Silent Corticotroph Adenomas

Ioachimescu, Adriana G.; Eiland, Leslie; Chhabra, Vaninder; Mastrogianakis, Gena M.; Schniederjan, Matthew; Brat, Daniel J.; Pileggi, Anthony V.; Oyesiku, Nelson M.

doi:10.1227/neu.0b013e318257c1f0

Cited by 56 publications

(20 citation statements)

References 22 publications

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“…Up to 60% of SCAs manifest with preoperative hypopituitarism, similar to rates observed in nonfunctioning adenomas [9,10,20,50,61] though one series reported rates as high as 76% in SCAs (n=33) compared to 50% in NFAs (n=126) [19]. Radiologic features are generally similar between SCAs and NFAs [9,10,19,43] though cavernous sinus invasion may be more prevalent in SCAs than in nonfunctioning adenomas [4,20,49,50,63]. One group identified multiple microcysts (<3 mm) of high intensity signal on T2 weighted images in 77% of SCAs compared 21% in CD and 5% of NFAs, and macrocysts in 23% of SCAs [7].…”

Section: Clinical Coursementioning

confidence: 59%

“…New onset postoperative hypopituitarism has been reported in up to half of SCAs [10,50,61] including postoperative adrenal insufficiency [10,29,42,45,46,61] though other studies did not confirm these differences [9,19]. While SCA recurrence rates of up to 60% have been documented [4,5,10,19,50,61], these rates do not differ from those observed with nonfunctioning adenomas [5,8,63].…”

Section: Clinical Coursementioning

confidence: 93%

“…However, in large series of SCAs, 2 groups each reported only 1 case transforming to biochemical Cushing disease but without clinical stigmata [19,61]. In the series of 75 SCAs, three type I SCAs developed CD 7 years on average after initial SCA diagnosis [20].…”

Section: Clinical Coursementioning

confidence: 99%

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Silent corticotroph adenomas

2018

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Purpose-Silent corticotroph adenomas (SCAs) comprise 20% of all corticotroph adenomas and 3-19% of nonfunctioning adenomas (NFAs). As they do not manifest clinical or biochemical hypercortisolism, they are diagnosed after pathologic examination of resected tumor tissue demonstrates positive ACTH expression. While preoperative features are similar to those of NFAs, SCAs may have more cavernous sinus invasion. Further, patients with SCAs tend to have more frequent and earlier recurrences than those with NFAs, often necessitating multiple surgeries and other modalities of treatment. This article reviews the incidence, pathogenesis, and clinical behavior of SCAs.Methods-A systematic literature review was performed using PubMed for information regarding silent corticotroph adenomas.Results-Up to date findings regarding epidemiology, pathogenesis, pathology, clinical presentation, postoperative course, and management of patients with SCAs are presented.Conclusions-This review highlights the necessity of rigorous monitoring for recurrences and hypopituitarism in patients with SCAs.

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Section: Clinical Coursementioning

confidence: 59%

Section: Clinical Coursementioning

confidence: 93%

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Silent corticotroph adenomas

2018

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“…5,10,14,19 Silent corticotroph cell adenomas are mostly macroadenomas and are commonly associated with cystic components; the diagnosis is often delayed when visual disturbances occur. 5,10,14,19 Although the possibility for these tumors to transform into Cushing disease was reported a few years after the first description, 6,17 this phenomenon and its clinical relevance have been poorly considered in the literature. Indeed, just a few similar cases have been reported, mostly in isolated case reports or series not focused on this topic, and no overall view of this phenomenon and its clinical implications has been provided.…”

Section: Discussionmentioning

confidence: 99%

“…5,9,10,14,19 A lesser-known peculiarity of tumors of this subtype is their ability to modify their clinical expression from an endocrinologically silent form to manifest Cushing disease and vice versa. This peculiar and unique clinical behavior of originally silent corticotropic adenomas has been reported sporadically.…”

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confidence: 99%

ACTH adenomas transforming their clinical expression: report of 5 cases

Zoli¹,

Faustini‐Fustini

Mazzatenta³

et al. 2015

FOC

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OBJECT Adrenocorticotropic hormone (ACTH) adenomas have been recognized as a more aggressive and invasive subtype of pituitary adenomas. An additional and clinically relevant peculiarity of these tumors is their ability to modify their clinical expression from a silent form to Cushing disease or vice versa. The aim of this study was to review a series of patients with pituitary adenomas and analyze the clinical implications of the transformation of clinical expression in 5 cases that showed this phenomenon. METHODS The authors retrospectively reviewed a series of patients with pituitary adenoma and collected clinical, biohumoral, and neuroradiological data of those who presented with a transformation from silent ACTH adenomas to functioning tumors or vice versa. In all the cases, preoperative assessment consisted of brain MRI, ophthalmological examination, and complete baseline endocrinological investigation. In patients with clinical and/or biochemical findings suspicious for Cushing syndrome, a low-dose dexamethasone suppression test was performed to rule in or out this diagnosis. Endocrinological evaluations were repeated 1 month after surgery, 3 months after surgery, and every 6 months or annually thereafter. Ophthalmological evaluations and brain MRIs were repeated after 3 months and then every 6 or 12 months thereafter. RESULTS Five patients (2 men and 3 women) included in this series had corticotropic tumors that showed transformation from an endocrinologically silent form to manifest Cushing disease and vice versa. The mean age at presentation was 40 years (range 18–51 years). In 3 of these patients, a transformation from silent to functioning ACTH adenoma with manifest Cushing disease occurred. In 1 patient, the authors observed the transition from a functioning to a silent adenoma with spontaneous resolution of hypercortisolism. Another patient's silent adenoma “shifted” to a functioning adenoma and then regressed back to a silent form with spontaneous resolution of Cushing disease. This patient again developed hypercortisolism, which finally resolved spontaneously. In this series, the transformation occurred after a mean of 3.5 years (range 6 months to 7 years). The shift from an ACTH-silent to a functioning adenoma was observed in 9% of the ACTH-silent adenomas in this series (4 of 44 cases), and the spontaneous remission of Cushing disease to a silent corticotroph cell adenoma occurred in 1.5% of cases of this series (2 of 132 functioning ACTH adenomas). At follow-up (mean 107 months; range 60–177 months), cortisol levels were within normal limits in all 5 cases. However, 1 patient required Gamma Knife radiosurgery and eventually adrenalectomy for disease control to be achieved. CONCLUSIONS The ability of silent ACTH adenomas to transform their secretion pattern poses a challenge for neurosurgeons and endocrinologists. Because the transformation is often unexpected, the clinical and biochemical data can be underestimated. Furthermore, this bizarre and unpredictable postoperative tumor behavior can lead to misinterpretation of clinical and endocrinological outcomes. Even if these cases are very rare, they are not anecdotal in large series. Thus, ACTH adenomas require careful biohumoral and neuroradiological follow-up to detect possible transformations.