British Journal of Radiology (2005) 78, 651-654
© 2005 British Institute of Radiology
doi: 10.1259/bjr/78900851
CT and MRI appearances of inflammatory pseudotumour of the cervical lymph nodes
R S Gunny, BSc, MRCP, FRCR
1
N Akhbar, MBBS, MRCPath, MD
2 and
S E J Connor, MBBS, MRCP, FRCR
1
Departments of 1 Neuroimaging and 2 Pathology, Kings College Hospital, Denmark Hill, London SE5 9RS, UK
Correspondence: Dr Roxana Gunny, Radiology Department, Great Ormond Street Hospital, Great Ormond Street, London, UK
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Abstract
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Inflammatory pseudotumour (IPT), also known as plasma cell granuloma and inflammatory myofibroblastic tumour, is a rare cause of benign cervical lymphadenopathy which mimics malignant causes of cervical lymphadenopathy. The imaging features of IPT affecting the cervical lymph nodes have not previously been described. We present cross sectional imaging in a case of IPT occurring in a 42-year-old African-Caribbean man, from his initial presentation to a subsequent spontaneous reduction in the extent of lymphadenopathy.
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Introduction
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Inflammatory pseudotumour (IPT), also known as plasma cell granuloma and inflammatory myofibroblastic tumour, is a rare cause of benign cervical lymphadenopathy. In its acute clinical presentation it mimics malignant and infective causes of cervical lymphadenopathy. The imaging features of IPT affecting the cervical lymph nodes have not previously been described. We present serial cross sectional imaging in a case of IPT occurring in a 42-year-old African-Caribbean man, from his initial presentation to a subsequent spontaneous reduction in the extent of lymphadenopathy. The non-specific imaging characteristics in this disease confirm the importance of making an early histological diagnosis.
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Case report
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A 42-year-old man of Jamaican-African origin presented with a 1 week history of a rapidly enlarging painful left neck mass, preceded by a week of generalized left neck pain. He was otherwise well with no symptoms of fever, weight loss, sweats or symptoms referable to the ear, nose or throat. He was resident in the UK and had not travelled abroad in the previous 2 years. He had no risk factors for HIV infection or known tuberculosis contact history.
Examination confirmed the presence of a lymph node mass fixed to the upper third of the left sternocleidomastoid muscle. There was no evidence of enlargement of other nodal groups or hepatosplenomegaly.
Initial investigations including full blood count, urea and electrolytes, liver function tests, and chest radiograph were within the normal range. Retroviral HIV and HTLV1, 2, hepatitis B, C, treponemal, Epstein-Barr virus (EBV) serology and autoimmune antibodies were also negative and bone marrow trephine and biopsy were normal.
Contrast enhanced CT of the neck demonstrated a mass of enlarged level II, III and V cervical lymph nodes on the left (Figure 1
). These were of uniformly low attenuation and did not demonstrate significant contrast enhancement. They were of variable size with maximum diameter of 2 cm. There was increased soft tissue stranding in the adjacent fat with intermittent loss of fat planes between the lymph nodes themselves and between the overlying sternocleidomastoid muscle. A contrast enhanced CT examination of the chest and abdomen performed at the same time revealed no evidence of hepatosplenomegaly or enlargement of any other nodal groups.
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Figure 1. Axial CT post intravenous contrast (Somatom Plus 4, Siemens, Erlangen, Germany; slice thickness/slice spacing 5/10, 100 ml Omnipaque 300) at the level of the mandible demonstrates a confluent mass of enlarged jugular chain and posterior cervical level II and V lymph nodes.
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An excisional lymph node biopsy demonstrated reactive changes with reactive lymphoid follicles surrounded by a lymphocytic and plasma cell infiltrate and co-existent dense fibrosis. The histology was felt to be inconclusive and no treatment was commenced at this point. The patient was kept under clinical review and reduction in the palpable bulk nodal disease was documented at a subsequent clinical assessment. MRI of the neck performed 3 months later confirmed that the mass of enlarged confluent cervical level lymph nodes had reduced in size compared with the earlier CT. The nodes were of peripheral intermediate signal intensity with central hypointensity on T1 weighted imaging, and peripheral hypointensity with central hyperintensity on T2 weighted and STIR imaging (Figure 2). With gadolinium the lymph nodes demonstrated avid rim enhancement extending into the adjacent fat planes and overlying sternocleidomastoid muscle (Figure 3).
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Figure 2. Neck MRI (Magnetom Harmony 1T; Siemens, Erlangen Germany). (a) Axial T2 turbo spin echo repetition time (TR)/echo time (TE) 4465/120) and (b) coronal short tau inversion recovery (TR/TE 4465/30) images through the neck show central homogeneously increased signal and peripheral lower intensity of the individual lymph nodes, with associated perinodal and sternocleidomastoid muscle hyperintensity consistent with extension of inflammatory infiltrate. Compared with the CT performed 3 months previously there has been some reduction in overall nodal bulk and extent of adjacent inflammatory change.
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Figure 3. Coronal T1 spin echo (repetition time (TR)/echo time (TE) 418/12) post gadolinium demonstrates peripheral enhancement of individual lymph nodes containing central low signal necrotic or cystic cavities, and extension of abnormal enhancement into the perinodal soft tissue and overlying sternocleidomastoid muscle.
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The diagnosis was only confirmed with a second excisional biopsy showing lymphoid tissue with focal areas of necrosis, large numbers of lymphocytes, plasma cells and fibroblasts and dense collagen formation. On the basis of this a diagnosis of inflammatory pseudotumour of the lymph nodes was made. The patient remains well 1 year after his initial presentation and there has been further reduction in the nodal bulk disease documented clinically.
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Discussion
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Inflammatory pseudotumour of the soft tissues is a benign and self-limiting condition which although rare is important because it mimics neoplasia clinically. Few large pathological series exist but there is no apparent age, ethnic or geographical predilection. One series of 84 extrapulmonary cases of IPT found a slightly greater number of female patients, but smaller series based on nodal IPT alone found no gender differences [1, 2]. Several triggers for its development have been described, including smoking, minor trauma, post-adenoidectomy and infection, however its exact aetiology is not known [1, 3]. It is suggested that inflammatory mediators such as cytokines and interleukin-1 are released in response to an insult, causing proliferation of fibroblasts, leaky and procoagulant endothelium and extravasation of polymorphous cellular infiltrate into the extracellular space. Genetic studies have found aberrant forms of anaplastic lymphoma kinase (ALK) in inflammatory pseudotumour/inflammatory myofibroblastic tumour (IMT). However there is some debate as to whether IPT with normal ALK function represents a separate pathology to IMT [4]. One study found no abnormal ALK function in IPT of the lymph nodes [5].
The key histological findings in establishing the diagnosis of IPT are the co-existence of variable numbers of inflammatory cells and spindle cells, consisting of fibroblasts and myofibroblasts and with varying degrees of fibrosis. The wide spectrum of histological appearances has led to a number of terms for this condition being used synonymously, including plasma cell granuloma, inflammatory myofibroblastic tumour, inflammatory myofibrohistiocytic proliferation and inflammatory fibromyxoid tumour. This variation in the extent of inflammatory infiltrate and fibrosis suggests that this is a dynamic and evolving inflammatory process. Moran et al [6] described three stages; in the first stage there are multiple small foci of spindle cell proliferation and inflammatory response with preserved nodal architecture and no fibrosis. In the following two stages there is progressive destruction of nodal architecture with initially increasing inflammatory and fibroblastic infiltrate and finally complete replacement of the node by sclerosis.
IPT was first described in the lung but has been found in diverse locations throughout the body, including the orbits, head and neck extranodal sites, liver, spleen, heart and lymph nodes. It may affect any nodal group but usually only one or two discrete nodal groups are affected simultaneously. One third of patients present with asymptomatic nodal enlargement. The rest have pain or constitutional symptoms such as fever and weight loss [1, 2].
Although the imaging of IPT in the spleen and in head and neck locations including orbits, paranasal sinuses, tonsils and deep spaces of the neck has been reported, there have been no descriptions of the MR or CT appearances of inflammatory pseudotumour of the lymph nodes. In the spleen IPT was seen as a hypodense lesion on CT, intermediate to low on T1 weighted sequences with relative T2 hypointensity, and showing delayed contrast enhancement on both CT and MRI [7]. In the liver, lesions have more usually been described as isointense or hyperintense on T2 weighted imaging with respect to adjacent liver [8, 9]. The imaging appearances in this patient were of confluent enlargement of the lymph nodes, peripheral rim enhancement and multiple areas of central cavitation corresponding to necrosis seen on histology. Interestingly necrotic areas have not been alluded to in previous histological descriptions of IPT [10]. There was extranodal spread of enhancing soft tissue into the surrounding fat, also confirmed histologically, and diffuse involvement of the sternocleidomastoid muscle seen on imaging, which are recognised pathological features of this entity.
Our case has shown some differences from the imaging appearances of IPT in extranodal head and neck sites. Cavitation within lesions was not a feature seen on CT or MRI. Some lesions were characterized by homogeneous contrast enhancement while others demonstrated more peripheral rim enhancement. There was imaging evidence of central fibrosis/relative lack of free water as shown by central T2 hypointensity on MRI, and bone marrow involvement was a feature of skull base lesions [11, 12].
The radiological differential of confluent cavitating lymphadenopathy includes metastatic spread from a head and neck squamous primary neoplasm, tuberculosis and atypical mycobacterial infection, other granulomatous diseases and rarer causes such as Kikuchi's disease. Many of these diseases may also demonstrate extranodal extension. Although extranodal extension and cavitation are less likely in Hodgkin's or non-Hodgkin's lymphoma, anaplastic forms of lymphoma may demonstrate these imaging characteristics and this is therefore not a specific finding for any one pathological process.
This description of the imaging of cervical node involvement in IPT suggests that radiologically the appearances are non-specific and that it is another lymphoma mimic to include in the differential of rapidly progressive cervical lymphadenopathy. It is important to make an early histological diagnosis, not only to exclude malignant and other treatable causes, but also to instigate early treatment with steroids. Eventual spontaneous regression of IPT in the liver, as with this case, is recognised [9, 13, 14]. However a dramatic response to steroids has been demonstrated for acute IPT occurring in extranodal head and neck locations. This compares with a less favourable response in more chronic disease once fibrosis has occurred [15].
Received for publication February 6, 2004.
Revision received January 13, 2005.
Accepted for publication February 14, 2005.
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References
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Abstract
Introduction
