Anaplastic carcinoma of the pancreas: Case report and literature review of reported cases in Japan

Abstract

We report a case of a 64-year-old woman with anaplastic carcinoma of the pancreas (ACP) with cyst formation and review 60 ACP cases reported in Japan. In 20% of cases, laboratory tests revealed severe anemia (hemoglobin level < 10.0 g/dL) and elevated leucocyte counts (> 12000/mm3), which were likely attributable to rapid tumor growth, intratumoral hemorrhage, and necrosis. Elevated serum CA19-9 levels were observed in 55% of cases. Cyst-like structures were observed on imaging in 47% of cases, and this finding appears to reflect subsequent cystic degeneration in the lesion. Macroscopically, hemorrhagic necrosis was observed in 77% of cases, and cyst formation was observed in 33% of cases. ACP should be considered when diagnosing pancreatic tumors with a cyst-like appearance, especially in the presence of severe anemia, elevated leucocyte counts, or elevated serum CA19-9 levels.

Cytopathology of anaplastic carcinoma of the pancreas: Review of a rare entity and description of a variant with signet ring cell features

“…ACP represents 0.1% of malignant exocrine pancreatic tumors according to one study, with autopsy series revealing higher incidences . It has a clinical presentation similar to PDAC, such as jaundice and abdominal pain; however, at time of diagnosis a bulky tumor is frequently detected, with involvement of organs in the vicinity . It tends to present more commonly in men versus women with a ratio of 2.5:1, and occurs more frequently in the head of the pancreas, with an average size of 4 cm …”

“…One case series of ACP reported that four out of five patients (80%) were diagnosed with ACP on EUS‐FNA . Another series reported that only three out of 60 patients (5%) were diagnosed preoperatively with ACP using EUS‐FNA . This highlights the importance of integrating the clinical and radiological findings with cytomorphology to arrive to the most accurate diagnosis possible, in the setting of an EUS‐FNA of a pancreatic mass .…”

“…Another series reported that only three out of 60 patients (5%) were diagnosed preoperatively with ACP using EUS‐FNA . This highlights the importance of integrating the clinical and radiological findings with cytomorphology to arrive to the most accurate diagnosis possible, in the setting of an EUS‐FNA of a pancreatic mass . EUS‐FNA is also useful in assessing chemosensitivity of pancreatic tumors, prior to chemotherapy .…”

“…Chemosensitivity is performed typically on surgical biopsies or resection specimen; however, since a big number of pancreatic neoplasms are often unresectable, EUS‐FNA has become an efficient way that provides tissue for testing . Some authors utilized it to diagnose and study the chemosensitivity of an unresectable ACP, and reported a good radiological response following treatment with Paclitaxel, to which ACP was found to be chemosensitive …”

“…[7][8][9] It has a clinical presentation similar to PDAC, such as jaundice and abdominal pain 10 ; however, at time of diagnosis a bulky tumor is frequently detected, with involvement of organs in the vicinity. 10 It tends to present more commonly in men versus women with a ratio of 2.5:1, 7 and occurs more frequently in the head of the pancreas, 7,10 with an average size of 4 cm. 7 Severe anemia (hemoglobin level < 10.0 g/dL) and elevated leucocyte count (>12 000/mm 3 ) have been observed in patients with ACP, and were attributed to rapid tumor growth, followed by hemorrhage and necrosis.…”