Primary Gastrointestinal Follicular Lymphomas: A Prospective Study of 31 Patients with Long-term Follow-up Registered in the French Gastrointestinal Lymphoma Study Group (GELD) of the French Federation of Digestive Oncology (FFCD).

BACKGROUND/AIMS: Primary gastrointestinal follicular lymphomas (PGFL) are very rare. Our aim was to analyze the clinical features, management, and long-term outcomes in a prospective series of patients diagnosed with PGFL. METHODS: All adult patients with PGFL, consecutively enrolled into the multicenter French study between 1990 and 2017, were evaluated and followed up prospectively after undergoing a complete work-up. Clinical, pathological and endoscopic features, as well as treatment outcomes, were analyzed. RESULTS: Thirty-one patients (16 men, median age 62 years, range 33 to 79 years) with PGFL were included. The median follow-up was 92 months (range, 6 to 218 months). In the majority of patients (n=14), lymphoma was incidentally diagnosed during endoscopy. Otherwise, the most frequent circumstances of diagnosis were abdominal pain (n=7) and dyspepsia (n=5). The duodenum was the most common site of involvement (n=19) and multifocal localizations were seen in seven patients (22%). The most frequent first line strategy was surveillance applied in 22 patients (71%), of whom nine reached spontaneous, complete remission and 11 had stable disease. Seven patients (23%) received chemotherapy as first line treatment, and two underwent resection. Of seven patients who received chemotherapy, four achieved complete remission. In three patients, transformation into a high-grade lymphoma occurred. CONCLUSIONS: The diagnosis of PGFL is frequently fortuitous. The most common localization is in the duodenum. The disease has an indolent course and a good prognosis, however, rare cases of transformation into aggressive high-grade lymphoma may occur. An appropriate characterization and follow-up of these lymphomas is mandatory for their optimal management.

Gastric MALT lymphoma in a population-based study in France: clinical features, treatments and survival.

BACKGROUND: Gastric mucosa-associated lymphoid tissue (MALT) lymphoma is a rare disease, and most available data on gastric MALT lymphoma (GML) come from clinical studies of selected patients treated in centres of excellence. AIMS: To analyse the clinical features, management and survival of GML patients in a population-based study in France METHODS: All new cases of GML diagnosed between 2002 and 2010 in 11 French areas covered by cancer registries were included. Pathology reports were verified and, if necessary, reviewed by an expert pathologist. All clinical data were retrospectively collected from medical files and analysed using stata V. 14 software. RESULTS: Four hundred and sixteen patients with confirmed GML (50% male, median age 67 years) were identified. Among them, 44 showed an early transformation into diffuse large B cell lymphoma and were considered to have had an initially missed high-grade lymphoma. At diagnosis, 76% of patients were at stage IE/II, and 24% at stage III/IV of the disease. Helicobacter pylori infection was found in 57% of the patients. Eradication treatment was administered to 76% of patients and complete remission (CR) was obtained in 39%. One hundred and ninety patients received at least one other treatment, including 10 already in CR after eradication. Altogether, CR was obtained in 70% of patients and the 5-year overall survival was 79% (95% CI [75-83]). CONCLUSIONS: In comparison to clinical series, in the general population, GMLs are more frequently diagnosed at an advanced stage, their clinical management is heterogeneous, and there is a risk of misdiagnosis and overtreatment. These results highlight the necessity of following currently available guidelines in this field.

Primary intestinal B-cell lymphoma: a prospective multicentre clinical study of 91 cases.

BACKGROUND & AIMS: Clinical presentation, diagnosis and prognosis of different primary intestinal lymphomas have not been well described and compared so far. Our aim was to prospectively analyse a series of consecutive patients presenting different types of B-cell primary intestinal lymphomas. METHODS: Adult patients with primary intestinal lymphoma, collected between 1991 and 2000 within the multicenter national study in France were evaluated and followed up prospectively. Clinical features and treatment outcomes were analyzed and compared among different groups of lymphomas. RESULTS: Among 91 cases of B-cell primary intestinal lymphomas identified, 38 (41%) were diffuse large B cell lymphomas, 34 (37%) mantle cell lymphomas, 12 (13%) follicular lymphomas, 5 (5%) marginal zone MALT-lymphomas, and 2 (3%) Burkitt's lymphomas. A differential diagnosis could be made on the basis of tumour cell morphology and phenotype assessed by immunohistochemistry. Clinical presentation of the different types of lymphomas varied with respect to age, symptoms, circumstances of diagnosis, and stage. Overall survival was the poorest for mantle cell lymphomas while diffuse large B cell lymphomas could be cured if in complete remission after first line treatment. CONCLUSIONS: This study underlines the existence, within the B-cell primary intestinal lymphomas, of several distinct entities with different clinico-pathological features and prognosis, whose identification is important for choosing appropriate therapeutic strategy.

Delineation of the infrequent mosaicism of KRAS mutational status in metastatic colorectal adenocarcinomas.

This study addresses the extent of the heterogeneity of KRAS status, present in a minority of metastatic colorectal carcinomas (mCRCs), on the basis of a thorough analysis of surgical resection specimens. Eighteen patients with mCRC were included. KRAS mutations (exon 2, codons 12 and 13) were determined using PCR and subsequent direct sequencing. This analysis included primary tumours (n=21), synchronous (n=10) and metachronous (n=18) matched metastases, and pelvic recurrence (n=1). Heterogeneity of KRAS status consisted in KRAS mutated in (i) the primary tumour but not in its synchronous metastasis, (ii) the metastasis but not in the primary tumour, (iii) the pelvic recurrence but not in the primary tumour, (iiii) some metastases and not in others from the same patient. Finally, the KRAS status varied among different areas of the same metastatic focus. This study defines the concept of KRAS mosaicism that affects a minority of mCRCs.