Open Sesame
Pathlet 010
Nathan Valencia Still '15
with the pathology team

These pathlets are edutainment. This site collects no information about visitors, and cannot substitute for your own doctor's care. There are many questions without clear right-or-wrong answers.

Baby Elmo
Courtesy of Sesame Street
Abby, a 12 month old girl is brought to Sesame Children’s Hospital by her parents after being admitted by her pediatrician. Abby’s parents tell you about a little girl who was delivered by C-section preterm but has been otherwise healthy until about a week ago. “We thought she was just gaining wait and growing, but then she stopped eating so much and continued to swell up,” explains her mom. The child had a red birthmark on the abdomen, which has faded some since she was born.

The baby’s vitals are: pulse 140 bpm, blood pressure 120/55, respirations 50, and temperature of 98.8 F. On physical examination you note the child appears unwell, is tachypnic, has generalized edema and a protuberant abdomen. and has generalized edema without a fluid wave. Her dad tells you, “We’ve also noticed changes in her birthmark.” The birthmark is red and raised compared to the skin around it. The liver edge is seven cm below the costal arch. The neck veins are full but there is little hepatojugular reflux. There is no fluid wave.

Auscultation of her chest and abdomen are significant for tachycardia and crackles bilaterally due to pulmonary edema. There is a loud continuous hum heard over the liver.

After reassuring her parents, you leave the room to discuss the case with your preceptor. After listening to your findings Dr. Grover says, “We need to find the cause of enlarged liver nad increased cardiac output. Focus on her enlarged liver and give me a working differential diagnosis.”

This is a real patient presentation, though the names have been fictionalized. Which of the following do you think accurately describe the child's health?

Biliary obstruction is likely
Child is in compensated shock
Child is in high-output heart failure
Congenital heart disease is likely
Enlarged liver is due primarily to congestion
Hepatic mass is likely
Leukemia is likely
Inferior vena cava syndrome is likely
Neuroblastoma is likely
Portal hypertension is likely
Wilms tumor is likely

The child is in HIGH-OUTPUT HEART FAILURE. The tachycardia, wide pulse pressure (systolic minus diastolic), and pulmonary and systemic edema should enable you to make the diagnosis easily. Since the baby is not anemia or febrile, perhaps there is a shunt between the arterial and venous systems.

Without hepatojugular reflux, it's unlikely that the very big liver is the result of hepatic congestion. So it's time to look for a HEPATIC MASS, or perhaps some liver disease producing liver enlargement.

Which are considerations now?

Hepatocellular adenoma
Hepatocellular carcinoma
Mesenchymal hamartoma

And what shall we order?

Routine chemistries / metabolic panel & electrolytes
Urine homovanillic acid
Plain film X-ray
CT of the abdomen
MRI of the abdomen
Needle biopsy

All the routine labs were normal. Alpha-fetoprotein was normal. Urine homovanillic acid (for neuroblastoma) was normal. There was no thrombocytopenia.

Plain films should not be your primary imaging modality, though calcifications might show up. We hope you did not try to needle-biopsy the liver; it might be some vascular tumor, after all, and there coudl be a terrible hemorrhage.

Abby was sent for a CT scan with injected contrast. These photos are borrowed from "Pediatric Consultant" 9(5), May 2010 (thanks). CT and delayed shot after injection of contrast, showing blood staying around in the mass.


This is an extremely vascular tumor, as shown by the hum and the imaging. Which would you like to do now?

Ask an invasive radiologist to embolize the mass
Get a surgical consult
Perform a needle biopsy of the mass
Send blood to the blood bank in case platelet transfusion become necessary
Treat with interferon-alpha-2A, which has been reported to shrink vascular tumors like this
Treat with prednisone, which has been reported to shrink vascular tumors like this

Again, we hope you did not needle-biopsy the mass, as these things bleed horribly. Embolizing these tumors isn't a good idea. Abby is not thrombocytopenic now, but it's reasonable to ask the blood bank to type and screen her blood as these tumors may start consuming platelets. Abby is probably too sick to wait to see if the mass responds to prednisone or interferon. Some radiologists have embolized these tumors with good results.

Abby went to surgery, and the mass was resectedl leaving enough liver behind. Here are the photomicrographs.


What is the diagnosis?

Hepatocellular adenoma
Hepatocellular carcinoma
Mesenchymal hamartoma

Good for you! We trust you recognized a hemangioblastoma because the tumor is made entirely of one kind of cell -- the kind that lines the vesels. You should have no trouble seeing the anastamosing vascular channels containing red blood cells.

Once you are done, what do you tell the parents?

A genetic workup will reveal the likely outcome
It would be best to send Abby to hospice now
The mass is likely to recur so be vigilant
The prognosis is excellent
We may get a cure with adjuvant chemotherapy


This is a fairly common pediatric tumor, and the most common of the benign liver tumors in children. The many vascular channels allow most of the artierial blood to take the path of least resistance to the venous circulation. If you are hard-core, you noticed this is a type II hemangioendothelioma, as it contains no bile ducts.

Often these are asymptomatic and self-cure, and perhaps most go undetected. The combination of high-output congestive heart failure and a big liver should make you think of this entity.

Children with hemangioblastomas are supposed to be more likely to have prominent vascular hamartomas (red birthmarks) on the skin as well. Nobody really understands how these things form.

Embolization, prednisone, and/or alpha-interferon have been used for symptomatic cases. Here, surgery gave a good result.

Post-operative recovery goes well and the edema present on admission resolves. The cutaneous hemangioma will spontaneously resolve as she gets older and is no cause for concern. If it does not, cosmetic procedures are available to remove or minimize its appearance. Abby’s parents happily leave the hospital with a healthy baby girl.

Nathan Valencia Still KCUMB '15

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