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Skin MS: Week 5 > Pathology: Soft Tissue Tumors > Flashcards

Pathology: Soft Tissue Tumors Flashcards

1
Q

Definition of soft-tissue tumors

A

Defined as mesenchymal proliferations that occur in the extraskeletal, nonepithelial tissues of the body, excluding organs, coverings of the brain, and lymphoreticular system.

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2
Q

Classification of soft-tissue tumors

A

the types of tissue that they recapitulate or differentiate to (e.g. fat, fibrous tissue, muscle, vessels, nerves). In each histogenetic category, they can be divided into benign and malignant (sarcoma) forms.

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3
Q

Soft-tissue tumor locations

A

Soft-tissue tumors can arise in any location; approximately 40% occur in the lower extremities, 20% in the upper extremities, 10% in the head and neck, and 30% in the trunk and retroperitoneum.

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4
Q

Benign vs malignant soft-tissue tumor prevalence

A

Benign soft-tissue tumors outnumber malignant soft-tissue tumors by 100:1. Sarcomas are relatively rare, accounting for 0.8% of all invasive malignancies, but 2% of all cancer deaths.

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5
Q

Soft-tissue malignancy metastasize pattern

A

In contrast to carcinomas, sarcomas usually metastasize via hematogenous routes (e.g. to lung and bone). - rare to lymphnodes

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6
Q

malignant soft-tissue tumor called

A

sarcoma

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7
Q

type of cell that give rise to soft-tissue tumors

A

Soft tissue tumors are believed to develop due to mutations in mesenchymal stem cells distributed throughout the body.

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8
Q

State the most common type of genetic abnormality in soft tissue tumors.

A

translocation –> fusion genes that encode chimeric transcription factors, leading to transcriptional deregulation (uncontrolled cell proliferation).
Other genetic abnormalities result in deregulation of kinase signaling.

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9
Q

Neurofibromatosis Type 1 –>

A

neurofibroma, malignant peripheral nerve sheath tumor

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10
Q

Gardner Syndrome –>

A

Fibromatosis

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11
Q

Osler-Weber-Rendu syndrome –>

A

telangiectasia

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12
Q

Environmental factors implicated in development of soft tissue tumors

A

Trauma, environmental agents, radiation therapy, viruses, chronic disease

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13
Q

dioxin –>

A

various sarcomas

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14
Q

vinyl chloride –>

A

hepatic angiosarocma

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15
Q

post-radiation –>

A

pleomorphic sarcoma/malignant fibrous histiocytoma, osteosarcoma

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16
Q

HHV8 –>

A

Kaposi sarcoma

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17
Q

Chronic lymphedema

A

Angiosarcoma

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18
Q

Some specific types of sarcoma tend to occur in certain _____. In general, incidence of sarcomas increases with ___.

A

Some specific types of sarcoma tend to occur in certain age groups. In general, incidence of sarcomas increases with age.

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19
Q

Factors that influence prognosis

A 
Histologic classification
Histologic grade
Stage of tumor
Superficial vs. deep
Size
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20
Q

Initial morphologic assessment is based on the ____________ of the tumor cells as well as the _______________. Numerous immunohistochemical stains are used in the histologic assessment of some tumors. In some instances, genetic analysis for specific fusion genes can aid in diagnosis.

A

Initial morphologic assessment is based on the cytologic appearance of the tumor cells as well as the architectural pattern of growth. Numerous immunohistochemical stains are used in the histologic assessment of some tumors. In some instances, genetic analysis for specific fusion genes can aid in diagnosis.

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21
Q

Histologic grade is based on the ______, ___________, and amount of _________

A

Histologic grade is based on the tumor differentiation score, mitotic count, and amount of tumor necrosis

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22
Q

Staging of soft-tissue sarcomas is based on _________ presence or absence of ___________, and presence or absence of __________. Most sarcoma metastases are ____________.

A

Staging of soft-tissue sarcomas is based on tumor characteristics (superficial or deep, size less than or greater than 5 cm), presence or absence of lymph node metastases (although lymph node metastases are rare in sarcomas,), and presence or absence of distant metastases (so staging is a T,N,M classification). Most sarcoma metastases are hematogenous.

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23
Q

Tx benign soft-tisue tumors

A

surgery (simple excision)

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24
Q

soft tissue sarcoma tx

A

surgery, with radiation therapy and chemotherapy used in certain clinical situations. Sometimes radiation therapy and/or chemotherapy is done before surgical excision (preoperative or neoadjuvant therapy)

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25
Q

Imaging/lab and soft-tissue tumors

A

Imaging studies (such as MRI) are often used in the assessment of soft-tissue tumors. Needle biopsy is often used to obtain diagnosis and plan therapy for large tumors.

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26
Q

Lipoma

A

most common soft tissue of tumor of adults (rare younger than 20)

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27
Q

Conventional lipoma

A

most common type, mature fat only

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28
Q

Angiolipoma

A

mature fat plus small vessel proliferation; can be painful

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29
Q

Spindle cell lipoma/Pleomorphic lipoma:

A

mature fat plus spindle cells/pleomorphic cells

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30
Q

Myolipoma

A

mature fat plus smooth muscle

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31
Q

Chondroid lipoma:

A

mature fat plus cartilage-like cells

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32
Q

Angiomyolipoma:

A

mature fat plus vessels plus smooth muscle (typically associated with the kidney, and 1/3 occur in patients with tuberous sclerosis)

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33
Q

Myelolipoma:

A

mature fat plus bone marrow elements (usually adrenal gland)

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34
Q

lipoma types that typically occur in a superficial location and are well circumscribed and encapsulated.

A

conventional lipoma, angiolipoma, myolipoma

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35
Q

Intramuscular lipoma:

A

infiltrating lipoma involving skeletal muscle

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36
Q

Lipoblastoma/Lipoblastomatosis:

A

refer to circumscribed (lipoblastoma) and diffuse (lipoblastomatosis) forms of fatty proliferation, usually occurring in infants

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37
Q

Liposarcoma

A

one of the most common sarcomas of adulthood and typically occurs in middle to older age adults. This tumor usually arises in the deep soft tissues of the proximal extremities and retroperitoneum.

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38
Q

Morphologic variants of liposarcoma

A

Well-differentiated liposarcoma (relatively indolent, local recurrence, but can be fatal when located in the retroperitoneum); when this tumor occurs in a location amenable to surgical excision (such as an extremity or on the trunk) it may be called an “atypical lipomatous tumor”

Myxoid/round cell liposarcoma (intermediate malignancy)

Pleomorphic liposarcoma (aggressive tumor, frequently metastasizes)

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39
Q

well circumscribed encapsulated mass of mature adipose tissue

A

conventional lipoma

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40
Q

longterm corticosteroid use –>

A

overgrowth of mature adipose tissue in the face (moon face) and on the back (buffalo hump), called “steroid lipomatosis”

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41
Q

________ are non-neoplastic, benign lesions that develop following local trauma/injury or are idiopathic. Clinically, they are worrisome as they grow rapidly and can have an infiltrative appearance. Two types.

A

Reactive pseudosarcomatous proliferations are non-neoplastic, benign lesions that develop following local trauma/injury or are idiopathic. Clinically, they are worrisome as they grow rapidly and can have an infiltrative appearance. Two types.

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42
Q

Two types reactive pseudosarcomatous proliferations

A

Nodular fasciitis, myositis ossificans

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43
Q

Usually on upper extremities (forearm) or trunk. At any age, usually young adults that present with a several week history of a solitary, rapidly growing, sometimes painful mass; history of previous trauma is present in 10-15%.

dermis, subcutis, or muscle
nodular, yet poorly marginated, cellular proliferation of immature fibroblasts and myofibroblasts in a myxoid stroma
Mitotic figures are numerous -can be confused with a true sarcoma.
Following excision, the lesion rarely recurs.

A

Nodular Fasciitis

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44
Q

athletic adolescents and young adults
follows episode of trauma in more than 50% of cases
typically found in muscles of the proximal extremities
often 3-6 cm in size.
Initially, lesion is cellular and consists of plump fibroblasts and myofibroblasts as seen in nodular fasciitis. Overtime, the outer margin of the lesion develops osteoblasts, which then deposit mineralized bone in the outermost layer (ossification). Eventually the lesion can become completely ossified. In the proliferative stage, the lesion can be confused with an extraskeletal osteosarcoma.

A

Myositis ossificans

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45
Q

Nodular Fasciitis:

Usually on ________. At any age, usually _______ that present with _______ ___________ mass. 10-15% hx of ____________.

A

Usually on upper extremities (forearm) or trunk. At any age, usually young adults that present with a several week history of a solitary, rapidly growing, sometimes painful mass; history of previous trauma is present in 10-15%.

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46
Q

Nodular Fasciitis:
____, _____, or _______
Circumscribed?
_________, cellular proliferation of immature ___ and ______ in a myxoid stroma
_____ figures are numerous -can be confused with a true _______.
Following excision, the lesion ____________.

A

dermis, subcutis, or muscle
nodular, yet poorly marginated, cellular proliferation of immature fibroblasts and myofibroblasts in a myxoid stroma
Mitotic figures are numerous -can be confused with a true sarcoma.
Following excision, the lesion rarely recurs.

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47
Q 
Myositis Ossificans:
population - \_\_\_\_\_\_\_\_\_ and \_\_\_\_\_\_\_\_\_\_\_
follows \_\_\_\_\_\_\_\_\_\_ in more than 50% of cases
typically found in \_\_\_\_\_\_\_\_
often\_\_\_\_\_\_\_\_\_ in size.
A

athletic adolescents and young adults
follows episode of trauma in more than 50% of cases
typically found in muscles of the proximal extremities
often 3-6 cm in size.

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48
Q

Myositis Ossificans:
Initially, lesion is ___ and consists of plump _________ and __________ as seen in __________. Overtime, the outer margin of the lesion develops _______, which then deposit _________ in the outermost layer (_________). Eventually the lesion can become completely _________. In the proliferative stage, the lesion can be confused with an ______________

A

Initially, lesion is cellular and consists of plump fibroblasts and myofibroblasts as seen in nodular fasciitis. Overtime, the outer margin of the lesion develops osteoblasts, which then deposit mineralized bone in the outermost layer (ossification). Eventually the lesion can become completely ossified. In the proliferative stage, the lesion can be confused with an extraskeletal osteosarcoma.

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49
Q

benign fibroblastic neoplasms involving the fascia or aponeuroses (broad tendons); lesions characterized by nodular or poorly defined broad fascicles of fibroblasts and myofibrobasts surrounded by abundant dense collagen. Many types exist, defined by location

A

Superficial fibromatoses

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50
Q

Superficial Fibromatoses: _____ fibroblastic neoplasms involving ______ or _________
lesions characterized by ____ or ____ ______ broad fascicles of _____ and _______ surrounded by abundant ________. Many types exist, defined by location

A

benign fibroblastic neoplasms involving the fascia or aponeuroses (broad tendons); lesions are characterized by nodular or poorly defined broad fascicles of fibroblasts and myofibrobasts surrounded by abundant dense collagen. Many types exist, defined by location

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51
Q

3 most common fibrous tumors and tumor-like lesions

A

Palmar fibromatosis (Dupuytren’s contracture), Plan, Penile (Peyronie)

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52
Q

These benign tumors consist of an infiltrative, poorly marginated proliferation of bland fibroblasts with collagen. While these lesions do not metastasize, they can recur if incompletely excised. They may occur at any age but are most frequent in the teens to 30’s, and some are associated with a history of previous trauma.

A

Deep-Seated Fibromatosis (Desmoid tumors)

53
Q

Desmoid Tumors: ____ tumors consist of an ____, _____ proliferation of ____ with ______. While these lesions ___ metastasize, they ____ recur if incompletely excised. They may occur at any age but are most frequent in the ______, and some are associated with a history of ________.

A

These benign tumors consist of an infiltrative, poorly marginated proliferation of bland fibroblasts with collagen. While these lesions do not metastasize, they can recur if incompletely excised. They may occur at any age but are most frequent in the teens to 30’s, and some are associated with a history of previous trauma.

54
Q

3 main types desmoid tumors

A

Abdominal fibromatosis
Extra-abdominal
Intra-abdominal

55
Q

Typically occurs in the musculoaponeurotic structures of the anterior abdominal wall in women during or after pregnancy.

A

Abdominal fibromatosis

56
Q

occurs in men and women with equal frequency and arises principally in the musculature of the shoulder, chest wall, back, and thigh

A

extra-abdominal fibromatosis

57
Q

typically occurs in the mesentery or pelvic side walls, often in individuals having familial adenomatosis polyposis (Gardner syndrome)

A

Intra-abdominal fibromatosis

58
Q

intra-abdominal fibromatosis associated with

A

familial adenomatosis polyposis (Gardner Syndrome)

59
Q

Virtually all deep-seated fibromatoses have ________ or _________ gene mutations

A

Somatic beta-catenin or adenomatous polyposis coli (APC)

60
Q

malignant, sarcomatous tumors of fibroblasts. These tumors can occur anywhere in the body, but are most common in the deep soft tissues of the extremities

A

Fibrosarcomas

61
Q

Fibrosarcomas are _______ malignancies, recurring in ___ of cases and metastasizing in ____

A

Fibrosarcomas are aggressive malignancies, recurring in more than 50% of cases and metastasizing in more than 25%

62
Q 
Benign fibrous histiocytoma
Very common, legs
Dermal proliferation of histiocytes/fibrocytes
Pinch test - indents
Tan/brown papules - not scaly, relatively normal
Etiology unknown (bug bites)
PUNCH
In dermis
A

dermatofibroma (benign fibrous histiocytoma)

63
Q 
Sarcoma of low malignant potential 
Usually younger adults/children (nodular usually)
Rarely metastasize/locally aggressive
Protuberant nodule within firmer plaque
Dense proliferation in dermis
PUNCH
A

dermatofibrosarcoma protuberans

64
Q

contain cellular elements that resemble both fibroblasts and histiocytes (tissue macrophages). As the phenotype of the neoplastic cells most closely resemble fibroblasts, the term fibrohistiocytic should be regarded as a descriptive term, not one indicating the cell of origin.

A

Fibrohistiocytic tumors

65
Q

kinds of fibrohistiocytic tumors

A

dermatofibroma, dermatofibrosarcoma protuberans
Malignant fibrous histiocytoma (group)
**Getting split into other classifications (fibroblast neoplasm)

66
Q

storiform

A

splindle-cells radiating out like spindles

67
Q

benign tumor showing skeletal muscle differentiation, very rare (heart or other sites)

A

rhabdomyoma

68
Q

most common soft-tissue sarcoma of childhood and adolescence - malignant tumor showing skeletal muscle differentiation

A

Rhabdomyosarcoma

69
Q

3 main types of rhabdomyosarcoma

A

Embryonal rhabdomyosarcoma
Alveolar rhabdomyosarcoma
Pleomorphic rhabdomyosarcoma

70
Q

most common subtype (60%) rhabdomyosarcoma. usually occurs in children age 10 years or younger, and often arises in the head and neck (e.g. nasal cavity, orbit, middle ear) and genitourinary tract.
botryoid subtype of embryonal rhabdomyosarcoma arises in the mucosal lining of hollow organs forming a polypoid, grape like growth (e.g. nasal cavity, urinary bladder, vagina).

A

Embryonal rhabdomyosarcoma

71
Q

subtype rhabdomyosarcoma - tends to occur in early to middle adolescence, commonly arising in deep soft-tissues of the extremities, and represents 20% of rhabdomyosarcomas.

A

alveolar rhabdomyosarcoma

72
Q

Genetic basis for prognosis w/ alveolar rhabdomyosarcoma

A

Specific cytogenetic abnormalities have been identified, some of which connote a worse prognosis. For example, patients with metastatic disease have a four year survival of 75% with a PAX7/FKHR fusion gene vs. only 8% with a PAX3/FKHR fusion gene.

73
Q

the rarest of the three subtypes (rhabdomyosarcoma), this sarcoma usually arises in the deep soft-tissues of adults.

A

pleomorphic rhabdomyosarcoma

74
Q

Rhabdomyosarcomas are______ malignancies, and are often treated with multimodal therapy (____ _____ _____). ______, _______, _______ and _______ influence survival, with the _____ subtype having the best chance of survival. ___ and ____ subtypes (especially with the _______) are more aggressive.

A

Rhabdomyosarcomas are very aggressive malignancies, and are often treated with multimodal therapy (surgery, chemotherapy, radiation therapy). Histologic subtype, size (smaller is better), patient age (infant/child better than adult), and location influence survival, with the botryoid subtype having the best chance of survival. Pleomorphic and alveolar subtypes (especially with the unfavorable genotype) are more aggressive.

75
Q

The benign tumor showing smooth muscle differentiation

A

leiomyoma

76
Q

most common places leiomyoma

A

They most commonly arise in the uterus, are present in 77% of women, and are often called “uterine fibroids.” They also arise from the arrector pili muscles in the skin, nipples, scrotum, and labia and less frequently develop in deep soft tissues and GI tract. Leiomyomas of the GI tract and uterus are usually well circumscribed tumors.

77
Q

malignant tumor showing smooth muscle differentiation

A

leiomyosarcoma

78
Q

leiomyosarcoma locations

A

They most frequently occur in the uterus or GI tract; soft tissue leiomyosarcomas occur in the retroperitoneum, skin, and deep soft tissues of the extremities.

79
Q

Histology of smooth muscle tumors

A

Both tumors consist of spindle cells showing histologic and immunohistochemical features of smooth muscle, arranged in fascicles that tend to intersect at right angles. Leiomyosarcoma shows increased nuclear pleomorphism and increased mitotic activity compared to the leiomyoma.

80
Q

Most leiomyomas NOT found in soft tissue - but in

A

uterus (uterine fibroids)

81
Q

Benign tumor and tumor-like conditions of blood vessels and lymphatics examples

A

Hemangioma
Pyogenic granuloma (eruptive hemangioma)
Lymphangioma

82
Q

most often occur in the skin, but any site is possible (e.g. liver); morphologically see various subtypes, such as capillary (skin), and cavernous (skin, deep tissues, liver, spleen).

A

Hemangioma (blood vessel tumor)

83
Q

typically occur in skin and subcutaneous soft tissue, look like a thin walled hemangioma without the red blood cells. Many of these occur in the neck or axilla of children. cystic hygroma (cavernous lymphangioma) of the neck in children can be associated with Turner syndrome

A

Lymphangioma

84
Q

Lymphangioma associated w/

A

Turner syndrome (cavernous lymphangioma)

85
Q

benign, typically painful tumor showing differentiation to the modified smooth muscle cells of the glomus body (arteriovenous structure involved in thermoregulation). These lesions arise most often in the distal digits.

A

Glomus tumor and glomangioma

86
Q

Special types of vascular ectasias

A

Nevus flammeus (port wine stain one type of these)
Spider Angiomas
Hereditary Hemorrhagic Telangiectasia

87
Q

Usually occur in head/neck of infant most regress. Port Wine Stain special type - does not regress. Those in trigeminal nerve can be associated w/ Sturge-Weber syndrome.

A

Nevus Flammeus.

88
Q

Nevus Flammeus associated with

A

if in trigeminal nerve association - Sturge-Weber syndrome (MR, seizures, masses of leptomeninges)

89
Q

skin spider-like vascular malformations seen in association w/ estrogen excess (pregnancy, cirrhosis)

A

Spider Angiomas

90
Q

Spider angiomas seen in association w/

A

excess estrogen (pregnancy, cirrhosis)

91
Q

autosomal dominant disorder with widely distributed vascular ectasias over the skin and oral mucous membranes, as well as in the respiratory, GI, and urinary tracts.

A

Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Disease)

92
Q

reactive vascular proliferation resulting from opportunistic infection from Bartonella bacteria in immunocompromised individuals. Lesions can occur anywhere in the body, and regress with successful antibiotic therapy.

A

bacillary angiomatosis

93
Q

are aggressive, malignant sarcomas showing endothelial differentiation

A

Angiosarcoma

94
Q

Angiosarcoma location

A

lesions occurring at any site but often involving skin (head and neck, especially the scalp), soft tissue, breast, and liver.

95
Q

Angiosarcoma associated with

A

Patients with lymphedema following axillary lymph node resection for breast cancer can get an angiosarcoma which presumably arises in association with dilated lymphatics. Angiosarcomas can also be secondary to previous radiation therapy.

96
Q

group of very rare malignant vascular tumors with clinical behavior less aggressive than angiosarcoma.

A

Hemangioendotheliomas

97
Q

malignant vascular tumor with an infectious etiology, caused by human herpesvirus-8 (HHV-8). It is believed that the virus, in concert with a cofactor, results in endothelial cell proliferation.

A

Kaposi’s sarcoma

98
Q

4 kinds of Kaposi’s Sarcoma

A

Chronic (classic), Lymphoadenopathic, Transplant-associated, AIDS-associated

99
Q

occurs in older men of Eastern European (especially Ashkenazi Jews) or Mediterranean descent. While the tumor can be associated with a second malignancy or altered immunity, it is not associated with HIV infection. The lesions are typically found in the skin and subcutaneous tissues of the lower extremities, do not progress further, and are asymptomatic.
Kaposi sarcoma

A

Chronic KS (classic or European KS)

100
Q

KS form - is endemic in Africa and afflicts young males and children, with involved lymph nodes and occasional organ involvement; this is an aggressive tumor.

A

Lymphadenopathic KS

101
Q

KS occurs in the setting of solid organ transplant with long-term immunosuppression; this is an aggressive tumor

A

Transplant-Associated KS

102
Q

KS - originally found in one-third of AIDS patients, incidence is now less than 1% due to the success of antiretroviral therapy. This tumor can involve lymph nodes and organs.

A

AIDS-associated KS

103
Q

caused by repeated minor trauma, typically of the interdigital plantar nerve between the third and fourth toes. This results in a distorted nerve, with concentric perineural fibrosis.

A

Morton neuroma

104
Q

when a peripheral nerve is severed or crushed, the proximal end regenerates. If the proximal end cannot reconnect with the distal end, a tangled mass of nerve fibers is produced.

A

Traumatic neuroma

105
Q

from neural crest-derived Schwann cell, benign tumors, typically encapsulated, well circumscribed and arise adjacent to a nerve. Some schwannomas are associated with neurofibromatosis type 2 (NF2).

A

Schwannoma

106
Q

Schwannoma associated with

A

Neurofibromatosis type 2

107
Q

benign tumor that presents as discrete localized masses – most commonly as a cutaneous form or in a peripheral nerve as a solitary form – or as an infiltrative lesion growing within and expanding a peripheral nerve (plexiform)

A

Neurofibroma

108
Q

The presence of multiple neurofibromas or plexiform neurofibroma strongly suggests the diagnosis of ________. Plexiform neurofibromas may result in significant neurologic deficits and have a significant potential for malignant transformation.

A

neurofibromatosis type 1 (NF1)

109
Q

highly malignant sarcomas usually arise in association with major nerve trunks → proximal portions of the upper and lower extremities and trunk.

A

Malignant peripheral nerve sheath tumor

110
Q

MPNST associated with

A

NF1

111
Q

Neurofibromatosis

___ ___ heredity w/ _____ expression

A

Autosomal dominant w/ variable expression

112
Q

Neurofibromatosis

Affects males vs females

A

equally

113
Q

Neurofibromatosis
Type 1 vs Type 2
prevalence, mutations

A

Type 1 - more common, chromosome 17 coding for neurofibromin (tumor suppressor)

Type 2 - less common, chromosome 22 coding for merlin (regulated membrane receptor signaling)

**Both act as growth suppressors

114
Q

NF1 Associated w/

A

Café au lait spots: coffee-colored skin macules (can also occur in NF2 but usually quite infrequent); axillary and inquinal freckling
Optic gliomas, astrocytomas
Lisch nodules (pigmented hamartomas of the iris)
Osseous lytic lesions
Plexiform neurofibromas (do not occur in NF2), can get sarcomatous transformation as discussed previously
Cutaneous/subcutaneous neurofibromas
Association with pheochromocytoma, Wilm’s tumor, juvenile CML
Neurodevelopmental problems

115
Q

NF2 associated w/

A

Bilateral acoustic schwannomas (cranial nerve VIII)

Meningiomas, spinal schwannomas, juvenile cataracts.

116
Q

benign tumors that occur either as a localized or diffuse form, and have a characteristic fusion gene abnormality. Mixture of giant cells, round histiocytic cells, collagen, foam cells, hemosiderin.

A

Tenosynovial giant-cell tumor

117
Q

solitary slow-growing painless mass, frequently involves tendon sheaths of fingers and wrists. Most common mesenchymal neoplasm of hand.

A

Localized, Giant cell tumor of tendon sheaths

118
Q

usually in knee/ankle/foot, pignmented villonodular synovitis when involving joint space. Rare.

A

Diffuse form of tenosynovial giant-cell tumor

119
Q

a misnomer, as this malignant tumor does not show synovial cell differentiation. Lesion uncommon in joint cavities, encountered in areas with no apparent relation to synovial structures. Primarily occurs in para-articular regions of the extremities in adolescents and young adults, and less commonly in the head and neck region.

A

Synovial sarcoma

120
Q

Dx Synovial sarcoma

A

There are often calcifications within the tumor which can be seen radiographically. Immunohistochemistry is helpful in the diagnosis as these tumors show positivity for cytokeratin (an epithelial marker).
There is also a characteristic fusion gene abnormality (SYT-SSX fusion gene).

121
Q

Two types synovial sarcoma

A

Microscopically, there are two types, a biphasic type with both epithelial and spindle cell components, and a monophasic type with spindle cell component only.

122
Q

Myxomatous lesions

A

extracullar mucin accumulation with associated cellular proliferation

123
Q

One myxoid lesion which is very common is the ________ which occurs as a cystic area of myxoid degeneration (not a neoplasm) in fibrous connective tissue, almost always located near a joint capsule or tendon sheath (wrist is the most common location).

A

ganglion cyst

124
Q

On fingers, ganglion cysts known as

A

digital mucus cysts

125
Q

Common benign mesenchymal/soft tissue tumors

A

lipomas, hemangiomas, fibrous histiocytoma (dermatofibroma), Dupuytren’s contracture, ganglion cyst, giant cell tumor of tendon sheath, leiomyomas (uterine)

126
Q

Common adult sarcomas

A

undifferentiated pleomorphic sarcoma/MFH, liposarcoma, and various fibrosarcoma variants

127
Q

Common childhood sarcomas

A

rhabdomyosarcoma, extraskeletal Ewings sarcoma/PNET, and neuroblastoma

128
Q

Key clinical questions to ask regarding soft tissue neoplasm or tumor

A

1) what are the chances of local recurrence, and 2) what are the chances of distant metastases?

Skin MS: Week 5 (6 decks)

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