Surgery Alone Is Effective in the Management of Pediatric Salivary Gland Acinic Cell Carcinoma.

PURPOSE: The treatment strategy for salivary gland acinic cell carcinoma in pediatric patients remains controversial. This retrospective study was undertaken to analyze the role of surgery in the treatment of acinic cell carcinoma of the major salivary gland in pediatric patients. PATIENTS AND METHODS: In this retrospective cohort study, we reviewed the medical records of all pediatric patients with acinic cell carcinoma of the major salivary gland who were treated at Beijing Stomatological Hospital of Capital Medical University from 1998 to 2015. The predictor variable was treatment modality. The outcome variables were disease-free survival (DFS), overall survival (OS), local control, and freedom from distant metastasis. Other variables of interest were as follows: age, gender, tumor site, T category, N category, recurrence history, pathologic grade, perineural invasion, extracapsular extension, positive margin, and resection condition. The data analysis methods used were descriptive, bivariate statistics and the Cox proportional hazards regression model. RESULTS: Of the 19 patients, 7 received surgery alone and 12 received initial surgery combined with postoperative radiotherapy (RT). During the median follow-up period of 86 months, the overall estimates of DFS, OS, local control, and freedom from distant metastasis were 82.6, 93.3, 89.5, and 94.4%, respectively. Good outcomes were achieved in patients who received surgery alone (100% OS and 85.7% DFS). Initial surgery combined with postoperative RT was appropriate for patients with risk factors (91.7% OS and 83.3% DFS). CONCLUSIONS: Surgery alone is appropriate for salivary gland acinic cell carcinoma in pediatric patients without risk factors, even if extracapsular excision is adopted. Preserving the invaded facial nerve during surgery is a good choice because a curative effect can be obtained when adjuvant RT is administered. Older age, high-grade pathology, incomplete resection, recurrence history, and extracapsular extension were identified as risk factors of poor prognosis.

Prostate cancer grading: a decade after the 2005 modified system.

This review article will cover the evolution of grading of prostate cancer from the original Gleason system in the 1960-1970s to a more patient-centric grading system proposed in 2013 from a group at Johns Hopkins Hospital, validated in 2014 by a large multi-institutional study, and subsequently accepted by the World Health Organization (WHO), College of American Pathology (CAP), and the AJCC TNM system. Covered topics include: (1) historical background; (2) 2005 and 2014 International Society of Urological Pathology Grading Conferences; (3) Description of Gleason patterns; (4) new approaches to display Gleason grades; (5) grading variants and variations of acinar adenocarcinoma; (6) reporting rules for Gleason grading reporting secondary patterns of higher grade when present to a limited extent; (7) reporting secondary patterns of lower grade when present to a limited extent; (8) reporting percentage pattern 4; (9) general applications of the Gleason grading system; (10) needle biopsy with different cores showing different grades; (11) radical prostatectomy specimens with separate tumor nodules; and (12) a new grading system for prostate cancer.

Postoperative [¹²5I] seed brachytherapy in the treatment of acinic cell carcinoma of the parotid gland: with associated risk factors.

BACKGROUND: This retrospective study was undertaken to analyze data from patients receiving iodine-125 ([(125)I]) seed brachytherapy postoperatively for the treatment of acinic cell carcinoma (ACC) of the parotid gland along with the following risk factors: residual tumor, recurrent tumor, facial nerve invasion, positive resection margins, advanced tumor stage, or tumor spillage. PATIENTS AND METHODS: Twenty-nine patients with ACC (17 females, 12 males; age range, 13-73 years; median age, 37.3 years) were included. Median follow-up was 58.2 months (range, 14-122 months). Patients received [(125)I] seed brachytherapy (median actuarial D90, 177 Gy) 3-41 days (median, 14 days) following surgery. Radioactivity was 18.5-33.3 MBq per seed, and the prescription dose was 80-120 Gy. RESULTS: The 3-, 5-, and 10-year rates of local control were 93.1, 88.7, and 88.7 %, respectively; overall survival was 96.6, 92, and 92 %; disease-free survival was 93.1, 88.4, and 88.4 %; and freedom from distant metastasis was 96.6, 91.2, and 91.2 %. Lymph node metastases were absent in all patients, although two patients died with distant metastases. Facial nerve recovery was quick, and no severe radiotherapy-related complications were noted. Recurrence history, local recurrence, and distant metastasis significantly affected overall survival. CONCLUSION: Postoperative [(125)I] seed brachytherapy is effective in treating ACC and has minor complications. Patients with a history of recurrence showed poor prognosis and were more likely to experience disease recurrence and develop metastases.

Role of Adjuvant Radiotherapy in Acinic Cell Carcinoma of the Salivary Glands: A Systematic Review.

A systematic review was carried out to evaluate if adjuvant radiotherapy for acinic cell carcinomas (ACCs) of salivary glands improves survival. Twelve retrospective studies published between 2000 and 2020 that analysed the effect of radiotherapy on salivary gland neoplasms and ACCs of salivary glands and met the inclusion criteria were included in the review. The overall quality of the studies was moderate to low. There was no high-quality evidence for improved survival with radiotherapy for ACCs of the salivary gland. Some evidence suggests that there may be an advantage for patients with high-grade tumours, but these data should be interpreted with caution due to the small number of patients and low-quality evidence. Good quality of evidence is lacking. Recommendation for adjuvant radiotherapy for tumours with poor prognostic factors will require discussion and shared decision-making with the patients.

Survival outcomes, prognostic factors, and effect of adjuvant radiotherapy and prophylactic neck dissection in salivary acinic cell carcinoma: A prospective multicenter REFCOR study of 187 patients.

BACKGROUND: Acinic cell carcinomas (AciCCs) are malignant tumours of the salivary glands. The aim of this work was to analyse data from the national REFCOR multicenter cohort (i) to investigate the prognostic factors influencing survival outcomes in AciCC, (ii) to assess the impact on survival of postoperative radiotherapy (RT) in patients treated for AciCC without high-grade transformation and (iii) to explore the prognostic impact of prophylactic neck dissection (ND) in patients treated for AciCC of the major salivary glands. PATIENTS AND METHODS: Data from all the patients treated for salivary AciCC between 2009 and 2020 were extracted from the REFCOR database. Survival outcomes and prognostic factors influencing Disease-Free Survival (DFS) and Overall Survival (OS) were investigated using univariate and multivariate analyses. Propensity score matching was used to assess the impact of postoperative RT and prophylactic ND on DFS. RESULTS: A total of 187 patients were included. After a median follow-up of 53 months, their 5-year OS and DFS rates were 92.8% and 76.2%, respectively. In multivariate analysis, male sex, older age, higher T and N status, and high grade were independently associated with a worse DFS. In the subpopulation analysed after propensity score matching, patients with cN0 AciCC without high-grade transformation who were treated by surgery and RT did not have an improved DFS compared to patients who were treated by surgery alone (hazard ratio (HR) = 0.87, p = 0.8). Factors associated with nodal invasion were T3-T4 status and intermediate/high histological grade. After propensity score matching, prophylactic ND was associated with a trend toward a better DFS (HR = 0.46, p = 0.16). CONCLUSIONS: These results suggest that (i) long-term follow-up (>5 years) should be considered in patients with AciCC, (ii) treatment by surgery alone could be an option in selected cN0 patients with AciCC without high-grade transformation and (iii) prophylactic ND may be considered preferentially in patients with T3-T4 status and/or intermediate/high histological grade.

[Acinic cell carcinoma of the nasal septum]. / Carcinome à cellules acineuses du septum nasal.

INTRODUCTION: Nasal cavity acinic carcinoma are exceptional and often of turbinal origin. We report a case of acinic carcinoma of septal origin and discuss this histological type rare in this site. OBSERVATION: A 47-year-old women, with no pathologic history, consulted for right nasal obstruction and hyposmia having evolved for a year. The clinical examination revealed a right nasal cavity lesion adhesive to the septum. Tomodensitometry showed a right nasal cavity and ethmoid opacity without bone destruction. The surgical treatment was endonasal tumor resection. The histological examination revealed a nasal fossa acinic carcinoma completely resected. A postoperative radiotherapy was initiated. The evolution was uneventful without recurrence after 4 years of follow-up. DISCUSSION: Acinic carcinoma is rarely located in the nasal cavity. Its septal origin is exceptional. It is usually located at the salivary gland level. Curative treatment is surgery associated or not to radiotherapy. The prognosis is related to tumor extension and quality of resection.

Inconsistency Between Contrast-Enhanced CT and Bone Scintigraphy in Revealing Radiation Nephritis.

A 34-year-old woman with pancreatic acinar cell carcinoma underwent a Tc-MDP bone scan to rule out potential bone metastasis because of back pain 18 months after radiation therapy. The bone scintigraphy revealed increased uptake over the upper pole of both kidneys corresponding to the portions of the kidneys included within the radiation field, consisting with radiation nephritis.

Parotid acinar cell carcinoma in a long-term survivor of childhood acute lymphoblastic leukemia.

Secondary malignancies are an important cause of morbidity and mortality in childhood cancer survivors. Salivary gland tumors account for about 6% of the second cancers. The majority of these are mucoepidermoid carcinomas (MEC) of the parotid gland. We report the clinical and pathological features of a rarer histological type, acinic cell carcinoma (ACC), in a childhood acute lymphoblastic leukemia (ALL) survivor. The behavior of secondary ACC appears similar to primary tumor and similar treatment may be adopted. Early recognition and complete resection is important for achieving a good outcome. Careful monitoring for recurrence or a third malignancy is needed.

Close Margins and Adjuvant Radiotherapy in Acinic Cell Carcinoma of the Parotid Gland.

Importance: The precise indications and oncologic effects of adjuvant radiotherapy in acinic cell carcinoma of the parotid gland are not well known, particularly in patients with negative, but close (≤1 mm), margins without other high-risk histopathologic factors. Objective: To evaluate the oncologic outcomes of patients with acinic cell carcinoma of the parotid gland and the results of adjuvant therapy for those with close (≤1-mm) margins. Design, Setting, and Participants: In a retrospective case series with medical record review at a single academic tertiary referral center, patients treated surgically from January 2000 to December 2014 for acinic cell carcinoma of the parotid gland were identified from an institutional database. All data analysis was performed in September 2017. Exposures: All patients underwent parotidectomy with or without adjuvant radiotherapy or chemoradiotherapy. Main Outcomes and Measures: The primary end point was locoregional control. Secondary end points included recurrence patterns and survival. Results: Forty-five patients were identified in this case series (23 [51%] female), with a mean (SD) age of 47.1 (19.5) years. The median follow-up in surviving patients was 56.7 months (range, 18.5-204 months). Four patients (9%) experienced recurrence (1 local and 3 distant) at a median of 67.3 months (range, 12.7-136 months) after surgery. Thirteen patients (29%) had at least one high-risk histopathologic factor (advanced T category, nodal disease, lymphovascular or perineural invasion, high-grade, or positive margins). The remaining 32 patients (71%) without these high-risk factors had significantly improved disease-free survival (hazard ratio, 0.08; 95% CI, 0.01-0.71). Of patients without high-risk factors, those with close (≤1-mm) margins were significantly more likely to receive adjuvant radiotherapy (10 [56%] vs 1 [7%]; difference, 49%; 95% CI, 16%-82%), although this was not associated with disease control. At a median follow-up of 64.3 months (range, 33-204 months) in the 18 patients with close (≤1-mm) margins without other high-risk factors (10 with adjuvant radiotherapy and 8 without adjuvant therapy), only 1 patient (who had received adjuvant radiotherapy) experienced a recurrence, at 136 months after surgery. Conclusions and Relevance: Patients with acinic cell carcinoma of the parotid gland whose only histopathologic risk factor is a close (≤1 mm) but negative margin do not appear to benefit from adjuvant radiotherapy. Recurrent disease is rare but may occur many years after initial treatment, and patients with acinic cell carcinoma could benefit from lifelong clinical surveillance.

Outcomes and prognostic factors for parotid acinic cell Carcinoma: A National Cancer Database study of 2362 cases.

OBJECTIVES: To evaluate the demographics, clinical features, survival outcomes, and prognostic indicators of patients with acinic cell carcinoma (ACC) of the parotid gland with emphasis on the roles of grade, tumor size, and nodal status in survival. MATERIALS AND METHODS: A retrospective analysis of cases diagnosed between 2004 and 2012 from the National Cancer Database was performed. Multivariable logistic regression was used to determine factors associated with survival. RESULTS: 2362 cases were identified. Most patients were females (61.3%) and Caucasian (85.4%) with a median age of 54 years (range, 18-90 years). Most tumors were <3 cm in size (75.8%). Regional metastases and high-grade histology were rare (8.2%, 5.1%). All patients received surgery as primary treatment with 42.7% of patients receiving adjuvant radiation therapy or chemoradiotherapy. 5 year overall survival was 88.6%. On multivariable analysis, age >70 years (hazard ratio [HR]: 10.05, 95% confidence interval [CI]: 5.64-17.91), high-grade (HR: 5.30, 95% CI: 3.39-8.29), tumor size of 3 to 6 cm (HR: 1.53, 95% CI: 1.10-2.12), tumor size >6 cm (HR: 2.98, 95% CI: 1.681-5.289), pN2+ (HR: 3.14, 95% CI: 2.10-4.69), T4 (HR: 2.89, 95% CI: 1.74-4.80) were significant prognosticators. CONCLUSION: Although patients with ACC generally are considered to have a favorable prognosis, an aggressive subgroup with poor outcomes was identified. This group is characterized by high-grade, advanced T classification, tumors larger than 3 cm, with regional metastases and age greater than 70 years. Histologic grade is a substantially stronger predictor of survival than T and N classifications.

Acinic cell carcinoma of buccal mucosa: An unusual presentation.

Acinic cell carcinoma (ACC) is a rare epithelial malignant neoplasm of salivary glands affecting predominantly the female population. Unusual occurrences of this neoplasm are reported in hard palate, maxillary sinuses, lip, etc. [1] We report one such case where a submandibular swelling that is provisionally diagnosed as pleomorphic adenoma due to its clinical and radiological findings, turned out to be ACC on histopathological evaluation.

Acinic cell carcinoma of the lacrimal gland.

An 18-year-old woman underwent exenteration of the right orbit for tumor recurrence 3 years subsequent to external-beam irradiation for a lacrimal gland tumor diagnosed as an "adenocarcinoma." Light microscopy of the exenteration specimen revealed an acinic cell carcinoma of the lacrimal gland, with a predominant microcystic (latticelike) pattern of growth. Cytoplasmic vacuoles and the secretion within the microcysts stained positive with periodic acid-Schiff with and without alpha-amylase, alcian blue (at a pH of 2.5), mucicarmine, and colloidal iron with and without hyaluronidase. This histochemical staining for epithelial mucins supports the theory that the lacrimal gland, although serous in type, may also function as a modified mucus gland. There was cytoplasmic immunopositivity for keratin (CAM 5.2, KAE 1-3); immunostaining for vasoactive intestinal polypeptide was negative. Electron microscopy disclosed undifferentiated features of intercalated duct cells. We speculate that the lack of immunoreactivity for vasoactive intestinal polypeptide may be correlated with the predominantly undifferentiated intercalated duct cell features observed ultrastructurally.

Periodontal attachment loss in patients after head and neck radiation therapy.

OBJECTIVE: The purpose of this study was to determine the potential impact of head and neck radiation therapy on the progression of periodontal attachment loss. STUDY DESIGN: Ten patients who received unilateral radiation fields that included the dentition were assessed before radiation treatment and after irradiation at a mean age of 6.01 years. Complete oral, dental, and periodontal examinations were completed by one examiner. The results were assessed through use of paired t tests. RESULTS: More teeth were extracted because of periodontal disease in the field of radiation after irradiation. Remaining teeth in the radiated volume showed an increase in probing depth of 0.82 mm in comparison with 0.40 mm for teeth in the nonradiated region (P = .05). Recession on the facial aspects was 1.88 mm for teeth in the radiated volume and 1.16 mm for teeth in the nonradiated region (P = .001), and recession on the lingual aspects was 2.10 for teeth in the radiated volume and 0.91 for teeth in the nonradiated region (P = .05). Mean total attachment loss was 2.81 mm for teeth in the radiated sites; this compared with 1.43 mm for teeth in the nonradiated sites (P = .003). Increased mobility of teeth in the high-dose fields was seen (P = .02). CONCLUSIONS: This study showed that tooth loss and greater periodontal attachment loss occur in teeth that are included within high-dose radiated sites of patients treated with irradiation therapy for cancer. These findings should be considered in preradiation treatment planning.

Utilization of custom electron bolus in head and neck radiotherapy.

Conventional methods of treating superficial head and neck tumors, such as the wedge pair technique or the use of multiple electron fields of varying energies, can result in excellent tumor control. However, in some cases, these techniques irradiate healthy tissue unnecessarily and/or create hot and cold spots in junction regions, particularly in patients with complex surface contour modification or varying planning target volume (PTV) thickness. The objective of this work is to demonstrate how bolus electron conformal therapy can be used for these patients. Two patients treated using this technique are presented. The first patient was diagnosed with malignant fibrous histiocytoma involving the right ear concha and was treated with 12-MeV electrons. The second patient was diagnosed with acinic cell carcinoma of the left parotid gland and was treated with 20-MeV electrons after having undergone a complete parotidectomy. Each patient's bolus was designed using bolus design tools implemented in an in-house treatment-planning system (TPS). The bolus was fabricated using a computer-controlled milling machine. As part of the quality assurance process to ensure proper fabrication and placement of the bolus, the patients underwent a second computed tomography (CT) scan with the bolus in place. Using that data, the final dose distribution was computed using the Philips Pinnacle(3) TPS (Philips Medical Systems, Andover, MA). Results showed that the 90% isodose surface conformed well to the PTV and that the dose to critical structures such as cord, brain, and lung was well below tolerance limits. Both patients showed no evidence of disease six months post-radiotherapy. In conclusion, electron bolus conformal therapy is a viable option for treating head and neck tumors, particularly patients having a variable thickness PTV or surface anatomy with surgical defects.

A single-field integrated boost treatment planning technique for spot scanning proton therapy.

PURPOSE: Intensity modulated proton therapy (IMPT) plans are normally generated utilizing multiple field optimization (MFO) techniques. Similar to photon based IMRT, MFO allows for the utilization of a simultaneous integrated boost in which multiple target volumes are treated to discrete doses simultaneously, potentially improving plan quality and streamlining quality assurance and treatment delivery. However, MFO may render plans more sensitive to the physical uncertainties inherent to particle therapy. Here we present clinical examples of a single-field integrated boost (SFIB) technique for spot scanning proton therapy based on single field optimization (SFO) treatment-planning techniques. METHODS AND MATERIALS: We designed plans of each type for illustrative patients with central nervous system (brain and spine), prostate and head and neck malignancies. SFIB and IMPT plans were constructed to deliver multiple prescription dose levels to multiple targets using SFO or MFO, respectively. Dose and fractionation schemes were based on the current clinical practice using X-ray IMRT in our clinic. For inverse planning, dose constraints were employed to achieve the desired target coverage and normal tissue sparing. Conformality and inhomogeneity indices were calculated to quantify plan quality. We also compared the worst-case robustness of the SFIB, sequential boost SFUD, and IMPT plans. RESULTS: The SFIB technique produced more conformal dose distributions than plans generated by sequential boost using a SFUD technique (conformality index for prescription isodose levels; 0.585 ± 0.30 vs. 0.435 ± 0.24, SFIB vs. SFUD respectively, Wilcoxon matched-pair signed rank test, p < 0.01). There was no difference in the conformality index between SFIB and IMPT plans (0.638 ± 0.27 vs. 0.633 ± 0.26, SFIB vs. IMPT, respectively). Heterogeneity between techniques was not significantly different. With respect to clinical metrics, SFIB plans proved more robust than the corresponding IMPT plans. CONCLUSIONS: SFIB technique for scanning beam proton therapy (SSPT) is now routinely employed in our clinic. The SFIB technique is a natural application of SFO and offers several advantages over SFUD, including more conformal plans, seamless treatment delivery and more efficient planning and QA. SFIB may be more robust than IMPT and has been the treatment planning technique of choice for some patients.

Cáncer de páncreas en edad pediátrica / Pancreatic cancer in pediatric age

Introducción: Los tumores malignos pancreáticos en pediatría son extremadamente infrecuentes. La sobrevida en el cáncer pancreático a cinco años es baja. Objetivo: Informar a la comunidad médica acerca de una variante poco frecuente de tumor maligno pancreático en edad pediátrica. Presentación del caso: Paciente masculino de 17 años de edad, de la raza negra, que asiste a consulta en julio de 2017 por dolor en hemiabdomen superior, se considera una gastritis y se medica con dieta y antiácidos. Posteriormente comienza con dolor abdominal recurrente, pérdida de peso, anorexia, dispepsias, ictericia en piel y mucosas. Acude al gastroenterólogo quien indica una endoscopia digestiva alta y realiza el diagnóstico del tumor mediante biopsia endoscópica transduodenal. Se opera y reseca gran tumor de cabeza del páncreas junto con primera, segunda y tercera porción del duodeno (pancreatoduodenectomía). El tumor en conjunto midió aproximadamente 15 X 20 cm de diámetro y fue una cirugía completa sin lesión microscópica residual. El resultado de la biopsia indicó que se trataba de un adenocarcinoma acinar del páncreas pobremente diferenciado. Conclusión: Existen pocos casos publicados en la edad pediátrica con esta variante de tumor pancreático. Se documenta la importancia de la cirugía en la cura de la enfermedad(AU)

Introduction: Pancreatic malignancies in pediatrics are extremely infrequent, among them ductal adenocarcinoma and acinar adenocarcinoma. Survival in pancreatic cancer at five years is low. Objective: To inform the medical community about an uncommon variant of pancreatic malignant tumor in pediatric age. Case presentation: Male patient of 17 yesar of age, of the black race, who attended consultation in July of 2017 for pain in upper abdomen, is considered a gastritis and is medicated with diet and antacids. Subsequently begins with recurrent abdominal pain, weight loss, anorexia, dyspepsia, and skin and mucous. Go to the gastroenterologist who indicates an upper gastrointestinal endoscopy and perform the diagnosis of the tumor by transduodenal endoscopic biopsy. A large head tumor of the pancreas is operated on and resected together with the first, second and third portion of the duodenum (pancreatoduodenectomy). The tumor as a whole measured approximately 15 X 20 cm in diameter and was a complete surgery without residual microscopic lesion. The result of the biopsy indicated that it was an acinar adenocarcinoma of the poorly differentiated pancreas. Conclusion: There are few cases published in the pediatric age with this variant of pancreatic tumor. The importance of surgery in the cure of the disease is documented(AU)

Dedifferentiated acinic cell tumour: the harlequin of salivary gland neoplasms--an unusual variant.

We present a case of a salivary gland tumour in a 25-year-old woman with lymphadenopathy and a clinical suspicion of lymphoma. The patient had a history of rapidly enlarging mass near angle of jaw which was resected and sent for histopathological examination. A final diagnosis of acinic cell tumour with dedifferentiation was made by histomorphological and immunohistochemical studies. Acinic cell tumour can mimic any salivary neoplasm phenotypically because of its varied architectural patterns of presentation with varied cell types, hence called the harlequin of salivary gland. Acinic cell tumour with dedifferentiation is a rare aggressive variant and requires adjuvant radiotherapy for better prognosis, hence the need for accurate diagnosis and communication to the surgeon.