BACKGROUND: ETV6 gene rearrangement is the molecular hallmark of secretory carcinoma (SC), however; the nature, frequency, and clinical implications of atypical ETV6 signal patterns by fluorescence in situ hybridization (FISH) has not yet been systematically evaluated in salivary gland neoplasms.
METHODS: The clinical, histopathologic, immunohistochemical and molecular features of seven salivary SCs, including four cases with atypical ETV6 FISH patterns, were retrospectively analyzed along with a critical appraisal of the literature on unbalanced ETV6 break-apart in SCs.
RESULTS: The patients were four males and three females (31-70 years-old). Five presented with a painless neck mass and two patients with recurrent disease had a history of a previously diagnosed acinic cell carcinoma of the buccal mucosa. Histologically, there were varied combinations of microcystic, papillary, tubular, and solid patterns. All tumors were diffusely positive for S100 and/or SOX10, while 2 cases also showed luminal DOG1 staining. Rearrangement of the ETV6 locus was confirmed in 5/7 cases, of which 3 cases showed classic break-apart signals, 1 case further demonstrated duplication of the ETV6 5`end and the other loss of one copy of ETV6. Two cases harbored ETV6 deletion without rearrangement. Two of the 4 cases with atypical ETV6 FISH patterns represented recurrent tumors, one with widespread skeletal muscle involvement, bone and lymphovascular invasion. Surgical treatment resulted in gross-total resection in all 7 cases, with a median follow up of 9.5 months post-surgery for primary (n = 3) and recurrent disease (n = 1).
CONCLUSIONS: Duplication of the distal/telomeric ETV6 probe represented the most common (26/40; 65%) variant ETV6 break-apart FISH pattern in salivary SC reported in the literature and appears indicative of an aggressive clinical course.

BACKGROUND: Salivary duct carcinoma (SDC) is an aggressive form of cancer, with cutaneous metastasis being a rare occurrence. Furthermore, cutaneous metastasis of SDC secondary to a scald is even rarer, and to the best of our knowledge, our case represents the first such instance. Considering the involvement of the fingers in the metastatic site, which may affect limb function and quality of life, we present this case to explore the reason why scald could lead to distant recurrence and better treatment options.
METHODS: An 85-year-old man diagnosed with SDC in the parotid gland found enlarged masses at the fingertips as a consequence of a burn, 6 years after his initial treatment.
METHODS: Cutaneous metastasis of SDC in the parotid gland and left thumb loss due to surgery.
METHODS: Radiotherapy was offered, targeting at the masses on the fingers, with dose at 15 Gy in 3 fractions, 12 Gy in 3 fractions, 15 Gy in 3 fractions for both hands and additional 21 Gy in 7 fractions only for left hand.
RESULTS: The tumors shrank after 2 months of radiotherapy and the patient recovered well. Side effects included nail hyperplasia and paronychia.
CONCLUSIONS: Connections between scald and distant metastasis of malignant tumors in this case needed further investigation. Considering reserving function of the fingers while dealing with metastasis, radiotherapy is recommended rather than surgery.

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Pleomorphic adenomas (PA) are the most prevalent benign salivary gland neoplasms. They may occur at any age, with a peak incidence between 40 and 60 years of age. They are more commonly observed in females (60%). These tumors can arise in both the major and minor salivary glands. Approximately 80% of these tumors are diagnosed in the parotid gland, whereas 10% arise in the minor salivary glands, mainly affecting the palates, followed by the lips and cheeks. This report describes two cases of unusual lesions that were diagnosed as (PA) in the minor salivary glands in our department via a review of the relevant literature. The first case involved an 83-year-old man who presented with a slow-growing swelling on the right side of the upper lip, and the second case involved a 45-year-old woman who presented with a slow-growing lesion on the palate. The presence of PA was confirmed histopathologically after surgical resection. Although relatively rare, PA is a benign lesion, the diagnosis of which must be known for appropriate therapeutic management.

Microsecretory adenocarcinoma (MSA) is a new type of salivary gland neoplasm identified in the 2022 World Health Organization Classification of Head and Neck Tumour (Skalova et al., Head Neck Pathol 16:40-53, 2022) and is characterized by a unique set of histomorphologic and immunohistochemical features and a recurrent MEF2C::SS18 fusion. MSA was initially misdiagnosed as another salivary gland tumour due to its similar morphology; until recently, only fewer than 50 cases were reported. We present a case of MSA of the hard palate with diverse architectural growth patterns, bland cytological features, abundant basophilic intraluminal secretions and fibromyxoid stroma. The tumour cells were positive for the SOX10, S100, and p63 protein and negative for the p40 protein according to immunohistochemistry. SS18 gene rearrangement was demonstrated via break-apart fluorescence in situ hybridization. We also provided a comprehensive literature review and integrated the clinicopathological features, immunophenotype, and molecular alterations of the disease. A comprehensive understanding of MSA enables us to accurately distinguish and categorize MSA from other salivary gland tumours with analogous morphologies.

Mucoepidermoid carcinoma is a malignant tumor that arises from the salivary glands. The recommended treatment strategy typically involves surgical intervention, sometimes complemented by radiotherapy, depending on the histological grade of the tumor. A case of a 22-year-old female patient without medical history was described. The clinical examination revealed a bluish lesion located on the hard palate. The histological examination confirmed the diagnosis of a low-grade mucoepidermoid carcinoma. Resection of the lesion was performed and oro-nasal communication was immediately closed by a prosthetic obturator and later on by a rotational palate flap. The patient was followed up for 12 months, and there was no evidence of any recurrence. This article highlights the importance of prompt clinical diagnosis of such lesions and provides an opportunity to review these cancer therapeutic measures to reduce postoperative morbidity.

BACKGROUND: Salivary gland cystadenoma (SGCA) is a rare benign tumor that predominantly occurs in the parotid gland. SGCAs affecting the minor salivary glands are uncommon and often resemble, clinically and histopathologically, other salivary gland lesions.
METHODS: This study aimed to describe a series of four cases of SGCA affecting intraoral sites and performed a literature review of well-reported SGCA published in the English-language literature.
RESULTS: SGCA cases included in this series were diagnosed in the buccal mucosa, lip, and hard palate of female patients aged between 19 and 78 years. All cases underwent excisional biopsy and were histologically characterized by a multicystic growth with variable degrees of capsule formation and were lined by several types of epithelium, including some cell types that are infrequently reported in SGCA. In some cases, a small collection of lymphocytes was observed adjacent to cystic formations. All SGCA were positive for periodic acid-Schiff, and immunohistochemical reactions were positive for CK7 and p63. The follow-up time ranged widely from 3 to 53 months, and to date, no recurrence has been observed.
CONCLUSIONS: The literature review revealed a total of 33 published studies accounting for 55 SGCA cases.

Paget disease is an intraepithelial neoplastic proliferation, commonly occurring in the breast and apocrine-rich areas, often associated with an underlying internal malignancy. Extramammary Paget disease (EMPD) of the oral cavity is exceedingly rare, with only eight reported cases, four of which were associated with an underlying internal malignancy. Here, we report a case of oral EMPD involving the buccal mucosa and gingiva of an 81-year-old male with no known underlying internal malignancy. The Paget cells were positive for CK7, CK20, CAM5.2, and androgen receptor, but negative for SOX10 and p63. The immunophenotype, association with internal malignancies, and treatment approaches for oral EMPD are reviewed.

Minor salivary glands are widely distributed in the mucosal surface of the lips, palate, nasal cavity, pharynx, and larynx, thus can arise from any of these primary sites. Intra-oral minor salivary gland tumors (IMSGTs), while considered rare in the general population are relatively more common when compared to all the other extra-oral sites. Pleomorphic adenoma, as seen in the index patient, is the most commonly diagnosed benign IMSGT. Intra-oral minor salivary gland tumors are not uncommon and depending on their size, nature, and location can be associated with severe limitation of the Patient\'s ability to breathe, speak clearly, and/or swallow and consequent severe morbidity and even mortality. In addition to these deleterious effects, they present a major surgical challenge to the surgeon, who has to determine the safest, most feasible access to ensure complete, or near-complete excision, as well as to the anesthetist, who needs to secure a definitive airway through the nose or mouth, both of which could be significantly restricted by the presence of the tumor. The aim is to present our successful management of one of the largest intra-oral minor salivary gland tumors documented in the literature, highlighting the specific measures we undertook to tackle the peculiar surgical and anesthetic challenges we faced. It had been two years since surgery and the patient is thriving with a markedly improved quality of life and no features of recurrence. The patient is a 50-year-old male with a slowly growing painless, left palatal mass in the roof of the mouth of 10 years duration with recurrent spontaneous bloody discharge effluent and snoring. There was an associated history of dysphagia to solid with associated choking spells, a left-sided facial asymmetry with no cheek swelling, odynophagia, sore throat, or difficulty with breathing. There was ipsilateral loss of upper incisors and dental anarchy about two years before presentation. No other nasal, otologic, or ophthalmic symptoms were present. No neck swelling, stiffness, cough, or chest symptoms. The oropharyngeal physical examination was highly restricted due to the intra-oral size of the mass. Figure 1. There was facial asymmetry with a bulge of the left maxilla, left-sided levels 1b and 2 non-tender lymph node enlargements, freely mobile, not adhered to the skin. A craniofacial CT scan revealed extensive isodense heterogeneously enhancing intra-oral soft tissue mass occupying the entire palate/oral cavity and encroaching laterally on the masticator and the parapharyngeal space with erosion of the left maxillary floor and hyoid bone Figure 2. The patient had an excision biopsy of the palatal mass with a free margin. No frozen section at the time of surgery. Histology revealed Pleomorphic adenoma and was followed up for 2 years with no evidence of recurrence. Prognosticators are delay in presentation leading to an increase in size of the mass and severe limitation of the patient\'s ability to breathe, speak clearly, and/or swallow and consequent severe morbidity and even mortality, the surgeon not being overwhelmed, the skillful Anaesthesist that could maneuver the nasal cavity without us doing tracheostomy and the successful outcome of the surgery.
Les glandes salivaires mineures sont largement réparties à la surface muqueuse des lèvres, du palais, de la cavité nasale, du pharynx et du larynx, et peuvent donc survenir à partir de l\'un de ces sites primaires. Les tumeurs des glandes salivaires mineures intra-orales (TGSMIO), bien que considérées comme rares dans la population générale, sont relativement plus courantes par rapport à tous les autres sites extra-oraux. L\'adénome pléomorphe, tel que celui observé chez le patient index, est la TGSMIO bénigne la plus fréquemment diagnostiquée. Les tumeurs des glandes salivaires mineures intra-orales ne sont pas rares et, en fonction de leur taille, de leur nature et de leur emplacement, peuvent être associées à une limitation sévère de la capacité du patient à respirer, à parler clairement et/ou à avaler, avec une morbidité sévère et même une mortalité. Outre ces effets délétères, elles présentent un défi chirurgical majeur pour le chirurgien, qui doit déterminer l\'accès le plus sûr et le plus faisable pour assurer une excision complète ou presque complète, ainsi que pour l\'anesthésiste, qui doit assurer une voie aérienne définitive par le nez ou la bouche, tous deux pouvant être significativement restreints par la présence de la tumeur. L\'objectif est de présenter notre prise en charge réussie de l\'une des plus grandes TGSMIO documentées dans la littérature, mettant en évidence les mesures spécifiques que nous avons prises pour relever les défis chirurgicaux et anesthésiques particuliers auxquels nous avons été confrontés. Deux ans après l\'intervention, le patient se porte bien avec une nette amélioration de sa qualité de vie et aucune manifestation de récurrence. Le patient est un homme de 50 ans présentant une masse palatine gauche en croissance lente et indolore dans le palais depuis 10 ans, avec des écoulements sanguins spontanés récurrents et des ronflements. Il y avait une histoire associée de dysphagie aux solides avec des épisodes d\'étouffement, une asymétrie faciale du côté gauche sans tuméfaction de la joue, une odynophagie, un mal de gorge ou des difficultés respiratoires. Il y avait une perte ipsilatérale des incisives supérieures et une anarchie entaire environ deux ans avant la présentation. Aucun autre symptôme nasal, otologique, ophtalmique n\'était présent. Aucun gonflement du cou, raideur, toux ou symptômes thoraciques. L\'examen physique de l\'oropharynx était fortement limité en raison de la taille intra-orale de la masse. Figure 1. Il y avait une asymétrie faciale avec une bosse du maxillaire gauche, des ganglions lymphatiques non douloureux des niveaux 1b et 2 du côté gauche, mobiles librement, non adhérents à la peau. La tomodensitométrie craniofaciale a révélé une masse tissulaire molle intraorale extensive, hétérogène, rehaussée de manière isodense occupant l\'ensemble du palais/cavité buccale et empiétant latéralement sur les muscles masticateurs et l\'espace parapharyngé, avec érosion du plancher du maxillaire gauche et de l\'os hyoïde. Figure 2. Le patient a subi une biopsie d\'excision de la masse palatine avec une marge libre. Aucune section congelée n\'a été réalisée lors de la chirurgie. L\'histologie a révélé un adénome pléomorphe et un suivi de 2 ans n\'a montré aucun signe de récurrence. Les facteurs pronostiques comprennent le retard de la présentation entraînant une augmentation de la taille de la masse et une limitation sévère de la capacité du patient à respirer, à parler clairement et/ou à avaler, avec une morbidité sévère voire une mortalité, le chirurgien ne se laissant pas dépasser, l\'anesthésiste compétent pouvant manœuvrer dans la cavité nasale sans avoir recours à une trachéotomie, et le succès de l\'intervention chirurgicale. MOTS-CLÉS: Intraoral; Glande salivaire mineure; Excision; Tumeur; Pronostiqueurs.

True malignant mixed tumors, also known as salivary gland carcinosarcoma (SCS), are uncommon yet highly aggressive lesions associated with a poor prognosis. These tumors exhibit a distinctive biphasic structure characterized by both epithelial and mesenchymal components. Recent research has shown that the majority of SCS cases stem from pre-existing pleomorphic adenomas (PAs), suggesting a stepwise developmental pattern. In this report, we present a case of a 73-year-old female with SCS and describe the clinical, radiographic, and pathologic observations. Notably, the SCS was associated with a residual PA. The SCS displayed a CTNNB1::PLAG1 gene rearrangement, providing a molecular basis for its origin from the PA. Further DNA genomic analysis exposed mutations in BAP1, PER1, and LRPB1. Our findings provide support to the theory that SCS emerges from a pre-existing PA while highlighting the multiple genetic changes that could contribute to malignant transformation.