Extra-nodal NHL

Epidymiology

  • 25 to 45% of all lymphomas
  • The most common lymphoma is skin lymphoma (majority T-cell histology)
  • For b-cell histology the most common are gastric lymphoma, Waldeyer's ring lymphoma, and brain.

Primary CNS Lymphoma

Head and Neck

Waldeyer' Ring Lymphomas

Treatment

  • Chemoradiation better than RT aloneAviles
  • RT: Involved field and draining nodes to 40 Gy (range 35-50 Gy )
  • Chemo: CHOP like

Outcome[2]

Treatment 5-FFS
Chemoradiation 83%
RT alone 48%
Chemo alone 45%
p-value <.001
  • OS 50-60% for IE and 25-50% for IIE but high relapse.

Paranasal Sinues and Nasal Lymphoma

  • B cell typically in US.
  • CMT is the treatments
  • RFS at 87% for stage I and 70% for stage II.

Salivary Gland Lymphomas

  • Increased risk with Sjogren's syndrome.
  • Myoepithelial sialoadenitis (MESA) characteristic of Sjogren's syndrome is a part of the spectrum of salivary gland
  • MALT are most common type
  • RT for low grade and CMT for intermediate/high grade.

Thyroid Lymphoma

  • Associated with Hashimoto's thyroiditis.

Treatment

  • Surgery and chemoradiation with 35-40 Gy of RT.
  • RT alone is appropriate for localized MALT or follicular lymphomas

Outcome

  • 95% local control for localized MALT or follicular lymphomas

Orbital Lymphomas

Lymphoma of the eye may be extraocular orbital tissues

  • conjunctiva
  • retorbulbar region
  • lacrimal gland

Lymphoma of the eye may be eye itself and is called primary intraocular lymphoma

MALT

  • 2/3 of the tumors of the conjunctiva are MALT
  • 10-15% are bilateral
  • Staging: MR of the orbit as well as the usual staging workup.
  • 20-30% give 95% 5-yr DSS.
  • 20% distant failure.
  • 20-30 Gy should be given
  • Some feel entire orbit should be treated to avoid marginal miss.

Orbial Lymphoma (Low-grade)[3]

  • Treat whole orbit to 25-30 Gy
  • Local Control Rate > 95%

DLBCL

  • DLBCL is most often seen in Retro-orbital area
  • 3-6 cycles of chemotherapy followed by 30-35 Gy in 1.5 to 1.75 Gy fractions RT

Intraocular Lymphoma

Thorax

Breast Lymphomas

Lung Lymphomas

Mediastinal (Thymic) Large Cell Lymphoma

GU

Testicular Lymphomas

Definition

  • Most all are DLBLC

Spread

  • 34% CNS relapse at 10 years
  • Contralateral testicular relapse occurs in 15% at 3 years and 42% at 15 years.

Treatment

  • Surgery (orchiectomy)
  • Scrotal RT (25-30 Gy) reduces contrlateral testis failure to 10%[4]
  • CNS prophylaxis need with IT chemotherapy
  • Systemic chemotherapy

Outcome

  • 5-OS 16-65%
  • median survival 12-24 months

Bladder Lymphomas

Femal Genital Tract Lymphomas

Intestinal Lymphoma

  • is initially resected and
  • if low grade then WART to 20-25Gy and
  • if intermediate grade the chemotherapy.

Cutaneous Lymphoma

Three broad categories

  • CTCLs of indolent clinical behavior (70&):
    • mycosis fungoides
    • primary cutaneous anaplastic large cell lymphoma (c-ALCL)
    • lymphomatoid papulosis
    • rare types (sucutaneous panniculitis-like T-cell lymphoma)
  • CTCL of aggressive clinical behavior (< 10%)
    • Sezary syndrome: leukemic phase of mycosis fungoides
    • peripheral T-cell lymphoma
    • NK cell lymphoma of nasal type
  • Cutaneous B-cell lymphoma (25%)
    • indolent histologies
    • follicular center lymphoma
    • MALT
    • Borrelia burgdorferi has been implicated.

General

  • in contrast to typical situation, clinical behavior of cutaneous lymphoma is governed by location and skin presentation.
  • leg worse outlook than skin surfaces eleswhere.

Treatment

Bone Lymphomas

  • Usually diffuse histiocytic tumor (ie intermediate grade).
  • Nodal metastases in 20% of the cases.
  • Very radiosensitive.
  • LC with RT alone is about 80-90% (100% in series by DTMarshall UF).
  • Treat the entire bone to 40-45Gy initially and then boost an additional 10-15Gy if with radiation alone.
  • 5 year survival is 60%.
  • CMT with doses of 35Gy minimum with RFS of 70% at 5 years.

== Gastric and non-gastric extra nodal MALT ==

Bibliography
1. Leitch HA, Gascoyne RD, Chhanabhai M, Voss NJ, Klasa R, Connors JM. Limited-stage mantle-cell lymphoma.
Ann Oncol. 2003 Oct;14(10):1555-61. PMID: 14504058
2. Aviles A, Delgado S, Ruiz H, de la Torre A, Guzman R, Talavera A. Treatment of non-Hodgkin's lymphoma of Waldeyer's ring: radiotherapy versus chemotherapy versus combined therapy. Eur J Cancer B Oral Oncol. 1996 Jan;32B(1):19-23. PMID: 8729614
3. Pfeffer MR, Rabin T, Tsvang L, Goffman J, Rosen N, Symon Z. Orbital lymphoma: is it necessary to treat the entire orbit? Int J Radiat Oncol Biol Phys. 2004 Oct 1;60(2):527-30. PMID: 15380588
4. Zucca E, Conconi A, Mughal TI, Sarris AH, Seymour JF, Vitolo U, Klasa R, Ozsahin M, Mead GM, Gianni MA, Cortelazzo S, Ferreri AJ, Ambrosetti A, Martelli M, Thieblemont C, Moreno HG, Pinotti G, Martinelli G, Mozzana R, Grisanti S, Provencio M, Balzarotti M, Laveder F, Oltean G, Callea V, Roy P, Cavalli F, Gospodarowicz MK; International Extranodal Lymphoma Study Group. Patterns of outcome and prognostic factors in primary large-cell lymphoma of the testis in a survey by the International Extranodal Lymphoma Study Group. J Clin Oncol. 2003 Jan 1;21(1):20-7. PMID: 12506165
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