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Common Extranodal Types:
UNCOMMON EXTRANODAL TYPES
In the News
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Primary malignant
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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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Images
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The ASCO Post, 2013: FDA Approves Mechlorethamine Gel for Cutaneous T-Cell Lymphoma http://bit.ly/18Ultxa This gel is the first and only FDA-approved topical formulation of mechlorethamine, which is commonly known as nitrogen mustard. The gel is applied topically once a day and dries on the skin. Mechlorethamine was approved previously for intravenous treatment of mycosis fungoides, the most common type of CTCL. Topical mechlorethamine preparations are currently recommended for the treatment of early-stage CTCL. Prior to approval of the gel, there were no FDA-approved topical mechlorethamine products; only nonstandardized, pharmacy-compounded petroleum ointment or aqueous-based topical preparations were available. |
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 T-cell lymphoma (CTCL)
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See Comparing types Also see T-cell Lymphomas PAL |
B-cell cutaneous lymphomas
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Follicular Center Cell Lymphomas of the Skin |
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Large B-cell lymphoma of the leg |
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T-Cell Rich B-Cell Lymphoma of the Skin |
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
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Lymphoma of the Skin Joseph M. Connors, Eric D. Hsi and Francine M. Foss Abstract- asheducationbook.org
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NCCN: Cutaneous B-Cell Lymphoma Guidelines Debut medpagetoday.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
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T-Cell Lymphoma Presenting as Benign Dermatoses - includes images - aafp.org
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ClinicalTrials.gov |
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Pilot Study: Zevalin for cutaneous lymphoma? informahealthcare.com/
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Rituximab monotherapy for primary cutaneous B-cell lymphoma: response and follow-up in 16 patients ingentaconnect.com
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Report: Zevalin® Effective for Treatment of Cutaneous B-Cell Lymphoma cancerconsultants.com
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NEW: Phase 2 Data for Romidepsin Showing Durable Response in Refractory CTCL businesswire.com
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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
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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.
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Cutaneous presentation of follicular lymphomas.
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Also see
MALT lymphomas
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]
"Primary hepatic lymphoma is extremely rare. Only around 110 cases have been reported in world literature." [1]
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About Intravascular large B-cell lymphoma |
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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 |
adnexa - Appendages of the eyeball, which includes the eyelids, muscles and soft tissue.
Eye Cancer Network -
Glossary:
eyecancer.com
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.
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2003 by American Society of Clinical Oncology
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Primary Radiation Therapy in Patients With Localized Orbital MALT Lymphoma http://bit.ly/9YUyKD May 2010
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Curative radiotherapy for primary orbital lymphoma.
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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.
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"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]
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About emedicine.medscape.com
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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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Ocul Immunol Inflamm. 2009
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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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About related abstracts |
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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:
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"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
"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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Blood. 2009 July 16; 114(3): 501–510.
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The common antibiotic doxycycline effectively treats a type of lymphoma associated with chlamydia infection biologynews.net
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Topical therapy selective for neoplastic cells - iwant2020.com
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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]
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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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 |