Hepatic epithelioid hemangioendothelioma associated with acute disseminated encephalomyelitis by a possible paraneoplastic process.
Adem AktasDaniel ProbstBrian Andrew Van TineKathryn MarlowPublished in: Rare tumors (2020)
Epithelioid hemangioendothelioma (EHE) is a low-grade, malignant vascular neoplasm that frequently involves the liver, lungs, bone, and soft tissue. Although not commonly associated with a paraneoplastic syndrome, paraneoplastic syndromes in the setting of EHE have been reported. Acute disseminated encephalomyelitis (ADEM) is an acute, autoimmune, demyelinating disorder of the central nervous system that most commonly occurs after an infection or vaccination. We present the case of a 23 year old female who developed the acute onset of fevers, tremors, right sided hemiplegia, global aphasia, and incontinence of urine and stool. MRI demonstrated findings consistent with a demyelinating disorder and brain biopsy confirmed the diagnosis of ADEM. The patient's work up revealed multiple liver lesions which were biopsy proven EHE. This case report discusses the diagnosis and treatment of two concurrent rare disease processes and the possible association of the processes via a paraneoplastic syndrome.
Keyphrases
- liver failure
- case report
- low grade
- respiratory failure
- drug induced
- aortic dissection
- soft tissue
- high grade
- magnetic resonance imaging
- multiple sclerosis
- hepatitis b virus
- intensive care unit
- computed tomography
- ultrasound guided
- radiation therapy
- magnetic resonance
- subarachnoid hemorrhage
- diffusion weighted imaging
- body composition