Sparsely Granulated Corticotroph Pituitary Macroadenoma Presenting With Pituitary Apoplexy Resulting in Remission of Hypercortisolism

Abstract

Objective: Pituitary corticotroph macroadenomas, which account for 7% to 23% of corticotroph adenomas, rarely present with apoplexy. This report aimed to describe a patient with a sparsely granulated corticotroph tumor (SGCT) presenting with apoplexy and remission of hypercortisolism.

Case report: A 33-year-old male patient presented via ambulance with sudden onset of severe headache and nausea/vomiting. Physical examination revealed bitemporal hemianopsia, diplopia from right-sided third cranial nerve palsy, abdominal striae, facial plethora, and dorsal and supraclavicular fat pads. Magnetic resonance imaging demonstrated a 3.2-cm mass arising from the sella turcica with hemorrhage compressing the optic chiasm, extension into the sphenoid sinus and cavernous sinus. Initial investigations revealed a plasma cortisol level of 64.08 (reference range [RR], 2.36-17.05) mcg/dL. He underwent emergent transsphenoidal surgery. Pathology was diagnostic of SGCT. Postoperatively, the following laboratory findings were found: (1) cortisol level, <1.8 ug/dL (RR, 2.4-17); (2) adrenocorticotropic hormone level, 36 pg/mL (RR, 0-81); (3) thyroid-stimulating hormone level, 0.07 uIU/mL (RR, 0.36-3.74); (4) free thyroxine level, 1 ng/dL (RR, 0.8-1.5); (5) luteinizing hormone level, <1 mIU/mL (RR, 1-12); (6) follicle-stimulating hormone level, 1 mIU/mL (RR, 1-12); and (7) testosterone level, 28.8 ng/dL (RR, 219.2-905.6), with ongoing requirement for hydrocortisone, levothyroxine, testosterone replacement, and continued follow-up.

Discussion: Corticotroph adenomas are divided into densely granulated, sparsely granulated, and Crooke cell tumors. Sparsely granulated pattern is associated with a larger tumor size and decreased remission rate after surgery.

Conclusion: This report illustrates a rare case of hypercortisolism remission due to apoplexy of an SGCT with subsequent central adrenal insufficiency, hypothyroidism, and hypogonadism.

Keywords: ACTH, adrenocorticotropic hormone; CD, Cushing disease; Cushing disease; DGCT, densely granulated cell tumor; FSH, follicle-stimulating hormone; IGF-1, insulin-like growth factor 1; LH, luteinizing hormone; MRI, magnetic resonance imaging; RR, reference range; SGCT, sparsely granulated corticotroph tumor; TSH, thyroid-stimulating hormone; TSS, transsphenoidal surgery; pituitary apoplexy; pituitary macroadenoma; pituitary tumor; sparsely granulated corticotroph tumor.

Fig. 1

A, Hyperattenuating 2.0 × 2.8 × 1.5-cm mass at the sella turcica on unenhanced computed tomography. Magnetic resonance imaging revealed a 1.9 × 3.2 × 2.4-cm heterogeneous mass on, (B) T1-weighted imaging and (C) T2-weighted imaging showing small hyperintense areas in the solid part of the sella mass with flattening of the optic chiasm and remodeling/dehiscence of the floor of the sella, extending into the right cavernous sinus with at least partial encasement of the internal carotid artery. ALF =; FLP =; HPR =; HRA =; RFA =.

Fig. 2

Representative images illustrating, (A) facial plethora; (B and C) abdominal striae; (D) supraclavicular fat pad; and (E) dorsal fat pad.

Fig. 3

A, Hematoxylin phloxine saffron staining showing an adenoma with a solid growth pattern; (B) immunohistochemical staining showing t-box transcription factor reactivity of tumor nuclei; (C) diffuse cytoplasmic staining for cytokeratin CAM5.2; and (D) regional moderately intense granular cytoplasmic staining for adrenocorticotropic hormone. Scale bar, 20 μm.

Fig. 4

Trend of select pituitary hormonal panel with key clinical events denoted by black arrows. ACTH = adrenocorticotropic hormone; POD = postoperative day.