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Abstract The aim of this study was to describe the prevalence, clinicopathological, and prognostic features of acinic cell carcinoma (AciCC) of the oral and maxillofacial region. AciCC cases were retrospectively retrieved from 11 pathology centers of three different countries. Medical records were examined to extract demographic, clinical, pathologic, and follow-up information. A total of 75 cases were included. Females (65.33%) with a mean age of 45.51 years were mostly affected. The lesions usually presented as an asymptomatic (64.28%) nodule (95.66%) in the parotid gland (70.68%). The association of two histopathological patterns was the most common finding (48.93%) and the tumors presented mainly conventional histopathological grades (86.11%). Surgical treatment was performed in the majority of the cases (59.19%). Local recurrence was observed in 20% of the informed cases, regional metastasis in 30.43%, and distant metastasis in 12.50%. The statistical analysis showed that the cases with a solid histopathological pattern (p=0.01), high-grade transformation (p=0.008), recurrence (p=0.007), and regional metastasis (p=0.03) were associated with poor survival. In conclusion, high histopathological transformation, presence of nodal metastasis, and recurrence were prognostic factors for AciCC of the oral and maxillofacial region.
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Lipase hypersecretion syndrome (LHS) is a rare paraneoplastic syndrome, associated with acinar cell carcinoma of the pancreas (ACCP) in 10% to 15% of patients. Clinically, LHS manifests itself with the appearance of subcutaneous fibrocystic nodules, associated with trophic changes in the overlying skin, such as ulcers or fistulas that are difficult to manage, mainly affecting the lower extremities. Additionally, lipolysis near the joints and in the intraosseous adipose tissue can cause bilateral arthralgias, especially of the knees and ankles. We report a 57-year-old man, with a history of insulin resistance and allergic rhinitis, who presented in June 2019 with multiple subcutaneous nodules in the lower extremities, predominantly in both ankles, associated with arthralgia in that region. Additionally, a CT scan of the abdomen revealed a significant abdominal mass, measuring approximately 17 cm and in contact with the body and tail of the pancreas, pathologically compatible with an ACCP. Treatment with capecitabine was started with a favorable progression. The patient currently presents a small left lateral retro malleolar fistula, which, given the analyzes, studies and reviewed literature is concluded to be a lesion in the context of LHS.
Subject(s)
Humans , Male , Middle Aged , Pancreatic Neoplasms/complications , Pancreatic Neoplasms/pathology , Pancreatic Neoplasms/diagnostic imaging , Skin Neoplasms , Carcinoma, Acinar Cell/complications , Carcinoma, Acinar Cell/pathology , Pancreas/pathology , Acinar Cells/pathology , Abdomen/pathology , LipaseABSTRACT
Objective:To analyze the clinical and pathological features and gene mutations of pancreatic acinar cell carcinoma (PACC).Methods:Clinical data of 34 patients with PACC admitted to the Department of Pancreatic Surgery of the First Affiliated Hospital of Naval Medical University from December 2009 to July 2018 were retrospectively analyzed to summarize its clinical characteristics, and the expressions of α1-ACT, CaM5.2, Syn and CgA in pancreatic tumor tissues were detected by immunohistochemistry. Next-generation gene sequencing technology was used to detect gene mutations in tumor specimens.Results:Among the 34 PACC patients, 23(68%) were males and 11(32%) were females; the age ranged from 25 to 75 years, with an average age of 54 years. The first symptom was abdominal pain or distension in 21 cases (62%), skin or scleral yellow staining in 4 cases(12%), and 9 cases(26%) were found in routine physical examination. BMI was 17.6-34.0 kg/m 2, of which 3 cases (9%) were <18.5 kg/m 2, 23 cases (68%) were 18.5-24.0 kg/m 2, and 8 cases (23%) were >24.0 kg/m 2. Preoperative examination showed elevated CA19-9 in 7 cases (20.6%), elevated CEA in 3 cases (8.8%), and elevated AFP in 7 cases (20.6%). Blood amylase was 16-247 U/L, with an average of 80 U/L. Enhanced CT showed that the lesion was irregular in shape, showing inhomogeneity and slightly low density, with areas of cystic degeneration and necrosis. The tumor was located in the head of the pancreas in 14 cases (41%), the body and tail of the pancreas in 19 cases (56%), and the neck of the pancreas in 1 case (3%). The largest tumor diameter was 1.5-15.5 cm, with an average of 5.4 cm. Postoperative pathologic stage I was confirmed in 4 cases (12%), stage Ⅱ in 14 cases (41%), stage Ⅲ in 14 cases (41%) and stage Ⅳ in 2 cases (6%). Immunohistochemical results showed that both α1-ACT and CaM5.2 were positively expressed (100%). Syn was positive in 8 cases (23.5%) and CgA was positive in 6 cases (17.6%). Ki-67 index was from 9% to 70%, with an average of 41%. Gene sequencing of pancreatic tumor tissue from 6 patients showed BRCA2 mutation in 2 patients (7155C>G), K-ras mutation in 1 patient (35G>T), RET mutation in 1 patient (200G>A), and LKB1 mutation (234G>T) in 1 patient, and one double mutation of K-ras and RET (35G>A, 1 798C>T). 30 patients were followed up, and the median survival was 38.3 months. Conclusions:PACC was a rare pancreatic tumor with no specific clinical manifestations. The positive expression rates of α1-ACT and CAM5.2 in tumor tissues were 100%. BRCA2, K-ras, RET and LKB1 were common gene mutations.
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Objective@#To investigate clinical, pathological and immunohistochemical features of pancreatic acinar cell carcinoma.@*Methods@#A retrospective review of surgical and pathological databases between 2011 and 2016 at PLA General Hospital was collected and 14 cases of acinar cell carcinoma (ACC) of the pancreas were identified. EnVision immunohistochemistry was used to detect the expression of Trypsin, bcl-10 and cytokeratin(CK) proteins.@*Results@#The patients included nine cases of pure ACC, 3 cases of mixed acinar ductal carcinoma, 1 case of mixed acinar-neuroendocrine carcinoma and acinar-ductal-neuroendocrine carcinoma, respectively. Tumors involved different anatomic locations of the pancreas, including eight involving the head of pancreas, four in the body and tail, one in the uncinate process and one in a heterotopic pancreas. Two patients had lymph node and liver metastases before surgery. Microscopically, the tumor was hypercellular with less fibroblastic proliferation and tumor cells arranged in acinar or solid pattern. The well differentiated tumor cells showed eosinophilic, granular cytoplasm with single prominent nucleoli, while the poorly differentiated tumor cells tended to grow in solid sheets. Immunohistochemically, the tumor cells were positive for pan-cytokeratin (14/14), Trypsin (12/14) and bcl-10 (11/14). Stains for CK7 and CK19 were negative (11/14 and 3/4). According to the pTNM staging, there were 7 cases at stageⅠ, 3 at stage ⅡA, 3 at stage Ⅲ and 1 at stage Ⅳ. With average postoperative follow-up of 6-58 months, the median disease-free survival time was 16 months.@*Conclusions@#Pancreatic acinar cell carcinoma is a rare and relatively indolent malignant tumor with characteristic histopathological and immunohistochemical features. Accurate pathological diagnosis plays an important role in patients′ treatment and evaluation of prognosis.
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Objective To explore the clinical and sonographic characteristics of pancreatic acinar cell carcinoma (PACC).Methods The clinical data and abdominal sonographic findings of 13 cases of PACC identified with pathology were reviewed,including contrast-enhanced ultrasound (CEUS) images.There were 9 males and 4 females with the average of 53.9 years old.Symptoms included upper abdominal pain,nausea,chest distress and weight loss.Results The serum level of tumor maker was elevated in only 5 cases.Eight masses (61.5％) were located in the pancreatic body-tail,4 (30.8％) in the head,the whole pancreas was involved in 1 case.The mean maximal diameter was 8.0 cm.Four lesions (30.8％) presented as solid-cystic mass.Well-defined border was showed in 6 cases (60.0％).Exophytic type was observed in 5 cases (38.5％).Pancreatic ductal dilation was seen in 3 cases (23.1％),in which 2 cases showed obstruction of common biliary duct.Liver metastasis was assessed in 4 patients (30.8％),and lymph node metastasis in 3 cases (23.1％).Vessels were invaded in 4 cases (30.8％).In 2 cases by CEUS,one showed arterial iso-enhancement,the other with strong enhancement.Conclusion Certain characteristic clinical and ultrasonographic features could be revealed in PACC,as a sizable,exophytic,solid-cystic and relatively hypervascular mass with well-definded border.
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Purpose To analysis the CT manifestations of acinar cell carcinomas of the pancreas (ACCs) in order to know more about its CT signs. Materials and Methods The plain and enhanced CT findings of 9 patients with AACs proved pathologically were analyzed retrospectively. Results The main image ifndings of the patients were as follows:①the pancreas grew with exophytic dilatability;②they tended to be large (average diameter was 4.7 cm), with round or oval shape; ③ the lesions showed hypodense on enhanced scan and the solid areas showed slight enhancement in the arterial phase compared with normal pancreas;④most lesions had uniform or partial thin enhanced ring;⑤most lesions demonstrated cystic or necrotic;⑥few had pancreatic/biliary ductal dilatation and peripancreatic involvement;⑦few showed internal calciifcation or intratumoral hemorrhage. Conclusion Plain CT scan and enhanced scan are signiifcant in locating and differentiating acinar cell carcinoma of the pancreas.
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Acinar cell carcinoma is a rare malignant epithelial neoplasm with predominantly exocrine acinar differentiation and is seen primarily in older men (mean age, 62 years). The presenting symptoms are usually non-specific, and jaundice is often not present. Symptoms relating to the overproduction and release of lipase into the circulation are present in 10-15% of patients. Characteristic cytomorphologic features include a population of cells with minimal pleomorphism, eccentrically placed nuclei with a single prominent nucleoli and moderate hyperchromasia. The cytoplasm is finely granular, and the background may contain granular debris secondary to cytolysis. A significant proportion of the cases also have a minor neuroendocrine component or scattered neuroendocrine cells. Approximately 50% of patients have metastatic disease at presentation, often restricted to the regional lymph nodes and liver. The prognosis is poor, only slightly better than that of pancreatic ductal adenocarcinoma.
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Humans , Male , Acinar Cells , Adenocarcinoma , Carcinoma , Carcinoma, Acinar Cell , Cytoplasm , Jaundice , Lipase , Liver , Lymph Nodes , Neuroendocrine Cells , Pancreas , Pancreatic Ducts , PrognosisABSTRACT
BACKGROUND: A new tumor entity of the salivary glands, mammary analogue secretory carcinoma (MASC) with ETV6-NTRK3 translocation, has recently been proposed. MASC was originally diagnosed as adenocarcinoma, not otherwise specified (ANOS), or acinic cell carcinoma (AciCC) by the current World Health Organization classification. We aimed to identify MASC cases by molecular tests, and to characterize their clinical, histological, and immunohistochemical features. METHODS: Thirty cases of MASC candidates were selected after review of 196 salivary gland tumors, and subjected to break-apart ETV6 fluorescence in situ hybridization (FISH), and immunohistochemical study for S100 protein, gross cystic disease fluid protein 15, DOG1, estrogen receptor, and progesterone receptor. RESULTS: Valid FISH results were obtained in 23 cases, and 13 positive cases were retrieved. MASCs were histologically varied, and the most frequent features observed in 10 cases were low-grade papillary/cystic/glandular patterns, intraluminal secretory materials, ovoid/wrinkled nuclei, and relatively abundant granular eosinophilic cytoplasms, corresponding to papillary-cystic or follicular types of AciCC. All cases showed diffuse immunopositivity for S100 protein. Three cases developed recurrences, but all patients remained alive. CONCLUSIONS: MASC could be a molecularly well-defined salivary gland neoplasm, encompassing some portions of AciCC and ANOS, but its histological spectrum and clinical implication require further investigation.
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Humans , Adenocarcinoma , Carcinoma, Acinar Cell , Cytoplasm , Eosinophils , Estrogens , Fluorescence , In Situ Hybridization , In Situ Hybridization, Fluorescence , Oncogene Proteins, Fusion , Progesterone , Recurrence , Salivary Gland Neoplasms , Salivary Glands , World Health OrganizationABSTRACT
Objective To investigate the histological features,biological features,clinical treatment and prognosis of pancreatic acinar cell carcinoma.Methods A retrospective review of 10 patients with pancreatic acinar cell carcinoma treated in our hospital from 1999 to 2008 was conducted and the clinical features,imaging changes,pathologic feature,treatment course and follow-up data were collected.Results There were 9 men and 1 woman with a mean age of (62±8) years old.Tumors were located in the uncinate process in 1 patient,head of pancreas in 7,body and tail in 2.The median size of these tumors was 4.5 cm×4.7 cm;common bile duct and intrahepatic bile duct,pancreatic duct dilation was detected in 7 cases,and superior mesenteric vein was invaded in 2 cases.Of the 10 patients,8 received pancreaticoduodenectomy,among these 8 patients,3 had extended lymph node dissection,2 had portal vein resection and replacement;2received resection of pancreatic body and tail as well as splenectomy.Histologically,the size of these tumors were 4.0 cm×3.3 cm×3.4 cm.Macrescopically,duodenum was invaded in 5 patients,superior mesenteric vein was invaded in 2 patients and neural invasion was present in 7 cases.Lymph node metastasis was noted in 6 cases.Follow-up data was available in 9 patients and 1 patient was lost in follow-up.The survival ranged from 3 to 51 months with a median survival 18 months,and 9 patients died of tumor recurrence and metastasis after operation.Conclusions Pancreatic acinar cell carcinoma should be recognized as a distinct tumor entity and it may not be sensitive to radiotherapy or chemotherapy.The biological features of pancreatic acinar cell carcinoma should be investigated further.
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Acinic cell carcinoma (ACC) is a rare, low-grade malignancy of the salivary glands. Most cases occur in the major salivary glands, especially the parotid gland, with only a few cases involving the minor salivary gland previously described. A 67-year-old male patient was admitted complaining of an obstructive feeling in the throat. On examination, a lobulated mass in the tonsillar surface was noticed. Tonsillectomy was performed under general anesthesia. Histopathological examination of the mass revealed sheets of large, polygonal acinar cells with granular, slightly basophilic cytoplasm, which led to the diagnosis of ACC. Here, we present a case of low-grade ACC of the palatine tonsil, which we believe to be the first reported case of ACC in this location.
Subject(s)
Aged , Humans , Male , Acinar Cells , Anesthesia, General , Basophils , Carcinoma, Acinar Cell , Cytoplasm , Palatine Tonsil , Parotid Gland , Pharynx , Salivary Glands , Salivary Glands, Minor , Secretory Vesicles , TonsillectomyABSTRACT
OBJECTIVE To study the diagnosis and treatment of parotid acinic cell carcinoma. METHODS The clinical data of 19 cases with parotid acinic cell carcinoma treated from 1985 to 2007 were retrospectively reviewed.Four patients underwent superficial parotidectomy,5 patients underwent total parotidectomy,6 patients underwent superficial parotidectomy plus partial deep parotidectomy,6 patients underwent total parotidectomy and neck dissection.Eight patients received postoperative radiotherapy.One patient only received radical radiotherapy after biopsy.All patients were followed up for 1 to 20 years.RESULTS Nobody died after operation.Five cases recurred after operation, and the recurrence rate was 26.3%(5/19) CONCLUSION The main treatment for parotid acinic cell carcinoma is complete resection and postoperative radiotherapy.
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Background and purpose:It is rare that gastric and colorectal cancer metastasize to the uterine cervix.The study aimed to explore the treatment,prognosis,and the clinical features of metastatases of uterine cervix from gastric and colorectal cancer.Methods:22 patients with metastatic carcinoma to the uterine cervix from gastric and colorectal cancer were treated from 1994 to 2007 in Cancer Hospital of Zhejiang Province.The clinical pathologic parameters and prognosis were analyzed retrospectively.Results:The ages of patients ranged from 29 to 74 years old with mean of 46.7 years.The main symptom of patients was vaginal bleeding(54.5%).The primary tumor was gastric cancer in 10 cases and colorectal cancer in 12 cases.6 cases received operation alone,3 cases received radiotherapy alone,1 case received chemotherapy alone,9 cases were treated by the combination of chemotherapy,operation or radiotherapy,3 cases were not treated.According to clinical pathologic parameters,if the primary tumor was gastric cancer,it metastasesed to endometrium of uterine body;if the primary tumor was colorectal cancer,the serosa and myometrial of uterine body were invaded.In 22 cases,3 cases were still under treatment,with one lost follow-up,and the others died,the mean overall survival was 14.6 months.The mean overall survival were 11.2 months for gastric cancer and 17.9 months for colorectal cancer respectively.The mean overall survival of signet-ring cell carcinoma,mucinous adenocarcinoma and adenocarcinoma(G1-G3)were 10 months,13 months,and 18.1 months respectively.The mean overall survival for the patients treated by either radiotherapy,operation or the combination were 10.7 months,13.2 months and 17.7 months respectively.The mean overall survival for 4 patients treated by radical operation and 5 patients treated by tumor cell reduction-extinction technique were 22.3 months and 7.8 months respectively.Conclusion:The mean overall survival for the patients with metastases of uterine cervix from gastric cancer was worse than that from colorectal cancer.The patients with signet-ring cell carcinoma had the shortest mean overall survival,followed by mucinous adenocarcinoma.The primary lesion of tumor,histological types and treatment modality may be the main factors that affect the prognosis.The strategy of the treatment for the disease should be multidisciplinary therapy including surgery,radiotherapy and chemotherapy.
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Background and purpose:Metformin is known to be an insulin sensitization agent and is a fi rst line treatment for patients with type 2 diabetes. Recent clinical studies have revealed that metformin treatment has been associated with reduced cancer risk,which indicated that metformin may be a potential anti-neoplastic agent. We investigated the effects of antidiabetic drug metformin on proliferation and apoptosis in human lung adenocarcinoma cell line A549 in vitro and explored the possible underlying mechanisms. Methods:A549 cells were treated with 0.5 mmol/L,2 mmol/L and 8 mmol/L metformin for 48 hrs. Growth inhibition rates of the cells were measured by MTT assay. Cell apoptosis were detected by ? ow cytometery(FCM). Expressions of three genes including p53,Bcl-2 and Bax mRNA in the cells were measured by Real-Time PCR. Results:The proliferation of A549 cells was inhibited by metformin in a dose-dependent manner. The Inhibition rates of metformin at dosage of 0.5 mmol/L,2 mmol/L and 8 mmol/L group were (29?5)%,(68?3)% and (84.1?2.6)%,respectively. Apoptosis was induced when the cells were treated with moderate to high concentrations of metformin.The percentage of cells in early and late stage of apoptosis was increased from (1.1?0.3)% and (1.78?0.22)% in controlled group to (2.1?0.5)% and (9?4)% in metformin 8 mmol/ L group,respectively. The expressions of p53,Bcl-2 and Bax mRNA were all up-regulated after metformin treatment while the Bcl-2/Bax ratio was signifi cantly decreased. Conclusion:Metformin can inhibit the proliferation of human adenocarcinoma cancer cell line A549 and induce cell apoptosis with moderate to high drug concentrations in vitro,which may partly be attributed to the up-regulation of p53 and down-regulation of the Bcl-2/Bax ratio.