Recent advances in intestinal lymphomas

Pg, Foukas; Leval, Laurence de

doi:10.1111/his.12596

Cited by 61 publications

(75 citation statements)

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“…The three major entities comprising the spectrum of NK malignancy are NK-cell enteropathy, extranodal NK/T-cell lymphoma (ENTL)-nasal type and aggressive NK-cell lymphoma 16 17. NK-cell enteropathy, a clinically indolent disorder characterised by a self-limiting lymphoproliferation in the GI tract, is inconsistent with the morphology and aggressive features of the cells characterised in this case 15. NK-cell enteropathies are routinely identified in the absence of EBV infection 17.…”

Section: Discussionmentioning

confidence: 70%

“…All primary GI lymphomas are non-Hodgkin lymphomas and, of these, nearly half are diffuse large B-cell lymphomas with marginal zone, mantle cell and follicular lymphoma frequently identified as well 10 13. The most commonly identified T-cell phenotype is the enteropathy-associated T-cell lymphoma, a rare but well-characterised disease of the small intestine 14 15…”

Section: Discussionmentioning

confidence: 99%

“…NK-cell enteropathies are routinely identified in the absence of EBV infection 17. The ENTL-nasal type, however, is an aggressive disease that, as the name suggests, is mostly localised to the upper aerodigestive tract 15 18 19. These lymphomas, by definition, require EBV infection as a pre-requisite for diagnosis, and are very occasionally found elsewhere in the GI system 20–22.…”

Section: Discussionmentioning

confidence: 99%

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The first report of a previously undescribed EBV-negative NK-cell lymphoma of the GI tract presenting as chronic diarrhoea with eosinophilia

et al. 2015

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A 74-year-old man presented with a 2-month history of watery diarrhoea. His complete blood count showed lymphopaenia and marked eosinophilia. Investigations for common infectious causes including Clostridium difficile toxin, stool culture, ova and parasites were negative. Endoscopy revealed extensive colitis and a CT of the abdomen identified numerous large abdominal lymph nodes suspicious for lymphoma. Multiple tissue samples were obtained; colon, mesenteric lymph node and bone marrow biopsy, as well as pleural fluid from a rapidly developing effusion, confirmed the presence of metastatic lymphoma with an immunophenotype most consistent with an aggressive variant of Epstein-Barr virus (EBV)-negative natural killer (NK)-cell lymphoma. The patient's clinical condition rapidly deteriorated and he died shortly following diagnosis. To the best of our knowledge, this is the first case report of a primary gastrointestinal EBV-negative NK-cell lymphoma, and its clinical presentation highlights the importance of a broad differential in the management of chronic diarrhoea.

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Section: Discussionmentioning

confidence: 70%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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The first report of a previously undescribed EBV-negative NK-cell lymphoma of the GI tract presenting as chronic diarrhoea with eosinophilia

et al. 2015

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“…As reported, the most common subtype of primary intestinal lymphoma is diffuse large B-cell lymphoma and extra-nodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) [10–12]. Among the T-cell lymphomas of GI tract, peripheral T-cell lymphomas (PTCL), enteropathy-associated T-cell lymphoma (EATL) and NK/T-cell lymphoma (NK/TL) are the common subtypes [12, 13]. In a retrospective Korean study, 42 primary GI T-cell lymphomas were assessed out of which 17 cases are located in small intestine.…”

Section: Discussionmentioning

confidence: 99%

Primary rare anaplastic large cell lymphoma, ALK positive in small intestine: case report and review of the literature

Cao

Liu

et al. 2016

Diagn Pathol

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BackgroundPrimary anaplastic large cell lymphoma, ALK positive in small intestine is clinically rare and the clinical, radiological and pathological information are generally not available. Here, we report a case of 32-year-old male with ALK positive anaplastic large cell lymphoma at the junction of jejunum and ileum, and highlight the clinicopathological features and the differential diagnosis of this type lymphoma.Case presentationThe patient presented with right middle abdominal mass for 1 month with sporadic pain. Computed tomography (CT) showed a mass measured 8.5 × 7.4 × 4 cm at the junction of jejunum and ileum. The diagnosis was made after pathological examination of the excised tissue by enterectomy. Grossly, the mass was located predominately in intestinal wall with grayish appearance and blurry boundary. Microscopically, almost all layers of the intestinal wall were infiltrated by pleomorphic tumor cells with diffuse and cohesive growth pattern. The neoplastic cells were mainly medium to large size with moderate basophilic cytoplasm. Most of them had hyperchromatic nuclei and prominent nucleoli. “Hallmark” cells were easily detected. Immunohistochemically, tumor cells are characterized by CD30, ALK, CD5, TIA-1, Granzyme B, EMA positive staining, and CD2, CD3, CD7, CD4, CD8, CD20, CD79a negative staining. The Epstein-Barr virus encoded RNAs (EBERs) genome was also negative. A diagnosis as primary small intestinal ALK positive anaplastic large cell lymphoma was finally made. The patient received CHOP chemotherapy and is alive till now without recurrence 5 months after enterectomy.ConclusionsPrimary small intestinal ALK positive anaplastic large cell lymphoma is rare. The accurate diagnosis should be based on combined consideration of clinical characteristics, CT image and pathological features, and should be distinguished from other lymphomas or solid tumors in small intestine.

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“…Table compares the main epidemiological, clinical, histolopathological, and genetic features of EATL and MEITL , …”

Section: Intestinal T‐cell Lymphomasmentioning

confidence: 99%

Breast implant‐associated anaplastic large cell lymphoma and other rare T‐cell lymphomas

Leval

2019

Hematological Oncology

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Peripheral T-cell lymphomas (PTCLs) account for less than 15% of all non-Hodgkin lymphomas worldwide. In the current WHO classification, more than 25 types of T-cell neoplasms are listed (Table 1). 1 Considering the noncutaneous T-cell lymphomas, despite substantial geographic variations in the incidence and the subtype prevalence, a few entities-essentially those presenting as nodal diseases-are relatively common and account for the majority of the cases, while extranodal entities are far less common or distinctively rare. Here, we will review the clinico-pathological features of breast implant (BI)-associated anaplastic large cell lymphoma and intestinal T-cell neoplasms, with a focus on the recent advances in the understanding of their molecular biology and pathogenesis. 2 | BREAST IMPLANT-ASSOCIATED ANAPLASTIC LARGE CELL LYMPHOMA Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a very rare form of T-cell lymphoma that arises in association with BIs. BIA-ALCL was introduced as a new provisional disease entity in the recently revised WHO classification of lymphoid malignancies, distinct from the other types of ALCLs already recognized (anaplastic lymphoma kinase [ALK]-positive and ALK-negative ALCL and primary cutaneous ALCL). 1 2.1 | Epidemiology Since the first case was described in 1997, more than 500 cases of BIA-ALCL have been reported in the literature worldwide. An increasing number of dioagnoses were recorded over the recent years reflecting a substantial increase in recognition and awareness of the disease. Notwithstanding these trends, the incidence of BIA-ALCL remains very low. Primary breast lymphomas represent 1% to 2% of all non-Hodgkin lymphomas, with most cases diagnosed as diffuse large B-cell lymphomas, follicular or marginal zone B-cell lymphomas. Among more than 400 breast lymphomas accumulated between 2010 and 2018 in the French lymphoma network, BIA-ALCL represented 10% of the diagnoses. 2Taking into consideration the very large number of women with BIs over the world, the individual risk of BIA-ALCL is very small. A recent population-based case-control study based on the pathology registry in the Netherlands established that BI is associated with a markedly increased risk (400 times) of developing ALCL, but the absolute cumulative risk is estimated to one case for every 50 000 women with BI by the age of 50 years and one per 7000 by the age of 75. 2 In another study based on US cases, the lifetime risk of BIA-ALCL in women with textured implants was estimated to be 1 in 30 000. 3 | Clinical featuresThe mean age at diagnosis is in the 50s. Approximately 60% of women who develop BIA-ALCL had implants for cosmetic reasons, and 40% had implants for reconstructive purposes after surgery for breast cancer. BIA-ALCL has also been reported in three transgender male to female individuals. The median time interval between implant insertion and lymphoma diagnosis is 8 to 9 years in different studies, but wide variations are observed. There is no significant difference in risk o...

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Recent advances in intestinal lymphomas

Cited by 61 publications

References 104 publications

The first report of a previously undescribed EBV-negative NK-cell lymphoma of the GI tract presenting as chronic diarrhoea with eosinophilia

The first report of a previously undescribed EBV-negative NK-cell lymphoma of the GI tract presenting as chronic diarrhoea with eosinophilia

Primary rare anaplastic large cell lymphoma, ALK positive in small intestine: case report and review of the literature

Breast implant‐associated anaplastic large cell lymphoma and other rare T‐cell lymphomas

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