CASE REPORT

Localized pleural amyloidosis: report of a case

Shohei Yoshiya • Riichiroh Maruyama • Takaomi Koga •

Yasunori Shikada • Tokujiro Yano • Yoshihiko Maehara

Received: 4 April 2011 / Accepted: 5 June 2011 / Published online: 31 January 2012

� Springer 2012

Abstract We herein describe an asymptomatic 31-year-

old male who was admitted for an investigation of an

abnormal pleural tumor detected by chest radiography. We

performed various preoperative investigations including

fluorodeoxyglucose-positron emission tomography. The

maximum standardized uptake value (SUVmax) was 2.2,

and malignancy could not be ruled out. We therefore car-

ried out a thoracoscopy-assisted partial resection of the

right upper lobe combined with a parietal pleurectomy. The

pathological examination showed that there was a tumor

localized with pleural amyloidosis.

Keywords Amyloidosis � VATS � Pleural tumor

Introduction

Amyloidosis is characterized by the extracellular deposi-

tion of an amyloid substance [1], and it is classified into

systemic and localized types. In the systemic disease,

amyloid is deposited in several organs, such as the kid-

neys, heart, liver, and peripheral nervous system, and

fatigue and weight loss are common presenting symptoms

[2]. However, the localized disease is asymptomatic in

many cases, because amyloid is deposited in only one

target organ. Although the lung parenchyma is sometimes

the target organ, amyloidosis is extremely rare in the

pleura. We herein report a rare case of localized pleural

amyloidosis.

Case

An asymptomatic 31-year-old male was admitted for the

investigation of an abnormal pleural tumor detected by

chest radiography. He had undergone an annual check-up

provided by his company every year, and had not been

noted to have any abnormality after any previous exam-

inations. He had a 20 cigarette/day smoking habit, and had

smoked since age fourteen. His occupation was in the

printing industry, and he had dust inhalation exposure. He

had also worked at a construction site approximately

10 years previously, and there was thus a potential that he

had experienced asbestos inhalation at that site. In addition,

he kept two dogs and seven cats in his house.

His physical examinations revealed that all values were

within the normal limits. In addition, the pulmonary

function tests were within the normal limits. Laboratory

examinations revealed: white blood cell count 4550/ll, red

blood cell count 438 9 104/ll, hemoglobin 13.4 g/dl,

hematocrit 40.3%, and a platelet count 20 9 104/ll.

Serological examinations, including the C-reactive protein

level, did not reveal any abnormalities. His serum levels of

carcinoembryonic antigen (CEA), cytokeratin 19 fragment

(CYFRA) and pro-gastrin releasing peptide (ProGRP) were

0.4 ng/dl (normal range 0–5 ng/ml), 0.9 U/ml (normal

range 0–2.0 U/ml) and 38.3 pg/ml (normal range 0–46 pg/

ml), respectively. Immunological investigations revealed:

soluble interleukin-2 receptor (sIL-2R), 302.6 U/ml (nor-

mal range 122–496 U/ml); QuantiFERON-TB-2G (QFT),

S. Yoshiya (&) � R. Maruyama � Y. Shikada � T. Yano �Y. Maehara

Department of Surgery and Science, Graduate School of Medical

Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku,

Fukuoka 812-8582, Japan

e-mail: yoshiya@surg2.med.kyushu-u.ac.jp

T. Koga

Department of Pathology, Graduate School of Medical Sciences,

Kyushu University, Fukuoka, Japan

123

Surg Today (2012) 42:597–600

DOI 10.1007/s00595-012-0122-z

negative; Cryptococcus neoformans antigen, negative; and

(1 ? 3)-b-D-glucan, negative.

Both chest radiography and enhanced computed

tomography (CT) scans showed a tumor mass along the

pleura out of the right apical segment, and the tumor

projected to the lung side between the second and third

ribs (Fig. 1a, b). The cortical layer of the bone close to

the tumor was normal. No mediastinal lymphadeno-

pathy, hilar lymphadenopathy or distant metastasis was

found. No abnormal findings of other organs were

observed.

Magnetic resonance imaging (MRI) demonstrated that

the tumor showed the same degree of signal as the muscle

(iso-slightly high signal) in T1WI and a partially high

signal in T2WI. The signal of this tumor was reinforced by

Gd enhancement, and the reinforcement was widely seen

along the pleura (Fig. 1c). 18F-fluorodeoxyglucose-positron

emission tomography (FDG-PET) showed that the maxi-

mum standardized uptake value (SUVmax) of this tumor

was 2.2, and therefore malignancy could not be ruled out

(Fig. 1d). There was no abnormal accumulation, thus

suggesting mediastinal lymphadenopathy, hilar lymphade-

nopathy or distant metastasis detected.

A transbronchial lung biopsy specimen showed only

mild chronic inflammatory cells, and there was no malig-

nancy. We were therefore unable to diagnose this tumor as

benign or malignant, but the patient requested surgical

treatment. Therefore, a thoracoscopy-assisted partial

resection of the right upper lobe combined with a parietal

pleurectomy was performed. The patient had an uneventful

postoperative recovery without any complications, and he

was discharged on postoperative day 9.

Fig. 1 Both chest radiography

(a) and enhanced computed

tomography (CT) scans

(b) showed a tumor mass along

the pleura out of the right apical

segment, and the tumor

projected to the lung side

between the second and third

ribs. c Magnetic resonance

imaging (MRI) demonstrated

that the signal of this tumor was

reinforced by Gd enhancement.

d 18F-fluorodeoxyglucose-

positron emission tomography

showed that the maximum

standardized uptake value

(SUVmax) of this tumor was 2.2

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Fig. 2 a The resected specimen

measured 28 9 18 mm. The

upper side is the area of the lung

parenchyma, and the lower sideis the pleural area. b The tumor

was a white, elastic, hard mass

Fig. 3 a Dylon staining 912.5, b amyloid P staining 912.5,

c amyloid A staining 912.5. The tumor was positive for dylon

staining and amyloid P staining, but negative for amyloid A staining.

d1 (H&E stain 920): There was a minute abscess that was of pleura

origin. d2 (H&E stain 9400) Aggregated plasma cells were detected

around the minute abscess

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The pathological examination of the resected specimen

showed a tumor which measured 28 9 18 mm that was

composed of amyloid deposition (Fig. 2). The pleural area

of this specimen showed diffuse positivity for dylon

staining and amyloid P staining. However, it was negative

for amyloid A staining (Fig. 3a–c). Almost all of the pul-

monary parenchyma area of this specimen was negative for

dylon staining. Moreover, a minute abscess that was of

pleura origin was observed, and there were aggregated

plasma cells around this abscess (Fig. 3d1, d2). There was

no lymph node metastasis. The tumor was pathologically

confirmed to be amyloidosis, excluding the AA type.

As of 7 months after the surgery, no symptoms of other

organ amyloidosis have been detected.

Discussion

Amyloidosis is characterized by the extracellular deposition

of an amyloid substance [1]. The classification of amyloidosis

is based on the different subunit proteins, which define the

organ involvement and disease manifestation. There are four

major categories of amyloidosis: primary (systemic or

localized) or immunoglobulin light chain (AL) disease, sec-

ondary or amyloid protein A (AA) disease, hereditary or

mutant transthyretin (ATTR) disease, and dialysis-associated

or b2-microglobulin (b2M) disease [3]. AA disease results

from the excessive production and organ-specific deposition

of an acute-phase reactant, serum amyloid A protein. On the

other hand, AL disease arises from the deposition of mono-

clonal j or k immunoglobulin light chains. In this case, the

pathological examination showed that the tumor was not an

AA type amyloidosis and the patient did not have any family

history of amyloidosis and had not received dialysis treat-

ment. Therefore, AL type amyloidosis was suggested.

In cases of the AL type, there are two sub-types of

disease: systemic and localized. In the systemic disease,

amyloid is deposited in several organs and ultimately leads

to organ failure. Fatigue and weight loss are common

presenting symptoms, but the diagnosis is rarely made until

symptoms referable to a particular organ appear. Kyle and

Gertz [2] reported that the most prominent clinical mani-

festations were in the kidneys, heart, liver and peripheral

nervous system. Various abnormalities appear depending

on the type of organ involvement: proteinuria in case of

renal amyloidosis; low voltage, various arrhythmias on

ECG, thickened ventricles, and dilated atria on UCG in

cardiac amyloidosis, and so on [4].

On the other hand, localized amyloidosis is associated

with no symptoms in many cases, because amyloid is

deposited in only one target organ. Berk et al. [3] showed

that localized amyloid deposits arise from a small number

of plasma cells surrounding the lesion, and indicated that

the most commonly involved sites included the skin, ure-

thra and urinary bladder, eyes, and lung parenchyma.

In our case, the patient had no symptoms and did not

show any abnormalities in our various examinations. At the

same time, the pathological examination showed aggre-

gated plasma cells around a minute abscess that was of

pleura origin. Therefore, we diagnosed the tumor as

localized pleural amyloidosis.

Localized pulmonary amyloidosis has been reported

many times [3, 5, 6], but localized pleural amyloidosis is

extremely rare, and only a very small number of people has

reported on this disease [7]. The previous reports have

shown that pleural amyloidosis is often accompanied with

pleural effusion as one symptom of the systematic disease

[8–10] and suggested that the amyloid deposited in the

pleura might be an incidental finding [8]. However, we first

detected the pleural lesion without any pleural effusion in

this case. Therefore, our present report emphasizes that

localized pleura amyloidosis should be considered as one

of the differential diagnoses when there is difficulty in

concluding the preoperative diagnosis of a pleural lesion.

Acknowledgment We thank Dr. Brian Quinn for critical comments

on the manuscript.

Conflict of interest Shohei Yoshiya and co-authors have no con-

flicts of interest.

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