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3rd year Soft Tissue Tumors


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t(11;22)(q24;12) / t(7,22)(p22;q12) / t(2;22)(q12;q12) Is a cytogenic alteration in ?
* Ewing Sarcoma
* Primitive Neuroectodermal tumor
* Neuroblastoma
t(2;13)(q35;q14) / t(1;13)(p36;q14) is a cytogenic alteration in ?
Alveolar Rhabdomyosarcoma
t(12;16)(q13;p11) is a cytogenic alteration in ?
* Myxoid liposarcoma
* Round cell liposarcoma
t(X;18)(p11;q11) is a cytogenic alteration in ?
Synovial sarcoma
t(3;12)(q24;q14) is a cytogenic alteration in ?
Is fibroma benign of malignant ?
Nodular fasciitis / Proliferative fasciitis / Proliferative myositis / Keloid are examples of what ?
Benign fibrous tissue TLLs (tumor like lesions)
What is in common to Fibrous hamartoma of infancy / Juvenile fibromatosis / giant cell fibroblastoma / calcifying aponeurotic fibroma / Hyaline fibromatosis ?
All are benign fibrous tumors
What are the types of Fibromatoses, give examples
*Superficial fibromatosis (fibromatosis of palms and soles)
*Deep fibromatosis (abdominal/extraabdominal fibromatosis)
Give 3 examples of malignant fibrous tumors
* Low grade Myofibroblastic sarcoma
* Adult fibrosarcoma
*Congenital fibrosarcoma
A benign lesion with fibroblasts and varying degree of collagen is known as :
Dense fibrous tissue with spindle or stellate mesenchymal cells, containing slit like vascular channels is characteristic of which benign tumor ?
Fibroma of tendon sheaths
Which tumor is common in individuals with a history of hard manual work, charecteristicly containing collagen, myofibroblasts and positive for elastic stains ?
What are the charecteristics of Giant cell fibroblastoma and at what age does it occur ?
*Superficial lesion on the back
*proliferation of fibroblasts
*myxoid stroma
*presence of atypical fibroblasts
*presence of giant cells
**Affects infants and children
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 r
*Calcifying aponeurotic fibroma -- also known as -- Juvenile aponeurotic fibroma
Describe Nodular fasciitis (mention benign/malignant, age, location, growth rate, size, margins, histological features, similarty to other tumors)
Nodular Fasciitis :
*Young adults
*Upper extremities
*Infiltrative margins
*spindle cells
*Loose mucoid matrix
*Vascular proliferation
*Wide collagen bundles
*Large no. of mitotic figures
*Confused with Fibrosarcoma
What is special about Cranial fasciitis ?
Happens in Children and results in bone erosion
Intravascular fasciitis is common in ?
Medium sized arteries and veins
What is the difference between Nodular fasciitis and Proliferative fasciitis ?
In Proliferative fasciitis there are large basophilic ganglion-like cells
What are the charecteristics of Proliferative Myositis ?
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
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 ?
What are the morphological features of Fibromatoses ?
*Large, firm, grayish-white, ill defined lesions
*Irregulr whorled cut surface
*Perivascular lymphocytic infiltrate
What is meants by a Desmoid tumor ?
A superficial tumor
What are the regional charecteristics of Muscuoaponeurotic Fibromatosis (aka Desmoid Tumor) ? Which region shows the best prognosis ? Which gender is affected more often ?
Regions : Shoulder, head, neck, abdomen
Best prognosis : Abdomen
Gender : Much more common in Men
What are 3 forms of Juvenile Fibromatosis ?
*Fibromatosis Colli
*Infantile digital fibromatosis
*Infantile myofibromatosis
Fibromatosis Colli is a type of which Fibormatoses group ?
What are its charecteristics ?
*Part of Juvenile fibromatoses
*Affects the lower third of SCM muscle
*Appears at birth or shortly after
What are the features of Infantile digital fibromatosis?
*Affects exterior surface of the end of phalanges
*Often multiple
*Shows Eosinophilic cytoplasmic inclusions
Does Infantile myofibromatosis occur in a solitary or multiple fashion ?
Either solitary or multiple
Which fibromatosis shows an abrupt transition from a rounded cells area to a spindle cells area ?
Infantily myofibromatosis
What are the other names for the following types of Fibromatoses :
1. Palmar fibromatosis
2. Plantar fibromatosis
3. Penile Fibromatosis
1. Dupuytren
2. Ledderhose
3. Peyronie
What are the charecteristics of LGMFS (Low grade myofibroblastic sarcoma) ?
Mention benign/malignant, age, region, recurrence, metastases, differences from Fibromatoses
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
Was fibrosarcoma a more common diagnosis during the 60's than it is today ?
Yes, in the 60's - very common. Today - RARE
What are the two types of Fibrosarcoma and which is more common ?
Adult, Infantile.
Infantile Fibrosarcoma much more common
When does infantile Fibrosarcoma develop ? Which part of the body does it affect ?
Often congenital, usually develops within the first 2 years
* Mostly affects the distal extremities
What are the pathological features of Fibrosarcoma ?
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
Is Fibrosarcoma + or - for the following IHC markers (S100, EMA, Keratin, Desmin) ?
What IHC feature is only found in the infantile form ?
NEGATIVE for S100, EMA, Keratin, Desmin
* Infantile form may show focal Actin +
Low grade fibromyxoid sarcoma (LGFMS) is a variant of which Malignant tumor ?
Define LGMFS vs. LGFMS
LGMFS - Low grade myofibroblastic sarcoma
LGFMS - Low grade fibromyxoid sarcoma (variant of Fibrosarcoma !)
What are the features of LGFMS ?
*Young adults
*Variable localization
*IHC : Vimentin +
Do Fibrohistocytic neoplasms occur more often in children or in adults ?
Also, do they contain true histiocytes ?
Fibrohistocytic neoplasms occur mostly in Adults
*They do not contain true histiocytes
What are the 2 types of Benign histiocytic neoplasms ?
1. Fibrous histiocytoma (skin = dermatofibroma,deep)
2. Atypical fibroxanthoma
Dermatofibrosarcoma Protuberans (DFSP) and Bednar's tumor are examples of ?
Intermediate malignancy Fibrohistiocytic tumors
Give an example of a malignant Fibrohistiocytic neoplams ?
Malignant fibrous histiocytoma
Fibrous histiocytoma (either skin, or deep) has the following features :
Fibrous Histiocytoma :
*foamy macrophages
*hemosiderin and RBCs
Fibrous histiocytomas IHC markers are :
CD68;Vimentin;FXIIIa : +
CD34 : -
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 ?
Atypical Fibroxanthoma,
a Benign Fibrohistiocytic neoplasm
What are the differences between Dermatofibrosarcoma (DFSP) and Fibrous histiocytoma ?
*No collagen in DFSP
*DFSP IHC : CD34+, Fibrous Histiocytoma IHC : CD34-
*In DFSP Dedifferentiation may occur and MHF may develop (MALIGNANCY)
What is Bednar's tumor and example of and what is its description ?
It is an intermediate-malignancy Fibrohistiocytic neoplasm.
It is a pigmented variant of DFSP (Dermatofibrosarcoma protuberans)
What is the most common diagnosis of a soft tissue sarcoma ?
MFH - Malignant fibrous histiocytoma
What are the variants of MFH ?
*Giant cell
What are the main features of Pleomorphic MFH and when is this diagnosis concluded ?
* Highly anaplastic
*No special light-microscope differentiating sign
*Storiform pattern
*Multinucleated giant cells

This diagnosis is given in case of unclassifiable high-grade sarcomas
What are the features of Myxoidfibrosarcoma (Myxoid MFH) ? Mention age,region, resemblance to other tuumors, cellular features, stroma, IHC marker)
Affects Adults
*Affects limbs (Retroperitoneum = rare)
*High grade resembles pleomorphic MFH
*Stellate and fusiform cells with vacuolisation (PAS+)
*Stroma is myxoid
*IHC : Factor XIII+
What are the names of two Peripheral nerve tumors which do not contain "malignant" in them ?
*Peripherial Primitive Neuroectodermal tumor (Peripheral PNET)
*Clear Cell Sarcoma
Is Traumatic Neuroma a tumor or a TLL ? Also, what are its main features ?
*It's a TLL
*All elements of a nerve can be recognzied
Which benign neoplasm shows two microscopic pattern called "Antoni A" and "Antoni B" ? What are the main differences ?
*Schwanomma (or a neurolimmoma).
*Antoni A : Cellular, with spindle cells (verocay bodies)
*Antoni B : Contains cystic areas, dark hyperchromatic nuclei
*Malignant transformation is very rare
What are the main features of a Neurofibroma ? (Encapsulation, focal or diffuse, special features)
What is neurofibromatosis ?
Neurofibroma :
*Not encapsulated
*Focal or diffuse
*No verocay bodies (contrast with schwanomma)
*Neurofibromatosis is multiple neurofibromas, usually with a malignant trans.
Main charecteristic of a Perineuroma ?
Very long cells arranged in parallel bundles
Name two peripheral nerve neoplasms which are S100 positive
1. Dermal sheath myxoma
2. Granular cell tumor
What is the difference between a Dermal sheath myxoma and a Myxoma ?
In Dermal sheath myxoma there are no epitheloid cells
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+
Granular cell tumor
What are the features of MPNST (Malignant peripheral nerve sheath tumor) (Source, microscopic features, IHC)
*Half arise de novo, half
from Neurofibromas
*Extreme cellular, spindle cells, epitheloid appearance, larg vascular spaces
What is the second name for a Peripheral primitive neuroectodermal tumor (Peripheral PNET) ?
Askins' tumor
What are the features of Peripheral PNET ? (Age, microscopic appearance, IHC)
*Affects young adults
*Uniform round cells containing glyocgen (PAS+)
*IHC : CD99+, NSE+
What tumor is parallel with Ewing's sarcoma of the soft tissue ?
What tumor is parallel with Malignant melanoma of soft tissues ?
*Ewing's sarcoma in soft tissue = Askin's tumor, or Peripheral PNET
*Malignant melanoma in soft tissue = Clear Cell Sarcoma
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 ?
Clear Cell Sarcoma (Malignant melanoma of soft tissues)
What's in common to Hemangioma, Glomus tumor, and Sinonasal Hemangiopericytoma ?
All are BENIGN neoplasms of vessels
What are the tumors of vessels which show intermediate malignancy ?
*Epitheloid hemangioendothelioma
What is in common to Angiosarcoma, Kaposi's sarcoma, and Glomangiosarcoma ?
All are MALIGNANT tumors of vessels
What is the most common location for a Capillary hemangioma ? Which IHC markers would it have ?
Most common location for capillary hemangioma is the skin. Since it is an endothelial neoplasm it is + for CD31,CD34,and FXIII
How is Hemangioma classified ? Mention two special types of Hemangioma and where they appear
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
What are the features of Glomus tumor (Glomangioma) (Localization, pain, microscopic, malignancy change)
*Classic localization is in subinguinal region, but can be elsewhere
*Normal endothelial in blood vesels surrounded by proliferation of 'epitheloid' cells
*May transform to malignant form (glomangiosarcoma)
Main features of Hemangiopericytoma (localization, malignancy, microscopic, IHC)
*Variable localization
*Intermediate malignancy
*IHC : Vimentin+, SMA-, CD34-, FXIIIa+/-
Main features of angiosarcoma (Source, age, variant, invasivness, IHC)
*Angiosarcoma arises from Endothelial cells of blood vessels
*Occurs in adults
*Variant is Lymphangiosarcoma
*Highly invasive
*IHC : FVIII+, CD31+, CD34+
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 ?
Kaposi's Sarcoma
What are the stages of Kaposi's Sarcoma ?
Patch -> Plaqu -> Nodular (with redish color)
How can you tell if an adipose tissue tumor is benign or malignant by its name ?
ONLY malignant adipose tissue tumor is Liposarcoma, all other tumors with 'lipoma' in their name OR 'hibernoma' are BENIGN adipose tissue tumors
What is the most common soft tissue tumor ? Is it benign or malignant ?
Lipoma, Benign
What is the difference in localization between a benign lipoma and a malignant liposarcoma ?
Liposarcomas are DEEP, lipomas are superficial
What are the common features of all lipomas, and which type is special in that it really does contain lipoblasts ?
*Most lipomas are superficial
*Usually encapsulated
*Yellowish, fat like tissue
*Only typical lipomas contain lipoblasts
What is special about the Angiolipoma variant of Lipoma, and what is special about the Angiomyolipoma variant ?
*Angiolipoma : Painful with fat cells and many capillaries
*Angiomyolipoma : Is common in the kidneys
With which malignant neoplasm is the benign tumor Lipoblastoma usually confused ?
What is the difference in age of onset ?
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
What is the origin of a Hibernoma and to what major group does it belong ?
Hibernoma is a subtype of benign adipose tissue tumors, it originates in brown adipose tissue
What is the most common Malignant soft tissue tumor in adults ?
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+ ?
What are the low & high grade variants of a Liposarcoma ?
Low Grade :
*Well defferentiated liposarcoma (resembles Lipoma)
*Myxoid (most common!)
High Grade :
*Round cell (small)
*Pleomorph (highly undifferentiated, contains giant cells)
What are the two components of a dedifferntiated Liposarcoma ?
*A well differentiated liposarcoma and a
*High grade non-lipogenous soft tissue sarcoma (Fibrosarcoma, MFH..)
What is the difference in nomenculture between a smooth muscle neoplasm and a striated muscle neoplasm ?
*Smooth muscle neoplasm contains "Leiomy" in its name
*Striated muscle neoplasm contains "Rhabdomy" in its name
What is the name of the malignant form of a smooth muscle neoplasm ?
A Leiomyosarcoma
What is common to all types of Leiomyoma ?
*Whitish-gray, well circumscribed firm masses
*Made up of fascicles of eosinophilic smooth muscle cells
*show NO mitotic activity *NO necrosis
What are the subtypes of Leiomyoma ?
What is the gross difference between a Leiomyoma and a Leiomyosarcoma ?
*Leiomyosarcomas are larger, softer, and have a tendency for necrosis
What are the features of a Leiomyosarcoma (frequency, microscopic, IHC)
*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+
What is the most common malignant tumor in children ? What is it in adults ?
Most common malignancy in children : Rhabdomyosarcoma
Most common malignancy in adults : Liposarcoma
What are the variants of a Rhabdomyosarcoma ? Which one shows the better prognosis ?
*Embryonal - excellent prognosis
*Alveolar - poor prognosis
Which rare neoplasm shows a localization to ther oral cavity, is found in adults, and contains large, round, well differentiated cells ?
What are the variants of Embryonal Rhabdomyosarcoma and what is their localization ?
*Embryonal : Head&neck, Genitourinary tract, extremities
*Botryoid : Genitourinary
*Spindle cell : Paratesticular
*Anaplastic : Lower extremities
What is the gross difference between a botryoid variant of an embryonal Rhabdomyosarcoma and the other variants ?
Other variants are fleshy, solid and well circumscribed, usually firmer.
The botryoid variant is softer and grows as grape like vesicles
What is the microscopic criteria for a Spindle shaped variant of an embryonal rhabdomyosarcoma, and what is it for an Anaplastic varaint ?
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
What are the features of an Alveolar Rhabdomyosarcoma (Age, localization, growth pattern, microscopic) ?
*Young patients
*Affects extremities, head/neck, trunk
*Grows in nests or clusters seperated by septa (like in the lung...)
*Variable cell types
What are the IHC markers of Rhabdomyosarcoma ?
Rhabdomyosarcoma :
SMA-, Desmin+, MyoD1+, Vimentin+, Myoglobin+
What are the benign forms of chondromatous tumors ?
*Myositis ossifican
*Fibrodysplasia ossificans
What are the common features of chondromatous tumors ?
*Very rare
*Arises from Chondromatous or osseous differentiation of pleuripotent mesenchymal cells
What are the benign types of synovial tumors ?
*Localized tenosynovial tumor (or Giant cell tumor of tendon sheaths)
*Diffuse tenosynovial tumor (or Soft tissue pigmented villnodular synovitis)
What is the only malignant form of a synovial tumor ?
*Malignant tenosynovial tumor
Synovial Sarcoma is NOT a synovial neoplasm !!!
What is the localization of a localized tenosynovial tumor (giant cell tumor of tendon sheaths) ?
Hands, Feet
What are the differences between a localized and a diffuse tenosynovial tumor ?
*Localized only : Circumscribed
*Diffuse only : Many xanthoma cells, hemosiderin pigmentation
How common is a malignant tenosynovial tumor ?
Rare, based on diagnosis of exclusion
What are myxomas ?
Myxomas are benign/intermediate malignancy neoplasms of uncertain differentation
Name malignant tumors of uncertain differenation and/or those which source is non-mesenchymal
*Small cell tumor with desmoplastic stroma
*Synovial sarcoma(!!)
*Epitheloid sarcoma
*Extrarenal rhabdoid tumor
What are the features of a Myxoma ?
*Have an Acid mucopolysaccharide matrix
*Many cases it is unknown whether it is neoplastic or reactive
*Best known tumor is Intramascular myxoma
Is an ossifying fibromyxoid tumor benign or malignant ?
Is it encapsulated ?
What is its IHC marker ?
*Benign tumor
*Encapsulated, well circumscribed
What are the two forms of Synovial sarcomas ?
BOTH DO NOT ARISE FROM JOINTS, but not far from them
What are the main features of Synovial sarcomas (Microscopic, age, gender, localization)?
*Spindle cells express EMA, cytokeratins == not found in normal synovial tissue
*Occurs in young adults, mostly males
*Localization to extremities
What are the two components of Biphasic Synovial sarcoma ?
*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
Which component of the biphasic synovial sarcoma is found in monophasic synovial sarcoma ?
The Spindle cells component
What is the cytogenic alteration arising in Synovial sarcoma ?
What are the IHC markers for synovial sarcoma ?
Translocation : X;18
IHC : S100+, EMA+
What are the features of an Alveolar soft part sarcoma (Age, metastasis, growth rate, microscopic, IHC)
*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)
What are the features of Epitheloid sarcoma (origin, age, variants, microscopic, IHC)
*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+

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