Algemeen

Introduction

Malignant tumors of the head and neck region form, at least in the Western countries, only a small percentage of the total number of malignancies in humans. In recent years, there appears to be an increase, possibly due to the aging and the increasing number of HPV-related tumors. Tumors in the head and neck have a special place in oncology because of their location in an area that is visible to everyone, and that for many, almost indispensable, life functions such as eating, drinking, breathing, talking, etc. is particularly important. The diagnosis and treatment of these relatively rare tumors should be made preferably in specialized centers by a multidisciplinary team, which must have a head and neck surgeon (ENT and oromaxillofacial surgery), radiation oncologist, reconstructive surgeon, pathologist, medical oncologist, radiologist, nuclear medicine  and a dentist prosthedontist, head and neck oncology nurse, physiotherapist, speech therapist and dental hygienist.

Anamnesis

Etiological factors

  • Alcohol
  • Tobacco smoking
  • HPV, EBV
  • Dental hygiene and irritation
  • Smokeless tobacco (chewing)
  • Beetle nut
  • Occupation: polluting industry / chemicals / building.
  • Wood and metal dust, leather industry
  • UV-irradiation

Risk factors

  • Previous radiation
  • Premalignant leasions (Leukoplakia, dysplasia)
  • HIV
  • Family history of cancer in head and neck, oesophagus and lungs
  • Immunosuppressive drugs
  • BRAF inhibitors

Complaints

  • Swelling in neck
  • Pain irradiating to neck-ears
  • sensory disorders
  • bleeding, ulcers
  • Foetor ex ore
  • Loss of teeth
  • Difficulties with dental prosthesis
  • Articulation / voice complaints
  • Difficulty swallowing and obstruction
  • trismus

Histology

  • Squamous Cell carcinoma
  • Undifferentiated carcinomas
  • Salivary Gland Carcinomas
  • Thyroid well differentiated carcinomas and rare variants
  • Basal cell carcinoma
  • Skin adnex carcinomas
  • Melanoma
  • Sarcoma
  • Metastases from elsewhere
  • Malignant lymphoma
  • Rare tumors

HP by core biopsy, incisional biopsy or cytology.

Diagnostics

T-classification

  • Head and neck and mucosal examination by inspection + palpation
  • Meticulous description of extension and infiltration of primary and metastases. Also indicate on figure in file / drawing / pictures
  • Examination under general anaesthesia in case of pharyngeal and laryngeal tumors to assess extension and exclude second primaries when imaging is insufficient or biopsies are difficult or teeth have to be extracted. Proximal esophagoscopy in case of hypo pharyngeal cancer.
  • OPG / panoramic X-ray of dentition in case of radiotherapy / mandible assessment
  • CT-scan/MRI/PET-CT/endoral ultrasound

N-classificatie

  • Inspection + palpation of the neck
  • Cytology
  • Ultrasound guided FNAC(cytologie) : see guidelines
  • CT-scan/MRI/PET-CT

M-classification: see guidelines

  • Chest X-rax
  • Liverfunction, serum markers (S100, SCC, thyroglobuline)
  • Imaging: mainly PET-CT (sometimes MRI/CT/bone scintigraphy/I scintigraphy)

Levels of the neck

Level I: submental (Ia) (bounded by anterior bellies of the musculi digastrici, chin and hyoid) and submandibular (Ib) (limited by mandible, hyoid and the musculus digastricus) triangle.
Level II: lymph nodes around the cranial 1/3 of the internal jugular vein and Accessory nerve. Bounded by the base of the skull, the hyoid (carotid bifurcation), rear edge of the musculus sternohyoïdeus and of the sternocleidomastoid muscle. IIa: under the accessory nerve, IIb: above the accessory nerve.
Level III: this area is below region II. The other limits are: the musculus omohyoïdeus (or bottom edge cricoid) and the posterior border of the sternocleidomastoid muscle.
Level IV: low-jugular lymph nodes. Boundaries: musculus omohyoïdeus (or bottom edge cricoid), the musculus sternohyoïdeus, the clavicle and the rear edge of the sternocleidomastoid muscle.
Level V: glands in the posterior neck triangle. Boundaries: the rear edge of the sternocleidomastoid muscle, the trapezius muscle and the clavicle. Va: above cricoid bottom edge or accessory nerve. Vb: below cricoid bottom edge or below accessory nerve.
Level VI contains the lymph nodes between hyoid, Jugulum (or anonymous artery) and the medial border of the carotid sheath.

TNM-classification

UICC 2017

T-classification: see individual primaries.

Clinical N-classification (different for skin, melanoma, thyroid and nasopharynx):

  • NX Regional lymph nodes cannot be assessed
  • N0 No regional lymph node metastasis
  • N1 Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension without extranodal extension
  • N2
    • N2a Metastasis in a single ipsilateral lymph node, more than 3 cm but not more than 6 cm in greatest dimension without extranodal extension
    • N2b Metastasis in multiple ipsilateral lymph nodes, none more than 6 cm in greatest dimension, without extranodal extension
    • N2c Metastasis in bilateral or contralateral lymph nodes, none more than 6 cm in greatest dimension, without extranodal extension
  • N3
    • N3a Metastasis in a lymph node more than 6 cm in greatest dimension without extranodal extension
    • N3b Metastasis in a single or multiple lymph nodes with clinical extranodal extension

Pathological N-classification

  • pNX Regional lymph nodes cannot be assessed
  • pN0 No regional lymph node metastasis
  • pN1 Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension without extranodal extension
  • pN2
    • pN2a Metastasis in a single ipsilateral lymph node, less than 3 cm  with extranodal extension or, more than 3 cm but not more than6 cm in greatest dimension without extranodal extension
    • pN2b Metastasis in multiple ipsilateral lymph nodes, none more than 6 cm, without extranodal extension
    • pN2c Metastasis in bilateral or contralateral lymph nodes, none more than 6 cm, without extranodal extension
  • pN3
    • pN3a Metastasis in a lymph node more than 6 cm in greatest dimension without extranodal extension
    • pN3b Metastasis in a lymph node more than 3 cm in greatest dimension with extranodal extension or, multiple ipsilateral, or any contralateral or bilateral node(s) with extranodal extension

M-classification:
M0:  no distant metastases

M1: distant metastases

Stage

  • Stage 0:  Tis N0
  • Stage I:  T1 N0
  • Stage II:  T2 N0
  • Stage III:  T3N0, T1-3N1
  • Stage Iva: T4aN0-1, T1-4aN2
  • Stage IVb: T1-4aN3, T4b
  • Stage IVc: M1

Prognostication

Besides the TNM stage and grading systems and many known prognostic factors, currently prognosis can be estimated through various programs:

Model V. Vanderpoorten, Leuven on parotiscarcinomen: Model

Model Oncologiq, R. Baatenburg de Jong: Old model / New model

Model Maastro: Model

Treatment

See per tumor localization.

The primary, curative treatment exists in principle, depending on the location and size of the tumor process, from primary surgery (including Robot, Laser, PDT), radiotherapy, cisplatin or cetuximab or a combination with the objective of maximizing oncological outcome and minimising functional handicaps. Laser therapy, Robot resections and photodynamic therapy (PDT) may be used for small tumors. Cryotherapy is appropriate in small pre-malignant skin tumors. (Adjuvant) chemotherapy and immunotherapy have no prominent place in the initial routine treatment of head and neck tumors.

With functional or anatomic unresectable tumors: chemoradiation or radiation with cetuximab is often possible.

Indications for postoperative radiotherapy

  • In case of close or positive resection margins, recession is preferred when feasible.
  • Depending on localization and T stage (per tumor type listed separately, in general all T4 (except when very small and well resected: should be discussed in HP meeting).
  • Three or more risk factors
    • T3
    • N1, N2a < 3cm
    • Perineural growth
    • Resection margin < 3 mm (depending on size and growth pattern, and in case recession is not an option)
    • Lymph(angio) invasion
    • Infiltrative growth

Lymph node metastases

  • More than one tumor positive lymph node: pN2b,c (not micrometastases)
  • Metastases > 3 cm, Level 4-5 metastases
  • Extranodal tumor localisations
  • Close resection margins in neck

Indications postoperative chemoradiation

  • Using Cisplatin 3×100 mg/m2
  • Evident (macroscopic) extranodal spread
  • Tumorpositive resection margins (so tumor in the margin, not close margin 1-2 mm) at primary or neck
  • N2c: it should be discussed
  • N3

Treatment of Locoregional Recurrence

After primary surgery if possible re-excision often followed by radiotherapy or radiotherapy / chemoradiation
After primary radiotherapy if possible surgery (sometimes additional radiation/ chemoradiation)
After combination treatment: individualize but if possible re-excision, PDT, chemotherapy

Distant Metastases

If cytostatic therapy is considered: preferably in a research context. In case one or two lung metastases during follow-up are found: if possible metastasectomie.

Surveillance

Consider offering rehabilitation !

1st year: depending on the tumor every 1-3 months
2nd year: every 4 months
3rd year: every 5-6 months
4th year: every 6 months
5th year: every 6 months
from the 6th year: only when indicated, adenoid cystic carcinoma

Routine Follow-up

  • Anamnesis focusing on symptoms that are the result of recurrence and / or metastasis and / or new tumor growth
  • weight
  • Inspection and palpation of the treated areas (cave 2nd primary tumor)
  • Inspection and palpation of the regional lymph node areas
  • Ultrasound FNAC of the neck: routinely every 3 months if the neck was not treated: see ultrasound guidelines
  • Thyroid Functions: every 6-12 months when the neck was irradiated
  • Further imaging and respons evaluation: MRI or PET-CT at 3 months post treatment after chemoradiation / radiotherapy in tumors larger than T2N1.
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