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Pituitary acromegaly: not one disease

Acromegaly has traditionally been regarded as a monomorphous disorder resulting from a benign pituitary adenoma. Increasing evidence, however, is highlighting that this disorder is associated with a spectrum of morphologically distinct pituitary tumors with variable clinical, biochemical and radiologic features and differing therapeutic outcomes that are attributed to different genetic and epigenetic changes. These data underscore the need for developing a more refined clinicopathological risk stratification system and implementing personalized targeted therapeutic approaches.
Source: Endocrine-Related Cancer - Category: Endocrinology Authors: Tags: Commentary Source Type: research

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Authors: Brotfain E, Sebbag G, Friger M, Kirshtein B, Borer A, Koyfman L, Frank D, Bichovsky Y, Peiser JG, Klein M Abstract Upper gastrointestinal tract (GIT) surgical procedures are more likely to cause nosocomial Candida peritonitis than lower GIT procedures and they thus constitute an independent risk factor for mortality. Because of the severity of postsurgical fungal infections complications, intensivists and surgeons need to be extremely aware of their clinical importance in critically ill postsurgical intensive care unit (ICU) patients. We analyzed the clinical and microbiological data of 149 oncologic patie...
Source: International Journal of Surgical Oncology - Category: Surgery Tags: Int J Surg Oncol Source Type: research
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Source: The American Journal of Surgical Pathology - Category: Pathology Tags: Special Article Source Type: research
We hypothesized that there is a relationship between the preexisting pleomorphic adenoma [PA]), histologic grade of epithelial-myoepithelial carcinomas (EMCAs), and genetic alterations. EMCAs (n=39) were analyzed for morphologic and molecular evidence of preexisting PA (PLAG1, HMGA2 status by fluorescence in situ hybridization, FISH, and FGFR1-PLAG1 fusion by next-generation sequencing, NGS). Twenty-three EMCAs were further analyzed by NGS for mutations and copy number variation in 50 cancer-related genes. On the basis of combined morphologic and molecular evidence of PA, the following subsets of EMCA emerged: (a) EMCAs wi...
Source: The American Journal of Surgical Pathology - Category: Pathology Tags: Original Articles Source Type: research
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Source: The American Journal of Surgical Pathology - Category: Pathology Tags: Original Articles Source Type: research
AbstractPurposeAcromegaly is a disease associated with an increased risk for several kinds of neoplasms including colon and thyroid cancer. Although the association between acromegaly and pancreatic neoplasms has not been elucidated, it has recently been reported thatGNAS gene mutations were found in 58% of intraductal papillary mucinous neoplasms (IPMNs), which are representative pancreatic cystic lesions, suggesting a link between IPMNs and acromegaly. To assess the prevalence of pancreatic cystic lesions in patients with acromegaly, we performed a retrospective cross-sectional single institute study.MethodsThirty consec...
Source: Pituitary - Category: Endocrinology Source Type: research
Somatic mosaicism has been implicated as a causative mechanism in a number of genetic and genomic disorders. X-linked acrogigantism (XLAG) syndrome is a recently characterized genomic form of pediatric gigantism due to aggressive pituitary tumors that is caused by submicroscopic chromosome Xq26.3 duplications that include GPR101. We studied XLAG syndrome patients (n = 18) to determine if somatic mosaicism contributed to the genomic pathophysiology. Eighteen subjects with XLAG syndrome caused by Xq26.3 duplications were identified using high-definition array comparative genomic hybridization (HD-aCGH). We noted that males w...
Source: Endocrine-Related Cancer - Category: Endocrinology Authors: Tags: Research Source Type: research
X-linked acrogigantism (X-LAG) syndrome is a newly described form of inheritable pituitary gigantism that begins in early childhood and is usually associated with markedly elevated GH and prolactin secretion by mixed pituitary adenomas/hyperplasia. Microduplications on chromosome Xq26.3 including the GPR101 gene cause X-LAG syndrome. In individual cases random GHRH levels have been elevated. We performed a series of hormonal profiles in a young female sporadic X-LAG syndrome patient and subsequently undertook in vitro studies of primary pituitary tumor culture following neurosurgical resection. The patient demonstrated con...
Source: Endocrine-Related Cancer - Category: Endocrinology Authors: Tags: Research Source Type: research
We report the case of a 40-year-old male without significant family history of endocrine disease who was found to have primary hyperparathyroidism, a pituitary tumor causing acromegaly, thyroid cancer, renal cell carcinoma, and pancreatic cysts. We posit that this represents a new version of MEN-4. While renal tumors (angiomyolipoma) have been reported as part of the MEN-4 phenotype, to our knowledge, this is the first case reported of the association of MEN-1 and/or MEN-4 phenotype with this unique constellation of tumors, including renal cell carcinoma. Interestingly, this patient tested negative (DNA sequencing/deletion...
Source: Cardiorenal Medicine - Category: Urology & Nephrology Source Type: research
More News: Acromegaly | Cancer | Cancer & Oncology | Endocrinology | Genetics | Pituitary Tumor