Considering treatment of colon NHL the most important role may be attributed to surgery in association with neo-adjuvant or adju-

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1 POLSKI PRZEGLĄD CHIRURGICZNY 2015, 87, 3, /pjs R E V I E W P A P E R S Colon s rare case of intestinal resection Tomasz Goryń, Cezary Rykała, Jacek Pawlak Department of General Surgery, John Paul II Western Hospital in Grodzisk Mazowiecki Ordynator: prof. dr hab. J. Pawlak Malignant s are a heterogenous group of tumors of the lymphatic system. According to the WHO classification the above-mentioned group includes more than 30 types of carcinomas. One of the group includes gastrointestinal s, which constitute up to 20% of all malignant s (1). Non-Hodgkin s s (NHL) may be present in all internal organs, although their most frequent location is the gastrointestinal tract. Gastrointestinal s represent 10% of all NHL (2). Amongst all gastrointestinal tumors NHL constitute 5 to 10% of all lesions (1, 3). NHL may be present in the entire gastrointestinal tract, although most commonly being diagnosed in the stomach (50%- 60%), and least likely found in the colon (4 to 15%) (2). Considering the above-mentioned location they are most often found in the ileocecal segment, amounting to 37% of all cases. In the remaining segments of the colon they are sporadically observed (3, 4). Amongst the best known etiological factors associated with non-hodgkin s s, one should include chronic gastritis with Helicobacter infection, celiac disease, inflammatory bowel diseases, acquired and congenital immunodeficiency disorders, HIV infections, and immunosuppression after transplantation. However, the definition of their role in the development of NHL requires further investigations (5). The dominant histopathological types of NHL present in the gastrointestinal tract include extranodal marginal zone B-cell of the mucosa associated lymphoid tissue (MALT-) and diffuse large B-cell (1, 6). However, colon NHL have a different histopathological structure, as compared to NHL of the stomach, which is also associated with different therapeutic methods and prognosis. Diagnostics of NHL located in the gastrointestinal tract is difficult, since quite often the course of the disease is symptomless with symptoms reported by the patients as uncharacteristic. The most commonly observed symptoms include gastrointestinal occlusion leading to nausea, vomiting, weight loss, and bowel habit disturbances (as in case of adenocarcinomas). Patients also complain of non-specific abdominal pain. NHL of the colon may also be responsible for gastrointestinal perforation and bleeding. In selected cases one may observe intestinal occlusion (5). The physical examination might reveal the presence of a large nodular mass located in the abdominal cavity. Diagnostics include laboratory, endoscopic, and radiological examinations. In selected cases bone-marrow biopsy might prove helpful. Imaging diagnostics include abdominal ultrasound and computer tomography, abdominal X-ray and barium enema (2). If the patient does not present acute symptoms requiring emergency surgery one should strive towards histopathological confirmation obtained by means of endoscopic examinations, biopsies under ultrasound control, or laparoscopy. It is important to determine the stage of the tumor, as well as assess the presence of distant metastases. Considering treatment of colon NHL the most important role may be attributed to surgery in association with neo-adjuvant or adju-

2 Colon s rare case of intestinal resection 149 vant chemotherapy and radiotherapy. Current algorithms and chemotherapy regimens include the use of cyclophosphamide, doxorubicin, vincristin, prednisolon, and immunochemotherapy with the use of anty-cd20 antibodies. In recent years, along with traditional radiotherapy one may also use radioimmunotherapy with monoclonal antibodies (7). In case of perforation, occlusion, or gastrointestinal bleeding which cannot be controlled by means of endoscopy or conservative treatment, surgery seems to be the method of choice (4). Due to the extent of the infiltration some patients require palliative resections leaving neoplastic tissue-cytoreduction operations. Case reports During the period between 2006 and 2012, 257 colon resections were performed at the Department of General Surgery, John Paul II Western Hospital in Grodzisk Mazowiecki, due to neoplastic lesions. Four (1.6%) patients were subjected to surgery, due to non-hodgkin s s. Table 1 presented patient characteristics. Two patients required surgery, due to occlusion symptoms. H.D, an 84-year old male patient (history nb /2012) required emergency surgery, due to gastrointestinal bleeding. The patient was hospitalized in another department of surgery because of gastrointestinal bleeding and vitamin K inhibitors overdose. After the restoration of coagulation disturbances abdominal CT was performed, which revealed the presence of a cecal tumor (fig. 1). Based on the CT result the patient was qualified for surgical treatment. After preparation right-sided hemicolectomy was performed on April 24, A large perforated cecal tumor infiltrating the right iliac vessels and compressing the right ureter causing dilatation was removed. During the postoperative period deterioration of heart failure and renal insufficiency were observed, and the patient died after three days. The histopathological result (nb ) revealed the presence of DLBCL. The infiltration comprised the entire colon wall with ulceration and perforation. Infiltration of the small bowel mesentery was also observed. Seven lymph nodes showed no signs of metastasis. Immunohistochemical staining was as follows: ki % CD20 (+). E.G, a 46-year old male patient (history nb -5319/2012) required emergency surgery, due to subocclusion symptoms caused by a cecal tumor. The patient had complained of medium intensity abdominal pain lasting for the past 2 3 months with progression of diarrhea and vomiting episodes. Weight loss amounted to 14 kg. The physical examination revealed the presence of a palpable, 10 cm in diameter tumor, located in the right iliac fossa. Laboratory results showed insignificant anemia RBC 4 mln/µl; HGB 10.9 mg%; HCT 33.4%. The remaining results were within normal limits. The abdominal ultrasound showed a cecal lesion of neoplastic character and distended intestinal loops. The examination showed no distant metastases. The patient was qualified for surgery. Laparotomy was performed which revealed the presence of a 20 cm cecal tumor infiltrating the retroperitoneal space. Right-sided hemicolectomy was performed. The postoperative course was uneventful. Abdominal CT performed after the operation showed right ureterectasia with an infiltrating lesion from the right abdominal cavity to the level of the iliac joint. The infiltra- Table 1. Indications for surgical intervention Lp. Data Gender Age Cause of surgery Risk factors 1 B.K. female 69 recurrence after pharmacological treatment none Histological type of high- grade 2 E.G. male 46 occlusion none high-grade b-cell 3 H.D. male 84 gastrointestinal circulatory insufficiency, arrythmias, DLBCL bleeding diabetes, renal failure 4 W.K. male 71 subocclusion diabetes, circulatory insufficiency, MALT- marginal arterial hypertension cell Location transverse colon

3 150 T. Goryń et al. tion comprised the wall of the abdominal cavity, greater iliac muscle, rectus muscle, inferior caval vein and intestinal loops modelling the abdominal aorta. Numerous packets of enlarged mesenteric lymph nodes were observed, 30 x 20 mm in size (fig. 2). The patient was directed for further adjuvant oncological treatment. The histopathological result (nb ) showed the presence of a high-grade B cell, which probably corresponds to Burkitt s. The infiltration comprised the entire colon wall and distal segment of the small bowel. 27 lymph nodes showed signs of malignancy. Immunohistochemical staining was as follows: ki67 up to 100% CD20 (+), Bcl2 (-), Bcl+ (-). W.K, a 71-year old male patient (history nb-29940/2008/) required emergency surgery, due to a transverse colon tumor with symptoms of subocclusion. The patient complained of unspecific abdominal pain lasting for the past 4 months with gradually intensifying constipation. Weight loss amounted to 5 kg. The physical examination showed no abnormalities, while laboratory results were as follows: RBC 4 mln/µl; HGB 10.9 mg%; HCT 33.4%. Abdominal X-ray showed signs of occlusion. Distant lesions of metastatic character were not observed. The patient was qualified for surgery. Laparotomy was performed which revealed the presence of a transverse colon tumor closing the lumen and infiltrating the Fig. 2. The postoperative abdominal CT presenting an infiltrating lesion from the right abdominal cavity to the level of the iliac joint Fig. 1. Abdominal CT - presenting a cecal tumor intestinal serosa. After tumor preparation the transverse colon was excised. The postoperative course was complicated by anastomosis dehiscence with the presence of an intraperitoneal abscess. The patient required reoperation, abscess drainage and colostomy. After surgery the patient remained at the ICU, due to cardiopulmonary insufficiency. After improvement of his general condition the future course was uneventful. The patient was discharged from the hospital on the 21-st day after surgery with recommendation for adjuvant oncological treatment. B.K, a 69-year old female patient (history nb-10507/2007/) underwent elective surgery, due to disease recurrence. The patient was primarily operated in 2004, due to small bowel NHL. After surgery she underwent adjuvant chemotherapy and bone marrow transplantation in Control abdominal CT revealed the possibility of relapse in the and distal segment of the ileum (fig. 3). The patient was qualified for surgery. Intraoperatively, thickening of the cecal wall and ascending colon and numerous enlarged intestinal mesentery lymph nodes were observed. Rightsided hemicolectomy and lymphadenectomy were performed. The operative course was uneventful. During the postoperative course the patient required ICU treatment, due to respiratory insufficiency. The patient was discharged from the hospital on the 10-th postoperative day with recommendations for adjuvant oncological treatment. She remains to date under outpatient observation, without recurrence.

4 Colon s rare case of intestinal resection 151 Fig. 3. Adominal CT presenting a relapse in the and distal segment of the ileum The histopathological result (nb ) showed the presence of recurrence. The infiltration comprised the entire large bowel wall. The collected sample showed 7 lymph nodes, 2 with infiltration. Discussion Non-Hodgkin s s (NHL) located in the gastrointestinal tract are a very rare disease, which may be observed by the surgeon and physicians of other specialties. Diagnostics of NHL is difficult, because symptoms reported by the patients are unspecific, comprising abdominal pain, weight loss, gastrointestinal bleeding, chronic diarrhea, and bloating. Often, one may observe intestinal perforation or massive bleeding as the first symptom and diagnosis is established intraoperativelly (6). Usually, colon NHL are responsible for the development of occlusion. Considering two patients operated in our department a palpable mass was observed in the right iliac fossa. Intraoperatively, cecal tumors were diagnosed infiltrating surrounding organs. The patient with the transverse colon tumor compalined of epigastric tenderness without a palpable mass. In all cases the above-mentioned lesions did not result in complete occlusion, and clinical symptoms developed in advanced stages. Diagnostics of NHL located in the gastrointestinal tract includes endoscopic and imaging examination (abdominal ultrasound and CT). Considering patients operated in our department imaging examinations were the method of choice. Three patients subjected to abdominal CT showed neoplastic lesions of the and transverse colon. In case of patient E.Gabdominal ultrasound showed a large neoplastic lesion located in the. In case of patient-b.k. the past medical history (NHL resection) indicated the possibility of recurrence. In the remaining cases patients were operated because of subocclusion symptoms or gastrointestinal bleeding. In these patients endoscopic histopathological confirmation was not obtained. Treatment of NHL located in the gastrointestinal tract comprises both chemotherapy and surgery. Surgical resections are performed in case of lack of response to chemotherapy, as well as in case of occlusion symptoms and gastrointestinal bleeding, which cannot be managed by means of endoscopic methods or pharmacological treatment (4). One of our patients required reoperation, due to recurrence not responding to chemotherapy. In the remaining three patients surgery was performed because of occlusion and gastrointestinal bleeding. These patients required emergency surgery after initial radiological diagnostics. Patient- H.D. with gastrointestinal bleeding was diagnosed in another medical center. Due to coagulation disturbances (INR>5) emergency colonoscopy was not performed. After normalizing the coagulation disturbances the patient underwent surgery for fear of bleeding recurrence. Patients E.G. and W.K were admitted to the Department, due to occlusion symptoms. In case of patient E.G., abdominal ultrasound was performed, which revealed the presence of a cecal tumor. In case of patient W.K, abdominal X-ray showed signs of obstruction. Considering the material obtained from 257 operations of colon tumors performed during the period between 2006 and 2012 at the Department of General Surgery, John Paul II Western Hospital in Grodzisk Mazowiecki, 4 (1.6%) patients were diagnosed with colon NHL. Three of the patients required emergency surgery, due to gastrointestinal bleeding and subocclusion symptoms. In two of the patients a palpable mass was observed in the right iliac fossa. Imaging examinations showed the presence of large

5 152 T. Goryń et al. neoplastic lesions located in the. In case of three patients endoscopic preoperative histopathological diagnosis was not possible. However, in case of possible safe endoscopy, one should strive to obtain histopathological confirmation, as in case of such large lesions neoadjuvant chemotherapy is the method of choice. In two cases, intraoperative diagnosis showed significant disease advancement. Despite the large size of the tumor both lesions were excised, considering the procedures as cytoreductive. In case of NHL located in the gastrointestinal tract, the advanced stage of the disease does not disqualify the patient from tumor resection, since chemotherapy and adjuvant radiotherapy significantly increase the chance for complete recovery. Conclusions Based on the presented clinical material, in any unclear case of gastrointestinal bleeding or colon tumors leading to obstruction, one should consider colon non-hodgkin s in the differential diagnosis. If it is possible to perform safe endoscopy histopathological diagnosis confirmation should be sought. Additionally, advanced tumors diagnosed intraoperatively do not disqualify from tumor resection. In case of uncharacteristic lesions, infiltration of the vessels and ureters does not disqualify the patient from recovery, since the method of choice in case of s is chemotherapy and radiotherapy. Surgical treatment is reserved for patients diagnosed with obstruction, perforation, or gastrointestinal bleeding. References 1. Kula Z, Weishof A: Chłoniak typu MALT żołądka, dwunastnicy, jelita krętego, kątnicy i wyrostka robaczkowego opis przypadku. Gastroenterol Pol 2005; 12(3): Tauro LF, Furtado HW, Aithala PS et al.: Primary Lymphoma of the Colon. Saudi J Gastroenterology 2009 Oct 15 (4): Daum S, Ullrich R, Heise W et al.: Intestinal non-hodgkin s : a multicenter prospective clinical study from the German Study Group on Intestinal non-hodgkin s Lymphoma. J Clin Oncol 2003 Jul 15; 21(14): Dziurkowska-Marek A, Marek T: Chłoniaki przewodu pokarmowego. Gastroenterol Pol 1999; 6(5): Howell J, Auer-Grzesiak I, Urbanski S: Hematologia 2010; 1(4): Walewski J: Pierwotne chłoniaki przewodu pokarmowego problemy diagnostyki, leczenia. Medipress Gastroenterologia 1998; 3: Cultrera JL, Dalia SM: Diffuse Large B-Cell Lymphoma: Current Strategies and Future Directions. Cancer Control July 2012; 19(3): Received: r. Adress correspondence: Grodzisk Mazowiecki, ul. Daleka 11 chirurgia@szpitalzachodni.pl.