73 yo male hypertension, incidental
discovery of a thoracic mass. Endoscopic biopsy negative.
Patient hypertendu, 73 ans. Decouverte
fortuite d'une masse thoracique.
evaluation proposed the diagnosis below, confirmed on the surgery specimen.
mesures 16 cm in greatest diameter, lobulated, surgically easily removed,
attached by a small pedicle to the pleura.
Cut surface shows a nodular
pattern, with large whorled white areas. Other are soft, yellowish, with
foci of necrosis.
Most of the sections
observed disclose a fascicular , moderately cellular, collagen producing
High Power, shows anisonucleosis,
Low power, sharp margins
of the nodules, and area of necrosis.
pattern, focally observed.
High power view on the
Other areas (grossly,
soft and yellowish), disclose a more cellular pattern.
These areas have a sarcomatous
Silver stain demonstrates,
a less pronounced meshwork than observed in the previous fields.
High power view, discloses
nuclear atypia, and high mitotic count.
Atypical mitotic figures
CD 34 is positive, along
with a high expression of p53. The other immunostains (cytokeratins, Actin,
Desmin, and neural markers) are negative.
fibrous tumor of the pleura.
Solitary fibrous tumor of pleura
Solitary fibrous tumor of
pleura (formerly called solitary fibrous mesotheliorna) is usually asymptomatic,
although on occasion patients present with pain, cough, dyspnea, hypoglycemia-related
symptoms, and/or prominent pulmonary osteoarthropathy that rapidly regress
when the tumor is removed.This tumor is not associated with asbestosis.
On rare occasions, it is found in association with a microscopically similar
tumor in the peritoneum or retroperitoneum. Grossly, the lesion is well
circumscribed, firm, lobulated, graywhite to yellow-white, with frequent
whorling and fasciculation. The mean diameter is 6 cm. The appearance
is reminiscent of uterine leiomyoma . Cystic degeneration is very unusual,
but solitary fibrous tumor can present as a mural nodule within a large
pleural-lined cyst. It may be found attached to the visceral pleura (80%)
or parietal pleura, within an interlobar fissure, or sometimes within
the lung parenchyma without a pleural connection.Lesions with an identical
microscopic appearance occur in the peritoneum, retroperitoneurn (including
kidney), mediastinum, nasopharynx, orbit, breast, and probably other sites.
both benign and malignant forms occur, the former greatly predominating.
The distinction between them can be difficult.In the typical benign case,
there is a tangled network of fibroblast-like cells, accompanied by deposition
of abundant collagen fibers, many of which have a keloid-like quality.
The degree of cellularity varies a great deal from area to area. Hemangiopericytornalike
areas are frequent. The most cellular types may be misdiagnosed as fibrosarcoma
or malignant schwannoma . If proper attention is paid to the lack of nuclear
aberrations and the rarity or absence of mitoses, confusion is unlikely
to occur. In some instances, clusters of cuboidal cells forming papillae,
tubules, or solid nests are found at the periphery of predominantly fibrous
tumors; these represent entrapped mesothelium. or bronchioloalveolar epithelium
and should not be misconstrued as evidence of a biphasic composition of
the tumor, as it has been done in the past.The malignant types are characterized
by increased cellularity, atypia, mitotic activity, and necrosis.
This neoplasm has been regarded
for many years as a form of mesotheliorna, largely on the basis of the
pattern of growth that the tumor cells exhibit in tissue culture; however,
on ultrastructural examination these cells show fibroblast-like rather
than mesotheliallike features, and by immunohistochernistry they are reactive
for vimentin and sometimes desmin rather than keratin. The most likely
interpretation of this tumor, therefore, is that it arises from noncommitted
mesenchymal cells present in the areolar tissue subjacent to the mesothelial
lining.This has led to the proposal of alternative terms such as submesothelioma
or submesothelial fibroma.
The tumor cells of this lesion
stain strongly and consistently for CD34, a finding that may be of use
in the differential diagnosis with some of the entities already described.
Almost 90% of these neoplasms
are cured by surgical excision. In a thorough review of the literature,
Briselli et al. found that 12% had caused death because of extensive intrathoracic
growth. Indicators of good prognosis were presence of a pedicle, good
circumscription, and absence of nuclear pleomorphism or mitotic activity.
(Ref. Ackerman's Surgical Pathology,
Some recent abstract
about Solitatry Fibrous Tumor of Plaura
Nihon Kokyuki Gakkai Zasshi
tumor of the pleura with elevated high-molecular-weight insulin-like growth
factor II and hypoglycemia.
Mori K, Ohtsuki Y, Hizuka
N, Division of Internal Medicine, Syuso Public Hospital, Ehime, Japan.
Recurrent hypoglycemia occurred
in a 58-year-old woman with a solitary fibrous tumor of the pleura and
exhibiting CD34 immunopositivity. During episodes of hypoglycemia, serum
high-molecular-weight insulin-like growth factor II (big IGF-II) was elevated.
After removal of the tumor containing IGF-II, the hypoglycemia and serum
big IGF-II disappeared. This was followed by an increase in normal IGF-II.
We speculate that the primary cause of hypoglycemia in this patient was
the presence of big IGF-II produced by the solitary fibrous tumor of the
pleura. To our knowledge, the presence of this type of IGF-II in CD34+
solitary fibrous tumors of the pleura has not been described to date in
Rev Clin Esp 1999 Jul;199(7):437-9
tumors of the pleura.
Roca Calvo MJ, Manes Bonet
N, Alix Trueba A, Freixinet Gilart J. Servicio de Cirugia Toracica, Hospital
Universitario Nuestra Senora del Pino, Las Palmas de Gran Canaria.
Seven cases of solitary fibrous
tumours of the pleura are here reported, formerly named benign fibrous
mesotheliomas. Histologically, these tumours are formed by fusiform cells
with a minimal cellular pleomorphism and absence of mitosis. These tumours
cause scarce symptoms which are usually diagnosed as a casual finding
when a chest X-ray is performed on account of other cause. Treatment should
be surgical, with removal of all tumoral mass, thus avoiding its growth
and the possibility of the reported degeneration towards malignancy.
Chirurg 1999 Aug;70(8):949-52
tumors of the pleura.
Meyer M, Krause U Klinik
fur Herz- und Thoraxchirurgie, Martin-Luther-Universitat, Halle-Wittenberg.
Solitary fibrous tumors of
the pleura (SFTP) are very rare neoplasms. The majority of these tumors
are benign, but about 10-20 % fulfill the criteria of malignancy. The
clinical presentation varies according to the size and intrathoracic localization.
In early stages, often asymptomatic, the tumors may grow to an enormous
size and then cause symptoms such as cough, chest pain and dyspnea, but
also paraneoplastic syndromes such as hypoglycemia or digital clubbing.
Between 1981 and June 1998 we treated in our institution 16 SFTP in 14
patients (4 M, 10 F, average age at first operation 58 years). Eight patients
showed symptoms, whereas in the other cases the tumors were found on routine
chest X-rays. The usually pedunculated SFTP were completely resected without
complications. Two patients developed malignant recurrences, which infiltrated
the right upper lung lobe and the diaphragm respectively. In these cases
the tumor was resected together with the adjacent structures. Since
late recurrences are more often malignant than primary SFTPs long-term
follow-up is mandatory even in benign lesions.
Ann Thorac Surg 1999 May;67(5):1456-9
of solitary fibrous tumors of the pleura.
de Perrot M, Kurt AM, Robert
JH, Borisch B, Spiliopoulos A Department of Surgery, University Hospital
of Geneva, Switzerland.
BACKGROUND: Solitary fibrous
tumors of the pleura are rare and presentunpredictable clinical behavior.
METHODS: Between 1981 and 1998, 11 solitary fibrous tumors of the pleura
were resected in 10 patients at the University Hospital of Geneva. Their
clinical behavior and outcome were reviewed. RESULTS: Seven tumors arose
from the visceral pleura, and three arose from the parietal pleura. Tumors
arising from the parietal pleura were revealed to be more difficult to
resect than those from the visceral pleura because of their size and adhesion
to the chest wall requiring extrapleural resection. Eight tumors showed
benign features, whereas two showed distinct features of malignity. One
additional patient presented marked pleomorphism that could represent
an intermediate form before frank malignity. Four tumors had been followed
expectantly for 2 to 10 years before surgery. Although three enlarged
rapidly, no signs of malignity were observed on histological examination.
All patients are alive, from 2 months to 14 years after surgery (mean
55 months). In one case, however, a malignant tumor recurred 6 years after
resection of a benign variant. CONCLUSIONS: Although histologically
benign, solitary fibrous tumors of the pleura may enlarge rapidly and
occasionally transform into malignant variants after several years. Therefore,
complete surgical resection and long-term follow-up is recommended for
Can J Surg 1998 Dec;41(6):467-9
solitary fibrous tumours of the pleura (localized "mesotheliomas"):
a case report.
Urschel JD, Brooks JS, Werness
BA, Antkowiak JG, Takita H Department of Thoracic Surgery, Roswell Park
Cancer Institute, Buffalo, NY 14263-0001, USA.
Solitary fibrous tumours
are uncommon pleural tumours that are not related to mesotheliomas. They
are typically benign and pedunculated and may grow to massive sizes. Surgical
resection is usually curative. Tumour recurrence and metachronous development
of multiple tumours are unusual. In this report a patient was treated
for 3 benign solitary fibrous tumours of the pleura over 23 years. The
authors hypothesize that this represents multifocal tumorigenesis as opposed
to local tumour recurrence. The importance of complete surgical excision
and lifelong radiographic follow-up are stressed.
Histopathology 1998 May;32(5):423-32
tumour: significance of p53 and CD34 immunoreactivity in its malignant
Yokoi T, Tsuzuki T, Yatabe
Y, Suzuki M, Kurumaya H, Koshikawa T, Kuhara H, Kuroda M, Nakamura N,
Nakatani Y, Kakudo K Department of Pathology, Wakayama Medical College,
Wakayama City, Japan.
AIMS: To clarify the association
of p53 and CD34 expression with development of malignant solitary fibrous
tumour we have studied 10 cases of solitary fibrous tumour arising in
the pleura, retroperitoneum and pelvic cavity with clinicopathological
features of malignancy. METHODS AND RESULTS: Tumours were localized solid
masses with or without necrosis in eight and they nearly totally occupied
the pleural cavity in two. Basic histology of the tumours was the proliferation
of spindle cells arranged in 'patternless' pattern or in interlacing bundles
with nuclear atypia and mitotic activities of various degree. In two,
high-grade foci were present within low or intermediate-grade tumours.
Recurrent tumours also showed more atypical features than primary tumours
in two. Immunohistochemical studies showed CD34 positivity in seven, but
three of them showed marked diminution or complete loss of CD34 expression
in high-grade foci or a recurrent tumour. Three high-grade cases showed
totally negative staining for CD34. p53 was strongly expressed in cases
with fatal outcome, clinical recurrence, nuclear atypia, high mitotic
activity or local invasion, whereas almost negative in benign tumours.
CONCLUSIONS: Malignant solitary fibrous tumours may occur de novo or by
transformation within benign or low-grade tumours and may be associated
with p53 mutation. Although CD34 is a useful marker in the diagnosis of
solitary fibrous tumour, one should bear in mind that its expression can
be lost in high-grade tumours.
Kyobu Geka 1998 Jun;51(6):504-7
Malignant recurrence of
solitary fibrous tumor of the pleura developed 20 years after the surgery:
a case report of re-extirpation.
Ito H, Arai T, Niino H, Inagaki
K, Morita T, Yano M, Nomura T Department of Thoracic Surgery, International
Medical Center of Japan, Tokyo,Japan.
A 63-year-old female developed
the chest wall tumor protruding into the right thoracic cavity. She consulted
our hospital complaining of chest pain. 20 years before this episode,
she had undergone tumor extirpation combined with the partial resection
of adherent anterior chest wall and the diaphragm for large benign solitary
fibrous tumor of the pleura. Because of the difficulty in making diagnosis
by needle biopsy, operation (re-tumor extirpation combined with the chest
wall) was done. Histological examination revealed that the tumor was malignant
solitary fibrous tumor. We concluded that the tumor was the malignant
recurrence of the benign solitary fibrous tumor resected 20 years before.
Occasionally, benign solitary fibrous tumor of the pleura recurs and behaves
more aggressively than primary, but such a long latent period as 20 years
is quite uncommon. So we present the case and the review of the literature.
Ann Thorac Surg 1998 May;65(5):1461-4
Giant solitary fibrous
tumor of the pleura.
Khan JH, Rahman SB, Clary-Macy
C, Kerlan RK, George TI, Hall TS, Jablons DM Department of Thoracic Surgery,
Mount Zion-UCSF Cancer Center 94115, USA.
Solitary fibrous tumors of
the pleura are rare. Approximately 600 cases have been described in the
literature. We report a case of a young man with a giant solitary fibrous
tumor of the pleura that filled his entire left hemithorax and anterior
mediastinum and extended into the right side of his chest. The diagnostic
modalities employed, the operation, and the postoperative management resulting
in complete resection of the tumor and full lung reexpansion are described.
Jpn J Clin Oncol 1998
of bcl-2 protein in solitary fibrous tumors.
Hasegawa T, Matsuno Y, Shimoda
T, Hirohashi S, Hirose T, Sano T Pathology Division, National Cancer Center
Research Institute, Tokyo, Japan.
The distinction of solitary
fibrous tumors from histologically similar neoplasms is often difficult
because they rarely occur at a variety of extrapleural sites. CD34 immunoreactivity
has recently been recognized to be an adjunct for the diagnosis of solitary
fibrous tumors. However, it is now known that CD34 staining is not entirely
specific for this entity. We evaluated 23 solitary fibrous tumors and
54 other spindle cell tumors often considered in the differential diagnosis
for immunoreactivity using monoclonal antibodies directed against bcl-2
protein, which protects cells from apoptosis and CD34. The patients with
solitary fibrous tumors comprised 11 men and 12 women, ranging in age
from 35 to 85 years (mean, 57.6 years). Fourteen tumors arose in the pleura,
four in the retroperitoneum, three in the superficial soft tissue and
one each in the mediastinum and uterine cervix. Nineteen of 23 solitary
fibrous tumors (83%), irrespective of tumor site, demonstrated diffuse
cytoplasmic staining for bcl-2 protein. bcl-2 immunoreactivity was also
observed in five of seven neurofibromas (71%), eight of 10 synovial sarcomas
(80%) and one of three spindle cell lipomas (33%). CD34 immunoreactivity
was present in all but one solitary fibrous tumor (96%), seven of seven
neurofibromas (100%), three of three spindle cell lipomas (100%), five
of five dermatofibrosarcomas (100%), three of three hemangiopericytomas
(100%) and two of seven malignant fibrous histiocytomas (29%). To date,
most of the pleural and extrapleural cases have not shown aggressive features.
We suggest that bcl-2 protein can be used together with CD34 in the diagnosis
of solitary fibrous tumor to distinguish this entity from other spindle
Chest 1997 Nov 5;112(5):1424-8
tumor of the pleura: a report of five cases diagnosed by transthoracic
cutting needle biopsy.
Weynand B, Noel H, Goncette
L, Noirhomme P, Collard P Department of Pathology, Universite Catholique
de Louvain, Cliniques Universitaires Saint-Luc, Brussels, Belgium.
Five patients had a solitary
fibrous tumor of the pleura; a well-known but rare entity. In all cases,
biopsy by a transthoracic cutting needle (Tru-Cut; Travenol; Deerfield,
IL) yielded specimens adequate for histologic analysis and gave the clue
to the diagnosis. In four patients, surgical resection confirmed the diagnosis.
The opportunity for and interest in diagnosing these tumors by transthoracic
cutting needle biopsy before surgery are discussed. An accurate diagnosis
of solitary fibrous tumors of the pleura can be made by a minimally invasive
procedure; this allows for a more informed allocation of surgical resources.
Cancer 1997 Apr 25;81(2):116-21
Solitary fibrous tumor. A
cytologic-histologic study with clinical, radiologic, and immunohistochemical
Ali SZ, Hoon V, Hoda S, Heelan
R, Zakowski MF Department of Pathology (Cytopathology), Johns Hopkins
Hospital, Baltimore, Maryland, USA.
BACKGROUND: Solitary fibrous
tumors (SFT) are rare neoplasms that most commonly involve the pleura,
mediastinum, and lung. They are believed to be submesothelial in origin.
Histologically, they are characterized by fibroblast-like cells and connective
tissue in varying proportions. The "patternless pattern" and
the hemangiopericytoma-like pattern are the most common arrangements.
The majority of SFTs have been immunoreactive for CD34. Very little has
been reported regarding the cytologic findings in these tumors. METHODS:
The authors reviewed the radiographic findings and studied fine-needle
aspiration biopsies performed on seven patients with SFT of the pleura,
and examined subsequent histologic material. The cell blocks or smears
of all cases were stained with a monoclonal antibody to CD34. RESULTS:
The cytologic preparations showed varying degrees of cellularity. Smears
were comprised of spindled cells in a bloody background with small amounts
of collagen. The three malignant tumors had a greater number of cells,
both dispersed and in clusters, with nuclear pleomorphism and prominent
nucleoli. The cell blocks characteristically showed varying degrees of
cellularity with thickened blood vessels and a hemangiopericytoma-like
pattern. The cells from the four benign tumors tended to be smaller in
size and grouped in more cohesive clusters than the malignant ones. CD34
stained the spindle tumor cells in the cell blocks or smears from five
cases; one malignant and one benign case failed to stain. The corresponding
histologic samples from these cases demonstrated the same CD34 staining
pattern in all cases except one. CONCLUSIONS: The differential diagnosis
of SFT includes neurogenic tumors, mesotheliomas, sarcomatoid carcinoma,
synovial sarcoma, hemangiopericytoma, and fibrosarcoma. CD34, when present,
may prove equally helpful as a positive marker in cytology and in histologic
preparations in the differential diagnosis of SFT
Am J Clin Pathol 1996
tumor of the soft tissue. An immunohistochemical and ultrastructural study.
Hasegawa T, Hirose T, Seki
K, Yang P, Sano T First Department of Pathology, University of Tokushima
School of Medicine, Japan.
Solitary fibrous tumor is
a rare spindle cell neoplasm of adults that usually arises in the pleura,
recently reported in other locations. The authors describe three cases
of solitary fibrous tumors in adults that occurred as circumscribed masses
in the somatic soft tissue, including the arm, back, and abdomen. Histologically,
they were characterized by a proliferation of spindle cells separated
by thick bands of collagen and prominent vascularity often showing a hemangiopericytoma-like
pattern. The spindle cells, having low mitotic figures and little nuclear
atypicality, exhibited a variety of growth patterns, including storiform,
fascicular and herringbone, and nuclear palisading. Vimentin and CD34
immunoreactivities were observed in many spindle cells of all tumors.
They had ultrastructural features of fibroblast and myofibroblast in two
cases examined. Solitary fibrous tumors seem to represent distinct mesenchymal
neoplasms that require us to identify their unusual location other than
the pleura and be familiar with their histologic appearances for arriving
at the correct diagnosis.
Acta Chir Belg 1995 May-Jun;95(3):127-9
Martens B, Bonnet P, Duquesne
L Department of Vascular thoracic surgery, Centre hospitalier Soignies-Braine-le-Comte,
The Solitary Fibrous Tumour
of Pleura has rarely been observed. A new case has been reported. This
tumour is often asymptomatic and mostly discovered by chance. A well delimited
and round opacity is demonstrated on the chest X-ray. computerized tomography
confirms the fleshy nature of this tumour with inhomogeneous enhancement.
The treatment is a complete surgical excision. The prognosis is excellent,
if diagnosed early.
Hum Pathol 1995 Apr;26(4):440-9
tumor: histological and immunohistochemical spectrum of benign and malignant
variants presenting at different sites.
Hanau CA, Miettinen M Department
of Pathology and Cell Biology, Jefferson Medical College of Thomas Jefferson
University, Philadelphia, PA 19107, USA.
Twenty-nine tumors (from
26 patients, including two with recurrent disease) diagnosed as solitary
fibrous tumor (SFT) of the pleura (n = 23), mediastinum (n = 4), abdominal
cavity (n = 1), and parotid gland (n = 1) were studied immunohistochemically.
Three histologically malignant tumors showed areas of high cellularity
and mitotic activity (more than 4 mitoses/10 high-power fields) with features
resembling malignant fibrous histiocytoma, malignant hemangiopericytoma,
or fibrosarcoma, together with areas typical of benign solitary fibrous
tumor. Formaldehyde-fixed, paraffin-embedded tissues and avidin-biotin-complex
immunostaining were used. All of the tumors showed vimentin positivity
and did not stain for cytokeratin, glial fibrillary acidic protein, or
muscle cell markers, except for focal desmin reactivity in seven tumors,
mostly seen in frozen sections, and focal keratin reactivity in one histologically
malignant tumor. The neoplastic cells were positive for CD34 and negative
for CD31; these patterns also were seen in the three histologically malignant
cases. In nine of the cases acetone-fixed frozen sections showed variable
focal positivity for neurofilament proteins of 68 kd. We conclude that
SFT is a neoplasm of fibroblasts/primitive mesenchymal cells with features
of multidirectional differentiation. We also report the finding of a novel
site for SFT, the parotid gland.
Hum Pathol 1995 Apr;26(4):428-31
CD-34 and keratin
expression distinguishes solitary fibrous tumor (fibrous mesothelioma)
of pleura from desmoplastic mesothelioma.
Flint A, Weiss SW Department
of Pathology, University of Michigan School of Medicine, Ann Arbor, USA.
Solitary fibrous tumors (SFTs)
often involve the pleura and also may encompass the peritoneum and nonserosal
sites. On occasion SFTs mimics other neoplasms, including desmoplastic
mesothelioma. CD-34, initially characterized as a hematopoietic progenitor
cell antigen, recently has been identified in a small number of SFTs.
Based on this observation, we compared the keratin, vimentin, and CD-34
expression of 19 SFTs and eight desmoplastic mesotheliomas. Fifteen of
19 SFTs (78.9%) expressed CD-34, whereas keratin expression was absent
in all SFTs. In contrast, none of the desmoplastic mesotheliomas expressed
CD-34 and keratin expression was found in seven of eight (87.5%). Vimentin
expression was noted in 18 of 19 SFTs and in seven of eight desmoplastic
mesotheliomas. We conclude that CD-34 expression distinguishes SFT from
desmoplastic mesothelioma. Additionally, the results of our study support
the idea that SFT is not derived from or related to conventional mesothelium.