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Case Reports
. 2025 Apr 11;3(5):luaf079.
doi: 10.1210/jcemcr/luaf079. eCollection 2025 May.

ACTH-dependent Cyclic Cushing Syndrome With Successful Pregnancy and Early Postpartum Relapse

Mohd Idris Mohamad Diah  1 , Jin Hui Ho  1 , Hwee Ching Tee  1
Affiliations
Case Reports

ACTH-dependent Cyclic Cushing Syndrome With Successful Pregnancy and Early Postpartum Relapse

Mohd Idris Mohamad Diah et al. JCEM Case Rep. .

Abstract

Cyclic Cushing syndrome (CS) is a rare form of CS characterized by intermittent episodes of hypercortisolism. We report the case of a 30-year-old female who was diagnosed with ACTH-dependent Cushing disease, confirmed by initial biochemical tests and pituitary imaging. Although surgery was planned, she experienced spontaneous remission for several months, followed by pregnancy, and subsequently relapsed in the early postpartum period. Transsphenoidal resection of a left-sided pituitary adenoma was then performed, confirming an ACTH-secreting tumor. A review of the literature revealed that this case contributes to the increasing number of patients with cyclic CS, with particular attention to the challenges of diagnosing hypercortisolism during pregnancy. While cases of Cushing disease recurrence after pituitary surgery in the immediate postpartum period have been documented, this is the first reported case of early postpartum relapse in cyclic CS. This case highlights the importance of long-term follow-up in patients with a high index of suspicion for cyclic CS, as well as the diagnostic challenges in managing the condition during pregnancy and the peripartum period.

Keywords: ACTH-dependent Cushing syndrome; cyclic Cushing syndrome; postpartum relapse; pregnancy.

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Figures

Figure 1.
Figure 1.
Clinical findings of Cushing syndrome during presentation in our case. (A) Round face; (B) acne with acanthosis nigricans; (C) red-purple striae on the abdomen.
Figure 2.
Figure 2.
Coronal T1-weighted MRI pituitary at diagnosis and after transsphenoidal surgery. (A) A hypointense lesion on the T1-weighted image in the left anterior pituitary region, measuring 0.6 × 0.5 × 0.5 cm (yellow arrow). (B) The lesion exhibited minimally delayed enhancement following gadolinium contrast administration (red arrow). (C) Follow-up MRI 6 months after transsphenoidal surgery. Abbreviation: MRI, magnetic resonance imaging.
Figure 3.
Figure 3.
Timeline changes of 24-hour urinary cortisol level over the years. Normal limit reference value for 24-hour urine cortisol: 11.8 to 485.6 nmol/24 hours.
Figure 4.
Figure 4.
ACTH-secreting pituitary adenoma. (A) Tumor cells arranged in solid sheets (low magnification, H&E stain). (B) Cytologically, the tumor cells have uniform round nuclei, stippled chromatin texture, inconspicuous nucleoli, and a modest to ample amount of basophilic cytoplasm (high magnification, H&E stain). (C) The tumor cells express strong and diffuse cytoplasmic reactivity for ACTH on immunohistochemistry. Abbreviation: H&E, hematoxylin and eosin.
Figure 5.
Figure 5.
Proposed diagnostic algorithm for suspected cyclic Cushing syndrome.
See this image and copyright information in PMC

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