—  SHORT COURSE #63  —

A Practical Approach to the Diagnosis of Common Hematopoietic and Solid Tumors of Childhood
D. Ashley Hill, M.D.
Washington University School of Medicine
St. Louis, MO

Mihaela Onciu, M.D.
St. Jude Children's Research Hospital
Memphis, TN

Click on each Case number below to display the text and references for that section

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Case #1 - Wilms Tumor

Case History:
A five-year-old female presented to her pediatrician with hematuria after being struck in the flank. Physical examination identified a left-sided abdominal mass. Radiographic studies showed a large left renal mass. A left radical nephrectomy was performed. Peri-aortic lymph nodes were sampled.




Case #2 - Neuroblastoma, Stroma-Poor, Differentiating

Case History:
A 14-month-old girl presented with an abdominal mass. CT scan showed a 7.0 x 6.5 x 5.0 cm nodular left adrenal mass with focal calcifications. The mass did not cross the midline. Bone marrow biopsies were normal. A left adrenalectomy was performed.




Case #3 - Alveolar Rhabdomyosarcoma

Case History:
A 1 year-old male presented to his pediatrician with an enlarging buttock mass. Physical examination showed a firm mass in the gluteal region. A pelvic CT scan showed a 10.0 x 8.0 x 7.0 cm mass involving the gluteus muscle with extension into the pelvis. An incisional biopsy was performed.




Case #4 - Pleuropulmonary Blastoma

Case History:
This five month old female presented acutely with fever, tachypnea and labored respirations. She was diagnosed with Influenza B and admitted to the hospital. Born at term with an uneventful neonatal course, she had no significant illnesses prior to admission, although her mother stated that her breathing had not been "normal" for some time. The family history was notable for two relatives with a history of childhood cancer including the patient's mother who had acute B-cell leukemia as a child and a maternal cousin who was being treated for neuroblastoma. A chest X-ray on admission showed hyperaeration of the left lung. A CT scan showed a markedly emphysematous left upper lobe with compressive atelectasis of the left lower lobe and mediastinal shift. There were multiple, fine septations in the hyperinflated lobe with no visible normal lung tissue. The radiographic differential diagnosis included congenital pulmonary airway malformation (CPAM) and congenital lobar emphysema. The patient recovered from her upper respiratory infection and returned approximately three weeks later for resection of the abnormal left upper lobe. At surgery, the left upper lobe showed massive overinflation with attenuation of the visceral pleura. The upper lobe was dissected off the lower lobe with ligation of upper lobe branches of the pulmonary vascular structures and the left upper lobe bronchus. No other cystic abnormalities involving the pleura, mediastinum or left lower lobe were noted.




Case #5 - Anaplastic Large Cell Lymphoma of T/null-cell Phenotype, Systemic Type (ALK-Positive), Monomorphic Variant

Case History:
A15-year-old boy presented with three-week fever, not responding to antibiotics, and isolated left inguinal lymphadenopathy. An excisional lymph node biopsy was performed. The excised inguinal lymph node measured 2.5 cm in greatest dimension. No additional disease was identified at staging work-up.




Case #6 - Anaplastic Large Cell Lymphoma of T/null Cell Phenotype, Systemic Type (ALK Positive), Lymphohistiocytic Variant

Case History:
A 10-year-old boy presented with left cervical lymphadenopathy. An excisional lymph node biopsy was performed. The excised cervical lymph node measured 2.3 cm in greatest dimension. The staging work-up revealed additional lymphadenopathy in the submandibular, paratracheal, axillary and inguinal areas.




Case #7 - Burkitt Lymphoma, Atypical Variant

Case History:
18-year-old boy with a history of blunt trauma to the abdomen followed by persistent abdominal pain. Physical examination prompted a clinical diagnosis of appendicitis and the patient underwent appendectomy. Introperative findings included purulent ascites, a massively enlarged appendix (18 cm in length and approximately 4 cm in diameter), and extensive small bowel involvement by tumor. Further staging work-up revealed extensive bone marrow involvement (ALL, L3) and many blasts present in the peritoneal fluid.




Case #8 - Precursor T-Cell Lymphoblastic Lymphoma

Case History:
12-year-old boy with a large anterior mediastinal mass and left cervical lymphadenopathy. A needle-biopsy of a left cervical lymph node was performed. Further work-up showed normal bone marrow and peripheral blood.




Case #9 - Diffuse Large B-Cell Lymphoma

Case History:
17-year-old previously healthy boy presenting with small bowel obstruction. An abdominal CT showed a terminal ileal mass. The patient underwent terminal ileal resection. The resected mass measured 8 cm in greatest dimension and was situated 13-14 cm proximal to the ileal-cecal valve. The margins of resection were grossly clear of tumor. No additional sites of involvement were found at staging work-up.




Case #10 - Mediastinal (Thymic) Large B-cell Lymphoma

Case History:
14-year-old girl with large anterior mediastinal mass and cervical lymphadenopathy. An incisional biopsy of the cervical lymph node was performed.




Case #11 - Medulloblastoma, Large Cell/Anaplastic Type

Case History:
A 13-year-old girl who presented with headache. Examination showed cranial nerve XI palsy. Radiographic studies showed an enhancing posterior fossa mass and cerebrospinal fluid dissemination of tumor.




Case #12 - Inflammatory Myofibroblastic Tumor

Case History:
18-year-old boy who presented with symptoms of esophageal dysfunction. Radiographic studies showed a lung/mediastinal mass.