대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Lymphomatoid granulomatosis

Radiologic Findings

Fig 1. Chest PA shows multifocal consolidation with nodules and interstitial thickening in both lungs showing middle to lower lung zone predominance.
Fig 2-4. CT scans reveals multiple variable sized nodules and peribronchovascular consolidation with surrounding peripheral GGOs in both lungs showing middle to lower lung zone predominance.
Fig 5-7. Follow-up CT scans after 5 weeks showed increased extent of conglomerated nodules and consolidation with some lesions showing internal low attenuation.

Brief Review

Lymphomatoid granulomatosis (LG) is a rare, aggressive extranodal Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative disease with reactive T-cells. While histopathologic features in patients with grade I LG are compatible with benign lymphocytic angiitis and granulomatosis, grade II LG follows an aggressive course, and grade III LG lesions should be considered diffuse large B-cell malignant lymphomas. LG usually manifests in the 5th or 6th decade of life, is more common among immunocompromised patients, and affects male subjects twice as often as it affects female subjects. The lungs are the most commonly involved site in patients with LG, with less frequent involvement of the skin, kidney, liver, and central nervous system. Although LG is a lymphoproliferative disorder, it is found less commonly in lymph nodes, the spleen, and bone marrow. Symptoms range from nonspecific systemic manifestations—such as fever, weight loss, and fatigue—to organ-specific symptoms—such as dyspnea, cough, and chest pain—in patients with lung involvement. The prognosis is generally poor, with a 5-year mortality rate of 60%–90%. However, early diagnosis and aggressive intervention may be life-saving in patients with this rare but potentially treatable disease. A standard treatment has yet to be established but in most of the published series, first-line treatment consists of corticosteroid therapy, either alone or in combination with cyclophosphamide.

Chung et al reported the imaging findings of LG as nodules and masses with lymphatic distribution as would be expected for a lymphoproliferative disease. Central low attenuation, ground-glass halo and peripheral enhancement of the nodules/masses can be also presented, likely related to the angioinvasive nature of this disease. LG can also be seen as diffuse ill-defined ground-glass opacities and nodules in a peribronchovascular distribution, with middle and lower zone predominance. The nodules may be confluent, and they may fluctuate in size over time. Of note, synchronous regression and progression of pulmonary nodules at chest CT may be observed. This has been attributed to focal attenuation of the local T-cell response and should be taken into account in the evaluation of treatment response. Our patient was proved as a LG, grade 3 on surgical lung biopsy and treated with chemotherapy.

References

1. Chung JH, Wu CC, Gilman MD, et al. Lymphomatoid Granulomatosis: CT and FDG-PET Findings. KJR 2011;12(6):672-678.
2. Rezai P, Hart EM, Patel SK. Case 169: Lymphomatoid Granulomatosis. Radiology. 2011 May;259(2):604-9.
3. Sheehy N, Bird B, O’Briain DS, Daly P, Wilson G. Synchronous regression and progression of pulmonary nodules on chest CT in untreated lymphomatoid granulomatosis. Clin Radiol 2004;59(5):451–454.

Keywords

lung, lymphoproliferative disease, Epstein Barr virus, lymphomatoid granulomatosis,