Soft Tissue Tumours
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Transcript of Soft Tissue Tumours
Soft Tissue Tumour
Fatty Tissue Tumour
Lipoma Liposarcoma
Nature Benign Malignant
Age 40-70 40-70
Site Subcutaenous
Upper back
Shoulder/ Back
Proximal extremities
Retroperitoneum
Paratesticular
Clinical Soft, Mobile, Painless (except angiolipoma) Painless, Deep seated
Subtypes Fibrolipoma, Angiolipoma, Myelolipoma, Spindle cell lipoma, Pleomorphic
lipoma
Well differentiated, Myxoid, Round cell, Pleomorphic, Dedi ferentiated
X-ray Well-circumscribed, Homogenous, Similar density as fat Lobulated with mass – contain strand of soft tissue slightly dense
HPE Lobules of mature adipocytes
Fibrous septae
Heterogenous population of adipocytes
Lipoblasts
Rx Complete resection Complete resection
Prognosis Good (except intramuscular type) Depend on location, type
Lipoma
Neoplastic adipocytes –
indistinguishable from normal
adipocytes
Spindle cell lipoma
Proliferation of adipocytes
Disorganized bands of collagen –
contain spindle cells
Liposarcoma
Large mass lesion
Yellowish, like adipose tissue
Well-differentiated
Liposarcoma
Adipocytes
Pleomorphism of neoplastic cells
(lipoblasts)
Pleomorphic lipoma
Floret cells (large pleomorphic cells
with nuclei around cell periphery)
Cellular angiolipoma
Well-circumscribed tumor
Compose d of vascular proliferation
Scattered fat cells
Thrombi (in blood filled spaces)
Liposarcoma Large bizarre lipoblasts
Liposarcoma
Well-differentiated atypical lipoma
Fibrous septa (cellular septa)
Liposarcoma
Well-differentiated atypical lipoma
Pleomorphic nuclei scattered among
fat cells
Myxoid, Liposarcoma
Branching capillary network
Small cells of myxoid liposarcoma
Lipoblasts
Lymphangioma-like cystic
degeneration
Pleomorphic, Li posarcoma
Cells with multiple grapelike vacuoles
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Smooth Muscle Tumour
Leiomyoma Leiomyosarcoma
Age 30-50 > 20
Sites Uterus
Skin, nipples, scrotum, labia
Intestine, less in deep soft tissue
Cutaneous (extremities & trunk)
Large blood vessels (IVC) & deep soft tissue
Uterus
Signs &
Symptoms
Painless soft tissue swelling
Painful in pilar leiomyoma
Intestinal obstruction & urinary symptoms
Soft tissue swelling
Mass effect : obstruction
Size 1-2 cm (e xcept uterus) > 5 cm
HPE Circumscribed
Bland smooth muscle
Whorl pattern
< 1 mitosis/ 10 HPF (high power field)
Degenerative changes
Interweaving fascicles of pleomorphic spindle cells
↑ mitosis
Necrosis
Sarcomatous like MFH (malignant fibrous histiocytoma)
IM Actin + Desmin + Actin + Desmin +
Prognosis Good Depend on size, location, grade
Retroperitoneum - incomplete excision
Uterine fibroids/ uterine leiomyoma
Leiomyoma
Cells do not vary greatly in size &
shape
Resemble normal smooth muscle
cells
Leiomyosarcoma
Alternating fascicle pattern
Blunt-edged nuclei
Minor pleomorphism present
Leiomyoma
Blunt-ended elongated nuclei
(smooth muscle proliferation) Bizarre leiomyoma
Enlarged pleomorphic nuclei
Non-mitotic
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Skeletal Muscle Tumour
Rhabdomyoma Rhabdomyosarcoma (RMS)
Cardiac rhabomyoma (hemartomatous)
1. Rare
2. < 5y/o
3. Frequently associated with tuberous sclerosis
4. HPE – large, polygonal cells with glycogen
vacuoles
Common soft tissue sarcoma – children, adolescent
Cytogenic abnormalities
Diagnostic cell = rhabdomyoblast
Adult rhadomyoma
1. > 40 y/o
2. Exclusively in Head & Neck region – eg.
Oropharynx
Sites
1. Head & Neck
2. Genitourinary tract
3. Extremities
Genital rhabdomyoma
1. 20-40 y/o
2. Polypoidal lesion (spindle cell) at vagina, vulva,
cervix
Histological subtypes
Embryonal RMS Alveolar RMS Pleomorphic/ anaplastic RMS
Conventional
Botyoid (sarcoma botryoides)
Spindle cell
Infant & children Adolescent Adult
1. Head & Neck, GUS,
retroperitoneum
2. Bladder, vagina, rectum,
nasal cavity, nasopharynx
3. Paratesticular
Extremities, paranasal sinuses,
retroperitoneum
Extremities
Spindle cells
Cross-striations
Rhabdomyoblast
Primitive cells
Cambium layers
Abundant collagen
Alveolar-like spaces
Fibrous septae
Cross-striation
Rhabdomyoblast
Large, pleomorphic
Multinucleated
↑ mitosis
Rhabdomyosarcoma (Embryonal
type)
Round cells with small oval nuclei
Scanty eosinophilic cytoplasm
Eccentric placement of nucleus in
cytoplasm
Rhabdomyosarcoma (Alveolar
type) Tumor cells – grip fibrous septa
around alveoli
Large pleomorphic cells with
abundant cytoplasm
Rhabdomyosarcoma (Botryoid
type) Orbital conjunctiva
Dense aggregation of cells below
epithelium
Rhabdomyosarcoma (Botyroid type)
Cambium layer
Loose texture
Differenting strap cells
Rhabdomyoma
(genital type) Fibrous tissue
Spindling groups of
cells
Long cytoplasmic
extensions with
parallel sides
IM – Vimentin, Desmin, Myogenin
Treatment – Surgery, Chemotherapy with or without radiotherapy
Prognosis – Staging, Age, Histological subtypes (Botryoid > Embryonal > Pleomorphic > Alveolar)
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Fibrous Tissue Tumour
Fibroma Fibromatosis Fibrosarcoma
Rare Superficial Deep (Desmoid tumor)
Palmar
Plantar
Penile
Extra-abdominal
shoulder, chest wall,
back, thigh
Abdominal
anterior abdominal wall
Intra-abdominal
mesentery, pelvic wall
Male ↑ Female ↑
Benign
Do not progress
Agressive locally
Frequenty recur but
never metastasize
Fibroblasts
Collagen deposit
Fibroblasts & collagen
Mitosis infrequent
Fibromatosis Fibroblasts proliferate on collagen
Cytoplasm is inapparent, but visible if cut en face
(center)
Rare
Fibroma-thecoma in ovary Diagnosis by exclusion
Renal fibroma – hamartoma Sites
Retroperitoneum, thigh, knee, distal extremities
Gross
Uncapsulated, soft, infiltrative with areas of
hemorrhage & necrosis
HPE
Fascicles of spindle cells
Herringbone pattern
Frequent mitosis & necrosis
Prognosis Aggressive
50% recur
25% metastasize
Fibrosarcoma
Fascicles of spindle cells
Lesion is cellular
Fibrosarcoma Herringbone pattern
Differential diagnosis – synovial sarcoma, malignant
peripheral nerve sheath tumor
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Fibrohistiocytic tumour
BFH (Benign fibrohistiocytic tumour) DFP (Dermatofibrosarcoma protuberans) MFH (Malignant fibrohistiocytic tumour)
Benign Borderline Malignant
Dermatofibroma Common pathway for ST sarcoma progression
Age 20-50 20-50 > 50
Sex Male ↑ Male = Female Male ↑
Sites Skin
Lower limb
Head & Neck
Trunk
Groin
Lower Limb
Head & Neck
Proximal extremities
Retroperitoneum
Signs &
Symptoms
Slow growing dermal / Soft tissue (ST) mass Exophytic
Multinodular painless growth
Large mass
Sometimes with pain
Gross Fairly circumscribed nodule/ mass Poorly circumscribed
with entrapped skin adnexae & fat
5-20 cm pse udo-capsule hemorrhage & necrosis
Sub-types - Classical
Bednar tumour
Storiform/ Pleo
Myxoid
Inflammatory
Giant cell
Angiomatoid
Histo Spindle cells
Infiltrative margin
Foam cells, hemosiderin, giant cells, epidermal
hyperplasia
Spindle cells
Infiltrative margin
Prominent storiform pattern
Mitosis <5/10 HPF
Depends on subtypes
Rx Simple excision Wide surgical resection Wide surgical resection
Prognosis Good Recurrence (50% )
Metastasis (5-15%)
Aggressive (except angiomatoid)
Metastasis (30-50%)
60% - 5 year survival rate (YSR)
Benign fibrous histiocytoma
Hyperplastic epidermis
Dermal spindle cell lesion – contain lakes of
blood
No endothelial lining of lakes
Dermatofibrosarcoma protuberans
Cartwheel/ storiform pattern
(without pleomorphism)
Thin nuclei, lack of perceptible cell borders, overall
tight quality of pattern
Malignant fibrous histiocytoma (Medium power)
Storiform/ pleomorphic
Pleomorphic nuclei scattered among smaller cells
Vague storiform pattern
Small fibrous histiocytomas Cartwheel/ storiform cells
Lack plaquelike growth in dermis/ subcutaneous fat
Malignant fibrous histiocytoma (High power)
Bizarre nuclei in huge cells found
Inflammatory infiltrate
Spindle cells – irregular nuclear shapes, lack well-
defined cell borders
Malignant fibrous histiocytoma (angiomatoid)
Dark cuff of lymphoid cells complete with
germinal center
Lighter tumour nodules proliferate
Malignant fibrous histiocytoma (inflammatory) Malignant nuclei are larger & vesicular (not bizarre)
Sea of neutrophils & other inflammatory cells
Malignant fibrous histiocytoma (Giant cell)
Osteoclast-like giant cells
Malignant tumour giant cells with enlarged nuclei
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Blood vessel tumour
Hemangioma Hemangioendothelioma Angiosarcoma
Nature Benign Borderline Malignant
Age Infant & children > 40 > 40
Sites Skin of Head & Neck
Liver
ST of extremities
Large veins
Skin of Head & Neck
Skin of Head & Neck
Breast, liver, spleen
ST of extremities
HPE Benign endothelial cells
In lobules
Proliferating endothelial cells
Cytoplasmic vacuoles containing RBC
Malignant pleomorphic cells
Anastomosing blood vessels
Prognosis Good Variable
Majority cured by excision
Up to 40% recur
Up to 30% metastasize
Very poor
Margin clearance difficult
60% recur
50% metastasize
Capillary Cavernous Lobular capillary
Infancy, early
childhood
Infant
Children
Any age
Female ↑ Female ↑ F=M
Skin &
subcutaneous of
Head & Neck
Skin &
subcutaneous
Deep ST
Liver
Gingiva, fingers,
lip & face
Lobules of small
size vessel
Central “feeder”
vessel
Large blood-
filled vessels
Thrombi &
calcification
As capillary type
Inflammation &
ulcer
Spontaneous
regression
No spontaneous
regression
No spontaneous
regression
Grade 2 Hemangioendothelioma Vessel-forming growth pattern
Blood-filled spaces
Angiosarcoma
Collagen as scaffold for endothelial cells
Too many cells line anastomosing spaces
Atypia
Lobular capillary hemangioma (pyogenic granuloma)
(Medium power)
Lobules are sharply demarcated by fibrous septa –
hypercellular & lack spindy stream of cells in Kaposi disease
Spindle cell hemangioendothelioma
Vacuolated endothelial cells
Lining blood-filled channels
Lobular capillary hemangioma (pyogenic granuloma)
(High power)
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Peripheral Nerve Tumour
Neurofibroma Schwannoma (neurilemmoma) Malignant peripheral nerve shealth tumour (MPNST)
20-40 y/o. 5-30 y/o (Diffuse)
Childhood (plexifor m)
20-50 y/o 20-50 y/o
Male = Female (but Male ↑ in neurofibromatosis)
Solitary
Diffuse
Plexiform
Soliary
Multiple (3%)
= Neurofibrosarcoma
= Malignant schwannoma
Any sites (especially Head & Neck) Extremities & Head & Neck
Cellular schwannoma – mediastinum, retroperitoneal,
pelvis
Slow growing painless skin lesions
Plaque-like in diffuse type
Tortous, worm-like nerve
Elephantiasis neuromatosa
Slow growing tumour
Pain with neurologic symptoms
Headache, nasal obstruction, epistaxis, otitis media
Hearing loss + tinnitus
Soft tissue mass with/ without pain
Long sweeping fascicles & herring bone patterns
Hypocellular myxoid areas (CT fibrosarcoma)
Nuclear palisading
Geographical necrosis
Metaplastic changes (eg. Bone)
Others – rosettes, glands formation, perivascular
condensation, neuroepithelial differentiation like
Ewing sarcoma
Epitheloid variant (CT melanoma or carcinoma)
Triton tumour (MPNST + RMS differentiation)
NF Type 1 (chromosome 17 ) NF Type 2 (chromosome 22 )
No capsule
Fusiform, plaque, plexiform
Well-circumscribed
Encapsulated
Dumbbell-shape d lesion
Spindle cell in haphazard arrangement
Hypocellular area
Mast cells
Antoni A area
Antoni B area
Verocay bodies
Hyalinized blood vessels
Cured by excision (solitary)
Recur & ↑ risk malignancy (NF)
Cured by excision Wide surgical resection
Neurofibroma
Plexiform neurofibroma
Schwannoma
Dimorphic pattern of cellular (Antoni A)
Loose myxoid (Antoni B)
Nuclear palisading (parallel rows of nuclei)
Malignant peripheral nerve sheath tumour
Mitotically active & cellular tumour
Nuclei bullet shaped
(blunt at one end, pointed at other)
Neurofibroma
Uniformly hypocellular
Slightly myxoid
Undulating fibrillar background
Spindle cell carcinoma
Nervelike whorls
Primitive Neuroectodermal tumour (PNET) (= Ewing sarcoma)
10-15 y/o
Swelling with pain & fever
Morphology
Small uniform round cells with scanty cytoplasm
Very little stroma
Rosette formation
Aggressive tumour
Treatment with resection & chemotherapy +/- radioRx
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Synovial Sarcoma
Carcinoma of soft tissue
20-40 y/o
Location
Lower > upper extremities & proximal > distal
Head & Neck, abdomen, retroperitoneum
Only 10% intra-articular
Radiographic findings
Deceptively well-circumscribed
Stippled calcifications
Mimic myositis ossificans (due to extensive calcification & metaplastic bone formation)
Pathologic findings
• Fibrous elements
o Spindle cells (monotonous, uniform) in short fasci cles
o Collagen (variable stromal)
o Alternating hypercellular & hypocellular regions
• Epithelial elements
o Gland formation
o Mucin production
• Intratumoral calcifications
• Cystic degeneration
• Metaplastic bone reaction
Subtypes Monophasic fibrous > Biphasic > Monophasic epithelial > Poorly differentiated
IM Vimentin, EMA, Cytokeratin, CD99
Treatment
Surgical resection (plus adjuvant therapy)
Risk factors for disease progression
Age > 25
Size > 5
High nuclear grade (poorly differentiated)
Necrosis > 50%
Rhabdoid morphology
↑ Tumour stage
Disease-free survival 88% in low-risk group (<25 y/o, <5cm, no poorly differentiated)
18% in high-risk group
Synovial sarcoma (Biphasic type)
Glands
Small, oval, overlapping nuclei in spindle component
Synovial sarcoma (Monophasic type)
Pericytomatous pattern
Solid areas
Synovial sarcoma (Monophasic type)
Focal cell cluster (indicate epithelial differentiation)
Synovial sarcoma (Reticulin stain)
Epithelial nesting pattern
Synovial sarcoma
Cytokeratin – glands, scattered spindle cells
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