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Corticotroph Pituitary Adenoma

ACTH-Secreting Pituitary Adenoma


Presentation

  • However, as with the case presented here, the coincident existence of a sellar mass should not preclude the possibility of an ectopic source.[ncbi.nlm.nih.gov]
  • Out of 70 patients undergoing transsphenoidal surgery for ACTH-secreting pituitary adenomas, four (6%) presented with primarily ectopic adenomas (2 primary operations, 2 reoperations; 3 with CD, one with Nelson's syndrome).[thieme-connect.com]
Easy Bruising
  • It is part of a group of diseases that cause Cushing’s syndrome, characterized by signs and symptoms that may include weight gain around the trunk and in the face, stretch marks, easy bruising, a hump on the upper back, muscle weakness, tiredness, thin[rarediseases.info.nih.gov]
  • Cushing’s Disease Symptoms The most common symptoms of Cushing’s disease are: unexplained weight gain, particularly around the neck and midsection, thinning of the arms and legs and muscle weakness, diabetes, high blood pressure, easy bruising, moodiness[mskcc.org]
  • Symptoms and signs of Cushings syndrome and disease may include: Change in body habitus: weight gain in face (moon face), above the collar bone (supraclavicular) and on back of neck (buffalo hump) Skin changes with easy bruising, purplish stretch marks[pituitary.org]
  • […] of Cushing’s syndrome: patients with Cushing’s disease usually present with one or more signs and symptoms secondary to the presence of excess cortisol Symptoms include: moon face (resulting in a large chin and large forehead) extra fat around neck easy[pietromortini.com]
  • The symptoms resulting from excess ACTH and cortisol are quite different and include mainly weight gain (central distribution), high blood pressure, fatigue, excess hair growth and menstrual irregularities in woman, skin thinning, easy bruising/ecchymosis[csrf.net]
Weight Gain
  • It is part of a group of diseases that cause Cushing’s syndrome, characterized by signs and symptoms that may include weight gain around the trunk and in the face, stretch marks, easy bruising, a hump on the upper back, muscle weakness, tiredness, thin[rarediseases.info.nih.gov]
  • Cushing’s Disease Symptoms The most common symptoms of Cushing’s disease are: unexplained weight gain, particularly around the neck and midsection, thinning of the arms and legs and muscle weakness, diabetes, high blood pressure, easy bruising, moodiness[mskcc.org]
  • Patients are often diagnosed with Cushing’s disease after several years of symptoms which might include progressive weight gain, new onset hypertension or diabetes and mood changes.[pacificneuroscienceinstitute.org]
  • Typically, patients have the following clinical features 6 : rounded face, sometimes described as 'moon-shaped' progressive centripetal obesity and weight gain prominent supraclavicular fat pad, sometimes known as a 'buffalo hump' purple skin striae easy[radiopaedia.org]
  • Symptoms and signs of Cushings syndrome and disease may include: Change in body habitus: weight gain in face (moon face), above the collar bone (supraclavicular) and on back of neck (buffalo hump) Skin changes with easy bruising, purplish stretch marks[pituitary.org]
Sputum
  • Promising preventive measures may include preoperative routine sputum smear and India-ink stain for screening, preoperative treatment of cryptococcal pneumonia, postoperative antibiotic management, and a more secure skull base reconstruction.[ncbi.nlm.nih.gov]
Pharyngitis
  • Stati, Identification of seven hormone producing cell types in the human pharyngeal hypophysis. J. Clin. Endocrinol. Metab. 60 , 212–216 (1985) PubMed CrossRef Google Scholar 31. R. Melchionna, R. Moore, The pharyngeal pituitary gland. Am. J.[link.springer.com]
Gastropathy
  • […] dexamethason suppression testデキサメタゾン抑制試験 diabetes糖尿病 diabetes mellitus (DM)[真性]糖尿病 diabetic bulla (pl. bullae)糖尿病[性]水庖症 diabetic coma 糖尿病[性]昏睡 diabetic complication糖尿病[性]合併症 diabetic diet糖尿病食 diabetic foot糖尿病[性]足病変 diabetic gangrene糖尿病[性]壊疽 diabetic gastropathy[tokyo-med.ac.jp]
Hyperpigmentation
  • Over the ensuing years, the patient developed Nelson's syndrome with hyperpigmentation and markedly elevated ACTH levels.[ncbi.nlm.nih.gov]
  • Avascular necrosis Death of bone due to decreased blood supply 0010885 Bipolar affective disorder Bipolar disorder 0007302 Cardiomyopathy Disease of the heart muscle 0001638 Cataract Clouding of the lens of the eye Cloudy lens [ more ] 0000518 Generalized hyperpigmentation[rarediseases.info.nih.gov]
  • Furthermore, certain patient groups may demonstrate additional or atypical clinical features depending on the etiology of Cushing syndrome 6 : patients with Cushing disease: hyperpigmentation, because melanocyte-stimulating hormone (MSH) and adrenocorticotropic[radiopaedia.org]
  • Hyperpigmentation is often seen and when the adenoma grows large enough there may be visual field defects or cranial nerve symptoms.[path.upmc.edu]
  • Neverthless, hyperpigmentation is a possible but rarely observed finding. Sometimes a psychiatric condition can be associated and, in a more advanced phase, osteoporosis and muscle wasting can become evident.[primarycare.imedpub.com]
Muscle Weakness
  • It is part of a group of diseases that cause Cushing’s syndrome, characterized by signs and symptoms that may include weight gain around the trunk and in the face, stretch marks, easy bruising, a hump on the upper back, muscle weakness, tiredness, thin[rarediseases.info.nih.gov]
  • Cushing’s Disease Symptoms The most common symptoms of Cushing’s disease are: unexplained weight gain, particularly around the neck and midsection, thinning of the arms and legs and muscle weakness, diabetes, high blood pressure, easy bruising, moodiness[mskcc.org]
  • Symptoms may include: upper body obesity round face increased fat around neck thinning arms and legs fragile and thin skin stretch marks on abdomen, thighs, buttocks, arms, and breasts bone and muscle weakness severe fatigue high blood pressure high blood[adrenaltumors.org]
  • Possible signs and symptoms of Cushing's syndrome include: Fat accumulation around your midsection and upper back Exaggerated facial roundness Thinning of the arms and legs with muscle weakness High blood pressure High blood sugar Acne Bone weakening[mayoclinic.org]
Recurrent Fractures
  • fractures Increased fracture rate Increased fractures Multiple fractures Multiple spontaneous fractures Varying degree of multiple fractures [ more ] 0002757 5%-29% of people have these symptoms Abdominal pain Pain in stomach Stomach pain [ more ] 0002027[rarediseases.info.nih.gov]
Fear
  • […] tumor in pituitary gland 0002893 Round face Circular face Round facial appearance Round facial shape [ more ] 0000311 Thin skin 0000963 Truncal obesity 0001956 30%-79% of people have these symptoms Acne 0001061 Anxiety Excessive, persistent worry and fear[rarediseases.info.nih.gov]
Amenorrhea
  • It is important to remember that the main cause of amenorrhea in females of reproductive potential is pregnancy![primarycare.imedpub.com]
  • Galactorrhea, amenorrhea, decreased libido, and infertility. Age is usually greater than 20-25 What pituitary tumors have a high recurrence rate after resection? TSH secreting tumors.[quizlet.com]
  • […] buffalo hump) Skin changes with easy bruising, purplish stretch marks (stria) and red cheeks (plethora) Excess hair growth (hirsutism) on face, neck, chest, abdomen, and thighs Generalized weakness and fatigue Loss of muscle Menstrual disorders in women (amenorrhea[pituitary.org]
  • Acromegaly is associated with enlargement of facial features, hands and feet, heart disease, hypertension, arthritis, carpel tunnel syndrome, amenorrhea, and impotence.[emedicine.medscape.com]
  • Much more often, it causes absent periods (amenorrhea). In men, it causes impotence and decreased sex drive.[drugs.com]
Sexual Dysfunction
  • In contrast, men usually present in the fourth or fifth decades of life and have sexual dysfunction and symptoms related to mass effect attributable to an enlarging sellar mass.[med.virginia.edu]
  • In the early phases, patients complain about one of the symptoms, but after a careful inspection, a constellation of signs and symptoms can be detected.[ 2, 3 ] Sexual dysfunction with decreased libido is one of the main initial complaints (eventually[primarycare.imedpub.com]
  • Signs and symptoms include: Nausea and vomiting Weakness Feeling cold Less frequent or no menstrual periods Sexual dysfunction Increased amount of urine Unintended weight loss or gain Adrenocorticotropic hormone-secreting (ACTH) tumors ACTH tumors produce[mayoclinic.org]
Oligomenorrhea
  • […] intolerance 0001952 Hirsutism Excessive hairiness 0001007 Increased circulating ACTH level High blood corticotropin levels 0003154 Kyphosis Hunched back Round back [ more ] 0002808 Mood changes Moody 0001575 Obesity Having too much body fat 0001513 Oligomenorrhea[rarediseases.info.nih.gov]
  • The clinical presentation can be subtle and generally patients refer to the GP for oligomenorrhea, irregular menstrual cycles, galactorrhea (spontaneous or expressive through squeezing on the nipples).[primarycare.imedpub.com]
  • Men may have erectile dysfunction, whereas premenopausal women often experience oligomenorrhea or amenorrhea 6, 11 ( Table 1 5 – 12 ).[aafp.org]
  • Oligomenorrhea or amenorrhea. Reduced fertility. Loss of libido. Erectile dysfunction. Galactorrhea in the estrogen-primed female breast.[cancer.gov]

Workup

  • […] ovarian tumors adrenocorticotropin (ACTH)-independent macronodular adrenocortical hyperplasia (AIMAH) : rare cause of ACTH-independent Cushing syndrome 4 corticotropin-releasing hormone-secreting tumor: very rare hypothalamic tumors ectopic production The workup[radiopaedia.org]
  • GENERAL PRINCIPLES OF DIAGNOSIS AND TREATMENT Patients presenting with signs and symptoms suggestive of a pituitary adenoma require a comprehensive workup and treatment plan reflecting a strong interdisciplinary effort with endocrinologists, neurosurgeons[med.virginia.edu]
  • While a majority of patients with endogenous Cushing syndrome have thyrotrophic adenomas (called Cushing Disease - see below), other tumors should also be considered as part of the endocrinologic workup, including adrenal tumors and especially ACTH-secreting[eyewiki.aao.org]
  • Dynamic pituitary MRI has high sensitivity and specificity for diagnosis of mild Cushing's syndrome and should be part of the initial workup. Horm Metab Res. 2007;39(6):451–456. 22. Rennert J, Doerfler A. Imaging of sellar and parasellar lesions.[aafp.org]
Enlarged Sella
  • Lateral skull radiograph in a patient with pituitary adenoma shows an enlarged sella and focal calcification in the adenoma (arrow) Pituitary adenomas are almost always benign with no malignant potential.[emedicine.medscape.com]

Treatment

  • Radiation and pharmaceutical treatment may be alternative for recurrent Cushing disease.[ncbi.nlm.nih.gov]
  • As the treatment time or treatment concentration increased, KML001 significantly increased the percentage of AtT-20 cells in apoptosis.[endocrine-abstracts.org]
  • Treatment for Cushing’s Disease The primary treatment for Cushing’s disease is surgery to remove the pituitary tumor.[mskcc.org]

Prognosis

  • Treatment and prognosis Management depends on the specific etiology 6.[radiopaedia.org]
  • Surgical removal has a guardedly favorable prognosis, but long-term follow-up is required.[skullbaseinstitute.com]
  • People with small tumors that do not need immediate treatment or can be completely removed with surgery have an excellent prognosis. Even for large tumors that interfere with vision, surgery can stop further deterioration of vision.[drugs.com]
  • Prognosis Remission can be obtained in up to 90% of patients with microadenomas and in about 50% to 60% of those with macroadenomas. [ 1 ][patient.info]
  • Additionally, it can result in Nelson syndrome in 10% to 30% of patients and carries a generally unfavorable prognosis.[ncbi.nlm.nih.gov]

Etiology

  • Treatment and prognosis Management depends on the specific etiology 6.[radiopaedia.org]
  • In none of these cases, however, was the diagnosis of an ACTH-secreting chromophobe adenoma established at the outset, and the present evidence favoring a pituitary etiology is based on retrospective reasoning and demonstration of abnormally high levels[nejm.org]
  • […] stimulation is also advocated in conjunction with the dexamethasone suppression test in order to increase sensitivity. 55 In patients where this testing is not compelling, inferior petrosal sinus sampling (IPSS) must be performed to confirm the pituitary etiology[ncbi.nlm.nih.gov]
  • Hyperprolactinemia Present Biologically inactive form of prolactin, with no pathologic association Useful to check in patients with hyperprolactinemia who have moderate elevations (100 to 300 mcg per L [4,348 to 13,043 pmol per L]) of serum prolactin with unknown etiology[aafp.org]
  • Etiologies of Hyperprolactinemia (adapted from M.E.[ocw.tufts.edu]

Epidemiology

  • Table 1 outlines the epidemiology, clinical and tumour features, genetics and treatment of different PPAs Table 1 Epidemiology, clinical and tumour features, genetics and treatment of different PPAs Molecular Pathogenesis In recent years genetic defects[karger.com]
  • Jorgensen, Epidemiology of Cushing’s syndrome. Neuroendocrinology 92 (1), 1–5 (2010) PubMed CrossRef Google Scholar 2. R.N. Clayton, D. Raskauskiene, R.C. Reulen, P.W.[link.springer.com]
  • Epidemiology [ 1 ] Pituitary tumours account for 10% to 25% of all intracranial neoplasms. Adenomas account for the largest portion of pituitary neoplasms (estimated at approximately 17%). Only a minority of adenomas are symptomatic.[patient.info]
  • PMID 12763000 The epidemiology of endocrine tumours. Monson JP Endocrine-related cancer. 2000 ; 7 (1) : 29-36. PMID 10808194 Incidence, Pathology, and Recurrence of Pituitary Adenomas: Study of 647 Unselected Surgical Cases.[atlasgeneticsoncology.org]
  • Cryptococcal meningitis : epidemiology and therapeutic options. Clin Epidemiol 2014;6:169–82. [5]. Lui G, Lee N, Ip M, et al. Cryptococcosis in apparently immunocompetent patients. QJM 2006;99:143–51. [6]. Nicholas J, Sarlis SJC, Lynnette K.[journals.lww.com]
Sex distribution
Age distribution

Pathophysiology

  • Because of their pathophysiological characteristics, anterior hypophysis tumors can be generally divided in functional and nonfunctional.[primarycare.imedpub.com]
  • Rovit RL, Duane TD : Cushing's syndrome and pituitary tumours: pathophysiology and ocular manifestation of ACTH- secreting pituitary adenomas. Am J Med1969; 46 : 416-427. 4. Rovit RL, Fein JM : Pituitary apoplexy: A review and reappraisal.[neurologyindia.com]
  • The exact pathophysiology of this phenomenon is unknown; however, as most of these lesions involve the chiasm and/or diencephalon, many have proposed that disruption of visual and vestibular input contribute to this pathology.[eyewiki.aao.org]
  • […] syndrome Symptoms from mass effect; hypopituitarism; silent (nonfunctioning) No specific corresponding test Pituitary carcinoma None No specific syndrome Symptoms from mass effect; hypopituitarism; silent (nonfunctioning) No specific corresponding test Pathophysiology[aafp.org]
  • It also suggests, however, that pituitary adenomas may arrive by different pathophysiologic pathways. Non-hormone-secreting pituitary tumors tend to grow slowly and insidiously.[glowm.com]

Prevention

  • Promising preventive measures may include preoperative routine sputum smear and India-ink stain for screening, preoperative treatment of cryptococcal pneumonia, postoperative antibiotic management, and a more secure skull base reconstruction.[ncbi.nlm.nih.gov]
  • It may be recommended as additional therapy after surgery to prevent regrowth of the tumor. Radiation may also be used as the sole treatment if the tumor cannot be removed surgically, or if the tumor regrows after surgery.[mskcc.org]
  • Prevention Pituitary adenomas are not associated with any known lifestyle or environmental risk factors, so there are no available methods to prevent the condition.[innerbody.com]
  • We believe seeing both specialists at the same time reduces anxiety and uncertainty for the patient, and prevents having to go back and forth between specialists.[uofmhealth.org]
  • Prevention Because doctors do not know why pituitary tumors develop, there is no way to prevent them.[drugs.com]

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