21 December 2020

Undifferentiated pleomorfic
Sarcoma
Oncology AY/RE

MOD: MX

SUPV: dr. M. Phetrus Johan, Ph.D, Sp.OT (K)

BAGIAN ORTOPEDI & TRAUMATOLOGI FAKULTAS KEDOKTERAN UNHAS MAKASSAR

 CASE 1
A 16-years-old male
patient, reported with the
complaint of swelling,
rapidly growing in nature,
since last 1½ months
which was present at the
left angle of the mandible
Datarkar et al. 2009. MFH case report. J Maxillofac
Oral Surg 8(2):196–198.
HISTORY TAKING
HISTORY OF PRESENT COMPLAIN
 The patient’s history revealed
pain in the lower left molar
region 1½ months ago for
which he consulted the
dentist. The swelling started
enlarging rapidly in relation to
the mandibular wisdom tooth
and ramus of the mandible.
 The increase in size was
associated with intense pain, and
numbness of the lower lip. The
mass was not fixed to the
adjacent soft tissues. Regional
lymphadenopathy was not
 evident. Medication in the form of
analgesics and antibiotics was
given. The patient was then
referred to our centre
Datarkar et al. 2009. MFH case report. J Maxillofac
Oral Surg 8(2):196–198.
 Radiographic examination
demonstrated a mixed radio
opaque and radiolucent lesion
which was poorly demarcated.
Destruction of the bone was
seen in the ramus perforating
the cortical plate, extending to
the angle and body of the
mandible (Fig. 2).
Datarkar et al. 2009. MFH case report. J Maxillofac
Oral Surg 8(2):196–198.
PHYSICAL EXAMINATION
 Hemi-mandibulectomy
with disarticulation
was performed with en
bloc removal of the
medial and lateral
contiguous soft tissue
Datarkar et al. 2009. MFH case report. J Maxillofac
Oral Surg 8(2):196–198.
 The tumor contains both fibroblast like and histiocyte like cells
in varying proportions with spindled and rounded cells and
numerous large bizarre tumor giant cells
Datarkar et al. 2009. MFH case report. J Maxillofac
Oral Surg 8(2):196–198.
 follow-up 18 months.
 recurrence after 36 months.
 CT scan of the patient revealed tumour spread
to infratemporal fossa and base of the skull.
 There was no metastasis other than the head
and neck region.
 kept on palliative therapy and passed away after
3 months
Datarkar et al. 2009. MFH case report. J Maxillofac
Oral Surg 8(2):196–198.
 CASE 2
 72-year-old woman, with a previous history of
hypertension, presented in the emergency
room complaining of significant pain and
functional disability of her right upper limb,
following direct trauma after a fall from her
own height,
Costa et al., Bone Undifferentiated Pleomorphic
Sarcoma–A Case Report. J Clin Case Rep 2018, 8:3
a two-part Neer proximal humeral
fracture
Costa et al., Bone Undifferentiated Pleomorphic
Sarcoma–A Case Report. J Clin Case Rep 2018, 8:3
Costa et al., Bone Undifferentiated Pleomorphic
Sarcoma–A Case Report. J Clin Case Rep 2018, 8:3
 postoperative period, despite intensive
physiotherapy, the patient-maintained pain
and swelling of the shoulder, with no
associated inflammatory signs or incision
drainage.

Costa et al., Bone Undifferentiated Pleomorphic

Sarcoma–A Case Report. J Clin Case Rep 2018, 8:3
 Two months later, an
articular CT scan showed
a poorly delimited
periarticular soft tissue
collection involving the
articular space, not
previously present

Costa et al., Bone Undifferentiated Pleomorphic

Sarcoma–A Case Report. J Clin Case Rep 2018, 8:3
 At that time, with the suspected shoulder
collection, the patient underwent an
exploratory arthrotomy, debridement and
periarticular tissue biopsy, being the main
finding an extensive hematoma

Costa et al., Bone Undifferentiated Pleomorphic

Sarcoma–A Case Report. J Clin Case Rep 2018, 8:3
 After this second
procedure, shoulder pain
and swelling intensified,
progressing to a massive
edema involving the
entire upper limb
Costa et al., Bone Undifferentiated Pleomorphic
Sarcoma–A Case Report. J Clin Case Rep 2018, 8:3
 The postoperative chest CT angiogram,
beyond the pulmonary embolism area,
showed a 2 cm left lung nodule located on
the superior lobe (Figure 6) and extrinsic
compression of the right axillary vein by
the shoulder soft tissue collection.

 These results arose the suspicion of a
neoplastic etiology, but the workup that
followed failed to identify any other
primary location
Costa et al., Bone Undifferentiated Pleomorphic
Sarcoma–A Case Report. J Clin Case Rep 2018, 8:3
 The PET scan
showed an intense
glycolytic
metabolism in the
right shoulder

Costa et al., Bone Undifferentiated Pleomorphic

Sarcoma–A Case Report. J Clin Case Rep 2018, 8:3
 Bronchofibroscopy and transthoracic needle biopsy of
the lung were normal.
 The periarticular tissue biopsy report described a
highly cellular smear, with pleomorphic spindle cells
and scattered mitosis; associated multinucleated
giant cells were present; but necrosis was not - these
aspects supported an UPS diagnosis.
Costa et al., Bone Undifferentiated Pleomorphic
Sarcoma–A Case Report. J Clin Case Rep 2018, 8:3
One month later, during the hospital stay, the restaging CT scan revealed
multiple bilateral lung lesions and axillary adenopathies suggesting
disseminated disease . The patient was proposed for palliative measures
Costa et al., Bone Undifferentiated Pleomorphic
and died a few days later. Sarcoma–A Case Report. J Clin Case Rep 2018, 8:3
introduction
 Malignant fibrous histiocytoma (MFH), a type
of sarcoma, is a malignant neoplasm of
uncertain origin that arises both in soft tissue
and bone.
 1961: by Kauffman and Stout : They described
MFH as a tumor rich in histiocytes with a 
storiform growth pattern.
introduction
 1977, MFH was considered the most common
soft tissue sarcoma of adult life.
 2002, the World Health Organization (WHO)
declassified MFH as a formal diagnostic entity
and renamed it as an undifferentiated
pleomorphic sarcoma not otherwise
specified, NOS
Epidemiology
 It is the most common soft-tissue sarcoma in
adults 55 to 80 years of age
 Male-to-female ratio = 2:1 4
 More common in Caucasian than in African
American or Asian populations.

AAOS comprehensive 2008

 Genetics/etiology—Expression of PD-1 in 30%
of undifferentiated pleomorphic sarcoma in
small series

AAOS comprehensive 2008

Location
orthobullets
Clinical Symptoms
 Usually a deep, slow-growing, painless mass.
 More common in the extremities (lower more common than
upper) than retroperitoneum.
 Patients occasionally present with fever, elevated white blood
cell count, and hypoglycemia.

AAOS comprehensive 2008

imaging
 Imaging
 Low signal
intensity on T1-
weighted MRI;
 high signal
intensity on T2-
weighted MRI

Undifferentiated pleomorphic sarcoma in a 68-year-old man who presented with a

painless soft-tissue mass in the right posterior thigh.
AAOS comprehensive 2008
orthobullets
 Macroscopy
 Gross: a gray-white
multinodular mass

AAOS comprehensive 2008

Histopathology
 Histologic subtypes
 pleomorphic (80% to 85%)
 giant cell (10%)
 inflammatory (<10%)
 Storiform or cartwheel growth patern is seen on low-power
histologic images
 Cells are plump, spindled, and arranged around narrow vessels.
 Haphazard histiocytic cells
 Multinucleate eosinophilic giant cells
 Marked atypia, mitotic activity, and pleomorphism
AAOS comprehensive 2008
Low magnification high magnification

orthobullets
, Low-power histologic image reveals a storiform pattern with bizarre pleomorphic tumor
cells and hyalinized collagen bundles consistent with undifferentiated pleomorphic sarcoma.
AAOS comprehensive 2008
Higher power histologic image reveals anaplastic tumor cells, multinucleated cells, and
mitotic figures. AAOS comprehensive 2008
 TREATMENT
▪ Radiation and wide surgical resection
▪ 50 Gy pre-operative and 66 Gy postoperative is the standard
dose for soft-tissue sarcomas
▪ Chemotherapy in selected cases (ongoing clinical trial with
pembrolizumab and neoadjuvant radiation)

AAOS comprehensive 2008

 prognosis
▪ Overall 5-year survival of 50% to 60% (depending on size,
grade, depth, presence of metastasis)

AAOS comprehensive 2008

Q
Thank You