pancreatic lymphoma

Anaplastisches

großzelliges Non-Hodgkin-Lymphom mit Pankreasbefall: Oben MRT T2-SPIR, axial und koronal T1-SPAIR nach Kontrastmittel; unten MRT DWI, PET-CT Fusion und MIP.

Wikipedia • CC BY-SA 4.0

Spectrum of

imaging findings in AIDS-related diffuse large B cell lymphoma. A 46-year-old female patient who had pancreatic lymphoma (a) had a negative PET CT at the end of treatment (b and c). Nine months later, there was local disease recurrence at the pancreatic head on follow-up FDG PET CT scan (d and e)

insightsimaging.springeropen.com • CC-by-4.0

Lymphoma •

Multifocal visceral lymphoma - Ganzer Fall bei Radiopaedia

Dixon, A. Multifocal visceral lymphoma. Case study, Radiopaedia.org. (accessed on 22 Oct 2022) https://doi.org/10.53347/rID-16513 • CC by-nc-sa 3.0 (modified)

Pancreatic

lymphoma • Lymphoma (pancreatic and mediastinal) - Ganzer Fall bei Radiopaedia

Gaillard F, Lymphoma (pancreatic and mediastinal). Case study, Radiopaedia.org (Accessed on 16 Jan 2023) https://doi.org/10.53347/rID-37106 • CC by-nc-sa 3.0 (modified)

Non-Hodgkin

lymphoma • Non-Hodgkin lymphoma - multi-organ involvement - Ganzer Fall bei Radiopaedia

Niknejad, M. Non-Hodgkin lymphoma - multi-organ involvement. Case study, Radiopaedia.org. (accessed on 22 Oct 2022) https://doi.org/10.53347/rID-95364 • CC by-nc-sa 3.0 (modified)

Radiopaedia • CC-by-nc-sa 3.0 • de

Pancreatic lymphoma is most commonly a B-cell sub-type of non-Hodgkin lymphoma.

Epidemiology

Pancreatic lymphoma is typically seen in middle-aged patients with a mean age of around 55 years old and is more common in immunocompromised patients.

Clinical presentation

Symptoms are often non-specific but may include :

abdominal pain ~ 85%
mass ~ 60%
weight loss ~ 50%
obstructive jaundice ~ 40% of cases
acute pancreatitis 10%

The classic B-symptoms of non-Hodgkin lymphoma (e.g. fever, chills, night sweats) are present in only 2% of cases .

Pathology

Etiology

It is classified as either primary or secondary:

primary pancreatic lymphoma is a rare extranodal manifestation of any histopathologic subtype of B-cell non-Hodgkin's lymphoma, representing <2% of extranodal lymphomas and 0.5% of pancreatic tumors
secondary lymphoma: found in 30% of non-Hodgkin lymphoma patients with widespread disease, it is the dominant form and is the result of direct extension from peripancreatic lymphadenopathy

Location

Two morphologic patterns of pancreatic lymphoma are recognized :

focal form: occurs in the pancreatic head in 80% of cases and has a mean size of 8 cm
diffuse form: infiltrative, leading to glandular enlargement and poor definition, features that can simulate the appearance of acute pancreatitis

Radiographic features

CT

minimal enhancement
peripancreatic lymph node enlargement
it typically has uniform low attenuation
diffuse enlargement (diffuse form) may simulate acute pancreatitis
encasement of the peripancreatic vessels may occur; vascular invasion is less common in lymphoma than in adenocarcinoma

MRI

focal form: low signal intensity on T1-weighted images and intermediate signal intensity on T2-weighted images (slightly higher signal intensity than the pancreas but lower signal intensity than fluid), and shows faint contrast enhancement
diffuse form: low signal intensity on T1- and T2-weighted MR images and shows homogeneous contrast enhancement, although small foci of reduced or absent enhancement are sometimes seen

Treatment and prognosis

Percutaneous or endoscopic core biopsy should be performed to establish the diagnosis .

Pancreatic lymphoma carries a better prognosis than adenocarcinoma because first-line treatment with chemotherapy is generally effective in producing long-term disease regression or remission. Surgery is not required in most cases.

Using complex treatment approaches, cure rates of up to 30% are reported for patients with primary pancreatic lymphoma. This prognosis is much better than the dismal 5% 5-year survival rate in patients with pancreatic adenocarcinoma .