Invasive ACTH-secreting pituitary macroadenoma in remission after transsphenoidal resection

Zhuang, Zhe; Liu, Xiaohai; Bao, Xinjie; Pan, Boju; Deng, Kan; Yao, Yong; Lian, Wei; Xing, Bing; Zhu, Huijuan; Lü, Lin; Wang, Renzhi; Feng, Ming

doi:10.1097/md.0000000000013148

Cited by 11 publications

(10 citation statements)

References 29 publications

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“…If the tumor extends into the suprasellar region, a transcranial approach may be needed ( 88 ). In a literature review of initial surgery for ACTHomas, a tumor with Knosp grades 3–4 have been identified in 12–20% of all tumors, and 12 of 36 patients (33.3%) have achieved the remission criteria ( 95 ), indicating that further treatment option is emergently required for these tumors. In Nelson’s syndrome, pituitary surgery is the first-line treatment; however, the complete remission rate depends on whether the pituitary tumor extends to the extrasellar region, similar to an ordinal pituitary tumor ( 96 ).…”

Section: Treatmentmentioning

confidence: 99%

Aggressive Cushing’s Disease: Molecular Pathology and Its Therapeutic Approach

et al. 2021

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Cushing’s disease is a syndromic pathological condition caused by adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas (ACTHomas) mediated by hypercortisolemia. It may have a severe clinical course, including infection, psychiatric disorders, hypercoagulability, and metabolic abnormalities, despite the generally small, nonaggressive nature of the tumors. Up to 20% of ACTHomas show aggressive behavior, which is related to poor surgical outcomes, postsurgical recurrence, serious clinical course, and high mortality. Although several gene variants have been identified in both germline and somatic changes in Cushing’s disease, the pathophysiology of aggressive ACTHomas is poorly understood. In this review, we focused on the aggressiveness of ACTHomas, its pathology, the current status of medical therapy, and future prospects. Crooke’s cell adenoma (CCA), Nelson syndrome, and corticotroph pituitary carcinoma are representative refractory pituitary tumors that secrete superphysiological ACTH. Although clinically asymptomatic, silent corticotroph adenoma is an aggressive ACTH-producing pituitary adenoma. In this review, we summarize the current understanding of the pathophysiology of aggressive ACTHomas, including these tumors, from a molecular point of view based on genetic, pathological, and experimental evidence. The treatment of aggressive ACTHomas is clinically challenging and usually resistant to standard treatment, including surgery, radiotherapy, and established medical therapy (e.g., pasireotide and cabergoline). Temozolomide is the most prescribed pharmaceutical treatment for these tumors. Reports have shown that several treatments for patients with refractory ACTHomas include chemotherapy, such as cyclohexyl-chloroethyl-nitrosourea combined with 5-fluorouracil, or targeted therapies against several molecules including vascular endothelial growth factor receptor, cytotoxic T lymphocyte antigen 4, programmed cell death protein 1 (PD-1), and ligand for PD-1. Genetic and experimental evidence indicates that some possible therapeutic candidates are expected, such as epidermal growth factor receptor tyrosine kinase inhibitor, cyclin-dependent kinase inhibitor, and BRAF inhibitor. The development of novel treatment options for aggressive ACTHomas is an emerging task.

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Section: Treatmentmentioning

confidence: 99%

Aggressive Cushing’s Disease: Molecular Pathology and Its Therapeutic Approach

et al. 2021

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“…ACTH secreting pituitary adenomas are known to have a lower prognosis when it comes to large size. Generally, for macroadenomas, it ranges from 12.5-71.1% (2) . However, with the inadequate tumour resection in this patient, the expected prognosis is low.…”

Section: Discussionmentioning

confidence: 99%

Invasive giant pituitary macroadenoma causing Cushing disease; a case report

Gamage

Pathmanathan

Somasundaram

et al. 2021

Sri Lanka J. Diabetes Endocrinol. Metab.

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Introduction: Cushing disease is a rare entity, and among them, giant pituitary adenomas causing Cushing disease is rarer. Here we present a case of Cushing disease secondary to a giant pituitary macroadenoma.Case report: A 41-year-old female with poorly controlled diabetes mellitus and hypertension diagnosed for six months presented with a history of unintentional weight loss, polyuria and polydipsia for the same duration. There was change in her facial appearance with increased pigmentation, frontal balding and she also noted increase in the size of her abdomen. There were features of proximal muscle weakness. Symptoms of hypothyroidism, hypoadrenalism or hyperprolactinemia were absent, and she was amenorrhoeic following hysterectomy done a year back. Visual impairment, headache, abdominal pain, features suggestive of internal malignancy, especially lung and renal, including features of pheochromocytoma were absent. She denied a family history of similar illness.Her BMI was 20.44 kg/m -2 . She had a prominent abdomen, round facies, hirsutism, male pattern hair loss, facial plethora and generalized pigmentation and thinning of skin. She also had hypertension and proximal muscle weakness. Rest of the examination was normal. Investigations revealed hypokalaemia, poorly controlled hyperglycaemia and, non-suppressed overnight dexamethasone suppression test. She had a elevated ACTH level, and a high dose dexamethasone suppression test showed suppression. Pituitary imaging with MRI revealed a 51x 62x 41 mm size sellar and a para sellar mass with heterogeneous echogenicity extending to the right temporal lobe. Further imaging for ectopic ACTH secretion was negative. She underwent transcranial surgery which was unsuccessful due to intraoperative bleeding, and radiotherapy was given postoperatively. Conclusion:Cushing disease due to giant pituitary macroadenomas are rare and may require secondary therapeutic measures in controlling the disease in case of failed surgery.

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“…It indirectly affects the body’s carbohydrate, protein, lipid and water-electrolyte balance. It takes part in maintaining the homeostasis of the neuroimmunoendocrine system [ 83 , 84 , 85 , 86 ]. ACTH is a physiological agonist of the melanocortin system, which affects cell proliferation and maintenance of normal cell homeostasis.…”

Section: Hormones With a Potential Nephroprotective Effectmentioning

confidence: 99%

The Role of Hormones and Trophic Factors as Components of Preservation Solutions in Protection of Renal Function before Transplantation: A Review of the Literature

Ostróżka-Cieślik

Dolińska

2020

Molecules

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Transplantation is currently a routine method for treating end-stage organ failure. In recent years, there has been some progress in the development of an optimal composition of organ preservation solutions, improving the vital functions of the organ and allowing to extend its storage period until implantation into the recipient. Optimizations are mostly based on commercial solutions, routinely used to store grafts intended for transplantation. The paper reviews hormones with a potential nephroprotective effect, which were used to modify the composition of renal perfusion and preservation solutions. Their effectiveness as ingredients of preservation solutions was analysed based on a literature review. Hormones and trophic factors are innovative preservation solution supplements. They have a pleiotropic effect and affect normal renal function. The expression of receptors for melatonin, prolactin, thyrotropin, corticotropin, prostaglandin E1 and trophic factors was confirmed in the kidneys, which suggests that they are a promising therapeutic target for renal IR (ischemia-reperfusion) injury. They can have anti-inflammatory, antioxidant and anti-apoptotic effects, limiting IR injury.

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Invasive ACTH-secreting pituitary macroadenoma in remission after transsphenoidal resection

Cited by 11 publications

References 29 publications

Aggressive Cushing’s Disease: Molecular Pathology and Its Therapeutic Approach

Aggressive Cushing’s Disease: Molecular Pathology and Its Therapeutic Approach

Invasive giant pituitary macroadenoma causing Cushing disease; a case report

The Role of Hormones and Trophic Factors as Components of Preservation Solutions in Protection of Renal Function before Transplantation: A Review of the Literature

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