scholarly journals A corticotroph pituitary adenoma as the initial presentation of familial glucocorticoid deficiency

2009 ◽  
Vol 161 (1) ◽  
pp. 195-199 ◽  
Author(s):  
Isabelle Benoit ◽  
Delphine Drui ◽  
Lucy Chaillous ◽  
Benoît Dupas ◽  
Jean-François Mosnier ◽  
...  
Keyword(s):  
Pituitary Adenoma ◽  
Tumour Size ◽  
Optic Chiasm ◽  
Initial Presentation ◽  
Pituitary Hyperplasia ◽  
Magnetic Resonance Imaging Mri ◽  
Glucocorticoid Deficiency ◽  
Type 3 ◽  
The Relationship

ContextFamilial glucocorticoid deficiency (FGD) is a rare autosomal recessive ACTH-resistance syndrome characterized by glucocorticoid deficiency in the absence of mineralocorticoid deficiency. Here, we report the case of a young woman with a corticotroph pituitary adenoma as the initial presentation of FGD.Case reportA 15-year-old girl was referred to our institution for a 16 mm pituitary adenoma associated with glucocorticoid deficiency. Clinical and biological features were evocative of FGD. DNA sequencing did not identify mutations in either the melanocortin 2 receptor (MC2R) or the MC2R accessory protein genes, indicating type 3 FGD. Despite adequate glucocorticoid replacement, plasma ACTH levels remained increased and pituitary magnetic resonance imaging (MRI) showed a progression of the tumour size resulting in optic chiasm compression with intra-tumoural haemorrhaging. When the patient was 26 years old, it was decided that she would undergo transsphenoidal surgery. The histomorphological analysis identified a well-individualized pituitary adenoma immunoreactive for ACTH. The proband's sister also exhibited type 3 FGD associated with pituitary hyperplasia upon MRI.ConclusionThis case highlights the relationship between FGD and hyperplasia of ACTH-producing cells, potentially leading to histologically proven pituitary corticotroph adenomas. This observation raises the question of the pituitary MRI's significance in the follow-up of FGD.

scholarly journals Pituitary MRI characteristics in 297 acromegaly patients based on T2-weighted sequences

2015 ◽  
Vol 22 (2) ◽  
pp. 169-177 ◽  
Author(s):  
Iulia Potorac ◽  
Patrick Petrossians ◽  
Adrian F Daly ◽  
Franck Schillo ◽  
Claude Ben Slama ◽  
...  
Keyword(s):  
Large Population ◽  
Optic Chiasm ◽  
Research Priority ◽  
Maximal Diameter ◽  
Magnetic Resonance Imaging Mri ◽  
Multimodal Management ◽  
Mri Characteristics ◽  
Weighted Sequences ◽  
The Relationship

Responses of GH-secreting adenomas to multimodal management of acromegaly vary widely between patients. Understanding the behavioral patterns of GH-secreting adenomas by identifying factors predictive of their evolution is a research priority. The aim of this study was to clarify the relationship between the T2-weighted adenoma signal on diagnostic magnetic resonance imaging (MRI) in acromegaly and clinical and biological features at diagnosis. An international, multicenter, retrospective analysis was performed using a large population of 297 acromegalic patients recently diagnosed with available diagnostic MRI evaluations. The study was conducted at ten endocrine tertiary referral centers. Clinical and biochemical characteristics, and MRI signal findings were evaluated. T2-hypointense adenomas represented 52.9% of the series, were smaller than their T2-hyperintense and isointense counterparts (P<0.0001), were associated with higher IGF1 levels (P=0.0001), invaded the cavernous sinus less frequently (P=0.0002), and rarely caused optic chiasm compression (P<0.0001). Acromegalic men tended to be younger at diagnosis than women (P=0.067) and presented higher IGF1 values (P=0.01). Although in total, adenomas had a predominantly inferior extension in 45.8% of cases, in men this was more frequent (P<0.0001), whereas in women optic chiasm compression of macroadenomas occurred more often (P=0.0067). Most adenomas (45.1%) measured between 11 and 20 mm in maximal diameter and bigger adenomas were diagnosed at younger ages (P=0.0001). The T2-weighted signal differentiates GH-secreting adenomas into subgroups with particular behaviors. This raises the question of whether the T2-weighted signal could represent a factor in the classification of acromegalic patients in future studies.

Outcomes of surgical and/or medical treatment in patients with prolactinomas during long-term follow-up: a retrospective single-centre study

2020 ◽  
Vol 0 (0) ◽  
Author(s):  
Gamze Akkus ◽  
Barış Karagun ◽  
Hilal Nur Yaldız ◽  
Mehtap Evran ◽  
Murat Sert ◽  
...  
Keyword(s):  
Medical Treatment ◽  
Tumour Size ◽  
Optic Chiasm ◽  
Initial Therapy ◽  
Post Treatment ◽  
Surgical Removal ◽  
Average Dose ◽  
Single Centre Study ◽  
Pre Treatment

AbstractObjectivesProlactinoma is the most common cause of pituitary tumours. Current medical guidelines recommend dopamine agonists (cabergoline or bromocriptine) as the initial therapy for prolactinoma. However, surgical removal can also be considered in selected cases, such as patients with macroadenomas with local complications (bleeding or optic chiasm pressure) or those not responding to medical treatment.MethodsThe present retrospective study included patients with prolactinomas (n=43; female, 24; male, 19) who were primarily managed with medical (n=32) or surgical (n=11) treatment.ResultsMacroadenoma (n=29.67%) was commonly detected in both genders (female, 54%; male, 84%). Moreover, the mean pre-treatment prolactin levels were similar in both genders (female, 683.3 ± 1347 ng/mL; male, 685.4 ± 805 ng/mL; p=0.226). Surgically treated patients had a greater reduction in tumour size (27.7 ± 17.9 mm pre-treatment vs. 8.72 ± 14.2 mm post-treatment) than non-surgically treated ones (12.5 ± 7.5 mm pre-treatment vs. 4.1 ± 4.2 mm post-treatment; p=0.00). However, the decrease in prolactin levels was similar between the two patient groups (p=0.108). During the follow-up period (10.6 ± 7.0 years), the average cabergoline dose of the patients was 1.42 ± 1.47 mcg/week.ConclusionsAlthough a surgical approach was considered for selected cases of prolactinoma, the average dose used for medical treatment was highly inadequate for the patients in the present study.

scholarly journals ACROSTUDY: the first 5 years

2009 ◽  
Vol 161 (suppl_1) ◽  
pp. S19-S24 ◽  
Author(s):  
Peter J Trainer
Keyword(s):  
Tumour Size ◽  
Safety Data ◽  
Liver Function Tests ◽  
Dose Titration ◽  
Serious Adverse Events ◽  
Once Daily ◽  
Injection Site Reactions ◽  
The Mean ◽  
Magnetic Resonance Imaging Mri

ACROSTUDY is an observational registry intended to collect safety and efficacy data on pegvisomant therapy. A total of 792 patients have been enrolled, of whom 83% had commenced pegvisomant prior to recruitment. The mean follow-up is 1.66 years with the mean duration of pegvisomant therapy 3.31 years representing 2625 patient years of treatment. About 90% of patients were on once daily pegvisomant, and 67% were on monotherapy. Disappointingly, IGF1 was normalised in <70% of patients; furthermore, in 80% of patients with an elevated IGF1, the daily dose of pegvisomant was 20 mg or less. A total of 56 serious adverse events (AEs) were reported, of which 13 were related to pegvisomant. A total of 276 AEs were reported, of which 56 were considered related to pegvisomant. The AEs most frequently attributed to pegvisomant were disturbed liver function tests and injection site reactions. Magnetic resonance imaging (MRI) was available in 684 patients. A total of 411 patients had at least one MRI on pegvisomant compared with a baseline. In 31 patients, a decrease in tumour size has been reported, of whom 20 had previously received radiotherapy. An increase in tumour size has been reported and confirmed in 22 patients. In 11 patients, there was contradictory data on tumour size, while, in six patients, central review of the films failed to confirm increase in tumour size. In conclusion, the safety data are generally reassuring, while the IGF1 normalisation rate is disappointing, which probably reflects a failure of dose titration. Further effort is needed to understand the reasons for the failure of dose titration.

scholarly journals Quantitative Volumetric Analysis Post Transsphenoidal Pituitary Adenoma Surgery

2012 ◽  
Vol 39 (5) ◽  
pp. 600-604 ◽  
Author(s):  
Alireza Mansouri ◽  
Sean Symons ◽  
Michael Schwartz ◽  
Joseph Chen ◽  
Farhad Pirouzmand
Keyword(s):  
Pituitary Adenoma ◽  
Volumetric Analysis ◽  
Point Of View ◽  
Size Difference ◽  
Resonance Imaging ◽  
Residual Mass ◽  
Postoperative Assessment ◽  
The Difference ◽  
Magnetic Resonance Imaging Mri

Background:Computed tomogram (CT) imaging is often used for immediate postoperative assessment of transsphenoidal pituitary adenoma resection while magnetic resonance imaging (MRI) is used for follow-up. The residual mass is known to decrease in size over time but the difference between the two imaging modalities has not been quantified. Our objective was to quantify the size difference of the residual mass on immediate postoperative CT compared with delayed MRI.Methods:Retrospective analysis of 69 patients who had undergone pituitary adenoma resection at our institution between 2004-2010. Sellar and suprasellar diameter, along with the overall volume of the residual mass were measured on both the immediate postoperative CT and delayed MRI.Results:Average preoperative sellar and suprasellar diameter was 22.2 ± 4.6mm and 20.9 ± 5.9mm, respectively. Average sellar residual diameter on immediate postoperative CT (16.5 ± 5.4 mm, 25% reduction) was significantly larger than delayed MRI (10.6 ± 6.2mm, 52% reduction). The average suprasellar component on CT (15.5±6.5mm, 26% reduction) was also significantly larger than that on MRI (3.3 ± 5.4 mm, 84% reduction). The postoperative CT showed a 46% reduction in volume while a 71% reduction was noted on the delayed MRI.Conclusion:A significant reduction in residual mass is noted on delayed MR imaging compared with immediate postoperative CT. Therefore, from a resource management and prognostication point of view, CT should be used for immediate postoperative assessment while delayed MRI should be used to assess operative success and for communication with patients.

scholarly journals Homoeopathic Treatment of Pituitary Adenoma: A Case Report

2020 ◽  
Vol 7 (2) ◽  
pp. 109-128
Author(s):  
Nur-E-Alam Rasel ◽  
Md. Sofiqul Alam ◽  
Md. Mostafizur Rahman Siddique ◽  
Narottam Debnath ◽  
Md. Jahangir Alam ◽  
...  
Keyword(s):  
Case Report ◽  
Pituitary Adenoma ◽  
Pituitary Adenomas ◽  
Combined Therapy ◽  
Optic Chiasm ◽  
Individual Case ◽  
The Body ◽  
Near Future ◽  
The Brain

Most pituitary adenomas are slow-growing and benign, which means they are not cancer and do not spread to other parts of the body. They generally have a slow but severe impact on vision due to compression of the optic nerves, optic chiasm, and cavernous sinus.  However, as they grow big they can put pressure on nearby structures, such as the nerves that connect the eyes to the brain and cause symptoms. Pituitary adenomas are the third most common intracranial tumor and arise from the pituitary gland. An individual case is presented in this paper with radiological evidence (MRI) of a large lobulated intrasellar mass (3.0 cm transversely, 3.5 cm craniocaudally and 2 cm anteroposteriorly) with supra-sellar extension is causing elevation of the floor of the 3rd ventricle of the brain. Pituitary macroadenoma and hemorrhagic foei, pituitary apoplexy, and pituitary adenomas were founded by different CT scan and MRI reports in different laboratories. The patient was treated with constitutional homoeopathic medicines – Natrum muriticum-m/1, 16 doses up to Natrum muriticum-m/20, 16 doses each from 26/04/2014 to 07/08/2017. Before treatment imaging was done several times from 6/04/2011 to 12/01/2012showed complete resolution of the pituitary adenoma and during treatment3 follow-up imaging was done on 25/07/2015 and 06/08/2017. This case report reviews the clinical presentation, homoeopathic management, and treatment of the major classification of pituitary adenomas and call attention to the need for repertorization in individualized homoeopathic prescription. It is hoped that if this type of clinical research continues in the future, homeopathy will have a breakthrough result and it’s symptomatic medical treatment will play a beneficial role in the near future from the deadly evils of various types of chemotherapy, radiotherapy, or combined therapy.

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.

scholarly journals Pancreatic Leiomyosarcoma With Schistosomiasis Hematobia: A Case Report and Literature Review

2021 ◽  
Vol 11 ◽  
Author(s):  
Qiang Li ◽  
Daniel Staiculescu ◽  
Yurong Zhou ◽  
Jiang Chen
Keyword(s):  
Case Report ◽  
Tumor Resection ◽  
Clinical Manifestations ◽  
Malignant Neoplasm ◽  
The Body ◽  
Imaging Characteristics ◽  
Magnetic Resonance Imaging Mri ◽  
The Relationship ◽  
Ct And Mri

Pancreatic leiomyosarcoma (PL) is a very rare, malignant neoplasm with a very poor prognosis. Here, we examine a novel case of PL with schistosomiasis hematobia. The patient had been initially misdiagnosed by the first magnetic resonance imaging (MRI). The second imaging examination demonstrated an enlarged heterogeneous tumor mass in the body-tail of pancreas. Following image analysis, the patient underwent a pancreatectomy, splenectomy and lymph node dissections. Sixteen months after the tumor resection, follow-up computed tomography (CT) and MRI revealed tumor metastasis in the liver and lung. PL has non-specific clinical manifestations and imaging characteristics, making early diagnosis very challenging. When it is difficult to distinguish between benign and malignant pancreatic lesions, short-term imaging follow-up is preferred. In this case report, we discuss the relationship between PL and schistosomiasis hematobia.

scholarly journals SUN-290 Pituitary Hyperplasia Due to Uncontrolled Primary Hypothyroidism: Case Series and Literature Review

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Juan Pablo Godoy Alonso ◽  
Germán González de la Cruz ◽  
Marlon Vladimir Vázquez-Aguirre ◽  
Andrea Rocha Haro ◽  
Karla Krystel Ordaz Candelario ◽  
...  
Keyword(s):  
Pituitary Gland ◽  
Feedback Inhibition ◽  
Case Series ◽  
Optic Chiasm ◽  
Primary Hypothyroidism ◽  
Autoimmune Hypothyroidism ◽  
Hormonal Replacement ◽  
Pituitary Hyperplasia ◽  
History Of

Abstract Background: Pituitary hyperplasia secondary to primary hypothyroidism results from the loss of thyroxine feedback inhibition and the subsequent overproduction of TSH. Case 1: A 18-year-old female presented with a chronic history of spontaneous galactorrhea, headache and malaise. Autoimmune primary hypothyroidism was diagnosed, with elevated TSH of 490 mIU/L (0.3-5) and low fT4 of 0.33 ng/dL (0.63-1.34). Pituitary MRI showed an enlarged pituitary with compression of the optic chiasm. Hormonal replacement with levothyroxine 75 mcg qd was started. Five months later she was asymptomatic, and normal TSH (1.64 mIU/L) and fT4 (0.9 ng/dL) levels. A new MRI revealed normal size of pituitary gland, with no compression of the optic chiasm and an intact infundibulum. Case 2: A 24-year-old female with type 1 diabetes and autoimmune primary hypothyroidism, presented with a five-year history of galactorrhea and oligomenorrhea. She was treated with insulin glargine 20U qd, and levothyroxine 200 mcg/day. However, patient’s adherence was bad. She consulted a primary health physician who suspected a prolactinoma after high prolactin levels (77.65, normal 2.64-13.13 ng/mL). Cabergoline was started without any clinical improvement. She then was referred to our service for follow-up. TSH results showed 500 mIU/L, with low fT4 (0.08 ng/dL). Prolactin levels was normal. Pituitary MRI revealed diffuse enlargement of the gland, with compression of infundibulum and optic chiasm. Treatment was modified to levothyroxine/liothyronine 100/20mcg 1 ½ tablet qd. After 7 months, we confirmed normal TSH (0.76 mIU/L) and fT4 (1.23 ng/dL), and the patient was asymptomatic. After 17 months, new MRI showed normal pituitary gland without any compression. Case 3: A 23-year-old female with a history of Addison′s disease and hypothyroidism diagnosed at age 17 presented with a 6-month history of somnolence, fatigue, headache and amenorrhea. She was previously treated with hydrocortisone 25mg/day, fludrocortisone 0.1mg/day, and levothyroxine 200mcg/day. Patient’s adherence was bad, and multiple hospitalizations because of adrenal crises were reported. Her initial hormonal evaluation revealed high TSH of 460 mIU/L and low fT4 of 0.25 ng/dL, mild hyperprolactinemia (32.16 ng/mL), and very high ACTH levels (2,700 pg/mL, normal 10-100). Pituitary MRI revealed an enlarged pituitary with mild compression of the optic chiasm. Hormonal replacement was modified to fasting levothyroxine alternating 200mcg and 300mcg qd. Her last follow-up showed normal TSH (0.53 mIU/L) and fT4 (1.18 ng/dL) levels. New MRI showed normal pituitary size Conclusion: We presented three young women, with autoimmune hypothyroidism, who developed pituitary hyperplasia and responded to proper hormonal replacement normalizing pituitary size. Reference: Endocrinol Diabetes Metab Case Rep. 2015; 2015: 150056.

Vestibular schwannoma and the only hearing ear

1996 ◽  
Vol 110 (4) ◽  
pp. 366-369
Author(s):  
Sanjaya Bhatia ◽  
Sandeep Karmarkar ◽  
Abdelkader Taibah ◽  
Alessandra Russo ◽  
Mario Sanna
Keyword(s):  
Vestibular Schwannoma ◽  
Tumour Size ◽  
Gamma Knife Radiosurgery ◽  
Nerve Function ◽  
Resonance Imaging ◽  
Facial Nerve Function ◽  
Watch And Wait ◽  
Brainstem Compression ◽  
Magnetic Resonance Imaging Mri

AbstractWith the recent advances in the management of vestibular schwannomas, it is possible not only to save the facial nerve function but also preserve hearing in a small percentage of cases. Difficulties arise while managing patients with vestibular schwannoma in their only hearing ear. In this article we summarize our experience in managing seven of these patients. We recommend a watch and wait policy with a regular follow-up with audiometric testing and gadolinium-enhanced magnetic resonance imaging (MRI). Gamma knife radiosurgery is advised in cases with deterioration of hearing or increase in tumour size. Surgery is usually avoided unless there are brainstem compression symptoms.

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

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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