Burkitt’s Lymphoma (Ileum) with Intussusception

Diagram 66



Chemotherapy

The patient was treated with 6 courses of

chemotherapeutic

agents,

which

included

cyclophosphamide, vincristine, methotrexate, doxorubicin, and cytarabine.

Follow-Up

(2005)

The patient remains well without evidence of recurrence, 9 years since the resection.

Comment

In Western countries, Burkitt’s Lymphoma is sporadic and accounts for 2% of all lymphomas. They



145



usually present as abdominal tumors, as in this case,

and may affect the jaw and peripheral lymph nodes,

particularly in the neck. It is associated with the

Epstein-Barr virus (EBV) in 20% of cases (Western

disease). The histology shows diffuse changes with

sheets of monomorphic neoplastic lymphoid cells

evenly interspersed with histiocytes, producing the

characteristic “starry sky” pattern.1 The prognosis

without treatment is very poor, however

chemotherapy has been effective in inducing dramatic and complete tumor regression, as in this

patient.



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67



Ileocecal Lymphoma

Male, 82 Years



History

The patient had noted the recent onset of abdominal distention with 2 episodes of acute lower abdominal pain within 2 months. These symptoms were

accompanied by a loss of weight. The stools had

become black in color over a period of 7 days. Examination revealed a large nontender spherical mass

occupying the right lower abdomen. A barium

enema demonstrated dilatation of the terminal

ileum with distortion of the mucosal pattern. A

computerized tomography (CT) examination

revealed a mass in the lower abdomen 10 × 10 ×

8 cm extending into the cecum and ascending colon.

A collection of contrast appeared within the mass,

suggesting a bowel perforation was present.

Colonoscopy revealed a large polypoid mass in the

medial aspect of the proximal ascending colon and

cecum, which obscured the ileocecal valve.

Operation

(12.4.93)

A large confluent mass was confirmed in the ileocecal region. Its bulky nature suggested the possibility of a lymphoma. The mass appeared to

infiltrate the ascending colon, terminal ileum, and

mesentery. Enlarged lymph nodes were present

along the ileocolic vessels. There was no other

pathology detected within the abdomen. A right



Figure 67.1: The left of the field shows 2 lymphoid

follicles with germinal centers. The lymphoma is on the

right. Small bowel villi are seen in transverse section.

146



hemicolectomy was performed, including removal

of 50 cm of terminal ileum.

Pathology

The mass “encased both the terminal ileum and the

proximal right colon.” On section, it was pale in

color with a central cavity that communicated with

the terminal ileum in two places. The mucosa of the

ileum was otherwise normal. Within the mesentery,

seven enlarged, firm lymph nodes were present. Histological examination confirmed the diagnosis of

lymphoma (Figure 67.1). The tumor was a nonHodgkin’s large B cell type with a diffuse pattern.

It was consistent with the so-called MALT lymphoma (arising from mucosal associated lymphoid

tissue). The mesenteric lymph nodes were not

involved with lymphoma. In the cecum, a flat pale

polyp was present measuring 50 × 50 mm, which

on examination was a dysplastic tubulovillous

adenoma.

Follow-Up

Six weeks after operation, a right-sided abdominal

mass was present and a gallium scan confirmed 4

foci of lymphoma within the abdomen. Over the

next 7 months, 5 courses of chemotherapy were

administered (cyclophosphamide, mitozantrone,

vinblastine). The response to treatment was satisfactory and the patient remained in remission until

1997, when the disease recurred. At the age of 87

years, no further treatment was advised. The recurrent lymphoma was not the cause of death 4 years

9 months after operation, when he died unexpectedly during sleep.

Comment

This patient’s clinical presentation and tumor

morphology were typical of small bowel lymphoma.

Such lesions represent 20% of small bowel tumors1

and may be associated with Coeliac or Crohn’s

disease as well as immunodeficiency states. Preoperative diagnosis is unusual, but at operation a

pale bulky tumor will suggest the diagnosis is lymphoma. In this patient, the MALT B cell tumor was

low grade, which is usually associated with a more

favorable prognosis. Adjuvant chemotherapy is frequently associated with resection of the disease, particularly if regional lymph nodes are involved.2



Diagram 67



147



C A S E



68



Multiple Lymphoma and

Ulcerative Colitis

Female, 49 Years



History

The patient had noticed a few weeks of minimal

dark red bleeding from the rectum and was examined by colonoscopy. There was diffuse low grade

colitis (Figure 68.1) affecting the colon with a loss

of vascular pattern and diminution of the haustral

indentation. In the lower third of the sigmoid colon

(at 20 cm), there was nodularity of the mucosa

with associated hyperemia and small submucosal

hemorrhages. Biopsy of this area revealed nonHodgkin’s lymphoma (NHL) of a diffuse type.

Immunochemistry was positive for the B cell

marker L26. Other biopsies of the mucosa showed

nonspecific chronic inflammation. Staging investigation by gallium and computerized tomography

(CT) scans revealed no other foci of lymphoma.

Surgical treatment was advised in preference to

chemotherapy.



Figure 68.2: Diffuse large B cell lymphoma.



Operation

(11.14.94)

The small bowel and colon were normal in appearance, with some thickening apparent in the lower

third of the sigmoid colon on palpation. Small firm



lymph nodes were palpable in the mesentery of

the sigmoid and descending colon. The spleen was

enlarged 40–50% of normal size. The extent of the

resection was related to distribution of the palpable



Figure 68.1: Endoscopy shows chronic inflammatory

changes in the mucosa of the ascending colon (1998).



Figure 68.3: Evidence of Colitis: surface ulceration and

distorted mucosal glands.



148



Diagram 68



lymph nodes. The proximal level was the upper

descending colon, and the distal level was mid

rectum. The anastomosis was performed with a

circular stapler.

Pathology

In the mucosa of the distal sigmoid, there were 5

pale, firm nodules 3–5 mm in size with surrounding

congestion and areas of mucosal hemorrhage. Histologically, the nodules were malignant lymphoma

of large cell B type (Figure 68.2). There were diffuse

as well as follicular areas of lymphoma in the bowel

wall. There was no evidence of lymphoma in any of

11 lymph nodes examined. Macroscopic evidence of

colitis was minimal, but microscopic examination

confirmed the presence of colitis (Figure 68.3).



colitis.2 It is not yet established whether the association of lymphoma and chronic ulcerative colitis

(CUC) is a definite risk factor or coincidence, but

some of the literature does support the former.3

Multifocal involvement is not uncommon in gastrointestinal lymphoma, and the small multiple

nodules were of interest in this patient. Wagonfeld

et al have described a patient with CUC and 22 lymphomas in the colon.4 The severity and duration of

the colitis is variable in patients with coexistent

lymphoma, and the patient described here has been

asymptomatic most of the time. Surgery remains

the mainstay for colorectal lymphoma. If the lymph

nodes are not involved, chemotherapy is usually

omitted.



Follow-Up

(2005)

No chemotherapy was administered, since the localized disease appeared to be adequately removed by

operation. Periodic examinations by gallium and CT

scans have shown no evidence of recurrent lymphoma. The patient remains well more than 10

years since operation except for occasional episodes

of diarrhea, which last for a few days. Surveillance

colonoscopies with random biopsies have continued

and reveal no change in the endoscopic or macroscopic appearances, which is consistent with a

diffuse chronic nonspecific colitis of moderate

degree (Figure 68.2).

Comment

Hopefully this patient’s prognosis is favorable due to

minimal localized lymphoma with no evidence of

recurrence in over 10 years. It is unusual for gastrointestinal lymphoma to be a small lesion at the

time of diagnosis. The obvious and numerous lymph

nodes in the sigmoid and left colon mesentery determined the proximal level of the resection but proved

negative for lymphoma. Chronic colitis coexisting

with malignant lymphoma is rare and was first

reported by Bargen in 1928.1 Baker reported an incidence of 5 lymphomas in 2500 patients with chronic



149



For a full-page image of this figure see the

appendix.



C A S E



69



Lymphoma of the Rectum

Male 63, Years



History

The patient presented with a history of bright blood

on the stool for only a few days. He otherwise felt

well. On rectal examination, a large firm polypoid

lesion was palpable on the left anterior aspect of the

lower third of the rectum 5 cm from the anal verge.

It was moveable on palpation. Colonoscopy revealed

no other pathology in the colon.

Operation

(10.28.99)

The lesion was attached to the wall of the rectum

by a broad pedicle, which could be readily constricted by closure of the diathermy snare. The

polyp was removed with coagulation current (Figure

69.1) as a total specimen. The underlying rectal

muscle remained intact.

Pathology

The polyp measured 25 × 22 × 12 mm. It was pale

pink in color, with a lobulated surface. Histologically, the lesion was covered by colonic mucosa

with some inflammatory reaction. Beneath the

mucosa, there was a nodular mass infiltrating the

submucosa composed of a “monotonous population” of intermediate to large sized lymphoid cells.

Numerous mitotic figures and apoptotic cells were



Figure 69.1: Endoscopic view of the defect after

diathermy snare removal. Blood clot in the proximal part

of the defect.

150



scattered throughout. Immunoperoxidase stains

identified the lesion as a malignant non-Hodgkin’s

lymphoma of diffuse large B cell type (Figure 69.2).

Further Treatment/Follow-Up

(2004)

Investigations revealed no other foci of lymphoma.

The patient was treated with 4 cycles of chemotherapy (cyclophosphamide, doxorubacin, vincristine,

and prednisolone). The site of the lymphoma

healed readily, and regular clinical, endoscopic, and

computerized tomography (CT) examinations have

shown no evidence of recurrent disease 4 years 6

months after removal of the rectal lesion.

Comment

Gastrointestinal lymphoma accounts for 2%–5% of

gastrointestinal tract (GIT) malignancy1 and is the

most common extranodal site.2 Au et al report the

incidence in the GIT as: stomach, 57%: small bowel,

22%; large bowel, 7%; gallbladder, 2%; and multiple sites, 11%.1 The prognosis for control of this

patient’s disease is estimated to be in the vicinity of

70%. The technique of diathermy snare with strong

coagulation current was used in this patient only

because the lesion was in the extraperitoneal

rectum.



Figure 69.2: The lymphoma is apparent beneath the

mucosa.



Diagram 69



151



P A R T



VIII



Anorectal Disease



C A S E



70



An Intrasphincteric Anal Tumor

Female, 59 Years



History

The patient had, for some years, been diagnosed

as suffering from an irritable bowel. For a “few

months,” she had noticed rectal pain on defecation

which had become severe and was aggravated by her

bowel frequency. She was not aware of any swelling,

but there was a sense of blockage during a bowel

action. On examination, there was a smooth,

rounded, nontender mass on the left side of the anal

canal, extending just above the anorectal junction

and bulging into the lumen beneath intact mucosa.

It extended into the ischiorectal fossa and was

readily palpable in the perianal region. Flexible sigmoidoscopy was normal to 30 cm. Transrectal ultrasound (TRUS) revealed a poorly echogenic mass

deep to the mucosa within the sphincter muscle. At

this examination, fine needle aspiration biopsy was

performed. Histologically, this revealed a “smooth

muscle tumor” with no evidence of malignancy.



side of the anal verge. Dissection lateral to the external sphincter, “stretched” over the mass, identified

the inferior hemorrhoidal nerve. Incision of a thin

rim of sphincter muscle revealed the surface of the

tumor that was then dissected free from its location

entirely within the external sphincter muscle

without loss of muscle tissue. The space within the

muscle was closed over a small soft rubber drain.



Operation

(9.2.91)

Under spinal anaesthetic and with the patient in the

prone jack-knife position, a hemicircumferential

incision was made over the palpable mass on the left



Pathology

The egg shaped tumor, with a well defined capsule

(Figure 70.1), measured 50 × 38 × 35 mm, and the

surface was smooth with some shallow lobulation.

On section, it was pale yellow in color and homogeneous in texture without hemorrhage or necrosis.

Histologically, it was uniformly cellular, being composed of spindle cells exhibiting mild atypia with 4

mitoses per 50 high-power fields (Figure 70.2). The

tumor was regarded as being “most likely benign.”

Since the original pathology examination, immunohistochemical staining with C117 has been

performed, confirming that the lesion is a gastrointestinal stromal tumor (GIST).



Figure 70.1: Section shows a circumscribed tumor with a

well defined capsule.



Figure 70.2: Higher power magnification shows a dense

field of plump spindle cells, typical of a GIST.



154



Diagram 70



Follow-Up

(2005)

There has been no recurrence of the tumor since

operation more than 13 years ago. Anal sphincter

function is normal without any degree of incontinence. The patient is examined by panendoscopy

and colonoscopy on a regular basis for a hiatus

hernia, gastric polyps, and symptomatic diverticular

disease.



Comment

Tumors of the anal sphincter are rare and stromal

tumors the more so.1 Topographical tumors, more

common in other sites, have been reported involving the anal canal: spindle cell lipoma;2 neurofibrous;3 and granular cell tumor.4 Other lesions that

may clinically suggest a tumor in this region are

chronic intersphincteric abscess, oleogranuloma,

and endometrioma.5 The cloacogenic carcinoma



155



may present as a subepithelial mass in the anal

canal. Tan et al have managed a similar tumor to the

one described here. Their tumor was initially locally

excised and found to have a mitotic count of 4 per

50 high-power fields.1 Subsequently, abdominoperineal excision of the rectum was performed, which

revealed residual stromal tumor with a mitotic

count of 2 per 10 high-power fields. This emphasizes

the difficulty of assessing the malignant potential of

these tumors. Precise preoperative imaging is essential in planning the surgical approach to a tumor

involving the anal sphincter, and currently either

endoanal ultrasound or magnetic resonance imaging

(MRI) with endorectal coil appear to be equally accurate.6 Local excision to provide a “total biopsy”

would appear to be the first step in management. If

there is then doubt concerning malignancy, the area

can be readily monitored by the available imaging

techniques.