Granulocyte-colony stimulating factor producing anaplastic carcinoma of the pancreas treated by distal pancreatectomy and chemotherapy: report of a case

Abstract

Granulocyte-colony stimulating factor (G-CSF) producing pancreatic cancers are extremely rare. These tumors have an aggressive clinical course but no established treatment. We encountered a patient with a G-CSF-induced pancreatic cancer who was treated by surgical resection, followed by steroid treatment and chemotherapy. A 68-year-old Asian male presented at a local hospital with a 3-month history of fever, loss of appetite, and 10-kg weight loss. Laboratory data showed leukocytosis and elevation of C-reactive protein. Computed tomography (CT) revealed a 50-mm mass in the tail of the pancreas, but no signs of infective foci. He was transferred to our hospital for further evaluation. Contrast-enhanced CT showed rapid growth of this tumor over 1 week, and (18) F-2-fluoro-2-deoxyglucose positron-emission tomography/computed tomography (FDG PET/CT) showed FDG accumulation in the tail of the pancreas (SUV max, 17.1) but at no other sites in his body. Magnetic resonance imaging showed a heterogeneous mass, similar to that observed by CT. Three weeks later, the patient underwent a distal pancreatectomy with splenectomy. The resected specimen was 154 mm in diameter, a threefold increase from the initial image. Histopathological examination identified the tumor as an anaplastic carcinoma of the pancreas. Following surgery, his leukocyte count and body temperature were reduced. He recovered well and was discharged from our hospital on postoperative day 18. Immunohistochemical expression of G-CSF in the resected specimen and elevated serum G-CSF concentration confirmed that the mass was a G-CSF producing anaplastic carcinoma of the pancreas. Subsequently, the patient experienced a high fever and loss of appetite. CT showed recurrence of cancer in the abdominal cavity, for which he was started immediately on tegafur-gimeracil-oteracil potassium combination S-1 and steroid. Unfortunately, he died on postoperative day 83. To our knowledge, this patient was the first with a G-CSF producing anaplastic carcinoma of the pancreas to be treated by surgical resection, steroid and adjuvant chemotherapy.

Keywords: Granulocyte-colony stimulating factor; Leukocytosis; Pancreatectomy; Pancreatic cancer; Steroid; TS-1.

Fig. 1

Contrast-enhanced CT on the day of first visit to our hospital, showing a heterogeneously stained tumor, 72 mm in diameter, at the tail of the pancreas. a plain, b arterial phase, c portal phase

Fig. 2

FDG PET ¹⁸ F-2-fluoro-2-deoxyglucose positron-emission tomography/computed tomography (FDG PET/CT) showed FDG accumulation in the tail of the pancreas (SUV, 17.1)

Fig. 3

Abdominal magnetic resonance imaging (MRI) 14 days after contrast-enhanced CT. a T1 weighted MRI, b T2 weighted MRI image, showing a heterogeneous mass, 100 mm in diameter, at the tail of the pancreas

Fig. 4

Macroscopic findings. The resected specimen was a large tumor (154 mm in diameter) at the tail of the pancreas, invading the transverse mesocolon

Fig. 5

Microscopic findings. a The tumor was pathologically diagnosed as an anaplastic carcinoma of the pancreas, containing pleomorphic tumor cells (b), spindle tumor cells (c), and moderately differentiated ductal carcinoma (d) (H& E × 40). b-d Positive staining of granulocyte-colony stimulating factor (G-CSF) immunohistochemistry in the cytoplasm of formalin-fixed paraffin-embedded specimen (×400). b Pleomorphic tumor cell variant, composed of bizarre, multinucleated giant cells that contain abundant eosinophilic cytoplasm. The nuclei were large, hyperchromatic, and contained variable numbers and sizes of nuclei. Numerous mitoses were easily identified, including bizarre mitoses. These cells were suspended in a sea of neutrophils. c Spindle-cell component resembling a sarcoma, with cells arranged in fascicles, sometimes in a herringbone pattern. There was less pleomorphism than in pleomorphic tumor cells, whereas significant atypia was common. d Moderately differentiated ductal carcinoma

Fig. 6

Serial leukocyte counts, granulocyte counts, and body temperature in this patient. Elevated leukocyte and granulocyte counts were reduced immediately after surgery. Body temperature was also reduced rapidly soon after the operation. These parameters were all elevated after tumor recurrence

Fig. 7

Contrast-enhanced CT at recurrence on postoperative day 36, showing peritoneal dissemination and liver metastases