scholarly journals Cushing’s Disease in a 7-Month-Old Girl due to a Tumor Producing Adrenocorticotropic Hormone and Thyreotropin-Secreting Hormone

1999 ◽  
Vol 31 (1) ◽  
pp. 7-11 ◽  
Author(s):  
J.V. List ◽  
S. Sobottka ◽  
A. Huebner ◽  
C. Bonk ◽  
J. Koy ◽  
...  
Neurosurgery ◽  
1983 ◽  
Vol 13 (2) ◽  
pp. 195-198 ◽  
Author(s):  
Mark N. Weissman ◽  
Larry K. Page ◽  
Raphael L. Bejar

Abstract A 7-year-old girl presented with the physical and endocrinological stigmata of Cushing's disease. An adrenocorticotropic hormone (ACTH)-producing pituitary microadenoma was excised. Three weeks after trans-sphenoidal adenomectomy, the patient developed benign intracranial hypertension. Although ACTH levels had decreased to normal, the serum cortisol had fallen to subnormal levels. The child responded to exogenous steroid therapy, which was gradually tapered and discontinued after 5 months. Normal pituitary and adrenal functions persist 2 years later.


2015 ◽  
Vol 38 (2) ◽  
pp. E7 ◽  
Author(s):  
Vivien Bonert ◽  
Namrata Bose ◽  
John D. Carmichael

Diagnosing Cushing's syndrome is challenging and is further hampered when investigations are performed in a patient with cyclic Cushing's syndrome. A subset of patients with Cushing's syndrome exhibit periods of abnormal cortisol secretion with interspersed normal secretion. Patients can have periods of clinical improvement during these quiescent phases or remain symptomatic. Initial diagnostic testing can be challenging because of the unpredictable durations of the peak and trough phases, and it is especially challenging when the diagnosis of cyclic Cushing's syndrome has not yet been determined. Here, the authors present the case of a patient with Cushing's disease with a pathology-proven adrenocorticotropic hormone (ACTH)–secreting pituitary adenoma and whose initial inferior petrosal sinus sampling (IPSS) results were deemed indeterminate; further studies elucidated the diagnosis of cyclic Cushing's syndrome. Repeat IPSS was diagnostic of a central source for ACTH secretion, and the patient was treated successfully with transsphenoidal resection. Literature concerning the diagnosis and management of cyclic Cushing's syndrome is also reviewed.


1990 ◽  
Vol 72 (3) ◽  
pp. 363-369 ◽  
Author(s):  
George T. Tindall ◽  
Carl J. Herring ◽  
Richard V. Clark ◽  
David A. Adams ◽  
Nelson B. Watts

✓ From 1977 to 1988, 56 patients with a preoperative diagnosis of Cushingαs disease were treated by transsphenoidal microsurgical exploration of the pituitary gland. In 42 patients, a discrete tumor was found and a selective adenomectomy was performed. Total hypophysectomy was performed in nine patients. In an attempt to preserve pituitary function, a technique of subtotal hypophysectomy was utilized in the remaining five patients. Regular and adequate follow-up results were obtained in 53 patients. A sustained remission was obtained in 45 of these 53 patients for a remission rate of 84.9%. Eight patients were classified as therapeutic failures. The causes for failure included: 1) invasive tumor; 2) hyperplasia mistaken for an adenoma; 3) a presumed ectopic source of adrenocorticotropic hormone; 4) misdiagnosis; 5) atypical tumor; and 6) recurrence of disease after remission. In cases of therapeutic failure, the original diagnosis of Cushing's disease must be reevaluated and treatment continued until sustained remission is achieved. Necessary measures to help avoid surgical failures and an approach for further diagnostic and therapeutic maneuvers in these cases are discussed.


2015 ◽  
Vol 38 (2) ◽  
pp. E16 ◽  
Author(s):  
Martin J. Rutkowski ◽  
Patrick M. Flanigan ◽  
Manish K. Aghi

After transsphenoidal surgery, Cushing's disease (CD) shows excellent long-term remission rates, but it may recur and pose a therapeutic challenge. Findings in recent published reports on the treatment of recurrent adrenocorticotropic hormone (ACTH)–secreting tumors suggest that repeat resection, radiation-based therapies such as Gamma Knife surgery and proton-beam radiosurgery, pharmacotherapy, and bilateral adrenalectomy all have important roles in the treatment of recurrent CD. Each of these interventions has inherent risks and benefits that should be presented to the patient during counseling on retreatment options. Radiation-based therapies increasingly appear to have efficacies similar to those of repeat resection in achieving biochemical remission and tumor control. In addition, an expanding retinue of medication-based therapies, several of which are currently being evaluated in clinical trials, has shown some promise as tertiary adjunctive therapies. Lastly, bilateral adrenalectomy may offer durable control of refractory recurrent CD. An increasing number of published studies with long-term patient outcomes highlight the evolving treatment patterns in the management of recurrent CD.


1987 ◽  
Vol 66 (2) ◽  
pp. 204-212 ◽  
Author(s):  
William F. Chandler ◽  
David E. Schteingart ◽  
Ricardo V. Lloyd ◽  
Paul E. McKeever ◽  
Gloria Ibarra-Perez

✓ The endocrinological, radiological, surgical, and pathological experience with 34 consecutive patients operated on for Cushing's disease is presented. Preoperative endocrine evaluation demonstrated that 19 patients had “typical” endocrine patterns for pituitary adrenocorticotropic hormone (ACTH)-dependent hypercortisolism and 11 had “atypical” testing. Pituitary pathology was found in 95% of the patients with typical preoperative endocrine testing but in only 55% of those with atypical testing. Eight of 34 preoperative computerized tomography scans demonstrated tumors, which correlated in all instances with the surgical findings. Microadenomas were removed from 25 patients, 22 of whom achieved postoperative remission of endocrine symptoms. Macroadenomas were found in three patients, only one of whom achieved remission after surgery. One patient had removal of an adenoma in the pharyngeal pituitary, and in another an ectopic lung carcinoid was excised; both of these patients were in postoperative remission. Four patients underwent transsphenoidal exploration but no definitive explanation was found for their hypercortisolism; these patients were not in remission. Immunohistochemical staining was performed on every specimen and all tumors showed excessive ACTH-secreting cells. A new rapid stain of the fibrovascular stroma is described.


2009 ◽  
Vol 49 (8) ◽  
pp. 365-369 ◽  
Author(s):  
Satoru SAKIHARA ◽  
Kazunori KAGEYAMA ◽  
Atsufumi MATSUMOTO ◽  
Hidetoshi IKEDA ◽  
Yuko TSUSHIMA ◽  
...  

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
W. Benothman ◽  
G. Saad ◽  
M. Kacem ◽  
K. Ach

The association of pregnancy and Cushing’s disease (CD) is rare. A 28-year-old woman was admitted for clinical suspicion of Cushing’s syndrome. The investigations confirmed the diagnosis of CD with secondary hypogonadotropic hypogonadism due to an invasive pituitary macroadenoma. The patient underwent transsphenoidal adenomectomy, and histopathology showed an adrenocorticotropic hormone pituitary adenoma. Initial remission of CD ensued, and fertility was restored as the patient had 2 uncomplicated pregnancies. Five years and 10 months after surgery, a third spontaneous pregnancy was confirmed with underlying recurrent CD. Having mild hypercortisolism, CD was managed expectantly. The outcome was a healthy full-term neonate with no maternal complications during pregnancy or labor. Our case highlights the challenge faced by physicians of choosing the optimal approach to active CD in pregnancy. In cases where maternal and fetal complications are mild, conservative approach may be used and specific treatment can be postponed until after delivery.


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