Natural history of pituitary apoplexy: a long-term follow-up study.

BACKGROUND AND AIMS: Pituitary apoplexy (PA) is a rare neurosurgical emergency, associated with deficiency of one or more pituitary hormones. Few studies have explored the relative outcomes associated with conservative and neurosurgical intervention. METHODS: A retrospective evaluation of all patients with PA reviewed at Morriston Hospital was undertaken and diagnosis was obtained from Morriston database (Leicester Clinical Workstation database) between 1998 and 2019 from clinic letters and discharge summaries. RESULTS: Thirty-nine patients with PA were identified with a mean age of 74.5 years and 20 (51.3%) patients were women. Patients were followed up for a mean±SD 68.1±61.7 months. Twenty-three (59.0%) patients had a known pituitary adenoma. Commoner clinical presentations of PA were ophthalmoplegia or visual field loss. Following PA, 34 (87.2%) patients were noted to have a non-functioning pituitary adenoma (either pre-existing or new), while 5 (12.8%) patients had a pre-existing functional macroadenoma. Neurosurgical intervention was taken in 15 (38.5%) patients of which 3 (20.0%) patients also received radiotherapy, 2 (13.3%) patients had radiotherapy alone and the remainder managed conservatively. External ophthalmoplegia recovered in all cases. Visual loss remained in all cases. One (2.6%) patient with chromophobe adenoma had a significant second episode of PA requiring repeat surgery. CONCLUSION: PA often occurs in patients with undiagnosed adenoma. Hypopituitarism commonly occurred following conservative or surgical treatment. External ophthalmoplegia resolved in all cases, however, visual loss did not recover. Pituitary tumour recurrence and further PA episodes are rare.

Intrasellar hemorrhagic chordoma masquerading as pituitary apoplexy: case report and review of the literature.

BACKGROUND AND IMPORTANCE: Chordomas are centrally located, expansile soft tissue neoplasms that arise from the remnants of the embryological notochord. Hemorrhagic presentation is exceedingly rare and can resemble pituitary apoplexy. Moreover, a purely intrasellar location of a chordoma is extremely uncommon. We report a case of a hemorrhagic intrasellar chordoma in an adult male, which presented similarly to pituitary apoplexy and was resolved with surgical resection. CLINICAL PRESENTATION: A 69-year-old male presented with a 4 week history of acute onset headache and concurrent diplopia, with significantly reduced testosterone and slightly reduced cortisol. His left eye demonstrated a sixth cranial nerve palsy. Magnetic resonance imaging of the brain showed a large hemorrhagic mass in the pituitary region with significant compression of the left cavernous sinus and superior displacement of the pituitary gland. The patient underwent an endoscopic endonasal transsphenoidal approach for the resection of the lesion. Near total resection was achieved. Final pathology revealed chordoma with evidence of intratumoral hemorrhage, further confirmed by immunopositive stain for brachyury. Post-operatively, the patient had improved diplopia and was discharged home on low dose hydrocortisone. At 3-month follow-up, his diplopia was resolved and new MRI showed stable small residual disease. CONCLUSIONS: Apoplectic chordomas are uncommon given chordoma's characteristic lack of intralesional vascularity and represent a diagnostic challenge in the sellar region. Our unique case demonstrates that despite our initial impression of pituitary apoplexy, this was ultimately a case of apoplectic chordoma that responded well to endoscopic endonasal surgery.

Pituitary Apoplexy: Risk Factors and Underlying Molecular Mechanisms.

Pituitary apoplexy is a rare syndrome, graded from asymptomatic subclinical apoplexy to a life-threatening condition due to pituitary ischemia or haemorrhage of an enlarged pituitary gland. The risk factors and the molecular underlying mechanisms are yet to be elucidated. We provide an overview of the general concepts, the potential factors associated with pituitary adenoma susceptibility for apoplectic events and the molecular mechanisms that could be involved such as HIF-1α/VEGF pathways and metalloproteinases activation, among others. The knowledge of the molecular mechanisms that could participate in the pathogenesis of pituitary apoplexy is crucial to advancement in the identification of future diagnostic tools and therapeutic targets in this rare but sometimes fatal condition.

Pituitary incidentaloma.

Pituitary incidentaloma are being accidentally found during imaging procedures in approximately 10% of normal population. Facing pituitary incidentalomas endocrinologists have to decide about further management based on the assessment of hormonal activity, aetiology, size, and possible compressive symptoms. Treatment of choice for prolactinomas is medical therapy. Surgical treatment is a treatment of choice for other hormonally active tumours and in case of complications as defects of visual field, pituitary apoplexy with compressive symptoms, ophthalmoplegia respectively. Tumour not indicated for surgical treatment are followed-up by imaging techniques and in indicated cases also by endocrinological or visual field evaluation.

Congenital nephrogenic diabetes insipidus accompanied with central nephrogenic diabetes secondary to pituitary surgery -a case report.

BACKGROUND: Diabetes insipidus (DI) can be a common cause of polydipsia and polyuria. Here, we present a case of congenital nephrogenic diabetes insipidus (CNDI) accompanied with central diabetes insipidus (CDI) secondary to pituitary surgery. CASE PRESENTATION: A 24-year-old Chinese woman came to our hospital with the complaints of polydipsia and polyuria for 6 months. Six months ago, she was detected with pituitary apoplexy, and thereby getting pituitary surgery. However, the water deprivation test demonstrated no significant changes in urine volume and urine gravity in response to fluid depression or AVP administration. In addition, the genetic results confirmed a heterozygous mutation in arginine vasopressin receptor type 2 (AVPR2) genes. CONCLUSIONS: She was considered with CNDI as well as acquired CDI secondary to pituitary surgery. She was given with hydrochlorothiazide (HCTZ) 25 mg twice a day as well as desmopressin (DDAVP, Minirin) 0.1 mg three times a day. There is no recurrence of polyuria or polydipsia observed for more than 6 months. It can be hard to consider AVPR2 mutation in female carriers, especially in those with subtle clinical presentation. Hence, direct detection of DNA sequencing with AVPR2 is a convenient and accurate method in CNDI diagnosis.

Apoplexy of Crooke cell tumour leading to the diagnosis of severe Cushing disease; a case report.

BACKGROUND: Patients with Crooke cell tumours present with features of Cushing syndrome or mass effect. There are few reports of patients with Crooke cell tumours presenting due to apoplexy. All of them had silent tumours. Patients with Cushing syndrome caused by Crooke cell tumours have not been reported to present with apoplexy. CASE PRESENTATION: A 35-year-old female presented with sudden onset headache and visual loss for 1 week. She had secondary amenorrhoea for 10 years. There were features of Cushing syndrome including central obesity, multiple monomorphic acne, dorso-cervical and supraclavicular fat pads, hypertension, proximal weakness, pigmentation and refractory hypokalaemia. She was found to have markedly elevated serum cortisol, central hypothyroidism and hypogonadotropic hypogonadism. There was a mass in the sellar region (4.7 cm × 1.9 cm × 5.3 cm) suggestive of a pituitary tumour extending to the suprasellar region. Imaging showed evidence of bleeding and compression of the optic chiasm. She underwent urgent trans-sphenoidal excision of the tumour. Histology was compatible with a pituitary neuroendocrine tumour. There was margination of ACTH reactivity to the cell periphery and ring like positivity in most of the cells in the cytokeratin stain. Features were in favour of a Crooke cell tumour. After surgery she improved gradually and became eucortisolaemic. CONCLUSIONS: This is a unique presentation of an apoplexy of Crooke cell tumour causing Cushing syndrome. Delayed health seeking behaviour of this patient despite severe Cushing disease could have led to this presentation which has not been reported before.

Pituitary apoplexy of a giant prolactinoma during pregnancy.

AIMS AND METHODS: Prolactinomas are a common cause of sexual dysfunction and infertility. We aimed, through this case report, to illustrate the difficulties of management of women with giant prolactinoma, especially in cases of desire of pregnancy. RESULTS: A 30-year-old woman was referred to our department for secondary amenorrhea. Investigations showed a prolactin level of 5168 ng/mL and giant pituitary adenoma of 4 cm in diameter. Cytoreductive surgery was performed after failure to normalize prolactin levels during three years with medical treatment by cabergoline. After seven months, menstrual cycles have resumed, and after 13 months, the patient became pregnant. At 22nd week of gestation, she was admitted in our hospital for pituitary apoplexy. Medical treatment with bromocriptine was chosen. The vaginal premature delivery at 28 weeks gave birth to twins weighing 1 Kg each who died on the 7th day of life. CONCLUSION: This is a relevant clinical case that illustrates the efficacy of cytoreductive surgery in case of insufficient response to dopamine agonists to restore gonadal function. The possibility of a pregnancy should be considered in these patients since it can be associated with high maternal and fetal risks.

Case Report: Polyneuropathy Pituitary Apoplexy with Normal Perimetry and Initially Normal Neuroimaging.

SIGNIFICANCE: Pituitary apoplexy is a syndrome with a varied appearance, which carries a significant risk of morbidity and mortality. It is important to recognize the potential numerous symptoms and clinical findings, urgently investigate with the proper neuroimaging tests, and coordinate care with the appropriate specialists without delay. PURPOSE: This study aimed to describe a patient with worsening headache and ophthalmoparesis attributable to pituitary apoplexy who initially had reportedly a normal neuroimaging result and were diagnosed with migraine. CASE REPORT: A 39-year-old Hispanic man with a history of migraine developed a new and worsening headache. He presented to a hospital emergency department where he underwent a non-contrast-enhanced computed tomography and MRI, whose results were subsequently interpreted as normal. His headache was attributed to migraine, and he was medicated as such and discharged. Three days later, he developed horizontal and vertical diplopia and sought a second opinion. His visual acuity and visual fields were normal. He manifested a right pupil-sparing, external partial cranial nerve III palsy and concurrent right sixth nerve palsy. He also complained of worsening headache and lethargy. He was immediately referred for contrast-enhanced MRI and magnetic resonance angiography with suspicion of pituitary apoplexy. Subsequent imaging revealed a hemorrhagic pituitary macroadenoma consistent with pituitary apoplexy that was expanding laterally into the right cavernous sinus. He underwent immediate neurosurgical surgical repair. CONCLUSIONS: New or worsening headache with signs and symptoms of hypopituitarism should immediately be investigated for pituitary apoplexy. Other possible findings include involvement of cranial nerves III through VI because of cavernous sinus involvement and visual deficits if the optic chiasm or intracranial portion of the optic nerve is involved. However, growth may be lateral, and no visual deficits may be found, as in this case. Multiple concurrent cranial neuropathies should increase suspicion for cavernous sinus involvement.

Pituitary adenoma and apoplexy during GnRH agonist treatment for IVF - case report.

In vitro fertilization is commonly used for treating infertility. One stage of this process is controlled hyperstimulation of the ovaries, achieved by administering gonadotropins. There are several stimulation protocols utilized that increase the number of ovarian follicles during IVF. The most common protocol employs desensitization - the inhibition of follicle-stimulating hormone and luteinizing hormone secretion by the pituitary gland. This is achieved by administering a gonadotropin-releasing hormone (GnRH) analog that is agonistic for the GnRH receptor. However the use of a this drug during therapy carries a risk of complications. This case report deals with a rare case of a woman who underwent pituitary tumor growth as a result of the treatment of GnRH analog.

Coagulative necrotic pituitary adenoma apoplexy: A retrospective study of 21 cases from a large pituitary center in China.

PURPOSE: Coagulative necrotic pituitary apoplexy (CNPA) is a clinical entity with unique intraoperative and histopathological manifestations. We aimed to improve the knowledge of this rare disease through the largest case series published to date. METHODS: A retrospective review of 21 CNPA patients was performed from among 5095 patients who underwent surgery for pituitary adenomas at a single institution between January 2009 and June 2017. The demographic, clinical, endocrine, neuroimaging, intraoperative, and histopathological findings, management and prognosis were summarized. RESULTS: Headache was the most common symptom that was observed in 21 patients, followed by visual disturbances (17/21, 81.0%), nausea and vomiting (16/21, 76.2%), electrolyte disturbance (13/21, 61.9%), and oculomotor palsies (10/21, 47.6%). Hypopituitarism with at least one anterior pituitary deficiency, especially panhypopituitarism (10/21, 47.6%), was present in 81.0% of patients. Most patients (81.0%) showed typical MRI appearances. All 21 patients underwent transsphenoidal surgery (TSS), and 16 patients had total tumor resection demonstrated by postoperative MRI. Cottage cheese-like necrosis was observed in 16 patients (76.2%) intraoperatively. Histopathology showed large areas of pink, acellular, coagulative necrotic areas in the central zone, and a pseudocapsule in the border zone. After follow-up for 4.3 ± 2.3 years, only 28.6% of patients still suffered from corticotropic deficiency, and 9.5% of patients had gonadotropic deficiency. These patients were administered the appropriate corresponding hormones for life. CONCLUSIONS: CNPA can be correctly diagnosed preoperatively by typical clinical and MRI characteristics. Early surgery combined with hyperbaric oxygen therapy early postoperatively usually yields satisfactory endocrine and neuro-ophthalmic outcomes.

Pituitary tumour apoplexy within prolactinomas in children: a more aggressive condition?

OBJECTIVES: To evaluate clinical presentations, diagnosis and management of paediatric patients presenting with pituitary apoplexy. METHODS: A retrospective case series describing a cohort of paediatric patients presenting with this condition from 2010-2016 to a tertiary referral children's hospital in the United Kingdom. RESULTS: Pituitary apoplexy is a rare condition that seems to have a higher relative incidence in children than adults. Our series suggests that pituitary apoplexy in paediatric patients with adenomas appears more common than previously described. All our patients required surgery, either as an acute or delayed procedure, for visual compromise. Two patients had commenced growth hormone (GH) for GH deficiency two weeks prior to the onset of pituitary apoplexy. CONCLUSIONS: With only a limited number of published case reports surrounding this topic our case series contributes to help further understand and manage this condition.

Pituitary Adenoma Apoplexy of the Orbit, Diagnosis, and Management With Presurgical Embolization.

Orbital invasion of pituitary adenomas has been previously reported. In this report, the authors describe a 71-year-old female with a prolactinoma that presented with invasion of and apoplexy within the orbit. The patient underwent exenteration, followed by rapid tumor recurrence and growth. Given the hemorrhagic nature of the tumor, she subsequently underwent preoperative embolization and surgical resection. This case is notable in that it illustrates both apoplexy of a pituitary tumor within the orbit and the benefit of presurgical embolization.

ISCHEMIC PITUITARY APOPLEXY, HYPOPITUITARISM AND DIABETES INSIPIDUS: A TRIAD UNIQUE TO NECROTIZING HYPOPHYSITIS.

- A rare case of necrotizing hypophysitis (NH) in a 52-year-old man presenting with pituitary apoplexy and sterile meningitis is described. This case indicates that the diagnosis of NH could be made without biopsy, based on concomitant presence of diabetes insipidus, hypopituitarism and radiologic features of ischemic pituitary apoplexy. Conservative management of pituitary apoplexy should be advised in NH. Additionally, this is the first report of a case of sterile meningitis caused by ischemic pituitary apoplexy.

Pituitary apoplexy and rivaroxaban.

Pituitary apoplexy (PA), defined by the occurrence of a massive hemorrhagic necrotic rearrangement within a pituitary adenoma, is rare. Its occurrence can be associated with certain risk factors, including anticoagulation. We report the first case of PA with rivaroxaban which is one of the new oral anticoagulants: a 73 year-old patient presenting with severe headache and visual field deterioration. Surgery was performed. Radiotherapy treatment was decided three months after surgery because of tumor residue.