(Chest. 1999;115:1735-1737.)
© 1999
American College of Chest Physicians
Finger Clubbing and a Lung Mass*
John D. Urschel , MD, FCCP;
Timothy M. Anderson , MD and
Brian P. Whooley , MD
*
From the Department of Thoracic Surgical Oncology, Roswell Park Cancer
Institute, Buffalo, NY.
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Introduction
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A
71-year old man complained of enlargement of his distal phalanges. He
did not have any other musculoskeletal complaints, and he denied
pulmonary symptoms. He was a heavy smoker. Medical history was
unremarkable. He did not have symptoms of cardiac or liver disease.
Physical examination showed obvious clubbing of his fingers. Chest
examination revealed dullness to percussion and decreased air entry at
the right base. There was no lymphadenopathy. Results of cardiac
examination were normal. The liver was not enlarged.
CBC and chemistry were normal. Fasting blood glucose level was also
normal. Chest radiography showed a mass in the right inferior-posterior
hemithorax (Fig 1)
that was best seen on the lateral radiograph (Fig 2) .
Chest CT scan showed a 12-cm mass arising from either the pleura or the
right lower lobe (Fig 3)
.
There was no lymphadenopathy. CT-guided needle biopsy specimen showed
benign fibrous tissue.
Bronchoscopy, done just prior to thoracotomy, was normal. Right lateral
muscle-sparing thoracotomy revealed a 12-cm mass within the lower lobe.
Right lower lobectomy and mediastinal lymph node sampling were
performed. The patient was discharged home from the hospital on the
second postoperative day. His finger clubbing completely resolved over
the ensuing 6 months.
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What is the diagnosis?
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Diagnosis: Solitary fibrous tumor of the pleura
Primary tumors of the pleura can be divided into two major
categories: diffuse and solitary.1
Diffuse pleural tumors
are mesotheliomas. They are more common than solitary pleural tumors,
arise from mesothelial tissue, are associated with asbestos exposure,
and almost always have a fatal course.2
Solitary tumors
have been known by a variety of names that are indicative of their
clinical course and controversies surrounding histogenesis. Previous
terminology included benign mesothelioma, localized mesothelioma,
subpleural fibroma, and localized fibrous tumor of the
pleura.1
3
4
These tumors are now known to be of
mesenchymal, as opposed to mesothelial, origin; the preferred term is
solitary fibrous tumor.5
They are not related to asbestos
exposure, and they follow a benign course in > 80% of
cases.1
There is no relationship between solitary fibrous
tumors and mesotheliomas.1
5
The pleural space is the
usual location for solitary fibrous tumors, but they may arise in a
wide range of tissues.5
This is not surprising considering
their mesenchymal histogenesis.
Patients with solitary fibrous tumors of the pleura may present
with cough, shortness of breath, chest pain, sensation of a mass moving
within the chest, hypoglycemia, hypertrophic pulmonary
osteoarthropathy, digital clubbing, fever, or an asymptomatic
radiographic abnormality.1
3
4
Both benign and malignant
solitary fibrous tumors can grow to massive proportions and cause
life-threatening compression of intrathoracic structures. Clubbing,
hypertrophic osteoarthropathy, and hypoglycemia are not typically seen
with mesothelioma, so the combination of a large pleural mass and one
of these paraneoplastic syndromes should suggest a diagnosis of
solitary fibrous tumor.1
Hypoglycemia is caused by tumor
production of insulin-like growth factor II.6
Hypoglycemic
coma and death have been caused by unrecognized solitary fibrous
tumors.1
7
Clinicians usually think of lung cancer with
brain metastases when presented with a patient with a large chest tumor
and altered mental status; solitary fibrous tumor should be kept in
mind in this clinical situation. Similarly, the combination of finger
clubbing and a chest mass is suggestive of lung cancer, but solitary
fibrous tumor is also possible. Paraneoplastic syndromes resolve with
surgical resection of the solitary fibrous tumor.3
Solitary fibrous tumors arise from the visceral pleura more
often than the parietal pleura.1
They are typically well
circumscribed, pedunculated masses, with large vessels within the tumor
pedicle. They can, however, be largely within the lung parenchyma, as
was the case in our patient. Radiologically, it is often difficult to
tell if the mass is pleural or pulmonary in nature. Bronchoscopy is
useful to exclude endobronchial tumors, such as lung cancer. A
transthoracic fine-needle aspiration biopsy specimen usually shows
benign-appearing spindle cells.
Ultimately, thoracotomy and tumor resection are usually required
for diagnosis. Histologic examination of benign solitary fibrous tumors
shows spindle cells with minimal nuclear pleomorphism and infrequent
mitoses. When malignancy occurs in a solitary tumor of the pleura, it
is characterized by pleomorphism, necrosis, and a high mitotic rate
(> 4 per 10 high-power fields).5
8
9
10
The morphologic
appearances of benign and malignant solitary fibrous tumors are
variable, and they may simulate a variety of soft-tissue
tumors.5
7
8
11
In addition, there are no specific
ultrastructural characteristics to separate solitary fibrous tumors
from some other soft-tissue tumors, such as fibrosarcoma. Until
recently, immunohistochemical characteristics (keratin negative,
vimentin positive, S-100 negative) were helpful, but not
diagnostic.8
The diagnosis of solitary fibrous tumor has
become more precise since CD34 antibody staining was found to be
positive in these tumors; it is now an essential pathologic feature for
the diagnosis of solitary fibrous tumors.5
11
Surgical resection of benign solitary fibrous tumors is usually
curative, but local recurrences can occur years after seemingly
adequate surgical treatment.1
3
5
7
Malignant solitary
fibrous tumors generally have a poor prognosis.1
3
7
9
Clinical follow-up and radiologic follow-up are indicated for both
benign and malignant solitary fibrous tumors.
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Footnotes
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Correspondence to: John D. Urschel, MD, FCCP, Thoracic Surgical
Oncology, Roswell Park Cancer Institute, Elm and Carlton Streets,
Buffalo, NY 14263-0001
Received for publication September 14, 1998.
Accepted for publication November 3, 1998.
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References
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Briselli, M, Mark, EJ, Dickersin, GR (1981) Solitary fibrous tumors of the pleura: eight new cases and review of 360 cases in the literature. Cancer 47,2678-2689[CrossRef][ISI][Medline]
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Qua, JC, Rao, UN, Takita, H (1993) Malignant pleural mesothelioma: a clinicopathological study. J Surg Oncol 54,47-50[Medline]
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Okike, N, Bernatz, PE, Woolner, LB (1978) Localized mesothelioma of the pleura: benign and malignant variants. J Thorac Cardiovasc Surg 75,363-372[Abstract]
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Robinson, LA, Reilly, RB (1994) Localized pleural mesothelioma: the clinical spectrum. Chest 106,1611-1615[Abstract/Free Full Text]
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Hanau, CA, Miettinen, M (1995) Solitary fibrous tumor: histological and immunohistochemical spectrum of benign and malignant variants presenting at different sites. Hum Pathol 26,440-449[CrossRef][ISI][Medline]
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Baxter, RC, Holman, SR, Corbould, A, et al (1995) Regulation of the insulin-like growth factors and their binding proteins by glucocorticoid and growth hormone in nonislet cell tumor hypoglycemia. J Clin Endocrinol Metab 80,2700-2708[Abstract]
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Witkin, GB, Rosai, J (1989) Solitary fibrous tumor of the mediastinum: a report of 14 cases. Am J Surg Pathol 13,547-557[ISI][Medline]
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Moran, CA, Suster, S, Koss, MN (1992) The spectrum of histologic growth patterns in benign and malignant fibrous tumors of the pleura. Semin Diagn Pathol 9,169-180[Medline]
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England, DM, Hochholzer, L, McCarthy, MJ (1989) Localized benign and malignant fibrous tumors of the pleura: a clinicopathologic review of 223 cases. Am J Surg Pathol 13,640-658[ISI][Medline]
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Carter D, Otis CN. Three types of spindle cell tumors of the pleura: fibroma, sarcoma, and sarcomatoid mesothelioma. Am J Surg Pathol 1988; 12:747–753
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van de Rijn, M, Lombard, CM, Rouse, RV (1994) Expression of CD34 by solitary fibrous tumors of the pleura, mediastinum, and lung. Am J Surg Pathol 18,814-820[ISI][Medline]
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Introduction
What is the diagnosis?
