Primary Pancreatic Lymphoma: Recommendations for Diagnosis and Management

Figures & data

Figure 1 PRISMA flow diagram.

Table 1 Patient Characteristics

Characteristics	n (%)
Presenting symptoms
Abdominal pain	175/232 (75.4)
Jaundice	100/239 (41.8)
B symptoms	77/241 (31.9)
GI obstruction	24/225 (10.7)
Acute pancreatitis	56/218 (25.9)
Laboratory values
LDH > upper limits	65/129 (50.4)
CA 19–9 > upper limits	32/119 (26.9)
Anemia	19/91 (20.9)
Thrombocytopenia	4/84 (4.8)
WBC > 10.000/mmc	17/93 (18.3)

Abbreviations: GI, gastrointestinal; LDH, lactate dehydrogenase; CA, carbohydrate antigen; WBC, white blood cells.

Table 2 Imaging Characteristics

Imaging	n (%)
Imaging method
US	214/256 (83.6)
CT	249 (97.3)
MRI	36 (14.1)
PET-CT	51 (19.9)
Pancreatic localization
Head	163/256 (63.6)
Body-tail	76 (29.6)
All gland	17 (6.6)
Tumor diameter, mean (range)	60.6 mm (range 16–200 mm)

Abbreviations: US, ultrasonography; CT, computed tomography; MRI, magnetic resonance imaging; PET-CT, positron emission tomography-CT.

Table 3 Diagnostic Methods, Histology and Clinical Stage

Diagnosis	n (%)
Diagnostic method
Percutaneous biopsy	68/224 (30.3)
Endoscopic biopsy	39 (17.4)
FNA	33 (14.8)
Surgical biopsy	26 (11.6)
Demolition surgery	49 (21.9)
Autopsy	9 (4.0)
Histology
DLBCL	143/266 (53.6)
FL	26 (9.8)
BL	20 (7.5)
High grade B cell lymphoma	14 (5.2)
T-NHL	18 (6.7)
HL	4 (1.5)
LPL	4 (1.5)
MZL	2 (0.7)
SLL	2 (0.7)
PTLD	2 (0.7)
Lymphoblastic lymphoma	1 (0.4)
Unknown	31 (11.6)
Clinical stage (Ann Arbor)
I–II	152/200 (76)
III–IV	48/200 (26)

Abbreviations: FNA, fine needle aspiration; DLBCL, diffuse large B cell lymphoma; FL, follicular lymphoma; BL, Burkitt lymphoma; NHL, non Hodgkin lymphoma; HL, Hodgkin lymphoma; LPL, lymphoplasmacytic lymphoma; MZL, marginal zone lymphoma; SLL, small lymphocytic lymphoma; PTLD, post-transplant lymphoproliferative disorder.

Figure 2 Temporal variation in biopsy techniques.

Table 4 Patient Survival According to Treatment and Histology

	Alive		Deceased
	n (%)	OS, Months (Range)	n (%)	TTD, Months (Range)
Treatment*
DS	4 (50)	11 (3–19)	4 (50)	2.5 (1–6)
DS-IC	13 (76.47)	37.8 (4–160)	4 (23.53)	13.5 (8–27)
DS-RT	2 (100)	34 (6–62)	/	/
DS-IC-RT	2 (100)	44.5 (24–65)	/	/
IC	26 (74.28)	34.7 (5–192)	9 (25.72)	13.4 (1–88)
IC-RT	15 (78.94)	45.4 (2–128)	4 (21.06)	36.5 (9–67)
Histology**
DLBCL	48 (68.57)	39.3 (2–132)	22 (31.43)	18.9 (1–88)
FL	11 (84.61)	31.4 (6–62)	2 (15.39)	67 (63–72)
T-NHL	3 (23.07)	10.3 (4–15)	10 (76.93)	4.3 (0–8)
BL	8 (66.66)	63.25 (8–192)	4 (33.34)	2.9 (1–7)
HGBCL	3 (50)	34 (3–94)	3 (50)	10.3 (8–12)

Notes: *Data available in 83 patients; **data available in 113 patients.

Abbreviations: OS, overall survival; TTD, time to death; DS, demolition surgery; IC, Immuno-chemotherapy; RT, radiotherapy; DLBCL, diffuse large B cell lymphoma; FL, follicular lymphoma; NHL, non Hodgkin lymphoma; BL, Burkitt lymphoma; HGBCL, high grade B cell lymphoma.

Table 5 PPL Characteristics in Pediatric vs Adult Patients

Characteristics	Patients <18 Year n (%)	Patients >18 Years n (%)
Presenting symptoms
Abdominal pain	16/21 (76.2)	159/211 (75.4)
Jaundice	13 (61.9)	87/218 (39.9)
B symptoms	2 (9.52)	75/220 (34.1)
GI obstruction	1 (4.76)	23/204 (11.3)
Acute pancreatitis	10 (47.6)	55/197 (27.9)
Laboratory values
LDH > upper limits	8/10 (80)	57/119 (47.9)
CA 19–9 > upper limits	0/3 (0)	32/116 (27.6)
Pancreatic localization
Head	13/21 (61.9)	150/235 (63.8)
Body-tail	2 (9.5)	74 (31.5)
All gland	6 (28.6)	11 (4.7)
Histology
DLBCL	5/21 (23.8)	138/245 (56.3)
BL	11 (52.4)	9 (3.7)
High grade B cell lymphoma	1 (4.8)	13 (5.3)
T-NHL	2 (9.5)	17 (6.9)
Unknown	2 (9.5)	29 (11.8)
Clinical stage (Ann Arbor)
I–II	5/13 (38.5)	147/187 (78.6)
III–IV	8 (61.5)	40/187 (21.4)

Abbreviations: GI, gastrointestinal; LDH, lactate dehydrogenase; CA 19–9, carbohydrate antigen; WBC, white blood cells; DLBCL, diffuse large B cell lymphoma; BL, Burkitt lymphoma; NHL, non Hodgkin lymphoma.