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15 Benign Tumors of the Anorectum
215
obstruction, the possibility of this disease should
be borne in mind (Manley and Skyring 1961 ).
The tumor usually arises from the submucosal
or the muscularis layer and is usually sessile.
Usually slow growing, the mucosa over the tumor
gradually gets stretched and thinned out, leading
to ulceration and bleeding (Kim and Kim 1988 ).
Confi rmation of diagnosis is by excision biopsy,
which is also an adequate treatment.
15.3.6 Neurilemmoma or
Schwannoma
Neurilemmomas or schwannomas are rare tumors
that arise from Schwann cells. They occur very
rarely in the rectum, presenting as intraluminal
polypoidal lesions. Occasionally the overlying
mucosa may ulcerate (Miettinen et al. 2001 ).
They can also present as tumors arising from the
presacral space, palpable on digital rectal examination. Assessment of the extent of the tumor is
best done by computed tomography imaging
(Kovalcik et al. 1978 ). They are treated by local
excision or by a local resection, by a transcoccygeal approach, or by a combined abdominotranssacral approach. Although these tumors are
benign, there have been reports of recurrences
following resection (Maciejewski et al. 2000 ).
15.3.7 Hemangioma
Only about 200 cases of hemangioma of the rectum have been reported (Hervías et al. 2004 ).
They are thought to be congenital in origin due to
embryonic sequestration of mesodermal tissues.
However, some authors consider it as neoplastic
in nature. They are usually of two types, the capillary hemangioma and the cavernous hemangioma (Fig. 15.10 ).
The capillary hemangioma is made up of narrow caliber, thin-walled vessels, lined by and
closely packed within a well-differentiated
hyperplastic endothelial lining. They are usually
well encapsulated and arise from the submucosal
vascular plexus. The cavernous hemangioma
consists of large caliber, thin-walled vessels, with
Fig. 15.10 Hemangioma
supportive stroma consisting of connective tissue
and smooth muscle fi bers (Amarapurkar et al.
1998 ). These may occur as (a) multiple phlebec-
tasia type, with multiple, less than 1 cm diameter
lesions, or (b) polypoidal type, or (c) diffuse,
expansive type, which varies in size and extent,
involving long and multiple segments of the
intestine. Hemangiomas usually present with
symptoms of bleeding from the gastrointestinal
tract. Cavernous hemangiomas tend to bleed
more frequently and massively, compared to capillary hemangiomas. Hemangiomas can also
enlarge and produce symptoms of intestinal
obstruction (Tan et al.
1998 ). Physical examina-
tion in these patients may also reveal hemangiomas in the skin or mucous membrane.
Contrast-enhanced computed tomography or
magnetic resonance imaging may be required to
assess their extent, as these lesions are known to
involve the perirectal and the gluteal region.
Treatment options include injection of sclerosing
agents, ligating the bleeding vessels, and localized
excision of the lesion (Richardson 1991 ). Although
abdominoperineal resections have been performed
in the past, sphincter-preserving operations are recommended if bleeding can be controlled (Wang
et al. 2010 ). Some success with radiotherapy has
been reported in the treatment of distal perirectal
hemangiomas (Chaimoff and Lurie 1978 ).
216
R. Mohan
15.3.8 Lymphangioma
Lymphangiomas can very rarely occur in the rectum; they are noted to occur more commonly in
the colon. The earliest report of a rectal lesion
was in 1932 (Chisholm and Hillkowitz 1932 ).
The lesion is thought to arise in the submucosal
lymphatic plexuses into which the villi drain. It
has also been suggested that these occur due to
obstructed mesenteric lymphatics (Dodd et al.
1970 ). Evaluation by proctoscopy shows numer-
ous extramucosal cystic masses, extending proximally from the anorectal junction. Microscopic
examination reveals submucosally located, nonencapsulated, and poorly circumscribed mass of
thin-walled, irregular vascular channels, with
dilated lymphatic vessels extending from the submucosa to the lamina propria. The vessels are
fi lled with fl uid resembling lymph, with large
number of lymphocytes within the lymphatic
channels and in the thin septae separating them
(Corman and Haggitt 1973 ). Lymphangioma
measuring less than 2 cm can be excised endoscopically, but larger lesions may require transanal excision (Poulos et al. 1997 ).
15.4 Benign Exogenous, Extrinsic, and Miscellaneous Tumors
15.4.1 Barium Granuloma
These occur as small, white, or yellowish plaques
and are felt as fi rm submucosal nodules, usually
in the distal rectum. They tend to occur at about
4–8 cm from the anal verge, on the anterior or
posterior rectal wall. Patients are usually asymptomatic, but the lesion may be palpable and may
mimic carcinoma of the rectum on digital rectal
examination (Gowda et al. 2014 ) (Fig. 15.11 ).
Trauma to the rectal mucosa caused during introduction of barium enema catheter tip or therapeutic interventions such as a polypectomy prior to a
barium study may cause a break in the rectal
mucosa, with barium getting retained in the submucosal plane. Microscopic examination usually
reveals a foreign body granulomatous reaction
(Lewis et al. 1975 ). No malignant transformation
Fig. 15.11 Barium granuloma
has ever been reported. Transanal excision is necessary to confi rm the diagnosis which is also
adequate treatment.
15.4.2 Endometriosis
Endometriosis can rarely present with the involvement of the anorectum or the perineum, occurring
only in females. Although very few cases of anorectal and perineal endometriosis have been
reported in the literature, involvement of the gastrointestinal tract is thought to occur in about
5.4 % of patients with endometriosis (Prystowsky
et al. 1988 ). Malignant transformation into adeno-
carcinoma has been reported in a patient with rectal endometriosis but is an extremely rare
occurrence (Magtibay et al.
cally present with cyclical pain, which is a dull
ache radiating to the pelvis, the rectum, the vagina,
or the perineum. There may be tenesmus, cyclical
bowel disturbances with painful defecation, dyspareunia, bowel obstruction, and occasionally
bleed per rectum. A small percentage of patients
are asymptomatic. Characteristic, small, tender
nodules, fi rm but sometimes hard in consistency,
may be palpable on bidigital rectovaginal examination. A clinical diagnosis can easily be made,
based on the history and clinical examination fi ndings, in most patients.
Histological examination usually reveals
characteristic features of endometriosis such as
2001 ). Patients typi-
15 Benign Tumors of the Anorectum
217
endometrial glands, endometrial stroma, with red
blood cells and hemosiderin pigment (fresh bleed)
or hemosiderin-laden macrophages (old bleed).
However, in patients where the physical fi ndings
are not very obvious, diagnostic laparoscopy or culdoscopy may be necessary to establish the diagnosis. A thorough evaluation of the uterus, the ovaries,
and the pelvis is to be done in all patients. Magnetic
resonance imaging is ideal for assessing the pelvic
structures and the extent of the disease and also
in patients in whom there is suspicion of the anal
sphincters involvement (Siegelman and Oliver
2012 ). Computed tomography imaging is useful if
proximal intestines and the ureters require assessment. Cystoscopy for assessment of the bladder
may be required, if there is suspicion of the involvement of the bladder (Hsu et al. 2010 ). Endorectal
ultrasound is useful in the assessment of perineal
involvement, suspected fi xation of endometriosis to
the rectal wall, and depth of rectal wall involvement
and can be utilized if surgical intervention is being
contemplated (Rossini et al. 2012 ).
Treatment decisions are made based on the severity of the patient’s symptoms, hormonal status, age,
and desire to have children. Medical management
options are medroxyprogesterone acetate, danazol,
and gonadotropin-releasing hormone (GnRH) agonists (Küpker et al. 2002 ). Surgical intervention is
indicated in patients with extensive bowel wall
involvement and in patients with obstructive symptoms. Disk excision of the rectum, excision of the
rectum, or a low anterior resection may be required
depending on the extent of rectal involvement
(Bailey et al.
anal lesions can be managed by wide excision, combined with immediate sphincteroplasty if the anal
sphincters are involved (Minvielle and De La Cruz
1968 ; Dougherty and Hull 2000 ).
1994 ; Urbach et al. 1998 ). Perineal and
15.4.3 Oleoma, Oil Granuloma,
Oleogranuloma,
and Paraffi noma
These tumors may be described as an intramural
pseudotumor that develops as a foreign body reaction. They are due to injection of mineral oil
(paraffi n) for the treatment of hemorrhoids or due
to the administration of enema for the treatment of
constipation. They usually occur in the anal canal
or proximal to the dentate line, in the distal rectum.
The patient may be asymptomatic for many years
after the administration of the agent or may manifest with rapidly increasing symptoms of discomfort and pain. One or more fi rm nodules may be
noted at the injection site. The overlying mucosa
and the adjacent perianal skin may appear congested and infl amed. The lesion may have a cystic
feel and is then referred to as an “oleocyst.”
Microscopic examination under low power
shows large mononuclear phagocytes, epithelioid
cells, eosinophilic leukocytes, and foreign-bodytype multinucleated giant cells, with intervening
large, clear spaces which give a Swiss cheese or
spongiform appearance to the tissues. Staining
with oil red O confi rms the presence of lipid. The
foreign-body-type reaction is usually limited to
the submucosa but may occasionally spread to
the mucosal lamina propria or extend into the
perirectal fat (Mazier et al.
by transanal excision of the lesion.
1978 ). Treatment is
15.4.4 Sarcoidosis
Sarcoidosis may manifest as a submucosal nodule
in the rectum. The patient may have other clinical
features of generalized sarcoidosis, but gastrointestinal presentation with symptoms such nausea,
vomiting abdominal pain, and bleeding per rectum
can uncommonly occur (Konda et al.
et al. 1982 ). The lesion may be palpable on digital
rectal examination as a fi rm submucosal nodule,
with mild mucosal infl ammation seen on proctoscopy. Excision biopsy is necessary to confi rm the
diagnosis and to differentiate it from tuberculosis
and Crohn’s disease. Histological examination
shows typical noncaseating granuloma, consisting
of histiocytes (Gould et al. 1973 ).
1980 ; Tobi
15.4.5 Tuberculosis
Tuberculosis of the anorectum is uncommon, but
tubercular involvement of the gastrointestinal tract
is relatively common occurrence. Patients typically
218
R. Mohan
Fig. 15.12 Rectal tuberculosis
present with clinical symptoms of common anorectal conditions, resulting in diagnostic delays
and errors (Chung et al. 1997 ). Presenting symp-
toms usually vary and common symptoms include
perianal pain, perianal purulent discharge, tenesmus, change in bowel habits, symptoms of bowel
obstruction, bleeding per rectum, and abdominal
pain (Candela et al. 1999 ). Anorectal manifesta-
tions include (a) fi stula in ano with purulent discharge, (b) shallow ulcer with an undermined
edge, (c) multiple mucosal ulcerations as a part of
military disease, (d) verrucous form with smooth
warty excrescences, (e) lupoid form with submucosal nodular mass with mucosal ulceration, and
(f) short and annular stricture with nodular surface
(Ibn et al.
2012 ) (Fig. 15.12 ).
Clinical differentiation from malignancy is
diffi cult when the disease manifests as a submucosal nodular mass or as a stricture. Histological
evaluation is necessary to confi rm the diagnosis
and also to differentiate from malignancy and
Crohn’s disease (Gupta 2005 ). Once the diagno-
sis is confi rmed by histopathology, antitubercular
therapy can be initiated. Surgical intervention is
indicated when routine histopathology is inadequate, inconclusive, or unable to conclusively
rule out malignancy (Yanagida et al. 1997 ).
Strictures with stenosis that persist in spite of a
completion of a course of antitubercular therapy
will also require surgical intervention.
Conclusion
Benign tumors of the anorectum pose diagnostic challenges and dilemmas in planning
treatment. In lesions presenting as a frank
infl ammatory condition, differentiation from
other infl ammatory pathologies affecting the
region is necessary, before commencing specifi c treatment. Some of these tumors are
benign at presentation but have potential to
turn malignant over time. Some can easily be
mistaken for malignancy, with aggressive
resections being contemplated. A high index
of suspicion regarding their benign nature,
with comprehensive background knowledge
of the common pathological conditions and
variations, combined with meticulous pathological evaluation, is necessary for proper
diagnosis and treatment of benign tumors of
the anorectum.
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