Cases reported "Mediastinal Neoplasms"

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1/71. Median sternotomy for parathyroid adenoma.

    Most mediastinal parathyroid tumours lie within the thymus gland and may be retrieved when cervical thymectomy is carried out in the course of neck exploration for primary hyperparathyroidism (HPT). We report 4 patients, each of whom required sternotomy for removal of a true mediastinal parathyroid adenoma. Subtraction isotope scintigraphy suggested the presence of a mediastinal tumour prior to cervical exploration in 2 individuals and prior to re-exploration in a third. When localisation before initial exploration for HPT suggests a parathyroid tumour within the chest, consideration should be given to proceeding to sternotomy, at first operation if a comprehensive neck exploration, including cervical thymectomy, fails to uncover the adenoma. Uniquely, one of our patients underwent sternotomy for HPT when 23 weeks pregnant.
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2/71. A new observation of the Carney's triad with long follow-Up period and additional tumors.

    The etiology of the Carney's triad (gastrointestinal stromal tumors, pulmonary chondromas, and paragangliomas) is unknown, and only 57 cases have been reported since its identification in 1977. We report the clinical course of a female with the complete triad and some additional tumors. Bilateral vagal paragangliomas were treated surgically and with radiotherapy between the ages of 24 and 26 years. Subsequently she underwent surgery for a gastric leiomyosarcoma (27 years), a pleomorphic adenoma of the parotid gland (49 years) and a multifocal breast cancer with axillary spread (50 years). A calcified lesion was also noticed in the left lung, the radiologic diagnosis of which was consistent with chondroma. A mediastinal paraganglioma, detected at 56 years on a control X-ray of the chest, was partially excised at 63 years. At the last control, performed at 66 years, the patient was alive with residual cervical and mediastinal paraganglioma. Her younger brother was affected by Hirschsprung's disease and died at 54 years of rectal cancer. Her daughter is 33 and has been suffering since birth with severe constipation. In conclusion, this is one of the longest followed-up patients with Carney's triad. Her case illustrates the need for early recognition of the setting in order to detect the component tumors at a stage when surgery may be curative, and careful and life-long follow-up, both because the multicentricity of the classic components tends to manifest metachronously and because of the tendency to develop other tumors, some of which may be malignant. Furthermore, the presence of Hirschsprung's disease in the patient's family, coupled with the alleged common origin of two component lesions from derivatives of the neural crest, open new avenues for the understanding of this disorder.
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3/71. Amphicrine tumor.

    The term amphicrine refers to cells, and tumors, which show both exocrine and endocrine features. Author s aim was to analyse the characteristics of these neoplasms. 40 suspicious cases were reviewed. Mucin-stains (PAS, diastase-PAS, Stains-all, Alcian-blue), immunohistochemistry (antibodies against Neuron-Specific Enolase (NSE), and chromogranin a (CGA), and electronmicroscopic studies were performed to demonstrate exocrine and/or endocrine features of the tumor cells. By means of these methods, 16 cases turned out to be amphicrine tumors. Among them, there were 4 sinonasal, 1 bronchial, 1 mediastinal, 8 gastrointestinal and 2 suprarenal gland neoplasms. In connection to the subject, a brief review is given of amphicrine tumor, regarding its etiological and pathological aspects. These tumors form a distinct clinicopathological entity and should be separated from both neuroendocrine tumors and adenocarcinomas.
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4/71. Neuroendocrine carcinoma of the posterior mediastinum: a possible primary lesion.

    A paravertebral mass was noted in the posterior mediastinum in a 47-year-old man. Microscopically, the tumor showed solid and trabecular patterns and consisted of poorly differentiated atypical cells that often formed Flexner-Wintersteiner rosettelike glands. Immunohistochemically, the tumor cells expressed both epithelial and neuroendocrine markers, including cytokeratin (AE1/3), carcinoembryonic antigen, epithelial membrane antigen, neuron-specific enolase, chromogranin a, and synaptophysin, but were negative for CD99 (MIC2). Ultrastructurally, numerous desmosomes and neurosecretory granules were identified in the tumor cells. The present lesion was a primary neuroendocrine carcinoma of the posterior mediastinum-an unusual site for such a lesion.
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5/71. Hurthle cell adenoma of the mediastinum: intraoperative cytology and differential diagnosis with correlative gross, histology, and ancillary studies.

    A 66-year-old man was found to have a 7.5 cm mediastinal mass detected on routine chest x-rays as part of his preoperative work up for an inguinal hernia repair. An orthotopic (normally located) nongoitrous thyroid gland without evidence of connection to the mediastinal mass was also identified. The clinical differential diagnoses included lymphoma, thymoma, and germ cell tumor. Fine-needle aspiration (FNA) biopsy smears and touch imprints of the mediastinal mass showed a loosely cohesive, highly cellular population of relatively uniform cells with abundant granular cytoplasm, low nuclear to cytoplasmic (N/C) ratios, and prominent nucleoli consistent with a Hurthle cell (HC) neoplasm. Subsequently, the diagnosis of HC adenoma was confirmed on the surgically excised mediastinal mass. To the best of our knowledge, the surgical pathology and cytologic features of an HC adenoma of the mediastinum have not been reported in the literature. The gross, histologic, immunohistochemical, and electron microscopic (EM) findings, in addition to the cytologic features, are presented along with a differential diagnosis of this mediastinal neoplasm.
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6/71. Thymic carcinoid and parathyroid hyperplasia detection with 99mTc-MIBI men type 1.

    We report a case of a 35-year-old male, with a history of diarrhea, renal lithiasis with frequent expulsions of calculus and hypercalcemia during the last 2 years. The patient was studied and diagnosed with a multiple endocrine neoplasia type I (MEN I), familiar (mother with MEN I). A scintigraphic study with 99mTc-MIBI was performed in order to localize hyperfunctioning parathyroid glands because of biochemical diagnosis of primary hyperparathyroidism. Double phase 99mTc-MIBI scan detected one hyperfunctioning parathyroid gland and a large anterior mediastinal mass. Subsequent, plain radiograph and CT of the chest showed a soft-tissue mass in that localization. Punch biopsy of the lesion guided by CT revealed malignant cells of neuroendocrine tumor. The tumor was removed and histologically confirmed as a carcinoid within a thymus in a MEN type I syndrome. MEN I patients can benefit from the examination with this agent which can potentially localize not only parathyroid endocrine pathology but also unknown associated tumors.
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7/71. A new pattern of multiple endocrine adenomatosis: chemodectoma, bronchial carcinoid, GH-producing pituitary adenoma, and hyperplasia of the parathyroid glands, and antral and duodenal gastrin cells.

    A female patient was found to have a chemodectoma, a GH-producing pituitary tumour and a bronchial carcinoid combined with hyperplasia of the parathyroids and of antral and duodenal gastrin cells. This combination of endocrine tumours and hyperplasias does not fit with the two multiple endocrine adenomatosis syndromes recognized at present. The case stresses the importance of scanning the patient for other endocrine tumours, once one has been diagnosed.
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8/71. Mediastinal extraadrenal myelolipoma: report of a case.

    We herein report a case of surgically resected mediastinal extraadrenal myelolipoma. myelolipoma is an uncommon tumor composed of adipose tissue and normal hematopoietic elements, and is most often found in the adrenal glands. We could find only five such cases of mediastinal myelolipoma in the English literature.
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9/71. Follicular carcinoma of the thyroid with functioning metastases and clinical hyperthyroidism.

    hyperthyroidism associated with metastatic follicular carcinoma of the thyroid gland is rare. In one patient the mass of functioning follicular tissue in the primary and metastatic tumour was so great that excessive amounts of thyroxine and triiodothyronine were produced and, as a result, clinical hyperthyroidism developed. This was in spite of the fact that the activity per unit of tissue was not supranormal and may even have been slightly subnormal. The initial response of the metastases to 131I ablative therapy was excellent.
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10/71. Laparoscopic management of a posterior mediastinal tumor mimicking an adrenal neoplasm.

    Background: Rarely, a posterior mediastinal mass may mimic an adrenal tumor on preoperative computed tomography scan. The intraoperative discovery that a mass thought to be associated with the adrenal gland actually is above the diaphragm in the posteroinferior mediastinum poses a challenge for the laparoscopic surgeon. Conversion to a thoracotomy or to videothoracoscopy incurs additional morbidity and risk for the patient. Materials and methods: We describe a technique for the transdiaphragmatic removal of a benign mass from the posterior mediastinum. A posterior mediastinal tumor was detected during a laparoscopic procedure for a suspected right adrenal tumor. Frozen section proved benign, and the mass was resected laparoscopically via transdiaphragmatic access to the posterior mediastinum. Results: No complications were noted during or after surgery. The patient was ready for discharge from the hospital on postoperative day 1. Conclusions: Transdiaphragmatic resection was done successfully instead of conversion to a thoracotomy or thoracoscopic procedure for a benign posterior mediastinal tumor found incidentally during laparoscopic surgery for a presumed adrenal lesion. This transdiaphragmatic approach can be applied to selected benign mediastinal masses.
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