Acinar Cell Carcinoma versus Solid Pseudopapillary Tumor of the Pancreas in Children: A Comparison of Two Rare and Overlapping Entities with Review of the Literature

BACKGROUND: Acinar cell neoplasms of the pancreas are rare but when encountered, the diagnosis is often established based on cytology specimens. Diagnostic accuracy is important because acinar cell carcinomas are aggressive yet may mimic tumors with different outcomes and management. METHODS: The authors identified all patients with a diagnosis of acinar cell neoplasm in the institutional database; assessed cytomorphology and immunocytochemistry for trypsin, chymotrypsin, synaptophysin, chromogranin A, and MIB-1; and compared all cytology and final histological diagnoses for diagnostic discrepancies. RESULTS: Cytological features were described for 16 histologically proven malignant acinar cell neoplasms: acinar cell carcinoma (8 cases), mixed acinar-neuroendocrine carcinoma (6 cases), mixed acinar-ductal carcinoma (1 case), and pancreatoblastoma (1 case).The majority of aspirates from acinar cell cystadenomas were nondiagnostic or negative (5 of 6 cases; 83%). Acinar and neuroendocrine differentiation that was detected by immunocytochemistry in >20% of tumor cells was found to be correlated with mixed acinar-neuroendocrine carcinoma histology. Cytohistological correlation included 32 patients with 17 discordant diagnoses (53%). The following preoperative cytology diagnoses proved to be acinar cell neoplasms on resection: neuroendocrine tumor (5 cases), adenocarcinoma (5 cases), atypical ductal cells (2 cases), solid pseudopapillary neoplasm, and hepatocellular carcinoma. Three aspirates diagnosed as acinar cell carcinoma by cytology proved to be chronic pancreatitis (2 cases) and ductal adenocarcinoma (1 case). CONCLU-SIONS: Acinar cell carcinoma has a distinctive cytological appearance but is frequently misdiagnosed on cytology. Immunocytochemistry is useful for identifying acinar differentiation. Cancer (Cancer Cytopathol) 2013;121:459-70. V C 2013 American Cancer Society.

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“…nuclear grooves, hyaline globules, branching capillaries. and myxoid stroma are not usually observed in ACC …”

Section: Discussionmentioning

confidence: 98%

Cytomorphologic and immunophenotypical features of acinar cell neoplasms of the pancreas

Sigel

Klimstra

2013

Cancer Cytopathology

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“…Acinar cell carcinoma is a particularly rare neoplasm, accounting for approximately 1% of all exocrine tumours of the pancreas in all ages [ 44 ]. Although rare in paediatrics, acinar cell carcinoma is more common than ductal cell adenocarcinoma, the most common pancreatic carcinoma in adults [ 25 ].…”

Section: Pancreatic Carcinomamentioning

confidence: 99%

International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines

Abib¹,

Chui²,

Cox³

et al. 2022

ecancer

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Most children with tumors will require one or more surgical interventions as part of the care and treatment, including making a diagnosis, obtaining adequate venous access, performing a surgical resection for solid tumors (with staging and reconstruction), performing procedures for cancer prevention and its late effects, and managing complications of treatment; all with the goal of improving survival and quality of life. It is important for surgeons to adhere to sound pediatric surgical oncology principles, as they are closely associated with improved local control and survival. Unfortunately, there is a significant disparity in survival rates in low and middle income countries, when compared to those from high income countries.The International Society of Paediatric Surgical Oncology (IPSO) is the leading organization that deals with pediatric surgical oncology worldwide. This organization allows experts in the field from around the globe to gather and address the surgical needs of children with cancer. IPSO has been invited to contribute surgical guidance as part of the World Health Organization Initiative for Childhood Cancer. One of our goals is to provide surgical guidance for different scenarios, including those experienced in High-(HICs) and Low-and Middle-Income Countries (LMICs). With this in mind, the following guidelines have been developed by authors from both HICs and LMICs. These have been further validated by experts with the aim of providing evidence-based information for surgeons who care for children with cancer.We hope that this initiative will benefit children worldwide in the best way possible.

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“…Table 2 summarizes the 26 reported pediatric cases, including the patients from the above reports and our case [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21]. Although the details are often unclear, the following characteristics of pediatric ACC can be suggested: (1) the tumors tend to be very large at diagnosis, (2) distant metastasis is rare and in most cases lesions are (9). e Immunohistochemical staining of AFP (9) resectable, (3) patients who undergo resection show long survival and a high long-term survival rate, and (4) many patients have a high AFP level.…”

Section: Discussionmentioning

confidence: 99%

“…Although the details are often unclear, the following characteristics of pediatric ACC can be suggested: (1) the tumors tend to be very large at diagnosis, (2) distant metastasis is rare and in most cases lesions are (9). e Immunohistochemical staining of AFP (9) resectable, (3) patients who undergo resection show long survival and a high long-term survival rate, and (4) many patients have a high AFP level. Similarly to adult ACC, long-term survival can be expected after radical surgical resection.…”

Section: Discussionmentioning

confidence: 99%

Acinar cell carcinoma of the pancreas in childhood

et al. 2016

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A 12-year-old Japanese girl with pancreatic acinar cell carcinoma is presented. She was referred to our hospital with upper abdominal pain on exercise. Computed tomography scan showed a 17 9 17 9 12 cm heterogeneous mass in the right abdominal cavity centering around the pancreatic head to the anterior pararenal space. We performed pylorus-preserving pancreatoduodenectomy, because the tumor invaded the pancreatic head. Macroscopically, the tumor was a 19 9 18 cm, encapsulated mass derived from the pancreatic head without invasion to the surrounding organs, and consisted of solid and cystic portions. Histological examination showed tumor cells proliferating in an acinar pattern and invading the duodenal muscle layer. Immunohistochemically, tumor cells were positive for a1 trypsin and a1 chymotrypsin. From these histological findings, we diagnosed the lesion as an acinar cell carcinoma of the pancreas. We report this case of childhood acinar cell carcinoma, which is extremely rare, with a review of the literature.

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Acinar Cell Carcinoma versus Solid Pseudopapillary Tumor of the Pancreas in Children: A Comparison of Two Rare and Overlapping Entities with Review of the Literature

Cited by 11 publications

References 35 publications

Cytomorphologic and immunophenotypical features of acinar cell neoplasms of the pancreas

Cytomorphologic and immunophenotypical features of acinar cell neoplasms of the pancreas

International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines

Acinar cell carcinoma of the pancreas in childhood

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