Gastrosplenic fistula occurring in lymphoma patients: Systematic review with a new case of extranodal NK/T-cell lymphoma

Abstract

Aim: To provide the overall spectrum of gastrosplenic fistula (GSF) occurring in lymphomas through a systematic review including a patient at our hospital.

Methods: A comprehensive literature search was performed in the MEDLINE database to identify studies of GSF occurring in lymphomas. A computerized search of our institutional database was also performed. In all cases, we analyzed the clinicopathologic/radiologic features, treatment and outcome of GSF occurring in lymphomas.

Results: A literature search identified 25 relevant studies with 26 patients. Our institutional data search added 1 patient. Systematic review of the total 27 cases revealed that GSF occurred mainly in diffuse, large B-cell lymphoma (n = 23), but also in diffuse, histiocytic lymphoma (n = 1), Hodgkin's lymphoma (n = 2), and NK/T-cell lymphoma (n = 1, our patient). The common clinical presentations are constitutional symptoms (n = 20) and abdominal pain (n = 17), although acute gastrointestinal bleeding (n = 6) and infection symptoms due to splenic abscess (n = 3) are also noted. In all patients, computed tomography scanning was very helpful for diagnosing GSF and for evaluating the lymphoma extent. GSF could occur either post-chemotherapy (n = 10) or spontaneously (n = 17). Surgical resection has been the most common treatment. Once patients have recovered from the acute illness status after undergoing surgery, their long-term outcome has been favorable.

Conclusion: This systematic review provides an overview of GSF occurring in lymphomas, and will be helpful in making physicians aware of this rare disease entity.

Keywords: Gastrosplenic fistula; Lymphoma; NK/T-cell lymphoma; Systematic review.

Figure 1

Flow diagram for the selection of studies.

Figure 2

On a coronal computed tomography image taken 2 mo after autologous stem-cell transplantation, the spleen was enlarged, measuring 17 cm in the longest dimension, and indicative of recurred lymphoma. The enlarged spleen abutted to the gastric fundus.

Figure 3

On an axial computed tomography image taken after chemotherapy, a huge fistula was shown between the gastric lumen and the spleen. The spleen was totally infarcted.

Figure 4

Microscopic specimen of the spleen and stomach. A: Atypical lymphoma cells were found in the spleen on hematoxylin-eosin stain (left). These cells showed positivity for CD3 on immunohistochemistry stain (middle) and EBV on EBV RNA stain (right). There was also extensive coagulative necrosis indicative of splenic infarction; B: Lymphoma cells were found in the stomach wall near the gastrosplenic fistula on hematoxylin-eosin stain (left), and which also showed positivity for CD3 (middle) and EBV (right). NK/T-cell lymphoma was diagnosed. These findings suggested that lymphoma cells may have infiltrated from the spleen to the stomach wall through the perforation site. EBV: Epstein-Barr virus.