RESUMEN
Adenoid cystic carcinoma (ACC) is a rare tumour of the salivary glands characterised by distant metastases, mainly to lungs and bone. Isolated metastasis to the liver is unusual. We present the case of a woman with an ACC of the submandibular gland (pT1N0) who underwent radical submandibular gland excision and selective neck dissection. Preoperative imaging identified a liver lesion with features suggestive of a haemangioma. Two-year postoperatively, a surveillance CT neck/trunk showed an increase in size of the left liver lobe lesion. Subsequent MR liver and US-guided biopsy confirmed the lesion to be metastatic ACC. The patient underwent a successful left lateral liver sectionectomy. She remains disease-free 2.5 years after her liver resection. A literature search revealed only four other similar cases. This report highlights that even early-stage ACCs of the salivary gland may present with synchronous solitary liver metastasis which can be effectively treated with curative surgery.
Asunto(s)
Carcinoma Adenoide Quístico , Neoplasias Hepáticas , Neoplasias de la Glándula Submandibular , Humanos , Carcinoma Adenoide Quístico/secundario , Carcinoma Adenoide Quístico/patología , Carcinoma Adenoide Quístico/cirugía , Carcinoma Adenoide Quístico/diagnóstico por imagen , Femenino , Neoplasias Hepáticas/secundario , Neoplasias Hepáticas/patología , Neoplasias Hepáticas/cirugía , Neoplasias Hepáticas/diagnóstico por imagen , Neoplasias de la Glándula Submandibular/patología , Neoplasias de la Glándula Submandibular/secundario , Neoplasias de la Glándula Submandibular/cirugía , Tomografía Computarizada por Rayos X , Persona de Mediana Edad , Glándula Submandibular/patología , Glándula Submandibular/cirugía , Hepatectomía , Imagen por Resonancia Magnética , Disección del CuelloRESUMEN
ABSTRACT: Adenoid cystic carcinoma (ACC) of the Bartholin gland is an exceedingly rare neoplasm. A 56-year-old woman with remote ACC resection (plus neoadjuvant chemoradiation and adjuvant chemotherapy) presented to an outside institution with shortness of breath. CT showed bilateral pulmonary nodules and mediastinal lymphadenopathy. With high clinical suspicion for metastatic disease, 18 F-FDG PET/CT was performed and showed scattered nodules with mild FDG uptake along with FDG-avid mediastinal, bilateral hilar, and bilateral cervical chain lymphadenopathy. Lung biopsy of a hypermetabolic nodule confirmed metastatic ACC.
Asunto(s)
Glándulas Vestibulares Mayores , Carcinoma Adenoide Quístico , Tomografía Computarizada por Tomografía de Emisión de Positrones , Humanos , Carcinoma Adenoide Quístico/diagnóstico por imagen , Carcinoma Adenoide Quístico/patología , Carcinoma Adenoide Quístico/secundario , Femenino , Persona de Mediana Edad , Glándulas Vestibulares Mayores/diagnóstico por imagen , Glándulas Vestibulares Mayores/patología , Fluorodesoxiglucosa F18 , Tomografía Computarizada por Rayos X , Metástasis de la Neoplasia , Imagen MultimodalRESUMEN
Importance: There is no systemic therapy for recurrent or metastatic adenoid cystic carcinoma (ACC) approved by the US Food and Drug Administration. Objective: To examine the efficacy, safety, and tolerability of vascular endothelial growth factor receptor (VEGFR) inhibitors in recurrent or metastatic ACC. Data Sources: PubMed, Embase, and Cochrane Library were systematically searched for studies of VEGFR inhibitors in recurrent or metastatic ACC from database inception to August 31, 2023. Study Selection: Inclusion criteria were prospective clinical trials of recurrent or metastatic ACC treated with VEGFR inhibitors, reporting at least 1 outcome of interest specifically for ACC. Of 1963 identified studies, 17 (0.9%) met inclusion criteria. Data Extraction and Synthesis: The Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) reporting guideline was followed to extract data. Data were pooled using a random-effects generalized linear mixed model with 95% CIs. Main Outcomes and Measures: The primary efficacy outcome was best overall response to VEGFR inhibitors, including objective response, stable disease, or progressive disease (PD). Safety and tolerability outcomes included incidence of grade 3 or higher adverse events, rates of exit from trial due to PD or drug-related toxic effects, and dose reduction rate (DRR). Results: A total of 17 studies comprising 560 patients with recurrent or metastatic ACC treated with 10 VEGFR inhibitors were included. The objective response rate was 6% (95% CI, 3%-12%; I2 = 71%) and stable disease was the most frequent best overall response (82%; 95% CI, 74%-87%; I2 = 67%). The 6-month disease control (defined as objective response and stable disease) rate was 54% (95% CI, 45%-62%; I2 = 52%). The rate of grade 3 or higher adverse events was 53% (95% CI, 42%-64%; I2 = 81%) and of DRR was 59% (95% CI, 40%-76%). Most patients (57%; 95% CI, 44%-70%; I2 = 83%) continued therapy until PD; 21% (95% CI, 15%-28%; I2 = 62%) of patients suspended therapy for toxic effects. In subgroup analysis by specific VEGFR inhibitor, the objective response rate was 14% (95% CI, 7%-25%; I2 = 0%), stable disease rate was 76% (95% CI, 63%-85%; I2 = 0%), proportion treated until PD was 61% (95% CI, 14%-94%; I2 = 94%), and DRR was 78% (95% CI, 66%-87%; I2 = 39%) with lenvatinib. Corresponding axitinib results were objective response rate of 8% (95% CI, 4%-15%; I2 = 0%) and stable disease rate of 85% (95% CI, 72%-92%; I2 = 69%), with 73% (95% CI, 63%-82%; I2 = 0%) of patients treated until PD, and the DRR was 22% (95% CI, 12%-38%; I2 = 77%). Rivoceranib had the highest objective response rate (24%; 95% CI, 7%-57%) but high heterogeneity among studies (I2 = 95%) and the lowest rate of patients who continued therapy until PD (35%; 95% CI, 20%-55%; I2 = 90%). Conclusions and Relevance: This systematic review and meta-analysis found that VEGFR inhibitors were associated with high rates of disease stabilization in recurrent or metastatic ACC. Of 10 included VEGFR inhibitors, lenvatinib and axitinib were associated with the best combined and consistent efficacy, safety, and tolerability profiles, substantiating their inclusion in treatment guidelines.
Asunto(s)
Carcinoma Adenoide Quístico , Recurrencia Local de Neoplasia , Receptores de Factores de Crecimiento Endotelial Vascular , Humanos , Carcinoma Adenoide Quístico/tratamiento farmacológico , Carcinoma Adenoide Quístico/secundario , Receptores de Factores de Crecimiento Endotelial Vascular/antagonistas & inhibidores , Recurrencia Local de Neoplasia/tratamiento farmacológico , Antineoplásicos/uso terapéutico , Inhibidores de Proteínas Quinasas/uso terapéutico , Metástasis de la NeoplasiaRESUMEN
Adenoid cystic carcinoma (ACC) is a rare malignant epithelial neoplasm that arises in secretory glands and commonly metastasizes to the lungs. MYBL1 is frequently overexpressed in ACC and has been suggested to be a driver of the disease. In this study, we identified a circular RNA (circRNA) derived from MYBL1 pre-mRNA that was accompanied by the overexpression of MYBL1 in ACC. Overexpression of circMYBL1 was correlated with increased lung metastasis and poor overall survival in patients with ACC. Ectopic circMYBL1 overexpression promoted malignant phenotypes and lung metastasis of ACC cells. Mechanistically, circMYBL1 formed a circRNA-protein complex with CCAAT enhancer-binding protein ß (CEBPB), which inhibited ubiquitin-mediated degradation and promoted nuclear translocation of CEBPB. In the nucleus, circMYBL1 increased the binding of CEBPB to the CD44 promoter region and enhanced its transcription. In addition, circMYBL1 was enriched in small extracellular vesicles (sEV) isolated from the plasma of patients with ACC. Treatment with sEVs containing circMYBL1 in sEVs enhanced prometastatic phenotypes of ACC cells, elevated the expression of CD44 in human pulmonary microvascular endothelial cells (HPMEC), and enhanced the adhesion between HPMECs and ACC cells. Moreover, circMYBL1 encapsulated in sEVs increased the arrest of circulating ACC cells in the lung and enhanced lung metastatic burden. These data suggest that circMYBL1 is a tumor-promoting circRNA that could serve as a potential biomarker and therapeutic target for ACC. Significance: circMYBL1 stabilizes CEBPB and upregulates CD44 to promote adhesion between cancer cells and endothelial cells and enables lung metastasis of adenoid cystic carcinoma, suggesting that inhibition of this axis could improve patient outcomes.
Asunto(s)
Carcinoma Adenoide Quístico , Células Endoteliales , Vesículas Extracelulares , Receptores de Hialuranos , Neoplasias Pulmonares , ARN Circular , Humanos , Neoplasias Pulmonares/secundario , Neoplasias Pulmonares/metabolismo , Neoplasias Pulmonares/patología , Neoplasias Pulmonares/genética , Receptores de Hialuranos/metabolismo , Receptores de Hialuranos/genética , Carcinoma Adenoide Quístico/patología , Carcinoma Adenoide Quístico/metabolismo , Carcinoma Adenoide Quístico/genética , Carcinoma Adenoide Quístico/secundario , Ratones , Animales , Vesículas Extracelulares/metabolismo , Células Endoteliales/metabolismo , Células Endoteliales/patología , ARN Circular/genética , ARN Circular/metabolismo , Proteína beta Potenciadora de Unión a CCAAT/metabolismo , Proteína beta Potenciadora de Unión a CCAAT/genética , Línea Celular Tumoral , Femenino , Ratones Desnudos , Masculino , Regulación Neoplásica de la Expresión Génica , Proteínas Proto-Oncogénicas/metabolismo , Proteínas Proto-Oncogénicas/genética , Ratones Endogámicos BALB CAsunto(s)
Carcinoma Adenoide Quístico , Neoplasias Hepáticas , Femenino , Humanos , Persona de Mediana Edad , Carcinoma Adenoide Quístico/patología , Carcinoma Adenoide Quístico/secundario , Carcinoma Adenoide Quístico/cirugía , Hepatectomía/métodos , Neoplasias Hepáticas/secundario , Neoplasias Hepáticas/patología , Neoplasias Hepáticas/cirugía , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVE: To analyze the failure patterns in patients with different histological subtypes of sinonasal malignancies (SNMs). STUDY DESIGN: Retrospectively gathered data. SETTING: Academic university hospital. METHODS: Patients with SNMs treated at a tertiary referral center between January 1999 and January 2019 were included. We assessed the failure patterns within different histological subtypes. RESULTS: The study included 897 patients. The median follow-up time was 100 months. Adenoid cystic carcinoma (ACC) had a moderate risk of developing local recurrence (LR) and distant metastasis (DM). Compared with ACC, squamous cell carcinoma (SCC), adenocarcinoma (AC), soft tissue sarcoma (STS), and mucosal melanoma (MM) were classified as a high LR risk group. For DM, neuroendocrine carcinoma (NEC), STS, and MM were in the high-risk group. CONCLUSIONS: ACC had intermediate local and distant failure risks, while SCC, AC, STS, and MM were at high LR risks. NEC, STS, and MM were at high DM risk.
Asunto(s)
Carcinoma Adenoide Quístico , Carcinoma de Células Escamosas , Melanoma , Humanos , Estudios Retrospectivos , Carcinoma Adenoide Quístico/patología , Carcinoma Adenoide Quístico/secundario , Carcinoma Adenoide Quístico/terapia , Carcinoma de Células Escamosas/patología , Recurrencia Local de Neoplasia/patologíaAsunto(s)
Carcinoma Adenoide Quístico/diagnóstico por imagen , Carcinoma Adenoide Quístico/secundario , Fosa Craneal Media/patología , Neoplasias Nasofaríngeas/diagnóstico por imagen , Neoplasias Nasofaríngeas/secundario , Anciano , Carcinoma Adenoide Quístico/patología , Fosa Craneal Media/diagnóstico por imagen , Femenino , Humanos , Imagen por Resonancia Magnética , Neoplasias Nasofaríngeas/patología , Nasofaringe/patologíaRESUMEN
Novel systemic agents and effective treatment strategies for recurrence adenoid cystic carcinoma (ACC) of the head and neck are still worthy of further exploration. Here, we analyzed the mutations and expression profiles of 75 Chinese ACC patients, characterized the prognostic value of the immune signature for recurrence or distant metastasis, and explored the potential of immunotherapeutic biomarkers in ACC. In general, MYB fusion and somatic mutations accounted for a high proportion, which was 46.7% (35/75). ACCs displayed an overall low mutation burden and lack of programmed cell death ligand-1 (PD-L1) expression. The antigen-presenting machinery (APM) expression score and immune infiltration score (IIS) were the lowest among ACC patients, compared with other cancer types. For 61 primary cases, the locoregional recurrence-free survival (LRRFS) was statistically significantly correlated with the IIS [univariate analysis; hazard ratio (HR) = 0.32; 95% CI, 0.11-0.92; p = 0.035] and T-cell infiltration score (TIS) (univariate analysis; HR = 0.33; 95% CI, 0.12-0.94; p = 0.037]. Patients with lower IIS (log-rank p = 0.0079) or TIS (log-rank p = 0.0079) had shorter LRRFS. Additionally, solid pattern was also a prognostic factor related to locoregional recurrence, whereas postoperative radiotherapy (PORT) exerted its beneficial effects. We further evaluated the pretreatment immune profile of five ACC patients treated with PD-1 inhibitors. Patients who responded to camrelizumab or pembrolizumab observed elevated APM and TIS, compared with patients with progressive disease. Our study highlights the immune infiltration pattern and messenger RNA (mRNA) signatures of Chinese ACC patients, which has the potential value for prognosis and immunotherapy.
Asunto(s)
Antígeno B7-H1/análisis , Biomarcadores de Tumor/análisis , Linfocitos T CD8-positivos/inmunología , Neoplasias de Cabeza y Cuello/inmunología , Linfocitos Infiltrantes de Tumor/inmunología , Microambiente Tumoral/inmunología , Antígeno B7-H1/antagonistas & inhibidores , Biomarcadores de Tumor/genética , Linfocitos T CD8-positivos/efectos de los fármacos , Carcinoma Adenoide Quístico/tratamiento farmacológico , Carcinoma Adenoide Quístico/genética , Carcinoma Adenoide Quístico/inmunología , Carcinoma Adenoide Quístico/secundario , China , Bases de Datos Factuales , Femenino , Perfilación de la Expresión Génica , Fusión Génica , Neoplasias de Cabeza y Cuello/tratamiento farmacológico , Neoplasias de Cabeza y Cuello/genética , Neoplasias de Cabeza y Cuello/patología , Humanos , Inhibidores de Puntos de Control Inmunológico/uso terapéutico , Linfocitos Infiltrantes de Tumor/efectos de los fármacos , Masculino , Persona de Mediana Edad , Mutación , Recurrencia Local de Neoplasia , Estudios Retrospectivos , Transcriptoma , Resultado del Tratamiento , Secuenciación del ExomaRESUMEN
BACKGROUND: Adenoid cystic carcinoma (ACC) of the head and neck often develops lung metastasis. At present, there are not many research reports on ACC lung metastasis, little is known about its exact clinical features and treatment results, and there is no consensus on the best treatment strategy. This study explored the effective treatment strategies, clinical outcomes and long-term prognosis of head and neck ACC lung metastases. METHODS: The clinical and follow-up data of 76 patients with head and neck ACC lung metastases were retrospectively analyzed. According to the initial treatment of patients, they are divided into 4 groups: surgery, surgery+chemotherapy or radiotherapy, chemotherapy or radiotherapy and supportive treatment. The patients were staged according to the International Registry of Lung Metastases Staging System (IRLM). Kaplan-Meier method and Log-rank test were used to compare the statistical differences of overall survival (OS) and progression-free survival (PFS) of patients with different treatment methods and different IRLM stages. RESULTS: The OS and PFS of patients undergoing surgery are better than those of supportive therapy or radiotherapy and/or chemotherapy (OS: P<0.000,1; PFS: P<0.000,1). The OS and PFS of patients with low stage IRLM are better than those with high stage (OS: P<0.000,1; PFS: P<0.000,1). Patients with single lung metastasis and without pleural effusion have better OS and PFS. CONCLUSIONS: The long-term prognosis of patients with lung metastasis of head and neck ACC who undergo surgery is better than other treatments, which is related to higher OS and PFS. For patients with ACC lung metastases who are operationally eligible, the significance of complete surgical resection should be higher than other treatment options.
Asunto(s)
Carcinoma Adenoide Quístico , Neoplasias de Cabeza y Cuello , Neoplasias Pulmonares , Adulto , Carcinoma Adenoide Quístico/mortalidad , Carcinoma Adenoide Quístico/secundario , Carcinoma Adenoide Quístico/terapia , Femenino , Neoplasias de Cabeza y Cuello/mortalidad , Neoplasias de Cabeza y Cuello/patología , Neoplasias de Cabeza y Cuello/terapia , Humanos , Neoplasias Pulmonares/mortalidad , Neoplasias Pulmonares/secundario , Neoplasias Pulmonares/terapia , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Estudios Retrospectivos , Análisis de SupervivenciaRESUMEN
Adenoid cystic carcinoma (ACC) is a rare malignant tumor, usually arising from salivary glands and rarely found in other locations. ACC is characterized by asymptomatic course, slow growth pattern, perineural invasion and high incidence of late metastasis. Renal localization of metastasis is rare. Only 12 cases of renal metastasis were published and, to our best knowledge, no case of bilateral metastasis to the kidney has ever been reported. We present a case of a 58 years old woman with bilateral renal metastasis from ACC of the right lung after fourteen years from lobectomy and radiotherapy for the primary presentation. The patient underwent bilateral robot-assisted partial nephrectomy in a two-stage approach. Despite its rare incidence and slow growth, ACC metastasis may simulate primary papillary renal carcinoma and occur many years after primary treatment. Therefore, lifelong follow-up, including abdominal imaging, is recommended.
Asunto(s)
Carcinoma Adenoide Quístico/secundario , Neoplasias Renales/secundario , Riñón/patología , Neoplasias Pulmonares/patología , Carcinoma Adenoide Quístico/diagnóstico por imagen , Femenino , Humanos , Neoplasias Renales/diagnóstico por imagen , Pulmón/patología , Persona de Mediana Edad , Tomografía Computarizada por Rayos XAsunto(s)
Carcinoma Adenoide Quístico/radioterapia , Carcinoma Adenoide Quístico/secundario , Embolización Terapéutica , Neoplasias Hepáticas/radioterapia , Neoplasias Hepáticas/secundario , Radiofármacos/uso terapéutico , Neoplasias de las Glándulas Salivales/patología , Radioisótopos de Itrio/uso terapéutico , Adulto , Anciano , Carcinoma Adenoide Quístico/diagnóstico por imagen , Resultado Fatal , Femenino , Humanos , Neoplasias Hepáticas/diagnóstico por imagen , Persona de Mediana Edad , Radiofármacos/efectos adversos , Criterios de Evaluación de Respuesta en Tumores Sólidos , Neoplasias de las Glándulas Salivales/diagnóstico por imagen , Factores de Tiempo , Resultado del Tratamiento , Radioisótopos de Itrio/efectos adversosRESUMEN
Malignant salivary gland carcinomas arising primarily within the jaw bones are extremely rare. The most common salivary malignancy in these locations is mucoepidermoid carcinoma followed by adenoid cystic carcinoma and adenocarcinoma. The clinical picture and imaging studies of these malignancies may be confused with odontogenic lesions which are more common in this location. Adenoid cystic carcinomas have a prolonged clinical course, tendency for perineural invasion and distant metastasis and multiple recurrences. The diagnosis of these tumors requires thorough histopathologic examination. Immunohistochemical studies may be required in cases showing solid growth pattern. High-grade transformation, earlier termed as dedifferentiation, has been observed in several salivary gland carcinomas including adenoid cystic carcinoma. These transformed tumors are reported to have an extremely poor prognosis. Here, we report a case of primary intraosseous adenoid cystic carcinoma with extensive skeletal metastases which showed a negative staining with p63 and positive staining with CD117. The tumor had a predominant solid growth pattern with areas indicative of high-grade transformation. A negative p63 staining may indicate an incomplete or focal loss of abluminal layer and this is one of the criteria for high-grade transformation in adenoid cystic carcinoma.
Asunto(s)
Neoplasias Óseas/secundario , Carcinoma Adenoide Quístico/secundario , Transformación Celular Neoplásica/patología , Neoplasias Mandibulares/patología , Femenino , Humanos , Persona de Mediana EdadAsunto(s)
Carcinoma Adenoide Quístico/secundario , Neoplasias Pulmonares/secundario , Metástasis Linfática , Neoplasias de la Tiroides/secundario , Neoplasias de la Tráquea/patología , Adulto , Broncoscopía , Carcinoma Adenoide Quístico/diagnóstico , Carcinoma Adenoide Quístico/terapia , Terapia Combinada , Diagnóstico Diferencial , Femenino , Humanos , Tomografía Computarizada por Rayos X , Neoplasias de la Tráquea/diagnóstico por imagen , Neoplasias de la Tráquea/terapiaAsunto(s)
Neoplasias de Cabeza y Cuello/radioterapia , Recurrencia Local de Neoplasia/radioterapia , Radiocirugia/métodos , Neoplasias de la Base del Cráneo/radioterapia , Anciano , Carcinoma Adenoide Quístico/patología , Carcinoma Adenoide Quístico/radioterapia , Carcinoma Adenoide Quístico/secundario , Carcinoma Adenoide Quístico/cirugía , Femenino , Neoplasias de Cabeza y Cuello/diagnóstico por imagen , Neoplasias de Cabeza y Cuello/patología , Neoplasias de Cabeza y Cuello/cirugía , Humanos , Masculino , Neoplasias del Seno Maxilar/patología , Persona de Mediana Edad , Recurrencia Local de Neoplasia/diagnóstico por imagen , Recurrencia Local de Neoplasia/patología , Recurrencia Local de Neoplasia/cirugía , Cuidados Paliativos/métodos , Neoplasias de la Parótida/patología , Neoplasias de la Parótida/radioterapia , Neoplasias de la Parótida/cirugía , Dosificación Radioterapéutica , Estudios Retrospectivos , Medición de Riesgo , Neoplasias de la Base del Cráneo/diagnóstico por imagen , Neoplasias de la Base del Cráneo/patología , Neoplasias de la Base del Cráneo/cirugía , Carga TumoralRESUMEN
Knowledge on the role of matrix metalloproteinases (MMPs) in adenoid cystic carcinoma (ACC) is limited. MMPs are capable of degrading almost all extracellular and pericellular components to promote invasion and metastasis. This study aimed to evaluate the immunohistochemical expression of MMP-7, -8, -9, -15, and -25 in ACC and to relate the results with clinicopathological factors and survival. The study included 68 patients with minor salivary gland ACC treated at the Helsinki University Hospital (Helsinki, Finland) in 1974-2012. Samples from 52 patients were available, consisting of 44 primary tumours and eight recurrent tumours. We scored immunostaining of MMP-7, -8, -9, -15, and -25 and analysed the immunoscore against clinical and pathological parameters using statistical correlation test. MMP-9 immunoexpression in pseudocysts of ACC and in peritumoural inflammatory cells associated with better survival and fewer treatment failures. High tumoural MMP-7 and -25 associated with better survival. High tumoural MMP-15 associated with poorer survival and high tumoural MMP-9 with advanced stage and regional recurrences. Tumour cells did not show MMP-8 immunopositivity. These results suggest that MMP-9 may contribute to ACC carcinogenesis in different roles. MMP-7, -8, and -9 can stimulate signalling pathways that may promote tissue modulation and metastatic potential. MMP-15 and -25 may reflect prognosis.
Asunto(s)
Biomarcadores de Tumor/análisis , Carcinoma Adenoide Quístico/enzimología , Metaloproteinasa 15 de la Matriz/análisis , Metaloproteinasa 7 de la Matriz/análisis , Metaloproteinasa 8 de la Matriz/análisis , Metaloproteinasa 9 de la Matriz/análisis , Metaloproteinasas de la Matriz Asociadas a la Membrana/análisis , Neoplasias de las Glándulas Salivales/enzimología , Glándulas Salivales Menores/enzimología , Carcinoma Adenoide Quístico/mortalidad , Carcinoma Adenoide Quístico/secundario , Carcinoma Adenoide Quístico/terapia , Femenino , Proteínas Ligadas a GPI/análisis , Humanos , Inmunohistoquímica , Masculino , Estadificación de Neoplasias , Neoplasias de las Glándulas Salivales/mortalidad , Neoplasias de las Glándulas Salivales/patología , Neoplasias de las Glándulas Salivales/terapia , Glándulas Salivales Menores/patología , Resultado del TratamientoRESUMEN
Adenoid cystic carcinoma is a rare neoplasm that arises from secretory glands, most frequently from the salivary glands. Primary cutaneous adenoid cystic carcinoma is microscopically identical to adenoid cystic carcinoma developing at other tissues. Therefore, differentiating between a primary cutaneous adenoid cystic carcinoma and an extracutaneous adenoid cystic carcinoma with cutaneous metastases is pivotal to determine its prognosis and management. We describe a case of primary cutaneous adenoid cystic carcinoma on the abdomen that was successfully treated with wide excision.
Asunto(s)
Carcinoma Adenoide Quístico/diagnóstico , Neoplasias Cutáneas/diagnóstico , Piel/patología , Abdomen , Anciano , Biopsia , Carcinoma Adenoide Quístico/patología , Carcinoma Adenoide Quístico/secundario , Diagnóstico Diferencial , Humanos , Masculino , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/secundarioRESUMEN
Adenoid cystic carcinoma (ACC) is most commonly seen in the salivary glands but may occur at other sites. Primary or metastatic involvement of the skin is unusual. We report 2 cases of ACC with unusual presentation. In the first case, a 55-year-old woman presented with a cutaneous lesion on the right shin, and final pathology showed ACC. An extracutaneous origin was excluded by clinical and imaging studies. In the second case, a 49-year-old woman presented with a nodule on the breast, and biopsy confirmed high-grade ACC (>30% solid areas). She underwent lumpectomy and subsequent mastectomy after recurrence. Sixteen months after the initial diagnosis of ACC of the breast, distant metastases at multiple sites, including the skin, were identified. This report will increase awareness of these rare presentations of cutaneous ACC and allow correct diagnosis and appropriate management of such cases.
Asunto(s)
Neoplasias de la Mama/patología , Carcinoma Adenoide Quístico/secundario , Neoplasias Cutáneas/patología , Biomarcadores de Tumor/análisis , Biopsia , Neoplasias de la Mama/química , Neoplasias de la Mama/terapia , Carcinoma Adenoide Quístico/química , Carcinoma Adenoide Quístico/terapia , Progresión de la Enfermedad , Resultado Fatal , Femenino , Humanos , Inmunohistoquímica , Persona de Mediana Edad , Clasificación del Tumor , Neoplasias Cutáneas/química , Neoplasias Cutáneas/secundario , Neoplasias Cutáneas/terapia , Resultado del TratamientoRESUMEN
OBJECTIVE: Our facility measures programmed cell death ligand 1 (PD-L1) expression in all patients before administering nivolumab. The aim of the present study is to clarify the association between overall survival (OS) and PD-L1 expression. PATIENTS AND METHODS: Subjects in this study were 52 patients with R/M-HNC cancer (45 men, 7 women) administered nivolumab in our facility between June 1, 2017 and January 31, 2019. Mean age was 62.2 years (median, 65 years; range, 28-81 years). Histopathological type was squamous cell carcinoma (SCC) in 48 cases, and non-SCC in 4 cases. We set OS as the primary endpoint and progression-free survival (PFS), overall response rate (ORR), association of OS and PD-L1 expression and association of PFS and PD-L1 expression as secondary endpoints. The cut-off for PD-L1 expression was set using the receiver operating characteristic (ROC) curve. We compared OS, PES and ORR using this PD-L1 cut-off for all patients and for the SCC group. OS and PFS were calculated using Kaplan-Meier methods. The log-rank test was used for statistical analysis, with values of p < 0.05 taken as significant. For PD-L1 immunohistochemistry assays, Dako 28-8 antibody was used. RESULTS: In the all-patients group, median OS was 9.6 months and 1-year OS rate was 40.4%. Median PFS was 4.0 months and 1-year PFS rate was 37.8%. The cut-off value of PD-L1 expression for OS was 40% for all patients and the SCC group. When PD-L1 expression was ≥40%, OS was significantly better in both all patients and the SCC group (p = 0.004, 0.007). The cut-off value of PD-L1 expression for PFS was also 40%. When PD-L1 expression was ≥40%, PFS was better in all patients and the SCC group (P = 0.003, 0.009). In the all-patients group, ORR was 19.2% and disease control rate (DCR) was 44.2%. When PD-L1 expression was ≥40%, ORR was 44.4% and DCR 83.3%. CONCLUSION: In the present study, when PD-L1 expression was high (≥40%), OS was significantly better (p = 0.004). This finding has not been reported in other research on R/M-HNC. PFS and ORR were also better with high PD-L1 expression. Regarding patterns of progression with a PD-L1 expression cut-off of 40%, hyperprogression was significantly more frequent for PD-L1 expression <40% (p = 0.039). Therefore, high PD-L1 expression could offer a predictor of prognosis and efficacy for nivolumab. The present findings may prove useful in considering treatment strategies.