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Types of Lymphoma >
Extranodal
Last updated:
06/06/2013
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TOPICS
Overview
| Cutaneous |
Gastric
| Hepatic
| Eye: Conjunctival / Ocular / Eyelid / Orbital |
Osteo (bone) | Oral |
Testis
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Overview
Extranodal
Extranodal 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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In the News
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News Medical: FDA grants Fast Track designation
to Elorac's naloxone topical lotion
http://bit.ly/ZleuKY
"topical lotion for
the relief of pruritus in patients with cutaneous
T-cell lymphoma (CTCL). There are currently no
approved therapeutic treatment options available to
patients and their physicians for pruritus in CTCL."
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Analysis of 581 patients with primary intestinal
NHL from the Consortium for Improving Survival of
Lymphoma
http://bit.ly/pzqV1V
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Extranodal
lymphomas: "Lymphomas
do not only only arise in lymph nodes or other areas of the lymphatic
system. 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? See
Lymphoma simplified
Background Resources
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2013: Cutaneous B-cell lymphomas: 2013 update on
diagnosis, risk-stratification, and management.
http://www.ncbi.nlm.nih.gov/pubmed/23379008
Approximately one-fourth of
cutaneous lymphomas are B-cell derived and are generally
classified into three distinct subgroups: primary cutaneous
follicle-center lymphoma (PCFCL), primary cutaneous marginal
zone lymphoma (PCMZL), and primary cutaneous diffuse large
B-cell lymphoma, leg type (PCLBCL, LT).
Both PCFCL and PCMZL are indolent lymphomas that
infrequently disseminate to extracutaneous sites and are
associated with an excellent long-term prognosis. In
contrast, PCLBCL, LT is an aggressive lymphoma with an
inferior prognosis. |
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Extranodal lymphomas
http://bit.ly/a6AhWt
Emanuele Zucca
and Franco Cavalli |
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Questions for your doctor PAL
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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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Radiotherapy for:
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2003 by American Society of Clinical Oncology
Localized Mucosa-Associated Lymphoid Tissue Lymphoma
Treated With Radiation Therapy Has Excellent
Clinical Outcome
http://jco.ascopubs.org/content/21/22/4157.full
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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:
UNCOMMON 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
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Primary
malignant
Lymphoma of the Breast
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 TOPIC
SEARCH: PubMed
"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- and T-cell Lymphomas presenting in the skin)
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Return to top |
TOPIC
SEARCH: Cutaneous lymphomas
PubMed:
Review |
Treatment
| Rituxan
Classifications:
Cutaneous
lymphomas present in the skin, and can have distinct
clinical courses depending on the underlying pathology.
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
Overview: Lymphoma of the Skin
Joseph M. Connors, Eric D. Hsi and Francine M. Foss
asheducationbook.hematologylibrary.org
Published in 2002 … but still seems relevant and informative
today.
Cutaneous Lymphomas in Alphabetical Order
Cutaneous
T-cell lymphoma (CTCL)
B-cell cutaneous
lymphomas
Cutaneous
pseudolymphoma (C-PSL), from Discussion:
"also called lymphoid infiltrates of the skin
mimicking lymphoma, is defined as reactive
polyclonal benign lymphoproliferative process
predominantly composed of either B-cells or T-cells,
localized or disseminated.
It heals spontaneously after cessation of the
causative factor (e.g. drugs) or after
non-aggressive treatment. It has many synonyms such
as lymphocytoma cutis and lymphadenosis benigna
cutis.
According to the dominant patterns, PSL were
classified into B-cell PSL (B-PSL) and T-cell PSL
(T-PSL).
However, there were no significant difference
between B-PSL and T-PSL. C-PSL affects all age group
with the predilection of Borrelia-induced B-PSL in
children and young adults, while drug induced T-PSL
more frequently are seen in adults.
Even though Borrelia-induced PSL may be precursor
for B-cell neoplasms of the skin, in genera, C-PSLs
are self-regressing and do not affect survival.
The etiology may involve microbial, physical,
chemical, Borrelia burgdorferi infection, insect
bite, tattoo, and drugs
Clinically, several variants of C-PSL exist. ....
In the author’s lymphoma experience the author have
experienced several C-PSL, but have not published
them because the author had been once concentrated
on hepatobiliary pathology. Herein reported is a
very interesting case of ordinary C-PSL with main
points of differential pathological diagnosis."
Int J Clin Exp Pathol. 2013; 6(5): 966–972.
Cutaneous pseudolymphoma: a case report with an
immunohistochemical study
ncbi.nlm.nih.gov
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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NCCN: Cutaneous B-Cell Lymphoma Guidelines Debut medpagetoday.com/
Primary cutaneous B-cell lymphoma is unlikely to
reduce a person's life expectancy, but even with definitive
therapy, the disease is likely to recur, doctors said in unveiling
new guidelines for treatment of the disease.
About 1,000 to 1,500 cases of cutaneous B-cell lymphoma occur each
year in the U.S., said Steven M. Horwitz, M.D., of Memorial-Sloan
Kettering Cancer Center in New York and a member of the guideline
panel. But because the disease is rarely fatal, there are tens of
thousands of people living with it.
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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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Clinical trials for cutaneous lymphomas
Related
Abstracts
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Pilot Study: Zevalin for cutaneous lymphoma?
informahealthcare.com/
"Ten patients, all but one, with relapsed PCBCL were included and
treated with rituximab (250 mg m−2/body surface) on
days 1 and 8 followed by a single dose of 90Y IT (11–15
MBq kg−1). The overall response rate was 100%. The
complete response rate was 100%. The median time to relapse was 12
months. Ongoing remissions were achieved in four patients (median
follow-up 19 months). " |
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Rituximab monotherapy for primary cutaneous B-cell lymphoma:
response and follow-up in 16 patients
ingentaconnect.com
On the basis of our results, single-agent
treatment with Rituxan appears to be feasible and safe and
results in a high rate of durable remissions. Judging from our
data, it appears to be an attractive treatment option and should
be directly compared with local radiotherapy.
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Report: Zevalin® Effective for Treatment of Cutaneous B-Cell Lymphoma
cancerconsultants.com
100% CR rate in small study (10 patients);
40% (4 of 10 pts) remain in complete remission at 19 months.
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NEW: Phase 2 Data
for Romidepsin Showing Durable Response in Refractory CTCL businesswire.com
"Romidepsin is a novel, cyclic peptide, pan-HDAC inhibitor
under investigation for hematologic malignancies"
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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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Primary
Gastric Lymphoma
Also see MALT lymphomas
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PubMed Topic Search: Primary
Gastric Lymphomas
"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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2003 by American Society of Clinical Oncology
Localized Mucosa-Associated Lymphoid Tissue Lymphoma Treated
With Radiation Therapy Has Excellent Clinical Outcome
jco.ascopubs.org
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Hepatic
(Lymphomas presenting in the liver)
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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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2003 by American Society of Clinical Oncology
Localized Mucosa-Associated Lymphoid Tissue Lymphoma Treated
With Radiation Therapy Has Excellent Clinical Outcome
jco.ascopubs.org
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Intra-Vascular Lymphoma
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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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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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TOPIC SEARCH: PubMed:
Secondary
orbital |
ocular
adnexal |
radiotherapy
for primary orbital
Types:
Ocular / Ocular Adnexa (eyelid) / Intraocular | Orbital
| Conjunctival
"Depending on the site of involvement,
ocular lymphoma can be
either intraocular or orbital and adnexal."
Important: Ocular and orbital lymphomas
can be effectively treated. However, how either is best treated can
vary significantly by the area effected, the grade, and the type.
Since ocular lymphoma are uncommon, we feel it's very important to
consult an expert experienced in these neoplasms
for a second opinion at diagnosis and certainly prior to receiving
therapy. Radiotherapy:
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2003 by American Society of Clinical Oncology
Localized Mucosa-Associated Lymphoid Tissue Lymphoma Treated
With Radiation Therapy Has Excellent Clinical Outcome
jco.ascopubs.org
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Primary Radiation Therapy in Patients With Localized Orbital
MALT Lymphoma
http://bit.ly/9YUyKD
Forty-three patients (93%)
achieved complete remission (CR), and three patients (7%)
achieved partial remission (PR). Five-year relapse-free
survival, cause-specific survival, and overall survival were
93%, 100%, and 100%, respectively.
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Curative radiotherapy for primary orbital lymphoma.
Int J Radiat Oncol Biol Phys. 2002 Nov 1;54(3):818-23. Review. PMID: 12377334
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Radiotherapy for extranodal, marginal zone, B-cell lymphoma of
mucosa-associated lymphoid tissue originating in the ocular adnexa:
a multi-institutional, retrospective review of 50 patients.
Cancer.
2003 Aug 15;98(4):865-71. PMID: 12910532
| Related
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
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Ocular
Lymphomas:
"Depending on the site of involvement, ocular lymphoma
can be either intraocular or orbital and adnexal." [1]
"Primary ocular lymphoma, an infrequent neoplasm, typically represents a diagnostic challenge masquerading as uveitis or
vitritis." [2]
Ocular
lymphoma - overview
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About
emedicine.medscape.com
"Orbital
and adnexal lymphoma is associated with systemic lymphoma in 30-35%
of cases. Hence, all patients with ocular lymphoma should have a
complete workup to rule out systemic lymphoma. "
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Intra-ocular
lymphoma:
A rare malignant form of
eye
cancer. Intraocular lymphoma may affect the eye secondarily from a
metastasis from a non-ocular tumor or may arise within the eye primarily
(primary intraocular lymphoma).
Primary intraocular
Lymphoma (PIOL) is a subset of primary central nervous system lymphoma (PCNSL).
PCNSL (and PIOL) are most commonly a diffuse large B-cell
immunohistologic subtype of non-Hodgkin's lymphoma according to the
World
Health Organization (WHO) classification of lymphomas.
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Primary Intraocular lymphoma (PIOL):
Most elusive intraocular
tumor to diagnose.
moffitt.org
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Ocular
adnexa
Lymphomas -
affecting the eyelid
TOPIC
SEARCH: PubMed
This extranodal area of presentation is not very common but can occur
in b-cell lymphomas, most often MALT.
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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Clinical features associated with survival of patients with lymphoma
of the ocular adnexa
http://bit.ly/6IoMsP |
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The Role of Infectious Agents in the Etiology of Ocular Adnexal
Neoplasia (full text)
ncbi.nlm.nih.gov |
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Linked to Chlamydia psittaci Infection (an infection
common to pet birds and transmitted to humans) Related
PubMed abstracts |
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Blood. 2009 July 16; 114(3): 501–510:
Extranodal marginal zone lymphoma of the ocular adnexa
"Besides radiotherapy and
chemotherapy, a variety of new treatment options have emerged in
the management of patients with ocular adnexal MALT lymphoma,
especially monoclonal antibody therapy and antibiotic therapy
against Chlamydia psittaci" |
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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 or may
manifest as a salmon patch- like thickening protruding into the
conjunctiva"
"Orbital lymphoma refers to a lymphoma
occurring in the conjunctiva, lacrimal gland, eyelid and ocular
musculature.
Primary non-Hodgkin's lymphoma of the orbit is a rare presentation,
representing 8-10% of extranodal NHL and only 1% of all NHL. Generally,
it has an indolent course.
Orbital lymphoma in contrast to ocular
lymphoma is rarely associated with primary central nervous lymphoma."
4
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Linked to Chlamydia psittaci Infection (an infection
common to
pet birds and transmitted to humans) -
ASCO
2003 (2273)
| Related
PubMed abstracts
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Orbital lymphoma: Role of radiation
ncbi.nlm.nih.gov
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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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Addition of a Short Course of Chemotherapy Did Not Improve
Outcome in Patients with Localized Marginal B-Cell Lymphoma of the
Orbit. Oncology. 2006 Jun 7;70(3):173-176
PMID:
16763405
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Primary radiotherapy for localized orbital MALT lymphoma.
Int J Radiat Oncol Biol Phys. 2002 Mar 1;52(3):657-63.
PMID:
11849787
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H. pylori molecular signature in conjunctival
mucosa-associated lymphoid tissue (MALT) lymphoma. Histol
Histopathol. 2004 Oct;19(4):1219-26.
PMID:
15375765
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Outcome and prognostic factors in orbital lymphoma: a Rare Cancer
Network study on 90 consecutive patients treated with radiotherapy.
Int J Radiat Oncol Biol Phys. 2003 Mar 15;55(4):892-8.
PMID: 12605966
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Indian J Ophthalmol. 2009:
Orbital lymphoma: Role of radiation
Available data support the acceptance
of radiotherapy as a standard therapeutic option in patients with
low to intermediate grade orbital lymphoma. Toxicity of radiotherapy
is mild if delivered precisely.
________________________
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." [Eyecancer.com]
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Subconjunctival interferon-alpha for the treatment of
conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma
biologictherapy.org
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Extranodal marginal zone lymphoma of the
ocular adnexa
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Blood. 2009 July 16; 114(3): 501–510.
Review Article: Extranodal marginal zone lymphoma of the ocular
adnexa
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2713468/
"Various treatment modalities are
available for the management of patients with OAML, including
surgical resection, radiotherapy, single-agent or combination
chemotherapy, and immunotherapy with monoclonal antibodies.
However, no prospective clinical trials have been conducted to
evaluate these therapeutic options or define the optimal
treatment approach for these patients. Recently, antichlamydial
antibiotic therapy was also proposed as a novel treatment
option." |
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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Oral
(Lymphomas presenting in the mouth)
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Oral
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TOPIC
SEARCH:
PubMed: oral
lymphomas
"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)
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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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TOPIC
SEARCH:
PubMed:
Bone
lymphoma: 67Ga scintigraphy | Primary
NHL Of bone
"Malignant
lymphoma of bone is rare. In many cases, its diagnosis is delayed
because of unspecific clinical signs and equivocal radiographs."
[1]
"
"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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Related
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
-
Importance of
combined-modality therapy for primary bone lymphoma.
Leuk
Lymphoma. 2003 Oct;44(10):1837-9.
PMID:
14692546 |
Relate
abstracts
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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
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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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