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CASE REPORTS
RETROCAVAL URETER WITH HYDRONEPHROSIS LYMPHOID TISSUE (MALT) LYMPHOMA
MOHAN J ALGOTAR*, MATHEW JOHN**, WAQAR ANSARI***
A 22-year-old male presented with episodic dull pain and lump in the infraumbilical region. Barium meal follow-through showed two large masses of the small intestine. CT scan showed concentric thickening of two separate segments of ileum and jejunum. During hospitalisation, the patient experienced sudden onset of severe pain with abdominal distension, following which the Xray showed free gas under the diaphragm. An emergency exploratory laparotomy with resection anastomosis of the perforated ileal mass along with that of the jejunal mass was done. Histopathology confirmed perforation in a mucosa-associated lymphoid tissue (MALT) lymphoma.
INTRODUCTION
Primary lymphomas of the small intestine are rare accounting for less than 2% of all gastrointestinal tract malignancies and 10-20% of small intestinal malignancies.1 They constitute 9% of all lymphomas. Three types of MALT lymphomas are seen, namely Western, Mediterranean and Childhood variety. Mediterranean lymphoma is the commonest type of intestinal lymphoma seen in Middle East and North Africa in the low-socioeconomic classes, mainly in the younger age groups. MALT lymphomas arise mainly in the stomach (66%), followed by small intestine (29%) and large intestine (6%).
Common symptoms include abdominal pain, vomiting, weight loss and altered bowel habits. Alleys of treatment include surgery, chemotherapy alone or in combination depending upon the grade of the tumour, clearance of margins etc.
CASE REPORT
A 22-year-old male presented with a two-month history of episodic dull pain and lump in the infraumbilical region. There was no history of fever, weight loss or altered bowel habits. The patient had received a one-month course of anti-tuberculous regimen from an outside institution, but without any relief (Tuberculosis is one of the differential diagnosis for a MALT lymphoma).2 On examination, there was a 10 x 7 cm, irregular, firm, nontender, intraperitoneal lump with restricted mobility, with no organomegaly or free fluid in the abdomen. On investigation, the patient was found to be anaemic with a haemoglobin of 7 gm% (20% of these patients have anaemia).2 Rest of the laboratory investigations were found to be normal. X-ray chest revealed normal findings. Ultrasound showed a long segment bowel mass with thickened wall in the pelvis most probably arising from the small intestine. Barium meal follow-through showed a large mass involving distal jejunum and proximal ileum with ulceration, shouldering and irregularity. Another similar mass was noted in the proximal jejunum. CT abdomen showed thickening of the ileal loop up to 4 cm with another thickening in the jejunum of about 2.5 cm. There was no evidence of ascites, lymphadenopathy with normal liver and spleen.
During hospitalisation, while building up the haemoglobin, he developed sudden onset of sharp and severe pain in the lump with abdominal distension. An urgent chest X-ray showed free gas under the diaphragm. Hence an emergency exploratory laparotomy was performed revealing a large mass of 15 x 10 x 10 cm of thickened proximal ileum, which was adherent to the sigmoid colon with an area of perforation of about 1 cm in size. Another mass of 10 x 5 x 5 cm was seen in the proximal jejunum. The liver and spleen were found to be normal with no mesenteric or para-aortic lymphadenopathy or ascites. Resection anastomoses of the two masses were done with an adequate margin of resection.
Histopathology revealed low-grade MALT lymphoma of the small intestine with resected margins free of the tumour. The tumour cells were found arranged diffusely in sheets and nests at places divided by fibrocollagenous stroma infiltrating all layers of the intestine. The tumour cell population was polymorphous consisting of lymphocytes, centriocyte-like cells, histiocytes and occasional centroblasts. Localised mesenteric lymph nodes were not involved by the tumour.
The patient had an uneventful postoperative course and is asymptomatic since the past two months.
DISCUSSION
Primary lymphomas of the small intestine account for less than 2% of all gastrointestinal malignancies and 9% of all lymphomas.1 Three types of primary small intestinal lymphomas are known, namely Western, Mediterranean and Childhood variety. Western-type lymphoma is seen predominantly in males presenting with abdominal pain and mass. 20% of these patients have anaemia.2 Most of these tumours are diagnosed intraoperatively. Perforation can occur without surrounding desmoblastic reaction and therefore primary closure is not therapeutic. Resection of the affected segment with its mesentery is the only acceptable procedure. Mediterranean lymphoma is the most common type of intestinal lymphoma seen in Middle East and North Africa in the low-socioeconomic classes, mainly in the younger age groups.
Predisposing factors include coeliac disease, immunoproliferative disorders. The common sites involved are stomach (66%), followed by small intestine (29%) and large intestine (6%). Symptoms manifested include abdominal pain (83%), weight loss, palpable mass, nausea, vomiting, bleeding, fever, altered bowel habits. 40% of these patients are seen as surgical emergencies, namely obstruction (23%), perforation (6%), bleeding (11%).
Dawson’s criteria3 for small intestinal lymphomas to be categorised as primary include:
No evidence of mediastinal or peripheral lymphadenopathy.
Normal white blood count and differential blood counts.
No splenic or hepatic involvement.
At laparotomy, bowel lesion predominates with only draining lymph nodes if at all affected.
Ann Arbor staging for GI lymphoma (Modified by Musshoff K.)4 are:
Stage I E : Confined to wall of the bowel.
Stage II E(1) : Regional lymph nodes contiguous to primary site.
Stage II E(2) : Regional lymph nodes not contiguous to primary site.
Stage III E : Lymph nodes on both sides of diaphragm, spleen (III S), or both (III ES).
Stage IV E : Bone marrow or other nonhaematolymphoid organ. 65% of stage I and stage II cases are curative following surgery. From stage III onwards chemotherapy and radiotherapy are required. In patients with evidence of residual lymphoma after surgery, chemotherapy is recommended.2 Chemotherapy is also recommended for all highgrade tumours even at stage I E.5 Multi-modality treatment has been found to be superior to surgery or chemotherapy alone.6
Prognostic features include stage of the lymphoma, presence or absence of perforation, tumour size, depth of tumour invasion, involvement of regional lymph nodes, use of multi-modality therapy.2,6 Average five-year survival rates are 63-95% for I E, 38-75% for II E, 15-53% for III E, and 8-31% for IV E.5
REFERENCES
1. Coit DG. Cancer of the small intestine. In : DeVita Jr., et al. (eds), Cancer: Principles and Practice of Oncology, 1993;(4th edition) I : 915-928.
2. Contreary K, Nance FC, Becker WF. Primary lymphoma of gastrointestinal tract. Ann Surg 1980; 191 : 593-598.
3. Dawson IMP, Cornes JS, Morson BC. Primary malignant lymphoid tumours of the intestinal tract: Report of 37 cases with a study of factors influencing Prognosis. Br J Surg 1961; 49 : 80-89.
4. Ann Arbor staging for GI lymphoma, modified by Musshoff K, Strahlentherapie, 1977; 153 : 219-21.
5. Rosenfelt F, Rosenberg SA. Diffuse histiocytic lymphoma presenting with gastrointestinal tract lesion - The Stanford Experience. Cancer 1980; 45 : 2188-2193.
6. Amer MH, El-Akkad D. Gastrointestinal lymphoma in adults - Clinical features and management of 300 cases. Gastroenterology 1994; 106 : 846-858.
DRUGS FOR ALCOHOL DEPENDENCE
‘Topiramate is more effective than placebo at reducing drinking and promoting abstinence in
alcohol-dependent individuals’
Although current psychosocial treatments for alcohol abuse and dependence have been useful in
reducing alcohol use and alcohol-related morbidity and mortality, they are not always completely
successful for all patients. Bankole Johnson and colleagues did a randomised controlled trial and
found that up to 300 mg per day of topiramate, a sulphamate fructopyranose derivative, was more
efficacious than placebo as an adjunct to standardised medication compliance management in
treatment of alcohol dependence. In a Commentary, Robert Swift notes that Johnson and colleagues’
results are important because they suggest that “different pharmacotherapies could be targeted at
different stages of alcoholism treatment - the initiation of abstinence, the maintenance of early
abstinence, or the maintenance of prolonged abstinence.
BMJ, 2003; 1666, 1677.
