Lymphoid lesion 57 - ed.dent.psu.ac.th

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8/17/2014 sompid/ lymphoid lesions 57 1 Dr. Sompid Kintarak Department of Stomatology Prince of Songkla University 2557 Lymphoid lesions Lymph node http://www.dartmouth.edu nhscience.lonestar.edu Barnes Surgical Pathol of HN 3 rd ed. (adenoid) Waldeyer ring http://ethesis.helsinki.fi www.pathology.washington.edu emedicine.medscape.com Foliate papillae Lymphadenopathy

Transcript of Lymphoid lesion 57 - ed.dent.psu.ac.th

8/17/2014

sompid/ lymphoid lesions 57 1

Dr. Sompid KintarakDepartment of Stomatology

Prince of Songkla University2557

Lymphoid lesions

Lymph node

http://www.dartmouth.edu

nhscience.lonestar.edu

Barnes Surgical Pathol of HN 3rd ed.

(adenoid)

Waldeyer ring

http://ethesis.helsinki.fi

www.pathology.washington.edu

emedicine.medscape.com

Foliate papillaeLymphadenopathy

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Reactive lymphoid hyperplasia

Barnes Surgical Pathol of HN 3rd ed.

Acute non-specific lymphadenitis

� Acute inflammatory response in a lymph node, normally associated with reactive changes in the follicles

� Cervical region: due to microbial drainage from infections of the head and neck region e.g. teeth or tonsils

� Axillary or inguinal regions: secondary to infections in the extremities

� Mesenteric lymph nodes: draining acute appendicitis

Acute non-specific lymphadenitis

� Macroscopic findings� swollen, gray-red, engorged

� Microscopic findings� prominent lymphoid follicles with large germinal centers� sinus dilation and filled with neutrophils, histiocytes, and a

moderate admixture of red cells� suppurative mass in follicles, within sinus

� Clinical findings� enlarged nodes due to cellular infiltration and edema

� tender due to capsular distention� redness of overlying skin

Chronic non-specific lymphadenitis

� Chronic immunologic reactions can produce several different morphologic alterations, depending on the underlying stimulus

� Morphology:� Reactive follicular lymphoid hyperplasia� Reactive paracortical lymphoid hyperplasia

� Sinus histiocytosis

� Clinical: enlarged lymph node, not tender

Reactive follicular lymphoid hyperplasia

� Increase in size and number of secondary lymphoid follicles

� Caused by stimuli that activate humoralimmune response

� Specific causes: � rheumatoid arthritis� toxoplasmosis

� early stage of HIV infection� cat scratch disease� cytomegalovirus lymphadenitis in immunocompetent hosts

� primary infection by Epstein-Barr virus (EBV)

library.med.utah.edu

Hyperplastic (Inflamed) Submandibular Lymph Node

© Photos: Dr. Charles Halstead, Virginia

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#46 Lesion: Hyperplastic Buccinator Lymph Node Prevalence (# Lesions/1,000) = 0.1 for Males, 0.1 for Females, 0.1 Total

© Photo: Dr. Jerry Bouquot, The Maxillofacial Center, Morgantown, West Virginia

Buccal Mucosa

Reactive paracortical lymphoid hyperplasia

� expansion of the paracortexbetween reactive or atrophic lymphoid follicles

� Characterized by reactive changeswithin the T-cell regions of the lymph node

� Caused by stimuli that trigger cellular immune responses� Causes:

� drugs (esp. dilantin)

� acute viral infection, particularly infectious mononucleosis, following vaccination against certain viral disease

Barnes Surgical Pathol of HN 3rd ed.

Sinus histiocytosis

� expansion of the sinuses by bland-looking histiocytes in the sinuses� distention and prominence of the lymphatic sinusoids

� the sinuses are filled with histiocytes� the endothelial cells are often grossly enlarged

� Nonspecific but prominent in lymph nodes draining cancer� A rare cause of cervical lymphadenopathy

pleiad.umdnj.edu webpathology.com

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease)

� Rare

� most cases resolve spontaneously

� marked capsular fibrosis, the sinuses are filled with large histiocytes; some have engulfed lymphocytes (emperipolesis)

� there is reactive plasmacytosis

pathologyoutlines.com ipath-network.com

Reactive lymphoid hyperplasia of extranodal sites in the head and neck

www.anatomyatlases.org

� Reactive lymphoid hyperplasia of the lingual tonsil (foliate papilla)

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� Lymphoid hyperplasia� Usually <1 cm in diameter� Posterior lateral tongue

(usually bilateral & symmetrical)

� Posterior hard palate

Angiofollicular lymphoid hyperplasia (Castleman’s disease)

� Rare condition, usually affects thorax� 15-20% of cases causes a cervical mass resembling a

lymphoma� unknown cause� Microscopy:

� Hyperplasia of lymphoid follicles

� Concentrically packed lymphocytes surround a vessel with thickened endothelium

� Highly vascular interfollicular tissue� Hyaline vascular type (90%) vs plasma cell type

Angiofollicular lymphoid hyperplasia (Castleman disease)

� Hyaline-vascular type: 90% of cases� young adults� asymptomatic, localized mass, enlarged cervical LN

� abnormal hyaline vascular follicles� hypervascular interfollicular zone� lack of nodal sinuses

� Plasma cell type:� associated with fever, weight loss, skin rash

� early loss of red blood cells (hemolytic anemia)� hypergammaglobulinemia

� commonly abdominal LN� reactive lymphoid follicles, sheet of mature plasma cells

between the follicles

Angiofollicular lymphoid hyperplasia (Castleman disease)

� Multicentric or generalized Castleman disease:� multiple peripheral nodes involvement with systemic symptoms� hepatosplenomegaly

� associated with HIV infection, HHV-8, overproduction of IL-6

� Management: surgery / high dose corticosteroids� Lymphomatous change is rare� Kaposi sarcoma in patients with the acquired immune deficiency

syndrome

webpathology.com

www.pathologyoutlines.com

Angiofollicular lymphoid hyperplasia (Castlemandisease)

Kimura disease � Rare in the West but common in China, Japan, and

Singapore� a mass forming chronic inflammatory condition of

unknown etiology, frequently involving the soft tissues, salivary glands (parotid, submandibular), cervical LNs, ocular adnexa in the HN region

� form deeper masses rather than mucosal nodules� young to middle-aged males� eosinophilia condition and high serum IgE� surgery may not be required except for cosmetic or

diagnostic reasons

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www.webpathology.com/image

www.scielo.br/scielo.php

Dr. Eiichiro Kimura

www.ispub.com/journal

Epithelioid hemangioma(Angiolymphoid hyperplasia with eosinophilia)

� Age 20-40 years; F > M� reactive process > neoplasm� affected skin of the head and neck region� subcutaneous lesions first appear as dull red pruritic

plaques� oral lesions (occasional) may resemble pyogenic

granuloma or may ulcerate� Microscopy:

� vascular proliferation with histiocytoid endothelial cells and stromal inflammatory reaction

� usually not associated with a heavy lymphoid infiltrate

dermatology.cdlib.org

ades.tmu.edu.tw

www.histopathology-india.net/ALHE.htm

� Angiolymphoidhyperplasia with eosinophilia

Drug-associated Lymphadenopathy

� Phenytoin-associated lymphadenopathy� can develop within a few weeks or months, but

more frequently after long-term treatment� not associated with serum-sickness-like

symptoms of fever, rashes, joint pain� cervical LNs are frequently first affected, then

becomes widespread

� Microscopic changes can closely mimic Hodgkin lymphoma

Oral lymphoepithelial cyst� Develops within a benign lymphoid aggregate or

accessary tonsil of oral or pharyngeal mucosa� Clinical presentation:

� yellow to white nodule� asymptomatic, slow growing

� overlying mucosa intact, smooth� most common in floor of mouth, followed by ventral tongue and

posterior lateral tongue

� Microscopy: lymphoid tissue with germinal centers surrounding true cyst lumen filled with epithelial debris

� Clinical DDX: lipoma, minor salivary gland neoplasm or sialolith, mucocele

� No treatment needed unless in traumatized area

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Benign lymphoepithelial lesion (myoepithelial sialadenitis)

� Microscopic features of lymphocytic infiltrate with an associated epimyoepithelial island

� Clinical features:� mostly develop as a component of Sjögren’s

syndrome (bilateral lesions): women� those not associated with Sjögren’s syndrome are

usually unilateral� may occur in association with other salivary gland

pathologic conditions e.g. sialoliths, benign or malignant epithelial tumors

Benign lymphoepithelial lesion (myoepithelial sialadenitis)

� Clinical features:� most often develops in adults, mean age 50 y� female (60% to 80% of cases)� parotid glands (85%), submandibular glands, minor

glands� usually appears as firm, diffused swelling of the

affected gland� asymptomatic or associated with mild pain

Benign lymphoepithelial lesion (myoepithelial sialadenitis)

� Histopathologic features:� a heavy lymphocytic infiltrate associated with the

destruction of the salivary acini� germinal centers may or may not be seen� ductal cells and surrounding myoepithelial cells

become hyperplastic, forming ‘epimyoepithelialislands’ throughout the lymphoid proliferation

www.brown.edu/Courses/Digital_Pa...lel.html

www.nature.com/modpathol/journal...2f1.html

Benign lymphoepithelial lesion (myoepithelial sialadenitis)

� Treatment and prognosis:� surgical removal with good prognosis in most cases� increased risk for lymphoma, either within the affected

gland or in an extrasalivary site (uncertain risk but one study showed the risk in patients with Sjögrensyndrome to be ~44 times greater than expected in general population – Kassan, et al. Ann Intern Med. 1978 Dec;89(6):888-92.

○ Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)

Benign lymphoepithelial lesion (myoepithelial sialadenitis)

� Treatment and prognosis: (continue)� a rare malignant counterpart of this lesion: malignant

lymphoepithelial lesion or lymphoepithelial carcinoma○ poorly differentiated salivary carcinoma with a

prominent lymphoid stroma○ most cases have occured in Inuits (Eskimos) and

Asians and appear to have arisen de novo as carcinomas

○ Epstein-Barr virus is the possible causative agent (not definite proof)

○ guarded prognosis (Asian patients have had a better survival rate)

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www.forpath.org/0201/html/case_9_10.htm

www.pathologyoutlines.com/lungtumor.html

Low-grade marginal zone B cell lymphoma of MALT

Hematolymphoid neoplasia� Hodgkin lymphoma� Non-Hodgkin lymphoma (more common)

Hematolymphoid neoplasia

� The most common hematolymphoid neoplasm in the HN region are � diffuse large B-cell lymphoma (DLBCL), follicular

lymphoma, classical Hodgkin lymphoma for LNs� DLBCL, extranodal marginal zone lymphoma of

mucosa-associated lymphoid tissue (MALT lymphoma), extranodal NK/Tcell lymphoma, and extramedullary plasmacytoma for extranodal sites

� mantle cell lymphoma of extranodal sites� Burkitt lymphoma of the jaw in children of central

Africa, and the sporadic form can affect the tonsils and lymph nodes of children and young adults

Hematolymphoid neoplasia

� Etiology: � Etiology of most types is unknown� Chromosomal translocations and oncogenes� Inherited genetic factors� Immunosuppression: posttransplant lymphoproliferative

disorder (PTLD), HIV/AIDS� Viruses: HTLV-1, EBV, KSHV/HHV-8� Environmental agents� Iatrogenic factors Regezi’s Oral Pathology. 6th ed.

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Hodgkin lymphoma

� Nodular lymphocyte-predominant Hodgkin lymphoma

� Classical Hodgkin lymphoma:� Nodular sclerosis classical Hodgkin lymphoma� Mixed cellularity classical Hodgkin lymphoma� Lymphocyte-rich classical Hodgkin lymphoma� Lymphocyte depleted classical Hodgkin lymphoma

Classical Hodgkin lymphoma

� predominantly a nodal-based disease (75% of cases) � mediastinal, supraclavicular, cervical LNs� Males, bimodal age distribution: 15-35 and >50 years� persistent enlarging, non-tender, discrete mass or

masses in one LN region� a better prognosis if no systemic signs & symptoms

(fever, night sweat, and weight loss)� characterized by neoplastic Reed–Sternberg cells and

their variants scattered in an appropriate inflammatory background

� ~40% of cases of classical Hodgkin lymphoma are associated with EBV

www.healthoma.com

www.wgabinecie.pl

Hodgkin’s lymphoma� CD45-, CD30+, CD15+/-

library.med.utah.edu

www.pathconsultddx.com

� Reed-sternberg cells and variants

Nodular lymphocyte-predominant Hodgkin lymphoma� Rare B-cell neoplasm� usually children to middle-aged adults, with marked

male predominance� most patients present with solitary enlarged lymph node

in the cervical, axillary, or inguinal region (stage I/II disease)

� large neoplastic cells (lymphocytic and histiocytic cells or L&H cells) have been nicknamed ‘‘popcorn cells’’ because of the characteristic lobulation of the nuclei with multiple deep notches.

� Large cells: CD45+, CD20+, CD30-, CD15-, EBV-

www.pathconsultddx.com

� Popcorn-like L&H cell

www.webpathology.com

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2008 WHO classification of lymphoma and histiocytic/dentritic cell tumors

� Precursor lymphoid cell neoplasms� B-lymphoblastic leukemia/lymphoma� T-lymphoblastic leukemia/lymphoma

� Mature B-cell neoplasms� Chronic lymphocytic leukemia/small lymphocytic lymphoma� B-cell prolymphocytic leukemia� Lymphoplasmacytic lymphoma� Splenic B-cell marginal zone lymphoma� Splenic lymphoma-leukemia, unclassifiable� Hairy cell leukemia� Plasmacytoma� Extranodal marginal zone lymphoma of MALT (MALT lymphoma)� Nodal marginal zone lymphoma� Follicular lymphoma� Primary cutaneous follicle center lymphoma� Mantle cell lymphoma

2008 WHO classification of lymphoma and histiocytic/dentritic cell tumors

� Mature B-cell neoplasms� Diffuse large B-cell lymphoma (DLBCL), not otherwise specified� DLBCL, distinct subtypes:

○ Primary mediastinal (thymic) large B-cell lymphoma○ Intravascular large B-cell lymphoma○ Primary effusion lymphoma○ T-cell/histiocyte-rich large B-cell lymphoma○ Plasmablastic lymphoma○ ALK-positive large B-cell lymphoma○ EBV+ diffuse large B-cell lymphoma of the elderly○ DLBCL associated with chronic inflammation○ Primary DLBCL of the central nervous system○ Primary cutaneous DLBCL, leg-type○ Large B-cell lymphoma arising in HHV8-associated multicentric

Castleman disease

2008 WHO classification of lymphoma and histiocytic/dentritic cell tumors

� Mature B-cell neoplasms� Lymphomatoid granulomatosis

� Burkitt lymphoma� B-cell lymphoma, unclassifiable, with features intermediate between

DLBCL and Burkitt lymphoma� B-cell lymphoma, unclassifiable, with features intermediate between

DLBCL and classical Hodgkin lymphoma

2008 WHO classification of lymphoma and histiocytic/dentritic cell tumors

� Mature T-cell and NK-cell neoplasms� T-cell prolymphocytic leukemia� T-cell large granular lymphocytic leukemia� Adult T-cell leukemia/lymphoma� Aggressive NK-cell leukemia� Chronic lymphoproliferative disorder of NK cells� EBV+ T-cell lymphoproliferative disease of childhood

○ Systemic EBV+ T-cell lymphoproliferative disease of childhood○ Hydroa vacciniforme-like lymphoma

� Extranodal NK/T-cell lymphoma, nasal type � Enteropathy-associated T-cell lymphoma � Hepatosplenic T-cell lymphoma � Angioimmunoblastic T-cell lymphoma � Peripheral T-cell lymphoma, not otherwise specified

2008 WHO classification of lymphoma and histiocytic/dentritic cell tumors

� Mature T-cell and NK-cell neoplasms� Anaplastic large cell lymphoma, ALK+� Anaplastic large cell lymphoma, ALK-� Mycosis fungoides/ Sézary syndrome � Subcutaneous panniculitis-like T-cell lymphoma � Primary cutaneous CD30-positive T-cell lymphoproliferative disorders

○ Lymphomatoid papulosis○ Primary cutaneous anaplastic large cell lymphoma

� Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma

� Primary cutaneous γδT-cell lymphoma� Primary cutaneous CD4+ small/medium T-cell lymphoma

2008 WHO classification of lymphoma and histiocytic/dentritic cell tumors� Immunodeficiency-associated lymphoproliferative disorders

� Lymphoproliferative diseases associated with primary immune disorders� HIV-related lymphomas� Posttransplant lymphoproliferative disorders� Methotrexate-associated lymphoproliferative disorders

� Histiocytic and dendritic cell neoplasms� Histiocytic sarcoma� Langerhans cell histiocytosis� Langerhans cell sarcoma� Follicular dendritic cell sarcoma� Interdigitating dendritic cell sarcoma� Indeterminate dendritic cell tumor� Fibroblastic reticular cell tumor� Disseminated juvenile xanthogranuloma� Blastic plasmacytoid dendritic cell neoplasm (formerly ‘‘blastic NK-cell

lymphoma’’)

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Non-Hodgkin’s lymphoma

� MALT lymphoma� Diffuse large B-cell lymphoma� Burkitt lymphoma

Non-Hodgkin lymphoma� relatively common in head & neck region� most common in middle-aged and older� non-tender, diffuse swelling with boggy consistency or

mass of reddish blue tissue with ulceration, pain� paresthesia particularly with a mandibular lesion (numb

chin syndrome)� initial presentation in oral cavity is uncommon (2%)� may rise within LNs or extranodal in soft tissue:

� Waldeyer ring, buccal vestibule, posterior hard palate, soft palate, gingiva, tongue

� Ill-defined radiolucency in bone, loss of lamina dura, uniform widening PDL space, displaced teeth, malocclusion and/or exfoliation

Lymphoma

MALT lymphoma

� Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue type

� accounts for 8% of all non-Hodgkin lymphomas� an indolent lymphoma � occurs in various mucosal and extranodal sites� most commonly involved salivary gland, thyroid gland,

and ocular adnexa� most patients have early stage disease (stage I/II) at

presentation

Diffuse large B-cell lymphoma

� a rapidly growing neck mass (lymph node), but 30% of patients present with extranodal disease and 71% have extranodal involvement during the course of disease

� about half of the patients have stage I/II disease� about 50% of cases express BCL2 protein� an aggressive but potentially curable disease, usually

treated by multiagent chemotherapy together with anti-CD20 (Rituximab)

� relatively common in head & neck region

Neville’s Oral Pathology. 3rd ed.

NHL of the palateRegezi’s Oral Pathology. 6th ed.

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� Diffuse large B-cell lymphoma - spleen

Robbin’s Pathology. 8th ed.

LCA

CD20CD3

Diffuse large B cell lymphoma

Burkitt’s lymphoma

� Highly aggressive B-cell lymphoma � Genetic mutations: C-MYC, p53 � Three forms:

� an endemic type in Africa� a sporadic form occurring in North America and Europe

� a form associated with immunodeficiency

� EBV associated (strong with African Burkitt)� Jaw involvement is characteristic of endemic BL; up to

50% of those afflicted have lesions of the maxilla or mandible

Burkitt’s lymphoma

� Children with peak prevalence ~ 7 y, male� Maxilla > mandible (2:1): posterior segments� Jaw involvement related to the age of patients� X-ray: ill-defined bone destruction

Neville’s Oral Pathology. 3rd ed.

Histopathology: � diffuse infiltration by monotonous

medium-sized cells with a starry-sky pattern

� tumor cells have round or molded nuclei, clumped chromatin, and multiple nucleoli

� numerous mitoses� almost 100% of tumor cells are in

reprication process� Over expression of c-myc resulted from

t(8;14)Treatment:� Death within 4-6 m if not treated� Intensive, multiagent chemotherapeutic

protocols � event-free survival rate 3 to 5 y after treatment ~ 85% to 95% with stage I or II disease, and 75% to 85% with stage III and IV

CD20CD3

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CD10

Bcl2

Burkitt Lymphoma