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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pituitary carcinomas, currently defined as primary adenohypophyseal neoplasms with evidence of either brain invasion or metastatic spread, are exceptionally rare. A case of corticotroph pituitary carcinoma is reported. A 17-year-old female first presented with an invasive and clinically non-functioning pituitary macroadenoma. The primary pituitary lesion lacked atypical histological features and retrospective immunohistochemical studies confirmed its corticotrophic nature. Repeated episodes of local recurrence followed together with the acquisition of severe Cushing's disease. Local disease control was not obtained despite repeated surgical decompression and courses of radiation therapy. Systemic dissemination with multiple bone metastases became manifest eleven years after the first presentation. Atypical histological features and Crooke's hyaline change were evident in both the recurrent and metastatic disease. She remains alive at last follow-up with severe complications relating to persisting sellar and metastatic disease and ongoing endocrine dysfunction. A further 32 cases of pituitary corticotroph carcinoma reported in English are reviewed to highlight the clinicopathological features of this rare form of pituitary neoplasm. Difficulties associated with the diagnosis and management of pituitary carcinoma are also discussed.
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PMID:Corticotroph pituitary carcinoma: case report and literature review. 1174 54

To understand the relationship between pituitary adenoma and carcinoma, four adrenocorticotropic hormone-producing pituitary adenomas and corresponding metastatic carcinomas were studied. All were functional macroadenomas (three cases of Nelson syndrome and one of Cushing disease) that initially invaded the sella turcica and occurred in women ranging in age from 17 to 66 years (mean 45 years). Metastases (two craniospinal and two systemic) occurred after latency periods of 6 to 13 years. Histological specimens were immunostained for pituitary hormones, Ki-67 antigen (MIB-1), p53 and p27 proteins, D-type cyclins, and glucocorticoid receptor messenger (m)RNA. The DNA content of the specimens was assessed using Feulgen stain. Reactivities were quantified by digital image analysis. Primary/recurrent lesions and metastatic tumors differed according to their respective mean mitotic indices (1.2/10 hpf compared with 4.3/10 hpf), MIB-1 labeling (1.7% compared with 8%), p53 staining (37.3% compared with 49.9%), and p27 labeling (48% compared with 25%). Cyclin D, immunoreactivity provided no prognostically significant information. Glucocorticoid receptor mRNA was detected in all cases. Results of a ploidy analysis were variable and nonprognostic. In keeping with the 2000 World Health Organization classification of endocrine neoplasms, our findings support the concept that primary tumors that exhibit mitotic activity, an increased (> 3%) MIB-1 labeling index, and/or p53 immunoreactivity should be termed "atypical adenomas" to denote their aggressive potential and the possibility of future malignant transformation.
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PMID:Corticotroph carcinoma of the pituitary: a clinicopathological study. Report of four cases. 1183 11

A 48-yr-old woman was evaluated 21 yr after receiving treatment for an ACTH-secreting metastatic pituitary carcinoma. She had been diagnosed with Cushing's disease 35 yr earlier at the age of 14 yr and had undergone bilateral adrenalectomy. Six years later she developed Nelson's syndrome, which was treated with resection of a pituitary adenoma followed by radiotherapy to the sella turcica. Eight years later she was found to have craniospinal metastases with three remote intracerebral lesions. Two of these lesions were surgically resected and stained positive for ACTH by immunofluorescence. She subsequently received whole-brain radiotherapy and is doing well 21 yr later with no lesions seen on magnetic resonance imaging and no evidence of recurrent metastatic disease. We present this case in detail along with a literature review of ACTH-secreting pituitary carcinoma.
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PMID:Long-term survival with ACTH-secreting carcinoma of the pituitary: a case report and review of the literature. 1210 5

We report five silent corticotroph carcinomas of the pituitary gland. They represent 0.05% of adenohypophyseal tumors surgically treated at Mayo Clinic during a 20-year period and about 5% of all reported pituitary carcinomas. The patients (three females and two males), ranging in age from 26 to 58 years (mean 39 years, median 35 years) presented with symptoms of mass effect; none had Cushing's disease. All tumors were initially invasive macroadenomas, recurred locally, and metastasized, three outside the central nervous system. The follow-up period ranged from 2 to 23 years (mean 10.6 years). All patients died, four of disseminated tumor and one of myocardial infarction. Histologically, three of the primary lesions were indistinguishable from an ordinary benign adenoma. Two were initially diagnosed as atypical adenomas as they featured nuclear pleomorphism, prominent nucleoli, mitotic activity, high MIB-1 labeling indices, and p53 overexpression. For the purpose of comparison, 17 silent corticotroph adenomas were also investigated. In addition, the clinicopathologic features of the silent carcinomas were compared with those of a meta-analysis of published Cushing's disease-associated pituitary carcinomas. The silent adrenocorticotropin carcinomas showed a propensity for extraneural dissemination and an outcome similar to those of the Cushing's disease-associated carcinomas. The two patients with initial atypical tumors died with metastases outside the central nervous system at 2 and 4 years, whereas the three patients with tumors lacking atypia died 16, 18, and 23 years after initial sellar surgery.
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PMID:Silent corticotroph carcinoma of the adenohypophysis: a report of five cases. 1265 32

Adrenal masses are one of the most common tumors in humans. They are a very heterogenous group of diseases and include benign and malignant adrenocortical lesions, metastases, pheochromocytomas and other entities. Adrenal masses originating from steroidogenic or chromaffin cells may be silent or the source of subclinical or overt hormone excess, such as primary aldosteronism, hypercortisolism or symptomatic catecholamine excess. On the other hand, adrenal hyperplasia may be the result of excess ACTH secretion in steroid biosynthesis disorders with deficient glucocorticoid secretion, in glucocorticoid resistance, in Cushing's disease, or ectopic ACTH syndrome. Algorithms for endocrine testing, imaging studies and their combination are available for defining the tumor entity and for the characterization of the hormone excess syndromes. Recent developments in molecular biology have provided tools for testing for hereditary tumor syndromes associated with adrenal tumorigenesis and to establish strategies for further treatment and follow-up.
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PMID:[Adrenal gland tumors]. 1768 15

The aim of this study was to analyze feasibility and outcomes of laparoscopic adrenalectomy (LA). Pathology, size and bilateral site of lesions were considered. Between December 1998 and May 2007 in our institution a total of 68 patients of mean age of 53 years underwent unilateral (n=57) or bilateral (n=11) LA. Adrenal masses averaged 5.4cm in size (range 1.2-13cm) and 56.7g in weight (range 10-265) including 71 benign and 8 malignant lesions. A total of 79 adrenal glands were resected, 44 right sided and 35 left sided. Removal was complete in 77 cases and partial (sparing adrenalectomy) in 1 patient affected by bilateral pheochomocytoma. Three left adrenalectomies for pheochromocytoma were robot-assisted. The transperitoneal lateral approach was preferred and the posterior retroperitoneal approach was adopted in 5 patients. The mean duration of surgery for each LA was 138+/-90min and 3.8 trocar were used on average (range 3-6). Conversion was needed in 3 cases owing to difficult dissection of large masses. Estimated mean blood loss for each LA was 95+/-30ml and it was greater for bilateral LA. Mortality was nil and morbidity was 5.8%. The average length of hospital stay (LOS) in surgical unit was 4+/-2.4 days (range 2-8). Patients affected by hormone secreting or bilateral lesions, by unilateral or bilateral pheochromocytoma and by bilateral Cushing's disease were transferred to the endocrinological ward so that their overall hospital stay was prolonged to 9+/-2.8 days on average (range 7-17). Mean duration of follow-up of patients was 38 months (range 2-100) and demonstrated acceptable endocrine results. Three primary cortical carcinomas were discovered as chance findings on histologic examination. While long-term results after LA for cortical carcinomas were poor and LA is not recommended in such cases, long-term results after LA for adrenal metastases were encouraging.
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PMID:Outcomes of laparoscopic adrenalectomy. Clinical experience with 68 patients. 1794 73

Corticotroph pituitary carcinomas are tumors, defined by the presence of distant metastases that determine their poor prognosis. The diagnosis and therapy of malignant corticotroph adenomas remains a clinical challenge. The molecular mechanisms of malignant transformation of pituitary adenomas are unclear, although they are believed to arise in an adenoma-to-carcinoma sequence. We describe two cases of malignant Cushing's disease with metastases in liver and bone, respectively. The primary pituitary tumors were treated by a combination of radiotherapy and transsphenoidal surgery, but recurred several times in both patients. The time interval between the diagnosis of Cushing's disease and the discovery of metastases was 32 and 17 years, respectively. In the first case the patient died within 6 months after diagnosis of metastasis, whereas the second patient is alive at a follow-up of 2 years after the discovery of the metastasis. Furthermore, we reviewed all available cases of corticotroph pituitary carcinomas reported in the literature and analyzed their clinical features and therapeutical management. In conclusion, frequent relapses of Cushing's disease, aggressive growth of macroadenoma, Nelson's syndrome after adrenalectomy or persistently high ACTH levels should prompt the clinician to consider the possibility of pituitary corticotroph carcinomas.
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PMID:Malignant pituitary corticotroph adenomas: report of two cases and a comprehensive review of the literature. 1817 44

Silent corticotroph pituitary adenomas (SCA) are defined as pituitary adenomas showing positive staining for adrenocorticotrophic hormone in immunohistochemical studies, but not associated with perioperative clinical or laboratory features of hypercortisolaemia. They account for 1.1-6% of surgically removed pituitary adenomas. Currently, two distinct pathologic subtypes of SCA are recognised. Their pathogenesis remains unclear. They present with local mass effects (headache, visual deterioration, cranial nerve palsies, endocrine dysfunction). The lack of manifestations of cortisol excess has not been conclusively explained. In surgical series, most tumours are macroadenomas with suprasellar extension present in 87-100% of the cases; this is in contrast to Cushing's disease, which is mostly attributed to microadenomas. Surgery remains the main therapeutic approach. Attempts to identify predictors of recurrence have not been successful. Management and follow-up protocols should be planned taking into account their potential aggressive behaviour, particularly upon recurrence. The development of florid pituitary Cushing's syndrome and local recurrence followed by metastatic disease (occasionally outside the central nervous system) have been rarely reported.
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PMID:Silent corticotroph adenomas. 1820 69

Gastrinoma tissue has been found frequently in lymph nodes located near the duodenum without a known primary tumor. Therefore, it has been suggested that a primary lymph node gastrinoma exists. We report on a 38-year-old woman suffering from multiple endocrine neoplasia type 1 (MEN1) confirmed by menin gene mutation analysis. MEN1 disease started with primary hyperparathyroidism followed by Cushing disease, the detection of tumors of the pituitary, adrenal cortex, and the pancreas and also an elevated serum gastrin level. An octreotide scan revealed 4 tumors in the upper abdomen. A selective arterial calcium stimulation test located the source of the hypergastrinemia to the area of the gastroduodenal and the superior mesenteric arteries. Total pancreatoduodenectomy was performed and conventional histopathologic examination revealed a well-differentiated cystic neuroendocrine tumor of the pancreas expressing glucagon and accompanied by several microadenomas. In addition, 3 suprapancreatic lymph nodes with gastrin-positive endocrine tissue were found. None of the pancreatic microadenomas expressed gastrin and no duodenal endocrine tumor was found despite careful macroscopic examination. Only after complete embedding of the duodenal and pancreatic tissue in 65 paraffin blocks, 2 microgastrinomas (0.45 and 0.8 mm in diameter) were identified in the duodenum. It is concluded that duodenal gastrinomas that give rise to lymph node metastases may be so tiny that they are easily overlooked in a routine examination and that systematic tissue monitoring is required to identify them.
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PMID:Primary lymph node gastrinoma or occult duodenal microgastrinoma with lymph node metastases in a MEN1 patient: the need for a systematic search for the primary tumor. 1852 Apr 36

Adrenocortical cancer (ACC) is a rare, challenging disease with a broad range of clinical presentations. Often presenting in an advanced stage with a large, locally invasive primary tumor or with Cushing's syndrome, it requires a multidisciplinary approach to treatment. We discuss controversies in the evaluation and management of ACC. We conclude that the role of (18)F-fluorodeoxyglucose positron emission tomography (FDG-PET) remains unclear and that it should be employed sparingly. Biopsies should be performed only when metastatic disease is present and a primary tumor has not been clearly established. Care should be taken in using the Weiss criteria to make decisions regarding prognosis. Surgery is the preferred intervention initially and at the time of recurrence, and every effort should be made to attempt a surgical resection. The latter should be an open resection; laparoscopic resection should not be performed if there is a high suspicion of ACC. The use of mitotane in patients without evidence of disease remains controversial. Systemic chemotherapy is effective in patients with widely metastatic disease or as an adjunct to a surgical intervention and should focus on regimens that have been shown to effect responses; "novel targeted therapies" should not be employed as first-line treatment. Radiofrequency ablation (RFA) and cryoablation can be very helpful but are still under evaluation; embolization with or without chemotherapy may be used as a surgical adjunct. The role of radiation remains to be defined. Finally, physicians caring for these patients need to recognize that Cushing's disease is a debilitating problem that should be managed aggressively; expecting chemotherapy to solve this complication is not appropriate.
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PMID:Practical considerations in the evaluation and management of adrenocortical cancer. 2116 80


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