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25 Rectal Polyps and Other Neoplasms
473
that not all small sessile polyps seen in the rectum
are hyperplastic and biopsy is essential to accurately identify the nature of the polyp.
Juvenile Polyps
Juvenile polyps (also called congenital polyps,
retention polyps, or juvenile adenomas) are typically pedunculated, spherical polyps with a
smooth surface. They are usually grey-white but
may be dark red [35]. The majority are 1.0–
1.5cm in diameter but they may be as large as
4cm. The cut surface of juvenile polyps shows
mucous lled cystic spaces. Histologically, juvenile polyps are quite distinctive, consisting of
normal columnar epithelial cells and goblet cells
arranged in cystically dilated glands set in an
abundant stroma (Fig.25.13). The surface epithelium is often ulcerated. These lesions are
thought to be hamartomatous or inammatory in
nature and have no malignant potential. Juvenile
polyps are most frequently found in children
under the age of 10 but can occur at any age [36].
Males are affected twice as often as females in
the pediatric population while in adults, the ratio
increases to 13:1.
The most common symptom of juvenile polyps is rectal bleeding, often due to autoamputation which occurs in 10% of cases [36]. Other
presentations include prolapse of a rectal mass,
intussusception, abdominal pain and diarrhea.
Juvenile polyps are typically diagnosed by
means of sigmoidoscopy or colonoscopy. Ninety
percent of juvenile polyps are located within
20cm of the anal verge and multiple polyps are
found in 30% of patients. McColl and colleagues
described a juvenile polyposis syndrome, which
is inherited in an autosomal dominant manner
[37]. Patients with this syndrome have multiple
juvenile polyps, some of which may be in the
stomach or small bowel, and a family history of
adenomas or adenocarcinomas of the colon. In
addition to juvenile polyps, patients with this
syndrome may have colorectal adenomas.
Patients with juvenile polyposis coli have a
higher recurrence rate (90%) than do those individuals who present with a single juvenile polyp
(20%) [38]. They may present with iron deciency anemia, hypoproteinemia, hypokalemia or
nger clubbing [39]. Juvenile polyposis coli is
considered a premalignant condition. Treatment
is subtotal colectomy with ileorectal anastomosis. In cases in which the rectum cannot be
cleared of polyps, restorative proctocolectomy
should be considered.
Cronkhite-Canada Syndrome
Cronkhite-Canada syndrome is characterized by
gastrointestinal polyposis, hyperpigmentation,
alopecia and nail dystrophy [40]. It is felt to be a
variant juvenile polyposis with ectodermal
changes and without evidence of genetic transmission. Diarrhea and malabsorption produce
severe vitamin deciency, hypoproteinemia and
uid and electrolyte abnormalities. Other symptoms and signs include anemia, rectal bleeding,
abdominal pain, weakness, nausea, vomiting, loss
of taste, and a variety of neurologic complaints.
Hair loss and nail and skin changes may be evident before the gastrointestinal symptoms become
apparent.
Fig. 25.13 Histology of hyperplastic polyp
Hamartomatous Polyps
The syndrome of characteristic hamartomatous
polyps of the gastrointestinal tract and pigmented
macules of the mucous membranes and skin was
initially described by Peutz [41] Jeghers and colleagues [42] later reported a number of cases of
474
K. M. BullardDunn
the syndrome. The disease is transmitted in an
autosomal dominant fashion; however, there are
sporadic cases [22]. In this syndrome, the small
bowel is the most frequent location for polyps,
particularly the jejunum.
Polyps may also be seen in the stomach, colon
and rectum [43]. Grossly, Peutz-Jeghers polyps
cannot be distinguished from adenomatous polyps. Peutz-Jeghers polyps may be sessile or
pedunculated, and they range in size from a few
millimeters to 6–7 cm. Histologically, these polyps are characterized by abnormal muscularis
mucosae, which branches out in a tree like fashion
(Fig.25.14) while the epithelium appears normal.
The cutaneous pigmentation is typically on
the buccal mucosa and on or around the lips
although ngers and toes may also show these
macules. The onset of pigmented areas is at birth
or infancy, but may regress later in life while the
polyps commonly occur in adolescence or early
adulthood. Abdominal pain, the most common
presenting symptom, can be difcult to control
because of obstruction from the polyp or intussusception. Rectal bleeding, polyp prolapse, passage of a polyp, anemia or hematemesis are other
reported signs and symptoms. Endoscopy and
contrast studies such as enteroclysis are used to
diagnose polypoid disease.
Patients not uncommonly undergo multiple
operations for bleeding or small bowel obstruction. Under these circumstances, if the diagnosis
is known, multiple polyps can be removed by
enterotomy and polypectomy. Invagination of the
bowel via an enterotomy or endoscopic polypectomy allows multiple polyps to be removed
Fig. 25.14 Histology of Peutz-Jeghers polyp
through one enterotomy site [43]. Massive small
bowel resection should be avoided.
The association of Peutz-Jegher syndrome
with malignancy is unclear. Konishi and colleagues reviewed 103 cases reported in the literature [44]. These 103 patients had a total of 117
neoplasms including 50 cancers of the gastrointestinal tract. These were most commonly located
in the colon and rectum. Spigelman and colleagues [45] found malignant tumors in 22% of
72 patients with Peutz-Jeghers syndrome in the
St. Mark’s Polyposis Registry. Giardiello and
colleagues [46] reported a 48% rate of malignancy in 31 patients studied. In contrast to the
above, Dozois and colleagues [47] failed to identify any cancers in a group of 48 patients from
the Mayo Clinic followed for a median of
33years.
Williams and colleagues [48] from St Mark’s
Hospital recommend upper and lower gastrointestinal endoscopy every other year, repeat evaluation if the patient becomes symptomatic, and
laparotomy for any small bowel polyp larger than
1.5cm in diameter. Periodic mammography and
ultrasound of the abdomen are useful since these
patients have a higher incidence of breast, ovarian and pancreatic cancers. The most important
issue is to distinguish Peutz Jeghers polyps from
familial polyposis coli, which are adenomatous
polyps with high malignant potential.
Lipomas
Lipomas are benign submucosal fatty lesions that
occur infrequently in the colon and rectum. [49]
Although these neoplasms are more commonly
found in the ascending colon, they can be found in
the rectum. The majority of lipomas are asymptomatic and discovered incidentally. The typical
endoscopic appearance of a lipoma is a smooth,
yellow, submucosal lesion that exhibits the characteristic “pillow sign” or “cushion sign” when
pressed with a forceps (Fig.25.15). Larger lesions,
usually greater than 2cm, may rarely may cause
bleeding or obstruction, or intussusception. Small
asymptomatic lesions do not require resection,
although ulceration can make differentiating these
benign lesions from malignancy more difcult.
Larger lipomas should be resected by transanal
25 Rectal Polyps and Other Neoplasms
Fig. 25.15 Endoscopic appearance of a lipoma demonstrating the “pillow sign”
excision, enucleation or limited proctectomy. It is
important to note that lipomas are difcult to
resect using an endoscopic snare because of the
energy required and the risk of bleeding and/or a
transmural burn [49–51].
475
with brisk and sometimes life threatening bleeding. Diagnosis is based upon endoscopic appearance, although confusion with other conditions,
especially inammatory, is not uncommon.
Hemangiomas are typically dark red or blue, and
cavernous lesions may appear as a polypoid mass
[52]. CT scan may show calcication in the submucosal plexus, especially with cavernous
hemangiomas.
Treatment for hemangiomas depends upon
extent of bleeding, size, and location. Capillary
lesions and small cavernous hemangiomas may
be successfully treated endoscopically, either by
excision, cautery, or sclerotherapy. Larger lesions
are more difcult to treat. Sclerotherapy, angioembolization, and cryotherapy have all been
reported. For larger, symptomatic lesions, resection is denitive therapy. Mucosal sleeve resection is sometimes successful in resecting
anorectal lesions [53].
However, abdominoperineal resection is occasionally required for large cavernous hemangiomas with bleeding that cannot be controlled by
other means [54–56].
Hemangiomas
Hemangiomas are vascular malformations
thought to be congenital in origin. Although
gastrointestinal hemangiomas are considerably
more rare than cutaneous hemangiomas, these
lesions do occur throughout the gastrointestinal
tract, including in the anorectum. Although
rare, patients with GI hemangiomas often manifest cutaneous hemangiomas as well.
Hemangiomas are classied as capillary hemangiomas that are comprised of ne, closely
packed capillaries, or cavernous hemangiomas
consisting of large, thin-walled blood vessels.
Cavernous hemangiomas often present as a
mucosal mass. In the colon and rectum, cavernous hemangiomas are far more common than
capillary lesions.
Painless bleeding is the most common symptom of anorectal hemangiomas, often beginning
early in life. Bleeding from capillary hemangiomas is typically slow and/or occult. Cavernous
hemangiomas, on the other hand, may present
Solitary Rectal Ulcer Syndrome/ Colitis Cystica Profunda
Solitary rectal ulcer syndrome and colitis cystica
profunda (Fig. 25.16) can present as a rectal
mass that can be difcult to differentiate from
other rectal neoplasms. These lesions are thought
to be associated with internal intussusception.
Patients may complain of pain, bleeding, mucus
discharge, or outlet obstruction. In solitary rectal
ulcer syndrome, one or more ulcers are present
in the distal rectum, usually on the anterior wall.
In colitis cystica profunda, nodules or a mass
may be found in a similar location. Biopsy of an
ulcer or mass is mandatory to exclude malignancy. Nonoperative therapy including highber diet, defecation training to avoid straining,
and laxatives or enemas is effective in the majority of patients. Biofeedback has also been
reported to be effective in some patients.
Abdominal or perineal repair of prolapse as
described above is reserved for highly symptomatic patients who have failed all medical interventions [49, 56].
476
K. M. BullardDunn
a
choice. Recurrence is common after local resection, but most small leiomyomas can be adequately
treated with limited resection. Lesions larger than
5 cm should be treated with radical resection
because the risk of malignancy is high [49, 55].
Malignant
Leiomyosacrcoma
Leiomyosarcoma is rare in the gastrointestinal
tract. When this malignancy occurs in the large
intestine, the rectum is the most common site.
Leiomyoma of the rectum is usually low grade,
and, as such, can be difcult to differentiate from
leiomyoma. Denitive diagnosis is usually made
b
after resection. Symptoms, when they occur, are
usually bleeding or obstruction. A radical resection is indicated for most of these tumors,
although local excision can be considered for
small lesions. Despite complete resection, recurrence is not uncommon and prognosis is generally poor [49, 57].
Fig. 25.16 (a) Solitary rectal ulcer syndrome. (b) Colitis
cystica profunda can be difcult to distinguish from
carcinoma
Leiomyomas
Leiomyomas are benign smooth muscle tumors
that arise from the muscularis mucosa or muscularis propria of the bowel. Although they are most
common in the upper GI tract, they can occur in
the rectum or, less commonly, in the anal canal.
Most patients are asymptomatic and lesions are
often diagnosed incidentally when a mass is seen
on endoscopy or felt on digital rectal examination.
Large lesions may ulcerate and cause bleeding or
result in tenesmus or frank obstruction.
Management of leiomyoma is based largely upon
the inability to differentiate a benign lesion from a
malignant leiomyosarcoma. As such, wide local
excision or radical resection is the treatment of
Gastrointestinal Stromal Tumors (GIST)
Are mesenchymal tumors that arise from the
interstitial cells of Cajal. The vast majority
(>95%) of GISTs express CD117 (KIT), and as
such, are sensitive to tyrosine kinase inhibitors
(TKIs), such as imatinib mesylate and sunitinib
malate. GISTs are most common in the proximal GI tract but do occasionally occur in the
colorectum (5–10%). While small GISTs may
be asymptomatic and discovered incidentally,
larger lesions can cause bleeding, obstruction,
or abdominal pain. Treatment of choice is surgical resection (either local excision or radical
resection) with microscopically negative margins if possible, however, local recurrence is
common. For larger marginally resectable
tumors, Tyrosine kinase inhibitors such as imatinib can be used to shrink the tumor. These
agents can also be considered for adjuvant therapy after resction and are useful for treating
metastatic disease [58].
25 Rectal Polyps and Other Neoplasms
477
Carcinoid Tumors
Carcinoid tumors are common in the gastrointestinal tract. Although nearly two thirds are found
in the small bowel, up to 25% are found in the
rectum. Most small rectal carcinoids are benign,
and overall survival is greater than 80%. However,
the risk of malignancy increases with size, and
more than 60% of tumors greater than 2 cm in
diameter are associated with distant metastases.
In addition, smaller carcinoids that invade the
muscularis propria or have other atypical features
are more likely to metastasize. Unlike small
bowel carcinoids, rectal carcinoid tumors are usually solitary and rarely present with synchronous
lesions. Rectal carcinoids are less likely to secrete
vasoactive substances than carcinoids in other
locations, and carcinoid syndrome is uncommon
in the absence of hepatic metastases. Serum and
urine levels of serotonin, 5-HT, and 5-HIAA are
typically normal. Very small carcinoids (<1cm)
can occasionally be endoscopically resected.
Tumors up to 2cm may be amenable to traditional transanal resection or to transanal endoscopic surgery. Larger tumors or tumors with
obvious invasion into the muscularis require more
radical surgery. In comparison to rectal carcinoids, carcinoid tumors in the proximal colon are
less common but more likely to be malignant.
Size also correlates with risk of malignancy in
this location, and tumors less than 2cm in diameter rarely metastasize. However, the majority of
carcinoid tumors in the proximal colon present as
bulky lesions and up to two-thirds will have metastatic spread at the time of diagnosis. These
tumors should usually be treated with radical
resection. Because carcinoid tumors are typically
slow growing, patients with distant metastases
may expect reasonably long survival. Symptoms
of carcinoid syndrome can often be alleviated
with somatostatin analogues including octreotide
and/or interferon-a. Tumor debulking can offer
effective palliation in selected patients [49, 59].
Carcinoid Carcinomas
Composite carcinoid carcinomas (adenocarcinoids) have histologic features of both carcinoid
tumors and adenocarcinomas. In comparison to
carcinoids, these tumors are usually high grade
and poorly differentiated. They tend to be aggressive and are associated with a poor prognosis.
The natural history of these tumors more closely
parallels that of adenocarcinomas than carcinoid
tumors, and regional and systemic metastases are
common. Carcinoid carcinoma of the colon and
rectum should be treated according to the same
oncologic principles as followed for management
of adenocarcinoma. Surgical resection for cure is
sometimes possible for localized lesions without
metastatic spread. Adjuvant chemotherapy and
radiation have also been utilized in this setting
[49, 60].
Lymphoma
Gastrointestinal lymphoma may be primary or
generalized/secondary. Primary GI lymphomas
occur most frequently in the terminal ileum and
cecum. Lymphoma involving the colon and rectum is rare, but accounts for about 10% of all gastrointestinal lymphomas. Presentation, treatment
and prognosis differ between patients with lymphoma occurring as a localized entity in the rectum versus those in whom there is generalized
lymphoma with rectal involvement. Symptoms in
isolated rectal lymphoma include bleeding,
obstruction, and pain. These tumors may be clinically indistinguishable from adenocarcinomas.
Bowel resection is the treatment of choice for
isolated colorectal lymphoma. Radiation has
been reported to be useful in unresectable cases.
Adjuvant therapy may be given based upon the
stage of disease and is useful for systemic disease
[49, 56, 61].
Retrorectal/Presacral Tumors
Retrorectal/presacral tumors are rare and can be
benign or malignant. The retrorectal space contains multiple embryologic remnants derived
from a variety of tissues including the neuroectoderm, notochord, and hindgut. As such, tumors
that develop in this space are often heterogeneous. Congenital lesions are most common,
478
K. M. BullardDunn
comprising almost two-thirds of retrorectal
lesions. The remainders are classied as neurogenic, osseous, inammatory, or miscellaneous
lesions. Malignancy is more common in the pediatric population than in adults, and solid lesions
are more likely to be malignant than are cystic
lesions. Inammatory lesions may be solid or
cystic and usually represent extensions of infection either in the perirectal space or in the
abdomen.
The majority of congenital lesions are developmental cysts. These lesions may arise from all
three germ cell layers. Dermoid and epidermoid
cysts are benign lesions that arise from the ectoderm. Enterogenous cysts arise from the primitive
gut. Anterior meningocele and myelomeningocele
arise from herniation of the dural sac through a
defect in the anterior sacrum. A “scimitar sign”, a
rounded, concave sacral border without any bony
destruction) is the pathognomonic radiographic
appearance of this condition (Fig.25.17).
Solid lesions include teratomas, chordomas,
neurologic tumors, or osseus lesions. Teratomas
are true neoplasms and contain tissue from each
germ cell layer and often contain both cystic and
solid components. Teratomas are more common
in children than in adults, but are more com-
monly malignant in adults. Chordomas are the
most common malignant tumor in this region and
arise from the notochord. These are slow growing, invasive cancers that show characteristic
bony destruction. Neurogenic tumors include
neurobromas and sarcomas, neurilemomas,
ependymomas, and ganglioneuromas. Osseous
lesions include osteomas and bone cysts, as well
as neoplasms such as osteogenic sarcoma,
Ewing’s tumor, chondromyxosarcoma, and giant
cell tumors.
Patients may present with lower back, pelvic,
or lower extremity pain, gastrointestinal symptoms, or urinary tract symptoms. Most lesions are
palpable on digital rectal examination. While
plain X-rays and CT scans are often used to evaluate these lesions, pelvic MRI is the most sensitive and specic imaging study. Myelogram is
occasionally necessary if there is central nervous
system involvement. Treatment is almost always
surgical resection. Although survival is excellent
after resection of benign lesions, local recurrence
is not uncommon. Prognosis after resection of
malignant lesions is highly variable and reect
the biology of the underlying tumor.
The role of biopsy for solid tumors in this
setting has been controversial. Historically, the
recommendation was to avoid biopsy because
of the risk of infection or needle tract seeding.
This recommendation has recently been challenged, especially for large and/or unusual
tumors that would be better treated with multimodality neoadjuvant therapy. A recent study
conrmed the utility of needle biopsy of solid
lesions and refuted concerns about needle tract
seeding [2]. As such, most solid lesions should
be biopsies regardless of resectability. In contrast, biopsy or aspiration of cystic lesions,
should still be avoided because of the risk of
infection [49, 62, 63].
Fig. 25.17 Presacral meningocele demonstrating scimitar sign and delineation of anatomy on MRI.With permission from Eric J.Dozois, MD
Melanoma
Anorectal melanoma (Fig. 25.18) comprises less
than 1% of all anorectal malignancies and 1–2%
of melanomas. Diagnosis is often delayed and
symptoms are attributed to hemorrhoidal disease.
Compared to cutaneous melanoma, prognosis for
patients with anorectal disease remains almost
25 Rectal Polyps and Other Neoplasms
Fig. 25.18 Anal melanoma presenting in a hemorrhoid
universally poor. Overall 5-year survival is less
than 10%, and many patients present with systemic metastasis and/or deeply invasive tumors at
the time of diagnosis. A few patients with anorectal melanoma, however, present with isolated local
or locoregional disease that is potentially resectable for cure, and abdominoperineal resection
(APR) and wide local excision have been advocated. Recurrence is common and is usually systemic regardless of the initial surgical procedure.
Local resection with free margins does not increase
the risk of local or regional recurrence and APR
offers no survival advantage over local excision. In
some patients, wide local excision may not be
technically feasible and APR may be required if
the tumor involves a signicant portion of the anal
sphincter or is circumferential. The addition of
adjuvant chemotherapy, biochemotherapy, vaccines, or radiotherapy may be of benet in some
patients, but efcacy remains unproven [49].
References
1. O'Brien MJ, Winawer SJ, Zauber AG, et al. The
national polyp study. Patient and polyp characteristics associated with high-grade dysplasia in colorectal
adenomas. Gastroenterology. 1990;98:371–9.
2. Whitlow CB, Opelka FG.Other rectal neoplasms. In:
Beck DE, Wexner SD, editors. Fundamentals of anorectal surgery. 2nd ed. London: W B Saunders; 1998.
p.382–99.
3. Fenoglio-Prieser CM, Pascal RR, Perzin
KH. Adenomas. In: Hartman WH, editor. Tumors
of the intestines. Washington, DC: Armed Forces
Institute of Pathology; 1990. p.69–150.
479
4. Fenoglio-Prieser CM.Colonic polyp histology. Semin
Colon Rectal Surg. 1990;2:234–45.
5. Wise PE.Polyps. In: Beck DE, Roberts PL, Saclarides
TJ, Senagore AJ, Stamos MJ, Wexner SD, editors.
The ASCRS textbook of colon and rectal surgery. 2nd
ed. NewYork: Springer; 2011. p.636.
6. Muto T, Bussey HJR, Morson BE.The evolution of cancer of the colon and rectum. Cancer. 1975;36:2251–70.
7. Tierney RP, Ballantyne GH, Modlin M. The adenoma to carcinoma sequence. Surg Gynecol Obstet.
1990;171:81–94.
8. Silverberg SG.Focally malignant adenomatous polyps of the colon and rectum. Surg Gyneeol Obstet.
1970;131:103–14.
9. Grinnell RS, Lane N. Benign and malignant adenomatous polyps and papillary adenomas of the colon
and rectum. An analysis of 1856 tumors in 1335
patients. Surg Gynecol Obstet. 1958;106:519–38.
10. Stryker JS, Wolff BG, Culp CE, et al. Natural history of untreated colonic polyps. Gastroenterology.
1987;93:1009–13.
11. Waye JO, O’Brien MJ.Cancer in polyps. In: Cohen
AT, Winawer SJ, editors. Cancer of the colon, rectum
and anus. NewYork: McGraw-Hill; 1995. p.456–76.
12. Pollard CW, Nivatvongs S, Rojanasakul A, et al.
The fate of patients following polypectomy alone
for polyps containing invasive carcinoma. Dis Colon
Rectum. 1992;35:933–7.
13. Whitlow CB, Gathright JB, Hebert SJ, et al. Longterm survival after treatment of malignant colonic polyps. Dis Colon Rectum. 1997;40:929–34.
14. Haggitt RC, Glotzbach RE, Soffer EE, Wruble
LE.Prognostic factors in colorectal carcinomas arising in adenomas: implications for lesions removed
by endoscopic polypectomy. Gastroenterology.
1985;89:328–36.
15. Nivatvongs S, Rojanasakul A, Reiman HM, etal. The
risk of lymph node metastasis in colorectal polyps
with invasive adenocarcinoma. Dis Colon Rectum.
1991;34:323–8.
16. Opelka FG, Hicks TE.Management of malignant polyps. Semin Colon Rectal Surg. 1991;2:296–304.
17. Whitlow CB, Beck DE, Gathright JB.Surgical excision of large rectal villous adenomas. Surg Oncol Clin
N Am. 1996;5:723–34.
18. Galandiuk S, Fazio VW, Jagelman OG, etal. Villous
and tubulovillous adenomas of the colon and rectum.
Am Surg. 1987;153:41–7.
19. Nivatvongs S, Nicholson JO, Rothenberger
OA.Villous adenomas of the rectum: the accuracy of
clinical assessment. Surgery. 1980;87:549–5l.
20. Wheat MW, Ackerman LV.Villous adenomas of the
large intestine. Ann Surg. 1958;147:476–87.
21. Keck JO, Schoetz OJ Jr, Roberts PL, et al. Rectal
mucosectomy in the treatment of giant rectal villous
tumors. Dis Colon Rectum. 1995;38:233–8.
22. Sakamoto GO, Mackeigan JM, Senagore
AJ.Transanal excision of large, rectal villous adenomas. Dis Colon Rectum. 1991;34:880–5.
23. Buess G, Kipfmuller K, Ibald R, etal. Clinical results
of transanal endoscopic microsurgery. Surg Endosc.
1988;2:245–50.
480
K. M. BullardDunn
24. Saclarides TJ, Smith L, Ko S, et al. Transanal
endoscopic microsurgery. Dis Colon Rectum.
1992;35:1183–91.
25. Jorgensen SJ, Ottsen M. Posterior rectotomy for
villous tumors of the rectum. Acta Chir Scand.
1975;141:680–2.
26. Wilson SE, Gordon HE.Excision of rectal lesions by
thoe Kraske approach. Am J Surg. 1969;118:213–7.
27. Thompson BW, Tucker WE. Transsphincteric
approach to lesions of the rectum. South Med J.
1987;80:41–3.
28. Redmond HP, Austin OMB, Clery AP, Deasy
JM.Safety of double-stapled anastomosis in low anterior resection. Br Surg. 1993;80:924–7.
29. Franklin R, McSwain B.Juvenile polyps of the colon
and rectum. Ann Surg. 1992;216:432–7.
30. Cohen AM, Enker WE, Monsky BD.Protectomy and
coloanal construction for rectal cancer. Dis Colon
Rectum. 1990;33:40–3.
31. Enker WE, Steams MW Jr, Janov AJ.Peranal coloanal
anastomosis following low anterior resection for rectal carcinoma. Dis Colon Rectum. 1985;28:576–81.
32. Chiu YS, Spencer RJ.Villous lesions of the colon. Dis
Colon Rectum. 1978;21:493–5.
33. Fenoglio-Prieser CM, Pascal RR, Perzin
KH.Nonneoplastic polyps. In: Hartman WH, editor.
Tumors of the intestines. Washington, DC: Armed
Forces Institute of Pathology; 1990. p.31–67.
34. Mazier WI, Mackeigan JM, Billinghan RP, Dignan
RD. Juvenile polyps of the colon and rectum. Surg
Gynecol Obstet. 1982;154:829–32.
35. McColl I, Bussey HJ, Veale AMO, Be M.Juvenile
polyposis coli. Proc R Soc Med. 1964;57:896–7.
36. Haggitt RC, Pitcock JA.Familial juvenile polyposis
of the colon. Cancer. 1970;26:1232–8.
37. Groseld JL, West KW. Generalized juvenile polyposis coli. Clinical management based on long-term
observations. Arch Surg. 1986;121:530–4.
38. Cronkhite LW Jr, Canada WJ.Generalized gastrointestinal polyposis. An unusual syndrome of polyposis,
pigmentation, alopecia and onychotrophia. N Engl J
Med. 1955;252:1011–5.
39. Peutz JLA. Very remarkable case of familial polyposis of mucous membrane of intestinal tract and
nasopharynx accompanied by peculiar pigmentations
of skin and mucous membrane. Nederl Maandschr V
Geneesk. 1921;10:134–46.
40. Jeghers H, McKusick VA, Katz KH. Generalized
intestinal polyposis and melanin spots of the oral
mucosa, lips and digits: a syndrome of diagnostic signicance. N Engl J Med. 1949;241:993–1005.
41. Utsunomiya J, Gocho H, Miyanaga T, et al.
PeutzJeghers syndrome: its natural course
and management. Johns Hopkins Med J.
1975;136:71–82.
42. Konishi F, Wyse NE, Muto T, et al. Peutz-Jeghers
polyposis associated with carcinoma of the digestive
organs: report of three cases and review of the literature. Dis Colon Rectum. 1987;30:790–9.
43. Spigelman AD, Arese P, Phillips RKS.Polyposis: the
Peutz-Jeghers syndrome. Br J Surg. 1995;82:1311–4.
44. Giardiello FM, Welsh SB, Hamilton SR, et al.
Increased risk of cancer in the Peutz-Jeghers
Syndrome. N J Med. 1987;316:1511–4.
45. Dozois HK Judd ES, Dahlin DC, et al. The PeutzJeghers syndrome. Is there a predisposition to the
development of intestinal malignancy? Arch Surg.
1969;98:509–17.
46. Williams CB, Goldblatt M, Delaney PV.Top and tail
endoscopy’ and follow up in Peutz-Ieghers syndrome.
Endoscopy. 1982;14:82–4.
47. Bullard Dunn KM, Rothenberger DA.Colon, rectum,
and anus. In: Brunicardi C, editor. Schwartz’s principles of surgery. 10th ed. NewYork: McGraw Hill;
2014. p.1175–240.
48. Silverstein FE, Tytgat GNJ, Polyps CI. Tumors in
gastrointestinal endoscopy. 3rd ed. London: Mosby;
1997. p.268–9.
49. Ghanem OM, Slater J, Singh P, Heitmiller RF,
DiRocco JD. Pedunculated colonic lipoma prolapsing through the anus. World J Clin Cases.
2015;3(5):457–61.
50. Silverstein FE, Tytgat GNJ, Polyps CI. Tumors in
gastrointestinal endoscopy. 3rd ed. London: Mosby;
1997. p.340–1.
51. Londono-Schimmer EE, Ritchie JK, Hawley
PR.Coloanal sleeve anastomosis in the treatment of
diffuse cavernous hemangioma of the rectum: long
term results. Br J Surg. 1994;81:1235–7.
52. Ugolini G, Rosati G, Montroni I, Manaresi A, Blume
JF, Taffurelli M.Diffuse cavernous haemangioma of
the rectum and anus: an unusual case of rectal bleeding with challenging management. BMJ Case Rep.
2009;2009:bcr02.2009.1545.
53. Nivatvongs S.Benign neoplasms of the colon and rectum. In: Gordon PH, Nivatvongs S, editors. Principles
and practice of surgery for the colon, rectum, and
anus. 2nd ed. St. Louis: Quality Medical Publishing;
1999. p.567–8.
54. Whitlow CB, Opelka FG. Other rectal neoplasms.
In: Fundamentals of anorectal surgery. 2nd ed.
Philadelphia: WB Saunders; 1998.
55. Nivatvongs S.Benign neoplasms of the colon and rectum. In: Gordon PH, Nivatvongs S, editors. Principles
and practice of surgery for the colon, rectum, and
anus. 2nd ed. St. Louis: Quality Medical Publishing;
1999. p.695–6.
56. NCCN Task Force Report. Update on the management of patients with gastrointestinal stromal tumors.
J Natl Compr Cancer Netw. 2010;8(2):S1–S44.
57. Demetri GD, von Mehren M, Antonescu CR, DeMatteo
RP, Ganjoo KN, Maki RG, Pisters PWT, Raut CP,
Riedel RF, Schuetze S, Sundar HM, Trent JC, Wayne
JD, Kwaan MR, Goldberg JE, Bleday R.Rectal carcinoid tumors: review of results after endoscopic and
surgical therapy. Arch Surg. 2008;143:471–5.
58. Barakat MT, Meeran K, Bloom SR.Neuroendocrine
tumors. Endocr Relat Cancer. 2004;11:1–18.
25 Rectal Polyps and Other Neoplasms
481
59. Nivatvongs S.Benign neoplasms of the colon and rectum. In: Gordon PH, Nivatvongs S, editors. Principles
and practice of surgery for the colon, rectum, and
anus. 2nd ed. St. Louis: Quality Medical Publishing;
1999. p.693–5.
60. Merchea A, Dozois EJ. Lesions originating
within the retrorectal space. J Gastrointest Surg.
2014;18(12):2232–3.
61. Dozois EJ, Jacofsky DJ, Billings BJ, Privitera A,
Cima RR, Rose PS, Sim FH, Okuno SH, Haddock
MG, Harmsen WS, Inwards CY, Larson DW.Surgical
approach and oncologic outcomes following multidisciplinary management of retrorectal sarcomas. Ann
Surg Oncol. 2011;18(4):983–8.
62. Merchea A, Larson DW, Hubner M, Wenger DE, Rose
PS, Dozois EJ. The value of preoperative biopsy in
the management of solid presacral tumors. Dis Colon
Rectum. 2013;56(6):756–60.
63. Chen H, Cai Y, Liu Y, He J, Hu Y, Xiao Q, Hu W, Ding
K. Incidence, surgical treatment, and prognosis of
anorectal melanoma from 1973 to 2011: a populationbased SEER analysis. Medicine. 2016;95(7):e2770.
Retrorectal (Presacral) Tumors
RamonA.Brown andDavidA.Margolin
26
Introduction
Retrorectal tumors encompass a heterogeneous
group of lesions and neoplasms that demonstrate
variable growth patterns. As a result of their anatomic location these rare lesions, manifest with
non-specic symptoms that makes their initial
diagnosis difcult. Often they are found incidentally on physical exam or imaging in the work up
of other disease processes. While these lesions
are fairly well documented in the literature they
are discussed largely in case studies. The largest
series is maintained by the Mayo clinic representing 1 in 40,000 hospital admissions [1, 2].
This chapter will illustrate and characterize the
etiology, diagnosis and management of these
lesions, specically focusing on minimally invasive approaches to this disease process.
Anatomy
The retrorectal space lies between the upper twothirds of the rectum and the sacrum, above the
rectosacral fascia. The boundaries of this potential space include the posterior wall of the rectum
(fascia propria) anteriorly and the sacrum posteriorly. This space extends superiorly to the peritoneal reection and laterally is bound by the lateral
stalks of the rectum, the ureters, the sacral nerve
roots and the iliac vessels. This retroperitoneal
space contains loose connective tissue. The presacral fascia protects the extensive vertebral
plexus of presacral vessels that lie deep to it.
The retrorectal space is thought to contain
totipotential cells from all three germ cell layers.
The wide range of histological variance may be
due to the presence of multiple embryologic remnants and heterogeneous tissue types. These
lesions range from benign cysts to malignant
masses which can invade the surrounding pelvic
structures [3].
R. A. Brown
The Ochsner Clinic Foundation, Ochsner Clinical
School, Ochsner Clinic, New Orleans, LA, USA
D. A. Margolin (*)
The Ochsner Clinic Foundation, Ochsner Clinical
School, Ochsner Clinic, New Orleans, LA, USA
The University of Queensland School of Medicine,
St. Lucia, QLD, Australia
e-mail: damargolin@ochsner.org
© Springer International Publishing AG, part of Springer Nature 2019
D. E. Beck et al. (eds.), Fundamentals of Anorectal Surgery,
https://doi.org/10.1007/978-3-319-65966-4_26
Classication
While a variety of classications systems have
been proposed for presacral tumors, the current
system is a modication of Uhlig and Johnson’s
system which places retrorectal tumors into broad
categories: congenital, neurogenic, osseous,
inammatory, and miscellaneous. In the current
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