Spontaneous hemorrhage simulating rapid growth of a benign subperiosteal plexiform neurofibroma

Netta M Blitman1, Jeffrey M Levsky, Esperanza Villanueva-Siles

  • 1Department of Radiology, Children's Hospital at Montefiore, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY 10467, USA. nblitman@montefiore.org

Pediatric Radiology
|July 13, 2007
PubMed

Insights

Spontaneous subperiosteal hemorrhage is a rare complication of von Recklinghausen's disease (neurofibromatosis 1). MR imaging revealed an underlying plexiform neurofibroma, aiding diagnosis and preventing misclassification as malignant transformation.

Area of Science:

  • Medical Imaging
  • Oncology
  • Genetics

Background:

  • Spontaneous subperiosteal hemorrhage is an uncommon complication associated with von Recklinghausen's disease (neurofibromatosis 1).
  • Limited literature exists detailing the specific MR imaging characteristics of this rare condition.
  • Distinguishing this benign entity from malignant tumors is crucial for appropriate patient management.

Observation:

  • A 12-year-old patient with neurofibromatosis 1 presented with a rapidly growing fibular mass.
  • Surgical and pathological examination confirmed subperiosteal hemorrhage within a plexiform neurofibroma.
  • MR imaging successfully visualized the underlying plexiform neurofibroma contributing to the hemorrhage.

Findings:

  • The study highlights the MR imaging features of spontaneous subperiosteal hemorrhage in the context of neurofibromatosis 1.
  • A unique case demonstrates the visualization of an underlying plexiform neurofibroma associated with the hemorrhage.
  • Pathological confirmation supports the diagnosis of hemorrhage into a benign neurofibroma.

Implications:

  • Accurate MR imaging interpretation is vital for differentiating this condition from malignant bone tumors.
  • Early and correct diagnosis can prevent unnecessary and potentially harmful surgical interventions.
  • Understanding the pathogenesis and imaging findings aids in the clinical management of neurofibromatosis 1 patients with subperiosteal hemorrhage.

Related Concept Videos

Hemorrhagic Stroke ll: Pathophysiology01:29

Hemorrhagic Stroke ll: Pathophysiology

A hemorrhagic stroke develops when a cerebral blood vessel ruptures, allowing blood to escape into the surrounding brain tissue, as in intracerebral hemorrhage (ICH), or into the subarachnoid space, as in subarachnoid hemorrhage (SAH). Because the skull is a rigid compartment, the sudden presence of extravascular blood rapidly increases intracranial pressure and compresses adjacent neural structures, leading to immediate tissue injury and impaired cerebral perfusion.Mass Effect and Primary...
Abnormal Proliferation02:23

Abnormal Proliferation

Under normal conditions, most adult cells remain in a non-proliferative state unless stimulated by internal or external factors to replace lost cells. Abnormal cell proliferation is a condition in which the cell's growth exceeds and is uncoordinated with normal cells. In such situations, cell division persists in the same excessive manner even after cessation of the stimuli, leading to persistent tumors. The tumor arises from the damaged cells that replicate to pass the damage to the daughter...
Abnormal Proliferation02:23

Abnormal Proliferation

Under normal conditions, most adult cells remain in a non-proliferative state unless stimulated by internal or external factors to replace lost cells. Abnormal cell proliferation is a condition in which the cell's growth exceeds and is uncoordinated with normal cells. In such situations, cell division persists in the same excessive manner even after cessation of the stimuli, leading to persistent tumors. The tumor arises from the damaged cells that replicate to pass the damage to the daughter...
Hemorrhagic Stroke l: Introduction01:17

Hemorrhagic Stroke l: Introduction

A hemorrhagic stroke is an acute neurological event that occurs when a weakened cerebral blood vessel ruptures, allowing blood to accumulate within or around the brain. The sudden release of blood forms a focal hematoma that increases intracranial pressure, displaces neural tissue, and can obstruct cerebrospinal fluid pathways. These effects may be compounded by intraventricular extension of the hemorrhage, cerebral edema, or compression of adjacent structures, all of which contribute to...
Regulation of Angiogenesis and Blood Supply01:24

Regulation of Angiogenesis and Blood Supply

Rapidly dividing tumors, embryos, and wounded tissues require more oxygen than usual, lowering the oxygen concentration in the blood. At low oxygen or hypoxic conditions, an oxygen-sensitive transcription factor called the hypoxia-inducible factor 1 or HIF1 is activated. HIF1 is a dimeric protein of alpha (ɑ) and beta (β) subunits.  Under optimal oxygen conditions, HIF1β is present in the nucleus while HIF1ɑ remains in the cytosol. HIF1ɑ is hydroxylated by prolyl hydroxylase and factor...
Vascular Spasm01:16

Vascular Spasm

The vascular phase, also known as vasospasm, is the initial stage of hemostasis, crucial for preventing excessive bleeding when a blood vessel is injured. After a vessel is cut, nerves in the damaged area trigger pain and other sensory impulses. Simultaneously, the smooth muscles in the vessel wall contract, resulting in a vascular spasm. This contraction reduces the vessel's diameter at the injury site, slowing or stopping blood loss through the vessel wall. Vascular spasms typically last for...