Intussusception due to Kaposi’s Sarcoma

K A Khorshid*, K Erzingatsian*, DA A K Watters* and A C Bayley*

2 patients with aggressive atypical Kaposi’s sarcoma presenting with intussusception are described and the implications of this disease in immunosuppressed patients discussed.

 

Kaposi’s sarcoma is regarded as a rare tumour of the small bowel¹. In the past 40 years there have been occasional case reports of Kaposi’s sarcoma presenting with haematemesis², intestinal perforation³, intestinal obstruction4, and diarrhea with protein-losing enteropathy5. Involvement of the stomach has been described radiologically6 and endoscopically7. 2 patients with intussusceptions have been reported, one small bowel8 and one large bowel9. Clinical studies of Kaposi’s sarcoma from East Africa have suggested that visceral involvement may occur frequently despite lack of abdominal symptoms and signs10 11, suggesting that spread of Kaposi’s sarcoma to the bowel is a late event or occurs in fulminating disease.

The endemic form of Kaposi’s sarcoma which is seen in Africa presents with localized lesions usually on the lower limbs. Aggressive atypical forms of the disease have been increasingly encountered since 1983 in Zambia12, Uganada13 and Zimbabwe14 and most of these patients are seropositive for antibody to the human immunodeficiency virus (HIV)15. Kaposi’s sarcoma of the gut is one such atypical form and may present with intestinal obstruction. We report 2 patients with Kaposi’s sarcoma of the small bowel presenting with intussusception.

 

Case Reports

Case 1. A 35-year-old-male civil servant, married with five children, was admitted with a 9-month history of lower limb swelling and the appearance of small nodules on the trunk and limbs.

Examination revealed a wasted, dysphonic man with on-tender abdominal distension, ascites, jaundice, symmetrical generalized lymphadenopathy and darkly pigmented plaques 2-3 cm in size on the limbs, trunk, head and neck. Oral examination revealed similar purple lumps on the palate. His haemoglobin was 7.4 g/dl, urea 12.5 mmol/l and liver function tests were indicative of obstructive jaundice. 3 days after admission the patient developed incomplete intestinal obstruction which did not resolve with conservative treatment. At laparotomy ascites was found and the small bowel appeared hypertrophied, oedematous and moderately distended. There were multiple subserosal purple lumps 1-2 cm in size throughout its length and the mesentery was markedly thickened with extensive lymphadenopathy. Near the caecum an irreducible ileo-ileal intussusception was found. This portion of the gut, including the gangrenous intussusceptum, was resected. A mesenteric lymph node and a nodule from the wall of the gut proximal to the resection were removed for histological examination. The larger bowel, liver and biliary system were macroscopically normal apart from the common bile duct, which was about 15 mm in diameter. In view of the poor condition of the patient no further diagnostic or operative procedure was undertaken.

Postoperatively the patient’s condition remained poor. He died on the 11th day. Histology of the resected specimen and nodes confirmed Kaposi’s sarcoma and the HIV antibody test was positive. Permission for autopsy was refused.

Case 2. A 16-year-old girl was admitted with a one-year history of recurrent colicky abdominal pain and bloody diarrhea. She was not pregnant and had no children. On examination there was generalized lymphadenopathy and wasting. She had obvious intestinal obstruction which was clinically and radiologically incomplete. She was in hypovolaemic shock but after resuscitation she underwent laparotomy.

Two laparotomies were performed within a period of 5 days. At the first operation multiple intussusceptions sue to sessile small bowel tumours were reduced and a segment of bowel with one polyp was excised. Further resections of the multiple tumours were not attempted at this stage because of the poor condition of the patient. To prevent recurrence of the intussusception she underwent further laparotomy 4 days later, and 4 separate areas of bowel containing all the palpable tumours were excised (Fig 1) as these had already begun to re-intussuscept. Extensive lymphadenopathy was noted but lymph node was not performed, and the large bowel was palpably free of tumour. Histology of the small bowel revealed Kaposi’s sarcoma (Fig 2). Examination of the oral cavity at this stage was normal.


Postoperatively her haemoglobin level remained at 3-4 g/dl despite repeated transfusions of blood. Episodes of epistaxis and later bleeding from the mouth occurred and 2 weeks following surgery she developed Kaposi plaques on the palate and tonsils. Subsequently she developed retinal haemorrhages, haemoptysis and blood-stained ascites. Her bloddy diarrhoea recurred and she required aspiration of a blood-stained pleural effusion on 3 occasions. The persistently low platelet count which developed a month postoperatively was the likely explanation for her bleeding tendency and precluded the use of standard chemotherapy; nevertheless, she received vincristine and actinomycin D on one occasion. In attempts to control her bleeding diathesis transfusions of fresh blood, platelet concentrates, vitamin K1 and haematinics were given without effect. Her condition continued to deteriorate and she died 2 months after admission. Her HIV antibody status was unknown, and permission for autopsy was refused.

 

Discussion

We report 2 cases in which small bowel intussusceptions developed in patients with aggressive, atypical Kaposi’s sarcoma. In the first patient a clinical diagnosis of Kaposi’s sarcoma had been made pre-operatively but in the second the diagnosis was made on histology of the resected specimen. Both patients died of progressive disease despite surgical correction of the small bowel obstruction.  

We suggest that on chronically ill, wasted patients who present with intestinal obstruction, aggressive atypical Kaposi’s sarcoma should be considered as part of the differential diagnosis. Careful general examination of the patient for lymphadenopathy, skin, palatal and tonsillar plaques is important if the diagnosis is not to be missed. Upper gastro-intestinal endoscopy of patients with endemic and with aggressive atypical Kaposi’s sarcoma has shown that oral lesions reliably predict the presence of further nodules in the oesophagus, stomach or duodenum16.

We can offer no fresh insight on treatment. Possibly the stress of surgery in these immuncompromised patients was the final insult which prompted rapid progression of their disease.

Acknowledgments. The authors wish to thank Dr Patil, Department of Pathology for preparation of slides and Dr M A Ansary, Medical Illustration Department, University Teaching Hospital, Lusaka for providing the figures.

 

References

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16 DIN N A. BAYLEY A C. Unpublished.
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