Clinical Assessment and Differential Diagnosis of a Child with by pptfiles


									Clinical Assessment and
Differential Diagnosis of a
Child with Suspected Cancer

Pediatric Resident Education Series
General Points
n Signs and symptoms of cancer are relatively
  non-specific and mimic a variety of more
  common childhood problems
n For an oncologist the index of suspicion for
  cancer is high
n For a primary care physician the opposite is
n You have to think about the possibility of
  cancer before you can make the diagnosis
General Points
n Nothing replaces a thorough medical history, family history and
   physical exam
    n Familial/genetic diseases associated with increased cancer
         n   Neurofibromatosis
         n   Familial polyposis
         n   Li-Fraumeni syndrome
    n   Major categories of diseases linked with an increased cancer
        risk include
         n   Immune deficiencies
         n   Metabolic disorders
         n   Disorders of chromosome stability
    n   Environmental exposures
         n   Previous diagnosis of cancer/cancer therapy
Common things are not always common…

n Symptoms and Signs of cancer mimicking normal
  childhood illnesses for which an initial evaluation for
  cancer is usually Not warranted include:
   n   Generalized malaise, fever, adenopathy
   n   Headache, rhinorrhea, epistaxis, febrile seizure,
       rhinitis, pharyngitis, earache
   n   Nausea, vomiting, diarrhea,
   n   Hepatomegaly, splenomegaly
   n   Hematuria, trouble voiding, vaginitis
   n   Masses (bony or soft tissue), pain/swelling
Symptoms and Signs of Cancer Mimicking Normal
Childhood Illnesses:
Initial Evaluation for Cancer Usually Not Warranted
Symptom / Sign                  Possible Malignancy

n Generalized malaise, fever,   n Lymphoma, leukemia, Ewings
  adenopathy                      (EWS), neuroblastoma (NBL)
n Head & Neck
   n Headache, nausea,             n   Brain tumor, leukemia
   n Febrile Seizure               n   Brain tumor
   n Earache                       n   Soft Tissue Sarcoma (STS)
   n Rhinitis                      n   STS
   n Epistaxis                     n   Leukemia
   n Pharyngitis                   n   STS
   n Adenopathy                    n   NBL, thyroid tumor, STS,
                                       leukemia, lymphoma,
Symptom / Sign                    Possible Malignancy

n Thorax
   n   Extrathoracic
                                        n   STS, PNET
        n   Soft tissue mass
                                        n   EWS, NBL
        n   Bony mass
   n   Intrathoracic
                                        n   Lymphoma, leukemia
        n   Adenopathy

n Abdomen
   n   External:
                                        n   STS, PNET
        n   soft tissue
   n   Internal:
                                        n   NBL, lymphoma,
        n   diarrhea, vomiting,
                                            hepatic tumor,
            hepatomegaly and/or
Symptom / Sign                Possible Malignancy

n Genitourinary
   n   Hematuria                 n   Wilms’, STS
   n   Trouble voiding           n   Prostatic or bladder STS
   n   Vaginitis                 n   STS
   n   Paratesticular mass       n   STS

n Musculoskeletal
   n   Soft tissue mass(es)      n   RMS, other STS, PNET
   n   Bony mass/pain            n   Osteosarcoma, EWS,
                                     Non-Hodgkin’s lymphoma
                                     (NHL), NBL, Leukemia
Signs and Symptoms in the
Child with Cancer
n If the signs and symptoms listed in previous
  table do not subside within a reasonable
  period, a consult with an oncologist is
n Exception to this rule – soft tissue mass in a
  child without a explanatory traumatic event
  warrants an early evaluation
Distribution of Lag Time in Days by
Diagnosis of Common Childhood Cancers
Diagnosis   n     Mean   Median       25th %       75th %
Brain       194   211      93           38           237
Ewing’s     82    182     127           79           255
Hodgkin’s   143   223     136           49           270
Leukemia    908   109      52           20           129
NHL         184   117      62           25           141
NBL         237   120      58           15           164
OS          67    127      98           40           191
RMS         126   127      55           25           161
Wilms’      223   101      31           9            120

                          Table 7-1. Pizzo & Poplack, 4th ed.
Common things are not always common… (part 2)

n Unusual Symptoms and Signs that warrant an
  immediate laboratory and/or imaging studies and
  consultation include:
   n   Hypertension, unexplained weight loss
   n   Focal neurologic abnormalities
   n   Masses
   n   Petechiae, pallor
   n   Adenopathy not responding to antibiotics
   n   Early morning vomiting
   n   Pain waking from sleep, not responsive to
       acetaminophen or NSAIDs
     Symptoms/Signs                  Laboratory, imaging        Major associated tumors
                                   studies, & consultations
Hypertension                      CXR, Abd US                   Renal or abdominal tumor,
Weight loss, sudden onset         Abd US                        Any malignancy
Petechiae                         CBC, manual diff              Leukemia, NBL
Adenopathy unresponsive to Surgical consultation, CXR,          Leukemia, Lymphoma
ABs                        CBC, manual diff
Endocrine abnormalities
nGrowth failure                   nHormonal assays              nPituitary tumors
nElectrolyte disturbances         nCT hypothalamic area         nHypothalamic tumors
nSexual abnormalities             nAbdominal CT                 nGonadal tumors
nCushing’s syndrome               nEndocrine consult            nAdrenal tumors

Brain                             Neurology and/or Neuro-       Brain Tumor
nHeadache, early AM vomiting      Surgery Consultation
nCranial nerve palsy, ataxia      followed by Imaging Studies
nDilated pupil, papilledema
nAfebrile seizures
nHallucinations, aphasia
nUnilateral weakness, paralysis
       Symptoms/Signs               Laboratory, imaging         Major associated tumors
                                  studies, & consultations
Eyes                             Ophthalmologic consultation    Retinoblastoma,
nWhite Spot, proptosis,                                         metastatic neuroblastoma,
blindness                                                       rhabdomyosarcoma (RMS),
nWandering Eye                                                  or other STS
nIntraorbital hemorrhage

Ears                                                            LCH, RMS
nBulging mass external canal     CBC, diff, Imaging studies
nMastoid tenderness, swelling
Puffy face & neck                CBC, diff, imaging studies     Mediastinal tumors
Pharyngeal mass                  CBC, diff, imaging studies     RSM, lymphoma, naso-
                                                                pharyngeal carcinoma
Periodontal mass, loose          Dental consultation, imaging   LCH, Burkitt’s lymphoma,
teeth                            studies                        neuroblastoma, osteosarcoma

Thorax                                                          Soft tissue tumors,
nExtrathoracic: mass             CBC, diff, imaging studies     mediastinal tumors,
nIntrathoracic: coughing, SOB                                   metastatic tumors
without fever or no history of
asthma, allergies
    Symptoms/Signs             Laboratory, imaging      Major associated tumors
                             studies, & consultations
Abdomen/Pelvis               Abd US; CBC, diff          Wilms’ tumor, soft tissue
nIntra-abdominal mass                                   sarcoma, neuroblastoma,
                                                        hepatoblastoma, hepato-
                                                        cellular carcinoma
Genitourinary                                           Germ cell tumor, RMS,
nTestes, vaginal mass        UA, CBC, diff              adrenal tumor
nMasculinization /           US of abdomen/pelvis
Musculoskeletal                                         Osteosarcoma, Ewings
nSoft tissue, bone marrow,   CBC, diff                  sarcoma, leukemia,
and/or pain                  Imaging studies            neuroblastoma, soft tissue
CNS Symptoms Concerning for Brain
n Masses can be suspected on the basis of a
  symptom complex that reflects the site of the
  tumor (seizures, weakness, difficulties with
n Pediatric tumors are often situated such that
  they interfere with CSF circulation resulting in
  increased intracranial pressure
   n   Headaches and vomiting are common
       presenting signs in these cases
Symptoms and/or Signs concerning for
n Unexplained fever > 101oF for more than a
n Petechiae
n Unexplained anemia / pallor
n Generalized lymphadenopathy
n Hepatosplenomegaly
n Bone or joint pain (30%) not relieved with
  pain medications or that wakes from sleep
Conditions Suggesting the Need for Radiographic
Evaluation in Children with Headaches
 n Presence of neurologic abnormality
 n Ocular findings, papilledema
 n Vomiting that is persistent, increasing or preceded by
     recurrent headaches
 n   Changing character of the headache
 n   Recurrent morning headaches or headaches that
     awaken or incapacitate the child
 n   Short stature or deceleration of linear growth
 n   Diagnosis of Neurofibromatosis
 n   Previous history of leukemia or CNS radiation
n Diagnosis
   n Lymph Node is considered large if > 10 mm;
      n Epitrochlear nodes > 5 mm

      n Inguinal node > 15 mm

n Most enlarged lymph nodes in children are related to
   n   Bacterial – Staph and Strep
   n   Atypical mycobacterium
   n   Cat scratch disease
   n   Viral – EBV and other herpes viruses
n Regional or generalized?
   n Generalized more likely malignant (except EBV)
   n Regional adenopathy not involving the head and neck
     more likely malignant
n Characteristics of the enlarged node(s)
   n Hard/rubbery, non-tender, matted (fixed, non-mobile)
     node is more likely malignant
n Location of the adenopathy
   n Adenopathy in the posterior auricular, epitrochlear or
     supraclavicular areas is abnormal
   n Mediastinal adenopathy is frequently malignant
Need for Lymph Node Biopsy is Suggested
by the Following Signs and Symptoms
n Enlarging nodes after 2-3 weeks of antibiotic therapy
n Nodes that are not enlarging but have not diminished
  in 6-8 weeks
n Nodes associated with any abnormal chest X-ray
n Adenopathy with associated weight loss,
  hepatosplenomegaly, unexplained fevers, and/or
  drenching night sweats
n Adenopathy in the posterior auricular, epitrochlear or
  supraclavicular areas
n Abdominal, Thoracic and Soft Tissue Masses
  (without a traumatic explanation)
  n   All require evaluation
Bone and Joint Pain
n Most pain associated with cancer is caused by bone,
  nerve or visceral involvement or encroachment
n Bone pain is usually not an early symptom of cancer
  except for malignancies involving bone
   n   Ewing’s sarcoma, osteosarcoma
        n Come and go early on disappearing for weeks or

n Bone or joint pain is a presenting symptom in about
  30% of patients with ALL
   n   Can be confused with rheumatic diseases
Bone and Joint Pain
n Evaluation should be performed when
  n Bone/joint pain is persistent
  n associated with swelling or mass
  n Limited mobility or joint motion
  n Consistently wakes from sleep at night
  n Not relieved by NSAIDs
Another way to think of things…..
n What is it?
n Where is it?
n Where can it go?

n The answer to any one of the above can help
  answer the other two
Work-up: Two Components
n Staging – find out where the tumor is (and isn’t)
   n X-ray of 1o site
   n CT body; CXR baseline, bone scan
   n Specialty tests
      n Gallium, MIBG

      n Tumor markers (HCG, HVA/VMA, ….

      n Bone marrow

n Evaluate for Complications of the tumor
   n CBC w/manual differential, TPN panel
   n Other studies
      n DIC screen, UA, …
Approach to the diagnosis….
n Tissue diagnosis
   n Incisional biopsy
   n Excisional biopsy
   n Special cases…
       n Calicified suprarenal mass + bone scan – in the

         absence of any desire for biologic studies, might
         consider getting diagnosis from bone marrow
   n FNA vs. excisional biopsy
       n Bias towards excisional -> sufficient sample to be

         representative and to send for special research
         studies (histology, chromosomes, special studies,
         research studies)
n Presenting signs and symptoms of childhood cancer
  are common to many childhood illnesses
n Early diagnosis of cancer may improve outcome
n If the possibility of cancer is not considered, delayed
  diagnosis is the result
n Although the incidence of childhood cancer is low,
  the impact of cancer makes it imperative that all
  professionals have a high index of suspicion of
n Tables from:
   n Principles and Practice of Pediatric Oncology, 4th
     edition, Pizzo PA & Poplack DG eds., Lippicott
     Williams & Wilkins, Philadelphia, 2002

n Bruce Camitta MD
  Michael Kelly MH PhD
  Kelly Maloney MD
  Anne Warwick MD MPH

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