British Journal of Radiology (2007) 80, e131-e133
© 2007 British Institute of Radiology
doi: 10.1259/bjr/52539691
Primary localized amyloidosis manifested as supraclavicular and mediastinal lymphadenopathy
H S Yong, MD
1
O H Woo, MD
1
J W Lee, MD
S-I Suh, MD
1
Y-W Oh, MD
1 and
E-Y Kang, MD
1
Departments of 1 Radiology and 2 Pathology, College of Medicine, Korea University, Korea University Guro Hospital, 97 Guro-dong, Guro-gu, Seoul, 152-703, Korea
Correspondence: Eun-Young Kang, Department of Radiology, Korea University Guro Hospital, 97 Guro-dong, Guro-gu, Seoul 152-703, Korea. E-mail: keyrad{at}korea.ac.kr
 |
Abstract
|
|---|
Thoracic involvement of amyloidosis is relatively rare, but mediastinal lymphadenopathy in the absence of pulmonary parenchymal involvement is extremely rare. The case presented here is of a previously healthy elderly woman who developed a palpable mass in the right supraclavicular area. The chest CT scan showed extensive, contiguous and homogeneous low attenuated lymphadenopathy with stippled calcification in the right supraclavicular area and mediastinum. Amyloidosis was confirmed histopathologically on a biopsy specimen from a right supraclavicular lymph node. Because there were no other sites found to be affected by amyloidosis and there was no underlying chronic disease, we made a final diagnosis of primary localized amyloidosis involving only the supraclavicular and mediastinal lymph nodes.
|
Introduction
|
|---|
Amyloidosis can involve any organ, but thoracic involvement in amyloidosis is relatively rare [1]. For cases of localized amyloidosis involving the thorax, patients are found to have three main types of disease distribution including a laryngotracheobronchial abnormality, discrete pulmonary nodule(s) (single or multiple), or rarely a diffuse interstitial pattern. However, the observation of mediastinal lymphadenopathy, in the absence of pulmonary involvement, is very rare [1, 2]. Only a few cases of mediastinal or hilar lymphadenopathy in the absence of pulmonary involvement have been reported in patients with the diagnosis of localized amyloidosis [3–6].
We report our recent experience with one patient who diagnosed with primary localized amyloidosis manifested only by supraclavicular and mediastinal lymphadenopathy.
|
Case report
|
|---|
A 58-year-old woman complained of a palpable mass located in the right supraclavicular fossa as well as mild dyspnoea. She was otherwise in good general health except for a history of thyroid nodular hyperplasia.
A routine chest radiograph showed a lobulated bulging contour in the right paratracheal area and cardiomegaly (Figure 1a
). A thoracic CT scan revealed extensive, contiguous, homogeneous and low attenuated lymphadenopathy in the right supraclavicular area and mediastinum (Figure 1b,c). In addition, subtle stippled calcifications were seen within the lymphadenopathy. There were no other abnormal lesions, such as pulmonary parenchymal or airway abnormalities. Initially, lymphoma or metastatic lymphadenopathy was suspected. Ultrasound-guided core biopsy was performed of the right supraclavicular mass.
View larger version (59K):
[in this window]
[in a new window]
|
Figure 1. (a) Routine chest radiograph shows a lobulated bulging contour in the right paratracheal area and cardiomegaly. (b) Thoracic CT scan at the level of the pulmonary trunk reveals a homogeneous low attenuated lymphadenopathy containing stippled calcification in subcarinal region. (c) Coronal reformatted image shows extensive, contiguous, homogeneous and low attenuated lymphadenopathy from the right supraclavicular area to mediastinum. (d) Supraclavicular lymph node biopsy specimen shows a homogeneous eosinophilic compound with aggregates of lymphoid cells after Congo red stain. (e) Under polarized light, homogeneous eosinophilic compound and aggregates of lymphoid cells exhibit apple-green birefringence.
|
|
The histopathological specimen revealed homogeneous eosinophilic materials and aggregates of lymphoid cells. These eosinophilic materials exhibited apple-green birefringence under polarized light after staining with Congo red and were positive for k and 
light chains (Figure 1d,e
). These pathological findings were consistent with amyloidosis. Abdominal and pelvic CT scans showed no specific abnormality. An echocardiogram showed slightly concentric left ventricular hypertrophy without systolic dysfunction. Bone marrow biopsy specimens obtained from the iliac crest showed normal cellularity and a mild increase of mature plasma cells. Serum and urine immunoelectrophoresis showed no definite abnormality. Therefore, the final diagnosis was made as primary localized amyloidosis involving only supraclavicular and mediastinal lymph nodes.
|
Discussion
|
|---|
Amyloidosis is a heterogeneous group of disorders characterized by extracellular deposition of abnormal protein and protein derivatives in a ß-pleated configuration that makes the proteins resistant to proteolysis. This deposition may occur because of a derangement in immunoregulation after a protracted antigenic challenge [2].
Amyloidosis can be classified according to its biochemical profile as either primary or secondary [7]. Primary amyloidosis occurs less frequently than the reactive or immune-related secondary form [3]. Secondary amyloidosis results from extracellular protein deposition caused by a number of chronic inflammatory disease processes (rheumatoid arthritis, tuberculosis, osteomyelitis, leprosy, Crohn's disease, cystic fibrosis, bronchiectasis). Primary amyloidosis consists of fragments of immunoglobulin light chains produced by plasma cells; thus it may be considered to be associated with a plasma cell dyscrasia. Primary amyloidosis may be associated with multiple myeloma or B-cell lymphoma [1, 2].
Amyloidosis also can be classified according to its anatomic placement as localized disease (10–20%) or systemic disease (80–90%) [8]. Systemic amyloidosis is frequently distinguished from localized amyloidosis by demonstrating protein in subcutaneous fat, rectal mucosa, bone marrow, urine, or serum. Localized amyloidosis has none of the above features. Instead, it is characterized by only discrete focal deposition of amyloid [2]. Localized amyloidosis is diagnosed primarily by exclusion. Shah et al [9] suggested the requisite criteria for diagnosis: positive immunohistochemical staining for k and light chains (AL) in a respiratory tract biopsy, or negative staining for serum AA protein, hereditary amyloid of transthyretin-type (TTR), and serum k and chains; no evidence of plasma cell dyscrasia on bone marrow aspirate, urine, or serum; characteristic distribution and pattern of localized respiratory tract disease on CT scan; negative radiolabelling of the serum amyloid P component (SAP scan); and negative echocardiogram. In this case, primary localized disease was diagnosed by the positive immunohistochemical staining for k or light chains (AL) in a lymph node biopsy specimen; localized supraclavicular and mediastinal involvement on CT scan; no abnormal findings on serum and urine immunoelectrophoresis; no evidence of plasma cell dyscrasia on bone marrow biopsy; and a slightly concentric left ventricular hypertrophy without systolic dysfunction on echocardiogram.
Amyloidosis may be associated with a variety of thoracic manifestations that depend in part on whether the condition is localized or systemic. Pickford et al [2] showed in his patients that adenopathy is the single most common abnormality in patients with systemic amyloidosis (9 of 12 patients, 75%). However, manifestations with a combination of both lymphadenopathy and pulmonary parenchymal involvement are more common. Three of nine patients who reported with adenopathy also had punctate calcification. In localized disease involving the thorax, adenopathy is a very unusual finding [2, 10]. Utz et al [10] reviewed the patients with pulmonary amyloidosis from 1980 to 1993. Only one of 38 patients with systemic disease showed bilateral hilar adenopathy. None of the 17 patients with localized pulmonary amyloidosis showed adenopathy.
The differential diagnosis of mediastinal lymphadenopathy includes: metastases, malignant lymphomas, sarcoidosis and other granulomatous disease such as tuberculosis. The observation of low attenuated mediastinal lymph nodes is common in patients with tuberculosis, fungal infections and neoplasms such as metastatic carcinoma and lymphoma. Extensive and conglomerated patterns of lymph nodes are often seen in patients with metastatic carcinoma and lymphoma. The findings of unusual stippled or faint lymph node calcification are rarely seen in untreated lymphoma, metastasis of mucinous adenocarcinoma, amyloidosis, or Castleman's disease [11]. Because only supraclavicular and mediastinal lymphadenopathy with stippled calcification was noted in our case, our initial differential diagnosis included lymphoma, metastasis, sarcoidosis and tuberculous lymphadenopathy. However, our findings show that amyloidosis could be considered in the differential diagnosis of lymphadenopathy that shows a pattern of homogeneous low attenuation, stippled or faint calcification. However, it is difficult to include amyloidosis in the differential diagnosis when mediastinal lymphadenopathy is the only abnormal lesion, because isolated mediastinal lymphadenopathy is a very rare manifestation of amyloidosis.
Lymphadenopathy with homogeneous low attenuation and stippled or faint calcification is a characteristic finding of amyloidosis. However, isolated mediastinal lymphadenopathy in the absence of pulmonary involvement is a very rare manifestation of thoracic amyloidosis. Furthermore, primary localized amyloidosis manifested as isolated mediastinal lymphadenopathy is an extremely rare finding.
Received for publication October 6, 2005.
Revision received February 9, 2006.
Accepted for publication February 20, 2006.
|
References
|
|---|
- Georgiades CS, Neyman EG, Barish MA, Fishman EK. Amyloidosis: review and CT manifestations. Radiographics 2004;24:405–16.[Abstract/Free Full Text]
- Pickford HA, Swensen SJ, Utz JP. Thoracic cross-sectional imaging of amyloidosis. AJR Am J Roentgenol 1997;168:351–5.[Abstract/Free Full Text]
- Conaghan P, Chung D, Vaughan R. Recurrent laryngeal nerve palsy associated with mediastinal amyloidosis. Thorax 2000;55:436–7.[Abstract/Free Full Text]
- Ordemann J, Braumann C, Rogalla P, Jacobi CA, Muller JM. Isolated amyloid tumor in the mediastinum: report of a case. Surg Today 2003;33:202–4.[CrossRef][Medline]
- Hiller N, Fisher D, Shmesh O, Gottschalk-Sabag S, Dollberg M. Primary amyloidosis presenting as an isolated mediastinal mass: diagnosis by fine needle biopsy. Thorax 1995;50:908–9.[Abstract/Free Full Text]
- Osnoss KL, Harrell DD. Isolated mediastinal mass in primary amyloidosis. Chest 1980;78:786–8.[CrossRef][Medline]
- Nomenclature of amyloid and amyloidosis. WHO-IUIS Nomenclature Sub-Committee. Bull World Health Organ 1993;71:105–12.[Medline]
- Urban BA, Fishman EK, Goldman SM, Scott WW Jr, Jones B, Humphrey RL, et al. CT evaluation of amyloidosis: spectrum of disease. Radiographics 1993;13:1295–308.[Abstract/Free Full Text]
- Shah PL, Gillmore JD, Copley SJ, Collins JV, Wells AU, du Bois RM, et al. The importance of complete screening for amyloid fibril type and systemic disease in patients with amyloidosis in the respiratory tract. Sarcoidosis Vasc Diffuse Lung Dis 2002;19:134–42.[Medline]
- Utz JP, Swensen SJ, Gertz MA. Pulmonary amyloidosis. The Mayo Clinic experience from 1980 to 1993. Ann Intern Med 1996;124:407–13.[Abstract/Free Full Text]
- Glazer HS, Molina PL, Siegel MJ, Sagel SS. High-attenuation mediastinal masses on unenhanced CT. AJR Am J Roentgenol 1991;156:45–50.[Abstract/Free Full Text]
Top
Abstract
Introduction
