Related Experiment Video
Updated: Jul 2, 2026
07:43
Endoscopic Endonasal Trans-sphenoidal Approach: Minimally Invasive Surgery for Pituitary Adenomas
Published on: January 17, 2018
Multiple pheochromocytomas in a patient with blurred vision
Kendra L Thoren1, Antonio G Balingit, Jerome Billingsley
1Department of Radiology, Nuclear Medicine Service, Madigan Army Medical Center, Tacoma, Washington 98431, USA.
Clinical Nuclear Medicine
|August 22, 2008
Summary
A 19-year-old male with multiple pheochromocytomas was diagnosed using I-123 metaiodobenzylguanidine (MIBG) scans. Genetic testing confirmed von Hippel-Lindau disease, highlighting the importance of comprehensive diagnostic approaches.
Area of Science:
- Endocrinology
- Oncology
- Genetics
Background:
- Pheochromocytomas are rare neuroendocrine tumors. Von Hippel-Lindau (VHL) disease is a genetic disorder predisposing individuals to various tumors, including pheochromocytomas.
- Early diagnosis and management are crucial for improving patient outcomes and preventing complications.
Observation:
- A 19-year-old male presented with bilateral retinal angiomas, leading to investigations for von Hippel-Lindau (VHL) disease.
- Imaging revealed multiple adrenal and extra-adrenal tumors, initially difficult to differentiate.
- I-123 metaiodobenzylguanidine (MIBG) scintigraphy confirmed the presence of functional pheochromocytomas.
Findings:
- Computed tomography (CT) and magnetic resonance imaging (MRI) localized the tumors but could not definitively differentiate pheochromocytomas.
- I-123 MIBG scan proved highly sensitive and specific for confirming pheochromocytomas and ruling out metastatic disease.
- Genetic testing identified VHL gene mutations associated with von Hippel-Lindau syndrome type II.
Implications:
- This case underscores the utility of I-123 MIBG scintigraphy in diagnosing and localizing pheochromocytomas, especially in the context of genetic syndromes.
- Multidisciplinary management, including surgical resection and genetic counseling, is essential for patients with VHL-associated pheochromocytomas.
- Long-term surveillance with imaging and laboratory tests is recommended for patients with VHL disease to monitor for tumor recurrence or newDevelopments.
Related Concept Videos
01:22Photoreceptors and Visual Pathways
At the molecular level, visual signals trigger transformations in photopigment molecules, resulting in changes in the photoreceptor cell's membrane potential. The photon's energy level is denoted by its wavelength, with each specific wavelength of visible light associated with a distinct color. The spectral range of visible light, classified as electromagnetic radiation, spans from 380 to 720 nm. Electromagnetic radiation wavelengths exceeding 720 nm fall under the infrared category, whereas...
01:25Glaucoma: Overview
Glaucoma is an eye condition characterized by increased intraocular pressure that damages the retina and optic nerve, leading to irreversible blindness if left untreated. The human eye has various components, including the cornea, iris, pupil, lens, and optic nerve. Aqueous humor is secreted by the epithelium of the ciliary body in the posterior chamber and flows through the trabecular meshwork and canal of Schlemm, maintaining normal intraocular pressure. The trabecular meshwork and the canal...
01:19Cushing Syndrome II: Pathophysiology
Cortisol production is normally governed by the hypothalamic–pituitary–adrenal (HPA) axis, which maintains hormonal balance through tightly regulated feedback mechanisms. Disruption of this regulatory system is central to the development of Cushing syndrome, whether the excess cortisol originates from external medications or internal pathology. Persistent cortisol elevation alters metabolism, immune function, and endocrine signaling, producing the characteristic clinical features of the...
01:16Focusing of Light in the Eye
Light rays enter the eye through the cornea, a transparent dome-shaped tissue that is the eye's outermost layer. The cornea bends or refracts, light rays traveling to the pupil. The shape of the cornea determines how much of the light is bent and whether the image will be focused correctly on the retina at the back of the eye. Once the light has passed through both refraction layers, it converges into a single focal point onto a small area. This is where photoreceptors start transforming...
01:27Adrenal Gland Disorders
Adrenal gland disorders manifest when the production of adrenal hormones deviates from the norm, resulting in either excessive or insufficient concentrations.
Adrenal insufficiency, characterized by insufficient cortisol and aldosterone production, leads to conditions like Addison's disease. This disorder, affecting the adrenal cortex, exhibits symptoms such as skin bronzing, dehydration, low blood pressure, fatigue, and weight loss. Congenital adrenal hyperplasia, a genetic ailment causing...
Adrenal insufficiency, characterized by insufficient cortisol and aldosterone production, leads to conditions like Addison's disease. This disorder, affecting the adrenal cortex, exhibits symptoms such as skin bronzing, dehydration, low blood pressure, fatigue, and weight loss. Congenital adrenal hyperplasia, a genetic ailment causing...
01:30Alterations in Blood Pressure
Alterations in blood pressure, such as hypertension (high blood pressure) and hypotension (low blood pressure), significantly affect human health. Understanding these conditions' classifications, causes, and symptoms is essential for effective management and treatment.
Hypertension (High blood pressure)
Hypertension occurs when blood pressure readings consistently exceed the normal range. It is diagnosed when systolic blood pressure (the top number, indicating pressure while the heart beats)...
Hypertension (High blood pressure)
Hypertension occurs when blood pressure readings consistently exceed the normal range. It is diagnosed when systolic blood pressure (the top number, indicating pressure while the heart beats)...