Morning Report 5/17/2013

Amy Rapp, MD

 11 year old female presents to ED for 1 year of chronic abdominal pain. Pain is located in RUQ, but child is unable to characterize it further, frequent I dont knows Pain is intermittent and comes every 2-3 months and lasts 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 decreased appetite. Family has tried Tylenol and heat packs, neither of which has helped. Family comes to the ED as this is worst pain episode and the child began to have emesis today. She denies prior episodes of emesis.

 Marked, reportedly unintentional weight loss that is 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 lifetime periods, still somewhat irregular Mother has also begun to notice right sided swelling in her cheek. Has seen multiple providers (poor continuity) for these complaints, including PCP, GI, and recently ENT (for swelling in cheek).

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 results are not yet available.

 PMH: Term, previously healthy PSH: None SH: Lives with mom, step-dad, 2 younger brothers. 6th grade, straight As. Has not missed significant amounts of school. Family is Spanish speaking but child speaks English. FH: No IBD, liver disease, childhood cancers, eating disorders, mental health disease. MGGM had lung cancer after smoking for years. Imm: UTD Development: Normal

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/AT HEENT: Right sided facial swelling over parotid gland without 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 mucosa shows 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 <2 sec, strong pulses in all ext., no c/c/e Skin: no rashes, skin is pink CNS: grossly intact, moves all ext equally and symmetrically. DTRs symmetric and normal.

11 yr female with acute on chronic abdominal pain, vomiting, right sided facial swelling and weight loss.

 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

Work Up
CMP/Mg/Ph/CBC/TSH/free T4/uric acid normal LDH 926 (<840) UA: 3+ ketones, 2+ protein ESR 115, CRP 16.7 CXR

Admitted to inpatient service for hydration, pain control, consideration of tube feeds and continued work up.

Abdominal CT
Impression: Right paraspinal soft tissue mass with epidural extension is most consistent with neuroblastoma. Associated multiple sclerotic lesions within the bone are characteristic of osseous metastases. Dx: Stage IV Neuroblastoma with multiple bony mets

Neuroblastoma
~10% of childhood cancer, most common malignancy in infants Generally dx at ~2yrs, 97% before age 10 Derived from neuroblasts of postganglionic sympathetic nervous system. Prognosis determined by age and stage, best prognosis if dx <1 yr 40% arise in abdomen within adrenal medulla, 30% abdomen non-adrenal, 20% paravertebral ganglia

 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 eye movements, can be associated with ataxia

 Dx: histologic evidence of neuronal origin or evidence of increased secretion of catecholamine metabolites in urine HVA/VMA can be followed for off-therapy screening 4S: infants <1 yr with dissemination to liver, skin or bone Stage 4 (other than 4S), disease-free survival at 2 years is 20-40% Lowest risk surgery alone Intermediate/High surgery and escalating intensity chemo, +/- radiation or stem cell tx