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Z. G. Schwam and M. Khan

– Oncocytoma: Oncocytes refer to cells with excessive mitochondria, with aci-

dophilic, granular cytoplasm. Associated with aging. – Hemangioma: Most common lesion of childhood. Similar growth and involu-

tion patterns as other hemangiomas. – Kuttner tumor (chronic sclerosing sialadenitis): heavy lymphoid inltrate in

the submandibular gland that can mimic a mass. – Benign lymphoepithelial cyst (BLEC): benign cystic parotid lesions that may

be bilateral and found almost exclusively in patients with HIV. Treatment

consists of anti-retroviral therapy and sclerotherapy. – Mucocele: frequently found on the lower lip as a result of trauma. – Myoepithelioma – Basal cell adenoma: may mimic adenoid cystic carcinoma – Canalicular adenoma: frequently found on the upper lip. – Sialadenosis: hypertrophic acinar cells. May be secondary to endocrinopathy,

alcohol dependence, malnutrition, or anti-cholinergics.

• Malignant lesions

– AJCC 8th edition staging (AJCC):

T1: 0–2cm, T2: 2–4cm, T3: >4cm or with extraparenchymal extension, T4a: gross invasion of the skin, mandible, facial nerve, or external ear canal. T4b: invades the skullbase, pterygoids, or encases carotid.

– Indications for neck dissection and radiation

Neck dissection is indicated for high-grade neoplasms and for those patients with clinically evident cervical lymphadenopathy. Elective neck dissection may be considered in those with T3/T4 tumors or extraparenchymal extension. Adjuvant radiation in the event of close/+ margins, extraparenchymal extension, perineural invasion, high-grade or recurrent low-grade lesion, lymphadenopathy.

– Mucoepidermoid carcinoma: most common malignant salivary lesion in both

adults and children. Low-grade tumors have glandular and microcystic fea-

tures that may be associated with a t(11;19) translocation. Higher grade

lesions associated with lymphadenopathy and a larger epithelial component

on histology. – Adenoid cystic carcinoma: Second most common malignant salivary lesions.

Characterized by perineural invasion with delayed distant mets. Nearly all

patients get adjuvant radiation.

Cribriform (Swiss cheese appearance, most common), tubular (low grade, best prognosis), solid (high grade, worst prognosis)

– Acinic cell carcinoma: predominantly low-grade neoplasm. – Epithelial-myoepithelial: locoregionally aggressive but with relatively low

mortality.

18 Salivary Gland Diseases

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– Salivary ductal carcinoma: high grade, very aggressive, early locoregional

mets, need adjuvant radiation. – Adenocarcinoma: high-grade lesion, most commonly in minor salivary glands. – Polymorphous low-grade adenocarcinoma: may have perineural invasion.

More common in minor salivary glands. Rare neck mets. – Carcinoma ex-pleomorphic adenoma: rapid growth in a long-standing lesion.

Poor overall survival. Adjuvant radiation common. – Lymphoma. May have nodal (non-Hodgkin lymphoma) or MALT lymphoma.

Sjogren disease patients at higher lymphoma risk. – Metastases: SCC > melanoma. BCC may invade directly. SCC > 2 cm or

>4mm thick or those located preauricular have increased incidence of mets to

parotid.

• Post-surgical complications

– Sialocele – Frey syndrome: gustatory sweating. From re-routing of postganglionic para-

sympathetics from the parotid to the sweat glands. – Facial palsy – Auricular numbness: from damage or transection of the great auricular nerve.

Further Reading

355

1. Baker EW, Michael S, Schulte E, Schumacher U.Head and neck anatomy for dental medicine. NewYork: Thieme; 2010.

2. Amin MB, Edge SB, editors. American Joint Committee on Cancer Cancer staging manual. 8th ed. Berlin: Springer; 2017.

3. Bastakis JG, Regezi JA.The pathology of head and neck tumors: salivary glands, Part 1. Head Neck Surg. 1978;1:59.

4. Bastakis JG, Regezi JA, Repola DA.The pathology of head and neck tumors: salivary glands, Part 2. Head Neck Surg. 1978;1:167.

5. Bradley PJ, Eisele DW, editors. Salivary gland neoplasms. Basel: Karger; 2016.

6. Brandwein MS, Ferlito A, Bradley PJ, Hille JJ, Rinaldo A.Diagnosis and classication of salivary neoplasms: pathologic challenges and relevance to clinical outcomes. Acta Otolaryngol. 2002;122:758–64.

7. Califano J, Eisele DW. Benign salivary gland neoplasms. Otolaryngol Clin N Am. 1999;32:861–73.

8. Chan Y, Goddard JC, editors. K.J.Lee’s essential otolaryngology: head & neck surgery. 11th ed. NewYork: McGraw-Hill Education; 2016.

9. Colevas AD, Yom SS, Pster DG, etal. NCCN guidelines insights: head and neck cancers, version 1.2018. J Natl Compr Cancer Netw. 2018;16:479–90.

10. Edgar WM.Saliva: its secretion, composition and functions. Br Dent J. 1992;172:305.

11. Eveson JW, Cawson RA.Salivary gland tumors. A review of 2410 cases with particular reference to histological types, site, age, and sex distribution. J Pathol. 1985;146:51.

12. Flint PW, Haughey BH, Lund VJ, etal., editors. Cummings otolaryngology: head and neck surgery. 6th ed. Elsevier/Saunders: Philadelphia, PA; 2015.

13. Frable MA, Frable WJ. Fine-needle aspiration biopsy of salivary glands. Laryngoscope. 1991;101:245–9.

356

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14. Hiatt JL, Gartner LP.Textbook of head and neck anatomy. 4th ed. Philadelphia, PA: Wolters Kluwer Health/Lippincott William & Wilkins; 2010.

15. Johnson JT, Rosen CA, editors. Bailey’s head and neck surgery: otolaryngology. 5th ed. Wolters Kluwer Health/Lippincott Williams & Wilkins: Philadelphia, PA; 2014.

16. McQuone SJ.Acute viral and bacterial infections of the salivary glands. Otolaryngol Clin N Am. 1999;32:793.

17. Myers EN, Snyderman CH, editors. Operative otolaryngology: head and neck surgery. 3rd ed. Philadelphia, PA: Elsevier; 2018.

18. Som PM, Curtin HD.Head and neck imaging. 5th ed. St. Louis: Mosby Elsevier; 2011.

19. Spiro RH.Salivary neoplasms: overview of a 35-year experience with 2,807 patients. Head Neck Surg. 1986;8:177–84.

20. Turner MD, Troulis MJ.Sialendoscopy and minimally invasive salivary gland surgery. Atlas Oral Maxillofac Surg Clin North Am. 2018;26:i.

21. Turner MD.Combined surgical approaches for the removal of submandibular gland sialoliths. Atlas Oral Maxillofac Surg Clin North Am. 2018;26:145–51.

22. Westra WH.The surgical pathology of salivary gland neoplasms. Otolaryngol Clin N Am. 1999;32:919.

Z. G. Schwam and M. Khan

Chapter 19

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Head andNeck Lymphoma andSarcoma

VivianF.Kaul, MohemmedKhan, andBrettA.Miles

Pearls

• The treatment of lymphoma is quite complex (multimodal) and requires accurate

diagnosis including open lymph node biopsy with ow cytometry analysis and architecture

• Unfavorable sarcoma histology—angiosarcomas, rhabdomyosarcomas espe-

cially of the alveolar morphological type, and osteosarcomas are devastatingly aggressive tumors with an extremely high incidence of distant metastasis

• Malignant brous histiocytoma is the most common soft tissue sarcoma in adults

• Rhabdomyosarcoma—embryonal subtype if found in the orbit has the most

favorable outcome

• Angiosarcoma is an aggressive vascular malignancy that often presents in the

cutaneous scalp of older Caucasian males; prognosis is poor

• Liposarcoma, if metastasizes, uniquely has a propensity for bone

• Synovial sarcoma is primarily a surgical disease

• Dermatobrosarcoma protuberans is a low-grade tumor with a predilection for

local recurrence

• Ewings sarcoma is associated with a mutation in the EWS gene, classically iden-

tied as a small blue round tumor

V. F. Kaul (*) · M. Khan Department of Otolaryngology - Head and Neck Surgery, Icahn School of Medicine at the Mount Sinai Hospital, New York, NY, USA e-mail: Vivian.zhu@mountsinai.org; mohemmed.khan@mountsinai.org

B. A. Miles Otolaryngology Head and Neck Surgery, Oral and Maxillofacial Surgery, Northwell Health System, New Hyde Park, NY, USA e-mail: bmiles4@northwell.edu

https://doi.org/10.1007/978-3-031-26048-3_19

357

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V. F. Kaul et al.

• Osteosarcoma is the most common malignancy of the bone, majority of which

are high grade

• Chondrosarcoma may present as a slowly expanding mass of the laryngeal or the

cricoid cartilages; treatment is surgical resection

• Radiation-induced sarcoma must occur after median latent period of 9–12years

Lymphoma

Epidemiology

• Lymphoma can also be nodal or extranodal (most common site as Waldeyer’s

ring—base of tongue, tonsils, and adenoids)

• Waldeyer’s ring is extranodal; however, in the staging, it is considered as nodal

location

• Hodgkin lymphoma (HL) or non-Hodgkin lymphoma (NHL)—B or T cell

(Table19.1)

Table 19.1 World Health Organization lymphoma classication

Lineage B cell T cell Hodgkin

Types

Precursor lymphoblastic leukemia

Chronic lymphocytic leukemia-small lymphocytic

Prolymphocytic Large granular lymphocytic Lymphocyte-rich

Lymphoplasmacytic Aggressive NK-cell Mixed cellularity Splenic marginal zone Adult T-cell—leukemia Lymphocyte

Hairy cell leukemia Extranodal NK-T-cell-nasal type Plasma cell myeloma/

plasmacytoma Extranodal marginal zone of

MALT type Follicular Subcutaneous panniculitis-like Mantle cell Mycosis fungoides—Sezary

Diffuse large Anaplastic large cell, T/null

Mediastinal large Peripheral T-cell—not otherwise

Primary effusion Angioimmunoblastic Burkitt variants Anaplastic large cell, Y/null

Precursor lymphoblastic leukemia

Prolymphocytic Nodular sclerosis

Enteropathic

Hepatosplenic

syndrome

cell—primary cutaneous type

characterized

cell—primary systemic type

Nodular lymphocytic predominance

classical

depleted

19 Head andNeck Lymphoma andSarcoma

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• 25% extranodal lymphomas occur in the head and neck, and 8% of ndings on

supraclavicular ne-needle aspirate biopsy yield a diagnosis of lymphoma

• Typically present as one or more slowly enlarging rubbery lymph nodes in

the neck

• Chromosomal abnormalities

– Aneuploidy occurs in HL – Translocations and deletions in NHL (c-myc translocation of Burkitt lym-

phoma and the bcl-2 translocation in follicular lymphomas)

• Infectious agents implicated

– Epstein–Barr virus – HIV-1 – Helicobacter pylori – Human T-cell lymphotropic virus-1 (HTLV-1) – Hepatitis B and C viruses – Human herpes virus 8 – Borrelia burgdorferi – Chlamydia psittaci – Campylobacter jejuni

• Chronic inammation increases the risk of lymphoma, i.e., MALT lymphoma

and Sjögren’s syndrome

• Hashimoto disease increases a patient’s risk of thyroid lymphoma approximately

70×, usually aggressive NHL

• Immunosuppression following organ transplantation increases the risk of lym-

phoma and may regress after cessation of immune suppression (EBVassociated NHL)

• HIV infection signicantly increases the incidence of lymphoma. The risk is

increased approximately 1000-fold for Burkitt lymphoma and 400-fold for aggressive lymphoma

359

Hodgkin Lymphoma

• Extends by means of contiguous nodal spread; therefore, it is often localized and

frequently occurs in the mediastinum

• Incidence of HL is increased tenfold in same-sex siblings

Non-Hodgkin Lymphoma

• Tends to spread hematogenously and is often systemic at diagnosis

• NHL may present as a mass in the oropharynx or nasopharynx (Waldeyer’s ring)

or parotid

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• Primary lymphoma of the oral cavity usually arises in the tongue base and is

rarer than a cervical presentation

• Lymphoma masses tend to be rubbery, rm, and non-ulcerating

• Worse prognosis relative to HL

V. F. Kaul et al.

Extranodal NK/T-Cell Lymphoma

• Aggressive ulcerative destructive locally invasive lesion of the nose, sinuses,

and face

• Associated with Epstein–Barr virus and worse prognosis compared with that of

patients with B-cell lymphoma

• Presents as a nasal mass, epistaxis, and/or nasal obstruction and pain

• More common in male adults, Asian and Native Americans

• Poor prognosis, 5-year survival of 50%

Burkitt Lymphoma

• Endemic (African form) manifests as a jaw or an abdominal tumor that spreads

to extranodal sites

• Nonendemic (North American form) has an abdominal presentation with mas-

sive disease

Imaging

• Standard protocol is CT scanning of the chest, abdomen, and/or pelvis

• CT/MRI often indicated for head and neck lymphoma for accurate staging

and therapy

• Positron emission tomography (PET) scanning indicated for staging of disease,

detection of recurrence, and monitoring treatment response

Evaluation andHistology

• Initial evaluation is generally ne-needle aspiration cytology to differentiate

from other malignancies

• Biopsy should be considered when a rm lymph node is larger than 1cm and is

not associated with infection and persists longer than 4weeks

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• May also have painless or mildly tender peripheral adenopathy in cervical, axil-

lary, inguinal, and femoral regions

• Flow cytometry may be utilized for preliminary classication; however, often

excisional lymph node biopsy is warranted for architecture for HL and NHL, submitted fresh for analysis

• CD20 is present in all B-cell lymphomas, an essential marker for

immunotherapy

• CD10 expression is more frequently seen in follicular type of well-differentiated

non-Hodgkin lymphoma

• Bcl-2 is a poor prognosis marker whereas Bcl-6 is a favorable marker for

prognosis

• Immunohistochemical analysis identies monoclonal antibody targets such as

CD20 (rituximab) or CD52 (alemtuzumab)

• Diffuse large B-cell lymphoma, uorescent in situ hybridization (FISH) for

t(8;14)/MYC translocation which is associated with a poor prognosis

• Bone marrow aspiration and lumbar puncture are also frequently indicated to

detect disseminated disease in the bone marrow or CSF via ow cytometry

• Diagnostic tonsillectomy may be indicated if lymphoma of the tonsils is sus-

pected in the setting of tonsillar asymmetry

361

Staging

Hodgkin and non-Hodgkin lymphoma new Lugano staging system (2017)

Stage I—Involvement of a single lymph node region or lymphoid structure or it

is found in only one area of single organ outside the lymph system

Stage II—Involvement of two or more lymph node regions on the same side of the diaphragm or localized contiguous involvement of only one extra-lymphatic site and lymph node region.

Stage III—Involvement of lymph node regions or lymphoid structures on both sides of the diaphragm or lymph nodes involved above the diaphragm and the spleen.

Stage IV—Disseminated involvement of one or more extra-lymphatic organs

Letter Designations

A—Asymptomatic.

B—Constitutional symptoms: Persistent or recurrent fever with temperature higher than 38°C or by recurrent and drenching night sweats within 1month, or by unexplained loss of more than 10% of the person’s body weight within 6months.

E—Extranodal: Direct extension into extra-lymphatic organ from an adjacent lymph node.

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X—Bulky disease: Width of the mediastinal tumor is greater than one-third of the transthoracic diameter at T5/6, or the diameter of the tumor diameter is larger than 10cm.

V. F. Kaul et al.

Management

• Generally treated with chemotherapy±radiotherapy

• Occasionally with early local disease radiotherapy alone may be an option

• Radiotherapy is the primary treatment modality in early-stage NK/T-cell

lymphomas

Hodgkin Lymphoma

• ABVD is a regimen of doxorubicin (Adriamycin), bleomycin, vinblastine, and

dacarbazine now considered the standard of care in HL (70% event-free survival

in favorable stages)

• BEACOPP (i.e., cyclophosphamide, doxorubicin, etoposide, procarbazine, pred-

nisolone, vincristine, and bleomycin with granulocyte colony-stimulating factor)

are being used for advanced HL

• Stanford V regimen (mechlorethamine, doxorubicin, vinblastine, vincristine,

bleomycin, etoposide, and prednisone) with consolidative radiotherapy to bulky

sites produces a progression-free survival of over 74%

• Modied CHOP—including pirarubicin, cyclophosphamide, vincristine and

prednisone (THP-COP) plus radiotherapy for early-stage aggressive non-

Hodgkin’s lymphoma of the head and neck has shown 5-year survival

rates of 89%

Non-Hodgkin Lymphoma

Indolent B-Cell Lymphoma

• Multiple subtypes are generally considered incurable with conven-

tional therapy

• Characterized by an indolent course, patients may remain stable for several years

without therapy

• Watch-and-wait strategy is often used

• Chemotherapy for symptomatic disease, hypersplenism, or bone marrow inltra-

tion causing cytopenia

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363

Aggressive B-Cell Lymphomas

• These include diffuse large BCL (most common type of non-Hodgkin lymphoma

of head and neck, Burkitt lymphoma), mantle-cell lymphoma, and lymphoblastic

lymphoma

• Potentially curable

– Chemotherapy and consolidation radiotherapy have 5-year survival rates of

up to 80% depending on stage

– Newer immunochemotherapy regimens (i.e., rituximab (DA-EPOCH-R)) but

may be useful in certain situations

– Burkitt lymphoma is a highly aggressive B-cell lymphoma and is curable and

treated with systemic and intrathecal chemotherapy – Three types: endemic, sporadic, and immunodeciency associated – Lymphoblastic lymphoma is usually of the T-cell phenotype and usually

affects the mediastinum; this is curable with aggressive chemotherapy – Mantle-cell lymphoma is a B-cell lymphoma that has a moderately aggressive

clinical course, rarely curable, overall survival of 3–5years – Patients with HIV infection have a signicantly increased incidence of lym-

phoma. The risk is increased approximately 1000-fold for Burkitt lymphoma

and 400-fold for aggressive lymphoma

T-Cell Lymphomas

• Rare in the head and neck region relative to B-cell lymphomas

• Associated with HTLV-1

• Extranodal NK/T-cell lymphoma, nasal type

– Nasal cavity, nasopharynx, and palate – Diagnosis difcult due to diffuse thickening – Localized disease treated with radiation, with or without chemotherapy – The prognosis for those with disseminated disease is poor – Common in East Asia and Latin America

• Anaplastic large-cell lymphoma (ALCL) tends to occur in young patients; longterm survival rate is approximately 70% with chemotherapy

• Angioimmunoblastic T-cell lymphoma is usually associated with immunodeciency. It tends to be aggressive with poor outcomes

Head andNeck Sarcoma

• Rare mesenchymal origin malignant tumor

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