Solitary fibrous tumors of the head and neck: a clinicopathologic and
radiologic review.Arch
Otolaryngol Head Neck Surg. 2006 May;132(5):517-25.
OBJECTIVE: To
describe the clinicopathologic and radiologic features of solitary
fibrous tumors of the head and neck. DESIGN: Retrospective analysis.
SETTING: Tertiary referral center that performs head and neck surgical
oncology. PATIENTS: Twelve patients with solitary fibrous tumors of
the head and neck identified from the pathology and soft tissue tumor
databases at Memorial Sloan-Kettering Cancer Center, New York, NY,
from 1990 to 2004. All cases were reviewed by 3 experienced
pathologists, 1 of whom is an experienced soft tissue tumor
pathologist. The diagnosis was confirmed by microscopic features on
hematoxylin-eosin staining and by positive staining for CD34 and Bcl2
on immunohistochemical analysis. Tumors were scored for mitotic
activity, cellularity, nuclear pleomorphism, necrosis, and the
presence of a malignant component. Details on patient characteristics,
tumor characteristics, previous treatment and surgery, adjuvant
treatment, and outcome were recorded from clinical records. RESULTS:
Solitary fibrous tumors occurred in patients over a wide age range
(27-78 years; median age, 52 years). Seven patients (58%) were women,
and 5 (42%) were men. Most tumors presented as a slow-growing painless
mass with a duration ranging from 2 months to 5 years. The tumors
ranged from 1 x 1 cm to 6 x 5 cm. Patients presented with a
subcutaneous mass of the scalp or face in 4 cases, intraoral mass in
4, sinonasal mass in 3, and paraspinal mass in 1. Computed tomographic
and/or magnetic resonance imaging scans of 7 of the 12 patients showed
well-circumscribed tumors that enhanced strongly with contrast.
Treatment for all of the patients was surgical resection. Pathologic
findings showed that 9 tumors were benign and 3 were malignant. Three
patients had a positive surgical resection margin. All patients were
alive at a median follow-up of 8 months (range, 1-76 months). Local
recurrence occurred in 1 patient who had positive surgical margins 3
years after the initial surgery. CONCLUSIONS: Solitary fibrous tumors
of the head and neck region are rare and most commonly benign. The
diagnosis depends on microscopic and immunohistochemical features,
although imaging may help. Patients with these tumors can be safely
treated with local excision, but tumors with positive margins require
close follow-up over several years owing to the potential for late
local recurrence.
Because of the occasional aggressive
behavior of this tumour, it should be considered potentially malignant.
The recurrence rate is very low after surgical resection, with a slight
increased risk of recurrence in the extrathoracic tumours.
Age:
Usually occurs in middle
aged adults between 20 and 70 years. The lesion may rarely occur in
adults.
Sites:
This tumour
has been reported in extrapleural sites, including the orbit, liver,
salivary glands, tongue, nose, paranasal sinuses, larynx, thoracic wall,
mediastinum, retroperitoneum, thyroid , gastrointestinal tract,
hypoglossal nerve & breast.
Other locations include adrenal, urinary bladder, prostate, spermatic
cord, testes, meninges, spinal cord and periosteum.
Clinical presentation:
This is
a well circumscribed slow growing painless tumour.
Clinical symptoms depend on the size and location of the tumor, where
symptoms are usually related to compression rather than infiltration of
the adjacent tissue.
Solitary fibrous tumour most often manifests as a
benign lesion; however, malignant transformation and metastasis have been
reported in a number of both pleural and extrapleural tumours.
Gross features:
Well circumscribed, lobulated, partially
encapsulated tumour.
The size of the tumour ranges from 1 to 25 cms in
diameter.
The tumour has a tan
white, firm, homogenous cut surface.
The solitary fibrous
tumors are composed of relatively uniform proliferating
ovoid or spindle-shaped cells that are arranged haphazardly, often in a
fascicular and whorled pattern,
in a heavily collagenized stroma.
Often there are abundant
blood vessels arranged in a hemangiopericytoma-like pattern .
The degree
of cellularity varies from area to area and in less cellular areas the
intercellular collagen is often thick and keloid-like.
Some tumours show
compact clusters of polygonal cells with a rather epithelioid appearance.
Mitotic activity is
generally low, ranging from 0 to 2/10 HPFs. Focal areas of necrosis are
identified in some cases.
Stromal cystic
degeneration and prominent myxoid change (Myxoid Solitary Fibrous Tumour)
are also present in some cases.
Prognostic factors for a
malignant potential include high cellularity, mitotic activity (>4 per 10
high-power fields), pleomorphism, extensive hemorrhage, and necrosis.
Also, tumours larger than 10 cm should be closely monitored.
Immunohistochemically
there is characteristic expression of vimentin, CD34, bcl-2, and CD99.
Factor XIIIa and alpha-smooth muscle actin are less commonly reactive and
a very few cells may be faintly positive for factor VIII-related antigen
and Ulex europaeus agglutinin 1.
All cases are essentially negative
for S-100 protein, desmin, CD31, and CD68.
Differential diagnosis:
Solitary
fibrous tumour may show a variety of growth patterns which may include
fascicular areas, storiform and herringbone formations and wavy
neurofibroma-like and monophasic synovial sarcoma-like areas in the solid
spindle cell component, thus simulating a variety of soft tissue neoplasms.
Solitary fibrous tumour of the soft tissue of the face: a case report.B-ENT 2006;2(4):201-4.
INTRODUCTION: Solitary fibrous tumour (SFT) is a rare mesenchymal
neoplasm described first in the pleura. Recently, SFTs have been found
in various extra-serosal locations, including the head and neck
region. CASE PRESENTATION: We report a case of SFT originating from
the periorbital region of the face. Preoperative cytological
examination by fine needle aspiration biopsy diagnosed a mesenchymal
tumour. The patient underwent surgical resection. The mass was
completely resected. Definitive histopathologic and immunohistologic
examination confirmed the diagnosis of SFT. DISCUSSION: The rare
localisation in extrapleural sites and the multiplicity of
histological patterns can explain the difficulty in arriving at a
definitive diagnosis in SFT. Usually, SFT is a benign tumour, although
malignant variants exist. Clinical behaviour is unpredictable and
recurrence or malignant transformation can also occur, especially in
cases with macroscopically or microscopically invaded margins.
Complete surgical excision and long follow-up is therefore always
recommended. The recent increase in reports of extrapleural SFT
indicates that this rare tumour should be included in the differential
diagnosis of soft tissue head and neck tumours.
Solitary fibrous
tumour of the face: a rare case report.Br
J Plast Surg. 2002 Jan;55(1):75-7.
Solitary
fibrous tumour is a rare mesenchymal neoplasm that most commonly
involves the pleura. The diagnosis of solitary fibrous tumour is
primarily histological. It consists of histological and positive
immunohistochemical findings of CD-34 and vimentin. Recently, solitary
fibrous tumour has been reported to occur in extrapleural soft
tissues, such as the orbit, nasal cavity, abdominal cavity, parotid
gland, scalp and neck. In an extensive review of the literature, we
found no reports of solitary fibrous tumour arising in the facial soft
tissue, other than in the parotid gland. This rare location of an
uncommon lesion can lead to a confusing diagnosis. We report a case of
solitary fibrous tumour originating in the temporal region of the
face, and call for awareness of this tumour among plastic surgeons.
