Utilidad diagnóstica del CD117 en un inusual carcinoma adenoide quístico del labio superior / Diagnostic utility of CD117 in an unusual adenoid cystic carcinoma of the upper lip / Utilidade diagnóstica do CD117 num carcinoma adenoide cístico invulgar do lábio superior

RESUMEN El carcinoma adenoide quístico (CAQ) es una neoplasia maligna de glándula salival, de evolución lenta pero de agresivo comportamiento, que se presenta frecuentemente en el paladar; sin embargo, en el labio superior, donde suelen encontrarse más neoplasias benignas, su presencia es escasamente relatada. Se expone un relato de una mujer de 63 años, derivada por presentar un nódulo en el labio superior derecho, de aspecto benigno, consistencia firme, circunscrito, móvil, doloroso a la palpación, sin tiempo de evolución determinado y cuyo estudio histopatológico exhibió una proliferación de células anguladas e hipercromáticas de escaso citoplasma, organizadas en patrón cribiforme y predominantemente tubular. Se realiza un estudio inmunohistoquímico, el cual mostró positividad para SMA, p63 y CD117 con un Ki-67 del 10 % aproximadamente. Estos resultados confirman diagnóstico de CAQ con patrón tubular, destacando el rol del CD117 para el diagnóstico diferencial con adenocarcinoma polimorfo. Se deriva a la paciente para tratamiento quirúrgico y radioterapia, y luego de un año de seguimiento mantiene un buen comportamiento clínico.

ABSTRACT Adenoid cystic carcinoma (ACCC) is a malignant neoplasm of the salivary gland, of slow evolution but aggressive behavior, which frequently occurs in the palate; however, in the upper lip, where more benign neoplasms are usually found, its presence is scarcely reported. We report the case of a 63-year-old woman, referred for presenting a nodule in the right upper lip, with benign appearance, firm consistency, circumscribed, mobile, painful to palpation, with no determined time of evolution and whose histopathological study showed a proliferation of angular and hyperchromatic cells with scarce cytoplasm, organized in a cribriform pattern and predominantly tubular. An immunohistochemical study was performed, which showed positivity for SMA, p63 and CD117 with a Ki-67 of approximately 10%. These results confirm the diagnosis of CAC with tubular pattern, highlighting the role of CD117 for the differential diagnosis with polymorphous adenocarcinoma. The patient was referred for surgical treatment and radiotherapy, and after one year of follow-up she maintained a good clinical behavior.

RESUMO O carcinoma adenoide cístico (CACC) é uma neoplasia maligna da glândula salivar, de evolução lenta, mas de comportamento agressivo, que ocorre frequentemente no palato; no entanto, a sua presença é raramente relatada no lábio superior, onde normalmente se encontram neoplasias mais benignas. Relatamos o caso de uma mulher de 63 anos que foi encaminhada por apresentar um nódulo benigno no lábio superior direito, de aspeto firme, circunscrito, móvel, doloroso à palpação, sem tempo de evolução definido. O exame histopatológico revelou uma proliferação de células angulosas e hipercromáticas com citoplasma escasso, organizadas em padrão cribriforme e predominantemente tubulares. Foi efetuado um estudo imuno-histoquímico que mostrou positividade para SMA, p63 e CD117 com um Ki-67 de aproximadamente 10 %. Estes resultados confirmam o diagnóstico de CAC com padrão tubular, realçando o papel do CD117 no diagnóstico diferencial com adenocarcinoma polimorfo. A paciente foi encaminhada para tratamento cirúrgico e radioterapia e, após um ano de seguimento, manteve bom comportamento clínico.

A clinicopathological analysis of cervical carcinoma with basaloid features / 实用医学杂志

Objective To investigate the clinical and pathological features of adenoid basal cell carcinoma(ABC),adenoid cystic carcinoma(ACC),and basaloid squamous cell carcinoma(BSCC)with basaloid charac-teristics and improve the diagnostic and differential diagnostic ability of clinicians and pathologists for these lesions.Methods A retrospective study was conducted on the clinical and pathological data of 4 cases of ABC,1 case of ACC,and 3 cases of BSCC diagnosed and treated at Shenzhen Maternal and Child Health Hospital,Southern Medical University from April 2018 to December 2022.Pathological slides were reviewed and relevant literature was analyzed and summarized.Results All three types of tumors were common in postmenopausal women and were associated with high-risk HPV infection.ABC was a low-grade cancer and patients were often clinically asymp-tomatic.It was usually detected incidentally during cervical screening due to cytological abnormalities,or after cervical cone biopsy or hysterectomy for HSIL.It presented as superficial cervical infiltration and clinical staging was often early.ACC and BSCC were intermediate to high-grade cancers and they often presented with postmenopausal vaginal bleeding.A visible mass was observed on the cervix.The clinical staging was intermediate to advanced.The three types of lesions could coexist.Careful observation of the morphological characteristics and immunohistochemical staining could help with differential diagnosis.None of the 8 patients experienced recurrence or metastasis during follow-up.Conclusion Cervical ABC,ACC and BSCC are rare and they originate from reserve cells.They share the similarities in clinical and pathological morphology,but differ in treatment and prognosis.So,accurate differen-tiation among them has important clinical significance.

Carcinoma adenoide quístico traqueal: presentación de caso clínico y revisión de literatura / Tracheal cystic adenoid carcinoma: Case report presentation and literature review

Introducción: El carcinoma adenoide quístico de la vía aérea es un tumor poco común, que se origina de las glándulas submucosas del árbol traqueobronquial. Por el usual retraso entre los síntomas y el diagnóstico, y por la propensión de este tumor para expandirse a través de los haces perineurales y submucosa, el tratamiento recomen dado es la resección quirúrgica con radioterapia posoperatoria. La supervivencia está determinada por la presencia de metástasis a distancia. Caso clínico: Paciente de sexo femenino de 70 años de edad con antecedentes de hipertensión arterial, EPOC (extabaquista 34 paquetes/año) que acude a servicio de urgencias con episodios de hemoptisis y disnea mMRC 3-4 previa. Discusión: Las neoplasias malignas de la tráquea son muy raras y los datos relacionados con ellos son limitados. Los más importantes factores pronósticos en las enfermedades primarias malignas de la tráquea constituyen el diagnóstico temprano, estadiaje del tumor, histología y opciones de tratamiento. Conclusiones: La detección temprana puede estar asociada con el incremento de las tasas de resecabilidad e, incluso, supervivencia prolongada.

Introduction: Adenoid cystic carcinoma of the airway is a strange tumor that arises from the submucosal glands of the tracheobronchial tree. For the usual delay between symptoms and diagnosis, and for the propensity of this tumor to expand through the perineural fibers and submucosa, the recommended treatment is surgical resection with postoperative radiation therapy. Survival is determined by the presence of distance metastases. Clinical case: 70 year-old female patient with a history of hypertension, COPD (former smoker 34 pack/year) who came to the emergency room with episodes of hemoptysis and previous dyspnea mMRC 3-4. Discussion: Malignant neoplasms of the trachea are very strange and data related to them is limited. The most important prognostic factors in primary malignant diseases of the trachea are early diagnosis, tumor stating, histology, and treatment options. Conclusions: Early detection may be associated with increased resectability rates and even prolonged survival.

Effects of ICMT gene silencing on the invasion and migration of human salivary adenoid cystic carcinoma cells in vitro / 口腔疾病防治

Objective @#To investigate the effect of isoprene cysteine carboxymethyltransferase (ICMT) gene on the migration and invasion of salivary adenoid cystic cancer cells (SACC) and the related mechanism, to provide experimental evidence for molecular targeted therapy of SACC.@*Methods@# Adenoid cystic cancer cells SACC-LM and SACC-83 were cultured in vitro, and siRNA was transfected into human SACC-LM and SACC-83 cells (experimental group) by transient transfection of a liposome vector. A blank control group and negative control group were set up respectively (transfected NC-siRNA). qRT-PCR was peformed to measure the mRNA expression of ICMT and RhoA in each group after transfection and to determine the silencing efficiency. The expression of ICMT, membrane RhoA, total RhoA, matrix metalloproteinase-2 (MMP-2), matrix metalloproteinase-9 (MMP-9) and Rho associated with coiled helical binding protein kinase 1 (ROCK1) in each group was detected by Western blot. The proliferation abilityies of SACC cells was detected by CCK-8 assay. The migration and invasion ability of SACC cells were detected by comparing the relative healing area of cell scratch assay and the number of Transwell assay cells. @*Results@#After transfection of ICMT-siRNA into SACC-LM and SACC-83 cells, the expression of ICMT gene and protein in the experimental group was significantly decreased compared with the negative control group and blank control group (P<0.05), but there were no significant differences in the expression of RhoA gene and total protein among all groups (P>0.05). The expression of RhoA membrane proteins, ROCK1, MMP-2, MMP-9 in the experimental group was significantly decreased compared with that in the negative control group and blank control group (P<0.05). Cell proliferation ability was significantly decreased (P<0.05). The migration and invasion abilities were significantly decreased (P<0.05). @*Conclusion @#In vitro silencing of ICMT gene can effectively inhibit the migration and invasion of human SACC-LM and SACC-83 cells, and the mechanism may be related to RhoA-ROCK signaling pathway.

Clinical Diagnosis and Treatment of Lacrimal Gland Adenoid Cystic Carcinoma / 肿瘤防治研究

Lacrimal gland adenoid cystic carcinoma (LGACC) is the most common primary malignant epithelial tumor of the lacrimal gland and has the characteristics of high recurrence rate, neurotropic growth, and risk of distant metastasis. The clinical manifestation is mainly a mass in the lacrimal gland area with a poor specificity. The combination of MRI and CT is important in the diagnosis of LGACC, and PET/CT is of great value in determining the nature and metastasis of LGACC. Histopathological examination is the gold standard for diagnosis, and the type of histopathology is significant for predicting the prognosis of patients. At present, the most commonly used treatment for LGACC is surgical resection combined with postoperative local radiotherapy. However, the overall prognosis is poor after active treatment because the pathogenesis of LGACC remains unclear. This article will combine relevant literature and the author's clinical experience to review the epidemiology, clinical manifestations, imaging examination, histopathological changes, treatment, and prognosis of LGACC. Results will contribute to improve the quality of diagnosis and treatment and the prognosis of patients.

The outcome of transnasal endoscopic total maxillectomy in the treatment of sinonasal adenoid cystic carcinoma / 临床耳鼻咽喉头颈外科杂志

Objective:To assess the prognosis of sinonasal adenoid cystic carcinoma with hard palatine invasion treated by transnasal endoscopic total maxillectomy. Methods:Clinical data of twenty-six patients with sinonasal adenoid cystic carcinoma invading hard palatine treated by transnasal endoscopic total maxillectomy between May 2014 and December 2020 was analyzed retrospectively. Survival rate, local recurrence and distant metastasis were analyzed using Kaplan-Meier method. Cox regression was used to investigate the prognosis factors. Masticatory function after maxillectomy has also been assessed using the questionnaire of patients' satisfaction about masticatory function. Results:Margins in 8 patients（30%） were positive. The median time of follow-up was 38 months（6 to 85 months）. Twenty-five patients recurred. Four patients died of distant metastasis. The 5-year overall survival rate and relapse-free survival rate was 79.5% and 89.1%, respectively. Independent predictors of outcome on multivariate analysis were positive margin（P=0.018）, recurrence（P=0.006） and distant metastasis（P=0.04）. Conclusion:Transnasal endoscopic total maxillectomy could be performed for the treatment of the sinonasal adenoid cystic carcinoma with hard palatine invasion. Positive margin, local recurrence and distant metastasis were important predictors for patients' prognosis.

Value of MRI in assessing regional invasion and neural spread in sinonasal and nasopharyngeal adenoid cystic carcinoma / 中国现代医生

Objective To explore the value of MRI in assessing regional invasion and neural spread of sinonasal and nasopharyngeal adenoid cystic carcinoma(ACC).Methods A retrospective collection of MRI and clinical data was analyzed in 22 patients with sinonasal and nasopharyngeal ACC confirmed by pathology.Results A total of 22 patients were included in the study.Invasion of the pterygopalatine fossa was observed in 15 cases,involvement of the masticatory muscles in 13 cases,skull base involvement in 13 cases,infiltration of the cavernous sinus in 9 cases,involvement of the infraorbital fissure in 9 cases,orbital involvement in 5 cases,and intracranial involvement in 6 cases.Only 3 patients presented with cranial nerve symptoms.MRI revealed thickening and enhancement of cranial nerves in 10 cases,with involvement of the maxillary and mandibular(V2,V3)branches of the trigeminal nerve(V)in 9 cases,and muscle denervation caused by nerve damage in 6 cases.Among the 12 patients who underwent surgery,9 cases were confirmed to have neural invasion by pathology,with MRI showing cranial nerve alterations in 7 cases of them,one case showed tumor involvement from the pterygopalatine fossa and medial pterygoid muscle to the cavernous sinus and Meckel cavity,while the other case showed tumor involvement from the pterygopalatine fossa to the sphenopalatine foramen.During the follow-up after treatment,4 of 11 patients experienced recurrence,all of which were accompanied by lung metastasis and pre-treatment neural invasion.Conclusion Sinonasal and nasopharyngeal ACC is characterized by a tendency for neural spread,extensive local invasion,and a high risk of recurrence.MRI is helpful in determining the extent of invasion and neural spread.

Misdiagnosis of adenoid cystic carcinoma of oropharynx: a case report / 临床耳鼻咽喉头颈外科杂志

Adenoid cystic carcinoma usually occurs in the salivary glands of the head and neck. It is a malignant tumor with a high degree of malignancy, resistance to radiotherapy and chemotherapy and poor prognosis. The clinical course of adenoid cystic carcinoma is slow and easy to be misdiagnosed. The main diagnosis and treatment means are individualized and precise treatment under the multi-disciplinary consultation mode, that is, surgical treatment and radiotherapy and chemotherapy. Adenoid cystic carcinoma is prone to relapse and hematologic metastasis, and the traditional radiotherapy and chemotherapy based therapies have not achieved satisfactory efficacy in the past three decades. How to detect, diagnose and treat early is an urgent task faced by clinicians.

Salivary gland tumor: atypical presentation of breast cancer

Breast cancer is a heterogeneous disease with various histological and molecular subtypes. Among them, salivary gland tumors are rare and can be divided into three groups: pure myoepithelial differentiation, pure epithelial differentiation and myoepithelial with mixed epithelial differentiation. In the last group, adenoid cystic carcinoma stands out, a rare entity with low malignant potential. It represents less than 0.1­3% of breast cancer cases and has the most frequent clinical presentation as a palpable mass. The diagnosis is confirmed by histology and immunohistochemistry. Classically, they are low-aggressive triple-negative tumors, with overall survival and specific cancer survival at five and ten years greater than 95%. However, there are rare reports of aggressive variants with a risk of distant metastasis and death. Treatment is based on surgical resection with margins. Lymphatic dissemination is rare, and there is no consensus regarding the indication of an axillary approach. Adjuvant radiotherapy is indicated in cases of conservative surgery and should be discussed in other cases. The benefit of chemotherapy remains uncertain, as most tumors are indolent. We report a case that required individualized decisions based on its peculiarities of presentation, diagnosed in an asymptomatic elderly patient during screening, in which mammography showed heterogeneous gross calcifications clustered covering 1.6 cm. Stereotacticguided vacuum-assisted biopsy was performed, and the area was marked with a clip. The anatomopathological examination led to a diagnosis of salivary gland-type carcinoma, triple-negative. The patient underwent segmental resection of the right breast and sentinel lymph node biopsy. The final anatomopathological result was similar to that of the biopsy, with an immunohistochemicalprofile of the adenoid cystic type and two sentinel lymph nodes free of neoplasia. Considering age and histological subtype, adjuvant therapy was not indicated. Follow-up for three years showed no evidence of disease

Carcinoma adenoide quístico / Adenoid cystic carcinoma

Introducción: El carcinoma adenoide quístico es una neoplasia maligna poco común y a menudo agresiva, que puede surgir en una variedad de órganos portadores de glándulas. Objetivo: Caracterizar la presentación de una neoplasia maligna agresiva infrecuente, el carcinoma adenoide quístico. Caso clínico: Paciente de 56 años que acudió a cuerpo de guardia con lesión en cuero cabelludo correspondiente con cilindroma maligno. Se estudia posteriormente y se constataron además lesiones a nivel de mamas, colon y recidiva en el cuero cabelludo, que dieron como resultado carcinoma adenoide quístico. Conclusiones: Es infrecuente; realizar su diagnóstico conlleva un alto índice de sospecha y su diagnóstico es histopatológico. Su pronóstico depende de la localización primaria y tiende a ser muy agresivo cuando se localiza fuera de la mama(AU)

Introduction: Adenoid cystic carcinoma is an uncommon and often aggressive malignant neoplasm that can arise in a variety of gland-bearing organs. Objective: To characterize the presentation of an uncommon aggressive malignancy, adenoid cystic carcinoma. Clinical case: 56-year-old patient who presented to the on-call department with a lesion on the scalp corresponding to malignant cylindroma. She was subsequently studied and lesions were also found in the breast, colon and recurrence in the scalp, which resulted in adenoid cystic carcinoma. Conclusions: It is infrequent; making its diagnosis involves a high index of suspicion and its diagnosis is histopathological. Its prognosis depends on the primary location and it tends to be very aggressive when located outside the breast(AU)

Clinico - pathological profile of Adenoid Cystic Carcinoma of the Lacrimal gland - A Retrospective Study at Tertiary Cancer Care Hospital, Punjab

Background: A retrospective study conducted in the department of radiation oncology to review the management aspects of the adenoid cystic carcinoma of the lacrimal gland and identify the outcome and corelation of the recurrence pattern with pathological and clinical findings. Methods: Data of 10 patients of adenoid cystic carcinoma of the lacrimal gland treated over a period of six years were reviewed with respect to presentation, treatment received , end points and outcome. Results: All of the 10 patients underwent surgery followed by adjuvant radiation therapy. Five patients received adjuvant chemotherapy . Eight (80%) patients were alive at a median follow up of 2 years. Five ( 50%) patients withou t evidence of disease and 3 patients with the disease. One patient developed scalp metastasis during the course of radiation therapy and another one developed lung metastasis. Conclusion: Ad enoid cystic carcinoma of the lacrimal gland is a rare neoplasm wi th aggressive outcome. It is often treated with surgery and adjuvant radiotherapy and needs long follow up.

Primary cutaneous adenoid cystic carcinoma – An onerous task for clinicians and pathologists

Adenoid cystic carcinoma (ACC) is an uncommon malignant neoplasm usually confined to the salivary glands, lungs, and breasts. Primary cutaneous adenoid cystic carcinoma (PCACC) is an extremely rare entity with solitary cases reported at sites away from the scalp and chest. Hence, one must follow the multidisciplinary approach to exclude any primary ACC elsewhere in the body. We report a rare case of PCACC arising from the skin of the left lower limb in a 55-year-old woman with a history of recurrent swelling, clinically diagnosed as a metastatic tumor.

Case report on adenoid cystic carcinoma of palate with review of literature

Adenoid cystic carcinoma (ACC) is a malignant tumor of minor salivary glands of the oral cavity, particularly the palate which accounts for about 5–10%. These tumors are slow growing with a high incidence of local recurrence, perineural invasion, and distant metastasis. Here, we report the case of a 69-year-old female who presented with swelling over the hard palate. Computerized tomography shows enhancing lesion along the undersurface of the left side of the hard palate, abutting the maxillary alveolus in the region of the second premolar and first two molars. There was no erosion of the inner cortex of the maxillary alveolus and no extension into the nasal cavity. Following the biopsy, she underwent a left hard palatectomy with upper alveolar resection. The final histopathological report confirmed ACC with no perineural invasion. Following she received adjuvant radiotherapy using an intensity-modulated radiotherapy technique. Now at 6 months of follow-up, there was no evidence of recurrence. Thus, our patient requires long-term follow-up to assess the benefit and survival outcome.

Treatment and prognosis analysis of perineural invasion on sinonasal adenoid cystic carcinoma / 中华肿瘤杂志

Objective: To analyze the efficacy of sinonasal adenoid cystic carcinoma (ACC) with perineural invasion (PNI), and explore the prognostic value of PNI on sinonasal adenoid cystic carcinoma. Methods: The clinical data of 105 patients with sinonasal ACC admitted to Cancer Hospital, Chinese Academy of Medical Sciences from January 2000 to December 2016 were retrospectively reviewed. All patients were restaged according to American Joint Committee on Cancer 8th edition. Follow-up visits were conducted to obtain information of treatment failure and survival outcome. The Log rank test was used for univariate analysis of prognostic factors, and Cox regression model was used for multivariate prognostic analysis. Results: The maxillary sinus (n=59) was the most common primary site, followed by the nasal cavity (n=38). There were 93 patients with stage Ⅲ-Ⅳ. The treatment modalities included surgery alone (n=14), radiotherapy alone (n=13), preoperative radiotherapy plus surgery (n=10), and surgery plus postoperative radiotherapy (n=68). The median follow-up time was 91.8 months, the 5-year local control (LC), distant metastasis-free survival (DMFS), progression-free survival (PFS), and overall survival (OS) rates were 72.6%, 73.0%, 52.9% and 78.0%, respectively. There were 33 patients (31.4%) with PNI-positive. The 5-year DMFS, PFS, and OS rates of PNI-positive group were 53.7%, 29.4% and 56.5%, respectively, which were significantly inferior to those of PNI-negative group (80.8%, 63.0% and 86.8%, respectively, P<0.05), while there was no significant difference in the 5-year LC rate between both groups (64.5% vs 76.5%, P=0.273). The multivariate Cox regression analysis showed PNI was one of the poor prognostic factors of DMFS (HR=3.514, 95%CI: 1.557-7.932), PFS (HR=2.562, 95%CI: 1.349-4.866) and OS (HR=2.605, 95%CI: 1.169-5.806). Among patients with PNI-positive, the 5-year LC, PFS and OS rates of patients received surgery combined with radiotherapy were 84.9%, 41.3% and 72.7%, respectively, which were significantly higher than 23.3%, 10.0% and 26.7% of patients receiving surgery or radiotherapy alone (P<0.05). Conclusion: The presence of PNI increases the risk of distant metastasis in patients with sinonasal ACC. Compared with patients with PNI-negative, the prognosis of patients with PNI-positive is relatively poor, and surgery combined with radiotherapy for PNI-positive sinonasal ACC results in good clinical outcomes.

Clinicopathological study in 28 cases of oral basaloid squamous cell carcinomas / 北京大学学报(医学版)

OBJECTIVE@#To investigate the clinicopathologic features and prognostic factors in oral basaloid squamous cell carcinoma.@*METHODS@#Retrospective analysis of oral basaloid squamous cell carcinomas patients who underwent tumor resection during the period from January 2002 to December 2020 in the authors' hospital, especially the clinicopathologic characteristics of 28 cases with confirmed diagnosis and follow-up data. Immunohistochemistry was performed to define the helpful markers for differentiation diagnosis. The factors influencing the prognosis were evaluated based on Kaplan-Meier method.@*RESULTS@#The tongue and mouth floor (11 cases, 39.3%) were the most frequently involved sites, followed by gingiva (6 cases, 21.4%), buccal (5 cases, 17.9%), palate (4 cases, 14.3%), and oropharynx (2 cases, 7.1%). The majority of basaloid squamous cell carcinomas were in advanced stage, with 12 cases in stage Ⅱ and 16 cases in stages Ⅲ-Ⅳ. Twelve of 28 patients were identified to have cervical lymph node metastasis, which was confirmed by histopathological examination. The incidence rate of lymph node metastasis was 42.9% (12/28). Nine tumors recurred, with one metastasized to the lung. At the meantime, the 28 conventional squamous cell carcinomas were matched with the same stage, among which 13 cases were identified with cervical lymph node metastasis. The incidence rate of lymph node metastasis was 46.4% (13/28). Five cases recurred, with two cases that metastasized to the lung and one to the brain. The 5-year overall survival rates of the basaloid squamous cell carcinoma and conventional squamous cell carcinoma patients were 54.6% and 53.8%, respectively. Histopathologically, basaloid cells consisted of tumor islands without evident keratinization but frequently with comedo-like necrosis within the tumor islands. CK5/6 and P63 exhibited strongly positive in all the 28 cases, whereas neuroendocrine markers, CgA and Syn, were negative. Eight cases positively expressed P16; one case showed focal SOX10 positive but CK7 negative.@*CONCLUSION@#The majority of oral basaloid squamous cell carcinomas present in advanced stage with a high tendency to lymph node metastasis, but the overall survival rates are not significantly different from conventional squamous cell carcinomas matched with the same stage. The human papilloma virus (HPV), as HPV-positivity rate is high, correlates to good prognosis. In addition, CK7 & SOX10 immunohistochemistry could contribute to differential diagnosis for basaloid squamous cell carcinoma with solid adenoid cystic carcinoma.

Clinical Features and Prognostic Factors of Adenoid Cystic Carcinoma of Head and Neck / 肿瘤防治研究

Objective To retrospectively analyze the clinical and pathological data of 48 patients with adenoid cystic carcinoma of the head and neck and screen out related factors that affect the patient's prognosis. Methods The overall survival rate of patients is used as the main indicator for observing prognosis. We selected 11 clinical and pathological factors as observation indicators that may have an impact on the survival and prognosis of patients. The relation between observation factors and the overall survival rate of patients was analyzed by Cox multivariate regression. Results The median follow-up time was 33.5(1-98) months. The 1-, 2- and 5-years overall survival rates were 95%, 91% and 87%. Local recurrence occurred in 14(29.2%) patients. Distant metastasis occurred in 22(45.8%) patients. Cox regression analysis showed that recurrence, distant metastasis and primary site were correlated with the overall survival rate of patients with head and neck adenoid cystic carcinoma (P < 0.05). Conclusion Adenoid cystic carcinoma is a relatively rare tumor of the head and neck with a low degree of malignancy. However, recurrence, distant metastasis and primary site have relatively great impact on the prognosis of patients.

Le carcinome adénoïde kystique des glandes salivaires accessoires: Particularités cliniques et modalités thérapeutiques / Adenoid cystic carcinoma of accessory salivary glands: clinical features and therapeutic modalities

Etudier les particularités cliniques des carcinomes adénoïdes kystiques des glandes salivaires accessoires et discuter les modalités thérapeutiques de ce type tumoral. Patients et méthodes: Il s'agit d'une étude rétrospective descriptive ayant colligé 11 patients présentant un carcinome adénoïde kystique (CAK) des glandes salivaires accessoires (GSA) pris en charge dans notre département sur une période de 20 ans. Résultats: L'âge moyen de nos patients était de 51 ans avec un sex ratio à 0,83. Le délai moyen de consultation était de 20 mois. Le site tumoral était: la cavité buccale dans 3 cas, le massif facial et le cavum dans 6 cas, la trachée et le larynx dans 2 cas. Les motifs de consultation étaient variables selon le site tumoral. L'extension péri neurale était objectivée dans 3 cas. Le traitement était chirurgical suivi de radiothérapie chez 5 patients. Une radiothérapie exclusive a été indiquée chez 3 patients et une radio chimiothérapie concomitante dans 3 cas. Un recul moyen de 42 mois a pu être obtenu pour 10 patients. Une patiente a été perdue de vue. L'évolution était favorable avec absence de récidive locale chez 8 patients. Conclusion: Le traitement de référence reste une chirurgie d'exérèse large, suivie de radiothérapie adjuvante. L'imagerie est indispensable pour le bilan d'extension locorégional et à distance qui conditionne l'attitude thérapeutique.

Avaliação da expressão da proteína EZH2 em carcinoma adenoide cístico e adenoma pleomórfico / EZH2 immunoexpression in adenoid cystic carcinoma and pleomorphic adenoma

As neoplasias de glândulas salivares apresentam comportamentos diferenciados, que não seguem os padrões clássicos das neoplasias benignas e malignas. A raridade de algumas destas lesões dificulta ainda mais o entendimento dos mecanismos envolvidos na etiopatogenia. Marcadores moleculares como a proteína EZH2 têm sido utilizados na investigação de alterações epigenéticas em diferentes neoplasias, auxiliando na definição do diagnóstico e prognóstico das lesões. O objetivo do presente trabalho é avaliar a expressão da proteína EZH2 e descrever as características clínicas e microscópicas de amostras de carcinoma adenoide cístico (CAC) e adenoma pleomórfico (AP) com ênfase na importância da definição da malignidade da lesão. A análise dos cortes microscópicos corados em Hematoxilina e Eosina dos casos de Adenoma pleomórfico mostraram células epiteliais e mioepiteliais glandulares dispostas em lençóis e estruturas ductiformes em meio a estroma variável. Os casos de Carcinoma adenoide cístico mostraram três padrões distintos de crescimento incluindo formações tubulares, cribriformes e sólidas. Todos os casos de AP e CAC foram positivos para reação imuno-histoquímica para EZH2. As amostras de CAC apresentaram expressão de EZH2 significativamente maior comparado ao AP. As covariáveis metástase em linfonodos, recorrência, padrão histológico, presença de áreas sólidas e invasão perineural foram descritas em relação à marcação de EZH2 em amostras de CAC. Dessa forma, os resultados do estudo melhoram o entendimento das características clínicas e histológicas do CAC, assim como sobre o comportamento das lesões. Além disso, a análise mostra que o EZH2 é um potencial marcador de malignidade e ressalta a importância da validação de marcadores moleculares de alterações epigenéticas.

Salivary gland neoplasms present different behaviors, which do not follow the classic patterns of benign and malignant neoplasms. The rarity of some of these lesions makes it even more difficult to understand the mechanisms involved in the etiopathogenesis. Molecular markers such as the EZH2 protein have been used to investigate epigenetic changes in different neoplasms, helping to define the diagnosis and prognosis of the lesions. The aim of the study was to evaluate the expression of the EZH2 protein and to describe the clinical and microscopic characteristics of adenoid cystic carcinoma (ACC) and pleomorphic adenoma (PA) which emphasizes the importance of defining the malignancy of the neoplasm. Histopathological analysis of PA cases showed myoepithelial and glandular epithelial cells arranged as duct-like structures and sheets intermingled in the variable stroma and ACC cases showed the three growth patterns, tubular, cribriform and solid forms. All ACC and PA cases were positive for EZH2, with diffuse nuclear staining in neoplastic cells. The ACC samples showed significantly higher EZH2 expression compared to the PA. The covariables nodal metastasis, recurrence, growth pattern, presence of solid areas and perineural invasion have been described in relation to EZH2 staining in ACC samples. The results of the study improve the understanding of the clinical and histological characteristics of ACC, as well as on the behavior of lesions. In addition, the analysis showed that EZH2 is a potential marker of malignancy and highlights the importance of validating molecular markers of epigenetic alterations.

High-Grade Transformation in Adenoid Cystic Carcinoma of the Bartholin Gland: Case Report / Transformação de alto grau em carcinoma adenoide cístico de glândula de Bartholin: relato de caso

Abstract Introduction In the present study, we report a case of primary adenoid cystic carcinoma (ACC) of the Bartholin gland with high-grade transformation (HGT). Adenoid cystic carcinoma of the Bartholin gland is a rare tumor and HGT has only been reported in head and neck tumors. Case Report A 77-year-old woman with a non-ulcerated vulvar lesion on the topography of the right Bartholin gland. The patient was submitted to tumor resection followed by V-Y island flap and adjuvant radiotherapy. The histopathological examination revealed primary ACC of the Bartholin gland, with areas of HGT and extensive perineural invasion. The immunohistochemical study with p53 showed a diffuse and strong positive reaction in areas with HGT. After 24 months of follow-up, the patient presented distant metastases and died, despite having undergone to chemotherapy. Conclusion As far as we know, this case is the first description in the literature of HGT in ACC of the Bartholin gland, and HGT appears to be associated with tumor aggressiveness.

Resumo Introdução Este estudo relata o caso de um carcinoma adenoide cístico (CAC) de glândula de Bartholin com transformação de alto grau. O CAC de glândula de Bartholin é um tumor raro, e sua transformação de alto grau é relatada somente em tumores de cabeça e pescoço. Relato de caso Paciente de 77 anos de idade, do sexo feminino, com lesão vulvar não ulcerada na topografia da glândula de Bartholin direita. A paciente foi submetida a ressecção do tumor e realização de retalho em V-Y, seguidas de radioterapia adjuvante. O exame histopatológico revelou CAC primário de glândula de Bartholin, com áreas de transformação de alto grau e invasão perineural. O estudo imunohistoquímico com p53 mostrou reação positiva difusa e intensa em áreas com transformação de alto grau. Após 24 meses de seguimento, a paciente apresentou metástases à distância e faleceu, apesar de ter sido submetida a quimioterapia. Conclusão Pelo que sabemos, este caso é a primeira descrição na literatura de transformação de alto grau em CAC de glândula de Bartholin, e a transformação de alto grau parece estar associada à agressividade do tumor.

Target delineation and dosimetry analysis of 125I interstitial brachytherapy for recurrent adenoid cystic carcinoma in the parotid gland / 中华放射医学与防护杂志

Objective:To analyze the target design and dosimetric parameters of patients with recurrent adenoid cystic carcinoma (ACC) in the parotid gland who were treated with 125I interstitial brachytherapy alone. Methods:A retrospective analysis was conducted for 25 patients with recurrent adenoid cystic carcinoma in parotid gland who were histopathologically diagnosed between January 2015 and October 2019. These patients were treated with 125I interstitial brachytherapy alone, with prescribed doses of 100-120 Gy. The target volume was designed according to the pathological characteristics of ACC and recurrence sites. The pre- and post-operation dosimetric parameters (i.e., local control rates) were calculated using the treatment planning system. Results:In this study, the local recurrence sites included the superficial lobe (10/25) and deep lobe (7/25) of the parotid gland primarily and the skull base region (four patients) and mastoid posterior region (four patients) secondarily. The number of 125I seeds ranged from 16 to 111, with a median number of 59. The activity of radioactive particles was 18.5-25.9 MBq. The 3- and 5-year local control rates were 81.5% and 61.5%, respectively. No significant differences were found between pre- and post-operative dosimetric parameters such as D90, V100, and V150. There was no significant difference in local control rates among the four different recurrence sites. Conclusions:125I interstitial brachytherapy is proven to be an effective approach in the treatment of recurrent adenoid cystic carcinoma in the parotid gland. Satisfying local control rates can be achieved through target delineation performed according to recurrence sites and perineural invasion characteristics of ACC.