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

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.

2021 ◽  
Vol 36 (6) ◽  
pp. 1130-1131
Author(s):  
Katelyn Yunes ◽  
Carolina Posada

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.


Author(s):  
Elżbieta Moszczyńska ◽  
Wiesława Grajkowska ◽  
Maria Maksymowicz ◽  
Joanna Malicka ◽  
Mieczysław Szalecki ◽  
...  

Abstract Objectives To describe the case of a 12-year-old girl with a rare plurihormonal pituitary macroadenoma secreting prolactin (PRL), growth hormone (GH), thyroid-stimulating hormone (TSH), and alpha subunit (α-SU). Case presentation The patient experienced recurrent headaches and progressing loss of vision in one eye. During the examination, abnormalities such as tall stature, coarse facial features, enlarged feet and hands, tachycardia, hand tremor, hyperhidrosis, galactorrhea, and goiter were observed. Head magnetic resonance imaging (MRI) revealed a solid tumor in the anterior and middle cranial fossa, measuring 80 × 50 × 55 mm. A stereotactic biopsy revealed plurihormonal Pit-1 positive pituitary adenoma secreting PRL, GH, and TSH. A pituitary hyperfunction with PRL, GH, TSH, and α-SU excess was diagnosed. The patient was successfully treated pharmacologically with dopamine agonists and somatostatin analogue, and a decrease of tumor volume (30%) was achieved. Conclusions When neurosurgery is not possible, long-term pharmacological treatment of plurihormonal pituitary macroadenoma can be a safe and relatively effective alternative.


2019 ◽  
Vol 38 (03) ◽  
pp. 199-202 ◽  
Author(s):  
Ricardo Lourenço Caramanti ◽  
Mário José Goes ◽  
Feres Chaddad ◽  
Lucas Crociati Meguins ◽  
Dionei Freitas de Moraes ◽  
...  

AbstractOrbital schwannomas are rare, presenting a rate of incidence between 1 and 5% of all orbital lesions. Their most common clinical symptoms are promoted by mass effect, such as orbital pain and proptosis. The best complementary exam is the magnetic resonance imaging (MRI), which shows low signal in T1, high signal in T2, and heterogeneous contrast enhancement. The treatment of choice is surgical, with adjuvant radiotherapy if complete resection is not possible. We report the case of a 24-year-old male patient with orbital pain and proptosis, without previous history of disease. The MRI showed a superior orbital lesion compatible with schwannoma, which was confirmed by biopsy after complete resection using a fronto-orbital approach.


2013 ◽  
Vol 12 (1) ◽  
Author(s):  
Dr Ellyda Muhammed Nordin

It is well known that ionizing radiation has an onco-genetic activity and has been implicated in the causation of brain tumors. However, when a new growth appears adjacent to the site of previous tumor, the diagnosis is more toward recurrence. In addition to that, the possible cause might be overlooked, when it occurs many years after radiation treatment. We report a case of radiation-induced meningioma developed 20 years after the patient received radiotherapy for pituitary adenoma.


Author(s):  
E. Ann Ellis ◽  
John R. Guy ◽  
Jeffery R. Fitzsimmons ◽  
Barbara L. Beck

Optic neuritis, a demyelinating autoimmune disease, is usually the initial site of multiple sclerosis (MS). Experimental allergic encephalomyelitis (EAE), the experimental model for MS, is induced by injecting spinal cord emulsion in complete Freund’s Adjuvant into susceptible animals; clinical symptoms of ataxia and hind quarter paralysis develop 8 to 14 days later. Damage to the bloodbrain barrier (BBB) and free radical derived oxidants, including hydrogen peroxide (H2O2), have been implicated in the pathology of EAE and other inflammatory diseases. Cytochemical localization of H2O2 in the optic nerve head of animals with EAE corresponds to areas of extravasation of horseradish peroxidase (HRP) at later stages of EAE. Damage to the BBB is monitored clinically in human cases of MS by magnetic resonance imaging (MRI) of gadolinium-DTPA (Gd-DTPA), contrast agent, leakage. Preliminary studies of EAE with MRI at 4 days post antigen sensitization showed leakage of Gd-DTPA in the optic nerve; light and electron microscopy (TEM) of sections from the same nerve showed substantial inflammation from the optic nerve head to the optic chiasm.


2009 ◽  
Vol 161 (1) ◽  
pp. 195-199 ◽  
Author(s):  
Isabelle Benoit ◽  
Delphine Drui ◽  
Lucy Chaillous ◽  
Benoît Dupas ◽  
Jean-François Mosnier ◽  
...  

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.


Author(s):  
Gulay Simsek Bagir ◽  
Soner Civi ◽  
Ozgur Kardes ◽  
Fazilet Kayaselcuk ◽  
Melek Eda Ertorer

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.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A592-A592
Author(s):  
Safa Ibrahim ◽  
David Wenkert

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.


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 ◽  
...  

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.


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