EPID-14. EPIDEMIOLOGY OF ADULT PITUITARY TUMORS IN THE U.S. ACCORDING TO THE 2017 WHO CLASSIFICATION OF ENDOCRINOLOGY TUMOURS

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi88-vi89
Author(s):  
Luz Castellanos ◽  
Catherine Gutierrez ◽  
Timothy Smith ◽  
Bryan Iorgulescu
Keyword(s):  
Tumor Size ◽  
Pituitary Tumor ◽  
Who Classification ◽  
Pituitary Tumors ◽  
Histopathological Diagnosis ◽  
Intracranial Tumors ◽  
Tumor Types ◽  
The U.S ◽  
Pituitary Carcinomas

Abstract INTRODUCTION Among adult intracranial tumors, the pituitary represents a frequent site of origin. We examine their contemporary epidemiology with a particular focus on uncommon pituitary tumor types. METHODS Adult patients presenting with pituitary or sellar tumors between 2004-2017 were identified from the U.S. National Cancer Database (comprising >70% of newly-diagnosed cancers). Their epidemiology was assessed in the context of the 2017 WHO Classification of Endocrine Tumours. RESULTS 12.5% of adult intracranial tumors arose in the pituitary region. 113,352 adults with pituitary tumors were identified. Histopathological diagnosis was obtained in only 59% of cases, in which 93% were pituitary adenomas and 6% were craniopharyngiomas. Among craniopharyngiomas, 71% were adamantinomatous and 29% were papillary, between which there were no differences in age, sex, or tumor size—however, papillary craniopharyngiomas were less common among Black nonHispanic patients (p< 0.001). Among the remaining 1% (n=680) of pituitary tumors, posterior pituitary tumors comprised 21%, chordomas 16%, meningiomas 15%, pituitary carcinomas 11%, GCTs 10%, hematolymphoid 8%, other mesenchymal and stromal 7%, neuronal/paraneuronal 6%, and schwannoma 4%. Meningiomas (84%), mesenchymal/stromal (64%), and neuronal/paraneuronal (64%) tumors displayed a female predominance, whereas GCTs (75%) and pituitary carcinomas (62%) exhibited a male predominance. Age at diagnosis, tumor size, and race/ethnicity varied widely across uncommon tumor types. We further examined the subtypes of uncommon pituitary tumors: for sellar chordomas, 19% were chordoid and none were dedifferentiated; for sellar meningiomas, 94% were grade I; for pituitary GCTs, 79% were pure germinomas; for hematolymphoid, 52% were DLBCL, 11% were plasmacytomas, and 9% were Langerhans cell histiocytosis; for neuronal/paraneuronal, 64% were gangliocytomas; and for mesenchymal tumors, 53% were vascular and 16% were SFTs/HPCs. CONCLUSIONS Using national registry data, we provide a detailed dissection of the epidemiology of adult pituitary tumors, with a particular focus on examining uncommon pituitary tumor types in the context of WHO2017.

scholarly journals The Progress of Immunotherapy in Refractory Pituitary Adenomas and Pituitary Carcinomas

2020 ◽  
Vol 11 ◽  
Author(s):  
Congxin Dai ◽  
Siyu Liang ◽  
Bowen Sun ◽  
Jun Kang
Keyword(s):  
Clinical Trial ◽  
Cd8 T Cells ◽  
Tumor Size ◽  
Clinical Studies ◽  
Alternative Therapy ◽  
Pituitary Tumors ◽  
Immune Microenvironment ◽  
Promising Alternative ◽  
Tumor Immune Microenvironment ◽  
Pituitary Carcinomas

Most pituitary adenomas (PAs) are considered benign tumors, but approximately 0.2% can present metastasis and are classified as pituitary carcinomas (PCs). Refractory PAs lie between benign adenomas and true malignant PC and are defined as aggressive-invasive PAs characterized by a high Ki-67 index, rapid growth, frequent recurrence, and resistance to conventional treatments, including temozolomide. It is notoriously difficult to manage refractory PAs and PC because of the limited therapeutic options. As a promising therapeutic approach, cancer immunotherapy has been experimentally used for the treatment of many tumors, including pituitary tumors. The purpose of this review is to report the progress of immunotherapy in pituitary tumors, including refractory PAs and PCs. The tumor immune microenvironment has been recognized as a key contributor to tumorigenesis, progression, and prognosis. One study indicated that the number of CD68+ macrophages was positively correlated with tumor size and Knosp classification grade for tumor invasiveness. The infiltration of CD4+ and CD8+ T cells was relatively scant in these adenomas, but pituitary growth hormone (GH) adenomas exhibited significantly more CD4+ and CD8+ T cells than non-GH adenomas. These results suggest an association of CD68+ macrophage infiltration with an increase in pituitary tumor size and invasiveness. Another study suggested that a lower number of CD8+ lymphocytes is associated with cavernous sinus invasion and resistance to treatment with first-generation somatostatin analogs in acromegaly patients, highlighting a potential role of the tumor immune microenvironment in determining the prognosis of somatotroph pituitary tumors. Preclinical studies have indicated that widely varying degrees of programmed death-ligand 1 (PD-L1) expression and tumor-infiltrating lymphocytes (TILs) are found among different subtypes. Functional PAs and aggressive PAs express significantly higher levels of PD-L1 and TILs than other subtypes, indicating that PD-1 blockade might be a promising alternative therapy for patients with aggressive PAs. PD-L1 transcript and protein levels were found to be significantly increased in functioning (GH and prolactin-expressing) pituitary tumors compared to nonfunctioning (null cell and silent gonadotroph) adenomas. Moreover, primary pituitary tumors harbored higher levels of PD-L1 mRNA than recurrent tumors. These findings suggest the possibility of considering checkpoint blockade immunotherapy for functioning pituitary tumors refractory to conventional management. Animal models of Cushing’s disease also demonstrated PD-L1 and TIL expression in cultured tumors and murine models, as well as the effectiveness of checkpoint blockade therapy in reducing the tumor mass, decreasing hormone secretion, and increasing the survival rate. Clinical studies show that immunotherapy may be an effective treatment in patients with pituitary tumors. One corticotroph carcinoma patient showed a significant reduction in hormone levels and shrinkage of the tumor size of primary and metastatic lesions immediately after investigational treatment with ipilimumab and nivolumab. However, another patient with corticotroph adenoma progressed rapidly after four cycles of anti-PD-1 (pembrolizumab) treatment. To date, there are two registered clinical trials of immunotherapy for pituitary tumors. One of them is the phase II clinical trial of nivolumab combined with ipilimumab for patients with aggressive pituitary tumors (NCT04042753). The other one is also a phase II clinical trial of the combination of nivolumab and ipilimumab for rare tumors, including pituitary tumors (NCT02834013). Both clinical trials are in the stage of recruiting patients and have not been completed. In summary, the results from preclinical research and clinical studies indicated that immunotherapy might be a promising alternative therapy for PCs and refractory PAs resistant to conventional treatments. The combination of immunotherapy and radiotherapy or temozolomide may have synergistic effects compared to a single treatment. More preclinical and clinical studies are needed to further indicate the exact efficacy of immunotherapy in pituitary tumors.

The new WHO classification of human pituitary tumors: comments

2005 ◽  
Vol 111 (1) ◽  
pp. 71-72 ◽  
Author(s):  
Dominique Figarella-Branger ◽  
Jacqueline Trouillas
Keyword(s):  
Who Classification ◽  
Pituitary Tumors ◽  
Human Pituitary

Indeterminacy in the WHO classification of tumors: an example of the histopathological diagnosis of brain tumors

2011 ◽  
Vol 28 (3) ◽  
pp. 247-251 ◽  
Author(s):  
Gaku Tanaka ◽  
Yoichi Nakazato ◽  
Tarou Irié ◽  
Tatsuya Okada ◽  
Masafumi Abe
Keyword(s):  
Brain Tumors ◽  
Who Classification ◽  
Histopathological Diagnosis ◽  
Classification Of Tumors

The new WHO classification of pituitary tumors: highlights and areas of controversy

2005 ◽  
Vol 111 (1) ◽  
pp. 80-81 ◽  
Author(s):  
Edward R. Laws ◽  
M. Beatriz S. Lopes
Keyword(s):  
Who Classification ◽  
Pituitary Tumors

scholarly journals Pituitary carcinoma: a review of the literature

2004 ◽  
Vol 16 (4) ◽  
pp. 1-9 ◽  
Author(s):  
Brian T. Ragel ◽  
William T. Couldwell
Keyword(s):  
Pituitary Tumor ◽  
English Literature ◽  
Treatment Options ◽  
Latency Period ◽  
Pituitary Tumors ◽  
Distant Metastases ◽  
Pituitary Carcinoma ◽  
Future Studies ◽  
Additional Surgery ◽  
Pituitary Carcinomas

Pituitary carcinomas, defined as distant metastases of a pituitary neoplasm, are rare; fewer than 140 reports exist in the English literature. The initial presenting pituitary tumor is usually a secreting, invasive macroadenoma, with adrenocorticotropic hormone (ACTH)– and prolactin (PRL)–secreting tumors being the most common. The latency period between the diagnosis of a pituitary tumor and the diagnosis of a pituitary carcinoma is 9.5 years for ACTH-producing lesions and 4.7 years for PRL-secreting tumors. Survival after documentation of metastatic disease is poor; 66% of patients die within 1 year. Treatment options include additional surgery, radiotherapy, and chemotherapy, all of which are associated with poor results. Future studies will focus on identifying those invasive pituitary tumors most likely to metastasize and treating them aggressively before they progress to pituitary carcinomas.

Overview of the 2017 WHO Classification of Pituitary Tumors

2017 ◽  
Vol 28 (3) ◽  
pp. 228-243 ◽  
Author(s):  
Ozgur Mete ◽  
M. Beatriz Lopes
Keyword(s):  
Who Classification ◽  
Pituitary Tumors

scholarly journals MPC-17 2021 WHO Classification of Tumors of the CNS, 5th ed

2021 ◽  
Vol 3 (Supplement_6) ◽  
pp. vi18-vi18
Author(s):  
Takashi Komori
Keyword(s):  
Molecular Markers ◽  
Risk Stratification ◽  
Who Classification ◽  
World Health ◽  
Grading System ◽  
Low Grade ◽  
Integrated Diagnosis ◽  
Classification Of Tumors ◽  
Tumor Types

Abstract The grading of gliomas based on histological features has been a subject of debate for several decades. While the traditional grading system has failed to stratify the risk of IDH-mutant astrocytoma, canonical histological and proliferative markers may be applicable to the risk stratification of IDH-wildtype astrocytoma. Numerous studies have examined molecular markers to obtain more clinically relevant information that will improve the risk stratification of gliomas. The CDKN2A/B homozygous deletion for IDH-mutant astrocytoma and the following three criteria for IDH-wildtype astrocytoma: the concurrent gain of whole chromosome 7 and loss of whole chromosome 10, TERT promoter mutations, and EGFR amplification, were identified as independent molecular markers of the worst clinical outcomes. Therefore, the 2021 World Health Organization (WHO) Classification of Tumors of the Central Nervous System adopted these molecular markers into the revised grading criteria of IDH-mutant and -wildtype astrocytoma respectively, as a grading system within tumor types. For diffuse gliomas in children, molecular alteration-based classification was adopted, dividing low-grade and high-grade subcategories. New tumor types and subtypes were introduced, some based on DNA methylation profiling. To achieve this novel classification in a resource-limited setting, an integrated diagnosis combining clinical, histological, and molecular information became more important.

scholarly journals Growth hormone–secreting macroadenoma of the pituitary gland successfully treated with the radiolabeled somatostatin analog 90Y-DOTATATE: case report

2016 ◽  
Vol 125 (2) ◽  
pp. 346-349 ◽  
Author(s):  
Joanna Waligórska-Stachura ◽  
Paweł Gut ◽  
Nadia Sawicka-Gutaj ◽  
Włodzimierz Liebert ◽  
Maria Gryczyńska ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Pituitary Gland ◽  
Tumor Size ◽  
Pituitary Tumor ◽  
Somatostatin Receptors ◽  
Somatostatin Analogs ◽  
Pituitary Tumors ◽  
Somatostatin Analog ◽  
Surgical Removal ◽  
Radiolabeled Somatostatin Analog

Pituitary tumors causing acromegaly are usually macroadenomas at the time of diagnosis, and they can grow aggressively, infiltrating surrounding tissues. Difficulty in achieving complete tumor removal at surgery can lead toward a strong tendency for recurrence, making it necessary to consider a means of treatment other than those currently used such as somatostatin analogs (SSAs), growth hormone (GH) receptor antagonist, surgical removal, and radiotherapy. The purpose of this paper is to describe a patient diagnosed with an aggressive, giant GH-secreting tumor refractory to medical therapy but ultimately treated with the radiolabeled somatostatin analog 90Y-DOTATATE. A 26-year-old male with an invasive macroadenoma of the pituitary gland (5.6 × 2.5 × 3.6 cm) and biochemically confirmed acromegaly underwent 2 partial tumor resections: the first used the transsphenoidal approach and the second used the transcranial method. The patient received SSAs pre- and postoperatively. Because of the progression in pituitary tumor size, he underwent classic irradiation of the tumor (50 Gy). One and a half years later, the patient presented with clinically and biochemically active disease, and the tumor size was still 52 mm in diameter (height). Two neurosurgeons disqualified him from further surgical procedures. After confirming the presence of somatostatin receptors in the pituitary tumor by using 68Ga-DOTATATE PET/CT, we treated the patient 4 times with an SSA bound with 90Y-DOTATATE. After this treatment, the patient attained partial biochemical remission and a reduction in the tumor mass for the first time. Treatment with an SSA bound with 90Y-DOTATATE may be a promising option for some aggressive GH-secreting pituitary adenomas when other methods have failed.

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