94 General Considerations of Lymphomas: Incidence Rates, Etiology, Diagnosis, Staging, and Primary Extranodal Disease Flashcards by Aria Cai

Lymphoma is more common in (men, women)

Men

The disease is more common in men with incidence rates of 23.9 and 16.2 in men and women, respectively.

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The highest reported incidence rate is in

Israel

It is lowest of all in Asia

There are more cases of NHL in Asia than in the United States.

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Incidence of different subtypes of NHL in different countries and continents

Follicular lymphoma: United States and Europe
Extranodal lymphoma: Japan
Burkitt lymphoma: sub-Saharan Africa
T-cell leukemia/lymphoma: southwest Japan, the southeastern United States, northeastern South America, and the Caribbean basin

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An exponential increase in incidence in NHL among men and women occurs with increasing age ECXEPT for:

Lymphoblastic lymphoma: most commonly in children
Burkitt lymphoma : 20- to 64-year-old age group
Primary mediastinal B-cell lymphoma: median age of 35 years

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Occupations with association with lymphoma

Crop farming but not animal farming, women’s hairdressers, cleaners, spray painters, carpenters and textile workers

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Environmental factors and lymphoma

Cigarette smoking: NHL, follicular lymphoma
BMI above 30 kg/m2: DLBCL
Radiation exposure

Surprisingly, some studies have suggested an inverse relationship between an individual’s exposure to ultraviolet light and lymphoma incidence (especially DLBCL)

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Infectious Agent: HTLV1

C-type RNA tumor virus

An acquired retrovirus that is not related to other known animal retroviruses

Also leads to a neurologic disorder called tropical spastic paraparesis

Adult T-cell leukemia/lymphoma (ATLL)

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Infectious Agent: Epstein-Barr Virus

DNA virus in the herpesvirus family

Burkitt lymphoma
Posttransplantation lymphoma
HIV-associated lymphomas (immunodeficiency-related Burkitt lymphoma, primary CNS lymphoma, primary effusion lymphoma, the immunoblastic-plasmacytoid type of DLBCL, and oral cavity plasmablastic lymphoma)
Extranodal NK/T-cell lymphoma, nasal type

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EBV binds to the _______

CD21 antigen (also the receptor for the C3d component of complement) on B lymphocytes

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A three-step process in the development of Burkitt lymphoma has been proposed:

(a) EBV initiates a polyclonal proliferation of B cells;
(b) malaria or other infections further stimulate the proliferating B-cells; and
(c) the transforming B cells incur specific reciprocal translocations of chromosome 8 with chromosome 2, 14, or 22, resulting in a clonal expansion of B lymphocytes

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Infectious Agent: Human Herpesvirus-8

In the homosexual population (mainly in the United States and Europe), HHV-8 is principally transmitted during repeated sexual contacts, whereas in Africa, it is mainly transmitted from mother to child and among siblings.

Saliva seems to play a major role in HHV-8 transmission.

Kaposi sarcoma
Castleman disease
Primary effusion lymphoma
Posttransplantation primary effusion lymphoma

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Infectious Agent: Hepatitis B

DLBCL and follicular lymphoma

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Infectious Agent: Hepatitis C

Associated with immunopathologic reactions, such as cryoglobulinemia, and frequently, clonality of infected B lymphocytes

DLBCL
Marginal zone lymphoma (MZL)
Lymphoplasmacytic lymphoma

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Infectious Agent: Helicobacter pylori

Major cause of peptic ulceration and gastric cancer

Marginal zone mucosa-associated lymphoid tissue (MALT) lymphomas of the stomach

Transformation of a MALT or de novo DLBCL

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Infectious Agent: Chlamydophila psittaci

Ocular adnexal lymphomas

The majority of ocular adnexal lymphomas are extranodal MALT lymphomas

Treated with doxycycline

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Infectious Agent:Campylobacter jejuni

Immunoproliferative disease of the small intestine

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Infectious Agent:Borrelia burgdorferi

B-cell lymphoma of the skin

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TRUE OR FALSE

With the exception of common variable immunodeficiency and the syndromes present in childhood, many of the inherited immune deficiencies are X-chromosome linked; as a result, males are affected more commonly than females.

TRUE

With the exception of common variable immunodeficiency and the syndromes present in childhood, many of the inherited immune deficiencies are X-chromosome linked; as a result, males are affected more commonly than females.

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This germline predisposition syndrome does not have an immunodeficiency phenotype. Rather, it is a nonsyndromic familial cancer syndrome transmitting susceptibility to mutations in p53. The cancers that occur in these families include lymphoma.

Li-Fraumeni syndrome

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There is increased incidence of NHL in patients with rheumatological diseases receiving _______________ inhibitors

Tumor necrosis factor (TNF) inhibitors

The OR is 1.93 (95% CI, 1.16–3.20), and it is possible that the risk is higher with the TNF fusion protein etanercept than it is with inhibitory monoclonal antibodes.

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Autoimmune disoreders: Sjögren syndrome

Parotid gland MZL (1000-fold increased risk)

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Autoimmune disoreders: Hashimoto thyroiditis

Thyroid MALT lymphoma
MZL

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Autoimmune disoreders: Lupus

MZL and DLBCL

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Autoimmune disorders: Autoimmune hemolytic anemia

DLBCL

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Autoimmune disorders: celiac disease, inflammatory bowel disease, and eczema

T-cell lymphoma

# TRUE OR FALSE The presence of such “B symptoms” has unfavorable prognostic significance in HL and NHL

FALSE The presence of such “B symptoms” has **unfavorable prognostic significance in HL**, but these symptoms **do not have independent prognostic significance for NHL**

Recurrence of these symptoms after treatment may herald disease relapse of HL

Fatigue, rash, pruritus, and alcohol-induced pain

# TRUE OR FALSE During the physical examination, all enlarged lymph nodes (>1.5 cm) should be recorded. Involved nodes typically are nontender, firm, and rubbery.

TRUE During the physical examination, all enlarged **lymph nodes (>1.5 cm)** should be recorded. Involved nodes typically are **nontender, firm, and rubbery.**

The single most important test in a lymphoma patient

Biopsy of affected tissue ## Footnote **Excisional lymph node biopsy** of one of the largest nodes available, or **generous incisional biopsy** of an extranodal site.

When is core biopsy indicated

When the only sites of disease involvement are **deep in the thorax or pelvis**

# TRUE OR FALSE Narrow-diameter core biopsies or fine-needle aspiration alone should never be used as the sole method of establishing the initial diagnosis of lymphoma.

TRUE Narrow-diameter core biopsies or fine-needle aspiration alone should never be used as the sole method of establishing the initial diagnosis of lymphoma.

FDG-avid lymphomas

HL, DLBCL, follicular lymphoma, mantle cell lymphoma, Burkitt lymphoma, anaplastic large-cell lymphoma, and most subtypes of PTCL | ABDE(man-tEl)F-HP

Non FDG-avid lymphomas Uses **Contrast-enhanced CT**

MZLs, chronic lymphocytic leukemia/small lymphocytic lymphoma, lymphoplasmacytic lymphoma/Waldenström macroglobulinemia, angioimmunoblastic T-cell lymphoma, mycosis fungoides, and cutaneous B-cell lymphomas

Preferred imaging for disease affecting the brain

**Magnetic resonance imaging** should be performed as well as a **lumbar puncture** ## Footnote PET/CT scans are of limited value for the detection of disease affecting the brain

# TRUE OR FALSE Although marrow aspiration and biopsy have been standard in lymphoma staging in the past, the high sensitivity of PET/CT for marrow involvement has rendered these procedures less essential for patients with any kind of lymphoma who have negative PET/CT imaging of the bones and marrow.

FALSE Although marrow aspiration and biopsy have been standard in lymphoma staging in the past, the high sensitivity of PET/CT for marrow involvement has rendered these procedures **less essential for patients with HL and DLBCL who have negative PET/CT imaging of the bones and marrow**.

Required diagnostics for full staging of other subtypes of lymphoma (aside from HL and DLBCL)

Single 2.5-cm core biopsy with flow cytometry and cytogenetics

Important serum prognostic markers that should be assessed at baseline for most lymphomas

Lactate dehydrogenase and β2-microglobulin

“B symptoms”

* Fevers greater than 38.3 °C * Drenching night sweats * Unexplained weight loss of more than 10% of body mass over 6 months

Bulky disease for HL

A nodal mass of **≥10 cm**, or **greater than one-third of the transthoracic diameter** at any level of thoracic vertebrae

Bulky disease for NHL

There is no consensus on the size of “bulk” for NHL with a suggestion that **6 cm** may be optimal for follicular lymphoma. Sizes between **6 cm and 10 cm** have been advocated to define bulk for diffuse large B-cell lymphoma

Indicate metabolic activity in tumor sites less than in the mediastinal blood pool, signifying **complete metabolic response and CR**

Deauville scores of 1–2 1- No significant FDG uptake in tumor site(s)above background 2- FDG uptake in tumor site(s) less than that in the mediastinal blood pool

Indicate residual abnormal metabolic activity, representing **treatment failure**

Deauville scores of 4 or 5 4- FDG uptake in tumor site(s) moderately higher than in the liver 5- FDG uptake in tumor site(s) markedly higher than that in the liver and/or new FDG-avid lesions likely to be lymphoma

Indicates metabolic activity greater than the mediastinum but less than the liver, it is **indeterminant**

Deauville score of 3 ## Footnote Most patients with HL or DLBCL who have a Deauville score of 3 at the end of treatment have a good outcome, but careful follow-up of such patients is required.

Recommended follow-up for HL and DLBCL

Visits **every 3 months during the first 2 years** are recommended and then reduced to **every 6 months for the next 3 years** and **annually** thereafter

Recommendation for followup imaging

NCCN: Contrast-enhanced CT imaging should not be performed more frequently than **every 6 months for 2 years** after the end of therapy for DLBCL and HL and then be discontinued Lugano IWG guidelines: **Discourage any surveillance CT imaging for asymptomatic patients with HL and DLBCL in CR** at the end of therapy

# TRUE OR FALSE There is general agreement that surveillance PET/CT imaging should not be carried out in asymptomatic patients in CR

TRUE There is general agreement that surveillance PET/CT imaging should not be carried out in asymptomatic patients in CR

Patients with **incurable histologies** require long-term follow-up and are usually observed every _____________

3–6 months

Extranodal involvement that occurs as the only initial evidence of lymphoma after staging procedures

Primary extranodal lymphoma

The histopathology of primary extranodal lymphoma is usually

MZL of MALT or DLBCL

Usual treatment for Primary extranodal lymphoma

* Immuno-chemotherapy multidrug regimen * Rituximab–cyclophosphamide, hydroxydaunorubicin, vincristine (Oncovin), and prednisone (RCHOP) or rituximab and bendamustine

Use of radiotherapy in Primary extranodal lymphoma

Selected cases of extranodal lymphoma, other stage I–II lymphomas and for consolidation therapy of bulky adenopathy

Primary CNS lymphomas are uncommon and almost always are of an aggressive histologic subtype, usually __________

DLBCL ## Footnote There is a relatively high incidence of **MYD88/CD79B** mutations

Treatment for Primary CNS lymphomas

Multiagent chemotherapy is usually given in younger fit patients with drugs that cross the blood–brain barrier such as a combination of **methotrexate, thiotepa, cytarabine, and rituximab (MATRix)** Consolidation is often given with either an **autologous stem cell transplant or whole-brain radiotherapy**

The most common orbital malignancy

Ophthalmic lymphoma

The most frequent subtype of Ophthalmic lymphoma

MZL of MALT

The most common site of ocular lesions

**Periorbital soft tissues**, particularly the conjunctival mucosal surfaces and the area surrounding the lacrimal gland

The therapy for orbital MZL

Radiotherapy

The therapy for orbital DLBCL

R-CHOP chemotherapy either alone or combined with local radiotherapy

Intraocular lymphomas are a rare presentation of lymphoma of the eye. Most cases are

DLBCL ## Footnote Lymphoma arising in the lacrimal sac was usually DLBCL

The diagnosis of intraocular DLBCL is established by

Vitrectomy

The therapy for intraocular DLBCL

High-dose methotrexate-based chemotherapy such as MATRix with or without intrathecal treatment and/or whole-brain and eye radiotherapy

Localized NHL involving the **nasal cavity and/or paranasal sinuses** may be

DLBCL, T-cell lymphoma, or NK/T-cell lymphoma

The predominant site of involvement in **T-cell and NK/T-cell lymphoma**

Nasal cavity

Nasal NK/T lymphomas are typically treated with

L-asparaginase plus radiotherapy

Patients with primary **sinus** lymphoma usually have

DLBCL

The three main types of cutaneous B-cell lymphomas

Primary cutaneous marginal zone B-cell lymphoma Primary cutaneous follicular center lymphoma Primary cutaneous large B-cell lymphoma (leg type)

Cutaneous B-cell lymphomas that are **indolent** types with an excellent prognosis Treated primarily with nonaggressive therapies, including simple excision, glucocorticoid injections, or local radiotherapy.

Primary cutaneous marginal zone B-cell and primary cutaneous follicle center lymphoma

A Cutaneous B-cell lymphoma with diffuse dermal infiltrate of neoplastic B-cells with extension to both the papillary dermis and the subcutaneous fat Aggressive lymphoma that should be treated primarily with aggressive chemotherapy

Primary cutaneous large B-cell lymphoma (leg type)

Primary pulmonary lymphoma histopathology is usually

Marginal zone B-cell lymphoma of the MALT or DLBCL

A rare occurrence pulmonary lymphoma that may follow lung transplantation

Primary endobronchial lymphoma

Most common location of lymphomatous masses in the heart

Right atrium

Most cases of Primary cardiac lymphoma are

B-cell lymphoma

**The most common form of extranodal lymphoma, accounting for one-third of cases**

Gastrointestinal lymphoma

Most commonly involved site of the GI tract

**Stomach** Followed by the **small bowel, ileum, cecum, colon, and rectum** ## Footnote In the bowel, the small intestine, rectum, and colon may be involved, in that order of frequency The intestinal location most often involved is the **ileocecal region** followed by small bowel, large bowel, and multiple intestinal sites.

Diagnosis of Gastrointestinal lymphoma is made by

Endoscopic biopsy

# TRUE OR FALSE GI Lymphomas: MALT lymphoma is common, but DLBCL also may arise de novo or may be found in the background of a MALT lymphoma. If both subtypes of lymphoma are present, the treatment should be directed in treating MALT

FALSE GI Lymphomas: MALT lymphoma is common, but DLBCL also may arise de novo or may be found in the background of a MALT lymphoma. **If both subtypes of lymphoma are present, the treatment should be directed at the large B-cell lymphoma**

The intestinal location most often involved is

Ileocecal region

The most common disease location in the colon

Cecum

# TRUE OR FALSE Primary lymphoma of the testes typically presents as a painless enlargement of the testis in an older man

TRUE Primary lymphoma of the testes typically presents as a **painless** enlargement of the testis in an older man

The histologic type usually of testicular lymphoma is

DLBCL

Relapses of testicular lymphoma usually occurs in

Either the CNS or contralateral testicle

The current international standard of care for testicular lymphoma

Orchiectomy, R-CHOP chemotherapy given every 21 days for six cycles, intrathecal methotrexate, and locoregional radiotherapy to the contralateral testicle

Characteristic of primary renal lymphoma:

Bilateral enlargement of the kidneys without obstruction and other organ or nodal involvement and absence of other causes of renal failure

When primary to the spleen, the lymphoma may be principally confined to the

Red pulp

Histopathology of Primary splenic lymphoma

DLBCL or MZL

Primary bone lymphoma may involve any bone but usually affects the

Long bones

Histopathology of breast lymphoma

DLBCL **B-cell lymphoma 2 (BCL-2) gene** expression is frequently present in the tumor cells.

Breast lymphoma that may be the consequence of **breast implants**, especially those with a “rough” surface

T-cell anaplastic lymphoma of the breast

94 General Considerations of Lymphomas: Incidence Rates, Etiology, Diagnosis, Staging, and Primary Extranodal Disease Flashcards

(88 cards)