Primary lymphoma of the appendix. Case report and review of the literature

Surgical oncology 1994; 3: 243-248

SHORT REPORT

Primary lymphoma of the appendix. Case report and review of the literature M. D. PASQUALE*, M. SHABAHANG*, P. BIl-TERMANt, E. E. LACK? AND S. R. T. EVANS* *Department

Malignant intestinal

of Surgery, and iDepartment

lymphomas

of Pathology, Georgetown University Hospita4 Washington, DC, USA

comprise

l-4%

of the

malignant

tract, but appendiceal lymphomas are exceedingly

case of a well differentiated

of the

gastro-

rare. Herein is presented a

lymphocytic lymphoma of the appendix found incidentally

at hernia repair. Forty-six cases of appendiceal

lymphoma

1696 with a mean patient age of 25.7 years. Thirty-one lower quadrant

neoplasms

pain, and a mass was an incidental

have been, reported

patients presented

since

with right

finding in five. Of the 46 cases,

follow-up was possible in 26. There were four deaths within 30 days of the operation and five deaths within 1 year. Although extensive follow-up is limited, there have been only two reported deaths secondary to primary appendiceal lymphoma since 1945 and these two cases are discussed in detail. Based on this extensive review, appropriate recommendations

are made. Surgical Oncology 1994; 3: 243-248.

Keywords: appendix, gastrointestinal

tract, lymphoma.

INTRODUCTION

approximately

While malignant

values (electrolytes and blood counts), chest roentgenogram, and electrocardiogram were normal. The patient’s medical history was remarkable only for

lymphomas

comprise

l-4%

of the

malignant neoplasms of the gastro-intestinal tract, the gastro-intestinal tract is the most common site

lesions

bowel

[l-3].

presenting Involvement

At the operation,

lymphoma literature.

CASE

along

with

of

primary

a review

the

hernia

through

was

the hernia

portion

of the appendix.

performed

world

via

the

An appendectomy

hernia

incision,

the

ligated and excised, and the transversalis reinforced.

The patient is a 77-year-old presented with a five month inguinal hernia. The hernia enlarged and on examination Correspondence:

indirect

he was

sac, a nodular mass was noted and was elevated into the hernia incision. This mass was in the distal was

sac was fascia was

Microscopic pathology of the appendix disclosed a well differentiated lymphocytic lymphoma. The

REPORT

Surgery, 4PHC,

for which

released. On manual palpation

appendiceal of

a large

laboratory

noted. When the hernia sac was opened, approximately 500 ml of sero-sanguinous fluid was

in the stomach or small of the colon is unusual

and appendiceal lesions are exceedingly rare, being reported in only 0.015% of all gastro-intestinal lymphomas [I, 2, 41. We report a case

Pre-operative

mild congestive heart failure, being treated with digoxin.

of primary extranodal lymphoma [l, 21. When occurring in this location, lymphomas are usually single

4 x 3 cm.

neoplasm

black male who history of a right had progressively was

noted

showed

exaggerated

growth

centres

(pseudofollicles) and infiltrated peri-appendiceal fat and fibrous connective tissue of the hernia sac (Figs l-3). Cytological examination of the peritoneal fluid was negative. Post-operative work-up consisted of a CT scan of the abdomen and thorax, a bone marrow

to be

Stephen R. T. Evans, MD, Department of Georgetown University Hospital, 3800

biopsy, upper gastrointestinal enema. These were normal.

Reservoir Road, N.W. Washington. DC 20007, USA.

243

series, and a barium No further treatment

M. D. Pasquale et al.

244

Figure 1. Malignant lymphoid infiltrate in the appendiceal crypts and expanded lamina propria characterized by a monotonous population of small round lymphocytes with clumped chromatin (haematoxylin and eosin).

Figure 2. Well differentiated lymphocytic lymphoma showing plasmacytoid features, brisk mitotic rate and pale, ill-defined aggregates of large cells (arrow), referred to as exaggerated growth centres (haematoxylin and eosin).

was

recommended.

post-operatively

I he patient

is now

5 months

and doing well.

1) there

were

19 females,

the sex was not reported. years

with

a mean

23 males,

and in 4 cases

Age ranged

from

age of 25.7 years

4 to 77

and a median

age of 25.5 years. Thirty-one

DISCUSSION

quadrant There

have been 46 cases of appendiceal

reported

since 1898. In reviewing

lymphoma

these cases (Table

common right

patients pain

and

symptom.

lower

quadrant

presented this Other

was,

with by

symptoms

mass,

diarrhoea,

right

far,

lower

the

noted

most were

nausea

a

and

Primary appendiceal lymphoma

245

Figure 3. Lymphoma infiltrates peri-

appendiceal fat and fibrous connective tissue (haematoxylin and eosin).

vomiting,

weight loss and diffuse abdominal

mass was found incidentally

pain. A

in five patients, and in

Involvement of the gastro-intestinal tract by malignant lymphoma has been recognized since the

four, the presenting complaints were not available.

earliest

Appendectomy while cecectomy

endothelial system [3]. It is, in fact, the most common site of extranodal involvement, followed by

and

right

dectomy performed

alone was performed in 31 cases, was carried out in three cases

hemicolectomy with

in four

bilateral

cases. Appen-

oophorectomy

in one patient who had extension

was of dis-

ease to the ovaries. One patient did not undergo operative intervention and in seven the operative treatment

was unknown.

Four patients were treated

with post-operative chemotherapy and one patient received post-operative radiation therapy. Of the 37 cases reported through to 1968, in which the old lymphoma nomenclature was used, there were 25 cases of lymphoblastic sarcoma, nine cases of giant follicular lymphoblastoma, and in three cases, classification was unknown. Since 1968, there have been three cases of well differentiated lymphocytic lymphoma, three of diffuse large cell lymphoma and three of Burkitt’s lymphoma. In one case, classification could not be obtained. Follow-up was available in 28 of the 46 cases. There were four deaths within 30 days of operation and five deaths within 1 year. Five patients, including ours, were reported to be alive within 1 year of operation. There was one patient alive within 5 years of operation, one at 9 years post-operatively and two at over 10 years post-operatively.

description

of the disease in the reticulo-

skin, bone, and the upper gastro-intestinal tract [5]. Despite this, primary appendiceal involvement is exceedingly

rare, being in the range of 0.015% of all

gastro-intestinal lymphomas [ 1, 41. The earliest report of a gastro-intestinal lymphoma was in 1883 by Debrunner, who described a case involving the large bowel [4]. Warren, in 1898, reported the first case of lymphoma reported

a series

of the appendix and in 1945 Knox of

23 patients

with

primary

appendiceal involvement [7, 81. Currently, there have been a total of 46 reported cases of appenditeal lymphoma. Primary involvement of the gastro-intestinal tract occurs in approximately 5% of all cases of lymphoma.

The most common

site is the stomach,

followed closely by the small bowel. The large bowel is affected less frequently [I]. Lewin, in a study of 117 patients with primary gastro-intestinal lymphoma, reported gastric involvement in 48 patients, small bowel involvement in 37 patients, ileocaecal involvement in 13 patients, large bowel involvement in 11 patients, and appendiceal involvement in two patients. In six patients there were multiple sites involved [2].

M. D. Pasquale et al.

246 Table 1. Appendiceal

lymphoma

Author

Year

Age/sex

Pathology

Treatment

Follow-up

Warren [7] Davis [I l] Paterson [I 21 Bernays [I 31 DeJong [I 41 Carwardine [I 51 Wilhelm [I 61 Powers [I 71 Wright [18] White [19] Wohl [20] Rohdenburg [21] Goldstein 1221 Lehman [23] Friend [24] Capecchi 1251 Stout [26]

1898 1900 1903 1905 1907 1907 1919 1911 1911 1913 1916 1919 1921 1925 1926 1927 1925

Lymphoblastic sarcoma Lymphoblastic sarcoma Lymphoblastic sarcoma Lymphoblastic sarcoma Lymphoblastic sarcoma Lymphoblastic sarcoma Lymphoma Lymphoblastic sarcoma Lymphoblastic sarcoma Lymphoblastic sarcoma Round cell sarcoma Lymphoblastic sarcoma Lymphoblastic sarcoma -

lleocaecectomy Appendectomy Appendectomy Appendectomy Appendectomy Appendectomy -

-

Appendectomy Right hemicolectomy Appendectomy Appendectomy Caecectomy Appendectomy -

10 weeks - dead 3 years - alive 4 years - alive 10 months - dead 10 months - alive 10 months - alive -

Appendectomy Appendectomy -

death

Post-operative -

death

1931 1932

Bizard [29] Ruggieri [30] Knox [8] Morehead [31 I

1938 1938 1945 1945

McSwain [32]

1945

Galloway [331 Jason [34] Clarke [35] Henley [36] Glick [3] Loehr [9]

1949 1949 1950 1954 1966 1968

Lewin [2]

1978

Sin [37]

1979

Saitou [38] Murakuni [39] Ghani 1401 Mori [4]

1981 1982 1984 1985

Stewart [l] Chawla [5]

1986 1989

33/F

Lymphoblastic sarcoma Lymphoblastic sarcoma Giant follicular lymphoblastoma Giant follicular lymphoblastoma Giant follicular lymphoblastoma Lymphosarcoma Lymphosarcoma Lymphoblastic sarcoma Giant follicular lymphoblastoma Lymphoblastic sarcoma Giant follicular lymphoblastoma Giant follicular lymphoblastoma Giant follicular lymphoblastoma Giant follicular lymphoblastoma Lymphosarcoma Giant follicular lymphoblastoma Giant follicular lymphoblastoma Lymphosarcoma Diffuse large cell lymphoma* Diffuse large cell lymphoma* Diffuse large cell lymphoma* Diffuse large cell lymphoma* Well differentiated lymphocytic lymphoma Burkitt’s Lymphoma Burkitt’s Lymphoma Lymphoma Diffuse large cell lymphoma* Burkitt’s lymphoma Well differentiated lymphocytic - lymphoma Diffuse infiltrating lymphoma Diffuse, mixed lymphoma

Post-operative -

Evans [27] Ullman [28]

--/M 51 /M 39/M 29/F --/M 45/F 17/M 12/F 17/M 25/F 35/M 4/M 25/F 20/F 9/F 8/M 8/F 9/F 55/M -

Pasquale

1993

77/M

27/M 39/M 4/M 33/F 12/M 26/M 39/F 47/F -/34/F 32/M 38/M 4/M -IF -/15/8/M 10/M 20/F -/22/M 70/F

34/F

*Described as lymphosarcoma Chemo = Chemotherapy.

Well differentiated lymphoma

by old nomenclature.

lymphocytic

Appendectomy Appendectomy Appendectomy Appendectomy Appendectomy Appendectomy Appendectomy Appendectomy Appendectomy Appendectomy No resection Appendectomy Right hemicolectomy Appendectomy Appendectomy -

5 months - alive Post-operative death 9 months - dead -

3 years 3.5 years 2 years 4 years -

alive

alive

alive alive

1 month - dead 3 years - alive 12 years - alive 9 years - alive 28 years - alive -

AppendectomylChemo AppendectomylChemo Right hemicolectomy Right hemicolectomy Appendectomy Appendectomy

3 years - alive 3 years - alive 7 months - dead -

CaecectomylChemo AppendectomylChemo Bilateral oophorectomy Appendectomy

18 months - alive 1 year - alive

3 years -

5 months -

alive

alive

Primary appendiceal

Gastro-intestinal the

lymphoid

extend

lymphomas

tissues

laterally

along

They may protrude

lamina

propria

the submucosal

plane

into the lumen forming

poid mass, or diffusely [2, 91. Affected

tend to arise from

of the

infiltrate

individuals

ponderance

[l,

pathology

disclosed diffuse large

[9].

reported to be positive. Four months postoperatively, the patient was re-explored for multiple

a poly-

male pre-

lymphoma,

colon. Microscopic cell lymphoma

into the bowel wall

21. Appendiceal

247

and

are usually in the fourth

to seventh decades of life, with a slightly

lymphoma

how-

of the appendix.

metastases.

intra-abdominal debulking, died

the patient

3 months

diagnosis

Lymph nodes were

later.

of primary

following

received Again,

tumour

chemotherapy. in this

appendiceal

setting

lymphoma

ever, affects males and females almost equally and

also be questioned

mean

have been colonic. Based on this extensive

and

median

approximately

ages

of those

affected

25 years. Typical symptoms

was

were the

presence of right lower quadrant pain or a mass in the right lower quadrant. Nonspecific complaints included

nausea and vomiting,

malaise, diarrhoea,

and weight loss. Prognosis, like other gastro-intestinal is related to tumour stage and histology

lymphomas, [I, 21. This

would argue for a full metastatic work-up in these patients. Stage of disease is then used to determine whether or not adjuvant therapy is required. Reported 5 year survivals

for patients with gastro-

intestinal lymphomas range from 30 to 50% [I, 2, 5, 91. Reports of appendiceal lymphoma since 1950 would

suggest

that

5 year

survival

is markedly

improved. The extent of resection remains controversial

and

it appears that gross and microscopic involvement of the tumour is the determining factor. Although follow-up is limited, there have been only two reported deaths secondary to primary appendiceal lymphoma

at autopsy

[35].

Post-mortem

examination

showed disseminated lymphoma to all the intraabdominal organs and obstruction of the common hepatic duct. The bulk of the neoplasm was in the appendix. However, a definitive diagnosis of primary appendiceal

of

and in fact, the primary

recommendations

primary

the could

appendiceal

may

review,

for the management

lymphoma

include

the

following: 1. If the

without

lymphoma extension

then appendectomy

is confined

to the appendix

to the caecum or other organs, is the recommended

treatment

of choice without adjuvant therapy. 2. Right hemicolectomy should be performed

if

there is extension of tumour beyond the appendix on to the caecum and should include resection with free margins if it extends to any other contiguous organs or to the abdominal sidewall. 3. Full staging work-up should be performed, as in all lymphomas, with adjuvant therapy being based on the stage of the disease. Although follow-up in the prior published cases is limited, there appears to be

no

advantage

isolated appendiceal

to

extended

resections

with

lymphoma.

since 1945. The first report in 1950 by

Clark is a patient who died secondary to a primary appendiceal lympho-sarcoma, with diagnosis being made

our current

He

lymphoma

might

not

be

correct

because of multi-organ involvement. The patient underwent no resection because of the extent of the disease and died one month after exploration. The second death due to appendiceal lymphoma was reported by Saitou in 1981 and was a patient who underwent exploration for a 24 x 8 x 10 cm mass in the right lower quadrant. The appendix was not identified at the time of operation [38]. An extended right hemicolectomy was performed, as it appeared that the tumour clearly involved the appendix, the base of the caecum and a segment of the right

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