A 30-year-old man with a history of epilepsy comes to the office for follow-up. He has a history of recurrent seizures that start as jerky, rhythmic movements of his left leg and gradually spread to involve the entire left side of his body. The patient frequently loses consciousness and has been witnessed to have a generalized tonic-clonic seizure. Neuroimaging has revealed a focal lesion in the right motor area. He has been treated with several antiepilepsy drugs, but his seizures have remained poorly controlled. Surgery is performed to remove the lesion, and the excised brain tissue shows a 1.5-cm mass composed of engorged purplish clusters. Microscopy shows numerous dilated capillaries with an endothelial lining separated by a thin layer of fibrous connective tissue. Which of the following is the most likely diagnosis?

Question

A 30-year-old man with a history of epilepsy comes to the office for follow-up.  He has a history of recurrent seizures that start as jerky, rhythmic movements of his left leg and gradually spread to involve the entire left side of his body.  The patient frequently loses consciousness and has been witnessed to have a generalized tonic-clonic seizure.  Neuroimaging has revealed a focal lesion in the right motor area.  He has been treated with several antiepilepsy drugs, but his seizures have remained poorly controlled.  Surgery is performed to remove the lesion, and the excised brain tissue shows a 1.5-cm mass composed of engorged purplish clusters.  Microscopy shows numerous dilated capillaries with an endothelial lining separated by a thin layer of fibrous connective tissue.  Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

11ec160b_39c1_dc4c_9249_533d6d6013c3_MD0001_00 Cavernous hemangiomas are vascular malformations that frequently involve the deeper tissues of the body, such as the liver and brain. Gross examination of hemangiomas reveals a "mulberry-like" appearance due to their purple vascular clusters. Histologically, they are composed of abnormal, dilated blood vessels with a thin adventitia lacking elastic fibers and smooth muscle. The reduced structural support gives them a tendency to leak, causing recurrent hemorrhage. Most patients with cavernous hemangiomas are asymptomatic, although hemangiomas in the brain may cause neurologic deficits and seizures due to compression of the surrounding tissue and irritation from recurrent bleeding. Surgical resection is indicated for lesions causing intractable epilepsy or progressive neurologic deficits. (Choice B) Ependymoma is a rare central nervous system tumor typically found intracranially in children and in the spinal cord in adults. The tumor cells may arrange themselves around blood vessels and form intervening processes, a histological appearance called perivascular pseudorosettes. (Choice C) Glioblastoma is a common and aggressive brain tumor characterized histologically by areas of necrosis surrounded by tumor cells (pseudopalisading necrosis). (Choice D) Kaposi sarcoma is a tumor that results from infection with human herpesvirus 8. It typically manifests as skin nodules but can also affect the gastrointestinal and respiratory tracts. Microscopic evaluation reveals highly vascular tissue containing elongated spindle cells. (Choice E) Saccular (berry) aneurysms are vascular outpouchings composed of a thickened intima due to myointimal hyperplasia with a stretched adventitial layer. They occur as a result of congenital blood vessel weakness and are found most commonly in the circle of Willis. Intracranial aneurysms are typically asymptomatic unless they rupture. Educational objective: Cavernous hemangiomas are vascular malformations composed of abnormally dilated capillaries separated by thin connective tissue septa. The lack of structural support gives them a tendency to bleed, and those that occur in the brain can present with neurologic deficits and seizures.

A 30-Year-Old Man with a History of Epilepsy Comes to the Office