SPC
骨科 住院醫師 楊碩文
骨科 李建和 主任
Basic Data

劉 先生 64 y/o
Chief Complain :
Palpable a solid painless mass over right
leg posterior aspect for 3 years and the
mass rapid enlargement with pain for last
2 weeks
Present illness





Denied any systemic disease
Old open fracture of right tibia and fibula s/p
external fixation 40+ years ago (fracture healed
well)
Sustained a solid painless mass about one egg
size over right posterior leg for about 3 years
No traumatic history at lesion area or infection
sign and discharge sinus
The mass rapidly enlarged with pain and tender
for last 2 weeks
Physical Examination


A solid, fixed, tumor mass with local
tenderness over right calf muscle about
10x8x6 cm in size
Right ankle weakness of dorsiflexion ,no
sensory disorder
Lab Data
WBC
RBC
8.93 10^3/uL [4.00-11.00]
4.17 10^6/uL [4.20-6.10]
Glucose(血液)
118 mg/dl [70-99]
BUN (血液)
16.2 mg/dl [6.0-20.0]
HGB
14.3 g/dL [12.0-18.0]
Creatinine(血)
0.9 mg/dl [0.5-1.2]
HCT
41.4 % [37.0-52.0]
eGFR
90 mL/min/1.73M2
MCV
99.3 fL [80.0-99.0]
GOT(血液)
43 IU/L [<37]
MCH
34.3 pg [26.0-34.0]
CRP (血液)
1.40 mg/dL [<0.5]
MCHC
34.5 g/dL [33.0-37.0]
Na (血液)
RDW
12.7 % [11.5-14.5]
K (血液)
PLT
316 x10^3 /uL [130-400]
MPV
8.90 fL [7.20-11.10]
RDW-SD 45.8 fL
PDW
9.5 fL
140 mEq/L [136-145]
3.2 mEq/L [3.5-5.1]
Impression


r/o Soft tissue malignant tumor of right
leg
Imaging studies




Plain radiographs of the affected area
MRI with contrast
Chest CT scan
Biopsy was arranged and done on 100/07/25
Leiomyosarcoma of
extremity
Leiomyosarcoma of Soft Tissue



Arise directly from the smooth muscle cells
lining small blood vessels
Extremely rare occurrence
Most malignant leiomyosarcomas arise
independently, and are not associated
with benign tumors
Leiomyosarcoma of Cutaneous
Origin



Man : women = 2:1
First diagnosed (1-2 cm), and prognosis is
generally good
Deeper lesions can metastasize in up to
30-40% of cases, usually hematogenously
to the lungs
American Joint Committee on
Cancer(AJCC) Staging System
Histological
Stage
Size
Grade
Location
(Relative to
fascia)
Systemic /
Metastatic
Disease Present
IA
Low
< 5cm Superficial or Deep
No
IB
Low
≥ 5cm Superficial
No
IIA
Low
≥ 5cm Deep
No
IIB
High
< 5cm Superficial or Deep
No
IIC
High
≥ 5cm Superficial
No
III
High
≥ 5cm Deep
No
IV
Any
Any
Yes
Any
Treatment

Surgery



Radiation Therapy



Achieving wide surgical margins is important in
preventing local recurrence
In this case: rescetable mass wild rescetion
Pre-operatively (neoadjuvant) or post-operatively
(adjuvant):
In this case: margin free, no consider R/T
Chemotherapy


Treatment of metastatic disease
In this case: no sign of metastasis, no consider C/T
Prognosis: Soft Tissue
Leiomyosarcoma

worse prognosis :


age >62 years, size >4cm, tumor necrosis,
vascular invasion, or previous intralesional
surgery
50% 3-year survival to 64% 5-year
survival
Prognosis: Cutaneous
Leiomyosarcoma


True intradermal leiomyosarcoma is
thought not to metastatsize
Wide excision of truly intradermal tumors,
if achievable, is curative
Bone scan
CT pre- & post-C
STIR
T1WI
STIR
T2WI
AT
PT
EDL
PL
Gd-T1WI
Soleus
GN
No pulmonary meta.
case I 劉x慶 11806 xxx
T11-9233, 9263
Frozen section and biopsy
Soft tissue, calf, right, intra-operative biopsy,
spindle cell tumor, favor sarcoma with myogenic
differentiation
T11-9983
Soft tissue, leg, right, wide excision, leiomyosarcoma
Frozen section: one tissue fragment measuring 1.6 x 0.7 x 0.7 cm in size
neoplastic spindle cells arranged in sheet and fascicular pattern with lymphocyte
spindle cell tumor, favoring sarcoma
neoplastic spindle cells arranged in sheet and fascicular pattern with areas of necrosis
Neoplastic spindle cells with pleomorphic and blunt-ended nuclei
A piece of skin 11.8 x 4.3 cm. One surgical scar measuring 2.5 cm in length
One part of calf muscle: 12.0 x 7.7 x 4.8 cm. and 240 gm.
with an intramuscular tumor: 4.9 x 4.7 x 3.5 cm
On section, the tumor is heterogeneous brown to yellow and soft with
focal hemorrhage and necrosis
The tumor is located intramuscularly. The overlying skin is not involved.
The tumor measures 0.4 cm, 3.8 cm, 4.0 cm, 3.9 cm and 2.2 cm away
from the lateral, medial, deep, superior and inferior section margins of the specimen
Necrosis: red area (left side), central tumor, normal muscle (right side)
Hemorrhage and necrosis
Tumor area
Hemorrhage and necrosis
Thick-walled vessel
Lymphoplasma cell
Tumor cells with
eosinophilic or clear
cytoplasm
Tumor cells with eosinophilic or clear cytoplasm and pleomorphic and
blunt-ended nuclei
Tumor cells with eosinophilic or clear cytoplasm and pleomorphic nuclei
Some lymphocyte and eosinophil infiltrate
Mitotic figure: 3/10 hpf
Atypical mitosis
Small separated tumor nodule is noted
Main tumor mass
Skin is free from the tumor
HE
Desmin (+)
Actin (+), h-caldesmon (+)
Vimentin (+)
CK (F+)
Myogenin (-)
CD31(F+), CD34 (-)
S-100, HMB45 (-)
Findings and Differential diagnosis
Gross findings: heterogeneous brown to yellow and
soft with focal hemorrhage and necrosis →
malignancy (sarcoma)
Microscopic findings:
1. Perpendicularly oriented fascicles of spindle cells
2. Brightly eopsinophilic cytoplasm
3. Blunt-ended nuclei
4. Nuclear atypia
→myogenic differentiation
Immunostudy:
(+) Vimentin, Actin, Desmin, h-caldesmon
Focal (+): CK, CD31
(-): myogenin, S-100, HMB45, CD34
Spindle cell sarcoma, with myogenic
differenitation
h-Caldesmon  leiomyosarcoma
(consultation
with Dr. Hsuan-Ying Huang in Kaohsiung CGMH)
MICROSCOPIC
Histologic Type: Leiomyosarcoma
FNCLCC (French Federation of Cancer Centers Sarcoma Group) grading
system:
Tumor differentiation:
Score 2: sarcomas of definite histologic type
Mitotic count:
Score 3: 20 or more than 20 mitoses per 10 HPF
(our case: 22/10 hpf)
Tumor necrosis:
Score 1: less than or equal to 50 % tumor necrosis
Histological grade: Grade 3: total score 6 -8
Margins: Margins negative for sarcoma
Distance of sarcoma from closest margin: 0.4 cm, lateral soft tissue
margins
Lymph-Vascular Invasion: Not identified
Pathologic Staging (pTNM) (AJCC/UICC TNM, 7th edition)
Primary Tumor (pT):
pT1b: Tumor 5 cm or less in greatest dimension, deep tumor
Regional Lymph Nodes (pN):
pNX: Regional lymph nodes cannot be assessed (not sampled)
Anatomic stage/prognostic groups: pStage IIA at least (pT1b NX MX G3)
Stage IA
Stage IB
Stage IIA
Stage IIB
Stage III
Stage IV
T1a N0, NX M0
G1, GX
T1b N0, NX M0
G1, GX
T2a N0, NX M0
G1, GX
T2b N0, NX M0
G1, GX
T1a N0, NX M0
G2, G3
T1b N0, NX M0
G2, G3
T2a N0, NX M0
G2
T2b N0, NX M0
G2
T2a, T2b N0, NX M0
G3
AnyT N1 M0
Any grade
AnyT Any1 M1
Any grade
Leiomyosarcoma - 1
1.
2.
3.
4.
5.
Definition: malignant neoplasm composed of cells
exhibiting smooth muscle differentiation
Etiology: EB virus associated in immunosuppressed
patient or associated with radiation
Incidence:
Rare: 10-15% of extremity sarcoma
(but common if including the uterine and visceral
lesions)
Age: middle-aged adults
Gender: no preference (women easily found in
retroperitoneal and inferior vena cava areas)
Leiomyosarcoma - 2
6.
Classification:
a. leiomyosarcoma of soft tissue
b. leiomyosarcoma of cutaneous origin
c. leiomyosarcoma of vascular origin
d. leiomyosarcoma in the
immunocompromised host
e. leiomyosarcoma of bone
Variant and special forms:
Myxoid leiomyosarcoma
Inflammatory leiomyosarcoma
Pleomorphic leiomyosarcoma
Leiomyosarcoma with osteoclastic-like giant cells
Epitheliod leiomyosarcoma
Immunohistochemistry for
leiomyosarcoma
•
•
•
•
•
•
•
•
•
•
Desmin
Actin-sm
Calponin
Caldesmon
CK-PAN
ER
CD34
PR
S-100
HMB-45
+
+
+
+
+
-
Leiomyosarcoma - 3
7.
8.
9.
S/S: deep soft tissue mass – often asymptomatic
a. retroperitoneal – abdominal pain
b. vena cava →
Upper portion: Budd-Chari syndrome (hepatomegaly, jaundice,
ascites)
Mid-portion: Renal obstruction
Lower portion: lower extremity edema
Treatment:
*surgical excision, radiation, chemotherapy
Prognosis: depend on site and stages of lesions
a. Restricted in cutis → essentialy never meta. As “atypical smooth
muscle tumor”
b. in subcutis: up to 1/3 meta. 10-20% die of disease
c. retroperitoneum: 80% die of disease, typical with metastasis
d. bone: up to ½ meta. 5 y survival: 65%
e. vena cava: 5y – 50%, 10y – 30% survival
f. head and neck: over ½ metastasis
北醫附醫， 萬芳醫院， 雙和醫院
(1995-2011/6)
(1998-2011/6)
(2008-2011/6)
1. leiomyoma: 8313
Uterus: 8033
age:10-96
(average: 47.3)
1. leiomyoma: 2161
Uterus: 1693
age:10-97
(average: 51.4)
1. leiomyoma: 423
Uterus: 355
age:17-82
(average: 47.6)
2. leiomyosarcoma: 39
Uterus: 20
age: 35-67
(average:52.1)
20/8033: 0.24%
2. leiomyosarcoma:12
Uterus: 3
age: 42-63
(average:51.3)
3/1693: 0.18%
2. leiomyosarcoma: 3
Uterus: 0
Non-Uterus: 23
age: 39-92 (mean: 69)
M:F: 9:14
Intraabdomen-5, extremity-5, G-I-4,
retroperitoneum-2, urinary bladder-2,
back-1, pararectum-1, cervix-1,
vagina-1, palate-1
Non-uterus: 9
age: 41-93 (mean: 58.2)
M;F: 3:6
extremity-2, retroperitoneum-2,
G-I-3, vagina-1, scrotum-1
Non-uterus: 3
age: 48-62
(mean:57.3)
M:F: 2:1
extremity-1, retroperitoneum-1,
G-I-1
SPC
100.08.26
王樂明醫師/劉偉民主任
PATIENT PROFILE
Name:楊O萍
 Chart No.: 11767181
 Gender: female
 Age: 61 years old
 Admitted data: 100.7.18

CHIEF COMPLAINT

Vaginal spotting for several weeks
PRESENT ILLNESS

This is a 61-year-old female patient, with history
of 1) duodenal ulcer, 2) myoma uteri, found 10
years ago. Her OBS/GYN history as followed:
1)G0P0AA0, 2)LMP: menopause at 55 y/o,
3)menarche at 14 years old, 4)duration for 7 days,
5)interval at about 30 days. She found vaginal
spotting recently, so she came to our OPD for
medical consultation and ultrasound showed
endometrium thickness of 13 mm with pelvic
mass closed to posterior wall (63x64x68mm) of
uterus. Under the impression of pelvic mass, she
was admitted to our ward for further surgical
intervention.
100.7.04

CA125 (血液) 12.21 U/ml [<35.00]
CBC (7/18)
 WBC:
6.75X103 /uL
 RBC: 4.36X106 /uL
 Hb: 13.2 g/dL
 MCV: 90.6 fL
 PT/aPTT: 12.2/33.2
 GOT/GPT: 17/15
 Na: 143 mEq/L
 Ca: /9.5 mg/dl
 K: 3.9 mEq/L
 Cl: 106 mEq/L
Endometrial hyperplasia:
13 mm
Pelvic mass: 83X64X68mm
Left ovary
Right ovary
CT
 1.
A fatty containing space-taking lesion
in pelvis with abutting to uterus & no
visible of ovaries ( possible due to age
status), highly suggestive of dermoid cyst
of ovary. Please correlate with clinical
information.
2. bil. renal cysts
3. Several gallstones with focal
adenomyosis of fundus
ARRANGED OPERATION

Laparoscopic assisted pelvic excision
One myomatous mass 8.0 x 7.7 x 5.8 cm in size
and 200 gm in weight.
On cut surface: white, yellow and elastic.
No hemorrhage, myxoid and cystic changes
Hyalinized vessels
Hyalinized vessels
Spindle smooth muscle
Focal myxoid background
Findings and differential diagnosis
• Gross: intrauterine tumor with white-yellow
and soft to elastic
• Micro: benign looking smooth muscle
bundles and scattered clusters or diffuse
large clear vaculated cell
• D/D:
1. angiomyolipooma
2. clear cell tumor metastasis
3. lipoleiomyma
Actin (+)
Ki 67(very low)
S-100(+)
P53 (-)
Final diagnosis
lipoleiomyoma
Lipoleiomyoma
1. A rare morphologic variant of uterine
leiomyoma characterized by the
presence of scattered islands of mature
adipocytes within the smooth muscle
neoplasm.
2. Histogenesis – controversial
3. Benign, case report: malignant
transformed
Lipoleiomyoma of the uterus is a rare condition.
• A perusal of the English literature revealed
approximately 140 cases.
• The adipose tissue of the present tumor was
free from atypia, and no lipoblasts were seen.
• p53 was negative and Ki-67 labeling was
very low. The MDM2 and CDK4, markers of
well-differentiated liposarcoma, were
negative.
The histogenesis of uterine LL is controversial.
1. Traditionally, the adipose tissue element of LL is thought to
be derived from degeneration of leiomyoma.
2. Sieinski (Int J Gynecol Pathol 8 (1989), pp. 357–363 ) and
Resta et al (Pathol Res Pract 190 (1994), pp. 378–383)
thought that LL arises from metaplasia (neometaplasia) of
immature perivascular pluripotent mesenchymal cells.
3. Lin and Hanai (Acta Pathol Jpn 41 (1991), pp. 164–169)
considered that the adipose tissue is derived from direct
metaplasia of the smooth muscle cells of leiomyoma.
4. Gentile et al (Pathologia 88 (1996), pp. 132–134)
thought that LL is derived from multipotential undifferentiated
mesenchymal cells.
5. Shintaku (Pathol Int 46 (1996), pp. 498–502) showed a case of
angiomyolipomatous LL with vascular proliferation and also
demonstrated cases of ordinary LL. He suggested that LL is a spectrum
of lipomyovascular tumors of the uterus and also suggested that adipose
tissue is derived from lipomatous metaplasia of leiomyoma.
6. Aung et al (Pathol Int 54 (2004), pp. 751–758 ) reported that 6 of 17
cases of LL showed angiomyolipoma-like vascular proliferation. They
showed that the Ki-67 labeling of smooth muscle element was 1.38%,
that of adipose tissue was 1.17%, and that of normal myometrium was
0.76%. They thought that both elements are proliferative lesions rather
than fatty degeneration. They also found that HMB45 was positive of
certain LLs. In the present tumor also, the Ki-67–positive cells were seen
in both elements; but HMB45 was negative. In the present study, Ki-67–
positive cells are seen in 0.2% to 0.3% of both adipose tissue and
muscular elements, suggesting that both elements have the capacity for
cell proliferation and that both the adipose tissue and leiomyomatous
tissue components in the present LL are neoplastic.
7. Uterine LL may be associated with metabolic diseases including
hyperlipidemia, hypothyroidism, and diabetes mellitus
(Int J Gynecol Obstet 67 (1999), pp. 47–49 ) This suggests that
changes in lipid metabolism after menopausal transition may play a
role in the development of LLs. The current case did not show
hypercholesterolemia, hypertriglycerolemia, and hyperglucosemia.
8. In the present case, hemoglobin A1c was normal; and no other
metabolic diseases including hypothyroidism were recognized. The
relationship between metabolic diseases and development of uterine
LL remains to be elucidated.
In large studies, 18.8% of patients with the uterine LLs were
associated with gynecologic malignancies that may originate from the
uterus, cervix, or ovaries
(Pathol Int 54 (2004), pp. 751–758 ) and (Int J Gynecol Pathol 25
(2006), pp. 239–242) In the present case, no gynecologic
malignancies were recognized.
Liposarcoma Arising in Uterine
Lipoleiomyoma:
A Report of 3 Cases and Review of the
Literature
AJSP: February 2011 - Volume 35 - Issue 2 - p 221–227
McDonald, Anna Greene MD*; Cin, Paola Dal PhD†;
Ganguly, Aniruddha PhD*; Campbell, Sharon*; Imai,
Yuki MD‡; Rosenberg, Andrew E. MD*; Oliva, Esther
MD*
北醫附醫， 萬芳醫院， 雙和醫院
(1995-2011/6)
1. leiomyoma: 8313
Uterus: 8033
age:10-96
(average: 47.3)
2. lipoleiomyoma: 21
Uterus: 20
age: 43-77
(average: 56.9)
20/8033: 0.25%
(1998-2011/6)
1. leiomyoma: 2161
Uterus: 1693
age:10-97
(average: 51.4)
2. lipoleiomyoma: 4
Uterus: 4
age: 40-64
(average: 54.2)
4/1693: 0.24%
(2008-2011/6)
1. leiomyoma: 423
Uterus: 355
age:17-82
(average: 47.6)
2. lipoleiomyoma: 1
Uterus: 1
age: 52
(average: 52)
1/355: 0.28%