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Case Rep Clin Pract Rev, 2007; 8: 269-272 w w w . c r c p r – on l i n e . c o m
Case Report
Received: 2007.04.11
A giant fibrous tumor of the pleura mimics plural
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Accepted: 2007.08.22
Published: 2007.11.05
effusion: a case report and review of the literature
Omer S. Alamoudi, Abdulrahman H. Demerdash
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Department of Medicine, King Abdulaziz University Hospital (KAUH), Jeddah, Saudi Arabia
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Summary
Background: A solitary fibrous tumor of the pleura (SFTP) is very rare with an incidence of less than 5%
of all pleural neoplasm. Although some tumors may reach an enormous size, they are often
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asymptomatic and have sometimes been mistaken as a pleural effusion.
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Case Report: Herein we describe a 33 yr-old male patient who diagnosed with a giant SFTP. Chest
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radiograph revealed a large homogenous opacity filling the right hemithorax that was
initially reported as a pleural effusion. Pleural tap was performed twice and turned out
to be dry. Computed Tomography) of the chest unexpectedly showed a large hypodense
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mass filling the right hemithorax. Transthoracic CT guided Tru-Cut biopsy revealed a
benign solitary fibrous tumor of pleura that was resected surgically, and the patient made
an eventual recovery.
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Conclusions: SFTP is a rare tumor. High index of clinical suspicion is essential to reach diagnosis. Long
term follow up is important to assess for recurrence.
Key words: Fibrous tumor • Pleural effusion
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Tables: -
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Figures: 3
References: 13
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Author’s address: Omer S. B. Alamoudi, Department of Medicine, KAUH, P.O.Box 80215, Jeddah 21589, Saudi Arabia,
e-mail: dramoudi@yahoo.com
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Case Report
Background He had no history of smoking, alcohol or drug abuse.
On examination: he was febrile with a temperature
A solitary fibrous tumor of the pleura (SFTP) is a of 37.8 C and his blood pressure was 110/70 mmHg.
rare disease, representing less than 5% of the total His height was 161 cm and weight 63 kg. His oxygen
pleural neoplasm. The majority of these tumors (88%) saturation was 97% at room air. He had no clubbing,
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are benign [1]. It arises in 75% of presenting patients cyanosis, or palpable lymph nodes. Chest examination
from the submesothelial connective tissue of the vis- showed signs of consolidation / collapse and pleural
ceral pleura, while in the remaining 25% from the effusion over the right hemithorax. Examination of
parietal pleura [2]. It affects men and women equal- the Cardiovascular, abdominal and central nervous
ly [3]. The peak incidence is usually between fourth systems were unremarkable. Initial investigations
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to sixth decades [2-4]. Forty percent of the patients showed Hg: 13 gm/dl, WCC: 8.5/cmm. ESR: 3 mm/h,
are asymptomatic at the time of presentation, while CRP: 150u (N <10u). Chest radiograph revealed a
the remaining may present with chest pain, cough, large homogenous opacity filling the right hemitho-
dyspnea, fever and/or pleural effusion [5]. Some tu- rax and pushing the mediastinal structures anteriorly
mors reach an enormous size and may be mistaken and to the left that was initially mistakenly reported
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as a pleural effusion [6, 7]. Some tumors have been by a senior radiologist as a massive pleural effusion.
discovered incidentally either during routine chest He consequently underwent 2 unsuccessful attempts
radiograph or by CT scan of the chest. Diagnosis is of pleural aspiration. CT scan of the chest was per-
usually obtained after surgical resection of the tumor formed and unexpectedly revealed a huge multilobu-
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and rarely reached preoperatively by Tru-cut biopsy lar, oval, well-defined encapsulated hypodense mass
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[5, 6, 8]. Surgical resection of the tumor is the main compressing the adjacent lower lobe of the right lung
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line of therapy [9]. Recurrence rate may reach up to and pushing the mediastinum anteriorly. There was
2%, and may require further resection [5, 9]. The no mediastinal lymph nodes enlargement (Fig 1).
aim of this study was to describe an extremely rare Tru-cut biopsy was performed and the histopathology
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case of a huge solitary fibrous tumor of the pleura showed benign fibrous tissue. Bronchoscopy showed
that was initially mistaken as pleural effusion and to narrowing of the right middle and lower bronchial
review the relevant literature. segments mainly due to external compression. The
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patient underwent surgical resection. The tumor was
case report large oval, encapsulated, firm in consistency, grayish
in color, measured 25 x 20 x 15cm and weighed 1600
A 33 yr old male Yemeni patient, who was work- grams and arose from the visceral pleura (Fig 2). The
ing as a salesman, was admitted to King Abdulaziz cut section was whorly. It was firmly adjacent to the
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university hospital (KAUH) through the emergency lower lobe of the right lung but macroscopically had
room (ER) in mid November, 2005. He presented no bronchial or diaphragmatic infiltration. The his-
with cough, dyspnea and pleuritic chest pain of 2 topathological diagnosis was localized benign fibrous
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months duration that was associated with fever and tumor of the pleura (Fig 3). Immunohistochemistry
rigors. There was no history of anorexia, weight loss of the neoplastic cell were positive for CD34 and vi-
or hemoptysis. Systemic review was unremarkable. mentin and negative for pankeratin (CK, AE1, and
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Figure 1. CT scan of the chest showing multi lobular well Figure 2. The tumor after resection showed a large multilobulated
encapsulated thin walled mass pushing the mediastinum oval, encapsulated mass that measured 25 x 20 x 15 cm and
anteriorly. weighed 1600 grams.
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A giant fibrous tumor of the pleura...
AE3) and calretinin confirming the diagnosis of soli-
tary benign fibrous tumor. Post-operatively, his right
lung fully expanded and regained normal size. Two
months later, he was seen in the outpatient clinic in
good general condition.
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discussion
Localized fibrous tumor of the pleura (LFTP) is rare,
accounting for less than 5% of all reported pleural
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tumors [1, 11]. In the literature, up to 500 cases have
been reported [1]. It usually considered benign, but
malignant changes may also occur (11). The peak
incidence is usually between the fourth to sixth de- Figure 3. Histopathology of the tumor showed cells scattered
cades of life [1, 8]. However, children may also be haphazardly (upper arrow) among strands of collagen fibers
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affected [1]. In our patient the tumor was diagnosed (lower arrow) (HE, ×165).
in his early thirties. Patients usually present with
chest pain, dyspnea, and cough and rarely with he- A CT scan is more sensitive than chest radiograph
moptysis. Extrathoracic manifestations may include in diagnosing SPFT. The usual finding is a lobulated
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a wide range of symptoms such as weakness, weight mass with variable heterogeneity according to the
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loss, nocturnal sweating, fever and chills [1, 11]. grade of necrosis with and without calcifications. It
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Symptoms tend to be more common when a larger is useful in assessing the relationship of the tumor
tumor is involved [1]. More than 50% of reported with the adjacent structures including mediastinum
cases were found incidentally during routine chest and to detect the presence of the pleural effusion, if
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radiographs [5, 10, 11]. In our patient, fever, chills, any [4, 9]. Magnetic Resonance Imaging (MRI) also
chest pain, and dyspnea were the main presenting helps surgeons to define the borders between the tu-
symptoms. Digital clubbing, and or hypertrophic os- mor and the surrounding structures before resection
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teoarthropathy have also been reported in up to 20% [11, 12]. The role of Positron Emission Tomography
of the patients [2, 11]. Interestingly, these changes (PET) has not yet been defined [9]. Bronchoscopy
are more common in patients with LFTP than with also has a minimal role in the diagnosis of such tu-
bronchogenic carcinoma. It is mainly attributed to mors as in our patient [5]. Transthoracic CT-guided
the fact that tumor cells may produce hyaluronic biopsy does seem to be a reliable diagnostic method,
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acid, which has an osteolytic effect [8]. Recurrent particularly if good tissue can be obtained [5, 9]. In
episodes of hypoglycemia have also been observed our patient the diagnosis was reached by Tru-cut
in 2–4% of reported cases [3, 11]. This is due to biopsy that was consistent with the histopathology of
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the production of the insulin-like growth factor II the excised tumor. The tumors appear macroscopi-
(IGF-II), which causes an increase of glucose utiliza- cally as encapsulated, firm and lobulated masses with
tion and an impairment of growth hormone counter- a characteristic whorled appearance. The size of the
regulatory response to hypoglycemia [10, 11]. Such tumors is variable and ranges from small nodular
symptoms may regress completely after surgical masses within the lung to large pedunculated tumors
resection [1, 11]. In our patient there was no digital within the pleural cavity [2, 13], which may weigh up
clubbing, hypertrophic osteoarthropathy, or hypo- to more than 1500 grams [2, 4]. In our patient the
glycemic episodes although the size of the tumor weight of the tumor was 1600 grams. Histologically,
was significantly large. The differential diagnoses the tumors are classified as benign or malignant
of LFTP should include; malignant mesothelioma, according to the criteria used by England et al. [3].
metastatic carcinoma, sarcomas, pleural lipoma, Malignancy should be suspected if one or more of the
bronchogenic cyst, pulmonary abscess, and bron- following criteria is present; 1) high mitotic activity,
chogenic carcinoma [1, 9]. Chest radiograph is an 2) high cellularity with crowding and overlapping of
important diagnostic tool, but may not be specific. nuclei, 3) presence of necrosis and 4) pleomorfism.
Large SFTP may appear as a homogeneous opac- The incidence of malignancy in the LFTP is variable
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ity in the chest radiograph that may be mistaken as in different studies and enncompasses 30% to 38%
pleural effusion as occurred with our patient [7]. of all reported cases [5, 11]. The management of
However, the majority of the patients with SPFT SFTP consists of adequate surgical resection dur-
have no pleural effusion [3, 5, 9]. A previous study ing which great care must be taken to avoid bleed-
by England et al. found that approximately 8% of ing tendency due to high vascularity of the tumor
138 patients with SPFT had a pleural effusion [3]. pedicle and surrounding adhesions [1, 5, 11]. The
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Case Report
intra-operative mortality rate is about 12% and primary tumor, and therefore, regular, long term
mainly due to decompression of the mediastinal follow up is highly recommended [5, 8, 9, 11].
structure that leads to fatal cardiopulmonary ar-
rest [4, 7]. The recurrence rate is low, reported as conclusions
occurring in 2% of patients presenting with benign
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tumor [9]. The factors increasing the incidence of SFTP is a rare tumor. Recurrence may occur up
recurrence include the size of the tumor, presence of to 17 years after initial resection of the primary
necrosis in excised tissue, cell polymorphism, number tumor, and therefore, regular and long term follow
of mitosis and resectability [3, 8, 13]. Recurrence up is highly recommended [5, 8, 9, 11] to assess for
may occur up to 17 years after initial resection of the recurrence.
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references:
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ra. Crit Rev Oncol Hematol 2003; 47: 29-33. ic surgery 2002 ; (18)4: 491-94.
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2. Briselli M, Mark EJ, Dickersin GR.. Solitary fibrous tumors of the pleu- 8. Suter M, Gebhard S, Boumghar M, Peloponisios N, Geton CY.
ra: eight new cases and review of 360 cases in the literature. Cancer Localized fibrous tumors of the pleura: 15 new cases and review of
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3. Egland DM, Hochholzer L, McCarthy MJ. Localized benign and ma- 9. Torre W, Sola S R , DelgadoA M. Localized Fibrous Tumors of the Pleura:
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10. Adhami N, Ahmed R, Lento P A, Shemshi M, Herman S D, Tierstien
4. Bicer M, Yaldiz S, Gursory S, Uigan M. A case of giant benign A S. Fibrous pleural tumor with hypoglycemia: Case Study. The Mount
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fibrous tumor of pleura Eur J of Cardio-Thoracic Surgery 1998(14): Sinai Journal of medicine, 2004: 71(5): 344-46.
211-21. 11. Rena O, Filosso PL, Papalia E, Molinatti M, Di Marzio P, Maggi G,
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5. Magdeleinat P, Alifano M, Petino A, Le Rochais J-P, Dulmet E, Galateau Oliaro A. Solitary fibrous tumor of the pleura: surgical treatment. Eur
F, Icard P, Regnard JF Solitary fibrous tumors of the pleura: clinical J Cardio-Thoracic Surg , 2001:19(2): 185-89.
characteristics, surgical treatment and outcome Eur J Cardio-Thoracic 12. Rosado-de-Christenson ML, Abbott GF, McAdams HP, Franks TJ,
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Galvin JR . From the archives of the AFIP: Localized fibrous tumor
6. Ulrik C.S., Viskum K. Fibrous tumor producing 171 liters of trans- of the pleura. Radiographics. 2003; 23(3): 759-83.
udate Eur. Respir J 1998: 12: 1230-32. 13. Chang YL, Lee YC, Wu CT. Thoracic solitary fibrous tumor: clinical
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