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ACTH-Secreting Pituitary Adenoma

Brain Tumor ACTH Secreting Pituitary Adenoma


Presentation

  • We present the case of a pediatric patient with an ACTH-producing tumor but little evidence of CS. No mutations in the coding sequence of NR3C1 were detected.[ncbi.nlm.nih.gov]
  • 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]
  • An 18-month old infant with Cushing's disease due to an ACTH producing pituitary tumor is presented. The case showed typical clinical and morphological sings of hypercortisolism.[ncbi.nlm.nih.gov]
  • A 19 years old Lebanese man presented in 2000, with overt Cushing's syndrome confirmed with markedly elevated urine free cortisols and failed dexamethasone suppression tests.[ncbi.nlm.nih.gov]
  • Here, we present a case of sphenoidal ectopic pituitary adenoma presenting as Cushing’s syndrome in association with a partial empty sella.[jpmsonline.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]
  • CASE REPORT A 16-year old female presented to our endocrinology department with 4-year history of unexplained weight gain of approximately 15 kg as the primary complaint.[jpmsonline.com]
  • 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]
Hyperpigmentation
  • Over the ensuing years, the patient developed Nelson's syndrome with hyperpigmentation and markedly elevated ACTH levels.[ncbi.nlm.nih.gov]
  • Aseptic 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 aetiology 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]
  • Nelson syndrome presents as hyperpigmentation from loss of cortisol inhibition and the stimulation of melanocytes by elevated ACTH.[ncbi.nlm.nih.gov]
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]
  • Patients with Cushing's may develop diabetes mellitus, high blood pressure, muscle weakness, clotting disorders, infections and obesity. Bone loss with pathologic fractures can occur.[pituitary.mgh.harvard.edu]
Amenorrhea
  • It is important to remember that the main cause of amenorrhea in females of reproductive potential is pregnancy![primarycare.imedpub.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]
  • 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]
  • 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]
  • 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]
  • 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]
Oligomenorrhea
  • […] glucose tolerance 0000833 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.[northshore.org]

Workup

  • […] tumours adrenocorticotropin (ACTH)-independent macronodular adrenocortical hyperplasia (AIMAH) : rare cause of ACTH-independent Cushing syndrome 4 corticotropin-releasing hormone-secreting tumour: very rare hypothalamic tumours 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]
  • Cortisol collection during GC treatment tended to increase (32.7% vs. 57.1%; p   0.068). Of surgeries performed without prior GC treatment, 31.7 and 55.0% had a cortisol nadir of  2 and  5 µg/dL, respectively, within 72 h postoperative.[ncbi.nlm.nih.gov]
  • Treatment for Cushing’s Disease The primary treatment for Cushing’s disease is surgery to remove the pituitary tumor.[mskcc.org]
  • As the treatment time or treatment concentration increased, KML001 significantly increased the percentage of AtT-20 cells in apoptosis.[endocrine-abstracts.org]
  • PRKCD might restrain corticotroph adenoma cells from acquiring an aggressive behavior, candidating PRKCD as a possible molecular target for the treatment of corticotroph adenomas.[ncbi.nlm.nih.gov]

Prognosis

  • Treatment and prognosis Management depends on the specific aetiology 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]
  • Additionally, it can result in Nelson syndrome in 10% to 30% of patients and carries a generally unfavorable prognosis.[ncbi.nlm.nih.gov]
  • 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]

Etiology

  • 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]
  • Das Ansprechen der Hormonsekretion variiert, bei Prolaktinomen beträgt es zwischen 20 und 75 % mit einer Latenz von Monaten. en The CHMP considered the MAH responses and agreed that although data may suggest that prolactin is involved in breast cancer etiology[de.glosbe.com]
  • 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]
  • 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 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]
  • Prevention Because doctors do not know why pituitary tumors develop, there is no way to prevent them.[drugs.com]

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