Ch12 - Malignant Flashcards by Austin Shackelford

What range (%) of fibrosarcomas occur in the head and neck? Where do most occur in general? Where do most occur in the head and neck?

10-19%…most occur in the extremities…nose / paranasal sinuses

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Buzz word for fibrosarcoma histology

“herringbone” pattern

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IHC for a fibrosarcoma should all be negative EXCEPT what two? (Fibrosarc is a dx of exclusion)

Vimentin and SMA (minimal staining)

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Fibrosarcoma recurrence? 5 year survival rate? (% range)

50% recur, 40-70% 5 yr survival

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What is the other term for Undifferentiated Pleomorphic Sarcoma?

Malignant fibrous histiocytoma

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___________ are a heterogeneous group of tumors from this former family, whose line of differentiation cannot be determined

Undifferentiated pleomorphic sarcomas

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Undifferentiated pleomorphic sarcoma: age? location?

older…deep tissues of trunk or extremities

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What is the most commonly described histologic pattern for Undifferentiated pleomorphic sarcoma?

storiform-pleomorphic type

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Undifferentiated pleomorphic sarcoma is considered to be an aggressive tumor that is usually treated by radical surgical resection. Approximately ___% of patients with head and neck tumors will have local recurrence, and almost ___% will develop metastases. The overall 5-year survival rate is 52% to 55%.

40% recur…30% met

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What is the most common soft tissue sarcoma?

LIPOSARCOMA (17% to 30% of all soft tissue malignancies in adults)

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What are the 3 most common sites for Liposarcoma? What % are in the head and neck?

thigh, retroperitoneum, inguinal…3% in head and neck

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Liposarcomas are seen primarily in _____; the mean age for head and neck tumors is ____ years.

adults…57 years

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What is the most common head and neck location for liposarcoma? What 2 intraoral sites?

neck…tongue and cheek

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What are the 4 variants of liposarcoma?

well-diff 2.myxoid/round cell 3.pleomorphic 4.dedifferentiated

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55-90% of all cases of liposarcoma in the oral cavity are which variant?

well-differentiated liposarcoma

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What histological variant of liposarcoma is related to myxoid liposarcoma, but is more aggressive?

round cell liposarcoma

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around ___% of all liposarcomas recur. The overall 5-year survival rate ranges from ___% to 70%. There is a 10-year survival rate of approximately 50%. The histopathologic subtype is extremely important in predicting the prognosis; the outlook for pleomorphic liposarcomas is much worse than for what two variants?

50% recur…59-70% survival…myxoid and well-differentiated tumors much better prognosis

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The prognosis for oral liposarcoma is __________ because of the predominance of ___________… Local recurrence has been reported in ___% to ____% of cases, but metastasis and death as a result of tumor is rare.

more favorable….well-differentiated subtypes…15-20% recurr

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What are the three names for malignant peripheral nerve sheath tumor?

malignant schwannoma, neurofibrosarcoma, neurogenic sarcoma

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Half of the cases of a malignant peripheral nerve sheath tumor occur in what patient population?

NF1 patients

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What % range of malignant peripheral nerve sheath tumors occur in the head and neck?

14-19%

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Malignant peripheral nerve sheath tumors are most common in ________. The mean age in patients with neurofibromatosis (____ to _____ years) is about one decade younger than in those without this condition

young adults…29 to 36 in NF1 vs 40-46 w/o neurofibromatosis 1

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What are three most common sites for Malignant peripheral nerve sheath tumors in the oral cavity?

mandible, lips, buccal mucosa

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Name that tumor: Radiographic examination of intraosseous tumors of the mandible may reveal widening of the mandibular canal or the mental foramen, with or without irregular destruction of the surrounding bone.

Malignant peripheral nerve sheath tumor

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The _________ is a rare neuroectodermal | neoplasm of the upper nasal vault that shows some similarities to neuroblastomas seen elsewhere in the body.

olfactory neuroblastoma

olfactory neuroblastoma arise from sensory olfactory _________ cells.

neuroepithelial

Unlike the usual neuroblastoma, the olfactory neuroblastoma is rare in patients younger than the age of ___ years. Instead, it is more common in adults, with a mean age at diagnosis of ___ to ___ years.

10...45 to 56

________ is a rare malignancy of vascular endothelium, which may arise from either blood or lymphatic vessels.

Angiosarcoma

More than ____% of all cases of angiosarcoma occur in the head and neck region, with the scalp and forehead being the most common sites. Oral lesions are quite rare

50%

The term ____________ is used to describe vascular tumors with microscopic features intermediate between those of hemangiomas and angiosarcomas. Such tumors also are rare and are considered to be of INTERMEDIATE malignancy (whatever the fuck that means)

hemangioendothelioma

What are the two more common sites for angiosarcoma in the oral cavity?

tongue and mandible

What three stains are used in angiosarcoma?

CD31 pos, factor VIII and CD34 (less consistent)

The prognosis for angiosarcoma of the face and scalp is poor, with a reported 10-year survival rate of only ___ to ___%.....angiosarcomas of the oral cavity and salivary glands appear to have a ______ prognosis

14% to 21%...better

Kaposi sarcoma is caused by infection with _______. The lesion most likely arises from _______ cells, which may express markers for both LYMPHATIC and BLOOD VESSEL differentiation

human herpesvirus 8 (HHV-8; Kaposi sarcoma-associated herpesvirus [KSHV])....endothelial

What are the 4 clinical presentations of Kaposi sarcoma?

1.classic 2.endemic (African) 3.iatrogenic (transplant-associated) 4. Epidemic (AIDS-related)

Classic (chronic) Kaposi sarcoma is primarily a disease of ____ adult life, and ____ to ____% of cases occur in MEN

late...70% to 95%

Classic KS typically occurs on the skin of _______...if found intraorally where is it found?

lower extremities..palate

Endemic Kaposi sarcoma was recognized as a relatively common neoplasm of WHAT AGE GROUP? in sub-Saharan Africa.

younger adults and children

Although _______ Kaposi sarcoma used to be the most common form of the disease seen in Africa, epidemic (AIDS-related) Kaposi sarcoma is seen more frequently now.

endemic

3 stages of kaposi sarcoma lesions

1. patch 2 plaque 3.nodular

What % of soft tissue sarcomas are leiomyosarcoma?

Age range of leiomyosarcoma

middle aged and older adults

Smooth muscle tumors with more then ____ mitoses per 10 HPF should be considered malignant

Leiomyosarcomas are positive for 4 IHC markers and which special stain?

1. desmin 2.muscle specific actin 3.smooth muscle myosin(SMMS) 4. SMA 5.PAS highlights glycogen in the cytoplasm

Half of all oral leiomyosarcomas occur where?

jaw bones

50% of soft tissue sarcomas in children are _____sarcomas

rhabdomyosarcoma- only 2-5% of adult sarcomas

Rhabdomyosarcomas occur ___% in the head and neck area

35%- most frequent site of this lesion

Female or male predilection for rhabdomyosarcoma

Male (60%)

3 subtypes of rhabdomyosarcoma

Alveolar, embryonal, pleomorphic

Age range for: embryonal rhabdomyosarcoma alveolar rhabdomyosarcoma pleomorphic rhabdomyosarcoma

E.R.: first 10 years of life AR: 10-25 Pleomorphic: patients older than 40

Most frequent locations for rhabdomyosarcoma

face and orbit, followed by nasal cavity

Most frequent intraoral site for rhabdomyosarcoma

palate

Two types of alveolar rhabdomyosarcoma

solid(cellular fields and no fibrous septa) and classic (fibrous septa with discohesive cells inside)

What are the 2 chromosomal translocations identified in alveolar rhabdomyosarcoma

PAX3-FOX01 (FKHR) and PAX7-FOX01 (FKHR)

Molecular changes seen in embryonal rhabdomyosarcoma

loss of heterozygosity at chromo11p15.5

What subtype of rhabdomyosarcoma has the best prognosis (5 year survival)

embryonal (66-72% with subtypes up to 95%(botryoid type) | alveolar type-44-53%

Synovial sarcoma represents ___to___% of all sarcomas

5-10%

% of synovial sarcomas occurring in the head and neck

1.9-3.7%

Balanced translocation seen in 90% of synovial sarcomas? What is the resulting fusion gene?

X;18(p11.2,q11.2)......SS18-SSX fusion gene

Age group for synovial sarcoma

teens and young adults, slight male predilection

Translocation for alveolar soft-part sarcoma and its gene fusion product...

der(17)t(X;17)(p11.2;q25)....ASPL-TFE3 fusion gene

Most common site for oral soft tissue mets

gingiva-54% of cases | 2nd most common: tongue 22%

Metastatic tumors represent ___% of all oral malignancies

route of blood borne mets to the head and neck in the absence of lung metastasis _____ plexus

batson's (valveless)

in what % of patients is metastatic oral malignancy the first sign of their disease

25%

What is the term for a malignant peripheral nerve sheath tumor with SKELETAL muscle differentiation?

malignant Triton tumor

What is the alternate term for olfactory neuroblastoma?

Esthesioneneuroblastoma

What 3 ancestries are prone to developing classic (chronic) kaposi sarcoma?

Italian, Jewish, Slavic men

Which kaposi stage? A proliferation of miniature vessels

patch stage

Which kaposi stage? further proliferation of these vascular channels along with the development of a significant spindle cell component

plaque stage

Which kaposi stage? the spindle cells increase to form a nodular tumorlike mass that may resemble a fibrosarcoma or other spindle cell sarcomas

nodular

The _______ subtype of embryonal rhabdomyosarcoma is sparsely cellular and has a pronounced myxoid stroma. Increased cellularity, or a so-called _______ layer, is usually seen just beneath the mucosal surface.

botryoid....cambium

What are two stains that can be positive in synovial sarcoma?

CK and EMA

What is the most common location for soft-part alveolar sarcoma? What are the 2 most common head and neck locations? *What is the median age for lingual soft-part alveolar sarcomas?

lower extremity...orbit and tongue...5-8 years old for lingual lesions

During the 0-30 age range soft-part alveolar sarcoma shows what sex predilection? What is the shift at age 30?

2:1 female early....more commen in men after 30

What IHC will stain positive in alveolar soft-part sarcoma?

TFE3 (transcription factor in the tumor nuclei)

What are the 3 most common oral soft tissue mets in men?

1. lung (1/3) 2.renal 3. melanoma (prostate favors bone, not soft tissue)

What are the 5 most common oral soft tissue mets in women?

1. breast (25%) 2.genital 3.kidney 4.lung 5.bone