Prof Dr Harzem Ozger Istanbul University Istanbul Faculty of Medicine Dept. Of Orthopaedics and Traumatology Orthopedic Oncologic Approach in Sacrum Tumors

Epidemiology %1,4 – 4 of all musculoskeletal tumors Benign aggressive > Malignant >>> Metastasis Low grade >> High grade Benign ABC GCT Malignant CS Chordoma EWS

Anatomical Considerations Delayed diagnosis Complicated radiotherapy Demanding surgical technique Increased perioperative morbidity / mortality Poor prognosis

Anatomical Considerations Bad prognostic anatomic site Delayed diagnosis Common pathologies are resistant to adjuvant treatments Hard to achieve WIDE MARGINS Perioperative morbidity / mortality Patient Large intrapelvic volume retards symptoms Large intrapelvic volume retards symptoms Delayed diagnosis Complicated radiotherapy Demanding surgical technique Increased perioperative morbidity / mortality Poor prognosis

Anatomical Considerations Bad prognostic anatomic site Delayed diagnosis Common pathologies are resistant to adjuvant treatments Hard to achieve WIDE MARGINS Perioperative morbidity / mortality Delayed diagnosis Complicated adjuvant treatment Demanding surgical technique Increased perioperative morbidity / mortality Poor prognosis Conventional RT Rectum, bladder, small bowel, Rectum, bladder, small bowel, dural sac and sacral roots at risk

Anatomical Considerations Bad prognostic anatomic site Delayed diagnosis Common pathologies are resistant to adjuvant treatments Hard to achieve WIDE MARGINS Perioperative morbidity / mortality Delayed diagnosis Complicated radiotherapy Demanding surgical technique Increased perioperative morbidity / mortality Poor prognosis Difficult exposure Difficult exposure Abundant hemorrhage Abundant hemorrhage Difficult 3D orientation Difficult 3D orientation Difficult reconstruction Difficult reconstruction

Anatomical Considerations Bad prognostic anatomic site Delayed diagnosis Common pathologies are resistant to adjuvant treatments Hard to achieve WIDE MARGINS Perioperative morbidity / mortality Delayed diagnosis Complicated radiotherapy Demanding surgical technique Increased perioperative morbidity / mortality Poor prognosisAnterior Posterior Posterior

Anatomical Considerations Bad prognostic anatomic site Delayed diagnosis Common pathologies are resistant to adjuvant treatments Hard to achieve WIDE MARGINS Perioperative morbidity / mortality Delayed diagnosis Complicated radiotherapy Demanding surgical technique Increased perioperative morbidity / mortality Poor prognosis Neighboring major neurovascular structures Rectum, bladder, ureters rectum sacrum

Anatomical Considerations Bad prognostic anatomic site Delayed diagnosis Common pathologies are resistant to adjuvant treatments Hard to achieve WIDE MARGINS Perioperative morbidity / mortality Delayed diagnosis Complicated radiotherapy Demanding surgical technique Increased perioperative morbidity / mortality Poor prognosis Loss of spinopelvic continuity

Biological ConsiderationsSurgeon Unfamiliar with the biology of sacral tumors Unfamiliar with the biology of sacral tumors Malignant behavior with benign histology in some cases! Malignant behavior with benign histology in some cases! Late MET and AWD for years with low-grade malignant! (chordoma) Late MET and AWD for years with low-grade malignant! (chordoma)

Psychological Considerations Is the surgeon ready to sacrifice? - Wide resection is the ONLY option for malignant tumors. - Insufficient resection to avoid complication: * Local recurrence which requires more morbid resection * Local recurrence which is inoperable * Metastasis

Psychological Considerations Is the patient ready to sacrifice? - Hard to convince a patient that he/she is going to / might have Sexual dysfunction Urinary incontinence Anal incontinence - colostomy Walking difficulties Wound problems and prolonged hospitalization after surgery and local recurrence is still possible. - Palliative treatment is always an option. BUT - The patient MUST BE WELL INFORMED that these complications are inevitable even if no surgery is performed and the tumor will be unresectable by then.

Preop assessment – Detailed MR imaging of sacral roots & margins and CT for osseous destruction RTx (especially IMRT – higher dose, less morbidity w/ 3D beam) CTx ???!!! (tumor-targeted CTx promising...) Preoperative embolisation (inform the interventional radiologist about the type of resection: intralesional / wide) Complex reconstructions (eg. lumbopelvic fixation – tumor surgeon cooperates w/ spine surgeon) Principals of Management

Team work beginning at biopsy Education of medical professionals: Prevention of wrong surgery !!! Extremely specialized management – experienced team:  Oncologic orthopedic surgeon  Radiation oncologist  Medical oncologist  Radiologist  Spine surgeon  General surgeon  Plastic surgeon  Vascular surgeon  Urologist  Physiotherapist  Medical psychologist Principals of Management

Hemorrhage – A/V iliaca, corona mortis – tumor itself Neurologic – Sacral roots Mechanic – Sacroiliac joint Neighbourhood – Anorectal complex – Bladder, ureters – Internal genitals Dead space ONCOLOGIC Surgical Considerations

Minimising intraoperative bleeding 13 y, F ABC of sacrum Intralesional resection following embolisation

Unilateral sacrif. causes mostly temporary palsies Bil. S3: Sexual dysfunction, urinary dysfunction Bil. S2: Anorectal dysfunction Bil. S1: Below knee extensor palsy For locomotion, quadriceps function is vital (try to protect L5) Preservation of nerve roots

16 y, F GCT of sacrum Intralesional resection (curettage and phenolisation) only NED at postop 4 yrs.

25 y, F ABC of sacrum Intralesional resection + phenolisation + PMMA NED at postop 5 yrs.

 %50 loss of SI joint causes vertical + rotational instability :  Lumbopelvic fixation !! Mechanical support

21 y, F LN of ABC of sacrum (curettage + PMMA in elsewhere hospital) Preop embolisation + removal of PMMA + curettage + high- speed burr + phenolisation Surgery had to be abandoned despite total spinopelvic discontinuity due to hemorrhage 1. stage postop

2. stage Posterior instrumentation performed after 2 wks for lumbopelvic fixation

42 y, M Chondrosarcoma of R iliac wing Loss of SI joint due to wide resection Spinal instrumentation from posterolateral and augmentation with a second rod for lumbopelvic fixation + prolen mesh to avoid abdominal hernia

Early postop xrays

Postop 3 months

Management of Urogenital and Colorectal Complications -20 y/o F -GCT of sacrum -Neurogenic bladder at postop 4 wks. -Life-long intermittent urinary catheterization unavoidable in some patients

- 17 y, M with OS of right hemipelvis - double J-catheterization preop to avoid intraoperative ureter injury

65 y, M Underwent surgery for sacrum chordoma Permanent colostomy due to rectum resection (tumor invasion) Temporary colostomy to avoid fecal contamination of the wound

- Dead space - Avascular flaps Management of Soft Tissue Complications

– Silicone prosthesis + Prolene Mesh – Live Tissue Gluteus maximus flap – If the gluteal arteries are not injured! VRAM (Vertical Rectus Abdominis Muscle Flap) VRAM supine prone

Adequate tumor resection – Benign (Intralesional) Curettage High-speed burr Phenolisation (chemical tumor ablation) PMMA ( thermal tumor ablation) – Malignant (Wide) No compromise on margins Adjuvant treatment can NEVER compensate for inadequate margins

65 y, M Sacrum chordoma arising from S2-3-4 and extending proximally along the tract of previous intervention Wide resection including the rectum + colostomy

Local recurrence at postop 1 yr - resected

Local recurrence at postop 3 yrs. - inoperable

All lessons learned!

Preoperative embolisation - 30 y / F - Sacrum chordoma

12 3 Before the resection of sacrum chordoma - Colostomy is prepared, - Vertical rectus abdominus myocutaneous flap is prepared, - VRAM flap is buried deep into the pelvis and the patient is turned to prone position.

After wide resection of sacrum chordoma and the rectum,VRAM flap is pulled out from posterior to fill the dead space.

Clinical photos at 8 months postop Permanent colostomy (planned preoperatively) NO complication NO local recurrence at postop 50 months

Extreme reconstructions - 16 y, F - osteosarcoma of right hemipelvis

- Internal hemipelvectomy (including partial sacrectomy) + hip transposition - Sciatic nerve was sacrified due to tumor invasion - Acetabular cup of uncemented total hip prosthesis was placed in L5& S1

Postop 5 months Postop 15 months

Ambulatory with a single crutch at 13 months postop

Life? Function? Psychic health? Wrong OP Morbidity   Pain   Quality of life  Death comes late

Surgery with WIDE MARGINS ? Urogenital & anorectal function If the surgeon does not sacrifice these functions, the tumor will do it in time (with high mortality!)

Conclusion For malignant sacral tumors, Marginal resection + Adjuvants do not provide safe margins. Intrapelvic recurrence is diffuse and mostly inoperable. Metastases appear late and the patient is usually Alive With Disease for a long time and also full of morbidities !!! If the surgeon does not sacrifice the function (nerve roots), the tumor does !!! The initial operation with WIDE MARGINS is the only chance for cure !!!