Glossary of 3rd year Soft Tissue Tumors
Other Decks By This User
- 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
- 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
- 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
*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
*Infiltration of fat and muscle at the periphery
*osteoclast-type giant cells
- *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 :
*Loose mucoid matrix
*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
- 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
*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
- 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 ?
- LGFMS :
*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 :
*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 ?
- What are the main features of Pleomorphic MFH and when is this diagnosis concluded ?
- * Highly anaplastic
*No special light-microscope differentiating sign
*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
*BOTH types are ENCAPSULATED
*Malignant transformation is very rare
- What are the main features of a Neurofibroma ? (Encapsulation, focal or diffuse, special features)
What is neurofibromatosis ?
- Neurofibroma :
*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
*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
*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
*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
*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 ?
- 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
*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
*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+
You must Login or Register to add cards