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Lymphoma

Authors: Feriyl Bhaijee, M.D., Israh Akhtar, M.D.

Last author update: 4 November 2013

Last staff update: 11 May 2023

Copyright: 2003-2024, PathologyOutlines.com, Inc.

PubMed Search: Lymphoma[TI] esophagus[TI]

Table of Contents

Cite this page: Bhaijee F, Akhtar I. Lymphoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/esophaguslymphoma.html. Accessed December 26th, 2024.

Definition / general

Lymphomas comprise a diverse group of clonal (malignant) lymphoproliferative disorders, classified by WHO based on lymphocytic origin (lymphoma)
- Hodgkin lymphomas arise from precursor B cells (Reed-Sternberg cells)
  - Classic Hodgkin lymphoma - 95%
  - Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) - 5%
- Non-Hodgkin lymphomas arise from monoclonal expansion of malignant B or T cells
  - B cell lymphomas
    - B cell acute lymphoblastic lymphoma (ALL)
    - Chronic lymphocytic lymphoma / small lymphocytic leukemia (CLL / SLL)
    - Mantle cell lymphoma
    - Follicular lymphoma
    - Marginal zone B cell lymphoma
    - Extranodal MALT type
    - Hairy cell leukemia
    - Plasmacytoma / plasma cell myeloma
    - Diffuse large B cell lymphoma (DLBCL)
    - Burkitt lymphoma
  - T / NK cell lymphomas
    - T cell acute lymphoblastic lymphoma (ALL)
    - T cell CLL
    - Mycosis fungoides / Sézary syndrome
    - Peripheral T cell lymphoma
    - Angioimmunoblastic T cell lymphoma
    - Enteropathy associated intestinal T cell lymphoma
    - Hepatosplenic T cell lymphoma
    - Anaplastic large cell lymphoma (ALCL)
    - Extranodal NK / T cell lymphoma, nasal type

Terminology

Primary esophageal lymphoma
- Arises de novo in esophageal mucosa associated lymphoid tissue (MALT)
Secondary esophageal lymphoma
- Spreads to esophagus by either direct extension or metastasis

Epidemiology

GI tract is most common site of extranodal non-Hodgkin lymphoma (4 - 20%)
- Esophagus is least common site of GI involvement
  - < 0.2% of all extranodal non-Hodgkin lymphoma
Lymphomas comprise < 1% of all esophageal cancers
- Secondary lymphoma (contiguous spread from mediastinal or cervical lymph nodes) >> primary esophageal disease
- Most originate from mature B cells
  - Non-Hodgkin lymphoma >> Hodgkin lymphoma
  - Mostly DLBCL and extranodal marginal zone B cell lymphoma, MALT type
Males > females
Wide age range: 17 - 86 years but usually middle age to older adults
- Younger age of onset in HIV+ patients (40 vs. 60 years)

Sites

Primary lymphoma:
- Arises in submucosal lymphoid patches
Secondary lymphoma
- Proximal esophageal involvement may result from adjacent cervical lymphadenopathy
- Middle esophageal involvement may be due to mediastinal lymphadenopathy
- Distal esophageal involvement often secondary to gastric disease

Risk factors

Chronic immune suppression or dysregulation
- HIV infection
- Chronic hepatitis C infection

Clinical features

Features of primary lymphoma (Dawson criteria):
- No palpable superficial lymphadenopathy
- Normal chest radiograph with no evidence of lymphadenopathy
- Normal peripheral white blood cell count
- Primary esophageal lesion with only regional adenopathy
- No liver or spleen involvement
Insidious onset
- Dysphagia (most common)
- Odynophagia
- Chest / abdominal pain
- Weight loss
Complications (Ann Otol Rhinol Laryngol 1994;103:843)
- Hemorrhage
- Vocal cord paralysis / hoarseness
- Stricture / obstruction
- Perforation with esophagomediastinal or esophagotracheobronchial fistula or mediastinitis
- Vocal cord paralysis
Relatively poor prognosis

Diagnosis

Biopsy with ancillary studies (immunohistochemistry, flow cytometry) to confirm histologic type

Laboratory

Complete blood count may show:
- Leukocytosis ± blasts (leukemic spread)
- Pancytopenia (bone marrow infiltration)

Radiology description

Nonspecific radiographic features
Local extension of gastric lymphoma may cause distal esophageal stricture
Barium swallow / CT: stricture, polypoid or intramural mass
PET useful for staging

Radiology images

Images hosted on other servers:

Strictures:

Marked 3 cm narrowing

Irregular outline and multiple filling defects

Luminal narrowing

Esophageal thickening ± mass lesions:

4 x 4 cm esophageal mass

Thickening of the esophageal wall

Thickening the aortic arch to the gastrointestinal junction

Endoscopic ultrasound:

Transmural thickening of the esophageal wall

Hypoechoic thickening

Primary esophageal Hodgkin lymphoma:

Submucosal nodules

Circumferential thickening

Aneurysmal dilatation

Nodular thickening of esophageal wall

Fistula to lobectomy

Prognostic factors

Stage at diagnosis
Feasibility of surgery or chemotherapy
- Successful management depends on accurate diagnosis and appropriate subsequent treatment
Histologic type: MALT lymphoma and Hodgkin lymphoma have better prognoses than DLBCL or T cell lymphomas
HIV / AIDS associated with worse outcomes
- Median survival: 4 - 6 months

Case reports

3 year old boy with anaplastic large cell lymphoma of esophagus (Pediatr Radiol 2012;42:627)
53 year old man with multiple lymphomatous polyposis with widespread involvement of GI tract (Arch Pathol Lab Med 2003;127:1028)
53 year old man with primary MALT lymphyoma of esophagus, manifesting as a submucosal tumor (Korean J Gastroenterol 2013;62:117)
56 year old man with primary esophageal lymphoma (Ann Thorac Surg 1998;66:1418)
58 year old man with primary extramedullary plasmacytoma of esophagus (Ann Diagn Pathol 2003;7:174)
59 year old man with MALT lymphoma of esophagus (Int J Clin Oncol 2012;17:174)
60 year old woman with dysphagia (Endoscopy 2012;44:E167)
61 year old men with Hodgkin lymphoma of esophagus (Am J Clin Pathol 1997;108:593, AJR Am J Roentgenol 2003;180:1335)
62 year old woman with MALT lymphoma arising in esophagus, stomach and lung (Gen Thorac Cardiovasc Surg 2011;59:826)
63 year old man with T cell lymphoma presenting as esophageal obstruction and bronchoesophageal fistula (Med Sci Monit 2011;17:CS66)
65 year old man with MALT lymphoma of esophagus coexistent with bronchus associated lymphoid tissue lymphoma of lung (Yonsei Med J 2005;46:562)
67 year old man with multiple polyposis of esophagus (Clin Gastroenterol Hepatol 2012;10:e65)
70 year old man with esophageal MALT lymphoma (Dis Esophagus 2013;26:349)
70 year old man with CD8+ mycosis fungoides with esophageal involvement (Oncol Lett 2013;5:73)
77 year old man with primary isolated non-Hodgkin lymphoma of esophagus (World J Gastroenterol 2009;15:1901)
80 year old woman with esophageal perforation and MALT lymphoma (University of Pittsburgh: Esophageal Perforation with an Unusual Etiology [Accessed 31 January 2018])
6 cases of primary esophageal lymphoma (Dig Dis Sci 2006;51:77)
Indolent T cell lymphoproliferative disease of GI tract (Blood 2013;122:3599)
Esophageal leukemic infiltration in children (J Pediatr Gastroenterol Nutr 2011;52:781)
Two immunocompetent patients with primary esophageal lymphoma (World J Radiol 2010;2:334)

Treatment

Primary esophageal lymphoma: local and systemic therapy
- Radiotherapy, chemotherapy
  - Non-Hodgkin lymphoma: CHOP (cyclophosphamide, vincristine, prednisone, doxorubicin), rituximab (anti-CD20 monoclonal antibody)
  - Hodgkin lymphoma: ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) or Stanford V (doxorubicin, vinblastine, mechlorethamine, vincristine, bleomycin, etoposide, prednisone)
- Surgical resection: diagnostic, curative or palliative
  - Endoscopic resection for early lesions (Dig Endosc 2014;26:478)
Secondary esophageal lymphoma: chemotherapy

Clinical images

Images hosted on other servers:

Endoscopy:

Multiple solid, firm nodular lesions

Ulcerated tumor

Large, rounded mass

Solid, firm nodular lesions

Multiple lymphomatous polyposis

Gross description

Submucosal mass with variable ulceration or exophytic growth

Gross images

Images hosted on other servers:

Ulcerated burgeoning masses

MALT: esophagus /
proximal stomach
are unremarkable

Microscopic (histologic) description

DLBCL: large centrocytes, centroblasts, immunoblasts and anaplastic large B cells
- Surface Ig+, CD20+, BCL6±, CD10±, CD43±, PAX5+
Marginal zone B cell lymphoma, MALT type: small lymphocytes, marginal zone B cells, plasma cells, reactive follicles, lymphoepithelial lesions
- Surface and cytoplasmic Ig+ (IgM > IgG or IgA), CD20+, CD5-, CD10-, BCL6-, BCL2+, CD43±, cyclin D1-
Mantle cell lymphoma: small to medium sized, slightly irregular cells with scant cytoplasm
- Surface IgMD+, CD20+, CD5+, CD10-, CD43+, cyclin D1+
Burkitt lymphoma: medium sized atypical lymphoid cells with round nuclei, basophilic cytoplasm and tingible body macrophages (starry sky appearance)
- Cytoplasmic lipid droplets are Oil red O+
- Surface IgM+, CD20+, CD10+, BCL6+, BCL2-, Ki67 ~100%
Follicular lymphoma: mixtures of centrocytes and centroblasts, follicular dendritic cells
- Surface Ig+, CD20+, CD10+, BCL6+, BCL2+, CD5-, CD43-, cyclin D1-
Classic Hodgkin lymphoma: Reed-Sternberg cells and variants in a reactive background
- R-S cells: CD30+, CD15±, PAX5+, ALK-, CD45-, CD3-
Nodular lymphocyte predominant Hodgkin lymphoma: vague nodules of small B cells and interspersed large tumor cells (LP cells) with thin nuclear membranes, fine chromatin and variable nucleoli
- LP cells: CD45+, CD20+, CD15-, CD30-

Microscopic (histologic) images

Images hosted on other servers:

Diffuse large B cell lymphoma

Mantle cell lymphoma

Mycosis fungoides

MALT lymphoma:

Dense submucosal lymphocytic infiltrate

Vaguely nodular growth pattern

Monocytoid appearance

Germinal centers

CD20+

BCL6+ in germinal centers

BCL6+

CD10

Ki67

Kappa / lambda

Cytology description

Discohesive, single abnormal lymphoid cells

Cytology images

Images hosted on other servers:

DLBCL

Burkitt lymphoma

MALT lymphoma

Peripheral smear description

Peripheral blasts if leukemic spread

Positive stains

CD45 (leucocyte common antigen): most hematopoietic neoplasms
- Notable exceptions: ALCL, classic Hodgkin lymphoma, plasma cell myeloma
CD2, CD3, CD4: most T cell neoplasms
CD5: CLL / SLL, mantle cell lymphoma
CD10: follicular lymphoma, Burkitt lymphoma, some DLBCL
CD19, CD20, CD79a: most B cell neoplasms
CD23: SLL / CLL
CD30: ALCL, classic Hodgkin lymphoma
Cyclin D1: mantle cell lymphoma
EBV: classic Hodgkin lymphoma
EMA: ALCL, plasmacytoma, LP cells in NLPHL, some T cell lymphomas
MUM1: classic Hodgkin lymphoma, plasmablastic lymphoma
PAX5: B cell marker, classic Hodgkin lymphoma

Negative stains

Most cytokeratins
Melanocytic markers (S100, HMB45, MelanA, MITF)
Smooth muscle markers (desmin, caldesmon, smooth muscle actin)
Neuroendocrine markers (chromogranin, synaptophysin, NSE)

Flow cytometry description

Useful to determine surface and cytoplasmic CD markers

Molecular / cytogenetics description

Common genetic features of mature B cell lymphomas
- Marginal zone B cell lymphoma, MALT type
  - IGH clonally rearranged
  - t(11;18)
- DLBCL
  - IGH clonally rearranged
  - t(8;14) c-myc-IGH
  - t(14;18) IGH-BCL2
- Burkitt lymphoma
  - IGH clonally rearranged
  - t(8;14)
  - t(2;8)
  - t(8;22)
- Mantle cell lymphoma
  - IGH clonally rearranged
  - t(11;14) BCL1-IGH

Differential diagnosis

Reactive lymphoid hyperplasia: polyclonal by IHC / flow cytometry

Additional references

eMedicine: Esophageal Lymphoma [Accessed 31 January 2018]

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