Enteral Fistula as Initial Manifestation of Primary Intestinal Lymphoma
Primary intestinal lymphoma (PIL) is a rare form of non-Hodgkin lymphoma that primarily affects the gastrointestinal tract. Among its complications, enteral fistula (EF) is an uncommon but significant manifestation, often presenting diagnostic and therapeutic challenges. This article provides a comprehensive overview of the clinical, diagnostic, and therapeutic aspects of PIL with EF as the initial manifestation, based on a detailed review of relevant literature.
Introduction
Enteral fistula is a pathological connection between the intestine and another organ or the skin, most commonly resulting from inflammatory bowel disease, duodenal ulcer, intestinal tuberculosis, or abdominal surgery. In rare cases, EF can be the initial presentation of primary intestinal lymphoma, a condition that is often misdiagnosed due to its nonspecific symptoms and radiographic findings. The early diagnosis of PIL in patients presenting with EF is crucial for appropriate management and improved outcomes.
Epidemiology and Clinical Presentation
The review of 12 cases from the literature revealed a male predominance (10 out of 12 patients), with a median age of 58 years, ranging from 3 to 86 years. The most common clinical manifestations included abdominal pain, diarrhea, and weight loss. These symptoms are nonspecific and can mimic other gastrointestinal disorders, such as Crohn’s disease, making early diagnosis challenging.
Medical History and Risk Factors
In the reviewed cases, 10 out of 12 patients had available medical histories. Two patients had human immunodeficiency virus (HIV) infection, two had a history of gastrointestinal surgery, and one had tuberculosis. These comorbidities may contribute to the development of PIL and its complications, including EF. However, the exact mechanisms remain unclear and warrant further investigation.
Diagnostic Imaging
Radiologic examination plays a crucial role in the diagnosis of PIL with EF. Enhanced computed tomography (CT) scan was the most commonly used imaging modality, revealing findings such as focal wall thickening, fistula formation, and lymph node enlargement. However, these findings are nonspecific and can overlap with other conditions, particularly Crohn’s disease. A retrospective study comparing CT enterography of PIL and Crohn’s disease found that circular thickening of the bowel wall, wall thickness greater than 8 mm, involvement of three or more segments, and aneurysmal dilation were more prevalent in PIL. These features may aid in the differential diagnosis, but they were not consistently reported in the reviewed cases.
Pathologic Findings
Pathologic examination is essential for the definitive diagnosis of PIL. In the reviewed cases, 8 out of 12 patients had B-cell lymphoma, with diffuse large B-cell lymphoma (DLBCL) being the most common subtype. Aggressive B-cell lymphomas, particularly DLBCL, have a higher risk of perforation and fistula formation compared to indolent types. This is consistent with previous studies that reported a significantly higher risk of perforation in aggressive B-cell lymphomas. Additionally, T-cell PIL has been associated with a higher perforation rate compared to B-cell types, further emphasizing the importance of pathologic subtype in the risk of complications.
Treatment Strategies
The management of PIL with EF involves a combination of supportive care, surgery, and chemotherapy. Supportive care is crucial in the initial treatment to stabilize the patient and prepare for definitive therapy. Surgery is often required to address the fistula and any associated complications, such as abscess formation or bowel obstruction. However, the decision to perform surgery must be carefully considered, given the risk of perioperative complications.
Chemotherapy is the cornerstone of treatment for PIL, particularly for aggressive subtypes such as DLBCL. In the reviewed cases, 6 out of 12 patients received combination therapy (surgery plus chemotherapy), and 5 of these patients were still alive during the follow-up period, with a median duration of 12 months. This suggests that combination therapy may lead to better outcomes compared to surgery alone.
An innovative approach reported in one case involved the use of a covered intestinal stent combined with chemotherapy. The stent was designed to exclude the defect and restore enteral nutrition, allowing for earlier initiation of chemotherapy without waiting for postoperative recovery. This approach highlights the potential for minimally invasive techniques in the management of PIL with EF.
Prognostic Factors
The prognosis of PIL with EF is influenced by several factors, including the pathologic subtype, stage of the disease, and treatment modality. Aggressive B-cell lymphomas, particularly DLBCL, are associated with a higher risk of complications and poorer outcomes. Advanced stage (III/IV), B symptoms, high international prognostic index score, absence of radical surgery, and lack of chemotherapy are also predictors of poor prognosis.
The 5-year survival rate of PIL varies widely, ranging from 24% to 71.6%, depending on the stage and treatment received. The development of effective chemotherapy regimens and the use of combination therapy have improved survival rates in recent years. However, long-term survival analysis is challenging due to the limited number of cases and follow-up information available in the literature.
Differential Diagnosis
The differential diagnosis of EF in the context of PIL is particularly challenging due to the overlap of clinical and radiologic features with other conditions, such as Crohn’s disease. The lack of specific diagnostic criteria for PIL with EF further complicates the diagnostic process. Radiologic findings such as circular thickening of the bowel wall, wall thickness greater than 8 mm, involvement of multiple segments, and aneurysmal dilation may help differentiate PIL from Crohn’s disease. However, these features were not consistently reported in the reviewed cases, highlighting the need for further research to identify reliable diagnostic markers.
Conclusion
Enteral fistula as the initial manifestation of primary intestinal lymphoma is a rare but significant condition that poses diagnostic and therapeutic challenges. The reviewed cases demonstrated a male predominance, with nonspecific clinical and radiologic findings that can mimic other gastrointestinal disorders. Pathologic examination is essential for definitive diagnosis, with aggressive B-cell lymphomas, particularly DLBCL, being the most common subtype. Combination therapy involving surgery and chemotherapy appears to offer the best outcomes, although the decision to perform surgery must be carefully considered.
The differential diagnosis of PIL with EF remains challenging, and further research is needed to identify reliable diagnostic markers. The use of innovative techniques, such as covered intestinal stents, may offer new avenues for treatment. Despite advances in therapy, the prognosis of PIL with EF remains variable, and long-term survival analysis is limited by the scarcity of cases and follow-up information.
In conclusion, early diagnosis and appropriate management are crucial for improving outcomes in patients with PIL presenting with EF. Clinicians should maintain a high index of suspicion for PIL in patients with unexplained EF, particularly in the presence of risk factors such as HIV infection or a history of gastrointestinal surgery. Further research is needed to better understand the pathogenesis of PIL with EF and to develop more effective diagnostic and therapeutic strategies.
doi.org/10.1097/CM9.0000000000000598
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