the choana or Rosenmuller fossa. The surface of the tumor often shows pronounced
vessels. In later stages there is swelling of the lateral part and the face of
the nasal skeleton, protrusion of the cheek, and possibly exophthalmos. Finally
the child has difficulty in eating. The tumor is very hard to palpation.
Palpation should be carried out carefully because damage to the surface of the
tumor with the fingernail may extensive bleeding.
Pathogenesis. The typical nasopharyngeal fibroma is histologically benign but
clinically malignant because of its expansive growth. It is a very coarse
angiofibroma rich in fibrous tissue which arises from the roof of the
nasopharynx. The tumor usually has a broad base from the body of the sphenoid
bone and is attached very firmly. It grows relatively quickly. After filling the
nasopharynx, the tumor sends offshoots into the nasal sinuses, the upper jaw,
the sphenoid sinus, the pterygopalatine fossa, the cheek, the ethmoid sinuses,
and the orbit. Finally, it may extend into the cranial cavity after eroding the
base of the skull.
Diagnosis. This is made by palpation, examination with the mirror and the
nasopharyngoscope loupe endoscope, and radiographs including tomograms in the
anterior, posterior, and lateral planes. Bilateral external carotid and internal
carotid artery angiograms are indicated for extensive tumor. A computed
tomography (CT) scan is done to assess involvement of the intracranial cavity.
Superselective angiography of the branches of the carotid is carried out to
allow therapeutic embolization.
Differential diagnosis. This includes hypertrophied adenoids, choanal polyp
(which is soft and does not bleed), lymphoma, chordoma, and teratoma.
Care should be taken in carrying out a biopsy: this can cause massive bleeding.
A nasopharyngeal tumor in an individual between the ages of 10 and 25 years and
which is suspected as being a juvenile nasopharyngeal angiofibroma should only
be submitted to biopsy in a hospital, and preparation should be made to proceed
immediately to further surgery if massive bleeding occurs.
Treatment. Response to radiotherapy is not to be expected because of the
histologic type of the tumor. Radiotherapy with above-average dose is therefore
only to be considered as a palliative measure, e.g., for massive extension of
the tumor into the middle cranial fossa, or where the risk of operation is too
high. Hormone therapy may also be tried but the results are uncertain.
The method of choice is operative removal. Immediate preoperative therapeutic
embolization of the supplying vessels may be undertaken in large tumors. This
reduces the otherwise massive bleeding during the operation, but there is a
definite risk of undesirable results of embolization. Feeding branches from the
external carotid artery identified by radiography may be ligated as a
preliminary step, often on both sides. There is a high risk of recurrence, in
about 20% of patients.
Rare Benign Nasopharyngeal Tumours
Chordoma. This develops from the notochord and occurs mainly in men between 20
and 50 years of age. It grows very slowly and erodes the base of the skull
causing lesions of the cranial nerves, and it may also extend into the sphenoid
sinus.
Treatment. Surgery should be performed if possible, but there is a considerable
risk of recurrence. Radiotherapy is only palliative. Metastases to the neck are
said to occur.
Other types of tumor include teratoma, dermoid, fibroma, and lipoma.
Treatment. Surgery should he undertaken if the patient has symptoms.
Malignant tumours of the nasopharynx
The most frequent of these relatively rare tumors is the squamous cell carcinoma
together with the lymphoepithelial tumor. These two together constitute 75% of
tumors of this region. Tumors of children include lymphoma, plasmacytoma, and
Burkitt's lymphoma in certain parts of Africa. Nasopharyngeal tumors affect men
twice as commonly as women.
Symptoms. These include nasal obstruction, disorders of tubal aeration causing
unilateral conductive deafness, middle ear discharge, middle ear effusion,
bloodstained purulent nasal discharge, and headaches felt deep within the skull.
Lymph node metastases are frequent and widespread in 90% of cases. Enlarged
jugulodigastric lymph nodes on one or both sides are often the first symptom
that brings the patient to the doctor. Lymph node metastases may also be
retropharyngeal and in the nuchal area. The primary tumor may remain
undiscovered despite careful endoscopic search because it grows concealed
beneath the mucosa. There is gradual protrusion and loss of mobility of the soft
palate and increasing, often unilateral pain in the head and the face
(trigeminal nerve). Exophthalmos, oculomotor paralyses of the IIIrd, IVth, and
VIth cranial nerves, involvement also of the Vth, IXth, Xth, XIth, and XIIth
cranial nerves, oral fetor, and massive nosebleeds occur. Blood-borne metastases
to the lung, the liver, and the skeleton are quite common.
Diagnosis. It is made by rhinoscopy, nasopharyngoscopy, palpation, retraction of
the soft palate, and biopsy. Tomograms in the anterior, posterior, and lateral
views and CT scans elucidate involvement of the base of the skull. Angiograms
may also be needed. The roof of the nasopharynx and Rosenmuller's fossa require
particular attention because these are often the site of origin of this tumor.
Treatment. Radiotherapy is the method of choice for mesenchymal tumors and
anaplastic carcinomas as they are very radiosensitive. It may be combined with
chemotherapy. Surgery should only be considered for very small circumscribed
nasopharyngeal tumors, combined with electrocoagulation, if it can be shown that
the tumor does not extend into the eustachian tube or the base of the skull.
Postoperative radiotherapy should be used. Advanced carcinomas are treated
solely by radiotherapy, at times combined with chemotherapy for the primary
tumor. Regional lymph node metastases may be treated by therapeutic neck
dissection provided that the primary tumor has been destroyed.
Prognosis. The 5-year survival in carcinoma of the nasopharynx is about 15%,
although the 5-year survival for stage I may be 30% or better. Unfortunately,
patients are seldom seen at this stage.
Tumours of the oropharynx
PATHOLOGY. The oropharynx consists of four sites as classified by the Union
Internationale Contre le Cancer (UICC):
Roof: Soft palate.
Floor: Posterior third of tongue and lingual epiglottis.
Lateral wall: Tonsil, anterior and posterior pillars.
Posterior wall: Posterior wall of pharynx from hard palate to hyoid level.
It contains squamous epithelium, lymph tissue and minor salivary glands and so
tumours of these three elements can occur. Their incidence is as follows:
squamous-cell carcinoma, 75%; lympho-epithelioma, 5%; lymphoma, 15%; minor
salivary gland tumours, 5%. The site incidence of squamous carcinoma is: lateral
wall, 45%; base of tongue, 40%; posterior wall, 10%; soft palate, 5%.
The UICC classification with respect to T staging is: T1: a tumour less than 2
cm; T2: a tumour between 2 and 4 cm; T3: a tumour greater than 4 cm, T4: a tumor
with extension to bone, muscle, skin, neck.
The maximum age incidence is in the eighth decade and the male/female ratio is
10: 1.
Clinical features. The history is usually of sore throat, otalgia or dysphagia.
Some patients have merely a feeling of a lump in the throat. About 20% of
tonsillar carcinomas present with a gland in the neck as the only symptom.
On examination all the structures in the oropharynx should be seen perorally and
with a nasopharyngeal and laryngeal mirror. The size of the tumour should be
assessed not only by eye but also by palpation. A carcinoma is nearly always
ulcerated, whereas lymphoma and salivary gland tumours are smooth enlargements.
The neck should also be palpated to complete the staging.
Investigations. Radiography is not usually helpful except to see if there has
been mandibular invasion. In a lymphoma, however, a chest radiographs are
essential.
Similarly laboratory investigations have little to add except in the assessment
of the general health of the patient. Biopsy should be performed under general
or local anaesthesia so that the extent of the tumour can be assessed at the
same time.
Treatment of carcinomas. Approximately 20% of patients with oropharyngeal
carcinoma are incurable for the following reasons: (1) highly anaplastic tumour;
(2) bilateral neck glands; (3) trismus; (4) distant metastases; (5) patient
refusal of treatment; (6) advanced age; (7) poor general condition. Not all of
these conditions are absolute contraindications to treatment, but the 2-3%
survival rates seen in patients with bilateral neck glands, trismus and
anaplastic tumours make one wonder if it is worth while subjecting the remaining
98% to radical surgery or radiotherapy.
Tonsil carcinoma. If there are no palpable neck glands then the primary
treatment is by irradiation because the results are just as good as with
surgery. If, however there is a palpable neck gland, primary irradiation cures
virtually no one. These patients should be treated with a commando operation
removing the fauces, part of the palate and base of tongue, part of the mandible
and a radical neck dissection. If the pharynx is closed primarily the oral
cavity will be crippled and so reconstruction is performed with either a
forehead flap or a myocutaneous flap including the pectoral muscle, which
enhances the «take» of the graft as well as supplying more bulk.
Posterior-wall carcinoma. Again, for the same reasons, radiotherapy is used as
the primary treatment. In the event of recurrence the posterior pharyngeal wall
can be widely excised via a lateral pharyngotomy and a reconstruction performed
with a medially based chest flap.
Treatment of Lymphomas. These should be irradiated, and, if the assessment shows
that they have spread outside the head and neck, chemotherapy should also be
used. If a tonsil is suspected of being involved with a lymphoma it should be
totally excised, since this gives the pathologist a better chance of diagnosing
the type than does a punch biopsy.
Prognosis. It depends on many factors such as type of tumour, size, neck gland
metastases, age, general state of a patient, etc. Lymphoma limited to the
oropharynx does well with around a 70% survival rate, but it falls dramatically
when it is out with the head and neck. Adequate excision of benign salivary
gland tumours should lead to a very low recurrence rate. Malignant salivary
gland tumours do well in the first 5-year period (70%), but poorly over 10 years
(5%).
With regard to squamous carcinoma, patients with tonsillar tumours survive much
longer than those with base of tongue tumours. Soft-palate and posterior-wall
tumours are so rare that survival patterns cannot be accurately predicted.
TUMOURS OF THE LARYNX
BENIGN TUMORS
Fibroma of the Larynx
Symptoms. These include hoarseness, aphonia and attacks of coughing. Dyspnea
occurs with large fibromas. If the polyp has a pedicle and is floating between
the cords, the voice may return to normal for short intervals.
Pathogenesis. This is the most common benign tumor of the vocal cords, mainly
affecting men. It is often initiated by agents causing laryngeal inflammation.
Hyperkinetic voice disorders and vocal abuse are important.
Diagnosis. Laryngoscopy shows the fibroma usually lying on the free edge of the
vocal cord either on a pedicle or sessile. It is seroedematous and occasionally
hemorrhagic. Older fibromas appear firm due to fibrosis and thickening of the
overlying epithelium.
Treatment. The fibroma is removed by endolaryngeal microsurgery, with
preservation of the vocal ligament and vocalis muscle. The patient is advised to
rest his voice until the defect epithelializes.
The fibroma may be removed by direct or indirect laryngoscopy after
premedication to inhibit reflexes and topical anesthesia.
The fibroma should always be examined histologically to establish the diagnosis.
Papillomas
Symptoms. Depending on the site and extent of the lesions, these include
hoarseness which is often severe and respiratory obstruction.
Pathogenesis. This disease has etiologic and morphologic similarities to the
common wart which occurs on the skin. A viral cause has been suggested. Some
juvenile papillomas resolve spontaneously about the time of puberty due to
hormonal influences. Many adult patients have suffered papillomas since early
childhood.
Diagnosis. This is made by indirect laryngoscopy, direct laryngoscopy and
histologic examination. Papillomas may be pedicled, solitary, or widespread.
Their surface is pale-yellow to red, granular, villous, and often has a
raspberry appearance.
Other areas of papillomatosis may lie in the oropharynx and the subglottic
space.
Treatment. Spontaneous regression rarely occurs. Treatment is combined. The
immunologic and antiviral treatment are used. Today there is no alternative to
surgery. Microsurgery is being progressively replaced by the laser. The problem
of surgery is the marked tendency of papillomas to recur, the appearance of new
foci, and the interference with the function of the vocal cords caused by
defects and scars due to repeated operations.
MALIGNANT TUMOURS
Laryngeal Carcinoma
Laryngeal carcinoma forms about 45% of carcinomas of the head and neck. It is
most common between the ages of 45 and 75 years. At the present time men are ten
times more frequently affected than women, although in the last few decades the
number of female patients has increased due to increased incidence of smoking in
women.
TNM-system for the Larynx
T – Tumor
T1 - Tumor is confined one anatomical site within the larynx
T2 – Tumor is confined one region of the larynx
T3 – Tumor extend beyond one region but it still confined to the larynx
(fixation of the vocal cord)
T4 – Tumor extend beyond the larynx
N – Regional lymph nodes metastases (for nose, pharynx and larynx)
N0 – none
N1 – mobile homolateral nodes
N2 – mobile contralateral or bilateral nodes
N3 – fixed nodes
M – Distant metastases
M0 – none
M1 – present
Regions Sites
Supraglottic lower part epiglottis
false cords
ventricles
arytenoids
Glottic vocal cords
anterior and posterior commissures
Subglottic walls of subglottis
Symptoms. Hoarseness is the first and main symptom when the tumor affects the
glottis. Further symptoms, which may occur alone or in combination depending
upon site and extent, include a feeling of a foreign body, clearing the throat,
pain in the throat or referred elsewhere, dyspnea, dysphagia, cough, and
hemoptysis. Regional lymph node metastases may also occur.
Hoarseness persisting for more than 2 to 3 weeks must always be investigated by
a specialist, and omission of this step is dangerous.
Pathogenesis. Invasive carcinoma may develop from epithelial dysplasia
especially from carcinoma in situ. More than 90% of laryngeal carcinomas are
keratinizing or nonkeratinizing squamous cell carcinomas. Unusual forms include
verrucous carcinoma, adenocarcinoma, carcinosarcoma, fibrosarcoma, and
chondrosarcoma.
Most patients with squamous carcinoma of the larynx were or are heavy cigarette
smokers and, in addition, often heavy drinkers. Chronic exposure to irritation
with heavy metals such as chromium, nickel, uranium, or asbestos, and
irradiation are rarer causes.
Laryngeal carcinoma infiltrates locally in the mucosa and beneath the mucosa and
metastasizes via the lymphatics and the bloodstream. The limits of vascular
spread are embryologically determined. Thus, supraglottic carcinomas usually
remain confined to the supraglottic space and spread anteriorly into the
preepiglottic space, whereas glottic carcinomas seldom spread into the
supraglottic area but rather into the subglottic space. A transglottic carcinoma
is a glottic carcinoma involving the ventricle and the vestibular folds in which
the site of origin can no longer be recognized. The characteristics of the
intralaryngeal lymphatics influence the frequency of regional lymph node
metastases. Other factors influencing the frequency of metastases are the
duration of the symptoms, the histologic differentiation, and the size and site
of the tumor. Lymph node metastases at the time of presentation are very rare in
carcinomas of the vocal cord, but are found in about 20% of subglottic
carcinomas, about 40% of supraglottic carcinomas, and in about 40% of
transglottic carcinomas.
Contralateral metastases are unusual in unilateral glottic tumors. Bilateral
metastases become more common if the carcinoma crosses the midline, e.g., at the
anterior or posterior commissure or in the trachea, or if the tumor arises
primarily in the supraglottic space.
Distant hematogenous metastases are relatively unusual in laryngeal carcinoma at
the time the patient is first seen. Second primary carcinomas of the respiratory
and digestive tracts also occur.
Diagnosis. The clinical diagnosis rests initially on the findings of indirect
laryngoscopy and telescopic laryngoscopy. The site and extent of the tumor and
the mobility of the vocal cord must be assessed. It is very important to carry
out microlaryngoscopy. This allows accurate evaluation of the site and extent of
the tumor, provides a view of hidden angles such as the ventricle and the
piriform sinus, and allows assessment of the superficial characteristics such as
nodular, exophytic, granulomatous, ulcerating, etc. Biopsy and histological
examination should be carried out.
Differential diagnosis. It includes chronic laryngitis and its specific forms,
and benign laryngeal tumors.
Treatment. If untreated, laryngeal carcinoma leads to death within an average of
12 months by asphyxia, bleeding, metastases, infection, or cachexia. The
existence of cardiovascular or pulmonary diseases and diabetes mellitus
determines the course of treatment and the course of the disease. The
indications for radiotherapy or surgery for laryngeal carcinoma vary depending
on the site and stage of the tumor. They are often used in combination.
Chemotherapy alone has so far proved to be useless for this type of tumor.
Radiotherapy achieves similar results to surgery for T1N0 glottic tumors and for
some T2N0 tumors. Radiotherapy must also be used for patients with inoperable
tumors or those unwilling to undergo surgery.
For all other sites and stages of tumor, especially if lymph node metastases are
present, surgery is clearly superior to radiotherapy.
Both methods of treatment may be combined, e.g., pre- or postoperative
radiotherapy or sandwich radiotherapy. This latter form of treatment gives the
best result for selected patients in advanced stages.
Complications after radiography include persistent edema which makes it
difficult to assess the local appearances and detect a recurrence. The edema is
usually due to chondroradionecrosis leading to cartilaginous necrosis and which
may require laryngectomy. Other complications include dysphagia, ageusia,
xerostomia and the sicca syndrome, recurrent tumor, or lymph node metastases. if
surgery must be undertaken after a full course of radiotherapy, the wound
healing and prognosis are considerably worse.
Surgical Procedures for Laryngeal Carcinoma
If larynx stenosis take place (3 or 4 stage) tracheostomy should be done.
1. Microsurgical decortication of the vocal cord is indicated for severe
dysplasia and some carcinomas in situ.
2. Cordectomy is indicated for a vocal cord carcinoma with a mobile vocal cord.
The breathing is normal after this operation. The voice is rough or hoarse
postoperatively, but may return to normal after several months as scar tissue
forms a pseudocord. Cordectomy can be carried out during direct laryngoscopy
also.
3. Vertical or horizontal partial laryngectomies are used for carcinomas for
which a cordectomy is not suitable because of the extent or site of the tumor,
but for which total laryngectomy is not necessary. Partial laryngectomies
preserve the vocal function and a normal airway. The prerequisites for success
are careful assessment and good surgical judgment to ensure that the tumor is
removed completely.
4. Total laryngectomy may on occasion be combined with removal of the
hypopharynx. This technique is indicated for tumors that cannot be removed by
cordectomy or partial laryngectomy and for tumors that have spread to
neighboring structures such as the tongue, the hypopharynx, the thyroid gland
and the trachea. Total laryngectomy is also indicated for tumors that have
recurred after radiotherapy or partial procedures.
SCLEROMA
Scleroma of the Nose
Scleroma is found in certain parts of Eastern Europe, Indonesia and Central and
Southern America, and is due to Klebsiella rhinoscleromatosis (bacilli of
Volcovich-Frich). It consists of a hard rubbery nodular infiltration of the
mucous membrane, reddish-brown in colour at first, but becoming pale pink later
when fibrosis occurs. There is nasal obstruction, crusted discharge and a slowly
progressive, but painless, stenosis of the nasal cavity. Prolonged antibiotic
treatment by ampicillin, streptomycin or tetracycline may be helpful. Local
excision of the lesions tends to destroy healthy tissue, and stenosis may be
treated by dilatation of the nasal cavities and the insertion of polythene tubes
which are retained for up to 2 months.
Scleroma of the Larynx
This disease starts in the nose and oral cavity and spreads downwards to involve
the pharynx and larynx. The typical lesion is in the subglottic region and takes
the form of a smooth red swelling covered by crusts. The patient complains of
nasal obstruction followed later by hoarseness, wheezing and stridor. Diagnosis
is made by biopsy when plasma cells and hyaline bodies are seen in granulation
tissue together with the diagnostic Mikulicz cells which are large cells looking
like enormous fat deposits. If it is untreated the condition progresses to
laryngotracheal stenosis. The first line of treatment is streptomycin with
steroids added if there is a danger of stenosis.
SYPHILIS
Laryngeal Syphilis
Isolated laryngeal syphilis is unusual, and it is much more often a
manifestation of oropharyngeal syphilis in the secondary generalized stage of
the disease.
Mucous plaques or hazy, smoke-colored mucosal lesions occur in the larynx
similar to those of syphilitic pharyngitis. The patient is also hoarse. The
disease is reportable.
Respiratory obstruction only occurs in the presence of marked mucosal swelling.
The cartilage is destroyed in a gumma in stage III. The differential diagnosis
from carcinoma is difficult to make. Diagnosis is by biopsy, but this must be
confirmed by the appropriate serological tests. Treatment should be supervised
by appropriate specialist.
Nasal Syphilis
Inherited Syphilis
This may appear in the form of coryza (snuffles) beginning in the first 3 months
of life. It is characterized by an obstinate nasal discharge which tends to dry
up and form crusts, while the irritation of the secretion causes fissures to
appear at the anterior nares. At a later stage, usually at puberty but sometimes
not until adult life, gummatous ulceration may destroy the tissues and lead to
atrophy and scarring, so that the bridge of the nose is depressed and foetid
crusts form within the nasal cavity. The permanent teeth and the cornea show the
well-known defects. In infants so affected other evidence of the disease should
be looked for and the family history must also be investigated.
Acquired Syphilis
Primary infections of the nose are very rare. Secondary lesions in the form of
mucous patches are met with less commonly than in the pharynx, and only slight
symptoms are produced. In its tertiary form syphilis may occur in the nasal
cavities. The septum is most commonly involved, but the lateral wall of the nose
may also be affected. The stage of gummatous infiltration is rarely seen
because, as a rule, ulceration and destruction of tissue have taken place before
the patient is examined. The gumma takes the form of an irregular mamillated
infiltration, dark red in colour, involving one or both sides of the septum.
Usually at this stage the only symptom is nasal obstruction, but there may be
headache and severe pain in the nose, which may be swollen and tender. When
ulceration occurs it is accompanied by a purulent discharge which tends to dry
and form crusts which emit a horrible stench. After removal of the crusts by
douching, the nose may be more fully inspected. If the septum is affected it
will be found to be perforated, and the perforation usually involves the bony
structures as well as the cartilaginous portion. If the process is still active
the edges of the perforation will be covered with granulations. The loss of
tissue may be so extensive that there may be sinking of the bridge of the nose
and even ulceration and destruction of the external nose. The structures of the
lateral wall of the nasal cavity may also be extensively ulcerated and in part
destroyed.
Diagnosis. When a granular lesion, especially one associated with foetid crusts,
is found on the nasal septum syphilis should be suspected. The diagnosis is made
on serological testing.
Treatment. Penicillin is the treatment of choice. The nose should be kept clean
by frequent douching, and all loose sequestra should be removed.
Syphilis of the Pharynx
Primary syphilis is uncommon, but the tonsil is second to the lip as the
frequent extragenital site. The chancre is unilateral, persists for several
weeks and is accompanied by enlarged cervical glands. Palpation by a gloved
fingers will disclose that the lesion is of cartilaginous hardness. The
discovery of Treponema pallidum may confirm the diagnosis.
Secondary syphilis in the pharynx is much more common and much more important in
that it is most contagious because the lesion teems with spirochaetes. Initially
there is congestion of the palate and fauces, and some tonsillar enlargement,
but soon the mucous patch develops. This may be found on any part of the mucosa
of the mouth or pharynx, the principal sites being, in order of frequency, the
tonsil, the palatine arches, the tongue and the inner aspect of the lips. The
patch is round or oval, bluish-grey in colour with a surrounding zone of
congestion. The patches may be multiple and symmetrical, and may become
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