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Presacral tumor

1. Presacral Tumours Presenter Dr.Harshavardhana H P SGE DNB Resident GEM Hospital. Centre for Colorectal Surgery, GEM Hospital & Research centre, presents PG Academic series Moderator Dr Rajapandiyan.S Head of Colorectal unit GEM hospital .

2. Introduction Retrorectal tumours

3. Anatomy Anteriorly Posterior wall of the rectum and mesorectum. Posteriorly Anterior aspect of the sacrum. Lateral borders lateral ligaments, the ureters, and iliac vessels.

4. ANATOMY Superior extent Pelvic peritoneal reflection. Inferior border Retrosacral fascia.

5. ANATOMY OF POSTERIOR SACRUM

6. ANATOMY OF POSTERIOR SACRUM

7. Content • Loose connective tissue • Middle sacral artery • Superior hemorrhoidal vessels • Branches of sympathetic and parasympathetic nerves

8. Presacral tumors and its challenges The sacral nerve rootlets injury to leads to • Defecatory • Urological • Sexual dysfunction. unilateral sacrifice of all of the sacral nerve, Atleast S1-3 roots present on either side: no issue. If bilateral S3 roots are damaged: fecal incontinence. may need a permanent colostomy

9. CLASSIFICATION OF PRE SACRAL TUMOURS CONGENITAL Benign Developmental cysts (teratoma, epidermoid, dermoid, mucus secreting) Duplication of rectum Anterior sacral meningocele Adrenal rest tumor Malignant Chordoma Teratocarcinoma NEUROGENIC Benign Neurofibroma Neurilemoma (Schwannoma) Ganglioneuroma Malignant Neuroblastoma Ganglioneuroblastoma Ependymoma Malignant Peripheral nerve sheath tumors

10. CLASSIFICATION OF PRE SACRAL TUMOURS OSSEOUS Benign Giant cell tumor Osteoblastoma Aneurysmal bone cyst Malignant Osteogenic sarcoma Ewing sarcoma Myeloma Chondrosarcoma MISCELLANEOUS Benign Lipoma Fibroma Leiomyoma Hemangioma Endothelioma Desmoid Hemangiopericytoma Malignant Liposarcoma Fibrosarcoma/malignant fibrous histiocytoma Leiomyosarcoma Metastatic carcinoma

11. CLINICAL PRESENTATION Vague and ill defined Discovered incidentally on routine pelvic or rectal examination Pelvic or low back pain, constipation, a palpable mass or obstructive type symptoms. Classically, pain is aggravated by sitting and ameliorated by standing or walking Urinary or fecal incontinence and sexual dysfunction. Persistent perianal discharge.

12. WORKUP

13. FOCUSSED PHYSICAL EXAMINATION Presence of Postanal Dimple, Rectal Mass on DRE, Rectal Mucosa Texture, Relation to Coccyx, Neurological Examination.

14. HIGH RESOLUTION CROSS SECTIONAL IMAGING

15. Evaluation MRI is the IOC.

16. Evaluation Plain radiographs: A "scimitar" sign on sacral views or sickle-shaped sacrum. Anterior sacral meningocele.

17. Approch for biopsy Transrectal or transvaginal biopsy is contraindicated A transperineal or parasacral approach within the field of the resection is recommended. Excision of the biopsy tract and site at the time of surgery advised.

18. ROLE OF PREOPERATIVE BIOPSY • Simple cystic lesions, which are uniformly benign, biopsy C/I • All solid or heterogeneous tumours , Consider biopsy prior to surgical intervention. potential impact upon the operative approach , (2) application of direct neoadjuvant therapy {Ewing sarcoma, osteogenic sarcoma, and neurofibrosarcoma} (3) better counsel the patient prior to Sx .

19. Dermoid and Epidermoid Cysts Failure of separation of cutaneous ectoderm from neural ectoderm. more common in females a postanal dimple or sinus up to 30% of present as a pelvic or perirectal abscess. may be misdiagnosed with fistula in ano if a communication between an abscess and a postanal dimple exists.

20. Both exhibit keratinizing stratified squamous epithelium Epidermoid cysts bear no skin appendages Dermoid cysts have sweat glands, hair follicles, or sebaceous cysts .

21. Enterogenous Cysts Duplication cysts of the rectum. Sequestration of the developing hindgut lined by squamous epithelium or columnar epithelium. Multilocular cyst. well-defined muscular wall with a myenteric plexus. Villi or crypts.

22. Tailgut Cysts Cystic hamartomas. Postanal gut cysts arise from remnants of the embryonic primitive gut . lesions are often multicystic and multilocular . Lining is a combination of squamous, glandular columnar, or transitional. No evidence of a well-defined muscular wall with myenteric plexus .

23. Calcification are uncommon. CT imaging reveals a well defined, homogeneous mass, with preservation of adjacent fat planes. Risk of malignancy 2 to 13%

24. TERATOMA AND TERATOCARCINO MA Presacral teratoma is the most common teratoma in infancy. A female predominance. Contain cells from all three germ layers. Tumor with recognizable hair, bone or teeth, are more likely to be benign. Benign lesions are usually cystic . Malignant lesions are having solid or heterogenous component.

25. 90% of SCT externally visible in infants. Teratomas in adults are most commonly intrapelvic. Type I is predominantly exter nal . Type Il is external tumor with intrapelvic extension. Type III is predominantly intrapelvic with external extension. Type IV is a presacral tumor

26. Features of Retrorectal cysts

27. Neurogenic tumours Represent about 10% of all retrorectal tumors . Schwannomas were the most common benign tumor. Malignant peripheral nerve sheath tumors, the most common malignant lesions. The most common presenting symptom is pain (most commonly sciatalgia ).

28. Preoperative biopsy in neurogenic tumour . 1.Pathology is unclear on imaging 2. >5 cm benign tumor on imaging 3. Neurogenic tumour with clinical features moderate or severe neurologic deficit. Indication of sx in benign neurogenic tumours >5 cm benign tumor on imaging. <5cm but symptomatic tumor. <5cm, asymptomatic tumor but rapidly growing. (10% growth in last 1 year on serial MRI).

29. SACROCOCCYGEAL CHORDOMAS Most frequently encountered malignant tumor of the retrorectal space Originate from the primitive notochord tissue either from the nucleus pulposi or from abnormal rests. anywhere along the spinal column M/C at the sphenoocipital and the retrorectal regions.

30. male predominance. Usually after 30 year age. long-standing history of vague pain. aggravated by sitting and ameliorated by standing or walking. Most important predictor of survival was a wide negative margin.

31. Osseous lesions 10% of all retrorectal tumors Most osseous lesions of the presacral space are metastatic. Pain remains the most common presenting feature. most aggressive of all the retrorectal tumors. locally destructive Pronounced metastatic potential.

32. Currarino syndrome Triad of • Presacral mass • Anorectal malformations • Sacral anomalies Most frequent component of the presacral mass is Meningocele Teratomas (20% to 40%). HLXB9 gene mutation located on chromosome 7q36.

33. SURGICAL INTERVENTION

34. MULTIDISCIPLINARY TEAM COLORECTAL SURGEON ORTHOPEDIC ONCOLOGIC SURGEON SPINE SURGEON VASCULAR SURGEON PLASTIC SURGEON RADIOLOGIST MEDICAL ONCOLOGIST RADIATION ONCOLOGIST ANAESTHESIOLOGIST REHABILITATION THERAPIST.

35. APPROACH TO THE RESECTION Anterior Transabdominal. Posterior Perineal or Parasacral Combined Anterior-Posterior approach

36. Tumors located below S3- The Posterior Approach Prone jackknife position. Incision over the lower sacrum and coccyx. Coccygectomy or Distal Sacrectomy only if malignancy invading it. Plane of dissection- between the retro rectal fat and the tumor (Pseudocapsule).

37. Combined Anterior- Posterior Approach. Indication- tumor above the level of S3. Anterior Phase Supine position, transabdominally. Modified Dorsal Lithotomy position- if rectal resection is planned. “Sloppy lateral position- two team approach. Plane of dissection- Anteriorly between its capsule and mesorectum. Posteriorly between the lesion and rectum. Small, Beningn tumours – Circumferential dissection and remove. Bulky tumors /Invasion – enblock resection of rectum with anastomosis and diverting ileostomy.

38. If bilateral S3 roots or S2 involved- End Sigmoid colostomy. If Major Sacrectomy is planned – Ligation of middle sacral artery and inter iliac artery and its branches. Preserve anterior division of internal iliac artery – as it gives of inferior gluteal artery and prevents potential perennial and gluteal necrosis. If extended sacral resection- Rectal abdominis flap.

39. Posterior phase Abdominal incision is closed and ostomy created before prone position. Midline incision over sacrum and coccyx. Anococcygeal ligament transected and levators retracted bilaterally. Dissection of gluteus maximus muscle on both sides. Transection of sacrospinous and sacrotuberous ligaments. Division of Piriformis to expose the sciatic nerves. Osteotomy – at S3 level or higher. Preseve atleast one S 3 nerve root. The neural sac may need to be ligated.

40. Minimal Invasive Approaches for Presacral Tumors Highly selected beningn tumours Lengyel et al described laparoscopic approach for advanced malignant lesion. Laparoscopic abdominal phase - Modified Lloyd davis position. Transsacral phase – prone jackknife position.

41. Follow up Beningn tumors Annual visit – DRE, Baseline CT (repeated at 5years interval if normal) Malignant tumours Annual Abdominopelvic MRI. Annual Chest CT scan Annual DRE Endoscopic examination. First 5 years

42. Management of Retrorectal tumors

43. THANK YOU