AJR 2003; 181:771-773
© American Roentgen Ray Society
Amyloidoma of the Clavicle
Franz Rachbauer1,
Alfons Kreczy2 and
Gerd Bodner3
1 Department for Orthopaedics, Provincial Hospital and University Clinic of
Innsbruck, Anichstra. 35, A-6020 Innsbruck, Austria.
2 Institute for Pathology, University of Innsbruck, A-6020 Innsbruck,
Austria.
3 Department for Radiodiagnostics I, Provincial Hospital and University Clinic
of Innsbruck, A-6020 Innsbruck, Austria.
Received October 14, 2002;
accepted after revision January 24, 2003.
Address correspondence to F. Rachbauer.
Introduction
The clavicle is an infrequent site for tumors. Approximately 1% of all
primary bone tumors arise in the clavicle
[1]. AL amyloidosis is a rare
disease and is estimated to occur 5.1-12.8 times per million person-years
[2]. Even though AL amyloidosis
has the widest spectrum of organ involvement
[2], localized tumorous
deposition of amyloid light chain protein in bone (amyloidoma) is rare
[3]. There are isolated reports
of amyloidomas of bone arising in the spine, the sternum, and the skull
[3-5].
We report a case of large amyloid deposition on the acromial end of the left
clavicle.
Case Report
A 74-year-old man was referred to our hospital because of a large tumor and
pain in the left shoulder that had lasted for 1 month. Three years previously
he had sustained a fracture of the distal left clavicle that healed
uneventfully; there were no radiographs available. Since that time, although
he felt well otherwise, he noted a slowly growing non-tender mass on the left
shoulder. At clinical examination the patient displayed a huge bulging tumor
of the left clavicle. On palpation the tumorous lesion was hard, only slightly
tender, with no signs of inflammation.
Radiologic examination showed a "bubbly" lesion expanding the
distal end of the clavicle and having margins that looked partly well defined
and sclerotic and partly ill defined (Fig.
1A). The polycystic, polylobulated lesion showed heavy
trabeculations, bone expansion, and scalloped margins. A triple-phase
whole-body bone scan showed focal increased uptake in the distal part of the
left clavicle and no other abnormalities.
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Fig. 1A. —74-year-old man with bulging tumor of left clavicle.
Radiograph of left clavicle shows destructive ballooning of distal end
(arrows). Polycystic, polylobulated lesion shows heavy trabeculations
(arrowheads), bone expansion, and partly well-defined and sclerotic
and partly ill-defined margins. Linear streaks of residual bone produce
trabeculated or bubbly appearance and may simulate intralesional
calcifications.
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Blood sedimentation rate was accelerated to 80 mm per first hour;
C-reactive protein was mildly elevated to 1.41 mg/dL. Hemoglobin was 13.3
g/dL, and blood leukocytes were 7400/µL; blood thrombocytes were 320/µL.
Relative concentrations of blood leukocytes were 69.5% neutrophiles, 16.6%
lymphocytes, 11.8% monocytes, 1.9% eosinophiles, and 0.2% basophiles. There
was no hypercalcemia, and serum electrophoresis did not disclose any
abnormalities.
Multiple percutaneous core biopsies were taken, revealing extensive
deposition of amorphous eosinophilic material surrounded at least focally by a
vigorous giant cell reaction and sparse focal lymphoplasmacytic infiltrates.
The tumor had an infiltrative margin, with eosinophilic deposits directly
applied against bony trabeculae. The eosinophilic deposits showed positivity
and apple-green birefringence when stained with Congo red
(Fig. 1D), proving amyloidosis.
There was no sensitivity to potassium permanganate treatment. Blood vessels
had amyloid deposits in their walls. Immunohistochemical staining proved
positive for lambda light chains, but negative for kappa and heavy chains.
There was reactive formation of new bone and cartilege. The predominantly
plasmacytic cells had eosinophilic cytoplasm with oval eccentric nuclei,
producing a cartwheel appearance, and showed only slight enlargement of
nucleoli.
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Fig. 1D. —74-year-old man with bulging tumor of left clavicle.
Photomicrograph of histopathologic specimen with positive findings shows red
fibrous stroma with lymphoplasmacytic infiltrates (asterisk) and
extensive deposition of amorphous material (arrowheads). Under
polarization microscopy, red-stained deposits show apple-green birefringence
characteristic of amyloid (inset, arrow). (Congo red, x60)
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Renal insufficiency was present, with serum creatinine levels of 3.03
mg/dL, serum urea of 60.8 mg/dL, and creatinine clearance of 26.7 mL/min.
Urinary protein was 168 mg/dL. There was a renal excretion level of 22 g of
protein per 24 hr. Immunoelectrophoresis of urine showed a peak of monoclonal
lambda light chain (Bence Jones protein lambda). Serum immunoelectrophoresis
revealed a peak of Bence Jones protein of 0.26 g/dL but was otherwise normal.
Quantities of IgG and IgM were normal, IgA level was slightly diminished, and
the kappa-lambda ratio was 1.23.
Further staging examinations consisted of CT scans of the thorax and
abdomen and radiographs of all four extremities, pelvis, spine, and skull.
There were no other lesions, and CT revealed the extent of tumorous
manifestation (Figs. 1B and
1C). Multiple bone marrow
samples were collected from the iliac crest. Cytology of bone marrow samples
revealed a normocelluar bone marrow. Immunocytology showed no elevation of
plasma cell ratio (< 5%), but plasma cells showed pathologic CD56 (natural
killer antigen) coexpression. Biopsy of intestinal mucosa revealed scarce
patchy amyloid deposition in vascular walls. There was no evidence of heart
involvement, no visceral enlargement, no kidney enlargement, and no
hepatomegaly. The patient did not have peripheral neuropathy.
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Fig. 1B. —74-year-old man with bulging tumor of left clavicle. CT scans
obtained through tumor show complete destruction of expanded distal end of
clavicle (arrow, B) with vanishing margins
(arrowheads, C) and remaining bone trabeculae.
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Fig. 1C. —74-year-old man with bulging tumor of left clavicle. CT scans
obtained through tumor show complete destruction of expanded distal end of
clavicle (arrow, B) with vanishing margins
(arrowheads, C) and remaining bone trabeculae.
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The patient underwent chemotherapy with prednisone and melphalan. At 1-year
follow-up, an interstitial bone marrow infiltration by a moderately
differentiated multiple myeloma was found. Despite further chemotherapy, the
patient's condition deteriorated and he died from disease 22 months after
first presentation at our department.
Discussion
Huge amyloid tumors of the bone are limited almost exclusively to patients
with amyloidosis caused by plasma cell dyscrasias. Most, if not all, AL
amyloidomas of bone are believed to represent solitary plasmacytomas of bone
or plasmacytoid lymphomas [3].
The rare exception is the deposits of amyloidlike material in bone of patients
receiving long-term hemo-dialysis for chronic renal failure
[6]. Clinical appearance must
be distinguished from the so-called shoulder pad sign
[2,
7], which is said to be
pathognomonic for amyloidosis but typically involves the soft tissues
surrounding the shoulder [7].
Amyloidomas of bone have most often been reported to occur in the spine and
flat bones such as the sternum, skull, pelvic bone, and ribs
[3-5].
The deposits of amyloid may calcify and may suggest the diagnosis of
chondrosarcoma on radiographs
[6]. Because imaging is
unspecific, diagnosis should be clarified by biopsy and histologic workup.
The diagnosis of primary amyloidoma requires histologic confirmation. In
amyloidomas, the mixed population of plasma cells, histiocytes, and
lymphocytes, widely separated by large masses of amyloid, frequently suggests
a reactive process [3]. Even
with optimal material it is usually impossible to assess the neoplastic nature
of the plasma cells without the use of immunohistochemistry or
gene-rearrangement studies to prove their clonality
[3].
Amyloidosis is related to altered proteins
[8], as evidenced by its
occurrence in approximately 10% of patients with myeloma
[3,
6]. Its distribution with
generalized deposition simulates that of primary systemic amyloidosis, a
diagnosis that depends on the exclusion of myeloma as well as the more obvious
causes of amyloidosis.
The modern classification of amyloidosis is based on the nature of the
precursor plasma proteins that form the fibril deposits
[2]. In AL amyloidosis, a
plasma cell dyscrasia related to multiple myeloma, clonal plasma cells in the
bone marrow (5-10%) produce immunoglobulins that are amyloidogenic
[2]. The amyloid deposits may
be massive and are usually associated with a giant cell reaction
[6]. They may also be subtle
and present only within vessels. Amyloid deposits can be found in a
myelomatous proliferation and may be abundant enough to mask the neoplasm
[2].
When reviewing 34 relatively well-documented cases of amyloid tumor of the
bone in the world literature of the past 100 years, Pambuccian et al.
[3] found the prognosis of
patients with these tumors to be generally poor, with most dying of local or
general complications of the disease in 8 months-3 years. With regard to the
biologic behavior of amyloidoma, progression to disseminated disease was
common [3]. Because of the
rarity of these lesions, there are no general recommendations on treatment
available. Given our patient and the preceding considerations on prognosis, we
believe a systemic approach and dealing with local complications are
justified.
References
- Unni KK. Introduction and scope of study. In: Unni KK, ed.
Dahlin's bone tumors: general aspects and data on 11,087
cases, 5th ed. Philadelphia: Lippincott Williams & Wilkins,1996
: 1-9
- Falk RH, Comenzo RL, Skinner M. The systemic amyloidoses.
N Engl J Med1997; 337:898
-909[Free Full Text]
- Pambuccian SE, Horyd ID, Cawte T, Huvos AG. Amyloidoma of bone, a
plasma cell/plasmacytoid neoplasm: report of three cases and review of the
literature. Am J Surg Pathol1997; 21:179
-186[Medline]
- Mizuno J, Nakagawa H, Tsuji Y, Yamada T. Primary amyloidoma of the
thoracic spine presenting with acute paraplegia. Surg
Neurol 2001;55:378
-382[Medline]
- Simoens WA, van den Hauwe L, Van Hedent E, et al. Amyloidoma of the
skull base. AJNR2000; 21:1559
-1562[Abstract/Free Full Text]
- Unni KK. Myeloma. In: Unni KK, ed. Dahlin's bone tumors:
general aspects and data on 11,087 cases, 5th ed. Philadelphia:
Lippincott Williams & Wilkins, 1996:225
-236
- Katz GA, Peter JB, Pearson CM, Adams WS. The shoulder-pad-sign: a
diagnostic feature of amyloid arthropathy. N Engl J
Med 1973;288:354
-355
- Gertz MA. Amyloidosis: recognition, prognosis and conventional
therapy. In: The American Society of Hematology, ed. Hematology
1999. New Orleans: American Society of Hematology,1999
: 339-347
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Introduction
Case Report
