**3. Discussion**

Current knowledge of MALT lymphoma is largely based upon studies in adults. MALT lymphoma is rare in children; the available evidence consists mostly of isolated case reports, except for one series of ten cases (Corr et al., 1997), and another including a total of 48 cases (children and young adults) (Taddesse-Heath et al., 1997) and a pediatric NHL trial recruiting children and adolescents from Germany, Austria and Switzerland (Kaatsch et al., 2004). MALT often develops within the context of a pre-existing inflammatory response due to infection or to autoimmune disorder. Many studies show the relationship between *H. pylori* infection and gastric MALT lymphoma (Isaacson & Whright, 1984; Kurugoglu et al., 2002); some authors have reported a regression of MALT lymphoma in parotid gland (Alkan et al., 1996), lip gland (Berrebi et al., 1998), small intestine (Fischbach et al., 1997) and rectum (Matsudo et al., 1997) following *H. pylori* eradication. Other risk factors for MALT lymphoma include autoimmune diseases like Hashimoto thyroiditis or Sjogren syndrome, and Borrelia burgdorferi for skin lymphoma. A further prerequisite for the development of MALT lymphoma in children may be the presence of HIV infection (Teruya-Feldestein et al., 1995; Mo et al., 2004). In some patients no risk factors can be identified. The most common sites are the stomach and salivary glands. Others sites are: ocular adnexa, the lungs, thyroid and the skin (Zucca et al., 2000). Some retrospective analyses of histopathological results of appendectomy specimens performed for acute appendicitis in a large sample of patients, including children, report a prevalence of appendiceal malignant tumors ranging from 0.4 (Tchana et al., 2006) to 1.5% (Ravi et al., 2006). Among the malignant tumors, carcinoids have the highest incidence (Tchana et al., 2006; Ravi et al., 2006) and 70–90% of these tumors are discovered incidentally because they are usually restricted to the distal appendix (Akerstrom, 1989; Aranha & Greenle, 1980). From a review of the literature we found only one case of appendix lymphoma in paediatric age presenting with intussusception symptoms (Karabulut et al., 2005). Our report probably represents the first case of MALT lymphoma of the appendix found accidentally in a child during an appendectomy. MALT lymphomas manifest with aspecific symptoms. In our case, the clinical presentation was characterized by recurrent abdominal pain, and the only element of suspicion was the enlargement of the distal portion of the appendix. The subsequent evolution to a mild form of IBD could be considered as an evolution of the appendiceal malt-limphoma for which the phenomenon should be considered a prodromal presentation of a more extensive bowel disease which require a close follow-up and specific therapy (Aomatsu et al., 2011). Otherwise we can't exclude that the subsequent IBD could be an autonomous, subsequent disease considered that, also in this case, there are no data in the Literature. Furthermore, given the previous appendiceal malt-lymphoma, the efficacy of mesalazine alone, without the use of immunosuppressive drugs, can be considered a very favorable factor in our case. In conclusion, even if the occurrence of malignant appendiceal pathology in children is rare (Setty & Termuhlen, 2010), the probability that it is asymptomatic is very high. According to our experience, our case suggests that histological examination should always be performed following appendectomy in children and that if a MALT lymphoma were discovered, a close follow-up is strongly recommended, not only for the MALT lymphoma recurrence but also for its possible evolution towards an inflammatory bowel disease.

#### **4. References**

426 New Advances in the Basic and Clinical Gastroenterology

Fig. 9. Mucosal lymphocytes nodule. Glands, well structured, present a reduction of mucus cells. The lamina propria is infiltrated by immunocompetent elements. (20x). 15 cm from the

A 6 weeks cycle of Mesalazine (5ASA), 2gr/day was administered to the patient obtaining the induction of remission and then repeated every 2 months for the prevention of recurrences. At present the patient is doing well and a strict clinical serologic follow-up with calprotectin, P-Anca and ASCA is scheduled every 6 months and, yearly, colonoscopy.

Current knowledge of MALT lymphoma is largely based upon studies in adults. MALT lymphoma is rare in children; the available evidence consists mostly of isolated case reports, except for one series of ten cases (Corr et al., 1997), and another including a total of 48 cases (children and young adults) (Taddesse-Heath et al., 1997) and a pediatric NHL trial recruiting children and adolescents from Germany, Austria and Switzerland (Kaatsch et al., 2004). MALT often develops within the context of a pre-existing inflammatory response due to infection or to autoimmune disorder. Many studies show the relationship between *H. pylori* infection and gastric MALT lymphoma (Isaacson & Whright, 1984; Kurugoglu et al., 2002); some authors have reported a regression of MALT lymphoma in parotid gland (Alkan et al., 1996), lip gland (Berrebi et al., 1998), small intestine (Fischbach et al., 1997) and

anal verge.

**3. Discussion** 


**20** 

**The Surgical Management** 

S. Burmeister, P.C. Bornman, J.E.J. Krige and S.R. Thomson

Chronic pancreatitis (CP) has been defined as a continuing inflammatory disease of the pancreas characterized by irreversible morphological changes, often associated with pain and with the loss of exocrine and endocrine function which may be clinically relevant (Clain JE Surg Clin North Am 1999). Pain is the principal cause of intractability and together with pancreatic insufficiency may have a significantly deleterious effect on a patient's quality of life as well as their ability to work and contribute to society, often leading to loss of their' social support network (Lankisch PG Digestion 1993). Progressive disease may culminate in severe and disabling symptoms requiring narcotic analgesia and frequent hospital admission with a consequent impact on health resources (Bornman PC W J Surg 2003; Braganza JM The Lancet 2011). The incidence and prevalence of disease has not been well documented however it is considered uncommon in Europe and the USA. This is in contrast to data available from South India where a prevalence of 114-200/100 000 people has been documented. Alcohol is the leading cause in western developed countries and some developing countries such as Brazil, Mexico and South Africa while idiopathic disease predominates in Asia and the subcontinent (Braganza JM The Lancet 2011; Garg PK J

Despite extensive study, the pathogenesis of chronic pancreatitis and the mechanisms which result in the development of pain remain poorly understood. As a result, treatment strategies have been largely empirical and based on symptoms, management of clinically evident exocrine and endocrine dysfunction and gross morphological abnormalities. Modalities employed have included medical support (with analgesics, anti-diabetic medication, pancreatic enzyme replacement, nutrient support and steroids in autoimmune disease), interventional endoscopy and surgery. The role of surgery has been primarily to relieve pain refractory to medical therapy, to address complications and to resect suspected or confirmed neoplastic disease (Bornman PC S Afr Med J 2010). The causes of pain in CP are likely multifactorial and proposed factors include excessive oxygen-derived free radicals, tissue hypoxia and acidosis, inflammatory infiltration accompanied by an influx of pain transmitted substances into damaged nerve ends and the development of pancreatic ductal and tissue fluid hypertension (Bornman PC W J Surg 2003). Surgical intervention for the relief of pain focuses primarily on the latter two proposed mechanisms. Two distinct principles have been applied in the development of

**1. Introduction** 

Gastroenterol Hepatol 2004).

**of Chronic Pancreatitis** 

*University of Cape Town* 

*South Africa* 

