JPOSNA Nov 2020: Tumor Quiz

This tumor quiz represents an interactive session where POSNA members are able to work through 9 clinical scenarios to challenge how they would address these problems.  Associated with each scenario is an OITE-style question and multiple choice options that seek the most preferred answer from the clinician.  In addition to the answer and follow-up radiographs, we have enlisted the opinions of three POSNA experts in oncology to provide an evidenced-based approach to thinking through each tumor problem. 
 
Our panel of experts include:
 
weber
Kristy Weber, MD
Chief of Orthopaedic Oncology at Penn Medicine and Director of the Sarcoma Program at the Abramson Cancer Center, served as the 87th president of the American Academy of Orthopaedic Surgeons (AAOS) and is a past president of the Musculoskeletal Tumor Society.
 
morris
Carol Morris, MD
Professor of Orthopaedic Surgery at John Hopkins University and Division Chief, Orthopaedic Oncology. Vice Chair, Clinical Operations, Orthopaedic Surgery, and president elect Musculoskeletal Tumor Society.
 
tolo
Vernon Tolo, MD
Chief Emeritus, Children's Orthopaedic Center at Children’s Hospital Los Angeles. Professor of Orthopedic Surgery, Keck School of Medicine of USC, past president of the Pediatric Orthopaedic Society of North America, the Scoliosis Research Society, and the American Academy of Orthopaedic Surgeons. Editor Emeritus of the Journal of Bone and Joint Surgery.
 

Quiz authors:

Alexandre Arkader, MD
Pediatric Orthopedics & Orthopedic Oncology
The Children’s Hospital of Philadelphia
Associate Professor of Orthopedic Surgery
Perelman School of Medicine at University of Pennsylvania

Tricia Bhatti, MD
Attending Pathologist
Scientific Director, Pathology Core Laboratory
Co-director, Pediatric Pathology Fellowship Training Program
Assistant Professor of Clinical Pathology and Laboratory Medicine
Perelman School of Medicine at the University of Pennsylvania

This tumor quiz represents an interactive session where POSNA members are able to work through 9 clinical scenarios to challenge how they would address these problems.  Associated with each scenario is an OITE-style question and multiple choice options that seek the most preferred answer from the clinician.  In addition to the answer and follow-up radiographs, we have enlisted the opinions of three POSNA experts in oncology to provide an evidenced-based approach to thinking through each tumor problem. 
 
Our panel of experts include:
 
weber
Kristy Weber, MD
Chief of Orthopaedic Oncology at Penn Medicine and Director of the Sarcoma Program at the Abramson Cancer Center, served as the 87th president of the American Academy of Orthopaedic Surgeons (AAOS) and is a past president of the Musculoskeletal Tumor Society.
 
morris
Carol Morris, MD
Professor of Orthopaedic Surgery at John Hopkins University and Division Chief, Orthopaedic Oncology. Vice Chair, Clinical Operations, Orthopaedic Surgery, and president elect Musculoskeletal Tumor Society.
 
tolo
Vernon Tolo, MD
Chief Emeritus, Children's Orthopaedic Center at Children’s Hospital Los Angeles. Professor of Orthopedic Surgery, Keck School of Medicine of USC, past president of the Pediatric Orthopaedic Society of North America, the Scoliosis Research Society, and the American Academy of Orthopaedic Surgeons. Editor Emeritus of the Journal of Bone and Joint Surgery.
 

Quiz authors:

Alexandre Arkader, MD
Pediatric Orthopedics & Orthopedic Oncology
The Children’s Hospital of Philadelphia
Associate Professor of Orthopedic Surgery
Perelman School of Medicine at University of Pennsylvania

Tricia Bhatti, MD
Attending Pathologist
Scientific Director, Pathology Core Laboratory
Co-director, Pediatric Pathology Fellowship Training Program
Assistant Professor of Clinical Pathology and Laboratory Medicine
Perelman School of Medicine at the University of Pennsylvania

A 3-year-old boy, otherwise healthy, presents with intermittent limp of a few weeks’ duration, two episodes of low-grade fever and no definitive complaints of pain. On exam, there was an antalgic gait, favoring the left lower extremity. The images and pathology slides are shown below. CD99 and MIC2 were performed and is also shown. What is the diagnosis and next steps in the management of this child?
 
q1a
q1b
q1c
Osteosarcoma. Finalize staging with a CT of the chest, pelvis and abdomen; bone scan and labs. Treatment should include pre-operative chemotherapy and limb salvage surgery with osteoarticular allograft.
Ewing Sarcoma. Finalize staging with a CT of the chest; bone scan or PET; bone marrow aspirate and labs. Treatment should include pre-operative chemotherapy and limb salvage surgery, followed by adjuvant chemotherapy.
Ewing Sarcoma. Finalize staging with a CT of the chest; bone scan or PET; bone marrow aspirate and labs. Treatment should include adjuvant chemotherapy and radiation therapy for local control.
Sub-acute osteomyelitis. Wait for cultures results and start empirical antibiotics. Restrict weight bearing.
Lymphoma of bone. Finalize staging with a PET scan and start chemotherapy.
This is a 14-year-old male who presents with pain in the right leg with activities. He is otherwise healthy, has no recollection of a trauma and states that the pain will sometimes respond to NSAIDS. There is no significant past medical history. Radiographs, advanced imaging and a biopsy is provided. What is the diagnosis and next steps in the management of this child?
 
q2a
q2b
q2c
Low grade osteosarcoma. Finalize staging with a CT chest and start pre-operative chemotherapy followed by limb salvage and adjuvant chemotherapy.
Chronic osteomyelitis. Proceed with saucerization of the tibia, antibiotic bead placement and long-term antibiotic therapy.
Fibrous Dysplasia of bone. Curettage with allograft bone placement.
Primary lymphoma of bone. Finalize the staging with bone marrow aspiration and plan on primary lesion resection/ limb salvage.
Primary lymphoma of bone. Finalize the staging with bone marrow aspiration and start definitive treatment with chemotherapy.
An 8 year old female presented to the ED following a ground level fall and inability to weight bear. She underwent a radiograph, followed by an MRI and further imaging and biopsy. What is the diagnosis and best treatment modality?
 
q3a
q3b
q3c
Osteosarcoma; long leg cast, followed by chemotherapy for local control and limb salvage with Van Ness Rotationplasty.
Osteosarcoma; external fixation, followed by chemotherapy for metastatic disease and delayed local control.
Metastatic neuroblastoma; immediate amputation, followed by adjuvant chemotherapy.
Osteomyelitis; debridement, spanning external fixation, followed by IV antibiotics.
Multifocal Eosinophilic Granuloma; external fixation for femur, followed by intralesional injection of steroid to right humerus.
A 7-year-old boy presented to our institution for a second opinion regarding a fungating mass in the distal aspect of the left femur. According to the family, the mass has been slowly growing for a year at least. After he underwent an open biopsy at an outside institution, the surgical site dehisced and the mass started fungating in that region. Based upon the clinical picture, radiographs, advanced imaging and a biopsy; what is the diagnosis?
q4a
q4aq4b
q4c
q4d
Malignant transformation of an osteochondroma
Osteosarcoma
Rosai Dorfman disease
Chronic Fungal Osteomyelitis
Juvenile Xanthogranuloma (JXT)
This is a 11-year-old girl who has been having pain with activities for a few months. She is a competitive soccer player and the pain seems to respond to NSAIDs and rest. She is otherwise healthy and there are no other symptoms. Based upon the radiographs, advanced imaging and the biopsy, what is the most likely diagnosis?
 
q5a
q5b
q5c
Sub-acute osteomyelitis.
Osteoblastoma/Osteoid Osteoma.
Chondroblastoma.
Langerhans Cell histiocytosis.
Fibrous Dysplasia.
An 8-year-old very active competitive soccer player presented with a few months’ history of left knee pain and mild swelling. The past medical history was significant for Rothmund-Thomson syndrome (RTS), presenting with history of GI problems and short stature. Based upon the radiographs, advanced imaging and biopsy, what is the most likely diagnosis and preferred treatment plan?
 
q6a
q6b
Aneurysmal bone cyst; curettage and bone grafting.
Nonossifying Fibroma; curettage and bone grafting.
High grade osteosarcoma; chemotherapy and local control with wide resection and Van Nes rotationplasty.
Langerhans Cell Histiocytosis; curettage and bone grafting.
Low-grade osteosarcoma; curettage with adjuvant and bone grafting.
16 year-old female complaining of chronic left hip pain with activities. Radiographs, CT and bone scan were obtained and are shown below. What is the most likely diagnosis (path is shown) and the best treatment approach?
 
q7a
q7b
q7c
Unicameral bone cyst; curettage and bone grafting +/- internal fixation.
Metastatic osteosarcoma; palliative chemotherapy and prophylactic intra-medullary fixation.
Melorheostosis; internal fixation.
Metastatic Lymphoma; curettage and bone grafting +/- internal fixation, palliative chemotherapy.
Fibrous dysplasia; internal fixation with intra-medullary device.
This is a 15 year-old female competitive soccer player who presents with a palpable mass and intermittent thigh pain for several months. She remembers being kicked in the thigh during regionals several months ago. Besides general anxiety, there was no other past medical issues. Based upon radiographs, advanced imaging and biopsy, what is the most likely diagnosis and preferred treatment?
 
q8a
q8b
q8c
Low-grade juxtacortical osteosarcoma; local control with wide resection and bone grafting.
Post-traumatic heterotopic ossification; NSAIDS and removal if painful.
Sessile Osteochondroma, reassurance and removal if painful.
Juxtacortical chondroma; observation.
High-grade juxtacortical osteosarcoma; chemotherapy followed Van Ness Rotationplasty.
This is a 10-year-old female who presents with a few weeks’ history of hip pain, worse at night. The past medical history is significant for Langerhans Cell histiocytosis at age 4 that was treated with chemotherapy (Prednisone and Methotrexate). Based upon the radiographs, advanced imaging and the biopsy, what is the most likely diagnosis and the next best diagnostic test?
 
q9a
q9b
q9c
Langerhans Cell Histiocytosis; skeletal survey.
Avascular necrosis from steroid use; GMERIC MRI.
Chronic fungal osteomyelitis; white blood cell scintigraphy.
Chondroblastoma; CT chest.
Leukemia; bone marrow aspirate.
0
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