Ch12 - Malignant Flashcards
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
Buzz word for fibrosarcoma histology
“herringbone” pattern
IHC for a fibrosarcoma should all be negative EXCEPT what two? (Fibrosarc is a dx of exclusion)
Vimentin and SMA (minimal staining)
Fibrosarcoma recurrence? 5 year survival rate? (% range)
50% recur, 40-70% 5 yr survival
What is the other term for Undifferentiated Pleomorphic Sarcoma?
Malignant fibrous histiocytoma
___________ are a heterogeneous group of tumors from this former family, whose line of differentiation cannot be determined
Undifferentiated pleomorphic sarcomas
Undifferentiated pleomorphic sarcoma: age? location?
older…deep tissues of trunk or extremities
What is the most commonly described histologic pattern for Undifferentiated pleomorphic sarcoma?
storiform-pleomorphic type
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
What is the most common soft tissue sarcoma?
LIPOSARCOMA (17% to 30% of all soft tissue malignancies in adults)
What are the 3 most common sites for Liposarcoma? What % are in the head and neck?
thigh, retroperitoneum, inguinal…3% in head and neck
Liposarcomas are seen primarily in _____; the mean age for head and neck tumors is ____ years.
adults…57 years
What is the most common head and neck location for liposarcoma? What 2 intraoral sites?
neck…tongue and cheek
What are the 4 variants of liposarcoma?
- well-diff 2.myxoid/round cell 3.pleomorphic 4.dedifferentiated
55-90% of all cases of liposarcoma in the oral cavity are which variant?
well-differentiated liposarcoma
What histological variant of liposarcoma is related to myxoid liposarcoma, but is more aggressive?
round cell liposarcoma
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
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
What are the three names for malignant peripheral nerve sheath tumor?
malignant schwannoma, neurofibrosarcoma, neurogenic sarcoma
Half of the cases of a malignant peripheral nerve sheath tumor occur in what patient population?
NF1 patients
What % range of malignant peripheral nerve sheath tumors occur in the head and neck?
14-19%
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
What are three most common sites for Malignant peripheral nerve sheath tumors in the oral cavity?
mandible, lips, buccal mucosa
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
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
- patch 2 plaque 3.nodular
What % of soft tissue sarcomas are leiomyosarcoma?
7%
Age range of leiomyosarcoma
middle aged and older adults
Smooth muscle tumors with more then ____ mitoses per 10 HPF should be considered malignant
5
Leiomyosarcomas are positive for 4 IHC markers and which special stain?
- 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
1%
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?
- 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?
- breast (25%) 2.genital 3.kidney 4.lung 5.bone
