1/49. Multiple endocrine adenomatosis of mixed type.A case of multiple endocrine adenomatosis (MEA) of mixed type is presented. The syndrome, observed in a 65 year-old female, consisted of multiple neurofibroadenomatosis, medullary thyroid carcinoma, multiple adenomata of the parathyroids, adrenal cortical adenoma and small cell anaplastic bronchogenic carcinoma. Thus, it was composed of type 1 as well as of type 2 MEA. On the basis of another seven cases, collected from the literature, the MEA syndrome of mixed type is reviewed with special reference to the phylogenetic origin of the cells of the APUD system.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/49. A child with adrenocortical adenoma accompanied by congenital hemihypertrophy: report of a case.We report herein the findings of a 7-year-old male child with a ruptured adrenocortical adenoma and congenital hemihypertrophy which was incidentally detected after suffering a trauma. A review of 21 pediatric cases of adrenocortical neoplasms in the literature was made. The patient showed precocious puberty such as pubis and advanced bone age, but an endocrinological examination revealed no definite abnormalities. The right adrenal tumor with hematoma was resected after these evaluations. adrenocortical adenoma is considered to occur more frequently in female children. However, the incidence of adrenocortical tumors accompanied by congenital hemihypertrophy does not differ between males and females. The outcomes were relatively good, although the observation periods were short in some patients. A large number of patients presented with a tumor and hemihypertrophy on the same side. This finding is of interest when considering the possible association between hemihypertrophy of the organs and tumor proliferation. However, their association in terms of development was unclear. It is necessary for patients with hemihypertrophy to have regular examinations for the possible development of malignant tumors, especially in the kidney, adrenal gland, and liver.- - - - - - - - - - ranking = 0.00017778542271377keywords = bone (Clic here for more details about this article) |
3/49. Adrenocortical oncocytoma in pregnancy.BACKGROUND: Adrenal oncocytomas are uncommon, nonfunctioning tumors occurring most often in endocrine organs. CASE: A 32-year-old woman presented at 25 weeks' gestation complaining of right flank pain. Abdominal ultrasonography and computed tomography revealed a 9 x 10-cm solid right-sided adrenal mass. Endocrine evaluation was normal. At 36 weeks' gestation, she underwent cesarean followed by resection of the adrenal mass. Histopathologic and ultrastructural studies revealed a benign adrenocortical oncocytoma. CONCLUSION: Although rare, adrenocortical oncocytomas should be included in the differential diagnosis of solid, nonfunctioning, adrenal tumors in pregnancy.- - - - - - - - - - ranking = 0.2keywords = endocrine (Clic here for more details about this article) |
4/49. association of Fallot Tetralogy with Carney's complex.The carney complex is an inherited, autosomal disease of multicentric tumors in many organs. Some components of Carney's complex are cardiac myxoma, spotty pigmentation, and endocrine overactivity. Primary pigmented nodular adrenocortical dysplasia (PPNAD) is an exceedingly rare cause of Cushing's syndrome in infants, children, and young adults. PPNAD occurs sporadically or as part of a familial syndrome called Carney's complex. Up to our knowledge, the association of Fallot Tetralogy with Carney's complex has not been previously reported. We presented, a 20-year-old woman, who had been operated for Fallot Tetralogy at the age of 3 years, had Carney's complex, i.e. left atrial myxoma, two facial spotty pigmented areas, and PPNAD.- - - - - - - - - - ranking = 0.2keywords = endocrine (Clic here for more details about this article) |
5/49. Adrenal tumor with caval extension--case report and review of the literature.Extension of adrenal cortical carcinomas into the IVC is rare. We describe one such tumor that extended to the level of the right atrium. In an effort to aid recognition and guide work-up of an upper pole lesion, we review the literature comprised of 77 similar cases and analyze the data in terms of patient demographics, anatomic distribution, clinical and laboratory evidence of endocrine function. Our review suggests that over half of all adrenal lesions with IVC extension will be clinically nonfunctional, including up to 17% of pheochromocytomas.- - - - - - - - - - ranking = 0.2keywords = endocrine (Clic here for more details about this article) |
6/49. Adrenal cortical diseases: international case conference.Six pathologists from japan and the United Kingdom evaluated four different cases of adrenocortical disorders independently. These adrenocortical disorders included an adrenal tumor in a 45-yr-old female without any endocrine abnormalities, bilateral adrenocortical lesions in a 55-yr-old female with cushing syndrome, an adrenocortical mass in a 44-yr-old man with hypertension, and an adrenocortical lesion in a 62-yr-old female with chronic hypertension for 30 yr. In this article, we provide a clinical summary, macroscopic and histologic findings, and histologic diagnosis of these four adrenocortical cases.- - - - - - - - - - ranking = 0.2keywords = endocrine (Clic here for more details about this article) |
7/49. Oncocytic adrenocortical carcinoma: a morphologic, immunohistochemical and ultrastructural study of four cases.We present the clinical, histologic, immunohistochemical, and ultrastructural findings of four cases of non-functioning oncocytic adrenocortical carcinomas. The patients' ages ranged from 39 to 71 years. There was no sex predilection. Large yellow-tan tumors (8.5 to 17.0 cm), well demarcated from the adjacent kidney, were seen with a thin rim of normal adrenal gland along one edge. One tumor invaded the inferior vena cava and extended up to the level of the right atrium, and another metastasized to bone. The other two tumors had similar morphologic features and therefore were considered carcinomas. Histologic sections of all four cases showed a diffuse proliferation of polygonal neoplastic cells with large nuclei containing prominent nucleoli and abundant granular and eosinophilic cytoplasm. Occasional mononuclear and binucleated giant cells were noted in one case. There were rare mitotic figures (less than one per 10 high power fields). All tumors were immunoreactive for cytokeratins (AE1/AE3 and CAM5.2). Inhibin was focally expressed by one tumor and its bone metastasis. Ultrastructurally, the cytoplasm of the neoplastic cells was packed with innumerable mitochondria. Cytologic atypia or mitotic rate cannot reliably predict the biologic behavior of oncocytic adrenocortical neoplasms. Large tumor size (4/4), extracapsular extension (3/4), blood vessel invasion (2/4), necrosis (4/4), and metastasis (1/4) are features of malignancy for oncocytic adrenocortical carcinomas. The treatment of these tumors is complete surgical excision.- - - - - - - - - - ranking = 0.00035557084542755keywords = bone (Clic here for more details about this article) |
8/49. Adrenal cortical adenoma with adrenalin-type neurosecretory granules clinically mimicking a pheochromocytoma.Adrenal tumors often present with clinical features that are specific and unique to their endocrine metabolism. When these features are in conflict with the pathologic appearance of the tumor, there can be great consternation for both the pathologist and the surgeon. In the case reported herein, an adrenalectomy was performed for clinical features of pheochromocytoma that on gross and histologic examination had the pathologic features of an adrenal cortical adenoma. Electron microscopy subsequently revealed that the tumor cells contained adrenalin-type granules, explaining the clinical outcome. It is crucial for both the surgeon and the surgical pathologist to be aware of this possibility when the clinical and pathologic features of an adrenal tumor are not congruent.- - - - - - - - - - ranking = 0.2keywords = endocrine (Clic here for more details about this article) |
9/49. Adrenal cortical phaeochromocytoma: a case report of a rare entity.Adrenal cortical phaeochromocytomas (pseudo-phaeochromocytomas) are a very rare entity and a diagnostic challenge. Of the few cases previously reported, most have incomplete data or lack clinical and biochemical follow-up documenting the cure of the excess secretion of catecholamines after resection of the tumour. We report herein a 62-year-old patient with clinical and biochemical findings diagnostic of a phaeochromocytoma associated with a 2-cm adrenal mass on CT scan. Surgery revealed the presence of an adrenal cortical adenoma with positive staining for the neuroendocrine marker synaptophysin, but negative for chromogranin, as has been previously reported for these rare cortical phaeochromocytomas. After removal of the tumour the clinical symptoms resolved and biochemical markers normalized, demonstrating the causal relationship between the cortical tumour and the excess production of catecholamines.- - - - - - - - - - ranking = 0.2keywords = endocrine (Clic here for more details about this article) |
10/49. Coexisting corticotroph and lactotroph adenomas: case report with reference to the relationship of corticotropin and prolactin excess.A 57-year-old obese woman with hypertension, diabetes mellitus, osteoporosis, and a 40-year history of secondary amenorrhea was diagnosed with corticotropin-dependent Cushing's syndrome. Dynamic endocrine testing and radiological evaluation did not reveal definitively the source of the excess corticotropin. Bilateral adrenalectomy was performed with resolution of the signs and symptoms of hypercortisolism. Four years later, the patient was noted to have rising serum corticotropin levels and an enlarging pituitary mass; hyperprolactinemia also was documented. A diagnosis of Nelson-Salassa syndrome was made, and she underwent a transsphenoidal adenomectomy. A histological examination of the specimen revealed two distinct, albeit contiguous, adenomas: a corticotroph adenoma and a lactotroph adenoma. Postoperatively, the serum prolactin and corticotropin levels decreased significantly. Although the stalk section effect resulting from compression by a pituitary adenoma can raise serum prolactin levels, a concurrent lactotroph adenoma should be considered in patients with nonfunctional or functional pituitary adenomas of other types associated with significantly elevated prolactin levels. The mechanisms underlying simultaneous adrenocorticotropic hormone and prolactin excess are discussed.- - - - - - - - - - ranking = 0.2keywords = endocrine (Clic here for more details about this article) |
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