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      A rare challenging case of co-existent craniopharyngioma, acromegaly and squamous cell lung cancer

      research-article
      1 , 2 , 3 , 1 , 2 , 3 , 2 , 3 , 1 , 2 , 3 , 4 , 1 , 2 , 3
      Endocrinology, Diabetes & Metabolism Case Reports
      Bioscientifica Ltd
      Adult, Male, White, United Kingdom, Pituitary, Pituitary, Gonadotropins, IGF1, Prolactin, TSH, ACTH, GH, Testosterone, Craniopharyngioma, Acromegaly, Hypergonadotropic hypogonadism, Pituitary adenoma, Visual impairment, Headache, Fatigue, Hypogonadism, Vision - acuity reduction, Diabetes insipidus, Hands - enlargement, Face - coarse features, Breathing difficulties, MRI, Prolactin, IGF1, Histopathology, Gonadotrophins, ACTH, TSH, Glucose tolerance (oral), GH, GH suppression, Visual field assessment, X-ray, CT scan, Fine needle aspiration biopsy, Immunohistochemistry, Synaptophysin, PET scan, Transsphenoidal surgery, Radiotherapy, Resection of tumour, Somatostatin analogues, Desmopressin, Hydrocortisone, Glucocorticoids, Levothyroxine, Testosterone, Lanreotide, Oncology, Unique/unexpected symptoms or presentations of a disease, March, 2018

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          Summary

          Co-existence of craniopharyngioma and acromegaly has been very rarely reported. A 65-year-old man presented with visual deterioration, fatigue and frontal headaches. Magnetic resonance imaging revealed a suprasellar heterogeneous, mainly cystic, 1.9 × 2 × 1.9 cm mass compressing the optic chiasm and expanding to the third ventricle; the findings were consistent with a craniopharyngioma. Pituitary hormone profile showed hypogonadotropic hypogonadism, mildly elevated prolactin, increased insulin-like growth factor 1 (IGF-1) and normal thyroid function and cortisol reserve. The patient had transsphenoidal surgery and pathology of the specimen was diagnostic of adamantinomatous craniopharyngioma. Post-operatively, he had diabetes insipidus, hypogonadotropic hypogonadism and adrenocorticotropic hormone and thyroid-stimulating hormone deficiency. Despite the hypopituitarism, his IGF-1 levels remained elevated and subsequent oral glucose tolerance test did not show complete growth hormone (GH) suppression. Further review of the pre-operative imaging revealed a 12 × 4 mm pituitary adenoma close to the right carotid artery and no signs of pituitary hyperplasia. At that time, he was also diagnosed with squamous cell carcinoma of the left upper lung lobe finally managed with radical radiotherapy. Treatment with long-acting somatostatin analogue was initiated leading to biochemical control of the acromegaly. Latest imaging has shown no evidence of craniopharyngioma regrowth and stable adenoma. This is a unique case report of co-existence of craniopharyngioma, acromegaly and squamous lung cell carcinoma that highlights diagnostic and management challenges. Potential effects of the GH hypersecretion on the co-existent tumours of this patient are also briefly discussed.

          Learning points:
          • Although an extremely rare clinical scenario, craniopharyngioma and acromegaly can co-exist; aetiopathogenic link between these two conditions is unlikely.

          • Meticulous review of unexpected biochemical findings is vital for correct diagnosis of dual pituitary pathology.

          • The potential adverse impact of GH excess due to acromegaly in a patient with craniopharyngioma (and other neoplasm) mandates adequate biochemical control of the GH hypersecretion.

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          Most cited references12

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          Pathogenesis of pituitary tumors.

          Pituitary adenomas may hypersecrete hormones (including prolactin, growth hormone and adrenocorticotropic hormone, and rarely follicle-stimulating hormone, luteinizing hormone or TSH) or may be nonfunctional. Despite their high prevalence in the general population, these tumors are invariably benign and exhibit features of differentiated pituitary cell function as well as premature proliferative arrest. Pathogenesis of dysregulated pituitary cell proliferation and unrestrained hormone hypersecretion may be mediated by hypothalamic, intrapituitary and/or peripheral factors. Altered expression of pituitary cell cycle genes, activation of pituitary selective oncoproteins or loss of pituitary suppressor factors may be associated with aberrant growth factor signaling. Considerable information on the etiology of these tumors has been derived from transgenic animal models, which may not accurately and universally reflect human tumor pathophysiology. Understanding subcellular mechanisms that underlie pituitary tumorigenesis will enable development of tumor aggression markers as well as novel targeted therapies.
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            Epidemiology of acromegaly: review of population studies

            Acromegaly is a rare condition necessitating large population studies for the generation of reliable epidemiological data. In this review, we systematically analysed the epidemiological profile of this condition based on recently published population studies from various geographical areas. The total prevalence ranges between 2.8 and 13.7 cases per 100,000 people and the annual incidence rates range between 0.2 and 1.1 cases/100,000 people. The median age at diagnosis is in the fifth decade of life with a median diagnostic delay of 4.5–5 years. Acral enlargement and coarse facial features are the most commonly described clinical manifestations. At the time of detection, most of the tumors are macroadenomas possibly relating to diagnostic delays and posing challenges in the surgical management. Increased awareness of acromegaly amongst the medical community is of major importance aiming to reduce the adverse sequelae of late diagnosis and treatment, improve patient outcomes and, hopefully, reduce the burden on the health care system.
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              Risk of colorectal neoplasm in patients with acromegaly: a meta-analysis.

              To examine the risk of colorectal neoplasm in acromegalic patients by meta-analyzing all relevant controlled studies. Extensive English language medical literature searches for human studies, up to December 2007, were performed using suitable keywords. Pooled estimates [odds ratio (OR) with 95% confidence intervals (CI)] were obtained using either the fixed or random-effects model as appropriate. Heterogeneity between studies was evaluated with the Cochran Q test whereas the likelihood of publication bias was assessed by constructing funnel plots. Their symmetry was estimated by the adjusted rank correlation test. For hyperplastic polyps the pooled ORs with 95% CI were 3.557 (2.587-4.891) by fixed effects model and 3.703 (2.565-5.347) by random effects model. The Z test values for overall effect were 7.81 and 6.984, respectively (P < 0.0001). For colon adenomas the pooled ORs with 95% CI were 2.486 (1.908-3.238) (fixed effects model) and 2.537 (1.914-3.364) (random effects model). The Z test values were 6.747 and 6.472, respectively (P < 0.0001). For colon cancer the pooled OR with 95% CI was identical for both fixed and random effects model (OR, 4.351; 95% CI, 1.533-12.354; Z = 2.762, P = 0.006]. There was no significant heterogeneity and no publication bias in all the above meta-analyses. Acromegaly is associated with an increased risk of colorectal neoplasm.

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                28 March 2018
                2018
                : 2018
                : 18-0018
                Affiliations
                [1 ]Institute of Metabolism and Systems Research , College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
                [2 ]Centre for Endocrinology , Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
                [3 ]Departments of Endocrinology and Radiology , Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
                [4 ]Departments of Radiology , Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
                Author notes
                Correspondence should be addressed to N Karavitaki Email: n.karavitaki@ 123456bham.ac.uk
                Article
                EDM180018
                10.1530/EDM-18-0018
                5881427
                d2dbbeb9-350e-4371-a060-d314a1e568dd
                © 2018 The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

                History
                : 26 February 2018
                : 12 March 2018
                Categories
                Adult
                Male
                White
                United Kingdom
                Pituitary
                Pituitary
                Gonadotropins
                Igf1
                Prolactin
                TSH
                ACTH
                Gh
                Testosterone
                Craniopharyngioma
                Acromegaly
                Hypergonadotropic Hypogonadism
                Pituitary Adenoma
                Visual impairment
                Headache
                Fatigue
                Hypogonadism
                Vision - acuity reduction
                Diabetes insipidus
                Hands - enlargement
                Face - coarse features
                Breathing difficulties
                MRI
                Prolactin
                IGF1
                Histopathology
                Gonadotrophins
                ACTH
                TSH
                Glucose tolerance (oral)
                GH
                GH suppression
                Visual field assessment
                X-ray
                CT scan
                Fine needle aspiration biopsy
                Immunohistochemistry
                Synaptophysin
                PET scan
                Transsphenoidal surgery
                Radiotherapy
                Resection of tumour
                Somatostatin analogues
                Desmopressin
                Hydrocortisone
                Glucocorticoids
                Levothyroxine
                Testosterone
                Lanreotide
                Oncology
                Unique/Unexpected Symptoms or Presentations of a Disease
                Unique/Unexpected Symptoms or Presentations of a Disease

                adult,male,white,united kingdom,pituitary,gonadotropins,igf1,prolactin,tsh,acth,gh,testosterone,craniopharyngioma,acromegaly,hypergonadotropic hypogonadism,pituitary adenoma,visual impairment,headache,fatigue,hypogonadism,vision - acuity reduction,diabetes insipidus,hands - enlargement,face - coarse features,breathing difficulties,mri,histopathology,gonadotrophins,glucose tolerance (oral),gh suppression,visual field assessment,x-ray,ct scan,fine needle aspiration biopsy,immunohistochemistry,synaptophysin,pet scan,transsphenoidal surgery,radiotherapy,resection of tumour,somatostatin analogues,desmopressin,hydrocortisone,glucocorticoids,levothyroxine,lanreotide,oncology,unique/unexpected symptoms or presentations of a disease,march,2018

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