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About
Lymphoma > Types of Lymphoma > Extranodal
Last updated: 01/03/2008
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Overview
| Cutaneous | Gastric
| Hepatic
| Conjunctival / Ocular / Eyelid / Orbital |
Osteo (bone) | Oral |
Testis
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Overview
Extranodal
Extra nodal means that the primary area of presentation of the
lymphoma is not in the lymph nodes.
Extranodal can be further categorized as primary or secondary,
with "primary" indicating that the lymphoma first presented
itself in an extranodal site.
Some kinds of extranodal lymphomas are: bone, breast,
eye, oral, skin, etc.
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Extranodal
lymphomas: "Lymphomas
do not only only arise in lymph nodes. Many arise [or present] in non-lymphoid
sites such as the skin and gastrointestinal tract. The
gastrointestinal tract is the most common extra-nodal site for
follicular lymphomas, the most common nodal based type of
lymphoma." - thedoctorsdoctor.com
What is lymphoma?
Briefly, lymphomas result when DNA
damage or changes occurs to an immune cell (a lymphocyte) that
alters the behavior of the cells. The damage to DNA results in the
abnormal production of proteins that prevents the cells from dying when
they should, or causes sustained rapid cell division that produces
more of its kind. These malignant cells
then may accumulate to form tumors that may enlarge the lymph nodes or
spread to other areas of the lymphatic
system, such as the spleen or
bone marrow. Lymphoma can also spread or first appear outside the
lymphatic system -- and is called extranodal
disease.
The underlying pathology (the type of cell, it's stage of
maturation, it's genetic characteristics, etc.) further classifies the
extranodal lymphoma. These properties may be more relevant to the the
likely behavior of the lymphoma and the best types of treatments.
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| Prognostic Factors in Primary Cutaneous B-Cell Lymphoma: The
Italian Study Group for Cutaneous Lymphomas. J Clin Oncol. 2006
Feb 21; PMID:
16492713
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Case analysis: Extranodal
follicular lymphoma: a clinicopathological and genetic
analysis of 15 cases arising at non-cutaneous extranodal
sites. Histopathology. 2004 Mar;44(3):268-76. PMID:
14987231 | Related
articles
The low incidence of t(14;18) and favourable outcome encountered in
Extranodal Follicular Lymphoma ( ENFL) in this study is similar to that previously found for primary cutaneous FL.
These results support the concept of a subtype of FL lacking t(14;18) involving the major breakpoint cluster region, and with a propensity to arise at extranodal sites. Despite a high relapse rate, patients with ENFL are more likely to achieve complete remission and may ultimately have a more favourable long-term prognosis than those with equivalent nodal disease.
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Extranodal nonorbital indolent lymphomas of the head and
neck: relationship between tumor control and radiotherapy.
Int J Radiat Oncol Biol Phys. 2004 Jul 1;59(3):788-95.
PMID:
15183482 | Related
articles
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[1375] Extranodal Follicular Lymphoma - a Retrospective Review
and Comparison with Localized Nodal Follicular Lymphoma. Session
Type: Poster Session 529-I - ASH
2004 | Terms
of Use
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Radiotherapy Suitable for Most Primary Cutaneous B-Cell Lymphomas
cancerpage.com
NEW YORK JAN 02, 2008 (Reuters Health) - Radiotherapy is appropriate for most patients with primary cutaneous B-cell lymphomas
(CBCL), according to a recent Dutch study, but more aggressive treatment is needed in patients presenting with lesions on the leg.
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Common Extranodal Types:
OTHER EXTRANODAL TYPES
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Follicular lymphoma of the salivary
gland: a clinicopathological and molecular study of six
cases. Int J Surg Pathol. 2001 Oct;9(4):287-93. PMID:
12574844
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| BREAST |
 TOPIC
SEARCH: PubMed |
Primary
malignant
Lymphoma of the Breast
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"Primary
lymphoma of the breast is rare, accounting for 1.7-2.2% of extranodal
lymphomas and 0.38-0.7% of non-Hodgkin's lymphomas (NHL)."
[1]
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Primary malignant lymphoma of the breast: clinicopathological
study of nine cases. Leuk Lymphoma. 2004 Feb;45(2):327-30. PMID:
15101719
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| CUTANEOUS
(B cell and T cell Lymphomas presenting in the skin) |
| TOPIC
SEARCH: Cutaneous lymphomas - PubMed: Review |
Treatment
| Rituxan |
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Cutaneous
lymphomas present in the skin, and can have distinct
clinical courses depending on the underlying pathology.
"Ultimately, the utility
of any classification system is to predict behavior of each
cancer and help to direct therapy. The EORTC
classification is one system that does a fine job
of organizing the myriad of names of cutaneous lymphomas.
Briefly, cutaneous lymphomas can be divided into T and B
cell lineage, with T cell lymphomas the most common. Within
these divisions, further distinctions based upon the
clinical behavior can be gleaned." - thedoctorsdoctor.com
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Standard and new treatments in cutaneous B-cell
lymphomas. J Cutan Pathol. 2006 Feb;33 Suppl 1:47-51. Review. PMID:
16412212 | Related
articles
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Lymphoma of the Skin (Nov_28_02) - asheducationbook.org
Joseph M. Connors, Eric D. Hsi and Francine M. Foss Abstract-
Scroll down to Section III for Cutaneous T-Cell Lymphoma
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T-Cell Lymphoma Presenting as Benign Dermatoses -
includes images - aafp.org
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Recommended reading:
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Lymphoma of the Skin Joseph M. Connors, Eric D. Hsi and Francine M. Foss Abstract- asheducationbook.org
"Describes the various ways in which the non-Hodgkin’s lymphomas can involve the skin, how these diseases should be assessed, standard treatments available in 2002, and new directions in research.
The goal of the session is to succinctly review recent developments in lymphoma classification and treatment as they apply to the unique aspects of lymphoma when manifest in the skin. "
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Classifications:
Clinical trials for cutaneous lymphomas - ClinicalTrials.gov
Related
Abstracts
| Cutaneous B-cell lymphoma responds to
rituximab: A report of five cases and a review of the literature.
Leuk Lymphoma. 2006 Sep;47(9):1902-7. PMID:
17065004 | related
articles
We treated five patients, each with either extensive lesions,
severe comorbidities or who refused treatment with radiation
therapy, with rituximab given as a single agent for four weekly
intravenous infusions...
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| Photopheresis Followed by Immunotherapy Improves Responses in Cutaneous
T-Cell Lymphoma - 411cancer.com
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| Intralesional
therapy with anti-CD20 monoclonal antibody rituximab in primary
cutaneous B-cell lymphoma. Arch Dermatol. 2000 Mar;136(3):374-8.
PMID:
10724200
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| Primary cutaneous diffuse large B-cell
lymphoma: a clinicopathologic study of 15 cases.
Am J Clin Pathol. 2002 Apr;117(4):574-80. PMID: 11939732 - PubMed
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| Cutaneous follicle center lymphoma: a
clinicopathologic study of 19 cases.
Mod Pathol. 2001 Sep;14(9):828-35. PMID: 11557777 - PubMed
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| Cutaneous presentation of follicular
lymphomas.
Mod Pathol. 2001 Sep;14(9):913-9. PMID: 11557789 - PubMed
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| Primary
Gastric Lymphomas (PGL) |
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PubMed Topic Search: Primary
Gastric Lymphomas |
Primary
Gastric Lymphoma
Also see MALT lymphomas
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"Primary
gastric lymphoma "account for 7% to 20% of all lymphoma, and the
gastrointestinal tract is the most common extranodal site of lymphoma.
PGL is the most common lymphatic tumor of the GI tract." [1]
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Chemotherapy Preferable to Surgery for Primary Gastric
Lymphoma - Medscape (free login required)
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Primary gastric lymphoma.
World J Gastroenterol. 2004 Jan;10(1):5-11. Review. PMID:
14695759
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Hepatic (Lymphomas presenting in the liver) |
| PubMed
Topic Search: Primary
hepatic lymphomas |
LIVER (HEPATIC)
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"Primary hepatic lymphoma is extremely
rare. Only around 110 cases have been reported in world literature."
[1]
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Primary hepatic lymphoma, single institution experience Year:
2002 - Abstract No: 2643
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Intra-Vascular |
Intra-Vascular
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Intra-Vascular Large B-Cell Lymphoma Revealed by a Nephrotic
Syndrome: A One Year Remission Induced by a High Frequency CHOP
and Rituximab. Leuk Lymphoma. 2004;45(8):1703-1705. PMID:
15370230 | Related
articles
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Ocular
/ Orbital (Lymphoma presenting in the eye) |
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TOPIC SEARCH: PubMed: Secondary
orbital | ocular
adnexal | radiotherapy
for primary orbital
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Ocular/Orbital
adnexa
- Appendages of the eyeball, which includes the eyelids, muscles and
soft tissue.
Eye Cancer Network
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Glossary:
eyecancer.com
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Conjunctival
Lymphoma
"Lymphomas can also occur in the conjunctiva.
These tumors look like salmon-colored patches on the eye and can be a
sign of systemic lymphoma." [1]
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Subconjunctival interferon-alpha for the treatment of
conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma - .biologictherapy.org
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Ocular
Lymphomas:
"Primary ocular lymphoma, an infrequent neoplasm, typically represents a diagnostic challenge masquerading as uveitis or
vitritis." [1]
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Lymphomas
affecting the eyelid (adnexa):
This extranodal area of presentation is not very common but can occur
in b-cell lymphomas, most often MALT. For further
reading, see lymphomainfo.net
TOPIC
SEARCH: PubMed
Adnexa " [Add-Nex-ah] - Appendages.
Structures around the eye that support and surround it (e.g. the eye
lids)." eyecancer.com
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Orbital
Lymphomas:
"Lymphomas of the orbit may arise spontaneously, or may be
associated with the development of systemic lymphoma. These
lesions may cause a painless, unilateral proptosis (#22128) or may
manifest as a salmon patch- like thickening protruding into the
conjunctiva"
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Ocular Adnexal Lymphomas:
TOPIC
SEARCH PubMed
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Research News:
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The common antibiotic doxycycline effectively treats a type of
lymphoma associated with chlamydia infection - biologynews.net
"The authors found that doxycycline treatment caused
lymphoma to regress in both patients who did not test positive for
the disease and those who did. They suggest that doxycycline may
be a useful therapy even in patients where other treatments have
failed, and it is a valid alternative to chemotherapy and
radiation without causing the same toxic side-effects. Patients
treated with doxycycline had a 66% rate of disease-free
survival."
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Topical therapy selective for neoplastic cells - iwant2020.com
Is it an alternative to radiotherapy for Lymphomas?
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Radiotherapy for extranodal, marginal zone, B-cell lymphoma of
mucosa-associated lymphoid tissue originating in the ocular adnexa:
a multiinstitutional, retrospective review of 50 patients. Cancer.
2003 Aug 15;98(4):865-71. PMID: 12910532 - Pubmed
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abstracts
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Curative radiotherapy for primary orbital lymphoma.
Int J Radiat Oncol Biol Phys. 2002 Nov 1;54(3):818. PMID:
12377334 - PubMed
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| Oral
(Lymphomas presenting in the mouth) |
| TOPIC
SEARCH:
PubMed: oral
lymphomas |
Oral
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"Extranodal lymphoma in the
oral cavity may be the first manifestation of lymphoma or may be part
of a disseminated process. Extranodal oral lymphoma of soft
tissue is typically a nontender, poorly circumscribed, compressible,
soft tissue enlargement, sometimes with erythema and ulceration of the
overlying mucosa." [1]
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Malignant Neoplasms of Oral Mucosa - dentalcare.com
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Osteo (Lymphomas presenting in the bone) |
| TOPIC
SEARCH:
PubMed: Bone
lymphoma: 67Ga scintigraphy | Primary
NHL Of bone |
OSTEO
(Bone)
Regarding new involvement in bone: "A lot of
times when follicular lymphoma involves bone, it does so locally, and
it's an extension of lymph nodes growing into bone.
On the other hand, bone involvement in follicular
lymphoma is generally uncommon, particularly if it's diffusely in many
bones. I've got to watch those terms. Under those circumstances I
always have a healthy suspicion about transformation, but I have seen
patients with follicular lymphoma with multiple bone sites who did not
transform. So you really have to try to get a biopsy that tells you
the answer." Dr.
Horning
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"Malignant
lymphoma of bone is rare. In many cases, its diagnosis is delayed
because of unspecific clinical signs and equivocal radiographs."
[1]
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"The data demonstrate that primary lymphoma
involving the bone has an excellent prognosis. Patients with
PBL treated with combined modality versus single modality therapy were
found to have a superior outcome, with a significantly better
survival. Cancer 2006. (c) 2006 American Cancer Society."
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Malignant lymphoma of bone. Arch Orthop Trauma Surg. 2002
Feb;122(1):10-6.
PMID: 11995873 - PubMed
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abstracts
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Excellent long-term
survival in patients with early-stage primary bone lymphoma
treated with doxorubicin-based chemotherapy and local
radiotherapy. Am J Clin Oncol. 2002 Dec;25(6):603-5.
PMID: 12478009 - PubMed
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Importance of
combined-modality therapy for primary bone lymphoma. Leuk
Lymphoma. 2003 Oct;44(10):1837-9. PMID:
14692546 | Relate
abstract
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Primary bone lymphoma: Treatment results and prognostic
factors with long-term follow-up of 82 patients. Cancer. 2006 May
12; PMID:
16700039 | Related
articles
"The data demonstrate that primary lymphoma involving the
bone has an excellent prognosis. Patients with PBL treated with
combined modality versus single modality therapy were found to
have a superior outcome, with a significantly better survival.
Cancer 2006. (c) 2006 American Cancer Society."
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Testis |
Testis
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Primary follicular lymphoma of the testis: excellent outcome
following surgical resection without adjuvant chemotherapy. J
Pediatr Hematol Oncol. 2004 Feb;26(2):104-7. Review. PMID:
14767197 | Related
abstracts
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