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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.
Subject(s)
Humans , Male , Aged , Parotid Neoplasms/diagnosis , Carcinoma, Acinar Cell/diagnosis , Prognosis , Parotid Neoplasms/therapy , Tomography, X-Ray Computed/methods , Carcinoma, Acinar Cell/therapyABSTRACT
Objective:To explore the differential diagnosis of pancreatic acinar cell carcinoma (PACC) and pancreatic ductal adenocarcinoma (PDAC) based on multidetector computed tomography (MDCT) features.Methods:The clinical, pathological and MDCT imaging data of 26 patients with pathologically confirmed PACC and 145 patients with pathologically confirmed PDAC who underwent MDCT from November 2013 to April 2021 were retrospectively studied. The differences of MDCT features including tumor location, tumor size, common pancreatic duct and bile duct dilatation, pancreatitis, lymph node metastasis, cyst, pancreatic parenchyma atrophy, duodenal involvement, bile ductal and vascular involvement between the two groups were compared. Univariate analysis and multivariate analysis by logistic regression models were performed to identify the independent predictive factors for PACC.Results:The tumor size, bile duct dilatation, lymph node metastasis, pancreatic parenchyma atrophy and vascular involvement were significantly different between PACC group and PDAC group (all P value<0.05). Multivariate analysis revealed that the tumor size ( OR=1.07, 95% CI 1.028-1.15, P=0.001), lymph node metastasis ( OR=0.23, 95% CI 0.065-0.800, P=0.02), pancreatic parenchyma atrophy ( OR=0.15, 95% CI 0.048-0.490, P=0.002) were closely associated with PACC. Conclusions:The tumor size, bile duct dilatation, lymph node metastasis, pancreatic parenchyma atrophy and vascular involvement evaluated by MDCT had a certain value in differentiating PACC from PDAC, and the tumor size, lymph node metastasis and pancreatic parenchyma atrophy were independent predictors for the diagnosis of PACC.
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@#Introduction: Acinic cell carcinoma (ACC) represents 1-6% of parotid gland neoplasms. Case Report: We report cytomorphological features of two uncommon variants of acinic cell carcinoma. The first case was an eleven-year-old female with a nodular mass in parotid and the FNA smears demonstrated a lymphoepithelial lesion composed of epithelial tumour cells with features of acinar cells in a lymphoid background. The second case was a 62-year-old male with a large parotid mass. The FNA smears revealed presence of extracellular, acellular amyloid-like material with tumour cells arranged in follicles. Discussion: Awareness of cytomorphological features of these unusual variants of acinic cell carcinoma may help to avoid diagnostic pitfall.
Subject(s)
Parotid NeoplasmsABSTRACT
Objective To assess the computed tomography (CT) and magnetic resonance imaging (MRI) features of acinar cell carcinoma of pancreas (ACCP). Methods The clinical data of 5 patients with ACCP confirmed by operation or biopsy were retrospectively analyzed. Among them, 2 patients underwent CT plain scan and enhanced scan, 2 underwent MRI plain scan and enhanced scan, and 1 underwent both CT and MRI plain scan and enhanced scan. The clinical data and imaging features of 5 patients were analyzed. Results The tumor of 1 case occurred in the uncinate process of head of pancreas, and 4 cases in the tail of pancreas. The maximum diameter of the lesion was 44.6-142.3 mm, with an average of 86.14 mm. The density or signal of tumors was not uniform in the 5 patients. Hemorrhage, necrosis and calcification were found in 1 patient, while the other 4 patients only had necrosis. The tumor capsule was intact in 1 case, with tumor located at the head of pancreas, and the capsules were incomplete in the other 4 cases. The enhancement of tumors in the 5 cases was lower than that of normal pancreatic parenchyma and reached the peak value in portal vein phase, and the tumors of all cases had internal necrosis. The patient whose lesion located at the uncinate process of pancreatic head had dilatation of pancreaticobiliary duct. Four tumors located at the tail of pancreas invaded splenic vein, and 2 of them had hepatic metastasis. Conclusion ACCP is characteristic on CT and MRI images, which is helpful for diagnosis and differential diagnosis.
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Resumen El carcinoma de células acinares es una neoplasia poco frecuente que se presenta principalmente en las glándulas salivales. Presentamos el caso de un paciente femenino de 48 años con dolor, paresia palpebral derecha y aumento de volumen. Biopsia que confirma diagnóstico, manejada con exenteración orbitaria derecha más RT. Durante seguimiento seis años después se presenta dolor columna dorsal, RMN con lesión osteoblástica en T2 biopsia con metástasis de carcinoma de células acinares. Debido a su baja incidencia el comportamiento del carcinoma de células acinares de la glán dula lacrimal es incierto, no hay reportes en la literatura de lesiones metastásicas únicas en columna.
Abstract Acinar cell carcinoma is a rare neoplasm occurs primarily in the salivary glands. We report the case of a female patient of 48 years with pain, right palpebral paresis, and increased volume. Biopsy confirmed diagnosis, handled right exenteration more RT. During follow-up six years after dorsal spine pain, MRI with T2 lesion biopsy osteoblastic metastatic carcinoma of acinar cells. Because of its low incidence behavior acinar cell carcinoma of the lacrimal gland is uncertain, there are no reports in the literature of metastatic lesions unique column.
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Humans , Female , Middle Aged , Spine , Carcinoma, Acinar Cell , Acinar Cells , Neoplasm MetastasisABSTRACT
PURPOSE: Pancreatic acinar cell carcinoma (ACC) is a rare cancer of the exocrine pancreas. Because of its rare incidence, the efficacy of chemotherapy in this patient population has been largely unknown. Therefore, we retrospectively analyzed the outcomes of patients with advanced pancreatic ACC who received chemotherapy. MATERIALS AND METHODS: Between January 1997 and March 2015, 15 patients with unresectable or metastatic pancreatic ACC who received systemic chemotherapy were identified in Asan Medical Center, Korea. RESULTS: The median age was 58 years. Eleven and four patients had recurrent/metastatic and locally advanced unresectable disease. The median overall survival in all patients was 20.9 months (95% confidence interval [CI], 15.7 to 26.1). As first-line therapy, intravenous 5-fluorouracil were administered in four patients (27%), gemcitabine in five (33%), gemcitabine plus capecitabine in two (13%), oxaliplatin plus 5-fluorouracil/leucovorin (FOLFOX) in two (13%), and concurrent chemoradiotherapy followed by capecitabine maintenance therapy in two (13%). The objective response rate (ORR) to chemotherapy alone was 23% and the median progression-free survival (PFS) was 5.6 months (95% CI, 2.8 to 8.4). After progression, second-line chemotherapy was administered in eight patients, while four patients received FOLFOX and the other four patients received gemcitabine. The ORR was 38%, and patients administered FOLFOX had significantly better PFS than those administered gemcitabine (median, 6.5 months vs. 1.4 months; p=0.007). The ratio of time to tumor progression (TTP) during first-line chemotherapy to TTP at second-line chemotherapy was significantly higher in patients administered FOLFOX (4.07; range, 0.87 to 8.30) than in those administered gemcitabine (0.12; range, 0.08 to 0.25; p=0.029). CONCLUSION: Our results suggest that oxaliplatin-containing regimens may have improved activity against pancreatic ACC.
Subject(s)
Humans , Acinar Cells , Antineoplastic Agents , Capecitabine , Carcinoma, Acinar Cell , Chemoradiotherapy , Disease-Free Survival , Drug Therapy , Fluorouracil , Incidence , Korea , Pancreas, Exocrine , Pancreatic Neoplasms , Retrospective StudiesABSTRACT
PURPOSE: Pancreatic acinar cell carcinoma (ACC) is a rare cancer of the exocrine pancreas. Because of its rare incidence, the efficacy of chemotherapy in this patient population has been largely unknown. Therefore, we retrospectively analyzed the outcomes of patients with advanced pancreatic ACC who received chemotherapy. MATERIALS AND METHODS: Between January 1997 and March 2015, 15 patients with unresectable or metastatic pancreatic ACC who received systemic chemotherapy were identified in Asan Medical Center, Korea. RESULTS: The median age was 58 years. Eleven and four patients had recurrent/metastatic and locally advanced unresectable disease. The median overall survival in all patients was 20.9 months (95% confidence interval [CI], 15.7 to 26.1). As first-line therapy, intravenous 5-fluorouracil were administered in four patients (27%), gemcitabine in five (33%), gemcitabine plus capecitabine in two (13%), oxaliplatin plus 5-fluorouracil/leucovorin (FOLFOX) in two (13%), and concurrent chemoradiotherapy followed by capecitabine maintenance therapy in two (13%). The objective response rate (ORR) to chemotherapy alone was 23% and the median progression-free survival (PFS) was 5.6 months (95% CI, 2.8 to 8.4). After progression, second-line chemotherapy was administered in eight patients, while four patients received FOLFOX and the other four patients received gemcitabine. The ORR was 38%, and patients administered FOLFOX had significantly better PFS than those administered gemcitabine (median, 6.5 months vs. 1.4 months; p=0.007). The ratio of time to tumor progression (TTP) during first-line chemotherapy to TTP at second-line chemotherapy was significantly higher in patients administered FOLFOX (4.07; range, 0.87 to 8.30) than in those administered gemcitabine (0.12; range, 0.08 to 0.25; p=0.029). CONCLUSION: Our results suggest that oxaliplatin-containing regimens may have improved activity against pancreatic ACC.
Subject(s)
Humans , Acinar Cells , Antineoplastic Agents , Capecitabine , Carcinoma, Acinar Cell , Chemoradiotherapy , Disease-Free Survival , Drug Therapy , Fluorouracil , Incidence , Korea , Pancreas, Exocrine , Pancreatic Neoplasms , Retrospective StudiesABSTRACT
Background and purpose:Acinar cell carcinoma of the pancreas (ACCP) is a rare malignant tumor and a few radiologic reports have been published. This study aimed to evaluate the CT characteristics of ACCP. Methods:CT signs of 9 cases of pathologically conifrmed ACCP were analyzed retrospectively.Results:The mean value of longest diameter of the 9 cases of ACCP was 52 mm. Among the 9 cases, 6 cases (66.7%) had ill-deifned bor-der, 6 cases (66.7%) showed exophytic type, 8 cases (88.9%) showed enhancement degree less than normal pancreatic tissue, 6 cases (66.7%) represented heterogeneous enhancement, 7 cases (77.8%) showed invaded vessel, 5 cases (55.6%) had lymph node metastasis, and none had hepatic metastasis. Dilated pancreatic duct was observed in only 1 case. Conclusion:When pancreatic mass is large, heterogeneous, exophytic and without dilated pancreatic duct, ACCP is suggested.
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Mixed acinar-neuroendocrine carcinoma (MANEC) is a malignant pancreatic tumor that rarely occurs in children. It is diagnosed pathologically according to the proportion of neuroendocrine cells present, highlighting the need for surgical biopsy. A 13-year-old boy presented with a 10-cm palpable mass on CT. Surgical resection was performed, and the pathological diagnosis was MANEC. There were no postoperative complications, and the patient was discharged from the hospital 10 days after surgery. He is presently undergoing adjuvant chemotherapy. We reviewed historical MANEC cases published in the English literature. We concluded that pathological analysis of a surgically resected specimen is necessary for an accurate diagnosis of MANEC, and that publication of more cases is needed to determine the optimal management strategy for MANEC.
Subject(s)
Adolescent , Child , Humans , Male , Biopsy , Carcinoma, Acinar Cell , Carcinoma, Neuroendocrine , Chemotherapy, Adjuvant , Diagnosis , Immunohistochemistry , Neuroendocrine Cells , Postoperative Complications , Publications , Surgical Procedures, OperativeABSTRACT
No abstract available.
Subject(s)
Humans , Acinar Cells , Arthritis , Carcinoma, Acinar Cell , PanniculitisABSTRACT
Salivary gland acinar cell carcinoma is a rare low grade malignant tumor,which always occurs in the parotid,submandibular and sublingual salivary glands,but extremely rare occurs in the nasal septum.This paper reports a case of the salivary gland acinar cell carcinoma that located in the nasal septum.
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Objective To investigate the clinical characteristics and surgical outcomes of pancreatic acinar cell carcinoma (PACC).Methods The clinical and follow up data of PACC patients treated with surgery in Zhongshan Hospital of Fudan University between 1999 and 2012 were analyzed retrospectively.Results A total of 16 PACC were identified including 13 male and 3 female patients.The age of the patients ranged from 38 to 71 with an average of 57 years old.Six patients presented as abdominal pain,while low back pain in 3 patients,abdominal distention in 2 patients,emaciation in 2 patients,jaundice in 2 patients and melena in 1 patient.Elevated CA19-9 level was observed in 8 patients and 2 patients had elevated serum CEA.The tumors were located in the uncinate process in 1 patient,head in 9,body and tail in 6.The superior mesenteric vein was invaded in 2 cases and 1 patient had hepatic artery invasion.The tumor invaded both the celiac trunk and splenic artery in 1 patient.One patient had just undergone intra-operative needle aspiration biopsy due to unresectable tumor.All the other 15 patients underwent surgical excision with R0 resection.Among the 10 patients received pancreaticoduodenectomy,2 had superior mesenteric vein resection and replacement and 1 had hepatic artery resection.Five patients underwent distal pancreatectomy without spleen preservation.The mean size of these tumors was 5.7 cm × 4.6 cm,12 cases had a surrounding envelope,while lymphatic metastasis was observed in 8 cases.The follow up data of 15 patients were collected and the median postoperative survival was 21 months,and the survival rate of 1,3,5 year was 71.4％,28.6％,7.1％.Conclusions Pre-operative diagnosis of PACC is extremely difficult due to lack of specific clinical features and lab tests.Surgery is the first line treatment for PACC,and the prognosis of PACC is better than that of pancreatic ductal adenocarcinoma.
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Objective:To discuss the clinical feature, diagnosis, and treatment course of pancreatic acinar cell carcinoma (ACC) to guide clinical practice and improve prognosis of patients. Methods:Clinical data of 15 patients with pathologically confirmed pancreatic acinar cell carcinoma between December 1994 and March 2014 in Tianjin Medical University Cancer Institute and Hospital were retro-spectively studied. Results:The patients include eight males and seven females with a median age of 44. Tumors in these patients appeared in different parts of the pancreas. Eight patients had tumor in the head, six in the body and tail, and one in the uncinate process. The tumor size ranged from 3 cm to 18 cm, with an average diameter of 6.67 cm. The patients presented less jaundice and the tumor markers remained constant, specifically, no increase was reported. Six patients had metastasis before their operation. Twelve patients received radical resection, while the other three received palliative treatment. The preoperative and intraoperative diagnoses were not exact. The final diagnosis depended on pathologic confirmation after surgery or puncture. The immunohistochemical results of trypsin and chymotrypsin were positive in the patients who were examined. The postoperative chemotherapy was usually based on gemcitabine. The average survival time was 20.6 months. Conclusion:Pancreatic acinar cell carcinoma has special clinical features, and clinicians tend to regard it as low-grade malignancy. The attitude towards ACC should be positive.
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Objective To investigate the imaging features of pancreatic acinar cell carcinoma (PACC),and to assess the role of CT and MRI in the diagnosis of the disease.Methods CT and MRI data of 7 cases with PACC confirmed by surgery and pathology were reviewed retrospectively.Plan and dynamic contrast-enhanced CT were performed in 4 cases.MRI with T1 WI,T2 WI,and dy-namic contrast-enhanced series were performed in 3 cases.Results All of the PACC lesions were manifested as a single solitary mass.1 lesion was located in the pancreatic head,and the other 6 in the pancreatic body-tail.On plan CT,all of the 4 lesions ap-peared hypodense and 3 lesions had irregular more hypodense region in the lesions.On the contrast-enhanced CT,the tumor paren-chyma showed mild to moderate enhancement with non-enhanced hypodense region in the arterial phase,and lower enhancement than that of the surrounding normal pancreatic tissue in the portal and delayed phase.All of the 3 cases were heterogeneous hypointensity on T1 WI and hyperintensity on T2 WI.The manifestations of the tumors on contrast-enhanced MRI were similar with that on the contrast-enhanced CT.Dilation of the pancreatic duct was seen in 4 cases.Liver metastasis was seen in 1 case.Surrounding tissues were invaded in 4 cases.Conclusion CT and MRI can display the features of PACC and help to improve the diagnostic accuracy.
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We report a case of alpha-fetoprotein (AFP)-producing acinar cell carcinoma (ACC) of the pancreas. The tumor was diagnosed in a 72 yearold female after radical subtotal gastrectomy (Billroth I) due to early gastric cancer six months before. The initial serum AFP levels were increased to 2,254.1 IU/ml and preoperative imaging studies showed a mass with approximately 2.5 cm in diameter near the neck of the pancreas. A pancreaticoduodenectomy was performed. The pathologic examination revealed an ill-defined lobulating tumor confined to the pancreas (T1 stage). Immunohistochemical study showed that the tumor cells expressed AFP. The Adenosine triphosphate-based chemotherapy response assay (ATP-CRA) suggested that cisplatin would be more desirable than gemcitabine in AFP-producing ACC of the pancreas as an adjuvant chemotherapy. However, the adjuvant chemotherapy was not performed due to the early pathological stage. The patient died from carcinomatosis and pneumonia. Even if the tumor was on a relatively early stage, an adjuvant treatment should be considered ACC.
Subject(s)
Female , Humans , Acinar Cells , Adenosine , alpha-Fetoproteins , Carcinoma , Carcinoma, Acinar Cell , Chemotherapy, Adjuvant , Cisplatin , Drug Therapy , Gastrectomy , Neck , Pancreas , Pancreatectomy , Pancreaticoduodenectomy , Pneumonia , Stomach NeoplasmsABSTRACT
ObjectiveTo investigate the clinicopathological features,immunohistochemical phenotype of pancreatic acinar cell carcinoma.MethodsEight cases of pancreatic acinar cell carcinoma admitted to our hospital from January 2001to January 2011were retrospectively analyzed. The clinicopathological characteristics,and immunohistochemical staining for phenotype were analyzed,then the follow-up data were summarized.ResultsAll 8 patients with pancreatic acinar cell carcinoma was male,with a median age of 47 years old.Tumors were located in the pancreatic head in 4 patients,pancreatic body and tail in 4 patients.The average tumor size was 4.5 cm × 4.0 cm × 3.2 cm,the section appeared as gray or gray-red and presented as solid or cystic lesions.Larger tumors were often accompanied by hemorrhage and necrosis.Microscopically,the tumor cells arranged in acinic,cord,trabecular or solid nests.The cytoplasm was abundant and eosinophilic.The nuclear was round,oval,slightly atypia.lmmunohistochemical staining showed diffusely positive for CAM5.2,α-AT,α-ACT and focally positive for CA19-9,CEA,E-cad,β-cat and MUC-1 and only occasionally positive for AFP,NSE,Syn and CgA.Follow-up data showed there was one case of postoperative death due to postoperative pancreatic leakage with abdominal infection.Liver metastasis occurred in 4 cases,among whom,2 cases died.ConclusionsPancreatic acinar cell carcinoma is a rare epithelial malignant tumor of pancreas,with distinct phenotype characteristics.
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Pancreatic panniculitis is a rare type of disorder associated with pancreatic diseases. We describe here a case of 54-year-old man who was admitted to the Department of Dermatology with the diagnosis of erythema nodosum. The patient presented with a 9-month history of painful erythematous nodules on the extremities, joint pain and swelling, and weight loss. A highly elevated level of pancreatic lipase was found on the laboratory examinations. The biopsy specimens from the skin lesions showed subcutaneous fat necrosis. Abdominal computed tomography (CT) revealed a large mass with central necrosis in the body and tail of the pancreas. Distal pancreatectomy, splenectomy and partial transverse colectomy were successfully performed on day 17 of the hospitalization. The histopathologic findings supported the diagnosis of acinar cell carcinoma of the pancreas (ACCP). Postoperatively, the level of serum lipase returned to normal, and the skin lesions and joint manifestations gradually regressed. However, the swelling did not significantly resolve in the left knee. In view of the non-specific clinical presentation of this disease, clinicians should be alert and have a high index of suspicion for pancreatic panniculitis.
Subject(s)
Humans , Middle Aged , Acinar Cells , Arthralgia , Biopsy , Carcinoma, Acinar Cell , Colectomy , Dermatology , Erythema Nodosum , Extremities , Hospitalization , Joints , Knee , Lipase , Necrosis , Pancreas , Pancreatectomy , Pancreatic Diseases , Panniculitis , Skin , Splenectomy , Subcutaneous Fat , Weight LossABSTRACT
Primary acinic cell carcinoma (ACC) of the lung is very rare and this tumor is thought to arise from pluripotent cells of the submucosal glands of the tracheobronchial tree. We report here on a case of primary ACC of the lung in a 68-year-old man who had a solitary pulmonary nodule in the left lower lobe. The patient was symptomless and the lesion was found on a chest X-ray taken during a regular health checkup. The video assisted thoracoscopic surgery wedge resection revealed an ovoid yellow tan solid mass that was 1.8 cm at the largest diameter. Microscopically, the neoplastic cells grew in solid sheets of round cells with eccentric nuclei and abundant basophilic granular cytoplasm. There were no mitotic figures or areas of pleomorphic or anaplastic cells. Immunohistochemical staining for cytokeratin (AE1/AE3) was positive, but the staining for chromogranin A and CD56 was negative. Ultrastructural examination revealed polyhedral cells with many zymogen granules of varying electron density. The patient is well 4 months postoperatively.
Subject(s)
Aged , Humans , Acinar Cells , Basophils , Carcinoma, Acinar Cell , Chromogranin A , Cytoplasm , Electrons , Keratins , Lung , Lung Neoplasms , Secretory Vesicles , Solitary Pulmonary Nodule , Thoracic Surgery, Video-Assisted , Thorax , Triacetoneamine-N-OxylABSTRACT
BACKGROUND/AIMS: Acinar cell carcinoma (ACC) of the pancreas is a rare malignancy. ACC has been considered a cancer with poor prognosis due to frequent metastasis, a high recurrence rate, and low resectability. The aim of this study was to examine the clinical, radiologic and pathologic features of ACC in Korean patients, and surgical outcome was also investigated. METHODS: We reviewed the clinical records of two patients with ACC who had undergone operation in January 1996 and December 2005 at Hanyang University Medical Center. Through searching of medical journal from 1983 to 2009, 27 patients reported on literatures as Korean ACC patients were reviewed together. The clinical, pathohistologic, and radiologic features, treatment, and prognosis were investigated for all 29 patients. RESULTS: ACC was more common in male, and age at diagnosis ranged from 25 to 68 years (median 54). Symptoms were, mostly abdominal pain and mass. Liver was most common organ of metastasis at diagnosis and recurrence after operation. The mean tumor size was 7.0 cm, and most common location was tail. Of the 29 patients, 22 underwent surgical resection. Excluding 7 cases of not-reported survival, the median survival with operation was 22.4 months compared to 1.5 months with non-operation. CONCLUSIONS: In Korea, the clinical features of ACC include young age, large size, tail location, and nonspecific tumor markers. Surgery should be actively performed in the treatment of ACC regardless of size.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Age Factors , Carcinoma, Acinar Cell/diagnosis , Pancreatic Neoplasms/diagnosis , Prognosis , Republic of Korea , Survival Analysis , Biomarkers, Tumor/analysisABSTRACT
Pancreatic tumors in children are relatively rare, and their prognosis differs from that in adults. The purpose of this study is to examine the clinical characteristics, treatment, and prognosis for children with pancreatic tumors. We retrospectively reviewed the medical records of children under 15 years of age with pancreatic tumors who were treated surgically at Asan Medical Center between January 1992 and November 2009. There were 16 patients, fourteen of whom were pathologically diagnosed with solid pseudopapillary tumor. The other two patients were diagnosed with pancreatoblastoma and acinar cell carcinoma, respectively. Six patients of the 16 patients (38%) were male, and there was a male-to-female ratio of 1:1.6. The initial presentations were upper abdominal pain in eight patients (50%), palpable abdominal mass in three, and vomiting in one. Four patients were diagnosed incidentally. Six patients' tumors were located in the pancreatic head, six in the pancreatic body, and four in the pancreatic tail, respectively. The surgical procedures performed included distal pancreatectomy (n=7, 44%), median segmentectomy (n=3), enucleation (n=3), pancreaticoduodenectomy (n=2), and pylorus-preserving pancreaticoduodenectomy (n=1). Three patients underwent laparoscopic surgery. The median tumor size was 6.5cm (1.8~20 cm). Early surgical complications included pancreatic fistula (n=4), bile leakage (n=1), and delayed gastric emptying (n=1). A late complication in one patient was diabetes. The median follow-up period was five years and four months, and all patients survived without recurrence. While pancreatic tumors in adults have a poor prognosis, pancreatic tumors of childhood are usually curative with complete resection and thus have a favorable prognosis.