scholarly journals An Update on Pituitary Neuroendocrine Tumors Leading to Acromegaly and Gigantism

2021 ◽  
Vol 10 (11) ◽  
pp. 2254
Author(s):  
Sylvia L. Asa ◽  
Shereen Ezzat

An excess of growth hormone (GH) results in accelerated growth and in childhood, the clinical manifestation is gigantism. When GH excess has its onset after epiphyseal fusion at puberty, the overgrowth of soft tissue and bone results in acromegaly. Persistent GH excess in gigantism also causes acromegalic features that become evident in the adult years. The causes of GH excess are primarily lesions in the pituitary, which is the main source of GH. In this review, we provide an update on the clinical, radiological and pathologic features of the various types of pituitary neuroendocrine tumors (PitNETs) that produce GH. These tumors are all derived from PIT1-lineage cells. Those composed of somatotrophs may be densely granulated, resembling normal somatotrophs, or sparsely granulated with unusual fibrous bodies. Those composed of mammosomatotrophs also produce prolactin; rare plurihormonal tumors composed of cells that resemble mammosomatotrophs also produce TSH. Some PitNETs are composed of immature PIT1-lineage cells that do not resemble differentiated somatotrophs, mammosomatotrophs, lactotroph or thyrotrophs; these tumors may cause GH excess. An unusual oncocytic PIT1-lineage tumor known as the acidophil stem cell tumor is predominantly a lactotroph tumor but may express GH. Immature PIT1-lineage cells that express variable amounts of hormones alone or in combination can sometimes cause GH excess. Unusual tumors that do not follow normal lineage differentiation may also secrete GH. Exceptional examples of acromegaly/gigantism are caused by sellar tumors composed of hypothalamic GHRH-producing neurons, alone or associated with a sparsely granulated somatotroph tumor. Each of these various tumors has distinct clinical, biochemical and radiological features. Data from careful studies based on morphologic subtyping indicate that morphologic classification has both prognostic and predictive value.

1985 ◽  
Vol 312 (1) ◽  
pp. 9-17 ◽  
Author(s):  
Shlomo Melmed ◽  
Calvin Ezrin ◽  
Kalman Kovacs ◽  
Robert S. Goodman ◽  
Lawrence A. Frohman

2008 ◽  
Vol 132 (7) ◽  
pp. 1055-1061 ◽  
Author(s):  
Teri J. Franks ◽  
Jeffrey R. Galvin

Abstract Context.—Tumors with neuroendocrine morphology are a distinct subset of lung neoplasms sharing characteristic histologic, immunohistochemical, ultrastructural, and molecular features. Objective.—To review the current histologic classification and the diagnostic criteria for the major categories of neuroendocrine tumors of the lung. Data Sources.—Published classification systems from the World Health Organization and pertinent peer-reviewed articles indexed in PubMed (National Library of Medicine) form the basis of this review. Conclusions.—Accurate classification of the neuroendocrine tumors of the lung requires knowledge of specific criteria separating the major categories, which is essential for determining prognosis and treatment.


Oncogene ◽  
2000 ◽  
Vol 19 (15) ◽  
pp. 1875-1884 ◽  
Author(s):  
L T Teixeira ◽  
H Kiyokawa ◽  
X D Peng ◽  
K T Christov ◽  
L A Frohman ◽  
...  

2011 ◽  
Vol 135 (1) ◽  
pp. 143-146 ◽  
Author(s):  
Joshua Anspach Hanson ◽  
Abiy B. Ambaye

Abstract Adult testicular granulosa cell tumors are rare sex cord–stromal tumors of which only 28 have been previously reported. As compared with their ovarian counterparts, these tumors may follow a more aggressive course because the proportion of malignant cases is higher. To date, there are no clinical or pathologic features that definitively predict malignancy. We reviewed all prior case reports for features that may predict their malignant potential. Tumor size greater than 5.0 cm is the only feature statistically associated with malignancy. Mitotic count, tumor necrosis, patient age, and the presence of gynecomastia do not, at present, predict benign versus malignant behavior.


2019 ◽  
Vol 12 ◽  
pp. 263177451984494
Author(s):  
Tania Franceschini ◽  
Deborah Malvi ◽  
Lorenzo Maroni ◽  
Matteo Ravaioli ◽  
Matteo Cescon ◽  
...  

We describe three cases of liver lesions, characterized by a discrepancy between presurgical imaging and histological features, in which the final histological diagnosis was quite different from what the surgeons expected. We present (1) a case of primary liver angiomyolipoma associated with focal nodular hyperplasia, (2) a case of perivascular epithelioid cell tumor, and (3) a case of liver splenosis associated with focal nodular hyperplasia. In all cases, a presurgical diagnosis of hepatocellular adenoma was made. Due to nonspecific clinical and radiological features, these rare liver lesions are often presurgically misdiagnosed, especially in young noncirrhotic patients. The association among different lesions represents one additional diagnostic challenge.


1965 ◽  
Vol 209 (3) ◽  
pp. 632-636 ◽  
Author(s):  
George L. Wolff

Daily injection of 0.05 USPU of ovine growth hormone had no effect on greater fat deposition in yellow dwarf ( Ayadwdw) mice of either sex as compared with nonyellow dwarf mice ( aadwdw). No differences between the accelerated growth rates of both genotypes during the injection period were noted nor were there any marked differences in the increase in tail length between the genotypes. Yellow dwarf mice continued gaining weight more rapidly than nonyellow dwarf mice after the end of the injection period. Analysis of the fat and water content of the carcasses indicates that the greater rate of weight gain of yellow dwarf mice is due almost entirely to increased fat deposition. The results of this experiment suggest that the obesity of yellow mice is not due to deficiencies of growth hormone or thyrotrophin.


2007 ◽  
Vol 86 (5) ◽  
pp. 463-468 ◽  
Author(s):  
J.R. Smid ◽  
J.E. Rowland ◽  
W.G. Young ◽  
K.T. Coschigano ◽  
J.J. Kopchick ◽  
...  

Growth hormone (GH) status affects dental development, but how GH influences tooth size/shape is unclear. Since GH affects dental epithelial proliferation, we hypothesized that GH influences the tooth crown and root dimensions. Dentin matrix dimensions were measured in longitudinal sections of decalcified first mandibular molars from 3 genetically modified mice: giant (GH-Excess) mice and dwarf (GH-Antagonist and GH-Receptor-Knockout) mice. GH status was found to influence crown width, root length, and dentin thickness. Analysis of these data suggests that GH influences both tooth crown and root development prior to dentinogenesis as well as during appositional growth of dentin. This is concordant with the expression of paracrine GH and GH receptors during tooth bud morphogenesis, and of GH receptors in the enamel organ, dental papilla, and Hertwig’s epithelial root sheath during dentinogenesis. Based on prior studies, these GH morphogenetic actions may be mediated by the induction of both bone morphogenetic protein and insulin-like growth factor-1 expression.


2011 ◽  
Vol 29 (4_suppl) ◽  
pp. 325-325
Author(s):  
T. M. Zagar ◽  
R. R. White ◽  
C. G. Willett ◽  
P. Papavassiliou ◽  
D. S. Tyler ◽  
...  

325 Background: Pancreatic neuroendocrine tumors (NET) are rare with improved prognosis compared to adenocarcinomas. Surgical resection remains the standard of care although many patients present with unresectable/metastatic disease. While many resected patients will fail distantly, little is known regarding the use of adjuvant radiotherapy. To define this and establish specific patterns of failure, an analysis of resected patients from a single institution was performed. Methods: From 1994 to 2009, 33 patients with NET of the pancreatic head underwent resection with curative intent at Duke University. Sixteen patients were treated with surgical resection alone, and an additional 17 underwent resection with adjuvant (n=10) or neoadjuvant (n=7) radiation therapy, usually with concurrent fluoropyrimidine-based chemotherapy (CMT). Median radiation dose was 50.4 Gy and median follow-up 28 months. Results: Patients receiving radiation therapy were more likely to have involved nodes (47% vs 19%, p=0.09), more mitoses per high power field (p=0.10) and involved margins (47% vs 31%, p=0.20) compared to surgery alone patients. Median survival for the whole cohort was 52 months. Two-year survival was 68% for the CMT group and 93% for the surgery alone group (p=0.03). Two-year local control was 85% for the CMT and 90% for the surgery group (p=0.49). Two-year metastasis-free survival was 45% and 69% for the CMT and surgery patients, respectively (p=0.02). Conclusions: Patients receiving CMT were more likely to have adverse pathologic features compared to surgery-alone patients. Survival outcomes were high in both groups, although less so in the CMT group. Distant metastasis development dominated patterns of failure. Local failure following resection of NETs is uncommon, and the role of adjuvant radiotherapy in this setting remains unclear. No significant financial relationships to disclose.


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