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Case Reports
. 2013 Sep 18:11:235.
doi: 10.1186/1477-7819-11-235.

Sarcoidosis lymphoma syndrome - the value of PET-CT in the diagnosis

Adrian Kis  1 , Noemi Eszes, Lilla Tamasi, Gyorgy Losonczy, Attila Csekeo, Judit Csomor, Veronika Muller
Affiliations
Case Reports

Sarcoidosis lymphoma syndrome - the value of PET-CT in the diagnosis

Adrian Kis et al. World J Surg Oncol. .

Abstract

We report a 52-year-old patient who developed B-cell non-Hodgkin's lymphoma subsequent to sarcoidosis. Sarcoidosis was diagnosed 16 years ago and remained asymptomatic for 14 years after steroid treatment. She presented with new symptoms of arthralgia, photosensitivity, butterfly erythema, autoimmune antibodies (ANA, chromatin positivity) associated with progression of the known left upper lobe lesion on the chest X-ray suggesting primary autoimmune disease (systemic lupus erythematosus). As steroid treatment was not effective, we started bolus cyclophosphamide therapy after which progression was seen on the chest X-ray. Computed tomography (CT)-guided needle biopsy confirmed malignancy of indefinable origin. Despite of the well-known fluorodeoxyglucose (FDG) avidity in active sarcoidosis, a FDG-positron emission tomography (PET) scan was performed to stage the primary tumour. Intensive FDG uptake was detected in the affected lung segment, with moderate uptake in mediastinal lymph nodes. The patient underwent left upper lobectomy. The histology showed pulmonary mucosa-associated lymphoma (bronchus-associated lymphoid tissue (BALT) lymphoma) in the lung tissue, while only sarcoidosis was present in the mediastinal lymph nodes. Bone marrow biopsy was negative.The association between sarcoidosis and lymphoma is known as sarcoidosis lymphoma syndrome, which is a rare disease. PET-CT was helpful in the differentiation of sarcoidosis and malignancy in this patient. It is important to be aware of the risk of lymphoma in sarcoidosis and FDG-PET, used for adequate purpose, can help the diagnosis.

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Figures

Figure 1
Figure 1
Chest X-ray of the patient. After 14 asymptomatic years, new symptoms occurred. The chest X-ray showed progression of the known left upper lobe lesion.
Figure 2
Figure 2
Repeat chest X-ray. Repeat chest X-ray showed rapid and significant worsening of the left upper lobe lesion.
Figure 3
Figure 3
Computed tomography (CT) guided needle biopsy of the left upper lobe lesion.
Figure 4
Figure 4
Fluorodeoxyglucose (FDG)-positron emission tomography (PET) scan.
Figure 5
Figure 5
Immunophenotyping of pulmonary mucosa-associated lymphoma. B cell lineage phenotype was demonstrated: CD20, BCL2 and lambda light chain positivity on the cell surface with CD10 and CD5 negativity.
Figure 6
Figure 6
Repeat chest computed tomography (CT) scan one year after surgery. One-year follow-up showed no progression of the diseases.
Figure 7
Figure 7
Repeat chest X-ray one year after surgery. One-year follow-up showed no progression of the diseases.
See this image and copyright information in PMC

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