Macroadenoma biochemical behavior in pediatric patients with Cushing’s disease differs from adult cases

Cushing’s disease in children is associated with similar biochemical measures whether the disease is due to macroadenomas or microadenomas, according to a presentation at the AACE 24th Annual Scientific & Clinical Congress.

This contrasts with the disease behavior in adults, in whom macrodenomas demonstrate less glucocorticoid suppression and adrenocorticotropic hormone (ACTH) response to laboratory tests than do microadenomas, according to researchers.

“Children with pituitary macroadenomas are more likely to have the classical response to Cushing’s disease functional testing as microadenomas,”Ricardo Correa, MD, a clinical and research endocrinology fellow at National Institutes of Health, told Endocrine Today.

Correa and colleagues conducted a retrospective review of patients with Cushing’s disease who were younger than 18 years when they were admitted to the NIH between 1997 and 2014. All Cushing’s diagnoses were confirmed by pathology.

Pituitary macroadenoma was identified in 13 patients (69% female) and microadenoma in 74 (58% female). The groups had similar mean age (14 years) and BMI (31.8 kg/m2 and 30.2 kg/m2 for macroadenoma and microadenoma, respectively). The macroadenoma group had a median (25% to 75%) 24-hour urine free cortisol of  263.60 mcg/24 hr (range 170.7-528) compared with 371.6 mcg/ 24 hr (range 244.2-625.3) in the microadenoma group (P = 0.47). Median 24-hr urinary 17-hydroxysteroid excretion in the macroadenoma group was 12.6 mg/24 hr (range 8.9-42.5) and 31.6 mg/24 hr (range 4.3-39.9) in the microadenoma group.

Mean morning serum cortisol was 38.9 ± 40.4 mcg/dL compared with  20.2 ± 15.8 mcg/dL in the macroademona and microadenoma groups, respectively (P = 0.16). Mean morning basal plasma ACTH was 106.3 ± 112.3 pg/mL compared with 49.9±44.3 pg/mL for the macroadenoma and microadenoma groups, respectively (P = 0.11), while ACTH responses to the ovine corticotropin-releasing hormone test revealed no statistically significant differences. Using the high dose dexamethasone suppression test, 58% (7/12) suppressed more than 69% in the macroadenoma group compared to 69% (44/64) in the microadenoma group (P = .51).

“Studies in adult patients have demonstrated that macroadenomas have less glucocorticoid suppressibility after the high-dose dexamethasone suppression test and attenuated ACTH response to CRH compared to pituitary microadenomas,” according to Correa. “However, the present study shows that this is not true in children; although patients with macroadenomas had a tendency for higher baseline serum ACTH and cortisol levels, their responses to dynamic testing were similar to those with microadenomas.”

Reference:

Correa R, et al. Abstract #803. Presented at: AACE 24th Annual Scientific & Clinical Congress; May 13-17, 2015; Nashville, Tenn.

Disclosure: The researchers report no relevant financial disclosures.

From http://www.healio.com/endocrinology/adrenal/news/online/%7Bb4fbf36f-ac88-4eff-9278-90f0a8d1aec2%7D/macroadenoma-biochemical-behavior-in-pediatric-patients-with-cushings-disease-differs-from-adult-cases?sc_trk=internalsearch

From Bangladesh ~ Pituitary Adenoma: When headache is a headache

Location of the pituitary gland in the human brain

Location of the pituitary gland in the human brain (Photo credit: Wikipedia)

“Got headache? Take a paracetamol and get relieved in a short while.”

This is common practice in our country. Almost everyone has had a headache, but rarely headache becomes a headache in our lives. Not all headaches require doctor’s attention but sometimes it represent the tip of a huge iceberg.

Mr Shafiul Islam, 38 years of age, an active male developed a gradual onset of headache, which worsened at the morning, followed by vomiting. He visited a general practitioner and took prescribed medicines, but that failed to cure the symptoms. Rather he was gradually experiencing loss of outer side vision of both eyes.

When he revisited a doctor and was advised for MRI of brain he was diagnosed with a core of “Pituitary Macrodenoma,” a tumor of a hormone producing gland of brain. Then Shafiul was referred to Neurosurgeon of Comfort Nursing home Assistant Professor Dr Moshiur Rahman, who decided to perform operation for removal of the tumor after the initial evaluation.

The pituitary gland is an endocrine gland about the size of a pea and weighing 5 grams (0.18 oz) in humans. It is a protrusion off the bottom of the hypothalamus at the base of the brain, and rests in a small, bony cavity (sella turcica). The pituitary gland secretes nine hormones. A pituitary adenoma is a slow growing and less harmful tumor arising from cells in the pituitary gland. Because they originate from cells in the pituitary gland, which is the master hormone gland, they often cause problems related to hormonal dysfunction.

Some pituitary tumors result in excessive production and over-secretion of hormones, which can result in a variety of syndromes. A large proportion of these tumors, however, do not produce any functional hormones, but instead grow to a size where they cause symptoms because they compress surrounding structures. For these reasons, larger pituitary tumors (called macroadenomas) often present with headache, visual loss and pituitary gland dysfunction.

The specific cause of pituitary adenoma development is unknown, although they are likely to be caused in part by random mutations in cells of the pituitary gland. Surgery is the first line of treatment for many symptomatic pituitary tumors in patients that are good surgical candidates, especially in patients with nonfunctioning macroadenomas.

Dr Moshiur approached the tumor by entering through nasal opening with the help of ENT specialist Associate Professor Dr Sajol Ashfaq, under general aenesthesia (fully unconscious) done by Aenesthesiologist Associate Professor Dr Shamsul Alam. After elevation of a thin membrane over the nasal partition and breaking a bone in the base of the skull they got a vision of the tumor through endoscope. After that, the tumor was removed through the nose. After three days of post-operative care, the patient was discharged. All his symptoms, headache, vomiting and poor vision improved dramatically and he got back to his normal life.

Dr Moshiur Rahman said: “The surgical approach for removing pituitary tumours is usually an endonasal (through the nostril) transsphenoidal (through the sphenoid sinus) approach. This procedure is Endoscopic Transnasal Transphenoidal Pituitary Adenomectomy, which is a safe, minimally invasive but effective, modern treatment option for Pituitary Adenoma, with few side effects and short post-operative hospital stay. This latest technology is being practiced in some centres of the capital for last few years.”

He also said, he performed three operations before successfully with no long term adverse effect. He also paid gratefulness to Associate Professor Dr Sajol Ashfaq and Associate Professor Dr Shamsul Alam for their sincere and great effort.

Once, people had to go outside of the country for this operation. Nowadays, this operation is often performed by many neurosurgeons of the capital. A few centres have also developed to provide these facilities of operation. People can take this oppurtunity confidently by choosing a competent surgical team.

Endoscopic transsphenoidal pituitary surgery: a good and safe primary treatment option for Cushings disease, even in case of macroadenomas or invasive adenomas.

Abstract – European Journal of Endocrinology, 06/24/2013  Clinical Article

Wagenmakers MA et al. – This study aims to gain insight in the role of endoscopic TS as a primary treatment option for CD, especially in patients with MRI negative CD and (invasive) macroadenomas.

The data suggest that in patients with non–invasive and invasive macroadenomas the endoscopic technique of TS should be the technique of choice as remission rates seem to be higher than remission rates reported for microscopic TS, although no comparative study has been performed.

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