Grading system may predict recurrence, progression of pituitary neuroendocrine tumors

The risk for recurrence or progression of pituitary neuroendocrine tumors in adults is significantly associated with age and tumor type, according to findings published in The Journal of Clinical Endocrinology & Metabolism.

Gérald Raverot, MD, PhD, of Hospices Civils de Lyon, Federation d’Endocrinologie du Pole Est in France, and colleagues evaluated 374 adults (194 women) who underwent surgery for a pituitary neuroendocrine tumor (mean age at surgery, 51.9 years) between February 2007 and October 2012 to test the value of a new classification system on prognostic relevance.

Tumors were classified using a grading system based on invasion on MRI, immunocytochemical profile, Ki-67 mitotic index and p53 positivity. Noninvasive tumors were classified as grade 1a, noninvasive but proliferative tumors were grade 1b, invasive tumors were grade 2a, invasive and proliferative tumors were grade 2b and metastatic tumors were grade 3.

Macroadenomas were the most common type of tumor based on MRI classification (82.1%), followed by microadenoma (13.6%) and giant adenoma (4.3%).

Information on grade was available for 365 tumors; grade 1a was the most common (51.2%), followed by grades 2a (32.3%), 2b (8.8%) and 1b (7.7%).

The progression-free survival analysis included 213 participants from the original cohort during a mean follow-up of 3.5 years. A recurrent event occurred in 52 participants, and progression occurred in 37 participants. The risk for recurrence and/or progression was associated with age (P = .035), tumor type (P = .028) and grade (P < .001). The risk for recurrence and/or progression was increased with grade 2b tumors compared with grade 1a tumors (HR = 3.72; 95% CI, 1.9-7.26) regardless of tumor type. Invasion was significantly associated with recurrence in grade 2a tumors (HR = 2.98; 95% CI, 1.89-4.7), whereas proliferation was not significantly associated with prognosis for grade 1b (HR = 1.25; 95% CI, 0.73-2.13).

“This prospective study confirms the usefulness of our previously proposed classification and may now allow clinicians to adapt their therapeutic strategies according to prognosis, but may also be used to stratify patients and evaluate therapeutic efficacy in future clinical trials,” the researchers wrote. “Further progress can be expected, in particular if an improved understanding of molecular abnormalities associated with pituitary tumorigenesis generates better biomarkers.” – by Amber Cox

Disclosures: The researchers report no relevant financial disclosures.

From https://www.healio.com/endocrinology/neuroendocrinology/news/in-the-journals/%7B4dbd524c-4534-42e3-a1dc-4e3a0d82a0f1%7D/grading-system-may-predict-recurrence-progression-of-pituitary-neuroendocrine-tumors

Rare neuroendocrine tumours may be misdiagnosed as Cushing’s disease

By Eleanor McDermid, Senior medwireNews Reporter

Ectopic tumours secreting corticotropin-releasing hormone (CRH) and adrenocorticotropic hormone (ACTH) are very rare in children and can result in a misdiagnosis of Cushing’s disease (CD), say researchers.

Three of the patients in the reported case series had pituitary hyperplasia and underwent transsphenoidal surgery for apparent CD before the tumour that was actually causing their symptoms was located. The hyperplasia was probably caused by release of CRH from the ectopic tumour, which stimulated the pituitary gland, giving the impression of an ACTH-secreting pituitary adenoma, explain Maya Lodish (National Institutes of Health, Bethesda, Maryland, USA) and study co-authors.

These three patients were part of a series of seven, which Lodish et al describe as “a relatively large number of patients, considering the infrequency of this disease.”

The patients were aged between 1.8 and 21.3 years. Three had neuroendocrine tumours located in the pancreas ranging in size from 1.4 to 7.0 cm, two had thymic carcinoids ranging from 6.0 mm to 11.5 cm, one patient had a 12.0 cm tumour in the liver and one had a 1.3 cm bronchogenic carcinoid tumour of the right pulmonary lobe.

Four of the patients had metastatic disease and, during up to 57 months of follow-up, three died of metastatic disease or associated complications and two patients had recurrent disease.

“Our series demonstrates that these are aggressive tumors with a high mortality rate,” write the researchers in the Journal of Clinical Endocrinology & Metabolism. “It is important to follow the appropriate work up, regarding both biochemical and imaging tests, which can lead to the correct diagnosis and to the most beneficial therapeutic approach.”

The team found the CRH stimulation test to be helpful, noting, for example, that none of the patients had a rise in cortisol that was consistent with CD, with all patients showing smaller responses ranging from 2% to 15%. Likewise, just one patient had an ACTH rise higher than 35% on CRH administration, and four patients had a “flat” response, which has previously been associated with ectopic neuroendocrine tumours.

Of note, six patients had normal or high plasma CRH levels, despite all having high cortisol levels, which would be expected to result in undetectable plasma CRH due to negative feedback, implying another source of CRH production. Five patients had blunted diurnal variation of both cortisol and ACTH levels consistent with Cushing’s syndrome.

The patients also underwent a variety of imaging procedures to identify the source of ACTH/CRH production, some of which, such as octreotide scans, are specialist and not available in most hospitals, the researchers note, potentially contributing to inappropriate diagnosis and management.

From http://www.news-medical.net/news/20141030/Rare-neuroendocrine-tumours-may-be-misdiagnosed-as-Cushinge28099s-disease.aspx

%d bloggers like this: