Abstract #507 Panhypopituitarism Secondary to Pituitary Macroadenoma with Optic Chiasm Compression and Possible Apoplexy

Abstract Purpose To evaluate whether the occurrence of chiasmal herniation coincides with visual field (VF) deterioration and to compare the course of VF defects in patients with and without radiological chiasmal herniation following treatment of pituitary adenoma. Methods This retrospective cohort study included 48 pituitary macroadenoma patients with chiasm compression, divided into three groups: Group 1 (N = 12), downward displaced optic chiasm and deteriorated VFs; Group 2 (N = 16), downward displaced optic chiasm; Group 3 (N = 20), control-group matched for tumour size and follow-up VFs, in mean deviation (dB). VFs were compared over time and a severity index, Chiasm Herniation Scale (CHS), for herniation based on radiological parameters was designed. Results After treatment, all groups showed improvement of VFs (Gr1: 2.97 dB p = 0.097, Gr2: 4.52 dB p = 0.001 and Gr3: 5.16 dB p = 0.000), followed by long-term gradual deterioration. The course of VFs between patients with and without herniation was not significantly different (p = 0.143), neither was there a difference in the course before and after herniation (p = 0.297). The median time till onset of herniation was 40 months (IQR 6 month-10 years) and did not significantly differ (p = 0.172) between the groups. There was no relation between VFs and the degree of herniation (p = 0.729). Conclusion Herniation does not appear to have clinical relevance with respect to VF outcome. The newly designed CHS is the first scoring system to quantify the severity of herniation and, in the absence of alternatives, may be useful to describe MRI findings to serve future added value in larger sized outcome studies.

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Loss of Vision after Transsphenoidal Surgery

Neurosurgery ◽

10.1227/00006123-199007000-00008 ◽

1990 ◽

Vol 27 (1) ◽

pp. 60-68 ◽

Cited By ~ 78

Author(s):

Daniel L. Barrow ◽

George T. Tindall

Keyword(s):

Radiation Therapy ◽

Transsphenoidal Surgery ◽

Optic Chiasm ◽

Empty Sella ◽

Significant Loss ◽

Pituitary Macroadenoma ◽

Previous Surgery ◽

Loss Of Vision ◽

Postoperative Hematoma ◽

Direct Injury

Abstract Eleven patients who experienced significant loss of vision after transsphenoidal surgery are reported on. The mechanisms involved in these visual complications include direct injury or devascularization of the optic apparatus, fracture of the orbit, postoperative hematoma, cerebral vasospasm, and prolapse of the optic chiasm into an empty sella. Factors that may increase the risk of visual complications include the presence of a pituitary macroadenoma, previous visual impairment, a “bottleneck” or dumbbell-shaped tumor, previous surgery and/or radiation therapy, and, possibly, use of a lumbar subarachnoid catheter during operation. A practical approach to the management and avoidance of these complications is presented.

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SAT-272 Pituitary Macroadenoma Co-Secreting TSH and PRL Responsive to Cabergoline

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.1940 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Saritha Tirumalasetty ◽

Robert Galagan ◽

Dragana Lovre ◽

Julia David

Keyword(s):

Tumor Size ◽

Pituitary Tumors ◽

Residual Tumor ◽

Optic Chiasm ◽

Pituitary Hormones ◽

Thyroid Stimulating Hormone ◽

Pituitary Macroadenoma ◽

Mri Imaging ◽

Free T4 ◽

Α Subunit

Abstract Background: Thyroid-stimulating hormone (TSH) secreting tumors (TSHoma) account for 0.5-2% of all pituitary adenomas with a prevalence of 1-2 cases per million, indicating that TSHomas are very rare. The majority of TSHomas solely secrete TSH however 9.7% co-secrete Prolactin (PRL). We are reporting a case of co-secreting TSH and PRL pituitary macroadenoma responsive to Cabergoline (CAB) Case: A 52 year old multiparous female presented with symptoms of galactorrhea and amenorrhea. Lab investigation revealed elevated PRL 73 ng/mL (n [normal] = 1-24), Total T3 305 ng/dL (n = 71-180), Free T4 (FT4) 2.37 ng/dL (n = 0.6-1.15), TSH 6.09 UIU/mL (n = 0.5-5.0), and α subunit 7.7 ng/mL. Estradiol was low at 16.9 pg/mL and FSH 6.3 MIU/mL LH 1.7 MIU/mL. Visual field testing showed a right nasal step. MRI imaging demonstrated a 21x24x32mm pituitary macroadenoma with optic chiasm distortion. Partial Transsphenoidal surgery (TSS) was performed and immunostaining of tumor tissue was positive for PRL and negative for other pituitary hormones. One month post-surgical MRI revealed 14x17x15mm residual tumor. One month post-op TFTs were normal: TSH 0.99 ng/dL, FT4 0.61 ng/dL; PRL decreased to 34.8 ng/mL. Six month post-op TSH increased to 5.86 UIU/mL, FT4 1.43 ng/dL, and PRL 44.7 ng/mL. Two years post-op TSH 8.06 ng/dL with elevated α subunit 3.4 ng/mL and PRL 56.8 ng/mL. Octreotide was then initiated for TSHoma treatment however she was unable to tolerate the medication due to diarrhea so was switched to CAB. After starting CAB at 0.5mg twice a week, residual sellar mass increased in size to 19.5x16x23mm with TFTs: TSH 5.66 UIU/mL, FT4 2.48 ng/dL. CAB dose was eventually uptitrated to 1mg twice a week. Repeat MRI showed slight decrease in pituitary lesion to 19x21x18mm and downtrending TFTs: TSH 2.28 UIU/mL, FT4 1.17 ng/dL. Discussion: In patients with pituitary tumors associated with elevated PRL and TSH, TSHoma should be part of the differential diagnosis. This patient’s initial lab evaluation with elevated PRL, TSH, FT4, Total T3, and α subunit confirm the diagnosis of a pituitary macroadenoma with co-secretion of PRL and TSH. Elevated PRL, TSH, FT4 and α subunit levels occurred 6 months after partial TSS resection with growing tumor size eventually requiring medical therapy. On CAB therapy, there were reductions in PRL, TSH, and FT4 levels as well as a decrease tumor size. This is the first reported case of a TSHoma responsive to CAB.

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Stubborn hiccups as a sign of massive apoplexy in a naive acromegaly patient with pituitary macroadenoma

Endocrinology Diabetes and Metabolism Case Reports ◽

10.1530/edm-17-0044 ◽

2017 ◽

Vol 2017 ◽

Author(s):

Gulay Simsek Bagir ◽

Soner Civi ◽

Ozgur Kardes ◽

Fazilet Kayaselcuk ◽

Melek Eda Ertorer

Keyword(s):

Pituitary Apoplexy ◽

Pituitary Surgery ◽

Optic Chiasm ◽

Oral Glucose ◽

List Type ◽

Pituitary Macroadenoma ◽

Large Tumor ◽

Gh Secretion ◽

Transsphenoidal Pituitary Surgery ◽

Magnetic Resonance Imaging Mri

Summary Pituitary apoplexy (PA) may very rarely present with hiccups. A 32-year-old man with classical acromegaloid features was admitted with headache, nausea, vomiting and stubborn hiccups. Pituitary magnetic resonance imaging (MRI) demonstrated apoplexy of a macroadenoma with suprasellar extension abutting the optic chiasm. Plasma growth hormone (GH) levels exhibited suppression (below <1 ng/mL) at all time points during GH suppression test with 75 g oral glucose. After treatment with corticosteroid agents, he underwent transsphenoidal pituitary surgery and hiccups disappeared postoperatively. The GH secretion potential of the tumor was clearly demonstrated immunohistochemically. We conclude that stubborn hiccups in a patient with a pituitary macroadenoma may be a sign of massive apoplexy that may result in hormonal remission. Learning points: Patients with pituitary apoplexy may rarely present with hiccups. Stubborn hiccupping may be a sign of generalized infarction of a large tumor irritating the midbrain. Infarction can be so massive that it may cause cessation of hormonal overproduction and result in remission.

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Macroprolactinoma Presenting With Hyponatremia; A Rare Case Report

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.1207 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A592-A592

Author(s):

Safa Ibrahim ◽

David Wenkert

Keyword(s):

Pituitary Adenoma ◽

Urine Osmolality ◽

Optic Chiasm ◽

Fluid Restriction ◽

Pituitary Macroadenoma ◽

Mechanical Pressure ◽

Tissue Mass ◽

Patient Reported ◽

Rare Case Report ◽

The Right

Abstract Background: Hyponatremia is a common electrolyte abnormality, investigating the etiology can be challenging to the clinician, especially when the cause has rarely been associated with hyponatremia. Macroprolactinoma has rarely been reported as a cause of hyponatremia. We are reporting a case of macroprolactinoma presenting with hyponatremia. Care Report: A 67 year old female previously healthy female, presented with a fall due to syncopal episode. Patient reported lightheadedness for a week prior to presentation. On physical examination, patient had bilateral periorbital ecchymoses, and swelling of the nose. Remaining of physical exam was unremarkable. Lab work showed hyponatremia with high urine osmolality and high urine sodium, consistent with syndrome of inappropriate antidiuretic hormone (SIADH). CT head showed Intrasellar soft tissue mass represents a pituitary macroadenoma. Pituitary hormonal workup showed high prolactin: 2,808 ng/mL, the rest of pituitary profile was normal, including pituitary-adrenal axis evaluation. Pituitary MRI confirmed a large pituitary macroadenoma measuring 1.7 x 2.9 x 2.5 cm, which displaces and compresses the optic chiasm and invades the right cavernous sinus. She was treated with fluid restriction and salt tablets with improvement of sodium level to normal. Cabergoline 0.25 mg twice a week was started with improvement of prolactin level to 156 ng/mL over 9 months period. Discussion: Pituitary macroadenoma rarely associated with hyponatremia, and when reported it is usually secondary to hypopituitarism (low ACTH or low TSH). However, there are few cases reported of SIADH related hyponatremia in the presence of normal pituitary function. Although the exact mechanism of exaggerated production of arginine vasopressin (AVP) is not fully understood, the likely theory thought to be related to the mechanical pressure on the axonal terminal of AVP neurons by the large pituitary tumor. Conclusion: Hyponatremia due to SIADH has been linked in few cases with nonfunctioning pituitary adenoma. Only two cases found in the literature reporting SIADH secondary to prolactin producing pituitary adenoma, with our case being the third reported case.

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SAT-273 Case of Mistaken Identity?

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.1229 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Carlos Pla

Keyword(s):

Muscle Weakness ◽

Pituitary Adenomas ◽

Complete Resolution ◽

Pituitary Apoplexy ◽

Optic Chiasm ◽

Visual Disturbance ◽

Pituitary Macroadenoma ◽

Patient Reported ◽

Work Up

Abstract Introduction Pituitary adenomas occur in 10-15% of patients and the majority are benign. Prolactinomas are the most common form of secretory pituitary adenoma. Pituitary apoplexy, a medical emergency with resulting visual loss and hormonal hyposecretion, requires rapid surgical intervention. We present a case of pituitary macroadenoma that underwent pituitary resection for acute visual disturbance which was later discovered to be caused by undiagnosed demyelinating disease. Clinical Case Patient is a 32-year-old male who presented initially with complain of fatigue and decreased libido. Work up revealed elevated prolactin level and low testosterone. MRI showed a 2x3cm pituitary macroadenoma. At moment of diagnosis, patient was otherwise asymptomatic. He was started on bromocriptine. During follow up visits, patient reported visual disturbance. First MRI in our clinic showed no suprasellar extension, no impingement of optic chiasm and nonspecific white matter disease. At that time, visual field testing showed left temporal defect in superior quadrant. Follow up MRI 1 year later continued to show a stable macroadenoma without impingement of the optic chiasm, but patient reported progressive left vision disturbance and new right vision loss. He was evaluated in the emergency room where he was treated for pituitary apoplexy with steroids and surgery. Vision improved the next day. Despite uncomplicated post-operative course, patient developed proximal muscle weakness and exam notable for diffuse motor deficit in bilateral lower extremities with hyperreflexia. Endocrinology workup was negative for hypercortisolism and ophthalmology diagnosed him with optic neuropathy. Neurology evaluation led to a diagnosis of multiple sclerosis (MS). Patient was started on natalizumab with complete resolution of all visual and muscle symptoms. Clinical lesson Our patient presented with complaints of fatigue, decreased libido and work up that showed a macroprolactinoma without MRI evidence of optic chiasm impingement. During treatment, he developed acute visual deficits that were attributed to pituitary apoplexy. This visual disturbance improved after surgery and use of high dose IV steroids, with the latter likely treating what had been an MS flare. In hindsight, ophthalmologic evaluation before surgery had shown new color blindness, a sign of optic neuropathy. Despite temporary relief, patient progressed to develop new muscle weakness and recurrent visual disturbance which led to the diagnosis of MS. Since being diagnosed and treated for MS, he has had complete resolution of his symptoms. This case stresses the importance of considering other etiologies for visual defects in patients with pituitary adenomas.

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Molecular Profile of a Pituitary Rhabdomyosarcoma Arising From a Pituitary Macroadenoma: A Case Report

Frontiers in Endocrinology ◽

10.3389/fendo.2021.752361 ◽

2021 ◽

Vol 12 ◽

Author(s):

Jinci Lu ◽

Liam Chen

Keyword(s):

Case Report ◽

Pituitary Adenoma ◽

Radiation Treatment ◽

Clinical Symptoms ◽

Mass Effect ◽

Optic Chiasm ◽

Molecular Profile ◽

Pituitary Macroadenoma ◽

Incidental Discovery ◽

Magnetic Resonance Imaging Mri

Pituitary sarcoma arising in association with pituitary adenoma is an uncommon finding. Most cases of secondary sarcoma have been noted to arise with a median interval of 10.5 years post radiation. In this case report, we describe a 77-year-old man with an incidental discovery of a pituitary macroadenoma on magnetic resonance imaging (MRI) and underwent radiotherapy. Three years after radiation treatment, there was an acute change in clinical symptoms and increase in tumor size and mass effect on the optic chiasm which prompted surgical resection. A pituitary adenoma along with a separate spindle-cell sarcomatous component was identified in histology. Immunohistochemical stain for muscle markers confirmed a development of pituitary rhabdomyosarcoma (RMS). Molecular profiling of the tumor identified mutations in TP53, ATRX, LZTR1, and NF1. Despite its rarity, characterization of pituitary RMS with immunohistochemistry and molecular studies may provide an insight to its pathophysiological relationship with pituitary adenoma.

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A-84 Interdisciplinary Collaboration: Neuropsychology’s Role in Elucidating Neurocognitive Symptoms in a Patient with Arachnoid Cyst

Archives of Clinical Neuropsychology ◽

10.1093/arclin/acab062.102 ◽

2021 ◽

Vol 36 (6) ◽

pp. 1130-1131

Author(s):

Katelyn Yunes ◽

Carolina Posada

Keyword(s):

Cystic Lesion ◽

Interdisciplinary Collaboration ◽

Mass Effect ◽

Middle Cranial Fossa ◽

Optic Chiasm ◽

Pituitary Macroadenoma ◽

Cognitive Changes ◽

Recent Onset ◽

Magnetic Resonance Imaging Mri ◽

Psychotic Features

Abstract Objective Arachnoid cysts (ACs) are typically cerebrospinal fluid-filled abnormalities that occur within the central nervous system. ACs are uncommon (prevalence rate of 1.2%), and typically occur within the left hemisphere middle cranial fossa or posterior fossa. Symptoms vary depending on location and size but often include headaches, weakness, cognitive changes, and hydrocephalus. We present an unusual case of a 68-year-old right-handed woman with 13 years of education who was admitted to the hospital following overdose. During a previous admission, Patient was found to have a large arachnoid cystic lesion and right-sided weakness, but was determined not to require acute intervention. Neuropsychology was consulted to assess current cognitive functioning. Method Patient was initially diagnosed with a severe major depressive episode with new-onset psychotic features following an overdose of baclofen and Tylenol. Suicide attempt was denied. Magnetic Resonance Imaging (MRI) confirmed previously identified arachnoid cystic lesion (Figure 1) causing mass effect. Patient’s MRI also revealed pituitary macroadenoma (Figure 2) that caused subtle distortion along the optic chiasm. Results The results (Table 1) suggested an abnormal cognitive profile characterized by deficits on measures of language, visuoperception, memory, and abstract reasoning in the context of relatively intact auditory attention and working memory. Conclusions The extent and severity of Patient’s deficits are likely attributable to the cystic lesion, with recent onset of visual hallucinations likely caused by location of pituitary macroadenoma. This case highlights the importance of having access to a neuropsychological inpatient consultation service to assist with clarification of symptom etiology and facilitation of appropriate diagnosis and intervention.

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Chiasmal Syndromes

Neuro-Ophthalmology ◽

10.1093/med/9780190603953.003.0011 ◽

2019 ◽

pp. 61-66

Author(s):

Matthew J. Thurtell ◽

Robert L. Tomsak

Keyword(s):

Visual Field ◽

Clinical Features ◽

Pituitary Apoplexy ◽

Optic Chiasm ◽

Surgical Decompression ◽

Visual Field Defects ◽

Pituitary Macroadenoma ◽

Visual Recovery ◽

Field Defects ◽

Pituitary Macroadenomas

Dysfunction of the optic chiasm typically produces bitemporal hemianopic visual field defects. Optic chiasmal dysfunction most often results from compression by extrinsic lesions, such as pituitary macroadenomas and meningiomas. In this chapter, we begin by describing the various bitemporal hemianopic visual field defects that can occur with optic chiasmal dysfunction. We next list potential causes of optic chiasmal dysfunction. We then review the clinical features and evaluation of pituitary apoplexy, which results from infarction of (or hemorrhage into) a pituitary macroadenoma. Lastly, we discuss the management of pituitary apoplexy, including the indications for and timing of surgical decompression, and review factors that affect the prognosis for visual recovery.

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SAT-467 A Case of Pituitary Hyperplasia Secondary to Uncontrolled Primary Hypothyroidism

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.1734 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Hebah Alhumaidi ◽

Sarika Rao ◽

Dhruv Kansal

Keyword(s):

Ethinyl Estradiol ◽

Field Testing ◽

Optic Chiasm ◽

Primary Hypothyroidism ◽

Pituitary Macroadenoma ◽

Pituitary Hyperplasia ◽

Morning Cortisol ◽

Free Cortisol ◽

Slight Elevation

Abstract 17 year old female presented for evaluation of galactorrhea of 3 months duration. Patient was diagnosed with papillary thyroid cancer and underwent total thyroidectomy in 2011. She was prescribed Synthroid 175 mcg but was not compliant with taking her medicine. In April 2019 she developed bilateral galactorrhea for which she was evaluated at an outside facility and was found to have a prolactin 143.7ng/mL (n 7.2-63) and TSH 996 mIU/L (n 0.5 -4.0). Pituitary MRI revealed pituitary macroadenoma measuring 1.5 x 1.4 x 1.2 cm with slight elevation of the optic chiasm and infundibulum. She was advised to restart Synthroid and was referred for the neurosurgery team at our facility for surgical resection of pituitary macroadenoma. Over the same period of time, she gained 25 lbs, developed headaches, excessive fatigue, constipation, hair loss, lower extremity swelling, and puffiness of her face. Her menstrual cycles were regular but this was only after she was placed on norelgestromin/ ethinyl estradiol transdermal patch. She denied visual changes. By the time she was seen at our clinic in June 2019, she was taking Synthroid daily for 1 month. TSH decreased to 1.0 mIU/L and prolactin improved to 68 ng/mL. IGF-1 was low at 98 ng/mL (n 149-509). ACTH, morning cortisol, and 24 hour urine free cortisol were within reference range. Visual field testing showed no visual defects. We advised patient to continue taking Synthroid and to follow up in 1 month. On the follow up visit in July 2019, TSH was 0.2 mIU/L, prolactin was 52 ng/mL and IGF-1 was 105 ng/mL. Pituitary MRI showed pituitary hyperplasia that has decreased compared to previous MRI, now measuring around 1 cm at the largest dimension without contact with the optic chiasm and the Infundibulum was at midline. Galactorrhea and headaches resolved and fatigue significantly improved.

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