Jejunocolic fistula presenting as chronic diarrhoea from advanced lymphoma
- Rogelio N Velasco, Jr. 1,
- Mark M Ando 1,
- Hashamiiya M Babaran 2,
- Ma. Corazon C Manuntag 3 and
- Dennis Lee Sacdalan 1
- 1 Division of Medical Oncology, Department of Medicine, University of the Philippines - Philippine General Hospital, Manila, Philippines
- 2 Division of Gastroenterology, Department of Medicine, Philippine General Hospital, Manila, Philippines
- 3 Department of Surgery, Philippine General Hospital, Manila, Philippines
- Correspondence to Dr Rogelio N Velasco, Jr.; rogervelascojr@gmail.com
Abstract
Jejunocolic fistula, a late complication of intestinal lymphoma, is a rare entity with only five reported cases in the literature. We report a young male presenting with a left lateral neck mass 5 years ago which then progressed to superior vena cava syndrome. Despite receiving radiotherapy and two cycles of chemotherapy, there was still tumour progression. He presented with a 2-week history of diarrhoea, haematochezia and weight loss for which antibiotics provided no relief. Esophagogastroduodenoscopy and colonoscopy showed a jejunocolic fistula. After discussion with the multidisciplinary team, nutritional upbuilding was initiated followed by exploratory laparotomy with segmental resection and anastomosis. On histopathology, diffuse large B-cell lymphoma involving the jejunum and colon was noted. Despite receiving palliative chemotherapy, he still succumbed to disease progression. This case highlights the importance of early recognition of jejunocolic fistulas among patients with intestinal lymphomas to facilitate early multidisciplinary intervention.
Background
The gastrointestinal tract, the most common site of extranodal lymphoma, is seen in 5%–20% of lymphoma cases.1 2 Diffuse large B cell lymphoma (DLBCL), the most common B cell neoplasm, is characterised by an aggressive course and predisposition among males.3 Gastrointestinal fistulas can arise following abdominal surgeries or secondary to pathologies such as tuberculosis and Crohn’s disease.4 Rarely, tumour bowel fistulas can complicate aggressive abdominal malignancies secondary to disease progression, chemotherapy or radiotherapy.5 Jejunocolic fistula, a rare complication of lymphoma, has only been reported in five cases in the literature.6–10 Because of the non-specific signs and symptoms of jejunocolic fistulas, early diagnosis and intervention are integral in the management.
Here, we present a case of advanced intestinal diffuse large B-cell lymphoma presenting with chronic diarrhoea secondary to jejunocolic fistula complicated by anaemia, malnutrition and electrolyte disturbance, with particular elaboration on the importance of early recognition and multidisciplinary management.
Case presentation
A man in his 20s and a smoker presented with a 2-month history of cough with associated haemoptysis 5 years prior. Two months later, he presented with progressive dyspnoea and plethora. He was assessed to have superior vena cava (SVC) syndrome based on CT scan findings of a mediastinal mass and compression of the SVC. Due to the urgency and clinical findings compatible with lymphoma in a young patient, he was treated with chemotherapy and radiotherapy. He received two cycles of cyclophosphamide, epirubicin, vincristine and prednisone and 23 sessions of radiotherapy with a total dose of 1200 cGy to the SVC area, 3400 cGy to the chest and 400 cGy to the gross tumour volume as a boost. There was complete resolution of the facial swelling and dyspnoea.
Biopsies of the neck lymph node and mediastinum were pursued to ascertain the aetiology. Leucocyte common antigen, cytokeratin, cluster of differentiation (CD) 15, 30 and 138 were all negative, while CD68 was positive in the supraclavicular lymph node consistent with chronic inflammation. Necrotic tissue was noted on the anterior mediastinal mass sample. Infectious aetiology for the mediastinal mass was also considered; hence, acid-fast bacilli and fungal tests were performed but turned out negative. A positron emission tomography scan showed a mildly metabolic mediastinal mass measuring 7.6 cm × 5.9 cm × 4.0 cm (stable in size compared with initial chest CT scan) with a maximum standard uptake value (SUVmax) of 2.5 extending to the prevascular and para-aortic regions, and posteroinferiorly to the pre-carinal and left peribronchial areas confluent with the mediastinal lymph nodes. In addition, an intensely avid right renal mass (5.0 cm × 5.2 cm × 4.5 cm, SUVmax 68.7) and prominent mesenteric lymph nodes up to approximately 1.9 cm with SUVmax of 24.8 were noted. In the interim, the patient was asymptomatic.
Investigations
Three years ago, he presented with a 2-week history of diarrhoea, haematochezia and progressive weight loss. He was seen at the clinic weak-looking and with markedly reduced weight. An infectious aetiology was initially entertained. However, stool culture was negative and the antibiotic was subsequently discontinued. Other causes for chronic diarrhoea were considered including gastrointestinal tuberculosis and malignancy. CT scan noted segmental wall thickening of the jejunum measuring up to 1.2 cm in thickness. Notably, there was air-filled and contrast-filled direct communication between the jejunum and the descending colon, consistent with fistula formation (figure 1). Upper endoscopy was unremarkable, while colonoscopy confirmed a fistula lined with small intestinal mucosa, located seven centimetres from the anal verge, and a circumferential fungating mass with exudates at the descending colon 35–43 cm from the anal verge (figure 2).
CT scan showing a contrast-filled fistulous tract between the jejunum (blue arrow) and the descending colon (yellow arrow) exhibiting thickened walls.
Colonoscopy findings demonstrating a fistula. (A) Fistulous tract opening (denoted by an asterisk) showing connection to the small intestine and surrounded by a circumferential ulcerating mass at the descending colon. (B) Fistulous tract opening into the small intestine.
Differential diagnosis
Because of his persistent watery diarrhoea, an infectious aetiology was initially considered. Antibiotic was given empirically but was eventually discontinued after stool culture yielded negative results. Other differentials presenting with chronic diarrhoea such as gastrointestinal tuberculosis, inflammatory bowel disease and lymphoma.
Treatment
On discussion with the colon multidisciplinary team, he underwent exploratory laparotomy with segmental resection of the jejunum distal to the ligament of Treitz and proximal to the ileocecal valve. An en bloc resection of the fistula was performed with anastomosis between the jejunojejunum and the descending colon. Gross examination of the specimen revealed that the jejunum, 53.5 cm in length, was adherent to the colon, 11.0 cm in length, at two points. A 4.0×3.0×2.2 cm yellowish, rubbery and fungating mass emanating from the mesentery of the jejunum was also noted. Cut sections of the intestines showed two fistulous tracts between the jejunum and the descending colon with necrotic mucosa adherent on the said tracts. Histopathological examination showed findings consistent with DLBCL diffusely involving the jejunum and descending colon (figure 3). He then underwent chemotherapy with rituximab, cyclophosphamide, vincristine, doxorubicin and prednisone (R-CHOP) for two cycles.
Histological sections of the specimen. (A) Jejunal mass showing sheets of dyshesive cells of varying sizes (H&E×10). (B) Lymphoid cells with oval to round, vesicular nuclei interspersed with normal lymphocytes (H&E×40).
Outcome and follow-up
Despite receiving two cycles of R-CHOP, he still suffered from diarrhoea and malnutrition. From a body mass index (BMI) of 34.3 kg/m2 before being diagnosed with lymphoma, his BMI dropped to 19.8 kg/m2 prior to chemotherapy and further declined when he was admitted for profound weakness and diarrhoea. His initial laboratories during his admission showed low serum sodium of 130 (N: 137–145 mmol/L), potassium of 1.4 (N: 3.5–5.1 mmol/L), magnesium of 0.42 (N: 0.7–1.0 mmol/L), albumin of 18 (N: 35–50 g/L) and random blood sugar of 71.4 (N: 74–106 mmol/L). His severe electrolyte imbalance was addressed through oral and intravenous replacement. He was also given intravenous fluid to address fluid losses and the resultant dehydration. He was seen by the dietary service for upbuilding. His total caloric intake was increased to 1800 kcal/day with high total protein content. Due to his poor performance status, he decided not to pursue further chemotherapy and opted for supportive care. He survived for 33 months since initial symptoms and 6 months after the last cycle of chemotherapy. He then eventually expired secondary to disease progression.
Discussion
Around 80% of small intestinal fistulas are secondary to postabdominal surgeries such as small bowel resection and lysis of adhesions.11 12 Other causes of fistulas include spontaneous formation from bowel diverticula, chronic diseases such as Crohn’s disease and postradiotherapy complications.13–16 Intestinal fistulas may also arise from malignancies including colon cancer as a consequence of tumour growth exerting pressure on the adjacent tissues disrupting the normal tissue barrier and forming fistulous tracts with adjacent surfaces.15 Rarely, jejunocolic fistulas complicate advanced malignant lymphomas, with only five reported cases in the literature (table 1).
Reported cases of enterocolic fistulas among patients with lymphoma
| Author | Lymphoma type | Age | Survival based on article | Fistula type | Therapy |
| Cessot6 | T-cell | 81/F | 9 months | Jejunocolic | Surgery and chemotherapy |
| Chun7 | T cell | 56/M | Died 2 months after surgery | Jejunocolic | Surgery |
| Gale22 | T cell | None | Died 1 month after chemotherapy | Ileocolic | Chemotherapy |
| Hopkins23 | Unspecified | 58/M | Alive | Duodenocolic | Surgery |
| Jangjoo8 | Low-grade B-cell | 36/M | Alive | Jejunocolic | Surgery and chemotherapy |
| Kumar (paper not available)9 |
Unspecified | -- | -- | Jejunocolic | -- |
| Matsumoto24 | DLBCL | 67/M | Died 4 weeks after surgery | Ileocolic | Surgery |
| McMahon10 | T cell | 61/F | Alive | Jejunocolic | Surgery |
| Okuda25 | Burkitt’s lymphoma | 3/M | Alive | Duodenocolic | Surgery and chemotherapy |
| Senyondo26 | DLBCL | 55 /M | Alive | Ileocolic | Surgery and chemotherapy |
| Wang27 | DLBCL | 86 /F | Alive | Enterocolic | Supportive care |
| Zhuang28 | DLBCL | 60/M | Alive | Ileocolic | Surgery and chemotherapy |
| DLBCL | 43 /M | Alive | Ileocolic | Chemotherapy |
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DLBCL, diffuse large B cell lymphoma; F, female; M, male.
The diagnosis of this complication from gastrointestinal lymphomas begins with a comprehensive history and physical examination. Signs and symptoms range from nonspecific weakness, fever and malaise to life-threatening diarrhoea and bleeding.17 Because of their non-specific symptomatology, lymphomas of the small intestine are usually diagnosed after the appearance of late complications such as perforation, haemorrhage and obstruction.7 In our patient, the presence of weight loss and bleeding pointed to a more advanced disease stage. Although the use of a CT scan already confirmed the presence of an enterocolic fistula, we performed a colonoscopy to obtain tumour tissue for histopathology and to determine the extent of the disease for treatment planning.
Management of patients with intestinal fistulas entails a holistic approach. Fluid and electrolyte imbalances are often encountered among patients with fistulas, notably hypokalaemia as seen in our patient, as a consequence of electrolyte loss from the effluent.18 To address this, the fluid and electrolyte derangements were addressed through oral and intravenous correction with careful monitoring of the fluid balance. We also obtained a stool culture to ensure no active infection was present. Supportive antidiarrhoeal medications were likewise given for symptomatic relief.
Protein-caloric malnutrition is almost universal among patients with fistulas as a result of bypassed absorptive segments of the intestine and lost enteric contents.18 He also presented with severe malnutrition, a consequence of tumour-induced metabolic demand, poor appetite and treatment-related effects.19 Espoused by the European Society of Parenteral and Enteral Nutrition, nutritional assessment and intervention can potentially circumvent the effects of malnutrition among cancer patients.20 He underwent nutritional upbuilding prior to surgical resection and palliative chemotherapy. Anaemia may arise from slow blood loss from a friable fistulous tract. Our patient underwent regular haematological monitoring and supportive blood transfusion before surgery and chemotherapy.
Due to the rarity of this complication, the course of treatment is not standardised. Surgery remains an important option especially in localised cases and should be prioritised when feasible.8 Reported cases of jejunocolic fistulas in literature underwent either surgery, chemotherapy or both depending on the patient profile. Cessot et al reported a patient presenting with diarrhoea and weight loss who underwent right hemicolectomy and small intestinal resection followed by chemotherapy.6 Jangjoo et al reported a 36-year-old man who underwent resection and anastomosis followed by chemotherapy.8 Chun et al presented a 56-year-old man who underwent right hemicolectomy and jejunectomy but eventually expired after surgery.7 McMahon et al reported a woman, 61 years old, who underwent resection and anastomosis and subsequent chemotherapy with CHOP.10 Similarly, two cases of duodenocolic fistula from colon adenocarcinoma were also reported in our institution. Owing to the advanced stage of their disease, complete resection was not feasible; instead, tube jejunostomy and ileostomy were performed after addressing malnutrition and electrolyte derangements.21
To our knowledge, this is the sixth reported case of lymphomatous jejunocolic fistula in the literature. Intestinal fistulas should be considered among cancer patients who present with chronic diarrhoea and malnutrition. Foremost in the management is addressing the fluid and electrolyte abnormality and underlying malnutrition. For patients with advanced disease stage, surgery may be performed followed by chemotherapy.
Patient’s perspective
It is very painful for us to know that our son had lymphoma. Urgent chemotherapy and radiation are both important in his treatment. We would like to thank all the members of the team for their financial support and health services they rendered to my son. Patients should follow the advice of their doctors. In addition, always maintain a steadfast spirit and unwavering faith. Prepare to face whatever challenges lie ahead and learn to trust .
Learning points
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Gastrointestinal lymphomas should be a differential in patients with chronic diarrhoea.
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Establishing a histopathological diagnosis is paramount in the management of lymphoma.
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Among patients with chronic diarrhoea and bleeding, prompt colonoscopy is essential in determining the aetiology.
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Management of gastrointestinal lymphoma and fistulas involves a multidisciplinary approach.
Ethics statements
Patient consent for publication
Footnotes
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Contributors RJV conceptualised the study, obtained the informed consent and drafted the final manuscript. MA drafted and revised the final version of the manuscript. MC contributed in the discussion of the case in the manuscript. HB collected the relevant images and revised the final version of the manuscript. DLS spearheaded the project and revised the final version of the manuscript.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
References
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