neuroblastoma dr. rapp 5.17.2013

7/28/2019 Neuroblastoma Dr. Rapp 5.17.2013

1/14

Morning Report

5/17/2013

Amy Rapp, MD


2/14

11 year old female presents to ED for 1 year of chronicabdominal pain.

Pain is located in RUQ, but child is unable tocharacterize it further, frequent I dont knows

Pain is intermittent and comes every 2-3 months andlasts 2-3 days.

For the last 3 months pain seems to be more frequent.

Recently she has begun to have back pain as well.

No associations with food, though markedly decreasedappetite.

Family has tried Tylenol and heat packs, neither ofwhich has helped.

Family comes to the ED as this is worst pain episodeand the child began to have emesis today.

She denies prior episodes of emesis.


3/14

Marked, reportedly unintentional weight loss thatis concerning to mother, though she doesnt

know how much. Soft, daily normal stools, no diarrhea, no blood or

mucous.

LMP was roughly 1 month prior, 3 lifetimeperiods, still somewhat irregular

Mother has also begun to notice right sidedswelling in her cheek.

Has seen multiple providers (poor continuity) forthese complaints, including PCP, GI, and recentlyENT (for swelling in cheek).


4/14

Work up thus far:

CBC, CMP normal

AXR showed stool on right side of colon

Treated with miralax for constipation Abdominal ultrasound revealed physiologic

right ovarian cyst

ENT biopsied cheek 2 days prior but resultsare not yet available.


5/14

PMH: Term, previously healthy

PSH: None SH: Lives with mom, step-dad, 2 younger

brothers. 6th grade, straight As. Has not missedsignificant amounts of school. Family is Spanish

speaking but child speaks English. FH: No IBD, liver disease, childhood cancers,

eating disorders, mental health disease. MGGMhad lung cancer after smoking for years.

Imm: UTD

Development: Normal


6/14

T 37.5 HR 130s 80 RR 20 BP 125/76 Sat 97% RA

General Appearance: cachectic, alert, does not appear to feel well, apathetic, NAD

Head: NC/ATHEENT: Right sided facial swelling over parotid glandwithout overlying

erythema. Rubbery mass palpable over right parotid gland, not tender to palpation.

There is no induration or fluctuance. The margins of the mandible feel smooth and

continuous without interruption or mass. Dry lips, conjunctivae clear, sclera

anicteric, TMs/OP clear, no nasal discharge, PERRL. Right buccal mucosashows a well healing 1.5cm incision.

Neck: supple, scant anterior cervical LAD

Resp: CTAB, no wheezing or stridor, no increased WOB or retractions

CV: mildly tachycardic, normal rhythm, normal S1 and S2; no murmurs, gallops

Abd: soft, nondistended, +BS, no HSM, no rebound tenderness, no CV tenderness.TTP over RU/RLQ.

Ext: WWP, CR


7/14


8/14

11 yr female with acute on chronic

abdominal pain, vomiting, right sided

facial swelling and weight loss.


9/14

GI

- Hepatitis

- Cholecystitis

- Cholelithiasis

- Celiac

- Pancreatitis

- IBD

- Gastritis

- GERD- PUD/H. pylori

- SMA syndrome

- Lymphadenitis

- Constipation

Neuro- Abdominal migraine

Psych

- Anorexia

- Bulemia

GU

- Ruptured ovarian cyst

- Ectopic pregnancy

ENT

- Parotitis

ID

- UTI/Pyelonephritis

- HIV

ONC- Lymphoma/leukemia

- Sarcoma

- Neuroblastoma


10/14

Work Up

CMP/Mg/Ph/CBC/TSH/free T4/uric acid normal

LDH 926 (


11/14

Abdominal CT

Impression: Right paraspinal soft tissue masswith epidural extension is most consistentwith neuroblastoma. Associated multiple

sclerotic lesions within the bone arecharacteristic of osseous metastases.

Dx: Stage IV Neuroblastoma with multiplebony mets


12/14

Neuroblastoma

~10% of childhood cancer, most commonmalignancy in infants

Generally dx at ~2yrs, 97% before age 10

Derived from neuroblasts of postganglionicsympathetic nervous system.

Prognosis determined by age and stage, bestprognosis if dx


13/14

MC presentation is non-tender abdominal

mass

Most are metastatic at diagnosis

Paraneoplastic syndromes are common on

boards but less so in real life- VIP syndrome: intractable secratory diarrhea

and abdominal distention

- Opsomyoclonus: jerking and random eyemovements, can be associated with ataxia


14/14

Dx: histologic evidence of neuronal origin orevidence ofincreased secretion of catecholamine

metabolites in urine HVA/VMA can be followed for off-therapy

screening

4S: infants

neuroblastoma dr. rapp 5.17.2013

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