Contributions
Abstract: PB2034
Type: Publication Only
Background
Duodenal-type follicular lymphoma was firstly recognized as a distinct entity in the 2016 revision of the WHO classification. Most cases are asymptomatic, confined to the duodenum, dissemination being very rare and limited to the small bowel. Currently, the best therapeutic approach is unknown, due to its rarity and to the lack of large series. In the few cases that do present symptoms or are at risk of local complications intrinsic to the localization of the tumour (obstruction, haemorrhage, perforation), the option watch and wait is unsuitable and the difficulty increases of having to choose among the various options: surgery, radiotherapy, immunotherapy or immunochemotherapy.
Aims
Evaluate if Rituximab in monotherapy is an adequate treatment for patients with duodenal-type follicular lymphoma with symptoms or complications.
Methods
We report 3 cases (patients aged 57, 68 and 74) which were diagnosed due to dyspeptic complaints (2) and by chance (1). All of the patients mentioned were followed from the diagnosis and after treatment until the present day.
Results
Duodenal lesions were described as nodular in 2 cases and extensively ulcerated in the third, measuring from 3.6 to 6 cm. Histologically, all were low-grade (1/2). Two cases presented as isolated duodenal disease and one had biopsy-confirmed ileal involvement. No extra-intestinal involvement was documented. The watch and wait approach was deemed inadequate due to the extension and ulceration in one case and the presence of symptoms in the other two. Patients were treated with rituximab in monotherapy. All became asymptomatic (sustained after a 12-month period), and the 6-month endoscopic evaluation showed complete regression of the lesions in 2 of the patients and partial regression in the other (40% reduction).
Conclusion
Duodenal-type follicular lymphoma is very rare and data regarding treatment options are limited due to the small number of reported cases. Concerning the therapeutic approach, it seems to be a remarkably indolent variant of follicular lymphoma, which may have a watch and wait approach in most cases. Nonetheless, although randomized studies are difficult in this rare disorder, when the presentation implies treatment, whether due to the existence of symptoms or to a risk of local complications, immunotherapy with an anti-CD20 antibody is an appropriate option.
Session topic: 20. Indolent Non-Hodgkin lymphoma – Clinical
Keyword(s): Follicular lymphoma, Indolent Non-Hodgkin's Lymphoma, Therapy
Abstract: PB2034
Type: Publication Only
Background
Duodenal-type follicular lymphoma was firstly recognized as a distinct entity in the 2016 revision of the WHO classification. Most cases are asymptomatic, confined to the duodenum, dissemination being very rare and limited to the small bowel. Currently, the best therapeutic approach is unknown, due to its rarity and to the lack of large series. In the few cases that do present symptoms or are at risk of local complications intrinsic to the localization of the tumour (obstruction, haemorrhage, perforation), the option watch and wait is unsuitable and the difficulty increases of having to choose among the various options: surgery, radiotherapy, immunotherapy or immunochemotherapy.
Aims
Evaluate if Rituximab in monotherapy is an adequate treatment for patients with duodenal-type follicular lymphoma with symptoms or complications.
Methods
We report 3 cases (patients aged 57, 68 and 74) which were diagnosed due to dyspeptic complaints (2) and by chance (1). All of the patients mentioned were followed from the diagnosis and after treatment until the present day.
Results
Duodenal lesions were described as nodular in 2 cases and extensively ulcerated in the third, measuring from 3.6 to 6 cm. Histologically, all were low-grade (1/2). Two cases presented as isolated duodenal disease and one had biopsy-confirmed ileal involvement. No extra-intestinal involvement was documented. The watch and wait approach was deemed inadequate due to the extension and ulceration in one case and the presence of symptoms in the other two. Patients were treated with rituximab in monotherapy. All became asymptomatic (sustained after a 12-month period), and the 6-month endoscopic evaluation showed complete regression of the lesions in 2 of the patients and partial regression in the other (40% reduction).
Conclusion
Duodenal-type follicular lymphoma is very rare and data regarding treatment options are limited due to the small number of reported cases. Concerning the therapeutic approach, it seems to be a remarkably indolent variant of follicular lymphoma, which may have a watch and wait approach in most cases. Nonetheless, although randomized studies are difficult in this rare disorder, when the presentation implies treatment, whether due to the existence of symptoms or to a risk of local complications, immunotherapy with an anti-CD20 antibody is an appropriate option.
Session topic: 20. Indolent Non-Hodgkin lymphoma – Clinical
Keyword(s): Follicular lymphoma, Indolent Non-Hodgkin's Lymphoma, Therapy