간암과 동반된 췌장 선방 세포 암종 1예

윤명희 고신대학교의과대학 2008 고신대학교 의과대학 학술지 Vol.23 No.1

Acinar cell carcinoma of the pancreas is a rare malignancy comprising about 1% of pancreas tumors with a generally poor prognosis. However, treatment proctocols for acinar cell carcinoma of the pancreas have not been standardized. A case of a 59-year-old man presented with epigastric pain and back pain for 2 months. Also he had 5 kg weight loss for 6 months. Computed tomography revealed a 3 cm sized pancreatic head mass, and a small lesion in the liver. There were not peripancreatic lymph node enlargements. The patients was diagnosed preoperatively as a pancreatic head cancer with metastatic liver lesion, and performed Whipple's operation and wedge resection of liver. Pathologic finding showed tumor cells were characterized by round or oval nuclei, only mild pleomorphism, single prominent nucleoli and abundant, eosinophilic and granular cytoplasm in pancreas head. In the liver hepatocellular carcinoma was showed with trabecular and pseudoacinar arrangement The patient was confirmed as a acinar cell carcinoma of pancreas head combined with hepatocellular carcinoma. This case illustrates the lack of a standardized approach of acinar cell carcinoma and emphasizes the need for further research.

췌장의 선방 세포 암종

전상교(Sang Kyo Jeon),박정근(Jung Kun Park),김동월(Dong Wol Kim),유선영(Seon Young Yoo),김정례(Jeong Rae Kim),김재홍(Jae Hong Kim),이진석(Jin Suk Lee),두창준(Chang Joon Doo),변종훈(Jong Hoon Byun),주재식(Jae Sik Joo) 대한소화기학회 1996 대한소화기학회지 Vol.28 No.4

Acinar cell carcinoma of the exocrine pancreas is a rare tumor with reported incidence of 1 to 2 % of pancreatic carcinoma. We reported a case of pancreatic acinar cell carcinoma in 58 year-old woman. Chief complaints were palpable mass and pain in left upper abdomen. Physical exatnination revealed huge aMominal mass in left upper abdomen. Abdominal ultrasonography and computed tomography revealed a huge well demarcated heterogeneous mass at the tail of the pancreas with liver metastasis. Distal pancreatectomy and excision of rnass were carried out. Pathological examination revealed acinar cell carcinoma in pancreatic tail. She died ninth day after operation. (Korean J Gastroenterol 1996;28: 592 - 596)

증례 : 혈액종양 ; 췌장 선방세포암 1예

김규영 ( Kyu Young Kim ),이화정 ( Hwa Jung Lee ),지준호 ( Jun Ho Ji ),이정림 ( Jung Lim Lee ),이원식 ( Won Sik Lee ),박종훈 ( Jong Hoon Park ),손경락 ( Kyung Rak Shon ) 대한내과학회 2009 대한내과학회지 Vol.76 No.4

최근 저자들은 매우 드문 종양인 췌장 선방세포암으로 근치적 원위부 췌장절제술과 비장절제술 시행 후 위의 단독 재발로 위전절제술과 다발성 간전이로 좌간절제술 후 보존적 capecitabine 항암화학요법을 실시하여 장기간 종양의 진행이 없는 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다. Acinar cell carcinoma of the pancreas is a rare tumor that constitutes 1~2% of all pancreatic cancers. The clinical and radiologic findings are inconclusive when diagnosing this disease. Acinar cell carcinoma progresses rapidly and metastasizes early, resulting in a poor prognosis. A 41-year-old man was admitted for abdominal pain. Abdominal computed tomography (CT) and positron emission tomography (PET)-CT showed a splenic mass involving the pancreatic tail with increased 18F-fluorodeoxyglucose (18F-FDG) uptake. A primary radical distal pancreatectomy and splenectomy were carried out. The pathology revealed acinar cell carcinoma of the pancreas. Three months later, a gastric recurrence was detected and a total gastrectomy was performed. Four months later, multiple hepatic metastases were found and a left hepatectomy was carried out. During treatment with capecitabine, no evidence of tumor progression was observed for 14 months. We report a case of metastatic pancreatic acinar cell carcinoma that did not progress for 14 months with capecitabine treatment. (Korean J Med 76:506-509, 2009)

Case Report : Pancreatic Panniculitis Associated with Acinar Cell Carcinoma of the Pancreas: A Case Report

( Zhen Jiang Zheng ),( Jun Gong ),( Guang Ming Xiang ),( Gang Mai ),( Xu Bao Liu ) 대한피부과학회 2011 Annals of Dermatology Vol.23 No.2

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. (Ann Dermatol 23(2) 225∼228, 2011)

췌장의 선방세포암 1예

최진호,권규흠,김장언,오지민,장유현,오소연,이윤경,황호경 대한노인병학회 2008 Annals of geriatric medicine and research Vol.12 No.3

Pancreatic acinar cell carcinoma is an uncommon malignancy of the exocrine pancreas. Because of a higher recurrence rate and frequent metastases, this tumor, generally, has a poor prognosis. We report a case of pancreatic acinar cell carcinoma in a 60-year-old man. His chief complaint was jaundice. Abdominal CT revealed a heterogeneous enhancing mass in the pancreatic head measuring 8.2×7.5 cm. After receiving the Whipple procedure, acinar cell carcinoma was diagnosed by pathological examination. Liver metastasis was found at one year after the curative resection.

Acinar Cell Carcinoma of the Pancreas

Jae Hoon Lee,Kyeong Geun Lee,Young Ha Oh,Seung Sam Paik,Hwon Kyum Park,Kwang Soo Lee 대한외과학회 2010 Annals of Surgical Treatment and Research(ASRT) Vol.79 No.4

Acinar cell carcinoma (ACC) of the pancreas is a rare malignancy making up approximately 1% of pancreatic non-endocrine malignant tumors. The common finding on computed tomography is a solitary, well-defined, heterogenous hypodense mass with enhancing capsule. ACC is a highly cellular tumor with minimal stroma and a lack of stromal desmoplasia. The accurate diagnosis of ACC cannot typically be done by histology alone but rather requires immunohistochemical staining or electron microscopy for the identification of pancreatic enzymes and zymogen granules. ACC has been considered a cancer with a poor prognosis due to frequent metastasis, a high recurrence rate, and low respectability. Surgical resection is the treatment of choice that can lead to long-term survival. ACC has a better prognosis than ductal carcinoma of the pancreas, but a worse prognosis compared to islet cell carcinoma. We report two cases of ACC with 5-year survival after surgical resection.

췌장 두부에 발생한 선방세포암 증례 및 문헌 고찰

강은혜 ( Eunhye Kang ),최유신 ( Yoo-shin Choi ),오형철 ( Hyoung-chul Oh ),도재혁 ( Jae Hyuk Do ),홍순억 ( Soon-uk Hong ),이승은 ( Seung Eun Lee ) 대한췌장담도학회 2022 대한췌담도학회지 Vol.27 No.1

췌장 선방세포암은 췌장암 중 1% 미만을 차지하는 매우 드문 암으로, 수술 전 영상 검사상 췌장관세포암, 췌장 고형성가유두상 종양, 신경내분비 종양 등과 감별이 어려운 경우가 많다. 현재까지 명확하게 정립된 치료법은 없으며, 조기 진단 및 종양의 완전 절제만이 생존율 향상을 가져온다고 보고되고 있다. 그러나 췌장 관세포암에 비하여 종양 절제 후 비교적 좋은 예후를 보여준다. 본 증례와 같이 비교적 젊은 남자 환자가 낭성 부분을 포함한 크기가 큰 고형성 췌장 종양을 주소로 내원하였을 경우 췌장 선방세포암을 염두에 두고 적극적인 수술 치료를 고려해야 할 것이다. Pancreatic acinar cell carcinoma (ACC) is a rare neoplasm accounting less than 1% of malignant pancreatic tumors. A 47-year-old male patient visited the emergency room with epigastric pain. Computed tomography or magnetic resonance imaging revealed a 4.7-cm heterogeneously enhanced solid and cystic mass with internal necrosis located in the head of the pancreas. Radiological diagnosis was borderline malignancy such as neuroendocrine tumor or solid pseudopapillary neoplasm. Two months later, the necrotic mass in the pancreas head had grown up to 11 cm, compressing the duodenum, superior mesenteric vein, and proximal transverse colon. Pylorus preserving pancreatoduodenectomy with segmental resection of transverse colon was performed. Histopathological examination revealed that the tumor was pancreatic ACC. The patient recovered without any complication and was doing well without recurrence for 12 months after surgery. Korean J Pancreas Biliary Tract 2022;27(1):54-59

Acinar Cell Carcinoma of the Pancreas: Clinical and Cytomorphologic Characteristics

Adam D. Toll,Ralph H. Hruban,Syed Z. Ali 대한병리학회 2013 Journal of Pathology and Translational Medicine Vol.47 No.2

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.

췌장 선방세포암 1예

이화정,지준호,박승찬,박정철,최은정,서혜진,이원식,이정림,배병조,손경락,이경희 영남대학교 의과대학 2008 Yeungnam University Journal of Medicine Vol.25 No.2

Acinar cell carcinoma is a rare tumor that represents 1~2% of al1 pancreatic cancers. Clinical and radiologic findings are inconclusive in this disease Acinar cell carcinoma is characterized by rapid progression and early metastasis, which lead to its poor prognosis. A 41-year-o1d man was admitted to our hospital for abdominal pain. Abdominal computed tomography (CT) and positron emission tomography-computed tomography (PET-CT) showed a splenic mass, which was being invaded by a pancreatic tail mass and which had increased ^(18)F-fluorodeoxyglucose (FDG) uptake Primary radical distal pancreatectomy and splenectomy were performed. Pathologic findings revealed an acinar cell carcinoma of the Pancreas The patient underwent a total gastrectomy three months later because of gastric recurrence Four months later, multiple hepatic metastases were discovered, and the patient underwent a left hepatectomy During treatment with capecitabine, there was no evidence of tumor progression for 14 months. We report a case of metastatic pancreatic acinar cell carcinoma, which did not progress for an extended period while the patient was being treated with capecitabme.

한국의 췌장 선방 세포암 -국내 문헌 발표된 27예와 본원의 2예의 임상병리학적 고찰-

이재훈 ( Jae Hoon Lee ),이경근 ( Kyeong Geun Lee ),박훤겸 ( Hwon Kyum Park ),이광수 ( Kwang Soo Lee ) 대한소화기학회 2010 대한소화기학회지 Vol.55 No.4

목적: 췌장의 선방 세포암은 외분비선에서 발생하는 종양으로 1-2%를 차지하는 매우 드문 악성종양이다. 국내의 선방 세포암에 대한 연구는 미흡한 실정이다. 이번 연구는 국내에서 보고된 선방 세포암에 대해 임상양상, 병리학적 특징, 치료와 예후를 알아보고자 시작되었다. 대상 및 방법: 2009년 10월까지 국내에 보고된 논문을 검색하여 병리학적으로 췌장 선방 세포암으로 확인된 총 27예의 환자와 본원에서 경험한 2예를 포함하여 총 29예에 대해 임상양상, 방사선 소견, 면역조직염색검사를 포함한 병리학적 특징, 수술과 재발 유무, 보조요법과 생존여부를 조사하였다. 이와 함께 외국의 연구와 비교하여 국내 선방 세포암의 특징을 고찰하였다. 결과: 선방 세포암은 50대의 남자에서 호발하며, 증상으로는 복부 동통과 종괴가 흔하다. 췌장 미부(41.4%)의 발생 빈도가 높았으며, 크기도 평균 7 cm으로 큰 양상을 보였다. 진단 당시 간 전이가 흔하고, 술 후 재발은 대부분 18개월 이내에 발생하였고 해당 장기로는 간이 가장 많았다. 29예 중 22예(75.9%)에서 근치적 절제술을 시행하였으며, 수술받은 환자의 생존기간은 22.4개월로 비수술적 치료의 1.5개월보다 큰 차이를 보였다. 5년 이상 생존한 예는 3예에 불과하였다. 결론: 국내의 선방 세포암의 특징은 평균 연령이 50대초반으로 더 젊었고, 크기는 평균 7 cm으로 더 크고, 췌장 미부의 발생빈도가 높았다. 예후는 비교적 나쁘지만 근치적 절제술을 시행한 경우 췌관 세포암보다 좋은 생존율을 보인다. 따라서 췌장 미부에 비교적 큰 종괴가 보일 경우 드물지만 선방 세포암을 감별해야 하고, 근치적 절제술을 위해 크기에 관계없이 적극적인 절제가 필요하다. 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. (Korean J Gastroenterol 2010;55:245-251)