1/6. Typical and atypical Carney's triad presenting with malignant hypertension and papilledema.This report encourages clinicians to consider a diagnosis of Carney's triad in patients with multifocal gastric stromal sarcoma, extraadrenal paraganglioma (predominantly mediastinal), or pulmonary chondroma. The authors conducted a retrospective 20-year survey at the Hospital for Sick Children and identified two children with Carney's triad. One child, presenting atypically with papilledema and fundal hemorrhages from malignant hypertension and benign intracranial hypertension from chronic iron-deficiency anemia, is the second patient ever to date be described with the complete Carney's triad of neoplasms at diagnosis. Another child presented more typically with gastric stromal sarcoma and pulmonary chondroma without paraganglioma. Carney's triad is a rare differential diagnosis for "idiopathic" hypertension or iron-deficiency anemia from chronic gastrointestinal bleeding. If missed, patients with Carney's triad may have the debilitating physical and mental consequences of chronic iron deficiency and may die of untreated prolonged hypertension and metastatic leiomyosarcoma.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/6. A case of familial paraganglioma syndrome type 4 caused by a mutation in the SDHB gene.BACKGROUND: A 40-year-old man was referred to our clinic with recurrent paragangliomas. He had undergone resection of a paraganglioma superior to the right adrenal gland at 19 years of age, resection of two para-aortic paragangliomas at 39 years of age, and resection of a paraganglioma in the interatrial septum at 40 years. The patient's mother had died at age 39 years of metastases from a carotid body tumor. INVESTIGATIONS: MRI and CT scanning, 131I-labeled metaiodobenzylguanidine scanning, and genetic testing for a mutation in the succinate dehydrogenase complex, subunit B gene. diagnosis: Familial paraganglioma syndrome type 4 caused by a mutation in the succinate dehydrogenase complex, subunit B gene. MANAGEMENT: The patient underwent two surgical procedures in our clinic. The first was to remove two para-aortic paragangliomas, and the second to remove a paraganglioma that involved both atria. The patient is at high risk for malignant disease and should undergo an annual monitoring program that consists of physical examination and measurement of his blood pressure and levels of urinary catecholamines and metanephrines. If these procedures suggest a recurrence of paraganglioma, 123I-labeled metaiodobenzylguanidine scanning should be performed. As he might develop nonfunctional tumors, however, he should also undergo CT scanning, MRI scanning, or both, of the neck, thorax, abdomen, and pelvis every 6-12 months. genetic testing has been offered to family members.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/6. glomus tympanicum--excision by radical mastoidectomy exposure with autograft reconstruction.Wide surgical exposure, total tumor removal and anatomic and functional reconstruction are the goals of tumor management. These goals are met by radical mastoidectomy exposure for the removal of glomus tympanicum tumors and reconstruction by autograft replacement of the posterior osseous canal, and if the tympanic membrane, malleus and incus are removed for tumor exposure, autograft replacement may also be accomplished. The history, physical findings, x-rays and details of surgical management of two patients with glomus tympanicum are reviewed. Wide removal of the posterior osseous canal, just lateral to the VIIth nerve with total replacement of this segment gives excellent exposure of the middle ear space and affords total reconstruction of the posterior osseous canal.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/6. Management of bilateral carotid body tumors and a glomus jugulare tumor in a child.This paper reports on a 14-year-old female child with bilateral carotid tumors and a glomus jugulare tumor, a rare combination of tumors in any patient and previously unreported in a child. The literature is reviewed and the details of the history, physical findings, results of laboratory tests, and the surgical management of the patient are discussed. The need for an exhaustive preoperative evaluation is stressed so that the proper surgery can be planned. The difficulties of surgical management, the complications, morbidity and potential hazards of surgery in these vascular tumors are presented. The unusual complications that a patient in the pediatric age group encountered as compared to those seen in adults are discussed.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/6. pain-inducing laryngeal paraganglioma: report of the ninth case and review of the literature.There are two distinct forms of laryngeal paraganglioma. The patient with the rare Type II has exquisite throat pain that is often clinically confusing. The small size or submucosal location of the lesion often results in a delay in correct diagnosis and treatment. The lesion is very aggressive, even if tiny, generally with widespread metastasis. This tumor should be considered in the differential diagnosis of severe persistent throat pain, especially in the absence of abnormalities on physical or radiologic examination.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/6. Angiographic detection of unsuspected carcinoma of the thyroid gland.angiography is seldom used to detect carcinoma of the thyroid. We have recently encountered four patients with hypervascular lesions of the neck demonstrated by angiography for evaluation of an unrelated medical problem. In each, carcinoma of the thyroid was detected and total thyroidectomy performed. Because of the possibility of carcinoma of the thyroid in patients with hypervascular lesions of the neck, we recommend exploration of the neck, regardless of symptoms, physical findings or scintiscan results.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |