Abdominal mass Flashcards
Case
CC: abdominal mass
K.P. 2/M , sees you for the first time with the chief complaint of
abdominal distention
HPI:
4 week history of intermittent low grade undocumented fever,
malaise, weight loss, anorexia
3 weeks history of progressive abdominal enlargement with note
of vomiting and anorexia. Consult with a pediatrician, Abdominal
CT scan done showed abdominal solid mass with stippled
calcifications. They were advised to seek further consult with a
specialist
2 weeks PTA, noted with constipation and difficulty urination.
Progression of abdominal enlargement prompted consult
ROS:
(+) fever
(-) cough/colds
(+) vomiting
(+) constipation
(+) bone pain
(-) epistaxis
(-) melena/hematochezia
(-) jaundice
(-) hematuria
PE: BP 90/60 HR 110 RR 24 Temp 37
Weight for age below -3 , Length for age below -3
Pink conjunctiva, anicteric sclerare, no cervical lymphadenopathy
ECE, clear breath sounds
Distended abdomen AG = 50cm, 20x20cm firm nonmoveable mass
Pink nailbeds, (-) edema
Normal external genitalia
Tanner of Breast - 1; Tanner of Pubic Hair – 1
Lab Results:
CBC
Hgb 9 (LOW) 12.0- 15.0 g/dL
Hct 28 (LOW) 36 – 48%
RBC 5 3.5 – 5.5 ml/UL
MCV 80 80-100 FL
MCH 30 25-35 PG
RDW 12 11 – 16 FL
WBC 9.0 4.5 -11.0 K/UL
Segmenters 60 40-74
Lymphocytes 25 14-46
Monocytes 10 4-13
Platelet Count 350 150 – 450 K/UL
Tumor Markers Patient Value Reference Range
Urine VMA 50 2-4 years: <13.0
mg/g creatinine
Urine HVA 55 2-4 years: <13.5
mg/g creatinine
AFP 25 <100ng/L
B – HCG 0.3 3 months-18 years
- 0.8 IU/L or less
Urinalysis: normal
CXR: normal
Abdominal CT scan with Contrast: shows a large heterogeneous low
attenuating lesion with calcification in the right suprarenal area
Bone scan: shows multiple randomly distributed focal lesions
scattered throughout the skeleton, particularly the spine, ribs, and
pelvis.
Bone marrow biopsy: small, round, blue cell tumor cells
What is the primary working impression?
Neuroblastoma (Adrenal gland, Right)
Basis for diagnosis?
History:
o Abdominal mass in childhood is considered a
malignancy unless proven otherwise
o Constitutional sx: fever, weight loss, malaise, anorexia
o Obstructive Sx: vomiting, constipation, difficulty in
urination
o CT scan: abdominal mass with calcification
o Possible mets: (+) bone pain
PE:
o Severely Stunted and severely wasted
o Abdominal mass
Laboratory:
o Anemia - Anemia or other cytopenias suggest bone
marrow involvement
o Urine VMA/HVA – elevated
o Abodminal CT clinches the diagnosis: Most cases of NB
arise in the abdomen, either in the adrenal gland or in
retroperitoneal sympathetic ganglia with characteristic
“calcifications”
o Bones mets in Bone scan: The most common sites of
metastasis are the long bones and skull, bone marrow,
liver, lymph nodes, and skin. Lung metastases are rare,
o Biopsy reveals the small round cell tumor characteristic
of NB
What are the differentials?
- Wilms Tumor
- Hepatoblastoma
- Rhabdomyosarcoma
- GCT’s/Teratoma
- NHL
Rule in and rule out your differentials
—–1. Wilms Tumor—–
Rule In:
age
constitutional signs and symptoms of malignancy
abdominal mass
mets: bone
Rule out: Wilms may present with
hematuria/ hypertension
UA: normal vs findings of
hematuria in Wilms
Urine VMA/HVA – elevation
not seen in Wlims
Abdominal CT clinches the
diagnosis – not renal in
location/ does not present
with calcification in Wilms
Mets: bone, brains, lungs
Histology in biopsy is not
small round cell
—–2. Hepatoblastoma—–
Rule In:
age
constitutional signs and symptoms of malignancy
abdominal mass
mets: bone
Rule Out:
Increased AFP found in hepatoblastoma
Urine VMA/HVA – elevation
not seen
Abdominal CT clinches the
diagnosis
Mets: bone Mets: bone and lungs
Histology in biopsy is not
small round cell
—–3. Rhabdomyosarcoma—–
Age
Constitutional signs and symptoms of malignancy
abdominal mass
mets: bone
histology: small round cell tumor
Rule out:
Urine VMA/HVA- elevation not seen
Abdominal CT clinches the diagnosis
Mets: lungs
—-GCTs/Teratoma—–
Rule In:
Age
Constitutional signs and symptoms of malignancy
abdominal mass (usual sites: sacrococcygeal, 40%, ovary 25%, testicle 12%, brain 5%
Other (including neck and medistinum-18%)
Rule Out:
Tumor markers: High hCG –
chorioCA, germinomas; High
AFP – yolk sac tumor
Urine VMA/HVA – elevation
not seen
Abdominal CT clinches the
diagnosis
mets: lungs
histology in biopsy is not small round cell
—–Non-Hodgkin Lymphoma—–
Rule In:
Age
Constitutional signs and symptoms
abdominal mass
mets: bone
histology: small round cell tumor
Rule out:
Urine VMA/HVA-elevation not seen
Abdominal CT clinches the diagnosis
Mets: lungs
Management for this case?
Management:
Diagnosis: discovered as a mass or multiple masses on plain
radiographs, CT, or MRI . Tumor markers, including homovanillic
acid (HVA) and vanillylmandelic acid (VMA) in urine, are elevated
in 95% of cases and help to confirm the diagnosis. A pathologic
diagnosis is established from tumor tissue obtained by biopsy.
NB can be diagnosed in a typical presentation without a primary
tumor biopsy if the patient has neuroblasts observed in bone
marrow and elevated VMA or HVA in the urine.
Imaging Studies:
o Obtain chest and abdominal radiographs to evaluate
for the presence of a posterior mediastinal mass or calcifications
o A CT scan of the primary site is essential to determine tumor extent.
o In cases of paraspinal masses, MRI aids in determining
the presence of intraspinal tumor and cord compression. Horner syndrome should be evaluated
with an MRI of the neck and head
o A technetium-99 bone scan can also be used to evaluate bone metastases.
Once the diagnosis has been histologically confirmed, and if
chemotherapy is needed, the following tests should be
performed.
o The CBC count, differential, and platelet count should
be evaluated.
o The glomerular filtration rate (GFR) or creatinine
clearance rate is used to establish baseline renal
function prior to platinum-based chemotherapy.
o Uric acid levels are used to assess the added risk from
tumor lysis.
o Liver function tests should be performed to assess
possible metastases and determine baseline results
prior to chemotherapy. These include assessment of
bilirubin, alkaline phosphatase, alanine
aminotransferase (SGPT), total protein, and albumin
levels
o Electrolytes, calcium, and magnesium levels should be
monitored daily during chemotherapy. Deficiencies
should be treated with supplements or changes in
intravenous therapy.
other management
Care is multidisciplinary (pediatric oncology, radiation oncologists,
surgeons, and anesthesiologists, as well as nurse practitioners,
nurses, pharmacists, psychologists, and physical and occupational
therapists dedicated to the special needs of these children)
The most important clinical and biologic prognostic factors
currently used to determine treatment are the:
o age of the patient at diagnosis
o stage of disease
o MYCN status
o Shimada histology
Treatment
Treatment:
Treatment options
Low risk (stage 1, 2)–>Surgery only
Low risk 4s–>Observation only as the tumor regresses on its own
Intermediate risk (stage 3,4
with favorable
characteristics) –> surgery, chemotherapy, and, in some cases, radiation therapy
High risk induction chemotherapy to achieve complete response
or very good partial response
→ resection of the primary
tumor followed by focal
radiation to residual tumor
→ Induction is then followed
by high-dose chemotherapy
and autologous stem cell
transplantation (SCT)
MC malignant abdominal masses in children:
Neuroblastoma, Wilm’s tumor, hepatoblastoma, lymphoma
1yo: hepatoblastoma
2 yo: Wilm’s tumor
3yo: neuroblastoma
Any age: lymphoma
What is lymphoma?
CH 496: LYMPHOMA
- neoplasms of lymphoid cells (lymphocytes, histiocytes,
& precursors)
- 6% of all childhood CAs
- Diffuse, aggressive CA in children
- Can present at 1-5yo, but more common in older
children and adolescents
- 60% NHL, 40% HL
- 1/3 of NHL present with abdominal disease
Patho
- HL: EBV + genetic predisposition à clonal
transformation of B-cells, giving rise to pathognomonic
binucleated Reed-Sternberg cells
- NHL: chromosomal translocation, infection, envt’l
factors, immunodeficiency, chronic inflammation –>
tumor of LN à progressive clonal expansion of B/T
cells, NK cells
What are the clinical manifestations of lymphoma?
CM
1. Abdominal pain
2. Vomiting
3. Diarrhea
4. Abdominal distention
5. Intussusception - >1yo: strong warning for lymphoma
6. Peritonitis
7. ascites
What are the diagnostics for lymphoma?
Dx:
1. CBC, Coagulation studies
2. U/a
3. Tumor markers
4. Plain abd XR/UTZ/CT scan
What is Hodgkin lymphoma?
Hodgkin lymphoma
patho: bimodal age distribution: 20-30 years and >50 years; most common cancer in adolescents and young adults; associated with viral infection infections (EBV, CMV, HHV-6)
Manifestations:
painless lymphadenopathy (cervical, supraclavicular, axillary or inguinal)
mediastinal mass
systemic symptoms (fatigue, anorexia and B symptoms: weight loss, fever and night sweats)
hepatic and splenic enlargement
ANN ARBOR STAGING
Stage I: only one lymph node group involved
Stage II: two LN groups involved on the same side of the diaphragm
Stage III: 2 LB groups involved on both sides of the diaphragm OR extra-lymphatic involvement
Stage IV: diffuse extra-lymphatic site involvement (usually BM, CNS, liver)
Diagnosis: Imaging studies (to determine extent of disease): CXR, CT, PET Scan
Excision biopsy of LN demonstrating Reed-Sternberg Cells (HL) and malignant cells (NHL) (fine needle may not be adequate
Others: to check for organ involvement: CBC, ESR, LDH,Creatinine, AST, ALT
Management: Chemotherapy, radiation therapy
What is Non-Hodgkin Lymphoma (NHL)?
NHL
*60% of all lymphomas in children
-Increasing prevalence with age
-associated with EBV, HIV
4 major subtypes:
lymphoblastic lymphoma, Burkitt lymphoma, diffuse large B-cell lymphoma, anaplastic cell lymphoma
CM: aggressive lymphomas
-Rapidly growing mass
-B symptoms:
*fever, night sweats, and weight loss)
Indolent lymphomas
Slowly growing LAD, hepatomegaly, splenomegaly, or cytopenia
ST. JUDE STAGING
Stage I: single extranodal tumor OR single nodal are involved
Stage II: single extranodal tumor with regional node involvement;
at least 2 nodal tumors on the same side of diaphragm; 2 extranodal tumors on same side of diaphragm
Primary GI tumor
Stage III:2 extranodal tumors on both sides of diaphragm
At least 2 nodal areas on both sides of diaphragm
Intrathoracic tumor
Extensive intraabdominal disease
Stage IV: CNS or bone marrow involvement
Diagnosis: Imaging studies (to determine extent of disease): CXR, CT, PET Scan
Excision biopsy of LN demonstrating Reed-Sternberg Cells (HL) and malignant cells (NHL) (fine needle may not be adequate
Others: to check for organ involvement: CBC, ESR, LDH,Creatinine, AST, ALT
Management: radiation for CNS involvement; chemotherapy for B symptoms, Stage III and IV
Possible BM transplant
What is neuroblastoma?
CH 498: NEUROBLASTOMA
- Occurs anywhere along the sympathetic chain or
adrenal medulla (MC, 35%)
o Abdominal in 65% of cases
- MC extracranial tumor in infants
- 75% <4yo
- Metastatic at dx: 75%
