Cardiac and vascular tumors: Pathology review

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A 15-year-old boy is being evaluated in the clinic after passing out in school during physical activity class. Medical history is significant for hyperactivity and behavioral problems since childhood. He often gets into fights, talks back to his teachers and has low performance in school. A recent IQ test evaluation was 71. Physical examination reveals a cardiac murmur. An echo is obtained and reveals a cardiac mass. Which of the following additional findings is most likely to be found on further evaluation of this patient?  

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Two people came to the clinic one day. Kara is a 66 year old woman who came to the clinic after noticing gradually developing left arm swelling and redness over the past 3 months. Physical examination reveals a tender purplish lesion along the left armpit. She has a history of hypertension, diabetes, and breast cancer that was treated 10 years ago with a modified radical mastectomy and radiation therapy.

Klay is a healthy 1 year old infant brought by his parents due to a rapidly growing “red bump” on his face. He has no history of trauma, and the lesion didn’t appear elsewhere. Physical examination reveals a raised, bright red nodule on the left side of his face and no other abnormal findings.

Now, both Kara and Klay have vascular tumors. There are many types so it’s best to classify them into benign and malignant tumors. Starting with the most common benign vascular tumor in children; the strawberry hemangioma, where Hemangioma means a benign tumor of the blood vessels. A strawberry hemangioma appears as a superficial, bright red skin lesion that looks kind of like a strawberry, and it commonly affects the face. Histologically, these lesions are confined to the epidermis. Now a typical strawberry hemangioma develops in infancy and grows pretty fast, but fortunately, it goes away on its own by 5 to 10 years of age. So in terms of management, exams like to bring up a very concerned parent, but the correct answer will almost always be to reassure the parent that the lesion will regress without treatment.

Now, a related disorder is cherry hemangioma, which is the most common benign vascular tumor in adults. This tumor appears dark red, like a cherry. Histologically, this lesion extends to the superficial papillary dermis, so they reach much deeper than strawberry hemangiomas. These tumors increase in frequency with age, and unlike strawberry hemangiomas, they do not regress spontaneously.

Cavernous hemangiomas are soft, bluish lesions, and unlike strawberry and cherry hemangiomas, they are usually seated in the deep dermis. The word “cavernous” means cavern-like. So it’s not surprising that histologically, these appear as large, endothelium-lined spaces filled with red blood cells. Cavernous hemangiomas can also be located in organs like the liver, spleen or even the brain. Also, Von-Hippel Lindau syndrome is an autosomal dominant condition that causes numerous tumors and cysts throughout the body, one of these being cavernous hemangiomas of the cerebellum and retina. Look for a history of bilateral pheochromocytoma or renal cell carcinoma.

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