par Jason Wasserman MD PhD FRCPC
5 mai 2026
Carcinome sécrétoire is a type of cancer that starts in the salivary glands — the glands that make saliva. It is one of the more recently described salivary gland cancers, and it has a few features that set it apart. First, it almost always carries the same specific change in its DNA — a la fusion of two genes that drives tumor growth. Second, that genetic change can be matched with a targeted drug, which means a small but important number of patients with advanced secretory carcinoma can be treated with a drug aimed at the cancer’s underlying cause. Third, secretory carcinoma is closely related to a similar tumor that occurs in the breast and shares the same DNA change — a connection that is reflected in the tumor’s older name (described below).
Cet article vous aidera à comprendre les résultats de votre rapport d'anatomopathologie : la signification de chaque terme et son importance pour vos soins.
Une remarque sur le nom
Le nom de cette tumeur a changé au fil des ans, et vous pourriez rencontrer des termes différents dans les rapports plus anciens :
- Secretory carcinoma — The current World Health Organization (WHO) name, in use since 2017 and confirmed in the 2022 update.
- Mammary analogue secretory carcinoma (MASC) — The original name when the tumor was first described in 2010. The word “mammary” refers to breast tissue, and the name reflected the discovery that this salivary gland tumor shares the same DNA change as a similar tumor of the breast.
- Acinic cell carcinoma — Before 2010, many secretory carcinomas were grouped with carcinome à cellules acineuses, another salivary gland cancer that looks similar under the microscope. If you have an older report (before about 2010 to 2015) that says “acinic cell carcinoma,” it is worth asking whether the tumor would be reclassified as secretory carcinoma today. This question is more than academic — secretory carcinoma can be treated with a class of drugs that does not work for true acinic cell carcinoma, as described later in this article.
Quelles sont les causes du carcinome sécrétoire ?
The cause of secretory carcinoma is not known in most cases. It is not linked to smoking, alcohol, infection, or any other lifestyle or environmental factor. We do know, however, that almost every secretory carcinoma has a specific change in its DNA. The most common change is a la fusion entre deux gènes appelés ETV6 et NTRK3. A fusion happens when two genes that are normally far apart on different chromosomes break and join together. The new combined gene then behaves abnormally. The ETV6-NTRK3 fusion creates an abnormal protein that tells the tumor cells to keep dividing, even when they should stop. A small number of secretory carcinomas have a fusion involving ETV6 et the RET gene, or, rarely, a fusion involving RET au another partner. These tumors behave the same way. These genetic changes happen by chance during a person’s lifetime. They are not inherited and cannot be passed to children.
Where does secretory carcinoma start?
Secretory carcinoma can start in any salivary gland, but is most often found in the parotid gland, which sits in front of and just below each ear. About 70% of secretory carcinomas arise in the parotid gland. The rest occur in the small minor salivary glands distributed throughout the lining of the mouth and throat — particularly in the inside of the cheek, the lip, and the palate (the roof of the mouth) — or in the submandibular gland (under the jaw). Tumors with the same DNA change can also occur in the breast (where they are called secretory carcinoma of the breast) and, rarely, in the skin and thyroid.
Secretory carcinoma can occur at any age but is most common between ages 30 and 60. It is slightly more common in men than in women.
Quels sont les symptômes du carcinome sécrétoire ?
Most secretory carcinomas grow slowly and produce few symptoms in the early stages:
- Grosse indolore — A slow-growing, painless mass in the salivary gland is the most common finding by far. In the parotid gland, the lump is felt under the skin in front of or below the ear. In a minor salivary gland, it appears as a firm bulge inside the mouth.
- Douleur ou sensibilité — Au début, ce n'est pas fréquent. L'apparition d'une douleur peut être un signe avant-coureur indiquant que la tumeur a envahi un nerf ou a subi une transformation de haut grade.
- Engourdissement ou faiblesse faciale — The facial nerve runs through the parotid gland. Tumors that press on or invade this nerve can cause weakness or paralysis of part of the face. This is uncommon in secretory carcinoma and, when present, suggests a more aggressive tumor.
- Difficultés à avaler ou à parler — Larger tumors of the minor salivary glands can interfere with normal mouth function.
- Gonflement du cou — Sometimes caused by the spread of the tumor to nearby lymph nodes.
Comment le diagnostic est-il posé?
Le diagnostic est établi après l'examen d'un échantillon de tissu au microscope par un médecin. pathologisteLa plupart des patients subissent d'abord un examen d'imagerie — généralement une échographie, un scanner ou une IRM — qui révèle une masse dans la glande salivaire. biopsie par aspiration à l'aiguille fine (FNAB) On procède souvent en premier lieu à une ponction à l'aiguille fine pour prélever un petit échantillon de cellules. Si la cytoponction à l'aiguille fine ne donne pas de résultat clair, on effectue une biopsie à l'aiguille. biopsie Cette méthode peut être utilisée à la place. Dans de nombreux cas, la tumeur entière est retirée en une seule intervention, et le diagnostic est établi sur cet échantillon plus important.
Under the microscope, the pathologist looks for a tumor made up of large cells with pink (eosinophilic) cytoplasm. The cells have round nuclei (the part of the cell that holds DNA), and most cells have a small dot called a nucléole in the center of the nucleus. The tumor cells are arranged in several typical patterns, often within the same tumor — they may form small round structures called tubules, large open spaces called Kystes, finger-like projections called papillae, or solid sheets. The hollow spaces of the tubules and cysts often contain a pink or blue fluid produced by tumor cells, which is why they are called “secretory” (the cells secrete a fluid). Figures mitotiques (cells in the act of dividing) are present but usually few.
Once the diagnosis is suspected, the pathologist confirms it using immunohistochimie, a staining technique that highlights specific proteins in tumor cells. Almost all secretory carcinomas show strong staining for two proteins called S100 and mammaglobin. The combination is one of the most reliable signs of the diagnosis. Other commonly observed proteins include SOX10 and cytokeratin 7. Secretory carcinoma is typically negative for proteins called p63 and DOG1, which are usually positive in other salivary gland tumors that can look similar under the microscope. The pattern of positive and negative stains, taken together, helps the pathologist tell secretory carcinoma apart from these other tumors. In some cases, additional molecular testing is performed to identify the underlying gene fusion, as described in the biomarker and molecular testing section below.
Transformation de haut niveau
High-grade transformation means that part of the tumor has changed into a much more aggressive form of cancer. In areas of high-grade transformation, the tumor cells lose the typical features of secretory carcinoma. They become very abnormal looking (atypique et pléomorphe), divide rapidly (with many mitotic figures), and often show areas of nécrose (cell death). High-grade transformation is uncommon in secretory carcinoma, but when it is present, the tumor is much more likely to spread to lymph nodes and to distant sites such as the lungs. Treatment is usually stronger when this finding is reported, often including a neck dissection (removal of lymph nodes from the neck) and radiation therapy after surgery.
Extension tumorale (extension extraparenchymateuse)
L'extension extraparenchymateuse signifie que la tumeur s'est propagée au-delà de la glande salivaire, dans les tissus environnants tels que le tissu adipeux, les muscles ou la peau. Ce phénomène est observé uniquement pour les tumeurs qui se développent dans l'une des trois glandes salivaires principales : la parotide, la sous-maxillaire ou la sublinguale. Les tumeurs présentant une extension extraparenchymateuse sont classées à un stade pathologique plus élevé (pT) et présentent un risque accru de récidive après l'intervention chirurgicale.
Invasion lymphovasculaire
Invasion lymphovasculaire means that tumor cells have entered small blood vessels or lymphatic vessels in or near the tumor. These vessels can carry the cells to lymph nodes or to distant parts of the body. When found, lymphovascular invasion raises the risk that the cancer will come back, and it may influence the decision to recommend radiation therapy after surgery.
Invasion périneurale
Invasion périneurale means that tumor cells are growing around or along a nerve. The facial nerve, which controls the muscles of facial expression, runs through the parotid gland and is the most commonly involved nerve when secretory carcinoma arises there. Perineural invasion can cause new pain, numbness, or facial weakness. When seen in a pathology report, it raises the risk that the tumor will come back near the original site, and your doctor may recommend radiation therapy after surgery to lower that risk.
Marges chirurgicales
A marge Il s'agit du bord du tissu que le chirurgien incise lors de l'ablation de la tumeur. Le pathologiste examine ces bords au microscope afin de déterminer si des cellules tumorales atteignent la surface de coupe.
- Marge négative — Aucune cellule tumorale n'est visible au niveau de la plaie. Cela suggère que la tumeur a été complètement retirée et que le risque de récidive est très faible.
- Marge réduite — Les cellules tumorales sont très proches de la marge de résection, sans toutefois l'atteindre. Le pathologiste pourra indiquer la distance exacte en millimètres. Une marge étroite peut accroître le risque de récidive tumorale à proximité du site initial et justifier une radiothérapie post-opératoire.
- Marge positive — Des cellules tumorales sont visibles au niveau de la marge de résection. Cela signifie que des cellules tumorales ont très probablement été laissées en place. Une marge positive conduit généralement à recommander soit une nouvelle intervention chirurgicale, soit une radiothérapie postopératoire.
L'évaluation des marges est particulièrement difficile en chirurgie parotidienne car le chirurgien doit contourner le nerf facial. C'est pourquoi des marges étroites sont fréquentes, même lorsque l'intervention a été réalisée avec soin.
Ganglions
Lymph nodes are small immune organs scattered throughout the body. The lymph nodes most likely to be involved by secretory carcinoma are those in the neck. During surgery, lymph nodes near the tumor may be removed and sent to the laboratory in a procedure called a neck dissection. This is more often performed when high-grade transformation is present, when the tumor is large, or when imaging or examination suggests that lymph nodes may be involved.
- Ganglion lymphatique négatif — Aucune cellule tumorale n'a été trouvée dans le ganglion.
- Ganglion lymphatique positif — Des cellules tumorales sont présentes à l'intérieur du ganglion. Le rapport précisera le nombre de ganglions contenant la tumeur, la taille du plus grand dépôt et si la tumeur s'est étendue au-delà de la paroi externe du ganglion (extension extraganglionnaire).
Spread to lymph nodes is uncommon in classic secretory carcinoma but is much more frequent when high-grade transformation is present.
Tests de biomarqueurs et de biologie moléculaire
A biomarker is something that can be measured in a tumor sample — most often a protein on the surface of the tumor cells or a change in the tumor’s DNA — that helps doctors predict how the tumor will behave or decide whether a treatment is likely to work. In some cancer types, biomarker testing is routinely performed because the results determine which drugs will be used. Secretory carcinoma is one of the few salivary gland cancers where this is true: the same gene fusion that defines the disease is also the target of an approved class of drugs. Biomarker testing is most important for patients whose tumor has recurred, spread, or cannot be removed by surgery, but it also confirms the diagnosis when other findings leave the diagnosis uncertain.
ETV6-NTRK3 and ETV6-RET fusion testing
Molecular testing looks for the specific gene fusion that drives secretory carcinoma. The most common fusion involves the ETV6 et NTRK3 genes — about 95% of secretory carcinomas have this fusion. A smaller number have a fusion involving ETV6 et RET, or, rarely, RET with another partner gene. The tests used to find these fusions include fluorescence in situ hybridization (FISH) and next-generation sequencing (NGS). Detecting one of these fusions confirms the diagnosis with very high accuracy. For patients with advanced disease (described in the next section), the same test result also identifies them as candidates for targeted drug therapy.
Thérapie médicamenteuse ciblée
The fusion that defines secretory carcinoma is more than a diagnostic clue — it is also a treatment target. ETV6-NTRK3 et d'autres NTRK fusions can be blocked by a class of drugs called NTRK inhibitors. Two NTRK inhibitors, larotrectinib and entrectinib, are approved for any cancer that has an NTRK fusion, which includes secretory carcinoma. Patients with advanced, recurrent, or unresectable secretory carcinoma can have substantial and long-lasting responses to these drugs. Tumors with the rarer RET fusion can be treated with a different class of drugs called RET inhibitors (selpercatinib and pralsetinib), which are approved for cancers with RET rearrangements. Most patients with secretory carcinoma do not need targeted drug therapy, because surgery alone is curative — but for patients whose tumor has come back or has spread, this is one of the most important conversations to have with the medical team.
Stade pathologique (pTNM)
Pathologic staging describes the size of the tumor and how far it has spread, based on the findings at surgery. It uses the TNM system: T stands for the size and extent of the primary tumor, N stands for involvement of nearby lymph nodes, and M stands for spread to distant parts of the body. Staging applies only to secretory carcinomas of the major salivary glands. Tumors of the minor salivary glands are staged using the system for the area where they started (such as the oral cavity or oropharynx).
Stade tumoral (pT)
- T1 — La tumeur mesure 2 cm ou moins et est localisée dans la glande salivaire.
- T2 — La tumeur mesure plus de 2 cm mais pas plus de 4 cm et reste confinée à la glande salivaire.
- T3 — La tumeur mesure plus de 4 cm ou s'est étendue au-delà de la glande salivaire dans les tissus mous environnants (extension extraparenchymateuse).
- T4a — La tumeur a envahi la peau, la mâchoire, le conduit auditif ou le nerf facial.
- T4b — La tumeur a envahi la base du crâne, les os voisins ou les principaux vaisseaux sanguins.
Stade nodal (pN)
- N0 — Aucune cellule tumorale n'a été détectée dans les ganglions lymphatiques examinés.
- N1 — Un seul ganglion lymphatique du même côté du cou contient une tumeur et mesure 3 cm ou moins, sans extension extraganglionnaire.
- N2a — Un seul ganglion lymphatique du même côté du cou mesure entre 3 et 6 cm, ou tout ganglion lymphatique présente une extension extraganglionnaire.
- N2b — Plusieurs ganglions lymphatiques du même côté du cou contiennent une tumeur, aucun ne dépassant 6 cm, sans extension extraganglionnaire.
- N2c — Les ganglions lymphatiques situés des deux côtés du cou ou du côté opposé à la tumeur contiennent des cellules tumorales, aucun ne dépassant 6 cm, sans extension extraganglionnaire.
- N3a — Un ganglion lymphatique de plus de 6 cm contient une tumeur.
- N3b — Tout ganglion lymphatique positif présente une extension extraganglionnaire (à l'exception de la catégorie de petit ganglion unique couverte par N2a).
Quel est le pronostic?
The outlook for most patients with secretory carcinoma is excellent. The tumor is generally slow-growing, and complete surgical removal cures most patients. The 5-year survival rate for classic secretory carcinoma is greater than 90%. Recurrence after complete surgery is uncommon but can occur years later, which is why long-term follow-up is recommended.
Plusieurs éléments du rapport d'anatomopathologie permettent d'identifier les patients présentant un risque plus élevé d'évolution défavorable :
- Transformation de haut niveau — Le signe avant-coureur le plus important. Les chances de survie diminuent considérablement en sa présence.
- Taille de la tumeur supérieure à 4 cm — Les tumeurs plus volumineuses sont plus susceptibles de s'être propagées et de récidiver.
- Extension extraparenchymateuse — Les tumeurs qui se sont développées au-delà de la glande salivaire présentent un risque plus élevé de récidive.
- Marges chirurgicales positives — Les tumeurs incomplètement retirées ont plus de risques de récidiver.
- Atteinte des ganglions lymphatiques — La propagation aux ganglions lymphatiques augmente le risque de métastases à distance et aggrave le pronostic global.
- Invasion périneurale et lymphovasculaire — Both are linked with a higher risk of the tumor coming back near the original site.
Que se passe-t-il après le diagnostic ?
Treatment for secretory carcinoma is led by a head and neck surgeon. The surgeon often works with a radiation oncologist, a medical oncologist (when high-grade or advanced disease is present), and a speech-language pathologist for any rehabilitation needs. The main treatment is surgery to remove the entire tumor.
- Parotidectomie — Removal of part or all of the parotid gland. Most secretory carcinomas of the parotid gland are treated with a superficial parotidectomy or, for deeper tumors, a total parotidectomy. The facial nerve is preserved whenever possible. Submandibular and minor salivary gland tumors are removed along with the entire affected gland or a rim of healthy tissue.
- Dissection du cou — Removal of lymph nodes from one or both sides of the neck. Done when there is clinical or imaging evidence of lymph node involvement, when high-grade transformation is present, or when the tumor is very large. Neck dissection is often not needed for small, classic secretory carcinomas.
- Radiothérapie après une intervention chirurgicale — Recommandée en cas de transformation de haut grade, de marges chirurgicales positives ou proches, d'envahissement périneural ou lymphovasculaire, d'atteinte ganglionnaire ou pour les tumeurs à un stade avancé. La radiothérapie est administrée sous forme de séances quotidiennes sur plusieurs semaines.
- Thérapie médicamenteuse ciblée — NTRK inhibitors such as larotrectinib or entrectinib are an option for patients with secretory carcinoma that has come back, has spread, or cannot be removed by surgery. Patients whose tumor has the rarer RET fusion may instead be treated with a RET inhibitor (selpercatinib or pralsetinib). These drugs are taken as pills.
- Chimiothérapie standard — Generally not very effective in secretory carcinoma and is mostly replaced by targeted drug therapy when systemic treatment is needed.
- Surveillance à long terme — Un examen clinique régulier de la tête et du cou, complété par des examens d'imagerie si nécessaire, est maintenu pendant de nombreuses années après le traitement.
Questions à poser à votre médecin
- Où exactement la tumeur a-t-elle commencé, et quelle était sa taille ?
- Quel est le stade pathologique (pT, pN et stade TNM global) de mon cancer ?
- Une transformation de haut grade a-t-elle été observée quelque part dans la tumeur ?
- La tumeur a-t-elle été complètement retirée ? Quelles étaient les marges chirurgicales ?
- Si la marge était positive ou proche, aurais-je besoin d'une nouvelle intervention chirurgicale ou d'une radiothérapie ?
- Une invasion périneurale ou lymphovasculaire a-t-elle été identifiée ?
- Des ganglions lymphatiques étaient-ils atteints par la tumeur, et y avait-il une extension extraganglionnaire ?
- Was the diagnosis confirmed with S100 and mammaglobin staining, or with molecular testing?
- Was the specific gene fusion identified — was it ETV6-NTRK3, ETV6-RET, or another fusion?
- If my tumor has come back or has spread, am I a candidate for an NTRK inhibitor or RET inhibitor?
- Aurais-je besoin d'une radiothérapie après l'opération ?
- Quel est mon risque estimé de récidive du cancer ?
- Quel est le calendrier des examens de suivi et des examens d'imagerie, et combien de temps cela va-t-il durer ?
- Aurais-je des séquelles permanentes au niveau du visage, des engourdissements ou une sécheresse buccale suite à l'opération ?
- Y a-t-il des essais cliniques que je devrais envisager ?
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