An Aberrant ACTH-producing Ectopic Pituitary Adenoma in the Sphenoid Sinus

Suzuki, Jirô; Otsuka, Fumio; Ogura, Toshio; Kishida, Masayuki; Takeda, Masaya; Tamiya, Takashi; Nishioka, Tatsuya; Tanaka, Yasushi; Hashimoto, Kozo; Makino, Hírofumi

doi:10.1507/endocrj.51.97

Cited by 33 publications

(24 citation statements)

References 29 publications

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“…Women are affected more than men [5,6]. The various clinical presentations depend on the space-occupying effect of the lesion or hormonal activity.…”

Section: Discussionmentioning

confidence: 99%

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Sphenoid sinus ectopic pituitary adenomas: CT and MRI findings

Yang¹,

Chong²,

Wang³

et al. 2010

BJR

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ABSTRACT. Ectopic pituitary adenomas (EPAs) are rare lesions. The purpose of this study was to describe the CT and MRI features of sphenoid sinus EPAs. Eight patients with histology-proven EPAs in the sphenoid sinus, all of whom underwent CT and MRI, were reviewed retrospectively. The following imaging features were analysed: (i) size, (ii) margin, (iii) CT attenuation characteristics and (iv) MRI signal intensity. In addition, the involvement of adjacent structures and the time-intensity curve (TIC) of dynamic contrast-enhanced (DCE) MRI were analysed. All EPAs had well-defined margins and showed no relationship to the intrasellar pituitary gland. The mean size was 28 mm (range, 20-46 mm). On non-enhanced CT, the lesions appeared isodense to grey matter in 7 (88%) patients and hypodense in 1 (12%) patient. Only two patients underwent post-contrast CT, and they showed moderate enhancement. On T 1 weighted images, EPAs appeared isointense in 6 (75%) patients and hypointense in 2 (25%). On T 2 weighted images, the lesions appeared hyperintense in 2 (25%) patients and isointense in 6 (75%). EPAs showed mild to moderate heterogeneous contrast enhancement and exhibited a cribriform-like appearance. Two patients underwent DCE MRI; the TIC showed a rapidly enhancing and slow washout pattern. The following features were also seen: an empty sella, bone changes and involvement of the cavernous sinus (5 patients; 62.5%). In conclusion, a high index of suspicion for EPA and a familiarity with the imaging findings may help to diagnose this rare entity accurately.

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“…Women are affected more than men [5,6]. The various clinical presentations depend on the space-occupying effect of the lesion or hormonal activity.…”

Section: Discussionmentioning

confidence: 99%

“…A review of the English literature has shown, to date, fewer than 40 cases [1][2][3][4][5][6]. Furthermore, some authors think a pre-operative diagnosis of EPA is often difficult to make.…”

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confidence: 99%

Sphenoid sinus ectopic pituitary adenomas: CT and MRI findings

Yang¹,

Chong²,

Wang³

et al. 2010

BJR

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“…Molecular analysis of circulating ACTH was performed by gel chromatography as previously reported [4,5]. Briefly, the plasma sample was extracted using SEP-PAK C 18 cartridge (Waters, Milford, MA).…”

Section: Analysis Of Preoperative Plasma Acth By Gel Chromatographymentioning

confidence: 99%

Periodic Secretion of Adrenocorticotropin in a Patient with Cushing's Disease Manifested during Pregnancy

et al. 2005

Self Cite

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Abstract. We report the case of 19-year-old woman with cyclical Cushing's disease, in whom plasma adrenocorticotropin (ACTH) was secreted periodically after her first pregnancy. Since the 33rd week of pregnancy, hypertension and proteinuria became clinically remarkable. She gave normal birth at 36th week of pregnancy; however she continued to gain body weight even after delivery and developed typical Cushingoid features. Her ACTH secretion lacked normal daily fluctuation but exhibited periodic change during 1-year observation, showing 119 pg/ml, 34.6 pg/ml and 115 pg/ml at the 4th, 7th and 13th months after delivery. Plasma ACTH levels were increased by corticotropin releasing hormone and metyrapone, while low-dose dexamethasone suppressed cortisol secretion. Gel filtration analysis of the patient's plasma detected big ACTH molecules being eluted with a peak of authentic 1-39 ACTH. Cranial magnetic resonance imaging revealed a 1-cm pituitary mass in right cavernous sinus. The pituitary tumor was removed by transsphenoidal surgery at 13th month after delivery and was pathologically compatible with ACTH-producing pituitary adenoma by immunohistochemistry. This case includes clinically rare subsets of Cushing's syndrome showing periodic ACTH secretion and aberrant ACTH molecules.

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“…Given that the cause was a functional pituitary adenoma, this result appears to be inconsistent with the known feedback-inhibition pattern found in functional ACTH-secreting pituitary adenomas. Suzuki et al 37 performed a literature review and found a variable response to dexamethasone suppression in multiple other cases. It is likely that these tumors behave differently than central adenomas in terms of glucocorticoid suppression.…”

Section: Discussionmentioning

confidence: 99%

Ectopic ACTH-secreting pituitary adenoma of the sphenoid sinus: case report of endoscopic endonasal resection and systematic review of the literature

Seltzer¹,

Lucas²,

Commins³

et al. 2015

FOC

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Ectopic pituitary adenomas are exceedingly rare entities that are often misdiagnosed. The resulting delay in diagnosis may be particularly concerning in the case of Cushing syndrome caused by an ectopic adrenocorticotropic hormone (ACTH)–secreting pituitary adenoma. Although the total resection of ectopic adenomas results in rapid and durable remission, persistent Cushing syndrome is often associated with permanently damaging invasive procedures and significantly higher risk of mortality. The authors report the case of a 48-year-old man with ACTH-dependent Cushing syndrome. On the morning before surgery, his serum cortisol measured 51 μg/dl, his ACTH level was 195.7 pg/ml, and his urinary free cortisol level was 2109 μg/day. Serum cortisol was not suppressed with the administration of high-dose dexamethasone. Imaging showed separate masses in both the sphenoid sinus and the pituitary gland, complicating the diagnostic process and requiring pathological assessment of both masses. No other abnormalities were found on thoracic, abdominal, or pelvic scans. Gross-total resection of both lesions was accomplished via an endoscopic endonasal transsphenoidal approach. Pathology confirmed an ectopic ACTH pituitary adenoma of the sphenoid sinus and a Crooke hyaline change of the pituitary gland. The patient achieved stable hormonal remission without significant postoperative complications, returned to full activity within 3 months, and remained disease free nearly 1 year after tumor resection. In a systematic literature review, the authors identified 41 cases of ectopic ACTH-secreting pituitary adenomas, including 18 arising in the sphenoid sinus without direct involvement of the sella. Including the case described here, the total number of ectopic ACTH pituitary adenomas arising in the sphenoid sinus was 19, and the total number of ectopic ACTH pituitary adenomas without regard to location was 42. For the 19 patients with adenomas found in the sphenoid sinus, ages ranged from 16 to 76 years, and there were 15 women and 4 men. The mean and median diameters of the resected sphenoid masses were 13.9 and 8 mm, respectively, with a range of 3–55 mm. Seven were microadenomas (< 1 cm). Fifteen of the 19 cases reported serum ACTH and morning cortisol levels, the means of which were 106.7 pg/ml and 32.5 μg/dl, respectively. Gross-total tumor resection was achieved in all patients except one, and in all of them durable hormonal remission of Cushing syndrome was achieved (mean follow-up time 20 months). Ectopic pituitary adenomas are rare but important causes of Cushing syndrome and related endocrinopathies, particularly because of the rapid onset and severity of symptoms with atypical presentation. Ectopic pituitary adenomas, especially those in the nasal cavity, nasopharynx, or paranasal sinuses, are easily misidentified. Any patient presenting with signs and symptoms of Cushing syndrome without any obvious pituitary adenoma or other sources of hypercortisolemia should be thoroughly screened for an ectopic adenoma. However, as with the case presented here, the coincident existence of a sellar mass should not preclude the possibility of an ectopic source. There should be a high degree of clinical suspicion for any mass in the general area surrounding the sella when evaluating Cushing syndrome.

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An Aberrant ACTH-producing Ectopic Pituitary Adenoma in the Sphenoid Sinus

Cited by 33 publications

References 29 publications

Sphenoid sinus ectopic pituitary adenomas: CT and MRI findings

Sphenoid sinus ectopic pituitary adenomas: CT and MRI findings

Periodic Secretion of Adrenocorticotropin in a Patient with Cushing's Disease Manifested during Pregnancy

Ectopic ACTH-secreting pituitary adenoma of the sphenoid sinus: case report of endoscopic endonasal resection and systematic review of the literature

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