THE ROLE OF ULTRASONOGRAPHY IN THE DIAGNOSIS OF MALIGNANT

SOLITARY FIBROUS TUMOR

dr. Anggita Putri Kantilaras , SpRad

Radiology Department Faculty of Medicine and Health Science Universitas Muhammadiyah Yogyakarta

Solitary Fibrous Tumor (SFT) is a rare tumor derived from cells that resemble primitive
fibroblasts on connective tissue. SFT components show a mix of connective tissue, cells and
hypervascularization area that consists of many various sizes of blood vessels. Incidence of SFT
is far more frequent in adults age 40-501 and there is no gender predilection1,2 . SFT can related
with hypoglycemia secondary to production insulin like growth factor, osteoarthropathy and
arthralgia.
SFT can be found in the head (mostly in orbita), chest wall, mediastinum, pericardium,
retroperitoneum, abdominal cavity or in rare sites such as meninges, salivary glands, adrenal,
bladder or testis.2 Forty percent case of SFT is found in the subcutaneous layer.3 Although SFT is
a benign tumor because it is a well-defined soft-tissue tumor, 10-15 percent of SFT will have
recurrence and or metastasis.2 According to Thigh Dae Ho Kim, et al, SFT located in the
extremities, especially in the legs have a high possibility of developing malignancy1 .
Ultrasound plays an important role in the diagnosis of a soft-tissue tumor.4 Radiologists
are expected to give useful information for clinician when the diagnosis is made, radiologists can
determine characterization of tumor lesion that can classify the tumor as benign or malignant.4,5
Correct classification is very important because it can help clinician to make decision and allow
optimal treatment for the patient.5

Case Report

A 27-year-old woman come to the Surgical Clinic of PKU Muhamadiyah Hospital

Gamping Yogyakarta on 26 January 2017 with a lump in her left upper thigh. The lump is initially
soft and growth since 10 months ago with the size of an egg. Because the patient feels the pain and
the lump is getting bigger, the patient go to the hospital. Physical examination shows a lump that
is hard in palpation and immobile. Clinicians suspect the patient with clinical diagnosis of
aneurysm, therefore the clinicians send the patient to Radiologist for ultrasound examination.
Ultrasound examination shows an isoechoic solid lesion in the subcutaneous femoral region,
measuring 7 cm x 6 cm with well-defined margin that deviates the femoral artery and vein inward
towards the medial region and the distance is 1.37 cm from the left femoral artery and vein.

Figure 1. Inhomogenous hypoechoic solid lesion in soft tissue of the left femur region with
intrauterine hyperechoic component. Mass invasion appears to m. rectus femoris by forming an obtuse
angle . Color flow mapping obtained hypervascularization in the hyperechoic area.

Performed on the side of the tumor the next day, looked solid mass with some fat
components with less diameter more 15 cm. Histopathological examination with san IHC
(immunohistochemistry) CD 34 and CD 31 showed malignant SFT with differential diagnosis of
hemangiopericytom a epitheloid he mangioendothelioma maligna. In microscopic examination, a
very cellular tumor cell is found that is composed of cells with round cell nuclei to spindles ,
granular core chromatin, partially solid, partially loose tumors that surround the rooms adjacent to
endothelial cells and found some mitosis.
Figure 2. The patient's macroscopic tissue . Solid yellow partial tumor with some fat components .

Discussion

In a gnosis, soft-tissue tumors based on ultrasound have limitations. 6 Plain radiography

is required to rule out the possibility of bone tumor diagnosis in any soft tissue mass . 6 , 7 In addition,
plain photographs are needed to see whether or not soft tissue mass infiltration to bone suggests
malignancy. 6 , 7 In this case, a plain photograph should be performed to see whether or not there is
mass infiltration to the bone because of a fixed lump in the underlying tissue.
Ultrasound in this case is instrumental in determining the extent, consistency,
vascularity pattern of the lesion and the presence or absence of invasion of the lesion to the bone.
4
The soft tissue tumor assessment algorithm with ultrasound includes: (1) Clinical and physical
examination of the patient, (2) Location and characterization of the tumor origin structure, (3)
Evaluation of gray scale image of the tumor, (4) Vascularity of the lesion, (5) Dynamic
examination , and (6) Advice given when ultrasound soft tissue mass can not be determined benign
or malignant. 4
Clinical and physical examinations provide important information for radiologists. The
long-standing soft tissue mass with relatively settled size leads to benign tumors. 4 When found in
children, the most likely diagnosis of osis is hemangioma or lymphangioma. 7
The history of
previous trauma leads to a picture of a hematoma or seroma. 7 However, according to George A
(2012) 21% of malignant soft tissue mass or sarcoma has a history of trauma at the site of the
tumor. 8.9
Patients in this case have no history of trauma, thus excluding the possibility of a
diagnosis of hematoma or seroma.
Patients in this case complain of pain due to a growing lump. Pain complaints are a
possible indicator of soft tissue sarcoma 4,5,7.
Spreading pain with emphasis leads to neurogenic
tumors. In addition, a lesion size of more than 5 cm also leads to a possible diagnosis of soft tissue
sarcoma. The size of the lesion in this case is 7 cm x 6 cm so that it leads to sarcoma. NICE
Guideline ( National Institute for Health and Care Excellence ) and ESMO ( European Society for
Medical Oncology ) uses a 5 cm size limit as lesions lead to malignancy, while some other
institutions use 3-5 cm constraints. 10
In identifying a tumor with ultrasound, radiologists need to mention the location of tumors
in subcutaneous, intramuscular, intermuscular, originating from nerves or blood vessels,
juxtaarticular or attached to tendo wrappers. 1 1 The superficial location refers to the subcutaneous
tissue and the skin outside the superficial layer that concerns the fascia that separates the
subcutaneous tissue with the muscle therein. Soft tissue tumors located in the inner layer (deep
soft tissue tumor), ekogenitas heterogeneous tumors, internal bleeding or necrosis leading to
malignant tumors than benign.
4.11

While superficial tumors have some slightly different indicators. The size of the
superficial tumor is not an important indicator, since the size limitations considered significant for
superficial tumors are less than 5 cm. 8.11 The presence of fascia edema, skin thickening, bleeding
and necrosis are significant factors leading to malignancy. 8.11
Peritumoral lobulation and edema
are also significant factors leading to malignancy. A blunt angle formed between the fascia with
the mass infiltrating the fascia shows the malignant mass. Malignant tumors have a tendency to
stick with the fascia rather than benign tumors .

Figure 3. Schematic image of tumor relationship with fascia. The tumor is not about the fascia (group 1) or minimal
contact with the fascia (group 2) leads to a benign tumor profile, whereas the tumor with the invasion of the fascia and
the obtuse angle (group 4) and invasion in the tumor to the fascia (kel 5) leads to malignance . 8.11

The tumor in this case lies superficially measuring 7 cm x 6 cm. As has been slightly
discussed in the above description, tumor size exceeding 5 cm leads to a diagnosis of sarcoma.
However, according to George et al size in superficial tumors is not an important indicator, since
the size limitations considered significant for superficial tumors are less than 5 cm. Of the various
malignant indicators mentioned above, the presence of necrosis in this case is an indicator of
malignancy. In color flow mapping examination in this case, hypervascularization is only found
in the components of hyperechoic lesions. The absence of vascularization in the hypoechoic lesion
component suggests a process of necrosis in the area.
Gray scale picture of the tumor is also instrumental in the approach to diagnosis of soft
tissue tumors. Intralesi erogenity, lesion form, edges and presence or absence of calcification help
lead to differential diagnosis. Gray scale appearance of a lesion will distinguish a cystic or solid
tumor. The SFT ultrasound picture is generally a hypoechoic mass that can sometimes be
heterogeneous. Ultrasound in this case shows an inhomogenous hypoechoic solid lesion.
Echogenitas lesions associated with many at least the content of collagen. Echogenitas increased
in lesions with a considerable amount of collagen. While the inhomogeneity of the lesions is
associated with many at least microsoid degeneration that will appear on histopathologic
examination.
Lenticular or flat lesions tend to lead to benign tumors. Most soft- tissue tumors have firm
limits. According to Ginat et al , the presence or absence of calcification in SFT does not
distinguish lesions benign or malignant, as both can be found calcification of benign and malignant
tumors. Calcification will be more clearly seen on ultrasound examination than MRI. Calcification
is not found in ultrasound in this case.
Dynamic examination is done by pressing the transducer to distinguish a cystic or solid
lesion. Sonopalpation can show the internal movement of echo showing a cystic lesion leading to
abscess. Although compression may be helpful to distinguish cystic or solid lesions, current
elastographic examinations are considered less likely to provide information needed by clinicians
than on gray scale scales from ultrasound.
Reporting of SFT ultrasound examinations should be able to provide descriptions of
lesions, diagnoses and even recommended treatments to the clinician. However, SFT diagnosis
based only on ultrasound findings has many limitations. Therefore, clinical findings, laboratory
data, additional findings of other modalities are indispensable.
Histopathological examination for SFT diagnosis requires immunohistochemical testing
CD 34 and CD 31 . According to England et al , the determination of benign or malignant tumors is
based on 4 criteria of malignant standards, namely (1) high mitotic activity (> 4 mitosis / 10 field
of view), (2) hyperelularity with solid cell nuclei and overlapping each other, (3) ) The presence
of necrosis, and (4) the discovery of pleomorphic cells. Histopathologic examination in this case,
in accordance with the above criteria of malignancy, namely hiperseluler tumor cells, overlapping
cell nuclei that show high mitotic activity and the discovery of pleomorphic cells.
Treatment of lesions by clinicians plays an important role, because incomplete tumor resection
will facilitate a recurrence of tumor. It is as proposed by Wignall et al, that extrathoracal SFT has
a significantly higher recurrence rate, especially if tumor resection is incomplete. According to ...
.. , factors affecting the recurrence of a tumor include (1) Extrathoracal location (especially
extremities), (2) Invasion of the lesion into surrounding structures making it difficult for
resection, (3) tumor size> 10 cm, and (4) maligna on histopathologic examination.

Long-term follow-up is necessary in this case, because according to Wignall et al , SFTs are
often found with clinical behavior that is incompatible with histopathologic features. SFT
management requires a multidisciplinary team of Orthopedic surgery, Radiologist and
Anatomical Pathologist. Any findings of soft-tissue tumors need to be evaluated for clinical,
imaging , and biopsy reports that will determine future action.