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MUCOSA ASSOCIATED LYMPHOID TUMOUR (MALT) OF THE ILEUM

Shilpa A Rao*, Prakash P Shilotri**, Pinky Thapar***
*Lecturer; **Prof.; ***Resident; Topiwala National Medical College and BYL Nair Hospital, Mumbai.

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A 64 year old male presented with episodic periumbilical abdominal pain of 1 year duration associated with vomiting and a lump in the said area. CT scan revealed a large mass involving the terminal ileum and infiltrating the anterior rectus sheath and abdominal wall. FNAC showed this to be a large cell lymphoma. Histopathology confirmed evidence of this being a lymphoma arising from the terminal ileum.

INTRODUCTION

Primary small bowel mucosa associated lymphoma tissue (MALT) constitute the least percentage of gastrointestinal neoplasm i.e. 1-4% of all gastrointestinal malignancies and 9% of all lymphomas. It commonly affects males more than 40 years of age and is mostly seen in the Middle East. These tumours are usually seen in the stomach (55%) followed by small intestine (30%). The commonest symptoms include abdominal pain, vomiting, weight loss and altered bowel habits. Histopathologically, there are two varieties - the Western (localised) variety and the Mediterranean immunoproliferative one. Surgery is curative in 65% cases for stage I and II. Palliative surgery without chemotherapy has 5 year survival rate of 0 to 17%. Palliative surgery with chemotherapy has a five year survival rate of 32 to 35%.

CASE REPORT

A 64 year old male presented to us with episodic periumbilical pain of one year’s duration aggravated by food and relieved by vomiting. There was no associated fever, jaundice or altered bowel habits. He also noticed a lump in the periumbilical region which was nontender and freely mobile initially. On examination there was no evidence of weight loss, fever, lymphadenopathy or oedema.

On per abdominal examination there was an irregular lump 8 cm x 5 cm, intraabdominal, non-tender, attached to left rectus muscle with restricted mobility. There was no hepatomegaly, splenomegaly or free fluid in the abdomen or any other lump palpable. Blood investigations were within normal range except for ESR (105 mm at the end of one hour). X-ray chest did not show any abnormality. CT scan showed a large mass arising from the terminal ileum and infiltrating the anterior abdominal wall and CT guided FNAC revealed it to be a large cell lymphoma. There was no hepatomegaly or splenomegaly on CT scan or intra operatively.

The patient underwent an exploratory laparotomy and a large 10 cm x 6 cm x 5 cm tumour arising one and a half feet from the ileocaecal junction involving the mesentery and fixed to the rectus muscle with partial volvulus of the segment was seen. Draining lymph nodes seemed enlarged and involved. Liver and spleen were normal. There was no evidence of free fluid or other lymphadenopathy. Wide resection of the segment with draining lymph nodes was done. The patient had an uneventful recovery. Post operatively he received 6 cycles of CHOP based chemotherapy and has been in remission since then.

Histopathology confirmed this to be microproliferative nodular growth arising from ileum extending from mucosa, submucosa upto serosa with pleomorphic cells arranged in sheets. The resected margins were clear. However omentum attached showed evidence of local spread. Draining lymph nodes were positive.

PRIMARY SMALL BOWEL LYMPHOMAS

These constitute the least of all gastrointestinal neoplasms and vary between 1 and 4% of all GI malignancies (Ellis 1986) and 9% of all lymphomas; although 43-70% of lymphomas affect the GIT secondarily (Rosenberg et al). They usually affect males more than 40 years of age, the male : female ratio being 2:1. They are mostly encountered in the Middle East. The common sites involved are Stomach - 55%, Small intestine - 30% (duodenum - 2.3%, jejunum - 41.3%, ileum - 56.4%), Large intestine - 1% and others - 4%.1 predisposing factors include coeliac disease (T cell type) (Swinson 1983), immunoproliferative diseases (Smith 1987) like ulcerative colitis / AIDS (B cell) (WHO meeting report 1976). Clinical symptoms/signs : These patients usually present with tumour mass or intestinal obstruction, sometimes intussusception or weight loss etc. [1]

The incidence of various presentations is as follows
Abdominal pain	70%
Weight loss	50%
Nausea and vomiting	30%
Bleeding	20%
Fever	08%
Altered bowel habits	04%

Spread usually occurs to other segments of the intestinal tract, liver, spleen etc. Extra abdominal spread is 4.7%. However there is usually no correlation between initial stage of lymphoma and extra abdominal spread.

Histopathology : Usually 2 types are recognised - Western localised lymphoma and diffuse Mediterranean immunoproliferative variety with a poor prognosis.

Our case fits into Dawson’s Criteria[2]updated in 1992 for diagnosing primary GUT lymphoma which suggests that there should be:

1. No evident superficial lymphadenopathy
2. Normal X-ray chest and CT scan thorax with no thoracic lymphadenopathy
3. Normal bone marrow
4. At laparotomy bowel lesion predominates with only draining lymph nodes if at all affected.
5. Liver, spleen normal on CT scan abdomen and at laparotomy

Staging (Ann Arbor) for primary GIT lymphoma[3]:

I E - confined to the gastro intestinal tract
II E - involvement of single lymph node
III E - tumour of GIT + spread above diaphragm
IV E - tumour of GIT + liver/marrow involvement.

65% of stage I and II cases are curative with surgery. Stage III onwards, surgery is mostly palliative and for symptomatic relief. Purely palliative procedures have a 5 year chemotherapy and to 27 to 30% with adjunct radiotherapy.

Post Operative

Chemotherapy given (every 21 days) - Cyclophosphamide 750 mg/sq m (1 gm for 1.6 sq m), Etoposide 75 mg, Vincristin 1, 4 mg/sq m, Prednisolone 20 mg (day 1 to 5). Patient received 6 cycles at 21 day intervals and has been symptom free. USG at 6 months had not revealed any evidence of this disease.

Prognosis : The overall survival for Lymphomas is better than for carcinoma at the same site. The most consistent prognostic factor is the stage of the disease. West Midlands Cancer Registry Data rates show that 61.3% were curatively resected. Of the remainder 12.1% had distant metastases and 4.6% died before diagnosis. Age adjusted survival for all cases was 22%. Curative treatment, i.e. surgery and radiotherapy, had a 32% 5 year survival. Palliative resection had a 0% 5 year survival which improved to 11.1% with radiotherapy. Chemotherapy is capable of producing long term benefit in advanced disease - 20-46% 5 year survival rates[4] There is a marked difference in survival between males and females, their 5 year survival rates being 35% and 22% respectively. [5]

REFERENCES

1. Blackledge G, Best JK, Crowther D. A study of gastrointestinal lymphoma. Cli Onco 1979; 5 : 209-19.
2. Dawson IMP, Cornes TS, Morson BC. Primary malignant lymphoid tumours of gastrointestinal tract. Ann Surg 1961; 191 : 593-8.
3. Carbone PP, Kaplan HS, Mussholt K, et al. Report of the committee of Hodgkin’s disease staging classification. Cancer Res 1971; 31 : 1860-1.
4. Gobbi PG, Didnigi P, Barbieri F, et al. The role of surgery in multimodal treatment of primary non-Hodgkin’s lymphoma - Report of 76 cases. Review of Ulcerative cancer 1990; 65 : 2528-36.
5. Fielding JWL. Role of surgery in non Hodgkin’s gastrointestinal lymphomas. RA in Surgery 16 : 143-61.

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