Primary pancreatic anaplastic large cell lymphoma, ALK negative: A case report

Table 1 Clinical and pathologic data of primary pancreatic ALCL reported cases in chronological order

	Maruyama et al[3]	Cohen et al[1]	Chim et al[4]	Present study
A/G	46/F	22/M	27/F	80/M
Clinical symptoms	Back pain, anorexia	Upper abdominal pain, upper GI bleeding	Upper abdominal pain, weight loss	FUO, anorexia, weight loss, fatigue
Physical examination	Icteric conjunctivae, flat and soft abdomen with mild tenderness	Pallor, epigastric mass	Epigastric mass	Leg edema
Radiological findings	CT: mass in pancreatic head, ERP: without pathologic changes in MPD	CT: mass in pancreatic head	CT: mass in pancreatic head	CT: mass in pancreatic head and uncinate process, MRI: CT findings + IVC compression
Endoscopy	Submucosal protrusion with erosion of the second portion of the duodenum	Duodenal bleeding near to Vater’s papilla	Bleeding ulceration of the first portion of the duodenum	Ulcerated mass-like deformity of the duodenal bulb
Laboratory findings	ALT, AST, ALP, total and direct bilirubin elevated, serum amylase decreased, LDH: ND	Peripheral eosinophilia, LDH: in normal range	LDH: in normal range	LDH: elevated, β2 microglobulin: elevated
Surgical operation	Whipple’s	Whipple’s	Not performed	Not performed
Pathologic (IHC) findings	CD30+, CD45+, CD45RO+, EMA-, ALK: ND	CD30+, CD45RO+, CD3+, EMA+, ALK-	CD30+, CD45-, CD45RO+, ALK: ND	CD30+, CD45+, CD45RO+, EMA+, CD3-, ALK-
Treatment	MACOP-P	CHOP	CHOP	Glucocorticoids (symptomatic)
Follow up	NED at 18 months	NED at 30 months	6 months, died of disease progression	Died due to haemodynamic instability 2 days after explorative laparotomy

A/G: age/gender, ALK: anaplastic lymphoma kinase, ALP: alkaline phosphatase, ALT: alanine aminotransferase, AST: aspartate aminotransferase, CHOP: chemotherapy regimen consisting of cyclophosphamide, adriamycin, vincristine and prednisolone, EMA: epithelial membrane antigen, ERP: endoscopic retrograde pancreatography, F: female, FUO: fever of unknown origin, GI: gastrointestinal, IHC: immunohistochemistry, IVC: inferior vena cava, LDH: lactate dehydrogenase, M: male, MACOP-P: chemotherapy regimen consisting of methotrexate, adriamycin, cyclophosphamide, vincristine, prednisolone and peplomycin, MPD: main pancreatic duct, ND: no data available, NED: no evidence of disease.