3Rd Year Soft Tissue Tumors

Front 1

t(11;22)(q24;12) / t(7,22)(p22;q12) / t(2;22)(q12;q12) Is a cytogenic alteration in ?

Back 1

* Ewing Sarcoma
* Primitive Neuroectodermal tumor
* Neuroblastoma

Front 2

t(2;13)(q35;q14) / t(1;13)(p36;q14) is a cytogenic alteration in ?

Back 2

Alveolar Rhabdomyosarcoma

Front 3

t(12;16)(q13;p11) is a cytogenic alteration in ?

Back 3

* Myxoid liposarcoma
* Round cell liposarcoma

Front 4

t(X;18)(p11;q11) is a cytogenic alteration in ?

Back 4

Synovial sarcoma

Front 5

t(3;12)(q24;q14) is a cytogenic alteration in ?

Back 5

Lipoma

Front 6

Is fibroma benign of malignant ?

Back 6

Benign

Front 7

Nodular fasciitis / Proliferative fasciitis / Proliferative myositis / Keloid are examples of what ?

Back 7

Benign fibrous tissue TLLs (tumor like lesions)

Front 8

What is in common to Fibrous hamartoma of infancy / Juvenile fibromatosis / giant cell fibroblastoma / calcifying aponeurotic fibroma / Hyaline fibromatosis ?

Back 8

All are benign fibrous tumors

Front 9

What are the types of Fibromatoses, give examples

Back 9

*Superficial fibromatosis (fibromatosis of palms and soles)
*Deep fibromatosis (abdominal/extraabdominal fibromatosis)

Front 10

Give 3 examples of malignant fibrous tumors

Back 10

* Low grade Myofibroblastic sarcoma
* Adult fibrosarcoma
*Congenital fibrosarcoma

Front 11

A benign lesion with fibroblasts and varying degree of collagen is known as :

Back 11

Fibroma

Front 12

Dense fibrous tissue with spindle or stellate mesenchymal cells, containing slit like vascular channels is characteristic of which benign tumor ?

Back 12

Fibroma of tendon sheaths

Front 13

Which tumor is common in individuals with a history of hard manual work, charecteristicly containing collagen, myofibroblasts and positive for elastic stains ?

Back 13

Elastofibroma

Front 14

What are the charecteristics of Giant cell fibroblastoma and at what age does it occur ?

Back 14

*Superficial lesion on the back
*proliferation of fibroblasts
*myxoid stroma
*presence of atypical fibroblasts
*presence of giant cells
**Affects infants and children

Front 15

Which tumor has the following charecteristics, and what are its two names :
*Ill defined border with foci of calcification
*Fibroblasts growth
*Infiltration of fat and muscle at the periphery
*osteoclast-type giant cells
*Local recurrence is frequent

Back 15

*Calcifying aponeurotic fibroma -- also known as -- Juvenile aponeurotic fibroma

Front 16

Describe Nodular fasciitis (mention benign/malignant, age, location, growth rate, size, margins, histological features, similarty to other tumors)

Back 16

Nodular Fasciitis :
*Benign
*Young adults
*Upper extremities
*Infiltrative margins
*spindle cells
*Loose mucoid matrix
*Vascular proliferation
*Wide collagen bundles
*Large no. of mitotic figures
*Confused with Fibrosarcoma

Front 17

What is special about Cranial fasciitis ?

Back 17

Happens in Children and results in bone erosion

Front 18

Intravascular fasciitis is common in ?

Back 18

Medium sized arteries and veins

Front 19

What is the difference between Nodular fasciitis and Proliferative fasciitis ?

Back 19

In Proliferative fasciitis there are large basophilic ganglion-like cells

Front 20

What are the charecteristics of Proliferative Myositis ?

Back 20

Proliferative Myositis :
*Ill defined scar like lesion ofthe muscle
*Proliferation of fibroblasts, surrounding muscle fibers
*Presence of large basophilic ganglion like cells or Rhabdomyoblast like cells
*Check IHC for Myofibroblasts
*Note link to Proliferative fasciitis

Front 21

Which tumor has the capacitiy to locally recurr, lacks the ability to metastasize, has little mitotic activity, shows proliferation of fibroblasts with an infiltrative growth pattern ?

Back 21

Fibromatoses

Front 22

What are the morphological features of Fibromatoses ?

Back 22

*Large, firm, grayish-white, ill defined lesions
*Irregulr whorled cut surface
*Perivascular lymphocytic infiltrate
*Calcification

Front 23

What is meants by a Desmoid tumor ?

Back 23

A superficial tumor

Front 24

What are the regional charecteristics of Muscuoaponeurotic Fibromatosis (aka Desmoid Tumor) ? Which region shows the best prognosis ? Which gender is affected more often ?

Back 24

Regions : Shoulder, head, neck, abdomen
Best prognosis : Abdomen
Gender : Much more common in Men

Front 25

What are 3 forms of Juvenile Fibromatosis ?

Back 25

*Fibromatosis Colli
*Infantile digital fibromatosis
*Infantile myofibromatosis

Front 26

Fibromatosis Colli is a type of which Fibormatoses group ?
What are its charecteristics ?

Back 26

*Part of Juvenile fibromatoses
*Affects the lower third of SCM muscle
*Appears at birth or shortly after

Front 27

What are the features of Infantile digital fibromatosis?

Back 27

*Affects exterior surface of the end of phalanges
*Often multiple
*Shows Eosinophilic cytoplasmic inclusions

Front 28

Does Infantile myofibromatosis occur in a solitary or multiple fashion ?

Back 28

Either solitary or multiple

Front 29

Which fibromatosis shows an abrupt transition from a rounded cells area to a spindle cells area ?

Back 29

Infantily myofibromatosis

Front 30

What are the other names for the following types of Fibromatoses :
1. Palmar fibromatosis
2. Plantar fibromatosis
3. Penile Fibromatosis

Back 30

1. Dupuytren
2. Ledderhose
3. Peyronie

Front 31

What are the charecteristics of LGMFS (Low grade myofibroblastic sarcoma) ?
Mention benign/malignant, age, region, recurrence, metastases, differences from Fibromatoses

Back 31

1. Malignant
2. Young to middle age
3. Wide anatomical distribution, esp. Head/neck
4. Recurrence is typical
5. Metastases occurs later on
6. Differences from Fibromatoses - show nuclear atypia, diffusely hypercellular

Front 32

Was fibrosarcoma a more common diagnosis during the 60's than it is today ?

Back 32

Yes, in the 60's - very common. Today - RARE

Front 33

What are the two types of Fibrosarcoma and which is more common ?

Back 33

Adult, Infantile.
Infantile Fibrosarcoma much more common

Front 34

When does infantile Fibrosarcoma develop ? Which part of the body does it affect ?

Back 34

Often congenital, usually develops within the first 2 years
* Mostly affects the distal extremities

Front 35

What are the pathological features of Fibrosarcoma ?

Back 35

Fibrosarcoma :
* Well circumscribed masses
* Deep (subfascial)
* Spindle cells with pale cytoplasm, elongated nuclei, arranged in a heringbone pattern (Fish bone)
*CELLULAR neoplasm, collagen is limited

Front 36

Is Fibrosarcoma + or - for the following IHC markers (S100, EMA, Keratin, Desmin) ?
What IHC feature is only found in the infantile form ?

Back 36

NEGATIVE for S100, EMA, Keratin, Desmin
* Infantile form may show focal Actin +

Front 37

Low grade fibromyxoid sarcoma (LGFMS) is a variant of which Malignant tumor ?

Back 37

Fibrosarcoma

Front 38

Define LGMFS vs. LGFMS

Back 38

LGMFS - Low grade myofibroblastic sarcoma
LGFMS - Low grade fibromyxoid sarcoma (variant of Fibrosarcoma !)

Front 39

What are the features of LGFMS ?

Back 39

LGFMS :
*Young adults
*Variable localization
*IHC : Vimentin +

Front 40

Do Fibrohistocytic neoplasms occur more often in children or in adults ?
Also, do they contain true histiocytes ?

Back 40

Fibrohistocytic neoplasms occur mostly in Adults
*They do not contain true histiocytes

Front 41

What are the 2 types of Benign histiocytic neoplasms ?

Back 41

1. Fibrous histiocytoma (skin = dermatofibroma,deep)
2. Atypical fibroxanthoma

Front 42

Dermatofibrosarcoma Protuberans (DFSP) and Bednar's tumor are examples of ?

Back 42

Intermediate malignancy Fibrohistiocytic tumors

Front 43

Give an example of a malignant Fibrohistiocytic neoplams ?

Back 43

Malignant fibrous histiocytoma

Front 44

Fibrous histiocytoma (either skin, or deep) has the following features :

Back 44

Fibrous Histiocytoma :
*Benign
*Storiform
*foamy macrophages
*hemosiderin and RBCs

Front 45

Fibrous histiocytomas IHC markers are :

Back 45

CD68;Vimentin;FXIIIa : +
CD34 : -

Front 46

Which tumor shows the following features :
Rapid growth, either symmteric or asymetric, well circumscribed (But my be infiltrative), shows fusiform orr round cells with with prominent cytological atypia ?
Is it benign or malignant ?

Back 46

Atypical Fibroxanthoma,
a Benign Fibrohistiocytic neoplasm

Front 47

What are the differences between Dermatofibrosarcoma (DFSP) and Fibrous histiocytoma ?

Back 47

*No collagen in DFSP
*DFSP IHC : CD34+, Fibrous Histiocytoma IHC : CD34-
*In DFSP Dedifferentiation may occur and MHF may develop (MALIGNANCY)

Front 48

What is Bednar's tumor and example of and what is its description ?

Back 48

It is an intermediate-malignancy Fibrohistiocytic neoplasm.
It is a pigmented variant of DFSP (Dermatofibrosarcoma protuberans)

Front 49

What is the most common diagnosis of a soft tissue sarcoma ?

Back 49

MFH - Malignant fibrous histiocytoma

Front 50

What are the variants of MFH ?

Back 50

*Pleomorphic
*Myxoid
*Giant cell
*Inflammatory
*Angiomatoid

Front 51

What are the main features of Pleomorphic MFH and when is this diagnosis concluded ?

Back 51

* Highly anaplastic
*No special light-microscope differentiating sign
*Storiform pattern
*Multinucleated giant cells

This diagnosis is given in case of unclassifiable high-grade sarcomas

Front 52

What are the features of Myxoidfibrosarcoma (Myxoid MFH) ? Mention age,region, resemblance to other tuumors, cellular features, stroma, IHC marker)

Back 52

Affects Adults
*Affects limbs (Retroperitoneum = rare)
*High grade resembles pleomorphic MFH
*Stellate and fusiform cells with vacuolisation (PAS+)
*Stroma is myxoid
*IHC : Factor XIII+

Front 53

What are the names of two Peripheral nerve tumors which do not contain "malignant" in them ?

Back 53

*Peripherial Primitive Neuroectodermal tumor (Peripheral PNET)
*Clear Cell Sarcoma

Front 54

Is Traumatic Neuroma a tumor or a TLL ? Also, what are its main features ?

Back 54

*It's a TLL
*All elements of a nerve can be recognzied
*Painful

Front 55

Which benign neoplasm shows two microscopic pattern called "Antoni A" and "Antoni B" ? What are the main differences ?

Back 55

*Schwanomma (or a neurolimmoma).
*Antoni A : Cellular, with spindle cells (verocay bodies)
*Antoni B : Contains cystic areas, dark hyperchromatic nuclei
*BOTH types are ENCAPSULATED
*Malignant transformation is very rare

Front 56

What are the main features of a Neurofibroma ? (Encapsulation, focal or diffuse, special features)
What is neurofibromatosis ?

Back 56

Neurofibroma :
*Not encapsulated
*Focal or diffuse
*No verocay bodies (contrast with schwanomma)
*Neurofibromatosis is multiple neurofibromas, usually with a malignant trans.

Front 57

Main charecteristic of a Perineuroma ?

Back 57

Very long cells arranged in parallel bundles

Front 58

Name two peripheral nerve neoplasms which are S100 positive

Back 58

1. Dermal sheath myxoma
2. Granular cell tumor

Front 59

What is the difference between a Dermal sheath myxoma and a Myxoma ?

Back 59

In Dermal sheath myxoma there are no epitheloid cells

Front 60

Which benign Peripheral nerve neoplasm shows the following features :
Small lesions, with large cells and a granular cytoplasm, rarely malignant, and is PAS+ and S100+

Back 60

Granular cell tumor

Front 61

What are the features of MPNST (Malignant peripheral nerve sheath tumor) (Source, microscopic features, IHC)

Back 61

*Half arise de novo, half
from Neurofibromas
*Extreme cellular, spindle cells, epitheloid appearance, larg vascular spaces
*S100+

Front 62

What is the second name for a Peripheral primitive neuroectodermal tumor (Peripheral PNET) ?

Back 62

Askins' tumor

Front 63

What are the features of Peripheral PNET ? (Age, microscopic appearance, IHC)

Back 63

*Affects young adults
*Uniform round cells containing glyocgen (PAS+)
*IHC : CD99+, NSE+

Front 64

What tumor is parallel with Ewing's sarcoma of the soft tissue ?
What tumor is parallel with Malignant melanoma of soft tissues ?

Back 64

*Ewing's sarcoma in soft tissue = Askin's tumor, or Peripheral PNET
*Malignant melanoma in soft tissue = Clear Cell Sarcoma

Front 65

Which malignant tumor of peripheral nerve has the following features :
Affects young adults, contains epitheloid or fusiform cells with large nuclei, cytoplasm may contain melanin, and is IHC + for HMB-45, Vimentin, and +/- for Keratin ?

Back 65

Clear Cell Sarcoma (Malignant melanoma of soft tissues)

Front 66

What's in common to Hemangioma, Glomus tumor, and Sinonasal Hemangiopericytoma ?

Back 66

All are BENIGN neoplasms of vessels

Front 67

What are the tumors of vessels which show intermediate malignancy ?

Back 67

*Epitheloid hemangioendothelioma
*Hemangiopericytoma

Front 68

What is in common to Angiosarcoma, Kaposi's sarcoma, and Glomangiosarcoma ?

Back 68

All are MALIGNANT tumors of vessels

Front 69

What is the most common location for a Capillary hemangioma ? Which IHC markers would it have ?

Back 69

Most common location for capillary hemangioma is the skin. Since it is an endothelial neoplasm it is + for CD31,CD34,and FXIII

Front 70

How is Hemangioma classified ? Mention two special types of Hemangioma and where they appear

Back 70

According to their clinical appearance and the caliber of the veseel.
Fetal hemangioma is seen in head&neck region.
Genital hemangioma is seen in vulvovaginal area of middle aged women

Front 71

What are the features of Glomus tumor (Glomangioma) (Localization, pain, microscopic, malignancy change)

Back 71

*Classic localization is in subinguinal region, but can be elsewhere
*Painful
*Normal endothelial in blood vesels surrounded by proliferation of 'epitheloid' cells
*May transform to malignant form (glomangiosarcoma)

Front 72

Main features of Hemangiopericytoma (localization, malignancy, microscopic, IHC)

Back 72

*Variable localization
*Intermediate malignancy
*IHC : Vimentin+, SMA-, CD34-, FXIIIa+/-

Front 73

Main features of angiosarcoma (Source, age, variant, invasivness, IHC)

Back 73

*Angiosarcoma arises from Endothelial cells of blood vessels
*Occurs in adults
*Variant is Lymphangiosarcoma
*Highly invasive
*IHC : FVIII+, CD31+, CD34+

Front 74

Which neoplasm shows the following featurs :
Low grade fusiform endothelial proliferation (often with immunodeficiency), with common stromal hemorrhage and hemosiderin deposition, and contains hyalin globules ?

Back 74

Kaposi's Sarcoma

Front 75

What are the stages of Kaposi's Sarcoma ?

Back 75

Patch -> Plaqu -> Nodular (with redish color)

Front 76

How can you tell if an adipose tissue tumor is benign or malignant by its name ?

Back 76

ONLY malignant adipose tissue tumor is Liposarcoma, all other tumors with 'lipoma' in their name OR 'hibernoma' are BENIGN adipose tissue tumors

Front 77

What is the most common soft tissue tumor ? Is it benign or malignant ?

Back 77

Lipoma, Benign

Front 78

What is the difference in localization between a benign lipoma and a malignant liposarcoma ?

Back 78

Liposarcomas are DEEP, lipomas are superficial

Front 79

What are the common features of all lipomas, and which type is special in that it really does contain lipoblasts ?

Back 79

*Most lipomas are superficial
*Usually encapsulated
*Yellowish, fat like tissue
*Only typical lipomas contain lipoblasts

Front 80

What is special about the Angiolipoma variant of Lipoma, and what is special about the Angiomyolipoma variant ?

Back 80

*Angiolipoma : Painful with fat cells and many capillaries
*Angiomyolipoma : Is common in the kidneys

Front 81

With which malignant neoplasm is the benign tumor Lipoblastoma usually confused ?
What is the difference in age of onset ?

Back 81

Lipoblastoma is often confused with myxoid Liposarcoma since they both have a myxoid stroma
*Lipoblastoma is usually in infants and young children
*Myxoid liposarcoma is found in ADULTS

Front 82

What is the origin of a Hibernoma and to what major group does it belong ?

Back 82

Hibernoma is a subtype of benign adipose tissue tumors, it originates in brown adipose tissue

Front 83

What is the most common Malignant soft tissue tumor in adults ?

Back 83

Liposarcoma

Front 84

Which malignant tumor shows localization in deep tissues such as lower extremities and abdomen, is usually well circumscribed but is not encapsulated, may be myxoid, contains lipoblasts and is S100+ ?

Back 84

Liposarcoma

Front 85

What are the low & high grade variants of a Liposarcoma ?

Back 85

Low Grade :
*Well defferentiated liposarcoma (resembles Lipoma)
*Myxoid (most common!)
High Grade :
*Round cell (small)
*Pleomorph (highly undifferentiated, contains giant cells)
*Dedifferentiated

Front 86

What are the two components of a dedifferntiated Liposarcoma ?

Back 86

*A well differentiated liposarcoma and a
*High grade non-lipogenous soft tissue sarcoma (Fibrosarcoma, MFH..)

Front 87

What is the difference in nomenculture between a smooth muscle neoplasm and a striated muscle neoplasm ?

Back 87

*Smooth muscle neoplasm contains "Leiomy" in its name
*Striated muscle neoplasm contains "Rhabdomy" in its name

Front 88

What is the name of the malignant form of a smooth muscle neoplasm ?

Back 88

A Leiomyosarcoma

Front 89

What is common to all types of Leiomyoma ?

Back 89

*Whitish-gray, well circumscribed firm masses
*Made up of fascicles of eosinophilic smooth muscle cells
*show NO mitotic activity *NO necrosis

Front 90

What are the subtypes of Leiomyoma ?

Back 90

*Cutaneous
*Genital
*Vascular
*Deep-seated

Front 91

What is the gross difference between a Leiomyoma and a Leiomyosarcoma ?

Back 91

*Leiomyosarcomas are larger, softer, and have a tendency for necrosis

Front 92

What are the features of a Leiomyosarcoma (frequency, microscopic, IHC)

Back 92

*Realtively rare
*Tumor bundles intersect each other, tumor cells are 'cigar shaped', varialbe cytological atypia, myxoid changes, giant cells
*IHC : Alpha-smooth muscle actinin+, Desmin+, Vimentin+

Front 93

What is the most common malignant tumor in children ? What is it in adults ?

Back 93

Most common malignancy in children : Rhabdomyosarcoma
Most common malignancy in adults : Liposarcoma

Front 94

What are the variants of a Rhabdomyosarcoma ? Which one shows the better prognosis ?

Back 94

*Embryonal - excellent prognosis
*Alveolar - poor prognosis

Front 95

Which rare neoplasm shows a localization to ther oral cavity, is found in adults, and contains large, round, well differentiated cells ?

Back 95

Rhabdomyoma

Front 96

What are the variants of Embryonal Rhabdomyosarcoma and what is their localization ?

Back 96

*Embryonal : Head&neck, Genitourinary tract, extremities
*Botryoid : Genitourinary
*Spindle cell : Paratesticular
*Anaplastic : Lower extremities

Front 97

What is the gross difference between a botryoid variant of an embryonal Rhabdomyosarcoma and the other variants ?

Back 97

Other variants are fleshy, solid and well circumscribed, usually firmer.
The botryoid variant is softer and grows as grape like vesicles

Front 98

What is the microscopic criteria for a Spindle shaped variant of an embryonal rhabdomyosarcoma, and what is it for an Anaplastic varaint ?

Back 98

Spindle shaped : More than 50% of the cells are spindle shaped
Anaplastic : Some scattered cells have a 3 times larger nuclei than the surrounding tumor cells

Front 99

What are the features of an Alveolar Rhabdomyosarcoma (Age, localization, growth pattern, microscopic) ?

Back 99

*Young patients
*Affects extremities, head/neck, trunk
*Grows in nests or clusters seperated by septa (like in the lung...)
*Variable cell types

Front 100

What are the IHC markers of Rhabdomyosarcoma ?

Back 100

Rhabdomyosarcoma :
SMA-, Desmin+, MyoD1+, Vimentin+, Myoglobin+

Front 101

What are the benign forms of chondromatous tumors ?

Back 101

*Chondroma
*Myositis ossifican
*Fibrodysplasia ossificans

Front 102

What are the common features of chondromatous tumors ?

Back 102

*Very rare
*Arises from Chondromatous or osseous differentiation of pleuripotent mesenchymal cells

Front 103

What are the benign types of synovial tumors ?

Back 103

*Localized tenosynovial tumor (or Giant cell tumor of tendon sheaths)
*Diffuse tenosynovial tumor (or Soft tissue pigmented villnodular synovitis)

Front 104

What is the only malignant form of a synovial tumor ?

Back 104

*Malignant tenosynovial tumor
***NOTE***
Synovial Sarcoma is NOT a synovial neoplasm !!!

Front 105

What is the localization of a localized tenosynovial tumor (giant cell tumor of tendon sheaths) ?

Back 105

Hands, Feet

Front 106

What are the differences between a localized and a diffuse tenosynovial tumor ?

Back 106

*Localized only : Circumscribed
*Diffuse only : Many xanthoma cells, hemosiderin pigmentation

Front 107

How common is a malignant tenosynovial tumor ?

Back 107

Rare, based on diagnosis of exclusion

Front 108

What are myxomas ?

Back 108

Myxomas are benign/intermediate malignancy neoplasms of uncertain differentation

Front 109

Name malignant tumors of uncertain differenation and/or those which source is non-mesenchymal

Back 109

*Small cell tumor with desmoplastic stroma
*Synovial sarcoma(!!)
*Epitheloid sarcoma
*Extrarenal rhabdoid tumor

Front 110

What are the features of a Myxoma ?

Back 110

*Have an Acid mucopolysaccharide matrix
*Many cases it is unknown whether it is neoplastic or reactive
*Best known tumor is Intramascular myxoma

Front 111

Is an ossifying fibromyxoid tumor benign or malignant ?
Is it encapsulated ?
What is its IHC marker ?

Back 111

*Benign tumor
*Encapsulated, well circumscribed
*S100+

Front 112

What are the two forms of Synovial sarcomas ?

Back 112

*Monophasic
*Biphasic
BOTH DO NOT ARISE FROM JOINTS, but not far from them

Front 113

What are the main features of Synovial sarcomas (Microscopic, age, gender, localization)?

Back 113

*Spindle cells express EMA, cytokeratins == not found in normal synovial tissue
*Occurs in young adults, mostly males
*Localization to extremities

Front 114

What are the two components of Biphasic Synovial sarcoma ?

Back 114

*Epithelial component which forms solid nests, or glands
*Spindle cells component which is made up of uniform spindle cells with collagen bundles, and calcification foci

Front 115

Which component of the biphasic synovial sarcoma is found in monophasic synovial sarcoma ?

Back 115

The Spindle cells component

Front 116

What is the cytogenic alteration arising in Synovial sarcoma ?
What are the IHC markers for synovial sarcoma ?

Back 116

Translocation : X;18
IHC : S100+, EMA+

Front 117

What are the features of an Alveolar soft part sarcoma (Age, metastasis, growth rate, microscopic, IHC)

Back 117

*Mostly young adults
*Growth rate is slow
*Metastasis does occur
*Tumor is divided into nodules, necrosis within the nodule with less cohesion in the center
*IHC : Actin+, myoD1+
(however no link to myoid nature)

Front 118

What are the features of Epitheloid sarcoma (origin, age, variants, microscopic, IHC)

Back 118

*Origin of Epitheloid sarcoma is of uncertain differenation, NOT epithel
*Young adults
*Distal variant is less aggresive and more common
*Proximal variant is much more aggressive
*Eosinophilic cells aggregated into nodules - carcinoma LIKE fashion
*IHC : EMA+, Vimentin+