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

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.

scholarly journals A case of pituitary apoplexy in pregnancy

2014 ◽  
Vol 2014 ◽  
Author(s):  
Aimee R Hayes ◽  
Anthony J O'Sullivan ◽  
Mark A Davies
Keyword(s):  
Pituitary Apoplexy ◽  
Rare Event ◽  
Visual Disturbance ◽  
List Type ◽  
The Subject ◽  
Magnetic Resonance Imaging Mri ◽  
Evidence Of Impact ◽  
Learning Points ◽  
Visual Abnormalities ◽  
In Pregnancy

Summary Pituitary apoplexy is a rare event in pregnancy. A 41-year-old woman with a known pituitary microadenoma presented with visual disturbance and headache during the second trimester of pregnancy. Magnetic resonance imaging (MRI) demonstrated pituitary apoplexy with chiasmal compression. After treatment with corticosteroid therapy, she underwent transsphenoidal excision of the pituitary adenoma. Visual abnormalities were completely restored and pituitary function preserved. There was no evidence of impact on the foetus. The literature on the subject is reviewed with emphasis on the management of the apoplectic patient with mild and stable neuro-ophthalmological signs. Learning points There are no clear guidelines on the management of pituitary apoplexy in pregnancy. A multidisciplinary approach can minimise morbidity and mortality. Pituitary apoplexy has an unpredictable clinical course and determining which clinical situations warrant early surgery needs to take into consideration the presence and severity of neurological signs and their stability. The management of conscious apoplectic patients with absent or mild and stable neuro-ophthalmological signs is controversial.

scholarly journals Dense calcification in a GH-secreting pituitary macroadenoma

2014 ◽  
Vol 2014 ◽  
Author(s):  
Ramez Ibrahim ◽  
Atul Kalhan ◽  
Alistair Lammie ◽  
Christine Kotonya ◽  
Ravindra Nannapanenni ◽  
...  
Keyword(s):  
Mutational Analysis ◽  
Residual Disease ◽  
Relevant Literature ◽  
Elevated Serum ◽  
Preoperative Imaging ◽  
Oral Glucose ◽  
Somatostatin Analogue ◽  
List Type ◽  
Pituitary Macroadenoma ◽  
Extensive Calcification

Summary A 30-year-old female presented with a history of secondary amenorrhoea, acromegalic features and progressive visual deterioration. She had elevated serum IGF1 levels and unsuppressed GH levels after an oral glucose tolerance test. Magnetic resonance imaging revealed a heterogeneously enhancing space-occupying lesion with atypical extensive calcification within the sellar and suprasellar areas. Owing to the extent of calcification, the tumour was a surgical challenge. Postoperatively, there was clinical, radiological and biochemical evidence of residual disease, which required treatment with a somatostatin analogue and radiotherapy. Mutational analysis of the aryl hydrocarbon receptor-interacting protein (AIP) gene was negative. This case confirms the relatively rare occurrence of calcification within a pituitary macroadenoma and its associated management problems. The presentation, biochemical, radiological and pathological findings are discussed in the context of the relevant literature. Learning points Calcification of pituitary tumours is relatively rare. Recognising calcification in pituitary adenomas on preoperative imaging is important in surgical decision-making. Gross total resection can be difficult to achieve in the presence of extensive calcification and dictates further management and follow-up to achieve disease control.

scholarly journals The possibilities and impossibilities of treating acromegaly 50 years ago illustrated by Diane Arbus, A Jewish Giant at Home with his Parents, 1970

2021 ◽  
Vol 2021 ◽  
Author(s):  
Wouter W. de Herder
Keyword(s):  
New York ◽  
Pituitary Surgery ◽  
Tumor Formation ◽  
High Dose ◽  
Living Room ◽  
Endocrine Disorders ◽  
List Type ◽  
Pituitary Macroadenoma ◽  
1960S And 1970S ◽  
At Home

Summary The iconic photograph ‘A Jewish Giant at Home with his Parents in the Bronx, NY, 1970’ by the famous American photographer Diane Airbus (1923–1971) shows the 2.34 m (7 ft. 8¼ in.) acromegalic giant Eddie Carmel (1936–1972) and his parents in the living room of their New York home. The picture is a typical example of Arbus’ style. The relationship between the artist and the tall subject is described. A growth hormone-secreting pituitary macroadenoma was unsuccessfully treated with two cycles of pituitary radiotherapy achieving a 7000 rad cumulative dose and by incomplete pituitary surgery. Hypopituitarism was treated according to medical standards in the 1960s and 1970s. The giant patient died of increased intracranial pressure and at autopsy a residual acidophil pituitary macroadenoma was found, but also a perisellar meningioma which was most probably induced by the high dose of pituitary radiotherapy. The case report illustrates the possibilities and impossibilities of treating acromegaly 50 years ago and demonstrates the potential risks of high dose pituitary radiotherapy (in acromegaly). Learning points Acromegaly is a very old disease. Therapy for acromegaly has evolved over the decades. In art museums one can come across artistic impressions of endocrine disorders. People suffering from disfiguring endocrine disorders like acromegaly were pre-WW2 ‘exposed’ in theaters and circuses. High dose pituitary radiotherapy can be associated with secondary brain tumor formation.

scholarly journals SAT-273 Case of Mistaken Identity?

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.

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

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.

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

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.

Chiasmal Syndromes

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.

scholarly journals Phaeochromocytoma and Acromegaly: a unifying diagnosis

2014 ◽  
Vol 2014 ◽  
Author(s):  
C Mumby ◽  
J R E Davis ◽  
J Trouillas ◽  
C E Higham
Keyword(s):  
Pituitary Gland ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Rare Condition ◽  
Ct Scanning ◽  
Pituitary Surgery ◽  
Pathogenic Mutation ◽  
Tolerance Test ◽  
Oral Glucose ◽  
List Type

Summary A 52-year-old lady was referred after a 5 cm left adrenal mass was detected on computed tomography (CT) scanning. She was asymptomatic although was noted to have acromegalic facies. Blood pressure (BP) was normal but plasma normetanephrines were raised to 2.81 mmol/l (<1.09) and urinary normetadrenaline excretion 5.3 μmol/24 h (0–4.3). Adrenal biochemistry screen was otherwise normal. Metaiodobenzylguanidine (MIBG) scan demonstrated uptake in the adrenal lesion. Growth hormone (GH) nadir on oral glucose tolerance test (OGTT) was 2.2 ng/ml with an elevated IGF1 level of 435 ng/ml (72–215), confirming acromegaly biochemically. The remainder of the pituitary screen was normal. A magnetic resonance imaging (MRI) scan of the pituitary revealed an enlarged pituitary gland with a microadenoma/cyst of 2–3 mm in diameter. Alpha blockade was achieved with a titrated dose of phenoxybenzamine before a successful laparoscopic hand-assisted left adrenalectomy. Postoperative biochemical testing revealed a normal plasma normetanephrine level of 0.6 nmol/l (<1.09) and a metanephrine level of 0.35 nmol/l (<0.46 nmol/l). Nadir on OGTT was normal at 0.07 ng/ml with an IGF1 level within the reference range at 111 ng/ml (75–215). Histology demonstrated a well-circumscribed and encapsulated oval mass with microscopic features typical for a phaeochromocytoma. The sections stained strongly positive for GHRH in 20% of cells on immunocytochemistry. Genetic analysis showed no pathogenic mutation. This is a report of the rare condition of a phaeochromocytoma co-secreting GHRH resulting in clinical and biochemical acromegaly. Neuroendocrine tumours can stain positive for GHRH without coexisting acromegaly, but the resolution of patient symptoms and normalisation of serum GH and IGF1 levels following surgery imply that this was functional secretion. Pituitary surgery should be avoided in such cases. Learning points Incidental findings on imaging require thorough investigation to determine the presence of serious pathology. Acromegaly and phaeochromocytoma are rarely coincident in the same patient. If this occurs, co-secretion of GHRH from the phaeochromocytoma or the presence of underlying genetic abnormalities must be considered. Acromegaly is due to ectopic GHRH-secreting neuroendocrine tumours in <1% of cases, most commonly pancreatic or bronchial lesions. Co-secretion of GHRH from a phaeochromocytoma is extremely rare. In such cases, the pituitary gland may appear enlarged but pituitary surgery should be avoided and surgical treatment of the neuroendocrine tumour attempted.

scholarly journals Pachydermoperiostosis: a rare mimicker of acromegaly

2017 ◽  
Vol 2017 ◽  
Author(s):  
Noor Rafhati Adyani Abdullah ◽  
Wong Lok Chin Jason ◽  
Azraai Bahari Nasruddin
Keyword(s):  
Differential Diagnosis ◽  
Serum Insulin ◽  
Rare Condition ◽  
Normal Serum ◽  
Growth Spurt ◽  
Oral Glucose ◽  
List Type ◽  
Excessive Sweating ◽  
Severe Insulin Resistance ◽  
Gh Secretion

Summary Pachydermoperiostosis is a very rare osteoarthrodermopathic disorder whose clinical and radiographic presentations may mimic those of acromegaly. In the evaluation of patients with acromegaloid appearances, pachydermoperiostosis should be considered as a differential diagnosis. In this article, we report a 17-year-old boy who presented with 2-year history of acral enlargement and facial appearance changes associated with joint pain and excessive sweating. He had been investigated extensively for acromegaly, and the final diagnosis was pachydermoperiostosis. Learning points There is a broad range of differential diagnosis for acromegaloid features such as acromegaly, pseudoacromegaly with severe insulin resistance, Marfan’s syndrome, McCune–Albright and a rare condition called pachydermoperiostosis. Once a patient is suspected to have acromegaly, the first step is biochemical testing to confirm the clinical diagnosis, followed by radiologic testing to determine the cause of the excess growth hormone (GH) secretion. The cause is a somatotroph adenoma of the pituitary in over 95 percent of cases. The first step is measurement of a serum insulin-like growth factor 1 (IGF1). A normal serum IGF1 concentration is strong evidence that the patient does not have acromegaly. If the serum IGF1 concentration is high (or equivocal), serum GH should be measured after oral glucose administration. Inadequate suppression of GH after a glucose load confirms the diagnosis of acromegaly. Once the presence of excess GH secretion is confirmed, the next step is pituitary magnetic resonance imaging (MRI). Atypical presentation warrants revision of the diagnosis. This patient presented with clubbing with no gigantism, which is expected in adolescent acromegalics as the growth spurt and epiphyseal plate closure have not taken place yet.

Long-term follow-up of five untreated acromegalic patients

1984 ◽  
Vol 106 (4) ◽  
pp. 437-442 ◽  
Author(s):  
J.W. R. Nortier ◽  
R.J. M. Croughs ◽  
J. H. H. Thijssen ◽  
F. Schwarz
Keyword(s):  
Tolerance Test ◽  
Clinical Activity ◽  
Oral Glucose ◽  
List Type ◽  
Treatment Protocols ◽  
Gh Secretion ◽  
Spontaneous Course ◽  
Long Term Follow Up ◽  
Plasma Gh

Abstract. The present study describes the clinical, biochemical and radiological follow-up of 5 patients with acromegaly, selected from a group of 53 patients, who did not receive treatment during a follow-up period of 5–16 years. The characteristics of these patients included: 1) older age (4 cases), 2) a long history of mild symptoms and signs (4 cases), 3) absence of diabetes mellitus (5 cases), 4) relatively low plasma GH levels (3 cases). The following observations were made during follow-up: clinical activity remained constant in 3 patients and lessonned in 2 patients, the lateral fossa area as calculated from a plain lateral X-ray of the skull remained constant and plasma GH levels measured during an oral glucose tolerance test remained constant in 3 cases, decreased substantially in one case and reached low levels in another case. In this last patient a reproducible increase of plasma GH was found after oral administration of a single dose of 2.5 mg bromocriptine, suggesting complete normality of GH secretion. It is concluded that acromegaly not necessarily progresses in all patients. The results re-emphasize the importance of taking the spontaneous course of pituitary adenomas into account when assessing the effect of various treatment protocols.

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