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Management of Presarcral
( Retro-rectal ) Tumors
Dr. Jawad Ahmed
First Year Resident
Surgical D unit LRH
Outlines
• Introductions
• Anatomy
• Classification
• Diagnosis
• Management
• Outcomes
Introduction
• Presacral masses are a group of lesions
that encompasses a wide spectrum of
diseases ranging from congenital lesions
to inflammatory diseases processes and
overt malignancy.
• In general retrorectal tumor are extremely
rare, with the incidence of tumors varying
in the reported literature.
• The Mayo Clinic has reported that
presacral tumors represent 1 in 40,000
hospital admissions.
• Diagnosis of these lesions is usually
incidental on physical examination or on
imaging studies, as symptomatology is
usually vague.
Anatomy
• The presacral or retrorectal space is not a
true space but rather a potential space.
• It is a unique area in that it represents a
developmentally critical location where
several types of embryological distinct cell
lines converge for the final steps prior to
the completion of ontogeny.
• The retrorectal space is the area posterior
to the rectum but, more specifically, its
superior extent is the pelvic peritoneal
refelections, its lateral limits are the
ureters and iliac vessels, posteriorly it is
defined by the sacrum, and anteriorly it is
defined as the posterior wall of the rectum.
• The inferior border is the levator complex
and the coccygeal muscles.
Classification
• As a result of the diverse nature of presacral
tumors, a variety of classification systems have
been proposed to categorize these lesions.
• The classification system first decribed by Uhling
and Johnson, has been most frequently used
and separate these lesions into the following
broad categories: congenital, neurogenic,
osseous, inflammatory and miscellaneous.
• Dozois have modified and updated this
classification systems to subcategorize tumors
in malignant and benign entities within these
broad categories.
Congenital Lesions
• These represent two-third of all retrorectal
lesions, which are thought to arise from
various combinations of the three
embryonic cell layers.
• These lesions can be cystic or solid.
• In general, these lesions are more
common in females than in males.
Dermoid and Epidermoid Cysts
• These cysts are lined with squamous epithelium
and may contain various skin appendeges such
as hairs or nails.
• These lesions are thought to arise from
ectodermal layer.
• Pateint can have post-anal dimple or sinus that
can be mistaken for an abscess and errantly
drained.
• This account for the high rate of infection of
these cysts.
Enterogenous Cysts
• Unlike dermoid or epidermoid cysts,
enterogenous cysts are multilocular.
• Enterogenous cysts arise from endoderm
of the primitive hindgut.
• These lesions can undergo malignant
degeneration.
Tailgut Cysts
• Tailgut cysts are also referred to as
retrorectal cystic hamartomas which arise
form the persistance of the hindgut.
• Rectal duplication cysts contain all of the
layers of the intestinal tract.
• Rectal duplication cysts can also undergo
malignant change.
Teratomas
• Teratoms also contain cells from all three germ
layers, but more importantly, these lesions are
true neoplasms.
• They can contain both solid and cystic
components.
• Upto 10% of these lesions contain cancer, and
thus aggressive extirpation should be pursued.
• Because of the diverse germ cell layers, these
lesions can become SSCs, rhabdomyosarcoma
or anaplastic tumors.
• These tumors can contain tissues from almost
any organ system including digestive and
respiratory or bony tissue.
• These are more common in females.
• They are also more common in children than
adults.
• Factors that are associated with malignat
degeneration and/or recurrence are incomplete
resection and resection where the coccyx is not
removed.
Chondromas
• The most common malignant tumors of the presacral space is the
sacrococcygeal chondroma.
• These tumors arise from what is believed to be vestigial notochord
tissue.
• These lesions are more common in male patients under 40 with an
incidence of about 0.08 per 100,000.
• These lesions can occur almost anywhere on the spinal cord but
most commonly found in the presacral area.
• These patients present with vague symptoms including low back
pain.
• The 5 and 10 year survival rates are 67 and 40% respectively.
• Surgery remain the mainstay of treatment, and it is associated with
a high recurrence rate.
Anterior Sacral Meningocele
• These lesions arise from protrusions of the dural
sac through a defect in the sacrum.
• The classic radiologic findings of these tumors
on plain Xray is “Scimitar Sign”.
• Patient often have vague symptomatology
including headache related to postural changes
and Valsala.
• MRI usually easily charaterizes these lesions,
and percutaneous biopsy should be avoided for
fear of bacterial contamination of the CSF and
itrogenic meningitis.
Scimitar Sign
Neurogenic Tumors
• Neurogenic tumors represent about 10% of all
the retrorectal tumors.
• They arise from peripheral nerves and include
neurofibromas, schwannoma, ganglionuroma,
neuroblastoma and ependymoma.
• Ependymomas are the most common of these
tumors.
• Differentiation between benign and malignant
variants can be difficult, and these tumors can
produce significant neuropathy as a presenting
symptoms.
Osseous Lesions
• Osseous lesions include giant cells tumors,
osteoblastoma, aneurysmal bone cysts,
osteogenic sarcoma, Ewings sarcoma, myeloma
and chondrosarcoma.
• These lesions represent 10% of all the
retrorectal tumors.
• These may be the most aggressive of all the
retrorectal tumors and can be very locally
destructive and have pronounced metastatic
potential.
Diagnosis
• HISTORY:
• Because of the location of these tumors in
presacral area, the symptomatology tends
to be vague and non-specific.
• Many patients will have lower back pain or
pelvic pain; however, in general, there is
no plethora of common findings.
• Patient with advanced tumors can have
constipation, sexual dysfunction, urinary
incontinence and other leg and gluetal
symptoms.
Physical Exam
• There is no specific exam finding in these
tumors.
• Many of these tumors are diagnosed incidentally
on rectal examination and in fact 97% of
presacral lesions are palpable in digital rectal
exam.
• Patient with congenital cyst or tumor may have a
post-anal sinus, however , the most likely
etiology of post-anal sinus is perianal fistulous
disease.
Imaging Studies
• Plain Xrays
• CT scan
• MRI
• Sigmoidoscopy
• Endo-rectal U/S
Plain Xrays
• Plain films have limited utility but can
sometimes demonstrate osseous
destruction of the sacrum or calcifications
within the tumor itself.
• In patient with anterior sacral meningicele,
the classic “scimitar sign” can often be
seen on plain films.
CT scan
A CT scan can be used to determine
whether a lesion is solid or cystic, evaluate
cortical bone destruction, and assess
involvement of adjacent viscera.
MRI
• MRI with gadolinium is the imaging
modality of choice for retrosacral tumors.
• MRI is critical in the management of these
tumors by facilitating accurate diagnosis,
determining the anatomic extent of the
lesion and selecting optimal surgical
approach.
Cystic teratoma
Sigmoidscopy
• The preoperative assessment of the recto-rectal
tumors should include intralumonal evaluation of
the rectum via flexible sigmoidscopy.
• Understanding the extent of the mass of the
tumor on the rectum and the ability to assess the
mucosal integrity of the rectum are both
important elements of the pre-op prepration.
• Flexible sigmoidscopy allows for a better
assessment of the upper and lower extents of
the sphincter complex.
Endo-rectal Ultrasound
• ERUS can be utilized to assess the relationship
of tumors to the muscular layers of the rectum
and anal sphincters; despite the fact that
majority of the lesions are well circumscribed,
the subset of tumors that are not circumscribed
can be quite locally advanced and destrcutive.
• ERUS can also allow a very preliminary
assessment of the sacral bony destruction by
tumors.
Preoperative Biopsy
• Biopsy of the presacral tumors presents a
twofold question.
• First, is the biopsy associated with a
higher rate of local recurrence?
• Second, does biopsy have proven utility in
the management of presacral tumors, i.e.,
does bit change the management?
• It is universally acknowledged that biopsy
of presacral tumors via transrectal or
transvaginal is strongly discouraged as it
is possible to infect a sterile cystic lesion.
• In additon, biopsy via these routes
necessitates either partial or complete
proctectomy or vaginectomy to remove the
biopsy tract in continuity with the sacral
tumors in order to prevent recurrence.
• More recent data suggest that percutaneous
biopsy of retrorectal tumors can be performed
without an increased risk of recurrence.
• There is a role for biopsy in unresectable,
sizeable or aggressive tumors such as Ewing’s
sarcoma or osteosarcoma where preoperative
radiation or chemotherapy could be of valve for
systemic or local control or to improve the
likelihood of resectability.
• It is our current practice to excise the biopsy
tract and site at the time of definitive surgery.
Treatment
Role of Preoperative Neoadjuvant Therapy:
• Retrorectal tumors can exhibit a diverse set of
behaviours and can be quite large and locally
advanced by the time they are diagnosed.
• In addition, a subset of pelvic sarcomas has
fairly significant systemic metastatic potential.
With this in mind, there is a definite role of
neoadjuvant chemotherapy for some of these
tumors.
• In locally advance tumors, where resectability is
an issue, neoadjuvant radiotherapy may render
some benefit in decreasing tumor size and
increasing resectability.
Surgical Treatment
• Unless the lesion is unresectable or there
is evidence of systemic metastasis,
presacral tumors should be resected, as
30-40% of the lesions will be malignant
and benign lesions can undergo malignant
transformation.
Preoperative planning
• The key to preoperative planning is
understanding the extent of the resection
field.
• In patients that have direct invasion of the
muscular wall of the rectum, proctectomy
must be anticipated.
• In cases of bony invasion, partial
sacrectomy is planned.
• Pelvic side wall involvement may
necessitate intraoperative radiotherapy
and vascular and ureteric reconstruction.
• The assembly of a multispecialty team of
colorectal, urologic, neurosurgical,
orthopedic, vascular and plastic surgeon is
a prerequisite for many of these
undertakings.
Surgical Apporach
• The location, the morphology and the
involvement of other pelvic structures dictate the
operative approach.
• In general, a well-circumscribed presacral
lesions whose uppermost extent can be
palpated on digital rectal examination can
usually be approached via a posterior approach.
• In lesions above S4 level, a purely abdomonal
approach can be considered.
• Lesions spanning both above and below are
best approached via a combined abdominal and
posterior approach.
Anterior Approach
• A midline incision is made,and a thorough exam of all
quadrants of the abdomen should be performed to
assure that there are no metastasis. The sigmoid colon
is mobilized along the white line of Toldtm and the
presacral space is entered at the level of sacral
promontory.
• The left & right hypogastric nerves are identified and
preserved.
• The rectum is pulled forward and dissected off the
anterior aspect of the lesion and then the posterior
aspect of the lesion is dissected off the presacral fasica.
• If the middle sacral artery is the main
arterial supply of the lesion, then it has to
be ligated prior to removing the lesion.
• With advances in minimally invasive
techniques, a laproscopic approach to
removing these tumors has been shown to
be a safe and feasible option.
Posterior Approach
• For low-lying tumors, the patient is placed in prone jack-
knife position with the buttocks spread with tape.
• An incision is made over the lower portion of the sacrum
and coccyx down to the anus taking care to avoid
demage to the external sphincter.
• Resection of the tumor may be facilitated by transection
of the anococcygeal ligament and coccyx.
• The lesion can then be dissected from the surrounding
tissues including the rectal wall, in a plane between
rectorectal fat and the tumor mass itself
• In case of very small lesions, the surgeon may
double-glove the left hand and, with index finger
in the anal canal and lower rectum, push the
lesion outward away from the depths of the
wound facilitating dissection of the lesion off the
wall of the rectum without injury.
• If necessary, the lower sacrum or coccyx or both
can be excised en bloc with the lesions to
facilitate excision.
Combined Abdominal and Perineal
Appraoch
• If the upper pole of the tumor extends clearly above S3
level, an anterior and posterior approach is usually
indicated.
• Patient may be placed in the supine or lateral position
depending on the surgeon preference and previous
experience.
• If the anterior-posterior approach is necessary, the
patient can be placed in a “sloppy-lateral position to
facilitate a simultaneous two-team approach.
• It is always recommended to do cystoscopy and bilateral
ureteral stent placement before laprotomy.
Conclusion
• Because of their rarity, the diagnosis of
presacral tumors can be challenging. Accurate
and reliable diagnostic imaging is essential to
identify the optimal surgical approach. Surgical
management should be determined by the
nature and location of the lesion and the extent
of involvement of surrounding structures while
minimizing the morbidity to the patient.
•

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Management of presarcral tumors

  • 1. Management of Presarcral ( Retro-rectal ) Tumors Dr. Jawad Ahmed First Year Resident Surgical D unit LRH
  • 2. Outlines • Introductions • Anatomy • Classification • Diagnosis • Management • Outcomes
  • 3. Introduction • Presacral masses are a group of lesions that encompasses a wide spectrum of diseases ranging from congenital lesions to inflammatory diseases processes and overt malignancy. • In general retrorectal tumor are extremely rare, with the incidence of tumors varying in the reported literature.
  • 4. • The Mayo Clinic has reported that presacral tumors represent 1 in 40,000 hospital admissions. • Diagnosis of these lesions is usually incidental on physical examination or on imaging studies, as symptomatology is usually vague.
  • 5. Anatomy • The presacral or retrorectal space is not a true space but rather a potential space. • It is a unique area in that it represents a developmentally critical location where several types of embryological distinct cell lines converge for the final steps prior to the completion of ontogeny.
  • 6. • The retrorectal space is the area posterior to the rectum but, more specifically, its superior extent is the pelvic peritoneal refelections, its lateral limits are the ureters and iliac vessels, posteriorly it is defined by the sacrum, and anteriorly it is defined as the posterior wall of the rectum. • The inferior border is the levator complex and the coccygeal muscles.
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  • 9. Classification • As a result of the diverse nature of presacral tumors, a variety of classification systems have been proposed to categorize these lesions. • The classification system first decribed by Uhling and Johnson, has been most frequently used and separate these lesions into the following broad categories: congenital, neurogenic, osseous, inflammatory and miscellaneous. • Dozois have modified and updated this classification systems to subcategorize tumors in malignant and benign entities within these broad categories.
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  • 11. Congenital Lesions • These represent two-third of all retrorectal lesions, which are thought to arise from various combinations of the three embryonic cell layers. • These lesions can be cystic or solid. • In general, these lesions are more common in females than in males.
  • 12. Dermoid and Epidermoid Cysts • These cysts are lined with squamous epithelium and may contain various skin appendeges such as hairs or nails. • These lesions are thought to arise from ectodermal layer. • Pateint can have post-anal dimple or sinus that can be mistaken for an abscess and errantly drained. • This account for the high rate of infection of these cysts.
  • 13. Enterogenous Cysts • Unlike dermoid or epidermoid cysts, enterogenous cysts are multilocular. • Enterogenous cysts arise from endoderm of the primitive hindgut. • These lesions can undergo malignant degeneration.
  • 14. Tailgut Cysts • Tailgut cysts are also referred to as retrorectal cystic hamartomas which arise form the persistance of the hindgut. • Rectal duplication cysts contain all of the layers of the intestinal tract. • Rectal duplication cysts can also undergo malignant change.
  • 15. Teratomas • Teratoms also contain cells from all three germ layers, but more importantly, these lesions are true neoplasms. • They can contain both solid and cystic components. • Upto 10% of these lesions contain cancer, and thus aggressive extirpation should be pursued. • Because of the diverse germ cell layers, these lesions can become SSCs, rhabdomyosarcoma or anaplastic tumors.
  • 16. • These tumors can contain tissues from almost any organ system including digestive and respiratory or bony tissue. • These are more common in females. • They are also more common in children than adults. • Factors that are associated with malignat degeneration and/or recurrence are incomplete resection and resection where the coccyx is not removed.
  • 17. Chondromas • The most common malignant tumors of the presacral space is the sacrococcygeal chondroma. • These tumors arise from what is believed to be vestigial notochord tissue. • These lesions are more common in male patients under 40 with an incidence of about 0.08 per 100,000. • These lesions can occur almost anywhere on the spinal cord but most commonly found in the presacral area. • These patients present with vague symptoms including low back pain. • The 5 and 10 year survival rates are 67 and 40% respectively. • Surgery remain the mainstay of treatment, and it is associated with a high recurrence rate.
  • 18. Anterior Sacral Meningocele • These lesions arise from protrusions of the dural sac through a defect in the sacrum. • The classic radiologic findings of these tumors on plain Xray is “Scimitar Sign”. • Patient often have vague symptomatology including headache related to postural changes and Valsala. • MRI usually easily charaterizes these lesions, and percutaneous biopsy should be avoided for fear of bacterial contamination of the CSF and itrogenic meningitis.
  • 20. Neurogenic Tumors • Neurogenic tumors represent about 10% of all the retrorectal tumors. • They arise from peripheral nerves and include neurofibromas, schwannoma, ganglionuroma, neuroblastoma and ependymoma. • Ependymomas are the most common of these tumors. • Differentiation between benign and malignant variants can be difficult, and these tumors can produce significant neuropathy as a presenting symptoms.
  • 21. Osseous Lesions • Osseous lesions include giant cells tumors, osteoblastoma, aneurysmal bone cysts, osteogenic sarcoma, Ewings sarcoma, myeloma and chondrosarcoma. • These lesions represent 10% of all the retrorectal tumors. • These may be the most aggressive of all the retrorectal tumors and can be very locally destructive and have pronounced metastatic potential.
  • 22. Diagnosis • HISTORY: • Because of the location of these tumors in presacral area, the symptomatology tends to be vague and non-specific. • Many patients will have lower back pain or pelvic pain; however, in general, there is no plethora of common findings.
  • 23. • Patient with advanced tumors can have constipation, sexual dysfunction, urinary incontinence and other leg and gluetal symptoms.
  • 24. Physical Exam • There is no specific exam finding in these tumors. • Many of these tumors are diagnosed incidentally on rectal examination and in fact 97% of presacral lesions are palpable in digital rectal exam. • Patient with congenital cyst or tumor may have a post-anal sinus, however , the most likely etiology of post-anal sinus is perianal fistulous disease.
  • 25. Imaging Studies • Plain Xrays • CT scan • MRI • Sigmoidoscopy • Endo-rectal U/S
  • 26. Plain Xrays • Plain films have limited utility but can sometimes demonstrate osseous destruction of the sacrum or calcifications within the tumor itself. • In patient with anterior sacral meningicele, the classic “scimitar sign” can often be seen on plain films.
  • 27. CT scan A CT scan can be used to determine whether a lesion is solid or cystic, evaluate cortical bone destruction, and assess involvement of adjacent viscera.
  • 28. MRI • MRI with gadolinium is the imaging modality of choice for retrosacral tumors. • MRI is critical in the management of these tumors by facilitating accurate diagnosis, determining the anatomic extent of the lesion and selecting optimal surgical approach.
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  • 34. Sigmoidscopy • The preoperative assessment of the recto-rectal tumors should include intralumonal evaluation of the rectum via flexible sigmoidscopy. • Understanding the extent of the mass of the tumor on the rectum and the ability to assess the mucosal integrity of the rectum are both important elements of the pre-op prepration. • Flexible sigmoidscopy allows for a better assessment of the upper and lower extents of the sphincter complex.
  • 35. Endo-rectal Ultrasound • ERUS can be utilized to assess the relationship of tumors to the muscular layers of the rectum and anal sphincters; despite the fact that majority of the lesions are well circumscribed, the subset of tumors that are not circumscribed can be quite locally advanced and destrcutive. • ERUS can also allow a very preliminary assessment of the sacral bony destruction by tumors.
  • 36. Preoperative Biopsy • Biopsy of the presacral tumors presents a twofold question. • First, is the biopsy associated with a higher rate of local recurrence? • Second, does biopsy have proven utility in the management of presacral tumors, i.e., does bit change the management?
  • 37. • It is universally acknowledged that biopsy of presacral tumors via transrectal or transvaginal is strongly discouraged as it is possible to infect a sterile cystic lesion. • In additon, biopsy via these routes necessitates either partial or complete proctectomy or vaginectomy to remove the biopsy tract in continuity with the sacral tumors in order to prevent recurrence.
  • 38. • More recent data suggest that percutaneous biopsy of retrorectal tumors can be performed without an increased risk of recurrence. • There is a role for biopsy in unresectable, sizeable or aggressive tumors such as Ewing’s sarcoma or osteosarcoma where preoperative radiation or chemotherapy could be of valve for systemic or local control or to improve the likelihood of resectability. • It is our current practice to excise the biopsy tract and site at the time of definitive surgery.
  • 39. Treatment Role of Preoperative Neoadjuvant Therapy: • Retrorectal tumors can exhibit a diverse set of behaviours and can be quite large and locally advanced by the time they are diagnosed. • In addition, a subset of pelvic sarcomas has fairly significant systemic metastatic potential. With this in mind, there is a definite role of neoadjuvant chemotherapy for some of these tumors. • In locally advance tumors, where resectability is an issue, neoadjuvant radiotherapy may render some benefit in decreasing tumor size and increasing resectability.
  • 40. Surgical Treatment • Unless the lesion is unresectable or there is evidence of systemic metastasis, presacral tumors should be resected, as 30-40% of the lesions will be malignant and benign lesions can undergo malignant transformation.
  • 41. Preoperative planning • The key to preoperative planning is understanding the extent of the resection field. • In patients that have direct invasion of the muscular wall of the rectum, proctectomy must be anticipated. • In cases of bony invasion, partial sacrectomy is planned.
  • 42. • Pelvic side wall involvement may necessitate intraoperative radiotherapy and vascular and ureteric reconstruction. • The assembly of a multispecialty team of colorectal, urologic, neurosurgical, orthopedic, vascular and plastic surgeon is a prerequisite for many of these undertakings.
  • 43. Surgical Apporach • The location, the morphology and the involvement of other pelvic structures dictate the operative approach. • In general, a well-circumscribed presacral lesions whose uppermost extent can be palpated on digital rectal examination can usually be approached via a posterior approach. • In lesions above S4 level, a purely abdomonal approach can be considered. • Lesions spanning both above and below are best approached via a combined abdominal and posterior approach.
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  • 45. Anterior Approach • A midline incision is made,and a thorough exam of all quadrants of the abdomen should be performed to assure that there are no metastasis. The sigmoid colon is mobilized along the white line of Toldtm and the presacral space is entered at the level of sacral promontory. • The left & right hypogastric nerves are identified and preserved. • The rectum is pulled forward and dissected off the anterior aspect of the lesion and then the posterior aspect of the lesion is dissected off the presacral fasica.
  • 46. • If the middle sacral artery is the main arterial supply of the lesion, then it has to be ligated prior to removing the lesion. • With advances in minimally invasive techniques, a laproscopic approach to removing these tumors has been shown to be a safe and feasible option.
  • 47. Posterior Approach • For low-lying tumors, the patient is placed in prone jack- knife position with the buttocks spread with tape. • An incision is made over the lower portion of the sacrum and coccyx down to the anus taking care to avoid demage to the external sphincter. • Resection of the tumor may be facilitated by transection of the anococcygeal ligament and coccyx. • The lesion can then be dissected from the surrounding tissues including the rectal wall, in a plane between rectorectal fat and the tumor mass itself
  • 48. • In case of very small lesions, the surgeon may double-glove the left hand and, with index finger in the anal canal and lower rectum, push the lesion outward away from the depths of the wound facilitating dissection of the lesion off the wall of the rectum without injury. • If necessary, the lower sacrum or coccyx or both can be excised en bloc with the lesions to facilitate excision.
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  • 52. Combined Abdominal and Perineal Appraoch • If the upper pole of the tumor extends clearly above S3 level, an anterior and posterior approach is usually indicated. • Patient may be placed in the supine or lateral position depending on the surgeon preference and previous experience. • If the anterior-posterior approach is necessary, the patient can be placed in a “sloppy-lateral position to facilitate a simultaneous two-team approach. • It is always recommended to do cystoscopy and bilateral ureteral stent placement before laprotomy.
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  • 55. Conclusion • Because of their rarity, the diagnosis of presacral tumors can be challenging. Accurate and reliable diagnostic imaging is essential to identify the optimal surgical approach. Surgical management should be determined by the nature and location of the lesion and the extent of involvement of surrounding structures while minimizing the morbidity to the patient. •