Update on the management of recurrent Cushing's disease

Rutkowski, Martin J.; Flanigan, Patrick M.; Aghi, Manish K.

doi:10.3171/2014.11.focus14703

Cited by 28 publications

(18 citation statements)

References 66 publications

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“…Prior series have reported treatment success in 97% of nonsecreting tumors and 45 to 93% secreting adenomas, with Pollock estimating an overall success rate of durable biochemical cure in at least 60% of recurrent secreting tumors. [174][175][176][177][178][179][180] Hypopituitarism is the most common complication, with 10 to 12% of adults requiring chronic hormonal supplementation after SRS. 16,48,181,182 Data on pediatric pituitary radiotherapy is more limited, due to its infrequent use; as our literature review demonstrates, radiation of any modality was reported in only 16% of children with recurrent or persistent disease.…”

Section: Management Of Progression or Recurrencementioning

confidence: 99%

Pediatric Pituitary Adenoma: Case Series, Review of the Literature, and a Skull Base Treatment Paradigm

et al. 2018

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Pediatric pituitary adenoma is a rare skull base neoplasm, accounting for 3% of all intracranial neoplasms in children and 5% of pituitary adenomas. Compared with pituitary tumors in adults, secreting tumors predominate and longer disease trajectories are expected due to the patient age resulting in a natural history and treatment paradigm that is complex and controversial. The aims of this study were to describe a large, single-institution series of pediatric pituitary adenomas with extensive long-term follow-up and to conduct a systematic review examining outcomes after pituitary adenoma surgery in the pediatric population. The study cohort was compiled by searching institutional pathology and operative reports using diagnosis and site codes for pituitary and sellar pathology, from 1956 to 2016. Systematic review of the English language literature since 1970 was conducted using PubMed, MEDLINE, Embase, and Google Scholar. Thirty-nine surgically managed pediatric pituitary adenomas were identified, including 15 prolactinomas, 14 corticotrophs, 7 somatotrophs, and 4 non-secreting adenomas. All patients underwent transsphenoidal resection (TSR) as the initial surgical treatment. Surgical cure was achieved in 18 (46%); 21 experienced recurrent/persistent disease, with secondary treatments including repeat surgery in 10, radiation in 14, adjuvant pharmacotherapy in 11, and bilateral adrenalectomy in 3. At the last follow-up (median 87 months, range 3-581), nine remained with recurrent/persistent disease (23%). Thirty-seven publications reporting surgical series of pediatric pituitary adenomas were included, containing 1,284 patients. Adrenocorticotropic hormone (ACTH)-secreting tumors were most prevalent (43%), followed by prolactin (PRL)-secreting (37%), growth hormone (GH)-secreting (12%), and nonsecreting (7%). Surgical cure was reported in 65%. Complications included pituitary insufficiency (23%), permanent visual dysfunction (6%), chronic diabetes insipidus (DI) (3%), and postoperative cerebrospinal fluid (CSF) leak (4%). Mean follow-up was 63 months (range 0-240), with recurrent/persistent disease reported in 18% at the time of last follow-up. Pediatric pituitary adenomas are diverse and challenging tumors with complexities far beyond those encountered in the management of routine adult pituitary disease, including nuanced decision-making, a technically demanding operative environment, high propensity for recurrence, and the potentially serious consequences of hypopituitarism with respect to fertility and growth potential in a pediatric population. Optimal treatment requires a high degree of individualization, and patients are most likely to benefit from consolidated, multidisciplinary care in highly experienced centers.

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Section: Management Of Progression or Recurrencementioning

confidence: 99%

Pediatric Pituitary Adenoma: Case Series, Review of the Literature, and a Skull Base Treatment Paradigm

et al. 2018

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“…In patients with evidence of incomplete tumor resection or a pituitary lesion on imaging, repeat TSS is recommended. Repeat TSS tends to have a lower remission rate than primary surgery, and carries with it a higher risk of panhypopituitarism (2,3). Medical therapy for CD may consist of steroidogenesis inhibitors such as ketoconazole, metyropone, mitotane, and etomidate, or pituitary-directed treatments such as pasireotide or cabergoline (1-3).…”

Section: Discussionmentioning

confidence: 99%

The Novel Use of Stereotactic Radiotherapy for Remnant Adrenal Tissue in Nelson Syndrome

Martirossian

Jacob

Riley

et al. 2020

AACE Clinical Case Reports

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Objective: Recurrent Cushing disease (CD) is a rare complication that occurs in patients who have undergone bilateral adrenalectomy (BLA). We report a case of recurrent CD in a patient with Nelson syndrome and adrenalectomy due to remnant adrenal tissue, and a novel treatment strategy using stereotactic body radiation therapy (SBRT) to the adrenal glands. Methods: We report a case of recurrent CD in a woman with Nelson syndrome and adrenalectomy and describe her clinical course and management. We also include a literature review of CD management and adrenal radiation. Results: The patient had persistent pituitary CD despite pituitary surgery and radiosurgery and underwent BLA. She developed recurrent CD due to a remnant adrenal gland post adrenalectomy. She then underwent SBRT to both adrenal beds to treat the remnant adrenal tissue. Her serum cortisol dropped rapidly after adrenal radiation and she experienced minimal side effects. She has been in remission for over 2 years. Conclusion: This is the first reported case of recurrent CD in a patient post adrenalectomy that was successfully treated with SBRT to the remnant adrenal tissue.

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“…Selective adenomectomy by TSS is the treatment of choice for Cushing’s disease [ 1 - 4 , 8 ]. The remission and recurrence rates after TSS have been reported to be in the ranges of 65%–95% and 8%–35%, respectively [ 14 - 16 ]. Several factors may influence remission in Cushing disease, such as preoperative ACTH level, UFC levels, adenoma size, localization on MRI, intraoperative tumor visualization, dural invasion, histological confirmation of corticotroph adenomas, skillful surgery, and age of patient.…”

Section: Discussionmentioning

confidence: 99%

A rare case of multiple pituitary adenomas in an adolescent Cushing disease presenting as a vertebral compression fracture

Song

Mun

Sung

et al. 2017

Ann Pediatr Endocrinol Metab

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Cushing disease in children and adolescents, especially with multiple pituitary adenomas (MPAs), is very rare. We report 17-year-old boy with MPAs. He presented with a vertebral compression fracture, weight gain, short stature, headache, and hypertension. On magnetic resonance imaging (MRI), only a left pituitary microadenoma was found. After surgery, transient clinical improvement was observed but headache and hypertension were observed again after 3 months later. Follow-up MRI showed a newly developed right pituitary microadenoma 6 months after the surgery. The need for careful clinical and radiographic follow-up should be emphasized in the search for potential MPAs in patients with persistent Cushing disease.

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Update on the management of recurrent Cushing's disease

Cited by 28 publications

References 66 publications

Pediatric Pituitary Adenoma: Case Series, Review of the Literature, and a Skull Base Treatment Paradigm

Pediatric Pituitary Adenoma: Case Series, Review of the Literature, and a Skull Base Treatment Paradigm

The Novel Use of Stereotactic Radiotherapy for Remnant Adrenal Tissue in Nelson Syndrome

A rare case of multiple pituitary adenomas in an adolescent Cushing disease presenting as a vertebral compression fracture

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