Long-Term Obesity Persists Despite Pituitary Adenoma Treatment In Childhood

Sethi A, et al. Clin Endocrinol. 2019;doi:10.1111/CEN.14146.

January 5, 2020

Obesity is common at diagnosis of pituitary adenoma in childhood and may persist despite successful treatment, according to findings published in Clinical Endocrinology.

“The importance of childhood and adolescent obesity on noncommunicable disease in adult life is well recognized, and in this new cohort of patients, we report that obesity is common at presentation of pituitary adenoma in childhood and that successful treatment is not necessarily associated with weight loss,” Aashish Sethi, MD, MBBS, a pediatric endocrinologist in the department of endocrinology at Alder Hey Children’s Hospital in Liverpool, United Kingdom, and colleagues wrote. “We have reported obesity, and obesity-related morbidity in a mixed cohort of children and young adults previously, but [to] our knowledge, this is the first time this observation has been reported in a purely pediatric cohort.”

In a retrospective study, Sethi and colleagues analyzed clinical and radiological data from 24 white children from Alder Hey Children’s Hospital followed for a median of 3.3 years between 2000 and 2019 (17 girls; mean age at diagnosis, 15 years). Researchers assessed treatment modality (medical, surgical or radiation therapy), pituitary hormone deficiencies and BMI, as well as results of any genetic testing.

Within the cohort, 13 girls had prolactinomas (mean age, 15 years), including 10 macroadenomas between 11 mm and 35 mm in size. Children presented with menstrual disorders (91%), headache (46%), galactorrhea (46%) and obesity (38%). Nine children were treated with cabergoline alone, three also required surgery, and two were treated with the dopamine agonist cabergoline, surgery and radiotherapy.

Five children had Cushing’s disease (mean age, 14 years; two girls), including one macroadenoma. Those with Cushing’s disease presented with obesity (100%), short stature (60%) and headache (40%). Transsphenoidal resection resulted in biochemical cure; however, two patients experienced relapse 3 and 6 years after surgery, respectively, requiring radiotherapy. One patient also required bilateral adrenalectomy.

Six children had a nonfunctioning pituitary adenoma (mean age, 16 years; two girls), including two macroadenomas. These children presented with obesity (67%), visual field defects (50%) and headache (50%). Four required surgical resections, with two experiencing disease recurrence after surgery and requiring radiotherapy.

During the most recent follow-up exam, 13 children (54.1%) had obesity, including 11 who had obesity at diagnosis.

“The persistence of obesity following successful treatment, in patients with normal pituitary function, suggests that mechanisms other than pituitary hormone excess or deficiency may be important,” the researchers wrote. “It further signifies that obesity should be a part of active management in cases of pituitary adenoma from diagnosis.” – by Regina Schaffer

Disclosures: The authors report no relevant financial disclosures.

From https://www.healio.com/endocrinology/adrenal/news/online/%7Bde3fd83b-e8e0-4bea-a6c2-99eb896356ab%7D/long-term-obesity-persists-despite-pituitary-adenoma-treatment-in-childhood

Paraneoplastic Cushing’s Syndrome Due to ACTH-Secreting Acinic Cell Carcinoma of the Parotid Gland: A Rare Case

​​​​​​​​​​​​​​Although acinic cell carcinoma — a cancer of the glands that produce saliva — is usually considered low risk, it can behave aggressively and cause Cushing’s syndrome, according to researchers who described such a case involving a 58-year-old man in Turkey.

They added that if not recognized and treated at an early stage, it can rapidly become life-threatening.

“The rarity of this entity poses a diagnostic challenge,” wrote the group of four researchers from a hospital in Turkey. “Awareness of this association is critical, as early identification and intervention may be lifesaving in selected patients.”

The case was described in a letter to the editor, titled “Paraneoplastic Cushing’s syndrome due to ACTH-secreting acinic cell carcinoma of the parotid gland: A rare case,” in the European Annals of Otorhinolaryngology, Head and Neck Diseases.

Pituitary adenoma growth rate influenced by multiple factors

Monsalves E. J. Clin Endocrinol Metab. 2013; doi: 10. 1210/jc.2013-3054.

The etiology of pituitary adenoma growth rate is multifactorial and may be influenced by patient age and gender, as well as adenoma subtype, hormonal activity, immunohistological profile and the direction of growth relative to the pituitary fossa, according to results of a retrospective study.

Researchers evaluated pre- and postoperative pituitary adenoma (PA) traits in relation to patient demographics, MRI specifications and histopathological factors. They examined 153 patients who underwent surgery for removal of a histologically-proven PA at Toronto Western Hospital between 1999 and 2011.

All patients had at least two preoperative and two postoperative MRIs to measure tumor volume doubling time. Both scans were completed a minimum of 3 months apart.

Patients all underwent a sella/pituitary imaging protocol, and volume was determined using partitioning and target volume software. Each patient was also reviewed by two endocrine pathologists, and standardized diagnostic synoptic pathology reports provided information on MIB-1 labeling index, p27 and N-terminally truncated fibroblast growth factor receptor 4 (FGFR4). Growth direction patterns were classified as superior, anterior, posterior and lateral in relation to the sellar fossa.

The researchers found a relationship between preoperative growth rate and age (P=.0001), as well as suprasellar growth (P=.003), existence of a cyst or hemorrhage (P= .004), the MIB-1 (P=.005), FGFR4 positivity (P=.047) and p27 negativity (P=.007).

Postoperatively, 34.6% of patients demonstrated residual volumes, while the remaining 100 patients did not. Residual volume was found to be associated with older patient age (57 vs. 51, P=.038), as well as growth patterns, including anterior, posterior, suprasellar and cavernous sinus extension (P=.001). There was a correlation between pre-and postoperative growth rates (r=0.497, P=.026). The rates of postoperative growth were linked with age (P=.015) and gender (P= .017).

“Due to the heterogeneity of PA, no single predictor of PA growth behavior can be taken in isolation as a means to predict its outcome,” the researchers wrote.  “These predictors must be combined in order to formulate the most accurate estimation of PA growth, which in turn will inform sound clinical management.”

Disclosures: The researchers report no relevant financial disclosures.

From http://www.healio.com/endocrinology/neuroendocrinology/news/online/%7B7cb2ec5d-eaa6-42a3-b279-2c2436d0fbd0%7D/pituitary-adenoma-growth-rate-influenced-by-multiple-factors