Follicular lymphoma classification: Difference between revisions
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==Overview== | ==Overview== | ||
According to the WHO criteria,follicular carcinoma is morphologically graded into low grade follicular lymphoma, high grade follicular lymphoma and diffuse large B cell lymphoma. Pediatric-type follicular lymphoma, primary intestinal follicular lymphoma, other extranodal follicular lymphomas and follicular lymphoma “in situ” (FLIS) are the other variants that are included under follicular lymphoma. | |||
==Classification== | ==Classification== | ||
According to the WHO criteria, the disease is morphologically graded into:<ref name="urlFollicular Lymphomas">{{cite web |url=http://pleiad.umdnj.edu/hemepath/follicular/follicular.html |title=Follicular Lymphomas |work= |accessdate=2008-07-26}}</ref> | According to the WHO criteria, the disease is morphologically graded into:<ref name="urlFollicular Lymphomas">{{cite web |url=http://pleiad.umdnj.edu/hemepath/follicular/follicular.html |title=Follicular Lymphomas |work= |accessdate=2008-07-26}}</ref> | ||
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:* Grades 1 and 2 now as low grade follicular lymphoma | :* Grades 1 and 2 now as low grade follicular lymphoma | ||
:* Grade 3A as high grade follicular lymphoma | :* Grade 3A as high grade follicular lymphoma | ||
:* Grade 3B as | :* Grade 3B as diffuse large B cell lymphoma. | ||
* Follicular lymphoma is graded according to the proportion of large cells (centroblasts). Studies suggest this histologic grading predicts clinical outcome, with more large cells behaving more aggressively and having a higher likelihood of transformation to diffuse large cell lymphoma. When any area of diffuse large-B-cell lymphoma is present in a follicular lymphoma the disease should be reported as diffuse large B-cell lymphoma.<ref name="seer">National Cancer Institute. Surveillance, Epidemiology, and End Results Program 2015. http://seer.cancer.gov</ref>. There was no difference in survival outcomes between patients with Grade 3A and 3B FL, whereas patients with FL3 with more than 50% diffuse component have an inferior survival similar to the survival of those with diffuse large cell lymphoma. FL3B with cytogenetic abnormalities of BCL6 (at 3q27) are thought to be genetically more akin to germinal center type diffuse large B-cell lymphoma than FL1-3A, and is associated with a more aggressive clinical course.Patients with FL3B with BCL2 translocation appear to have a clinical course similar to patients with FL1-3A. Since FL3B is rare, the clinical behavior of FL3 in most studies is based mainly on FL3A cases. | |||
* | *Pediatric-type follicular lymphoma, primary intestinal follicular lymphoma, other extranodal follicular lymphomas and follicular lymphoma “in situ” (FLIS) are the other variants that are included under follicular lymphoma. <ref>{{cite web | title = Non-Hodgkin's Lymphomas (NCCN.org) | url = http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf }}</ref> | ||
::* Pediatric-type follicular lymphoma | |||
:::* Rare variant of follicular lymphoma | |||
:::* Characterized by lack of BCL2 rearrangement and t(14,18) | |||
:::* Better prognosis than adult follicular lymphoma | |||
:::* Often cured with minimal therapy. | |||
::*Primary intestinal follicular lymphoma | |||
:::* Common in the small intestine with the vast majority of cases occurring in the duodenum. | |||
:::* The morphology, immunophenotype, and genetic features are similar to those of nodal follicular lymphoma. | |||
:::* Most patients have clinically indolent and localized disease. | |||
:::* Survival appears to be excellent even without treatment. | |||
::*Other extranodal follicular lymphoma | |||
:::* The morphology, immunophenotype, and genetic features are similar to those of nodal follicular lymphoma. | |||
:::* Patients usually have localized disease and systemic relapses are rare. | |||
::*Follicular Lymphoma “in situ” | |||
:::* The occurrence in the general population appears to be rare,reported in patients with prior follicular lymphoma or concurrent follicular lymphoma (at other sites), as well as in individuals with no known history of follicular lymphoma. | |||
:::* Preservation of the lymph node architecture | |||
::* | :::* Incidental finding of focal strongly positive staining for BCL2 (restricted to germinal centers) and CD10 in the involved follicles | ||
:::* The detection of t(14;18) by FISH. | |||
: | |||
::* | |||
Other extranodal follicular lymphomas occur in almost any extranodal site. Patients usually have localized extranodal disease and systemic relapses are rare. Testicular follicular lymphoma are reported with increased frequency in children, but also are reported in adults. | Other extranodal follicular lymphomas occur in almost any extranodal site. Patients usually have localized extranodal disease and systemic relapses are rare. Testicular follicular lymphoma are reported with increased frequency in children, but also are reported in adults. | ||
Revision as of 15:34, 24 August 2015
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Risk calculators and risk factors for Follicular lymphoma classification |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
According to the WHO criteria,follicular carcinoma is morphologically graded into low grade follicular lymphoma, high grade follicular lymphoma and diffuse large B cell lymphoma. Pediatric-type follicular lymphoma, primary intestinal follicular lymphoma, other extranodal follicular lymphomas and follicular lymphoma “in situ” (FLIS) are the other variants that are included under follicular lymphoma.
Classification
According to the WHO criteria, the disease is morphologically graded into:[1]
- Grade 1 (<5 centroblasts per high-power field (hpf))
- Grade 2 (6–15 centroblasts/hpf)
- Grade 3 (>15 centroblasts/hpf)
- Grade 3A (centrocytes still present)
- Grade 3B (the follicles consist almost entirely of centroblasts)
- The WHO 2008 update classifies
- Grades 1 and 2 now as low grade follicular lymphoma
- Grade 3A as high grade follicular lymphoma
- Grade 3B as diffuse large B cell lymphoma.
- Follicular lymphoma is graded according to the proportion of large cells (centroblasts). Studies suggest this histologic grading predicts clinical outcome, with more large cells behaving more aggressively and having a higher likelihood of transformation to diffuse large cell lymphoma. When any area of diffuse large-B-cell lymphoma is present in a follicular lymphoma the disease should be reported as diffuse large B-cell lymphoma.[2]. There was no difference in survival outcomes between patients with Grade 3A and 3B FL, whereas patients with FL3 with more than 50% diffuse component have an inferior survival similar to the survival of those with diffuse large cell lymphoma. FL3B with cytogenetic abnormalities of BCL6 (at 3q27) are thought to be genetically more akin to germinal center type diffuse large B-cell lymphoma than FL1-3A, and is associated with a more aggressive clinical course.Patients with FL3B with BCL2 translocation appear to have a clinical course similar to patients with FL1-3A. Since FL3B is rare, the clinical behavior of FL3 in most studies is based mainly on FL3A cases.
- Pediatric-type follicular lymphoma, primary intestinal follicular lymphoma, other extranodal follicular lymphomas and follicular lymphoma “in situ” (FLIS) are the other variants that are included under follicular lymphoma. [3]
- Pediatric-type follicular lymphoma
- Rare variant of follicular lymphoma
- Characterized by lack of BCL2 rearrangement and t(14,18)
- Better prognosis than adult follicular lymphoma
- Often cured with minimal therapy.
- Primary intestinal follicular lymphoma
- Common in the small intestine with the vast majority of cases occurring in the duodenum.
- The morphology, immunophenotype, and genetic features are similar to those of nodal follicular lymphoma.
- Most patients have clinically indolent and localized disease.
- Survival appears to be excellent even without treatment.
- Other extranodal follicular lymphoma
- The morphology, immunophenotype, and genetic features are similar to those of nodal follicular lymphoma.
- Patients usually have localized disease and systemic relapses are rare.
- Follicular Lymphoma “in situ”
- The occurrence in the general population appears to be rare,reported in patients with prior follicular lymphoma or concurrent follicular lymphoma (at other sites), as well as in individuals with no known history of follicular lymphoma.
- Preservation of the lymph node architecture
- Incidental finding of focal strongly positive staining for BCL2 (restricted to germinal centers) and CD10 in the involved follicles
- The detection of t(14;18) by FISH.
Other extranodal follicular lymphomas occur in almost any extranodal site. Patients usually have localized extranodal disease and systemic relapses are rare. Testicular follicular lymphoma are reported with increased frequency in children, but also are reported in adults.
References
- ↑ "Follicular Lymphomas". Retrieved 2008-07-26.
- ↑ National Cancer Institute. Surveillance, Epidemiology, and End Results Program 2015. http://seer.cancer.gov
- ↑ "Non-Hodgkin's Lymphomas (NCCN.org)" (PDF).