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3 Peculiar Patterns ofSpread ofNose Vestibule Malignancies
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Fig. 3.8 A less frequent case: the visible lesion on the skin arises from the internal surface of the
lateral alar cartilage, and from here it comes to invade the skin
3.4 Typical Patterns ofSpread fromtheMedial Wall
When a primary lesion of the medial wall of the vestibule spreads posteriorly, it
typically runs along the septal cartilage (Fig. 3.9). For the above cited cartilage
resistance, septal perforation is rare except in very bulky primaries or as a consequence of extensive tissue sampling during a diagnostic biopsy. Such posterior
spread is not an issue until it remains within the anatomical limits of the nose vestibule (anterior to the coronal plane of pyriform aperture) (Fig.3.9). Septal/columellar primaries can also share the inferior pattern of spread of inferior wall primaries.

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Fig. 3.9 Posterior spread from the septum leading to a cT3 lesion according to our newly proposed T classication
F. Bussu et al.
3.5 Typical Patterns ofSpread fromtheInferior Wall
The inferior spread of nose vestibule primaries (mainly from the inferior and medial
walls) typically involves the superior lip (Figs.3.10 and 3.11). It is therefore another
typical route for skin invasion in nose vestibule malignancies.
Primaries of the inferior wall can also spread posteriorly; in this case, as the
anteroposterior dimension of the inferior wall is minimal (see Fig.3.2), it is associated to two orders of issues:
– invasion of the nasal spine and/or of the hard palate with bone involvement
(Fig. 3.12), which determines an upstaging according to Wang classication and
to our newly proposed T classication [10–12], and most probably a prognostic
impairment;
– overcoming of the plane of the pyriform opening, the newly proposed posterior
limit of the nose vestibule (Fig.3.9), which in our opinion should determine an
upstaging of the lesion as well (see Chap. 5) as it determines clear issues for
treatment by exclusive interstitial brachytherapy (see Chap. 12), which is being
demonstrated to be a valuable option in this group of malignancies [10–12].

3 Peculiar Patterns ofSpread ofNose Vestibule Malignancies
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Fig. 3.10 A typical route
of invasion involving the
upper lip
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F. Bussu et al.
Fig. 3.11 The tumor invades the lter and runs to the upper lip

3 Peculiar Patterns ofSpread ofNose Vestibule Malignancies
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Fig. 3.12 Inferior
spread: invasion of the
nasal spine and/or hard
palate
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References
1. Jeannon J-P, Riddle PJ, Irish J, O’sullivan B, Brown DH, Gullane P.Prognostic indicators in
carcinoma of the nasal vestibule. Clin Otolaryngol. 2007;32:19–23.
2. Bussu F, Tagliaferri L, Mattiucci G, etal. Comparison of interstitial brachytherapy and surgery
as primary treatments for nasal vestibule carcinomas. Laryngoscope. 2016;126:367–71.
3. Mor N, Blitzer A.Functional anatomy and oncologic barriers of the larynx. Otolaryngol Clin
North Am. 2015;48:533–45.
4. Sandell LJ.Novel functions for type II procollagen. Connect Tissue Res. 2014;55:20–5.
5. Caccialanza M, Piccinno R, Percivalle S, Rozza M.Radiotherapy of carcinomas of the skin
overlying the cartilage of the nose: our experience in 671 lesions. J Eur Acad Dermatol
Venereol. 2009;23:1044–9.
6. Caccialanza M, Piccinno R, Moretti D, Rozza M.Radiotherapy of carcinomas of the skin over-
lying the cartilage of the nose: results in 405 lesions. Eur J Dermatol. 2003;13:462–5.
7. Caccialanza M, Piccinno R, Gaiani F, Contini D.Relevance of dermatologic radiotherapy in the
therapeutic strategy of skin epithelial neoplasms: excellent results in the treatment of lesions
localized on eyelids and skin overlying the cartilage of the nose. G Ital Dermatol Venereol.
2013;148:83–8.
8. Caccialanza M, Bertani E, Piccinno R, Rozza M, Brambilla R, Percivalle S.Radiotherapy of
T4 squamous cell carcinoma of the skin of nasal pyramid. J Eur Acad Dermatol Venereol.
2006;0:060606032107062.
9. Bussu F, Tagliaferri L, De Corso E, et al. Functional results of exclusive interventional
radiotherapy (brachytherapy) in the treatment of nasal vestibule carcinomas. Brachytherapy.
2021;20:178–84.

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10. Bussu F, Tagliaferri L, Piras A, Rizzo D, Tsatsaris N, De Corso E, Parrilla C, Paludetti
G. Multidisciplinary approach to nose vestibule malignancies: setting new standards
approccio multidisciplinare ai tumori maligni del vestibolo del naso: verso la denizione
di nuovi standard. Acta Otorhinolaryngol Ital. 2021;41(1):S158–S165. https://doi.
org/10.14639/0392-100X-suppl.1-41-2021-16.
11. Bussu F. New standards for the management of nose vestibule malignancies. Acta Oto-
Laryngologica. 2023;143(3):215–22. https://doi.org/10.1080/00016489.2023.2179662.
12. Scheurleer WFJ, Tagliaferri L, Rijken JA, Crescio C, Rizzo D, Mattiucci GC, Pameijer FA, de
Bree R, Fionda B, de Ridder M, Bussu F. Evaluation of staging systems for cancer of the nasal
vestibule. Cancers. 2023;15(11):3028. https://doi.org/10.3390/cancers15113028.
F. Bussu et al.

Current Staging Systems forNose
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Vestibule Malignancies
AntonioPiras, DavideRizzo, EmiliaDegni, ClaudiaCrescio,
AlessiaRe, GiovanniMariaFadda, GiancarloMattiucci,
andAntonioPazzola
4.1 Introduction
4.1.1 Principles ofTNM Classification
In the management of cancer, an accurate and reliable staging of the tumor holds the
key to successful treatment strategies, to the prediction of clinical outcomes, and to
international communication.
The tumor-node-metastasis (TNM) staging system describes the anatomic extent
of the primary tumor (T) as well as the involvement of the regional lymph nodes (N)
and distant metastasis (M). It should be based on a thorough knowledge of the
natural history of tumors at various sites and subsites and on a clear denition of
4
A. Piras · D. Rizzo (*)
Otolaryngology Division, Sassari University Hospital, Sassari, Italy
Department of Medicine, Surgery and Pharmacy, University of Sassari, Sassari, Italy
e-mail: davide.rizzo@aouss.it; drizzo@uniss.it
E. Degni · C. Crescio
Otolaryngology Division, Sassari University Hospital, Sassari, Italy
e-mail: emilia.degni@aouss.it; claudia.crescio@aouss.it
A. Re
Radiation Oncology Unit, Mater Olbia Hospital, Olbia, Italy
G. M. Fadda · A. Pazzola
Medical Oncology Division, Sassari University Hospital, Sassari, Italy
e-mail: giovanni.fadda@aouss.it; antonio.pazzola@aouss.it
G. Mattiucci
Radiation Oncology Unit, Mater Olbia Hospital, Olbia, Italy
Università Cattolica del Sacro Cuore, Istituto di Radiologia, Rome, Italy
e-mail: giancarlo.mattiucci@unicatt.it
© Springer Nature Switzerland AG 2023
F. Bussu (ed.), Malignancies of the Nasal Vestibule,
https://doi.org/10.1007/978-3-031-32850-3_4
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A. Piras et al.
prognostic parameters, as it is expected, rst of all, to properly stratify prognosis
and hence to guide therapeutic decision.
TNM was developed and is periodically updated by the Union for International
Cancer Control (UICC). It is also used by the American Joint Committee on Cancer
(AJCC) and by the International Federation of Gynecology and Obstetrics (FIGO).
In 1987, the UICC and AJCC staging systems were unied into a single TNM staging system. The generation of scientic evidence leads to continuous updates in the
TNM system for all the malignancies.
4.1.2 Current UICC/AJCC TNM Classification forNose
andParanasal Sinuses Malignancies
Like many other cancers, a fully satisfying stratication of nose/paranasal malignancies has been challenging, mainly because of the numerous anatomic sites and
subsites from which tumors can arise and because of the heterogeneity of histologic
types that show different clinical behavior and outcomes.
T classication of nose and paranasal sinuses follows two different orders of
criteria for maxillary sinus primaries on the one hand and nasal cavities/ethmoid
primaries on the other (Tables 4.1 and 4.2).
Many histotypes exist and are also acknowledged as key factors when determining the prognosis. Melanoma, lymphoma, and sarcoma are not included in the
UICC/AJCC staging of nose/paranasal sinuses primaries.
The nasoethmoidal complex is divided into two subsites: the nasal cavity and the
ethmoid sinuses. The ethmoids are further subdivided into two subsites: left and
right, separated by the nasal septum (perpendicular plate of ethmoid). The nasal
Table 4.1 UICC/AJCC 2017, Eighth Edition. Classication of Primary Tumor (T): Maxillary Sinus
T category T criteria
TX Primary tumor cannot be assessed
Tis Carcinoma in situ
T1 Tumor limited to maxillary sinus mucosa with no erosion or destruction of bone
T2 Tumor causing bone erosion or destruction including extension into the hard palate
T3 Tumor invades any of the following: bone of the posterior wall of the maxillary
T4 Moderately advanced or very advanced local disease
• T4a Moderately advanced local disease
• T4b Very advances local disease
and/or middle nasal meatus, except extension to posterior wall of maxillary sinus
and pterygoid plates
sinus, subcutaneous tissues, oor or medial wall of orbit, pterygoid fossa, ethmoid
sinuses
Tumor invades anterior orbital contents, skin of cheek, pterygoid plates,
infratemporal fossa, cribriform plate, sphenoid or frontal sinuses
Tumor invades any of the following: orbital apex, dura, brain, middle cranial fossa,
cranial nerves other than maxillary division of trigeminal nerve (V2), nasopharynx
or clivus

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Table 4.2
and ethmoid sinus
T category T criteria
TX Primary tumor cannot be assessed
Tis Carcinoma in situ
T1 Tumor restricted to any one subsite, with or without bony invasion
T2 Tumor invading two subsites in a single region or extending to involve an adjacent
T3 Tumor extends to invade the medial wall or oor of the orbit, maxillary sinus,
T4 Moderately advanced or very advanced local disease
• T4a Moderately advanced local disease
• T4b Very advanced local disease
UICC/AJCC 2017, Eighth Edition. Classication of Primary Tumor (T): Nasal cavity
region within the nasoethmoidal complex, with or without bony invasion
palate, or cribriform plate
Tumor invades any of the following: anterior orbital contents, skin of the nose or
cheek, minimal extension to anterior cranial fossa, pterygoid plates, sphenoid or
frontal sinuses
Tumor invades any of the following: orbital apex, dura, brain, middle cranial fossa,
cranial nerves other than maxillary division of trigeminal nerve (V2), nasopharynx
or clivus
cavity is divided into four subsites: the septum, the oor, the lateral wall, and the
edge of the naris to the mucocutaneous junction, but without any differences in the
denition of c- and/or p-T between such different subsites, which are all included in
the same topographic code C30.0 (Tables 4.5 and 4.6).
4.1.3 Evolution ofUICC/AJCC TNM ofNose andParanasal Sinus
The current complete 8th UICC/AJCC staging system (2017) for maxillary sinus
and nasoethmoid lesions is therefore shown in Tables 4.1, 4.2, 4.3, and 4.4 [1].
The T category (Tables 4.1 and 4.2) is identical to the UICC/AJCC Cancer
Staging Manual 7th edition (2009) [2], according to the changes introduced in the
6th edition (2002) concerning the classication of the nasal cavity and paranasal
malignancies [3]. Here a new site, the nasal cavity (C30.0), was added for inclusion
into the staging system of nose malignancies. In the 6th UICC/AJCC edition, in
addition to maxillary sinus, the nasal cavity was merged with the ethmoid in the
same T classication, forming the nasoethmoid complex, divided into two regions:
the nasal cavity (four subsites: septum, oor, lateral wall, and vestibule) and the
ethmoid sinuses (two subsites: right and left).
The sites in this classication are listed by code numbers of the International
Classication of Diseases for oncology (Chapter 2 of the 2019 edition of the
ICD-10-CM, World Health Organization) [4] (Tables 4.5 and 4.6).
As shown, the nasal vestibule (NV) is included in C30.0 code, the same “billable” ICD code used to specify a diagnosis of malignant neoplasm everywhere in
the nasal cavity, considering NV only an unspecied subsite of the nasal cavity [5, 6].

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Table 4.3
N category N criteria
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Metastasis in a single ipsilateral lymph node, 3cm or smaller in greatest
N2 Metastasis in a single ipsilateral node larger than 3cm but no larger than 6cm in
• N2a Metastasis in a single ipsilateral node larger than 3cm but no larger than 6cm in
• N2b Metastasis in multiple ipsilateral lymph node, none larger than 6cm in greatest
• N2c Metastasis in bilateral or contralateral lymph nodes, none larger than 6cm in
N3
• N3a
• N3b Metastasis in any node(s) with clinically overt ENE (+)
ENE extranodal extension
Table 4.4
N category N criteria
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Metastasis in a single ipsilateral lymph node, 3cm or smaller in greatest
N2 Metastasis in a single ipsilateral node, 3cm or smaller in greatest dimension and
• N2a Metastasis in single ipsilateral or contralateral node 3cm or less in greatest
• N2b Metastasis in multiple ipsilateral lymph node, none larger than 6cm in greatest
• N2c Metastasis in bilateral or contralateral lymph nodes, none larger than 6cm in
N3
• N3a
• N3b Metastasis in a single ipsilateral node larger than 3cm in greatest dimension and
Denition of regional lymph node (N): Clinical N (cN)
dimension and ENE (−)
greatest dimension and ENE (−); or metastasis in multiple ipsilateral lymph node,
none larger than 6cm in greatest dimension and ENE (−); or in bilateral or
contralateral lymph nodes, none larger than 6cm in greatest dimension and ENE
(−)
greatest dimension and ENE (−)
dimension and ENE (−)
greatest dimension and ENE (−)
Metastasis in a lymph node larger than 6cm in greatest dimension and ENE (−);
or metastasis in any node(s) with clinically overt ENE (+)
Metastasis in a lymph node larger than 6cm in greatest dimension and ENE (−)
Denition of regional lymph node (N): Pathological N (pN)
dimension and ENE (−)
ENE (+); or larger than 3cm but no larger than 6cm in greatest dimension and
ENE (−); or metastasis in multiple ipsilateral lymph node, none larger than 6cm
in greatest dimension and ENE (−); or in bilateral or contralateral lymph nodes,
none larger than 6cm in greatest dimension and ENE (−)
dimensión and ENE(+); or a single ipsilateral node larger than 3cm but not larger
than 6cm in greatest dimensión and E N E (−)
dimension and ENE (−)
greatest dimension and ENE (−)
Metastasis in a lymph node larger than 6cm in greatest dimension and ENE (−);
or in a single ipsilateral node larger than 3cm in greatest dimension and ENE (+);
or multiple ipsilateral, contralateral or bilateral node, any with ENE (+)
Metastasis in a lymph node larger than 6cm in greatest dimension and ENE (−)
ENE (+); or multiple ipsilateral, contralateral or bilateral nodes, ant with ENE (+)
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