(Chest. 2000;117:1505-1507.)
© 2000
American College of Chest Physicians
A 63-Year-Old Woman With a 2-Month History of Dyspnea*
Santiago E. Rossi, MD;
Holman P. McAdams, MD;
Jeremy J. Erasmus, MD and
Thomas A. Sporn, MD
*
From the Departments of Radiology (Drs. Rossi, McAdams, and Erasmus) and Pathology (Dr. Sporn), Duke University Medical Center, Durham, NC.
Correspondence to: Holman P. McAdams, MD, Duke University Medical Center, Box 3808, Durham, NC 27710; e-mail: mcada003{at}mc.duke.edu
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Introduction
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A 63
-year-old woman presented with a 2-month history of progressive dyspnea
and a 5-kg weight loss. She had no cough, hemoptysis, or chest pain.
There was no history of anorexia, diarrhea, or abdominal pain. She had
a 20-pack-year history of smoking but had stopped 20 years prior to
presentation. The patient’s medical history was otherwise
unremarkable.
On physical examination, mucous membranes, temperature, pulse rate,
respiratory rate, and BP were normal. The results of auscultation of
the lung and heart were normal. There were no abdominal, rectal, or
breast masses palpated, and there was no adenopathy. All laboratory
tests, including WBC count, RBC count, and hemoglobin level, were
normal.
The chest radiograph showed a well-circumscribed soft-tissue mass
overlying the left hilum (Fig 1
). Contrast-enhanced chest CT revealed a solitary periaortic mass of
heterogeneous attenuation in the posterior aspect of the left
hemithorax. The mass displaced the left lower lobe bronchus and
partially encased the descending aorta (Fig 2
). There were no other parenchymal abnormalities, mediastinal or hilar
adenopathies, or pleural effusions. The results of transbronchial and
transthoracic needle aspiration biopsies of the mass were
nondiagnostic. A video-assisted thoracoscopic lung biopsy was
performed.
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What is the diagnosis?
|
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Diagnosis: Inflammatory Pseudotumor of the Lung
Biopsy specimens showed the proliferation of a heterogeneous
population of inflammatory cells including macrophages, lymphocytes,
and neutrophils with peripheral fibrosis (Fig 3
). Immunohistochemical staining for
1-antitrypsin and CD-68 confirmed a
histiocytic phenotype.
Inflammatory pseudotumors typically consist of variable amounts of
stromal and cellular elements, with the myofibroblast, a cell involved
in tissue repair, now recognized as the principal cell
type.1
2
3
4
Depending on the major histopathologic features,
inflammatory pseudotumors are divided into the following types: fibrous
histiocytoma, lymphoplasmacytic, and organizing pneumonia. Because of
their variable histology, these masses have several synonyms, including
inflammatory myofibroblastic tumor, plasma cell granuloma, xanthoma,
xanthogranuloma, xanthomatous pseudotumor, and plasma cell-histiocytoma
complex.5
6
7
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Discussion
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Inflammatory pseudotumors are rare lesions that were first
described in the lung in 1939.8
They have been reported
subsequently in numerous locations including the liver, spleen, lymph
nodes, salivary glands, breast, soft tissues, and skin.6
The precise etiology of inflammatory pseudotumors is unknown, but they
may be caused by an exaggerated response to injury resulting in a
localized inflammatory lesion.7
Although these lesions are
inflammatory in nature and are thought to originate from organizing
pneumonia in most cases, aggressive features that mimic malignancy,
such as local invasion or recurrence, have been
reported.3
5
9
10
11
The age of the patient at presentation is variable, but the majority of
patients are < 40 years old (range, 13 months to 77
years).1
6
8
10
Although pseudotumors constitute < 1%
of all lung tumors, they are reported to be the most common cause of
solitary lung masses in children.12
Inflammatory
pseudotumors occur with equal incidence in men and women, and one third
of patients have a history of upper respiratory tract infection or
pneumonia.5
6
8
Forty to 70% of patients are symptomatic
at presentation with cough, fever, chest pain, dyspnea, or
hemoptysis.6
8
Laboratory investigations usually yield
normal results, although some patients have elevated erythrocyte
sedimentation rates and WBC counts.
Chest radiographs usually show a solitary, well-circumscribed,
lobulated nodule or mass that is 1 to 10 cm in diameter. The
lesions are typically located in the peripheral portions of the
lower lobes.10
Calcification is detected within the
lesion in 
40% of cases, but cavitation is uncommon.10
A chest CT scan usually reveals a nodule or mass of heterogeneous
attenuation; the masses typically show variable degrees of enhancement
after the IV administration of a contrast agent.10
Inflammatory pseudotumors uncommonly are manifested as multiple
pulmonary nodules and consolidation, mediastinal, or endobronchial
masses.10
Inflammatory pseudotumors that extend into the
hilum or central airways can result in lobar
atelectasis.10
Hilar or mediastinal adenopathy and small
ipsilateral pleural effusions are reported in 7% and 10% of
cases, respectively.
Because of the variable cellular composition of these masses,
diagnosis with transthoracic fine-needle aspiration or transbronchial
lung biopsy is difficult. Furthermore, because inflammatory
pseudotumors can demonstrate aggressive behavior and have overlapping
clinical and pathologic features with inflammatory sarcomas, complete
surgical excision is generally considered the treatment of
choice.3
7
8
13
14
15
Steroid therapy is usually not
helpful.3
Chemotherapy has been used to treat multifocal
lesions and recurrence.16
In conclusion, inflammatory pseudotumors are uncommon benign lesions of
the lung that may show aggressive behavior. The majority of patients
present with nonspecific symptoms and are < 40 years of age. These
lesions typically are manifested on a radiograph as solitary,
well-circumscribed lung nodules or masses. The diagnosis is seldom
confirmed preoperatively, and the treatment of choice is surgical
resection.
Received for publication June 11, 1999.
Accepted for publication July 23, 1999.
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References
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-
Mountney, J, Suvarna, SK, Brown, PW, et al (1997) Inflammatory pseudotumour of the lung mimicking thymoma. Eur J Cardiothorac Surg 12,801-803[Abstract]
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Bakhos, R, Wojcik, EM, Olson, MC (1998) Transthoracic fine-needle aspiration cytology of inflammatory pseudotumor, fibrohistiocytic type: a case report with immunohistochemical studies. Diagn Cytopathol 19,216-220[CrossRef][ISI][Medline]
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Bahadori, M, Liebow, AA (1973) Plasma cell granulomas of the lung. Cancer 31,191-208[CrossRef][ISI][Medline]
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Ishida, T, Oka, T, Nishino, T, et al (1989) Inflammatory pseudotumor of the lung in adults: radiographic and clinicopathological analysis. Ann Thorac Surg 48,90-95[Abstract]
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Matsubara, O, Tan-Liu, NS, Kenney, RM, et al (1988) Inflammatory pseudotumors of the lung: progression from organizing pneumonia to fibrous histiocytoma or to plasma cell granuloma in 32 cases. Hum Pathol 19,807-814[ISI][Medline]
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Coffin, CM, Dehner, LP, Meis-Kindblom, JM (1998) Inflammatory myofibroblastic tumor, inflammatory fibrosarcoma, and related lesions: an historical review with differential diagnostic considerations. Semin Diagn Pathol 15,102-110[ISI][Medline]
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Agrons, GA, Rosado-de-Christenson, ML, Kirejczyk, WM, et al (1998) Pulmonary inflammatory pseudotumor: radiologic features. Radiology 206,511-518[Abstract/Free Full Text]
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Gal, AA, Koss, MN, McCarthy, WF, et al (1994) Prognostic factors in pulmonary fibrohistiocytic lesions. Cancer 73,1817-1824[CrossRef][ISI][Medline]
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Cohen, MC, Kaschula, RO (1992) Primary pulmonary tumors in childhood: a review of 31 years’ experience and the literature. Pediatr Pulmonol 14,222-232[ISI][Medline]
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Lal, RB, Thompson, JR (1974) Postinflammatory tumor (xanthogranuloma) of the lung—an enigma. Ann Thorac Surg 17,174-180[ISI][Medline]
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Mandelbaum, I, Brashear, RE, Hull, MT (1981) Surgical treatment and course of pulmonary pseudotumor (plasma cell granuloma). J Thorac Cardiovasc Surg 82,77-82[Abstract]
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Berardi, RS, Lee, SS, Chen, HP, et al (1983) Inflammatory pseudotumors of the lung. Surg Gynecol Obstet 156,89-96[ISI][Medline]
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Boman, F, Champigneulle, J, Boccon-Gibod, L, et al (1995) Inflammatory myofibroblastic tumor of the lung with endobronchial, infiltrating, multifocal and recurrent form. Ann Pathol 15,207-210[ISI][Medline]
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TOP
Introduction
What is the diagnosis?
