RESUMEN
BACKGROUND: Pancreatic acinar cell carcinoma (PACC) is a rare pancreatic neoplasm. Recently, molecular analysis revealed that PACC shows a high frequency of the BRCA1/2 mutation and is likely to be considered a cancer associated with hereditary breast and ovarian cancer (HBOC). Hereditary cancers, including HBOC, are characterized by multifocal and/or metachronous tumors. However, no case reports exist of germline BRCA1-mutated synchronous and metachronous PACC. CASE: A 58-year-old man was diagnosed with synchronous and metachronous PACC at the age of 56 and underwent two surgeries. Ten months after the second surgery, the patient developed multiple liver metastases. Gemcitabine plus nab-paclitaxel therapy was administered as first-line chemotherapy. After seven cycles, computed tomography examination revealed progressive disease (PD). Therefore, modified FOLFIRINOX (mFFX) was administered as second- line chemotherapy. After 19 cycles of mFFX, comprehensive cancer genomic profiling (CGP) identified a BRCA1 pathogenic variant that was confirmed to be germline origin. Accordingly, we treated the patient with olaparib; however, he was diagnosed with PD after 4 months. He subsequently died 5 years and 9 months after the initial surgery, and 3 years and 10 months after chemotherapy. Based on the genetic data of the patients, his family members received genetic counseling followed by cascade testing. Consequently, the same gBRCA1 pathogenic variant was detected in the son and his surveillance for HBOC-related cancers was initiated. CONCLUSION: We diagnosed a 58-year-old man with a synchronous and metachronous PACC with germline BRCA1 pathogenic variant. Considering that PACC is likely to have BRCA1/2 mutations responsible for HBOC, we need to be aware of the possible presence of multifocal and/or metachronous tumors in patients with PACC. Additionally, patients with PACC should undergo genetic examinations, which would be beneficial in determining treatment strategies and health care for blood relatives.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica , Proteína BRCA1 , Carcinoma de Células Acinares , Mutación de Línea Germinal , Neoplasias Pancreáticas , Humanos , Persona de Mediana Edad , Masculino , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/patología , Proteína BRCA1/genética , Carcinoma de Células Acinares/genética , Carcinoma de Células Acinares/patología , Carcinoma de Células Acinares/diagnóstico , Carcinoma de Células Acinares/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Primarias Secundarias/genética , Neoplasias Primarias Secundarias/patología , Neoplasias Primarias Secundarias/diagnóstico , Neoplasias Primarias Múltiples/genética , Neoplasias Primarias Múltiples/patología , Neoplasias Primarias Múltiples/terapia , Oxaliplatino/administración & dosificación , Leucovorina/administración & dosificación , Irinotecán/administración & dosificación , Paclitaxel/administración & dosificación , Fluorouracilo/administración & dosificaciónRESUMEN
Pancreatic acinar cell carcinoma (PACC) is a rare cancer with no specific treatment. The treatment and chemotherapy for PACC are selected according to pancreatic ductal adenocarcinoma (PDAC). Herein, we describe a recurrent PACC case of an older adult patient. The patient was treated with systemic chemotherapy, chemoradiotherapy, and maintenance therapy based on the pathologic germline BRCA2 variant, resulting in long-term survival. The pathogenic BRCA variant is detected more frequently in patients with PACC than in those with PDAC. The BRCA variant significantly impacts treatment selection and prognosis; therefore, early genomic analysis is recommended when treating PACC.
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Carcinoma de Células Acinares , Quimioradioterapia , Recurrencia Local de Neoplasia , Neoplasias Pancreáticas , Humanos , Neoplasias Pancreáticas/terapia , Carcinoma de Células Acinares/terapia , Quimioradioterapia/métodos , Recurrencia Local de Neoplasia/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Anciano , Masculino , Quimioterapia de Mantención , Proteína BRCA2/genética , Mutación de Línea GerminalRESUMEN
PURPOSE: We performed a population-based cohort study to investigate the clinical characteristics and survival rates of primary malignant parotid tumors (MPT) in children and adolescents. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was used to identify all pediatric and adolescent patients with MPT who were diagnosed between 2000 and 2018. Based on a number of parameters, survival curves were produced using Kaplane-Meier estimates. The log-rank test was used to compare survival curves. The influence of each component on overall survival (OS) was examined using a multivariate Cox proportional hazards model. RESULTS: There were 352 identified pediatric and adolescent patients with MPT. At diagnosis, the age ranged from 1.0 to 19 years, with a median of 15 years. Mucoepidermoid carcinoma (MC) (46.5 %) was the most common histological subtype, followed by acinar cell carcinoma (ACC) (36.4 %) and others (17.1 %) such as adenoid cystic carcinoma and squamous cell carcinoma. All patients had overall survival rates of 98.8 %, 95.6 %, and 94.6 % at 1-year, 3-year and 5-year, respectively. The results of the Cox proportional hazard regression showed that tumor grade, SEER stage, radiotherapy, and treatment regimens were significant independent predictors of overall survival. CONCLUSIONS: In pediatric and adolescent MPT, tumor grade, SEER stage, adjuvant radiation, and treatment regimens were found to be important independent predictors of survival. More research is required to validate the role of adjuvant radiation.
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Carcinoma Mucoepidermoide , Neoplasias de la Parótida , Programa de VERF , Humanos , Adolescente , Niño , Neoplasias de la Parótida/epidemiología , Neoplasias de la Parótida/diagnóstico , Neoplasias de la Parótida/patología , Neoplasias de la Parótida/terapia , Neoplasias de la Parótida/mortalidad , Masculino , Femenino , Programa de VERF/estadística & datos numéricos , Pronóstico , Preescolar , Lactante , Carcinoma Mucoepidermoide/epidemiología , Carcinoma Mucoepidermoide/diagnóstico , Carcinoma Mucoepidermoide/patología , Carcinoma Mucoepidermoide/terapia , Carcinoma Mucoepidermoide/mortalidad , Tasa de Supervivencia , Adulto Joven , Modelos de Riesgos Proporcionales , Carcinoma de Células Acinares/epidemiología , Carcinoma de Células Acinares/diagnóstico , Carcinoma de Células Acinares/patología , Carcinoma de Células Acinares/terapia , Estudios de Cohortes , Estados Unidos/epidemiología , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/epidemiología , Carcinoma de Células Escamosas/patología , Carcinoma de Células Escamosas/terapia , Carcinoma Adenoide Quístico/epidemiología , Carcinoma Adenoide Quístico/diagnóstico , Carcinoma Adenoide Quístico/terapia , Carcinoma Adenoide Quístico/patología , Carcinoma Adenoide Quístico/mortalidad , Estadificación de Neoplasias , Estimación de Kaplan-MeierRESUMEN
OBJECTIVES: To characterize the presentation and treatment of children presenting with primary salivary gland neoplasms. METHODS: A retrospective review of primary salivary tumor patients presenting to Children's Hospital Colorado between January 2000 and August 2020. RESULTS: Fifty children were identified with primary salivary gland tumors, comprising of 39 (78 %) benign and 11 (22 %) malignant lesions. Pleomorphic adenoma was the most common benign tumor (36/39, 92 %), while acinic cell carcinoma was the most common malignancy (7/11, 64 %). The parotid gland was the most common site, followed by the submandibular gland (66 % vs. 34 %). No tumors were found in the sublingual glands. Benign neoplasms accounted for 70 % of parotid lesions and 94 % of submandibular tumors. No significant differences in age (13.6 years, SD 4 vs. 13.0 years, SD 4.3) were noted between patients with benign and malignant disease, but tumors in females were more frequently malignant (M:F 1:1.3 vs. 1:2.7 for benign and malignant tumors, respectively). Neck dissection and/or facial nerve sacrifice were required in 27 % (3/11) and 9.1 % (1/11) of malignancies, respectively. Local recurrence was observed in 7.7 % (3/39) of benign cases and 9.1 % (1/11) of malignant cases. No salivary malignancies required chemotherapy, though one patient with neurofibromatosis received imatinib prior to resection. Two patients with locoregional malignancy received adjunctive radiation. The average duration of follow up for benign and malignant disease were 12.6 ± 25 and 45.1 ± 32 months, respectively. CONCLUSIONS: This study presents one of the larger single institutional experiences of pediatric primary salivary neoplasms in the past 20 years, identifying pleomorphic adenoma and acinic cell carcinoma as the most common benign and malignant etiologies, respectively. While this review found most neoplasms presented as a localized mass effectively managed with conservative surgical resection, aggressive tumors required multidisciplinary care.
Asunto(s)
Adenoma Pleomórfico , Carcinoma de Células Acinares , Neoplasias de la Parótida , Neoplasias de las Glándulas Salivales , Femenino , Humanos , Niño , Adolescente , Adenoma Pleomórfico/patología , Carcinoma de Células Acinares/terapia , Carcinoma de Células Acinares/patología , Neoplasias de las Glándulas Salivales/epidemiología , Neoplasias de las Glándulas Salivales/terapia , Neoplasias de las Glándulas Salivales/patología , Glándula Parótida/patología , Glándula Submandibular , Estudios Retrospectivos , Neoplasias de la Parótida/patologíaRESUMEN
Acinar cell carcinoma (ACC) is a rare pancreatic malignancy with distinctive clinical, molecular, and morphological features. The long-term survival of ACC patients is substantially superior to that of pancreatic adenocarcinoma patients. As there are no significant patient series about ACCs, our understanding of this illness is mainly based on case reports and limited patient series. Surgical resection is the treatment of choice for patients with the disease restricted to one organ; however, with recent breakthroughs in precision medicine, medicines targeting the one-of-a-kind molecular profile of ACC are on the horizon. There are no standard treatment protocols available for people in which a total surgical resection to cure the condition is not possible. As a result of shared genetic alterations, ACCs are chemosensitive to agents with activity against pancreatic adenocarcinomas and colorectal carcinomas. The role of neoadjuvant or adjuvant chemoradiotherapy has not been established. This article aims to do a comprehensive literature study and present the most recent information on acinar cell cancer.
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Carcinoma de Células Acinares , Neoplasias Pancreáticas , Humanos , Carcinoma de Células Acinares/genética , Carcinoma de Células Acinares/terapia , Carcinoma de Células Acinares/patología , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/terapia , Neoplasias Pancreáticas/patología , Inmunohistoquímica , Mutación , Neoplasias PancreáticasRESUMEN
BACKGROUND: Acinic cell carcinoma (ACC) is a salivary gland malignancy that rarely can involve the sinonasal cavity. There are limited outcomes data available to guide management. OBJECTIVE: We sought to use the National Cancer Database (NCDB) to characterize treatment outcomes in sinonasal ACC. METHODS: A retrospective analysis of the NCDB from 2004 to 2016 for patients with ACC involving the sinonasal cavity was conducted. Demographic, treatment, and survival information were obtained. Unadjusted Kaplan-Meier estimates, log-rank tests, and a multivariable Cox proportional hazard model were used to assess overall survival (OS). RESULTS: A total of 28 patients were included in the analysis with an average age of 58.6 ± 15.5 years. Half the patients (n = 14, 50%) were male, mostly white (n = 23, 82.1%), and with private insurance (n = 16, 57.1%). The nasal cavity was the most common subsite (n = 18, 64.3%), followed by the maxillary sinus (n = 5, 17.9%). Most patient received surgery alone (n = 17, 60.7%), with the remaining patients undergoing surgery followed by radiation (n = 8, 28.6%), radiation alone (n = 1, 3.6%), and no treatment (n = 2, 7.1%). The 1-, 5-, and 10-year survival in this cohort was 100% (95% CI: 100%-100%), 84.3% (95% CI: 71.2%-99.7%), and 72.2% (95% CI: 55%-94.8%), respectively. On multivariate analysis, older age was associated with worse OS (hazard ratio (HR): 1.27; 95% CI: 1.11-1.46, P < .001). Disease of the sphenoid sinus correlated with worse survival (HR: 198, 95% CI: 10.4-3,739, P < .001) and large tumor size was associated with worse OS on log-rank test, but not on multivariate analysis. CONCLUSION: Sinonasal ACC is a rare entity with relatively good long-term outcomes. Older age and primary disease of the sphenoid sinus are associated with worse outcomes. Most patients are treated with surgical resection. Future research is needed to assess the optimal timing and indications for radiation therapy.
Asunto(s)
Carcinoma de Células Acinares , Neoplasias de los Senos Paranasales , Adulto , Anciano , Carcinoma de Células Acinares/epidemiología , Carcinoma de Células Acinares/terapia , Femenino , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Neoplasias de los Senos Paranasales/epidemiología , Neoplasias de los Senos Paranasales/terapia , Modelos de Riesgos Proporcionales , Estudios RetrospectivosRESUMEN
OBJECTIVES: To analyze the demographic data, surgical and adjuvant treatment data and the survival outcomes in adult patients affected by acinic cell carcinoma of the parotid gland (AciCC). METHODS: A retrospective multicenter analysis of patients treated for AciCC of the parotid gland from 2000 to 2021 was performed. Exclusion criteria were pediatric (0-18 years) patients, the absence of follow-up and patients with secondary metastatic disease to the parotid gland. Multivariable logistic regression was used to determine factors associated with survival. RESULTS: The study included 81 adult patients with AciCC of the parotid gland. The median age was 46.3 years (SD 15.81, range 19-84 years), with a gender female prevalence (F = 48, M = 33). The mean follow-up was 77.7 months (min 4-max 361, SD 72.46). The 5 years overall survival (OS) was 97.5%. The 5 years disease-free survival (DFS) was 60%. No statistical differences have been found in prognosis for age (< 65 or ≥ 65 years), sex, surgery type (superficial vs profound parotid surgery), radicality (R0 vs R1 + Rclose), neck dissection, early pathologic T and N stages and adjuvant therapy (p > 0.05). CONCLUSION: This study did not find prognostic factor for poorest outcome. In contrast with the existing literature, our results showed how also high-grade tumours cannot be considered predictive of recurrence or aggressive behaviour.
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Carcinoma de Células Acinares , Neoplasias de la Parótida , Adulto , Humanos , Femenino , Niño , Adulto Joven , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Carcinoma de Células Acinares/terapia , Carcinoma de Células Acinares/patología , Glándula Parótida/patología , Neoplasias de la Parótida/cirugía , Glucosamina , Pronóstico , Estudios Retrospectivos , Estadificación de NeoplasiasRESUMEN
OBJECTIVE: Acinic cell carcinoma (AciCC) is a rare, usually low-grade salivary malignancy. Evidence on rates of lymph node metastases (LNMs) is limited in pediatric patients and varies significantly (4%-45%) in adults. We set out to determine and compare rates of LNMs in pediatric and adult AciCC and to analyze their impact on survival, using the National Cancer Database. STUDY DESIGN: Historical cohort study. SETTING: National Cancer Database. METHODS: All AciCCs of the major salivary glands with complete clinical and pathologic nodal staging were selected between 2010 and 2016. Patient demographics, tumor characteristics, treatment, and survival were analyzed. Univariable and multivariable regression were performed to determine factors associated with LNMs and survival. RESULTS: We identified 57 (4.6%) pediatric patients (<18 years) and 1192 (95.4%) adults with AciCC. Clinical LNMs were rare in pediatric patients (n < 10) and adults (n = 88, 7.4%). Occult LNMs were uncommon in pediatric patients (n < 5) and adults (n = 41, 4.6%). Three-year overall survival for pediatric patients was 97.8%. Adults with LNM had worse 3-year overall survival than those without (66.0% vs 96.3%, P < .001). In multivariable regression, high-grade disease (hazard ratio, 10.15 [95% CI, 5.60-18.80]; P < .001) and T3-T4 tumors (hazard ratio, 2.80 [95% CI, 1.56-4.97]; P < .001) were associated with LNM in adult patients. CONCLUSION: LNMs in AciCC of the major salivary glands are rare in children and adults. However, high-grade and T3-T4 tumors are associated with an increased risk of LNM. LNM is associated with worse survival.
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Carcinoma de Células Acinares , Neoplasias de las Glándulas Salivales , Adulto , Humanos , Niño , Carcinoma de Células Acinares/terapia , Carcinoma de Células Acinares/patología , Estudios de Cohortes , Glándulas Salivales/patología , Metástasis Linfática , Neoplasias de las Glándulas Salivales/terapia , Neoplasias de las Glándulas Salivales/patología , Estudios RetrospectivosRESUMEN
Resumen Los tumores malignos de glándulas salivales (TMGS) constituyen un grupo infrecuente de cánceres de una gran variedad histológica. Dentro de las neoplasias de estirpe epitelial encontramos al carcinoma de células acinares (CCA), que representa entre un 6%-10% de todos los TMGS. No posee patrones clínicos ni radiológicos específicos, pero comparte características comunes con otros TMGS que serán discutidas a lo largo del reporte. Cabe destacar que en términos generales es un cáncer de bajo grado y poco recidivante, por lo que un tratamiento oportuno y un seguimiento estricto mejoran el pronóstico para este tipo de pacientes. Su diagnóstico se establece con evidencia histopatológica que confirme la presencia de diferenciación epitelial de tipo acinar. El tratamiento consiste en la exéresis tumoral con márgenes libres por parotidectomía suprafacial o total, asociado a vaciamiento cervical si se detecta compromiso nodal. Adicionalmente, se debe sugerir terapia adyuvante ante la presencia de un factor de mal pronóstico. Se presenta un caso y se realiza revisión de literatura.
Abstract Malignant salivary gland tumors (TMGS) constitute an infrequent group of cancers of a wide histological variety. Within the epithelial lineage neoplasms, we find acinar cell carcinoma that represent between 6%-10% of all TMGS. It does not have specific clinical or radiological patterns, but it shares common characteristics with other TMGS that will be discussed throughout the report. It should be noted that in general terms it is a low-grade cancer with low recurrence rates, so timely treatment and strict follow-up improve the prognosis for this type of patient. Its diagnosis is established with histopathological evidence that confirms the presence of acinar-type epithelial differentiation. Treatment consists of tumor excision with free margins by suprafacial or total parotidectomy, associated with cervical lymph node dissection if nodal locoregional metastasis is detected. Additionally, adjuvant therapy should be suggested in the presence of a poor prognostic factor. A case is presented and a literature review is carried out.
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Humanos , Masculino , Anciano , Neoplasias de la Parótida/diagnóstico , Carcinoma de Células Acinares/diagnóstico , Pronóstico , Neoplasias de la Parótida/terapia , Tomografía Computarizada por Rayos X/métodos , Carcinoma de Células Acinares/terapiaRESUMEN
PURPOSE: Hospital-based clinical studies have limitations in holistic assessment of cancer treatment and prognosis, as they omit out-of-hospital patients including elderly individuals. This study aimed to investigate trends in initial treatment and corresponding prognosis of patients with exocrine pancreatic cancer (EPC) in Korea. MATERIALS AND METHODS: The Korea Central Cancer Registry data of patients with EPC from 2006 to 2017 were retrospectively reviewed. We defined the first course of treatment (FT) as the cancer-directed treatment administered within four months after cancer diagnosis according to the Surveillance, Epidemiology, and End Results (SEER) program. RESULTS: Among 62,209 patients with EPC, localized and regional (LR) SEER stage; patients over 70 years old; and ductal adenocarcinoma excluding cystic or mucinous (DAC) accounted for 40.6%, 50.1%, and 95.9%, respectively. "No active treatment" (NT, 46.5%) was the most frequent, followed by non-surgical FT (28.7%) and surgical FT (22.0%). Among 25,198 patients with LR EPC, surgical FT increased (35.9% to 46.3%) and NT decreased (45.0% to 29.5%) from 2006 to 2017. The rate of surgical FT was inversely related to age (55.1% [< 70 years], 37.3% [70-79 years], 10.9% [≥ 80 years]). Five-year relative survival rates of LR DAC were higher after surgical FT than after NT in localized (46.1% vs. 12.9%) and regional stage (23.6% vs. 4.9%) from 2012 to 2017. CONCLUSION: Less than half of overall patients with LR EPC underwent surgical FT, and this proportion decreased significantly in elderly individuals. Clinicians should focus attention on elderly patients with EPC to provide appropriate medical advice.
Asunto(s)
Adenocarcinoma/epidemiología , Carcinoma de Células Acinares/epidemiología , Neoplasias Pancreáticas/epidemiología , Adenocarcinoma/terapia , Adulto , Anciano , Anciano de 80 o más Años , Carcinoma de Células Acinares/terapia , Atención a la Salud/estadística & datos numéricos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neoplasias Pancreáticas/terapia , Sistema de Registros , República de Corea/epidemiología , Estudios RetrospectivosRESUMEN
Secretory carcinoma (SC) and acinic cell carcinoma (AciCC) are two rare tumors originating in the salivary gland of the head and neck. Before the World Health Organization (WHO) classified SC as a new entity in 2017, the majority of SC cases were incorrectly diagnosed as AciCC. Indeed, they are similar in biological behaviors, clinical manifestations and histomorphological features. Especially, SC and zymogen granule-poor AciCC are difficult to differentiate, which brings a tough challenge in clinical diagnosis. This article provides an updated understanding of the differential diagnosis in SC and AciCC from two main perspectives: histopathology and molecular genetics. The targeted therapies for both tumors are also mentioned. It aims to give some hints in clinical diagnosis and treatment, in hopes that patients with adequate diagnosis could obtain the opportunityformore effective treatment.
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Carcinoma de Células Acinares , Carcinoma , Neoplasias de las Glándulas Salivales , Biomarcadores de Tumor , Carcinoma/diagnóstico , Carcinoma/terapia , Carcinoma de Células Acinares/diagnóstico , Carcinoma de Células Acinares/terapia , Diagnóstico Diferencial , Humanos , Neoplasias de las Glándulas Salivales/diagnóstico , Neoplasias de las Glándulas Salivales/terapiaRESUMEN
OBJECTIVES: Acinar cell pancreatic carcinomas (ACPCs) are rare neoplasms accounting for 1% to 2% of pancreatic tumors in adults. The objective of this study is to evaluate the benefit of chemotherapy in the adjuvant setting in resected ACPC and in the palliative setting for metastatic ACPC. METHODS: Data were obtained from all US hospitals that contributed to the National Cancer Database between 2004 and 2014. Cases were identified using the histology code 8550. RESULTS: A total of 593 patients with ACPC were identified. The mean age was 64.4 years (range, 18-90 years), with a male preponderance (72.8%, n = 432). Localized stage disease comprised 52.3% (n = 310) of patients. Among localized ACPC patients, 88.0% (n = 191) underwent surgery and 50.6% (n = 91) received adjuvant chemotherapy. The 5-year overall survival in those who received adjuvant treatment was slightly higher than those who did not receive adjuvant treatment (46.7% vs 44.8%, P = 0.3271). Among advanced-stage ACPC patients, 67.6% received chemotherapy, which translated into improved 5-year overall survival compared with no chemotherapy (8.1% vs 0%, P < 0.0001). CONCLUSIONS: Chemotherapy in the palliative setting for advanced-stage ACPC patients was associated with improved survival. Adjuvant therapy did not translate into significant survival benefit.
Asunto(s)
Carcinoma de Células Acinares/terapia , Bases de Datos Factuales/estadística & datos numéricos , Evaluación de Resultado en la Atención de Salud/estadística & datos numéricos , Neoplasias Pancreáticas/terapia , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Carcinoma de Células Acinares/patología , Quimioterapia Adyuvante/métodos , Estudios de Cohortes , Terapia Combinada/métodos , Femenino , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Evaluación de Resultado en la Atención de Salud/métodos , Pancreatectomía/métodos , Neoplasias Pancreáticas/patología , Radioterapia Adyuvante/métodos , Estados Unidos , Adulto JovenRESUMEN
ABSTRACT: Cutaneous metastases from parotid tumors are uncommon and imply a poor prognosis. In this article, we report 2 new cutaneous metastasis cases from 2 different parotid malignancies and 42 additional cases from a literature review. Clinical manifestations, localization, and outcomes of skin metastasis from parotid tumors are described. Although infrequent, parotid neoplasms may develop skin metastasis many years after the initial diagnosis, mostly in the head and neck region. Therefore, long-term follow-up and periodic skin examination of these patients is mandatory. Dermatologists and surgeons must have a high index of suspicion when evaluating any skin lesion arising on these patients because cutaneous metastasis from parotid neoplasms generally implies a poor prognosis.
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Adenoma Pleomórfico/patología , Carcinoma de Células Acinares/secundario , Neoplasias de la Parótida/patología , Neoplasias Cutáneas/secundario , Adenoma Pleomórfico/terapia , Anciano , Carcinoma de Células Acinares/terapia , Femenino , Humanos , Persona de Mediana Edad , Neoplasias de la Parótida/terapia , Neoplasias Cutáneas/terapia , Factores de Tiempo , Resultado del TratamientoRESUMEN
OBJECTIVES: To evaluate the rate of lymph node metastasis in parotid gland acinar cell carcinoma, to identified factors associated with increased risk of metastasis, and to evaluate the effect of nodal metastasis on survival. METHODS: This is a retrospective cohort study utilizing data from a large population-based cancer database. Data were extracted from the Surveillance, Epidemiology, and End Results (SEER) 18 database. The study cohort included patients with parotid gland acinar cell carcinoma diagnosed between 2000 and 2015. RESULTS: The overall rate of lymph node metastasis was 6.8%. T3/T4 (OR 6.17, 95% CI, 3.03 to 13.16) disease along with High Grade (OR 15.95) disease were associated with increased risk of nodal metastasis. Non-white, non-Black race was associated with decreased risk. Age and sex were not associated with nodal metastasis. Nodal metastasis was associated with worse OS (HR 6.27, 95% CI, 3.85 to 10.20) and DSS (HR 6.96, 95% CI, 3.81 to 12.73) after adjusting for covariates. CONCLUSION: Parotid gland acinar cell carcinoma carries a low risk of nodal metastasis. Both advanced T stage and high grade are associated with increased risk of nodal metastasis. Nodal metastasis is associated with decreased overall survival. LEVEL OF EVIDENCE: 3 Laryngoscope, 131:E1125-E1129, 2021.
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Carcinoma de Células Acinares/patología , Metástasis Linfática/patología , Neoplasias de la Parótida/patología , Carcinoma de Células Acinares/terapia , Femenino , Humanos , Metástasis Linfática/terapia , Masculino , Persona de Mediana Edad , Clasificación del Tumor , Estadificación de Neoplasias , Neoplasias de la Parótida/terapia , Estudios Retrospectivos , Factores de Riesgo , Programa de VERF , Estados UnidosRESUMEN
This is a review of the clinical and histopathological published data on very rare heterotopic acinic cell carcinomas (AcCCs) with suggested optimal management. Extrasalivary AcCCs originate primarily in parotid lymph nodes. They present at low clinical stage, show mostly low-grade histopathology and are circumscribed with a complete nodal capsule. Extracapsular dissection was advocated as adequate therapy. In rare cases with positive surgical margins, a completion parotidectomy or adjuvant radiotherapy should follow. Heterotopic high-grade AcCCs are rare, necessitating radical surgery including neck dissection and adjuvant radiotherapy. The short term prognosis is excellent, long term outcomes are not known. Longer term follow-up is essential.
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Carcinoma de Células Acinares , Carcinoma de Células Acinares/diagnóstico , Carcinoma de Células Acinares/terapia , HumanosRESUMEN
BACKGROUND/OBJECTIVES: Mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) of the pancreas and periampullary region are extremely rare and heterogeneous malignancies. Literature is sparse, clinical management is not standardized and little is known about survival outcomes. The aim of this study was to identify pathological and radiological features of MiNEN and assess the outcome of surgical management. METHODS: Patients undergoing surgery for pancreatic and periampullary MiNEN between 2001 and 2019 were retrospectively analysed based on a prospective database. Histological, radiological and clinical features were assessed. Survival was analysed in a nested case-control study and matched-pair analyses with pure neuroendocrine neoplasms (pNEN) and ductal adeno- or acinar cell carcinomas of the pancreas. A literature review with focus on survival after surgical resection was additionally performed. RESULTS: Of 13 patients with MiNEN, 5 had acinar-MiNEN and 8 adeno-MiNEN. Two of 5 (40%) acinar-MiNEN and one adeno-MiNEN patients had liver metastases. All but one adeno-MiNEN (88%) showed preoperative radiological features of pancreatic adenocarcinoma, 3 of 5 (60%) acinar-MiNEN exhibited mainly neuroendocrine features. No surgical mortality was observed. The 5-year overall survival rate in all MiNEN was 40%. Five-year survival rate was 58% in adeno-MiNEN and comparable to that of matched ductal adenocarcinomas (36%) and pNEN (48%). Five-year overall survival rate was 20% in acinar-MiNEN, compared to 39% in acinar carcinoma patients and 59% in matched pNEN patients. CONCLUSIONS: MiNEN are rare and difficult to distinguish from pure adenocarcinoma or neuroendocrine neoplasm preoperatively. Surgical resection would therefore be the treatment of choice in localized tumors.
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Neoplasias Complejas y Mixtas/diagnóstico , Neoplasias Complejas y Mixtas/terapia , Tumores Neuroendocrinos/diagnóstico , Tumores Neuroendocrinos/terapia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/terapia , Adenocarcinoma/diagnóstico , Adenocarcinoma/cirugía , Adenocarcinoma/terapia , Adulto , Anciano , Anciano de 80 o más Años , Carcinoma de Células Acinares/diagnóstico , Carcinoma de Células Acinares/cirugía , Carcinoma de Células Acinares/terapia , Carcinoma Ductal Pancreático/diagnóstico , Carcinoma Ductal Pancreático/cirugía , Carcinoma Ductal Pancreático/terapia , Estudios de Casos y Controles , Femenino , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Metástasis de la Neoplasia , Neoplasias Complejas y Mixtas/cirugía , Tumores Neuroendocrinos/cirugía , Pancreatectomía , Neoplasias Pancreáticas/cirugía , Estudios Retrospectivos , Análisis de Supervivencia , Resultado del TratamientoRESUMEN
BACKGROUNDS: Acinar cell carcinoma of the pancreas is a rare malignancy, and its features remain unclear. We aimed to analyze the clinical characteristics, treatment and prognosis of acinar cell carcinoma with our institutional case series. METHODS: Patients diagnosed with acinar cell carcinoma in our hospital between 2005 and 2019 were reviewed. Investigations on clinicopathological features, treatment details and long-term survival were performed. RESULTS: A total of 45 pathologically confirmed acinar cell carcinomas were identified. The median age at diagnosis was 58 years with a male-to-female ratio of 3.1:1. There were 24 (53.3%) localized, 5 (11.1%) locally advanced and 16 (35.6%) metastatic cases, with a pancreatic head-to-body/tail ratio of 1:1.4 for all the primary lesions. In the localized group, there were 10 pancreatoduodenectomy, 12 distal pancreatectomy, 1 total pancreatectomy, and 1 distal pancreatectomy combined with proximal gastrectomy. Among the locally advanced and metastatic cases, 13 patients received chemotherapy, 1 received concurrent radiochemotherapy, 1 underwent synchronous resection of primary tumor and liver metastasis, 1 underwent palliative operation, 1 underwent exploratory laparotomy, and 4 required no treatment. The median overall survival of this series was 18.9 months with a 5-year survival rate of 19.6%. Moreover, the resected acinar cell carcinoma patients were associated with prolonged survival compared with the unresected cases (36.6 vs. 8.5 months, P < 0.001). CONCLUSIONS: Surgical resection could improve the long-term survival of acinar cell carcinoma patients, which might also improve the prognosis of selected metastatic cases. Large-scale studies are needed to further clarify the biological behavior and clinical features, and to seek the optimal treatments.
Asunto(s)
Carcinoma de Células Acinares/terapia , Neoplasias Hepáticas/terapia , Pancreatectomía/estadística & datos numéricos , Neoplasias Pancreáticas/terapia , Pancreaticoduodenectomía/estadística & datos numéricos , Anciano , Carcinoma de Células Acinares/mortalidad , Carcinoma de Células Acinares/secundario , Quimioterapia Adyuvante/estadística & datos numéricos , Femenino , Estudios de Seguimiento , Gastrectomía/estadística & datos numéricos , Hepatectomía/estadística & datos numéricos , Humanos , Estimación de Kaplan-Meier , Neoplasias Hepáticas/mortalidad , Neoplasias Hepáticas/secundario , Masculino , Persona de Mediana Edad , Cuidados Paliativos/estadística & datos numéricos , Páncreas/patología , Páncreas/cirugía , Neoplasias Pancreáticas/mortalidad , Neoplasias Pancreáticas/patología , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
BACKGROUND: To explore the clinicopathologic characteristics, treatment and prognostic factors of head and neck acinar cell carcinoma (HNACC) comprehensively. METHODS: A population-based study was conducted using data from the Surveillance, Epidemiology, and End Results database (1975-2016). Overall survival (OS) and HNACC-specific survival of patients with different clinicopathologic variables were compared using the Kaplan-Meier method and Cox multivariate regression. RESULTS: A total of 2624 primary HNACC cases (1052 males, 1572 females) were identified. There was a significant difference in gender distribution. Among the total cohort, 2416 cases originated from salivary glands, including 2325 parotid gland ACC cases. Regardless of confounding factors, the 10-year and 20-year disease-specific survival (DSS) was 93.6 and 90%, respectively. Surgery was favourably associated with better DSS and OS [HR = 0.13, P = 0.0092 and HR = 0.23, P = 0.0203]. Gender was the only demographic independent prognostic factor for both DSS and OS [Male vs female, HR = 3.3, P = 0.0028 for DSS; HR = 2.44, P = 0.0376 for OS]. Higher pathological grade was adversely associated with DSS and OS [Grade II, HR = 4.03, P = 0.0444; Grade III + IV, HR = 35.64, P = 0.0000 for DSS; Grade III + IV, HR = 4.49, P = 0.0000 for OS, Grade I as reference]. In addition, TNM/AJCC stage was commonly associated with prognosis. CONCLUSION: Surgery was the only favourable prognostic indicator for both DSS and OS. Gender, age, pathological differentiation and TNM/AJCC stage were independent prognostic factors for survival.
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Carcinoma de Células Acinares/mortalidad , Neoplasias de Cabeza y Cuello/mortalidad , Neoplasias de las Glándulas Salivales/mortalidad , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Carcinoma de Células Acinares/patología , Carcinoma de Células Acinares/terapia , Niño , Preescolar , Femenino , Estudios de Seguimiento , Neoplasias de Cabeza y Cuello/patología , Neoplasias de Cabeza y Cuello/terapia , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Clasificación del Tumor , Estadificación de Neoplasias , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Programa de VERF/estadística & datos numéricos , Neoplasias de las Glándulas Salivales/patología , Neoplasias de las Glándulas Salivales/terapia , Glándulas Salivales/patología , Glándulas Salivales/cirugía , Factores Sexuales , Tasa de Supervivencia , Resultado del Tratamiento , Estados Unidos/epidemiología , Adulto JovenRESUMEN
PURPOSE: Acinic cell carcinoma (ACCs) is uncommon malignant epithelial neoplasm of the salivary glands; the most common presentation is a well-defined painless solid mass. Diagnosis of ACCs is frequently complicated, due to its similarity with benign tumors. METHODS: A review of the literature available on ACCs was carried out. Studies were sourced from PubMed with searching of relevant headings and sub-headings and cross-referencing. RESULTS: There are no clear characteristics of ACCs found on CT, MRI and ultrasound imaging. The management of the ACC, a rare malignancy of the parotid gland, is often difficult and controversial. Radical surgery is the best treatment option. The role of radiotherapy remains controversial: the precise indications and oncologic effects of adjuvant radiotherapy in ACC of the parotid gland are not well known. There is insufficient literature regarding the chemotherapy for metastatic ACC. CONCLUSION: Knowledge about ACC, a rare malignancy of parotid gland, has changed over the past few decades. More clinical randomized works would be needed, both to assess the real effectiveness of radio and chemotherapy and to have an unanimous consensus about their indications.
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Carcinoma de Células Acinares , Carcinoma , Neoplasias de la Parótida , Carcinoma de Células Acinares/diagnóstico , Carcinoma de Células Acinares/terapia , Humanos , Glándula Parótida/diagnóstico por imagen , Neoplasias de la Parótida/diagnóstico , Neoplasias de la Parótida/terapia , Radioterapia Adyuvante , Glándulas SalivalesRESUMEN
BACKGROUND: Acinic cell carcinoma (AciCC) is rare in children; therefore, reaching a consensus on its management is challenging and radiotherapy is limited by concerns about long-term toxicity. The purpose of this study is to analyze the effectiveness and safety of surgery plus postoperative 125 I interstitial brachytherapy (IBT) for children and adolescents with AciCC of the parotid gland (PG) treated at a single institution. PROCEDURE: Sixteen patients ≤ 18 years old with AciCC of the PG treated with surgery plus 125 I IBT from 2007 to 2018 were included. Surgery was the primary treatment; ten patients underwent total gross excision and six subtotal gross excision. The matched peripheral dose was 60-120 Gy. Overall survival, disease-free survival (DFS), local control rate, distant metastasis, and radiation-associated toxicities were analyzed, and factors influencing outcomes were evaluated. RESULTS: During follow-up (1.8-12.6 years; mean, 6.3 years), lymph node metastasis was observed in one case, 2.6 years after 125 I IBT treatment. The five-year overall and DFS rates were 100% and 91.7%, respectively. On univariate analysis, tumor size ≥ 3 cm (100% vs 50%; P = 0.025) and extraglandular extension (100% vs 50%; P = 0.025) were significant prognostic indicators for DFS. No severe radiation-associated complications occurred. CONCLUSIONS: Children and adolescents with AciCC of the PG with high-risk features can be managed using surgery plus postoperative 125 I IBT with excellent local control. Radiation-related complications were minor. Patients with facial nerve involvement can have their facial nerves preserved. Residual tumors can be safely managed using adjuvant 125 I IBT.