Clinical History. TumorBoard: Recurrent Chondrosarcoma of the Ethmoid Sinus. chondrosarcoma. Clinical History (cont ) Present Illness

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1 TumorBoard: Recurrent Chondrosarcoma of the Ethmoid Sinus Part 1 of 2 Najat Daw, MD Sandeep Samant, MD Jeffrey Buchsbaum, MD, PhD Christine Fuller, MD Kathleen Helton, MD May 9, 2003 Clinical History 7 y.o. BF with h/o nasal discharge Tearing of the left eye and sleep apnea in Nov Evaluated by ENT: mass in nasal cavity Biopsy showed a benign cartilaginous lesion (condroma or cartilaginous hamartoma) Clinical History (cont ) Present Illness Resection of nasal sinus tumor with pericranial graft reconstruction and microsurgical skull base repair in 12/99 Tumor encapsulated (6x4x3 cm) removed in piecemeal fashion Pathology: low-grade chondrosarcoma No follow-up Headache in 1/03 CT head showed recurrent mass in the ethnoid sinus Referred to St. Jude in 3/03 No nasal symptoms, no weight loss or change in appetite PE: scar of befrontal craniotomy, and bilateral shotty cervical lymph nodes Diagnostic Work-up CBCD, chemistries and coagulation studies WNL Referred CT head: mass centered in ethmoid sinus involving L nasal cavity and medial wall of L orbit MRI face/brain: enhancing lobulated lesion in ethmoid region (2.6x2.4 cm), no invasion of EOM or brain, but possible infiltration of L maxillary sinus CT chest and none scan negative Management Surgical resection of tumor through facial incision and nasal approach on 3/26/03 Pathology: chondrosarcoma grade 1-2 Resection margins presumed positive Endoscopic debridment of nasal cavity on 4/10/03 Started RT on RT-SARC on 4/30/03 1

cranial and facial bones were reviewed Tumors had propensity to occur in the wall of a maxillary sinus, at the junction of sphenoid and ethmoid sinuses and vomer, and at the undersurface of the

2 Imaging of Chondrosarcoma Diagnostic Imaging Kathleen J. Helton, M.D. Division of Diagnostic Imaging Department of Radiological Sciences St. Jude Children s Research Hospital University of Tennessee Memphis, TN Craniofacial chondrosarcomas: imaging findings in 15 untreated cases Radiographic findings of 15 untreated chondrosarcomas of the cranial and facial bones were reviewed Tumors had propensity to occur in the wall of a maxillary sinus, at the junction of sphenoid and ethmoid sinuses and vomer, and at the undersurface of the sphenoid bone Slowly-growing tumors, chondrosarcomas tended to be a large, multilobulated, and sharply demarcated Craniofacial chondrosarcomas: imaging findings in 15 untreated cases Frequent bone changes: a combination of erosion and destruction, with sharp transitional zones and absent periosteal reaction Tumor matrix calcifications, not necessarily chondroid (ares and whorls), are almost always present Both CT and MR may be necessary for thorough evaluation of tumor extent AJNR Am Journal Neuroradiol 1989 Jan-Feb Lee YY, Van Tassel P. MD Anderson Hospital Lateral Nasal Wall Medial Nasal Wall (Septum) 2

3 Initial CT, demonstrated a large, expansile, destructive mass in the sinonasal cavity MRI at recurrence, enhancing left ethmoid, maxillary tumor, no intracranial extension

WHO classification of Bone Tumors Chondrosarcoma Central (intramedullary) Peripheral (cortical) Dedifferentiated Mesenchymal Clear cell Primary and secondary (osteochondroma, enchondroma,

rare mitoses, some necrosis Grade 3: very cellular and pleomorphic; mitoses easy to find, frequently prominent necrosis Mesenchymal and dedifferentiated- always high grade Clear cell- low grade

4 WHO classification of Bone Tumors Chondrosarcoma Central (intramedullary) Peripheral (cortical) Dedifferentiated Mesenchymal Clear cell Primary and secondary (osteochondroma, enchondroma, syndromic) 2003 Chondrosarcoma Grading Grade 1: moderately cellular, hyperchromatic nuclei of fairly uniform size and small Grade 2: greater cellularity, greater nuclear atypia and nuclear size; rare mitoses, some necrosis Grade 3: very cellular and pleomorphic; mitoses easy to find, frequently prominent necrosis Mesenchymal and dedifferentiated- always high grade Clear cell- low grade Stereotactic Image-Guided Navigational Surgery Application in skull-base and paranasal sinus neoplasms Stereotactic Image-Guided Navigational Surgery Application in skull-base and paranasal sinus neoplasms 4

Intra-operative Patient prep - Skin Prep - Dry

fit Intra-operative Set Up Position LandmarX

side of surgeon to ensure good line of site

points (if using FAZER Contour Laser, this step

over 3D volume to better describe patient (for

(nasal angle), anterior/superior (nasion)

Correlation - Touch same points stored on your

options for sinus instrumentation - Passive

5 Intra-operative Patient prep - Skin Prep - Dry Forehead - Apply reference frame - Ensure snug fit Intra-operative Set Up Position LandmarX Evolution ENT Image Guidance System opposite side of surgeon to ensure good line of site Pre-operative Preplan - Select registration points (if using FAZER Contour Laser, this step is not necessary) - Minimum 4 points - Spread over 3D volume to better describe patient (for sinus: Posterior (tragus), Anterior/inferior (nasal angle), anterior/superior (nasion) Intra-operative Registration: - CT/Patient Correlation - Touch same points stored on your system or Accessories Sinus - Multiple tracking options for sinus instrumentation - Passive (wireless, IR reflective) - Fixed array - SureTrak 2 Universal Adapter array Intra-operative Accuracy confirmation Evaluate accuracy / regognize error Use known anatomic structures 5

6 WW Recurent chondrosarcoma ethmoid sinuses 6

7 Thank You End of Part 1 of 2 TumorBoard: Recurrent Chondrosarcoma of the Ethmoid Sinus Part 2 of 2 Najat Daw, MD Sandeep Samant, MD Jeffrey Buchsbaum, MD, PhD Christine Fuller, MD Kathleen Helton, MD May 9,

Chondrosarcoma Second most common primary bone malignancy in adults Malignant tumor of cartilage with entirely chondroid matrix To be distinguished from chondroblastic osteosarcoma, cartilagenous

8 Chondrosarcoma Second most common primary bone malignancy in adults Malignant tumor of cartilage with entirely chondroid matrix To be distinguished from chondroblastic osteosarcoma, cartilagenous tumor with osteoid production Low-grade chondrosarcoma may be difficult to distinguish from benign enchondroma by pathology Chondrosarcoma Types Conventional 90% - Low to intermediate grade - High grade 5-10% Dedifferentiated Mesenchymal Clear cell Conventional Chondrosarcoma Grade 1 Cytologically similar to enchondroma, Slowly growing, locally aggressive with indolent course, does not metastasize Grade 2 Increased cellularity, myxoid change, Locally aggressive with potential for local recurrence, 10-15% 15% metastasize Grade % of chondrosarcomas - rise in cellularity, nuclear atypia, and mitoses, rapidly growing, 50% metastasize Symptoms and Radiographic Findings Pain and local swelling X-Ray Typically radiolucent area with punctate opacities, but can be lytic or heavily calcified Cortical thinning or endosteal scalloping- cortical thickening Cortical disruption and destructive (moth- eaten) pattern in high grade lesions 8

Treatment and Behavior Surgery is the primary treatment modality - Complete wide excision RT and chemo play a minor role Prognosis depends on size, location and grade 5-year

Chondrosarcoma in Children and Adolescents 12 children over 23 yrs, mean age 14 yrs Pelvis most common location (n=6) 10 primary and 2 secondary after RT or in osteochondroma 8

9 Treatment and Behavior Surgery is the primary treatment modality - Complete wide excision RT and chemo play a minor role Prognosis depends on size, location and grade 5-year survival for grade 1-90%, grade 2-81%, grade 3 29% Recurrence occurs 5 to 10 years after surgery, may be associated with higher grade, dedifferentiation or metastasis Chondrosarcoma in Children and Adolescents 12 children over 23 yrs, mean age 14 yrs Pelvis most common location (n=6) 10 primary and 2 secondary after RT or in osteochondroma 8 patients died Treatment is radical excision, RT and chemo reserved for recurrences or distant metastases Aprin et al, Clin Orthop 198 Pediatric Nasal Chondrosarcoma 5 y.o. boy with 2 mo h/o epiphora and nasal obstruction, grade 2, complete resection and chemo, NED 1 yr after diagnosis 20 mo girl with 6 mo h/o nasal obstruction and CN defects, grade 3, subtotal resection, DOD 6 mo after surgery 8 y.o. boy with recurrent tumor 2 years after GTR, grade 3, wide local excision, NED 6.5 yr after surgery Lacovara et al, Am J Ped Hem Oncol

10 Primary Chondrosarcoma of the Head and Neck in Pediatric Patients 14 cases from Tumor Registry of AFIP M/F 8/6; mean age 11.8 yrs (range 3 to 18) Symptoms: nasal stuffiness or discharge, sinusitis, HA, or mass Location: Maxillary sinus 4 Mandible 3 Nasal cavity 2 Neck 2 NP, orbit, skull base 1 each Gadwal et al, Cancer 2000 Primary Chondrosarcoma of the Head and Neck in Pediatric Patients Tumor size 2 to 15 cm (mean 3.1 cm) Grade 1 (n=9); grade 2 (n=1), grade 3 (mesenchymal [n=2], dedifferentiated [n=2]) All patients treated by surgery, followed by RT (n=5) and/or chemo (n=2) All 11 with F/U available are alive a mean of 14.8 years Gadwal et al, Cancer 2000 Condrosarcoma of the Head & Neck The UCLA experience, patients aged 15 to 77 yrs Nasal cavity and paranasal sinuses (8), mandible (5), laryngeal cartilage (2), hyoid bone (2) and orbit (2) 9/10 low grade NED, 1/7 high grade NED 5-yr survival 68% Grade, tumor size and completeness of surgical resection prognostic factors Mark et al, Am J Clin Oncol 1993 Condrosarcoma of the Head & Neck The UCLA experience, Patients with low-grade tumors should undergo adequate surgical resection Further surgery or RT for residual disease High-grade grade tumors should be treated aggressively - RT may be beneficial - Role of chemo not defined Mark et al, Am J Clin Oncol 1993 Recurrent Chondrosarcoma: Post-Op Intensity Modulated Radiation Therapy May 9, 2003 Clinical Data No good prospective pediatric data on recurrent chondrosarcoma therapy, especially in this scenario Head and neck sarcomas are very uncommon, and make up 5-10%. The most comprehensive pediatric data set consists of a retrospective paper on 14 patients. Only 5 were under 10 years old at diagnosis. (Gadwal, et al., Cancer 88(9): ) 2188) The best (base of skull) adult series (MGH) used XRT from 64.2 to 79.6 (72.1) Gy with LC and EFS in the high 90 s with 63 month median f/u (2.1m to 18y). (Rosenberg, et al., Am. J. Surg. Path. 23(11): ) 1378) 10

Some issues in this case Patient age and the use of radiation Her tumor recurred post a G.T.R. Her initial tumor volume (to be shown on CT) Vision status Tumor type - to be discussed by Dr.

11 Some issues in this case Patient age and the use of radiation Her tumor recurred post a G.T.R. Her initial tumor volume (to be shown on CT) Vision status Tumor type - to be discussed by Dr. Fuller Anatomy - to be addressed by Drs. Samant and Helton Organ Lens (cataract) /Lid Retina Optic N./Chi. Brain Bone Growth Dose Tolerances 5% 10 Gy / Gy Gy 50 Gy Gy 50% 18 Gy / Gy 65 Gy 65 Gy Gy Variable, generally less than 20 Gy, but effects can be seen at lower doses. Intensity Modulated Radiation Therapy Overview of IMRT versus Standard Therapy IMRT: 3D data set based MLC used Planned Forward or Inversely More heterogeneity introduced Setup errors can be magnified more quickly Conventional 3D/Other: 0 to 3D data set based Open field to static MLC Forward planned More homogeneous overall Setup errors magnified less quickly Data Sets Multileaf Collimator Clinical Exam Plain Film U/S Tomogram CT MR PET May be moved with each field May be moved dynamically Complex devices Rapid advances are being made in size, speed, and cost 11

Forward I have beams with which to work If I aim them this

and error and clinical experience, blocks, etc. Intuitive I.E.

Planning Reverse/Inverse Where do I want the final dose to be

at what doses So, where should my beams be placed Often

Heterogeneity Q/A Setup More Critical: Tetris MLC Shape MLC

the first of 8 fields used to produce the IMRT distribution on

12 Forward I have beams with which to work If I aim them this way, I get a certain pattern This can be improved via trial and error and clinical experience, blocks, etc. Intuitive I.E.: If I aim the flashlight I get an image. Planning Reverse/Inverse Where do I want the final dose to be (dose cloud, isodose level) What things do I need to avoid and at what doses So, where should my beams be placed Often counter-intuitive Monte-Carlo methods (cost functions) used Heterogeneity Q/A Setup More Critical: Tetris MLC Shape MLC Shape MLC Shape MLC Shape Load Verify Deliver Record Shown is the first of 8 fields used to produce the IMRT distribution on the bottom right corner. All 8 fields are processed this way. (Resume if needed) Complexity Complexity 12

13 13

14 Downsides: Heterogeneity Integral Dose Cost in time both for the physician and the physics team Longer treatment time (GA, comfort) More machine dependent Re-treatment can be far more complex 14

15 Questions Thank You End of Part 2 of 2 15

Imaging: When to get MRI, CT or PET-CT?

Imaging: When to get MRI, CT or PET-CT? Alina Uzelac, D.O. Assistant Clinical Professor Neuroradiology UCSF Department of Radiology and Biomedical Imaging San Francisco General Hospital Overview CT MRI