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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 .
Introduction
Retrorectal
tumours
Heterogeneous
Can arise from any of the germ cell layers
Incidence of less than 1%
Two-thirds are congenital
More common in females than males
Malignancy is more common in men.
The most common malignant tumor is a sacrococcygeal
chordoma.
Anatomy
Anteriorly
• Posterior wall of the rectum and
mesorectum.
Posteriorly
• Anterior aspect of the sacrum.
Lateral borders
• lateral ligaments, the ureters, and iliac
vessels.
ANATOMY
Superior extent
• Pelvic peritoneal
reflection.
Inferior border
• Retrosacral fascia.
ANATOMY OF POSTERIOR SACRUM
ANATOMY
OF
POSTERIOR
SACRUM
Content
• Loose connective tissue
• Middle sacral artery
• Superior hemorrhoidal vessels
• Branches of sympathetic and
parasympathetic nerves
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
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
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
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.
WORKUP
Focused physical
examination,
High-resolution cross-
sectional imaging, and
Percutaneous biopsy of solid
or heterogeneous tumors.
FOCUSSED PHYSICAL
EXAMINATION
• Presence of Postanal Dimple,
• Rectal Mass on DRE,
• Rectal Mucosa Texture,
• Relation to Coccyx,
• Neurological Examination.
HIGH RESOLUTION CROSS SECTIONAL IMAGING
CT
To differentiate
Cystic, solid or
heterogenous
areas.
Cortical
involvement.
MRI
Involvement of
adjacent
structures.
Marrow
involvement
Vascular and
neural
involvement.
Evaluation MRI is the IOC.
Evaluation
Plain radiographs:
• A "scimitar" sign on sacral views or sickle-shaped
sacrum.
Anterior sacral meningocele.
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.
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.
(1) 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.
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.
• Both exhibit keratinizing stratified squamous epithelium
• Epidermoid cysts bear no skin appendages
• Dermoid cysts have sweat glands, hair follicles, or sebaceous cysts.
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.
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.
• Calcification are uncommon.
• CT imaging reveals a well defined, homogeneous mass, with
preservation of adjacent fat planes.
• Risk of malignancy 2 to 13%
TERATOMA AND
TERATOCARCINOMA
• 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.
• 90% of SCT externally visible in
infants.
• Teratomas in adults are most
commonly intrapelvic.
• Type I is predominantly external.
• Type Il is external tumor with
intrapelvic extension.
• Type III is predominantly intrapelvic
with external extension.
• Type IV is a presacral tumor
Features of Retrorectal cysts
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).
• 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
1. >5 cm benign tumor on imaging.
2. <5cm but symptomatic tumor.
3. <5cm, asymptomatic tumor but rapidly growing.
(10% growth in last 1 year on serial MRI).
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.
• 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.
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.
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.
SURGICAL INTERVENTION
Once
identified,
Pre sacral
tumours
should be
treated
surgically.
Risk of malignancy
Risk of Meningitis in Anterior Sacral Meninigocoele.
Risk of infection in Cystic lesions.
Dystocia in young women.
Locoregional symptoms.
MULTIDISCIPLINARY
TEAM
• COLORECTAL SURGEON
• ORTHOPEDIC ONCOLOGIC SURGEON
• SPINE SURGEON
• VASCULAR SURGEON
• PLASTIC SURGEON
• RADIOLOGIST
• MEDICAL ONCOLOGIST
• RADIATION ONCOLOGIST
• ANAESTHESIOLOGIST
• REHABILITATION THERAPIST.
APPROACH TO
THE RESECTION
Anterior
Transabdominal.
Posterior
Perineal or Parasacral
Combined
Anterior-Posterior approach
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).
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.
• 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.
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.
Minimal Invasive
Approaches for
Presacral Tumors
• Highly selected beningn tumours
• Lengyel et al described laparoscopic
approach for advanced malignant lesion.
I. Laparoscopic abdominal phase -
Modified Lloyd davis position.
II. Transsacral phase – prone jackknife
position.
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
Management of
Retrorectal tumors
THANK YOU

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  • 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 Heterogeneous Can arise from any of the germ cell layers Incidence of less than 1% Two-thirds are congenital More common in females than males Malignancy is more common in men. The most common malignant tumor is a sacrococcygeal chordoma.
  • 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.
  • 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 Focused physical examination, High-resolution cross- sectional imaging, and Percutaneous biopsy of solid or heterogeneous tumors.
  • 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 CT To differentiate Cystic, solid or heterogenous areas. Cortical involvement. MRI Involvement of adjacent structures. Marrow involvement Vascular and neural involvement.
  • 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. (1) 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 TERATOCARCINOMA • 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 external. • Type Il is external tumor with intrapelvic extension. • Type III is predominantly intrapelvic with external extension. • Type IV is a presacral tumor
  • 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 1. >5 cm benign tumor on imaging. 2. <5cm but symptomatic tumor. 3. <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 Once identified, Pre sacral tumours should be treated surgically. Risk of malignancy Risk of Meningitis in Anterior Sacral Meninigocoele. Risk of infection in Cystic lesions. Dystocia in young women. Locoregional symptoms.
  • 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. I. Laparoscopic abdominal phase - Modified Lloyd davis position. II. 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

Editor's Notes

  1. Focused physical examination- POSTANAL DIMPLE, RECTAL MASS, MUCOSA TEXTURE, RELATION TO COCCYX, NEUROLOGICAL EXAMINATION.