trichoadenoma.

    An 8-year-old girl had a painless circumscribed nodule in the right parotid gland for 4 weeks. A tumour (1.3 cm diameter) within the salivary gland parenchyma showed small cystic spaces with horn-like material macroscopically. The tumour tissue contained solid squamous cell formations and cystic spaces limited by multilayered squamous epithelium and covered by layers of ortho- and parakeratotic cells. The cystic spaces contained keratotic lamellae. In some areas pin-like epithelial proliferations were seen. All epithelial cells were characterized by uniform nuclei, and no atypical mitoses were seen. Keratinizated masses with partial calcification were occasionally located in the interstitial tissue and replaced by multinucleated giant cells. The tumour was classified as a choristoma and resembled a trichoadenoma. The ectodermally derived oral and salivary gland epithelium may be the source of skin-like or adnexal tumours.
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3/59. Clinical and morphological features of undifferentiated monomorphous GH/TSH-secreting pituitary adenoma.

    A 41-year-old male presented with progressive visual defects, acromegaly and hyperthyroidism. After clinical evaluation a giant GH/TSH-secreting pituitary adenoma was diagnosed. Administration of the somatostatin analog octreotide at doses of 150 microg s.c. per day inhibited the secretion of both GH and TSH. A three-week treatment with octreotide prior to surgery led to slight visual improvement and CT scan showed some new necrotic areas within the tumor mass. Transcranial surgery was performed. By immunohistochemical analyses of the adenoma tissue GH, prolactin and beta-chorionic gonadotropin were detected; TSH was negative. Electron microscopy revealed an undifferentiated, monomorphous adenoma with morphological features of an acidophil stem cell adenoma such as the presence of misplaced exocytoses, fibrous bodies and mitochondrial gigantism. However, the tumor cells contained small secretory granules (up to 250 nm) accumulated along the cell membrane characteristic of thyrotrope cells. Furthermore, some adenoma cells were fusiform with long cytoplasmic processes resembling thyrotropes. Two months after the operation CT scan revealed a large residual tumor. serum GH and TSH levels had increased again and the TSH level was even higher than before the treatment. The patient died suddenly, most probably of lethal arrhythmia. Specimens of the adenoma tissue obtained at autopsy confirmed the previous findings with the exception of positive immunostaining for TSH which was found in less than 1% of the adenoma cells. This undifferentiated, monomorphous GH/TSH-secreting pituitary adenoma represents an entity that is unusual both in its ultrastructural features and clinical manifestations suggesting a cytogenesis from an early, undifferentiated stem cell.
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4/59. pituitary apoplexy probably due to TRH and GnRH stimulation tests in a patient with acromegaly.

    pituitary apoplexy is the most serious and life-threatening complication of pituitary adenomas. Most of the cases occur spontaneously but it may occur also after a number of events such as the pituitary stimulation tests. We report a case of acromegaly due to a giant pituitary adenoma in which pituitary apoplexy developed 88 hours after TRH/GnRH stimulation test. The patient had severe headaches, nausea, vomiting, visual disturbance and mental alteration and the computed tomography (CT) scans revealed intratumoral and intraventricular bleeding. The pituitary mass was removed by transsphenoidal approach. The patient developed pneumonia and died on the 9th postoperative day. pituitary apoplexy was confirmed at surgery and on histological examination. Immunohistochemical staining was positive for GH and PRL. This case indicates that pituitary apoplexy may develop several days after TRH/GnRH stimulation test.
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5/59. Brown tumour of hyperparathyroidism in the mandible associated with atypical parathyroid adenoma.

    The brown tumour of hyperparathyroidism is a localized bone tumour and an uncommon manifestation of hyperparathyroidism. A 27-year-old woman presented with a mandibular 8 x 10 cm solid mass diagnosed as central giant cell granuloma. Chemical blood analysis revealed increased serum calcium levels of 12.46 mg/dL and the parathyroid hormone level was 124 pg/dL. The patient underwent surgery with removal of a parathyroid mass. Histologically, this parathyroid tissue was seen to be limited by a fibrous capsule with morphological features consistent with atypical parathyroid adenoma. The mandibular tumour has receded and the patient declined further procedures. This is the first case reported of brown tumour as the primary manifestation of an atypical parathyroid adenoma, a lesion that shares some features with parathyroid carcinoma without the unequivocal properties of malignancy.
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6/59. Highly aggressive brown tumour of the maxilla as first manifestation of primary hyperparathyroidism.

    A case is presented of a 62-year-old man with a right maxillary swelling for the previous three months. The lesion was expansive and osteolytic, with invasion of the adjacent maxillary sinus, nasal and pterygomaxillary fossae and floor of the orbit. histology revealed the presence of an intrabony giant cell lesion. blood tests demonstrated elevations in calcium (16.2 mg/dl) and parathyroid hormone (PTH) concentrations (841 pg/ml). This suggested the diagnosis of hyperparathyroidism initially manifesting as a brown tumour of the maxilla. Posterior explorations confirmed the existence of an underlying ectopic parathyroid adenoma as the cause of the condition.
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7/59. Two familial giant pituitary adenomas associated with overweight: clinical, morphological and genetic features.

    OBJECTIVE: Pituitary adenomas are usually sporadic, although rare familial cases have been described. Here we report two first degree female cousins with giant pituitary adenoma and overweight. Both presented with secondary amenorrhoea, occasional headache and weight gain. MATERIALS AND methods: In both patients clinical, morphological and genetic studies were performed. Both patients underwent surgery and post-operative medical therapy with somatostatin analogues and dopamine agonist, followed by a conventional radiotherapy course. RESULTS: Clinical examination at presentation revealed an acromegaloid habitus only in the second patient. Basal and dynamic hormonal evaluation showed high serum GH and serum IGF-I values, higher in the second than in the first patient, and a mild hyperprolactinaemia only in the first patient. On optical and electron microscopy, both tumours were oncocytic adenomas, immunopositive for GH in the first patient and GH/prolactin in the second. The genetic analysis for germ-line mutations of the multiple endocrine neoplasia type 1 gene was negative. Two years after radiotherapy a remarkable shrinkage of both tumours was observed, whereas the overweight worsened in both patients, accompanied by high plasma leptin values. CONCLUSION: To our knowledge, this is the first report of familial pituitary adenomas including one case of a clinically silent GH-secreting adenoma. In addition, it provides further evidence that familial pituitary tumours can occur as a multiple endocrine neoplasia type 1 unrelated disease.
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8/59. A giant Brunner's gland adenoma presenting as gastrointestinal hemorrhage.

    Brunner's gland adenomas are rare tumors of the duodenum that are usually small in size. Only a few cases of tumors more than 4 to 5 cm in size are reported in the literature. Although the majority of patients are asymptomatic, hemorrhage and obstruction are the most clinically significant manifestations. We report a case of Brunner's gland adenoma in which the patient presented with major gastrointestinal bleeding. Endoscopic, radiologic, and endosonographic appearances are illustrated.
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9/59. Pigmented adrenal hyperplasia with myelolipomatous changes and bilateral testicular enlargement in an untreated man with 21-hydroxylase deficiency.

    OBJECTIVE: To report the occurrence of a giant left adrenal tumor and bilateral testicular masses (adenomatous hyperplasia of leydig cells) in a young man with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. methods: The clinical, radiologic, endocrinologic, and pathologic features of this case are correlated with the findings in the literature. RESULTS: The interesting elements in this case are the rare pathologic features of the left adrenal lesion (pigmented adrenal hyperplasia with myelolipomatous changes) and the association with the infrequent testicular adrenal rest tumors. The absence of enlargement of the right adrenal gland was unexplained. CONCLUSION: The presence of these two rare complications seemed to be associated with poor adherence to medical treatment recommendations for congenital adrenal hyperplasia.
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10/59. Giant intracranial granular-cell tumor arising from the abducens.

    We present a case of an adolescent with a giant intracranial granular-cell tumor, who presented with diplopia and worsening visual acuity. The tumor measured 8.2 cm at the maximum diameter and occupied the right middle cranial fossa, with intrasellar and suprasellar involvement. It was composed of solid nests of oval or polygonal eosinophilic cells with a focal pseudo-papillary configuration. Pituitary macroadenoma was suspected from examination of the intraoperative frozen section, but immunohistochemical and ultrastructural findings were consistent with granular-cell tumor, and they suggested the origin to be the peripheral nerve sheath. Considering that abducens palsy was the first clinical manifestation, the tumor was thought to arise from abducens. From histological findings (focal cellular spindling, mitotic activity and a relatively high Ki-67 labeling index [10.1%]) and according to Fanburg-Smith's criteria, the tumor was suspected to have low-grade malignant potential. Granular-cell tumor should be included in the differential diagnosis of parasellar tumors, even in young patients or patients with large lesions. Both immunohistochemical and electron microscopic studies are essential for diagnosis.
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