Malgnant Mesenchymal (3) Flashcards
Which malignant mesenchymal tumor is of fibrous tissue?
Fibrosarcoma
Which malignant mesenchymal tumor is of nervous tissue?
Malignant peripheral nerve sheath tumor (MPNST)
Which malignant mesenchymal tumor is of endothelial cell tissue?
Kaposi sarcoma
Which malignant mesenchymal tumor is of muscle tissue?
Rhabdomyosarcoma
What are the clinical features of a fibrosarcoma?
Slow-growing, pain in late stage
What are the histo features of a fibrosarcoma?
Spindle shaped cells in a “herringbone” pattern
T/F: Treatment for fibrosarcoma includes radiation and chemotherapy.
False
Wide surgical excision
What is the 5 year survival rate of fibrosarcoma and how does it metastasize?
50%
Via blood
What are two other names for a MPNST?
Neurogenic sarcoma or neurofibrosarcoma
What are some clinical features of MPNST?
50% associated with NF1
May arise spontaneously
May have pain
Those with NF1 will develop lesion earlier in life (late 20s)
What are the histo features of MPNST?
Spindle-shaped cells with wavy nuclei
+ mitosis
What is the treatment and prognosis for MPNST?
Surgical resection and radical excision
50% 5-year survival without NF-1
Worse prognosis with NF-1
What causes Kaposi sarcoma?
Infection with HHV-8
What are the clinical features of classic kaposi syndrome?
Lower extremities of older patients
Painless purple. Goes from macule -> plaque -> tumor.
Does not blanch with pressure
What are the clinical presentations of endemic kaposi syndrome?
Seen in Africa
Varies from indolent skin lesions to aggressive tumors
T/F: Latrogenic kaposi syndrome will be seen weeks after organ transplant.
False
Months-years
What tissues are affected with latrogenic kaposi syndrome?
Skin and oral mucosa
T/F: Oral cavity is often the initial site of presentation in patients with classic kaposi syndrome.
False
AIDS-related kaposi syndrome
Where are there likely to be oral lesions with AIDS related kaposi syndrome?
Hard palate, gingiva, tongue
Will invade bone leading to mobility
What is the treatment and prognosis for kaposi syndrome?
Excision of small lesions, radiation and chemo injections
Classic and AIDS related -fair
Endemic - poor
latrogenic -fair to poor
What are the three types of Rhabdomyosarcoma?
- Embryonal
- Alveolar
- Pleomorphic
What is the most common soft tissue sarcoma in children under 15 y.o.?
Rhabdomyosarcoma
What is the most common intraoral site for Rhabdomyosarcoma?
Palate
What is the proper term and appearance when a Rhabdomyosarcoma grows within a body cavity?
Sarcoma botryoides
Looks like a bunch of grapes
What are the histo features of rhabdomyosarcoma?
Small round cells with hyperchromatic nuclei
Strap-shaped rhabdomyoblasts
What is treatment/prognosis for Rhabdomyosarcoma?
Wide excision, chemo, radiation
Good; 90% 5 year survival
Which lymphoreticular malignancy involves a spectrum of disorders characterized by proliferation of histiocyte-like cells with varying numbers of eosinophils, plasma cells, lymphocytes, and giant cells?
Langerhans cell histiocytosis
What is a langerhans cell?
Tissue-resistant macrophages that serve as antigen-presenting cells
T/F: Langerhans cell histiocytosis is a neoplastic process.
True
Which of the three types of LCH is often seen as a skin rash in infants and has an aggressive course?
Acute disseminated histiocytosis
____________ is not as aggressive as acute disseminated histiocytosis and is often seen in older children.
Chronic disseminated histiocytosis
What is the classic triad of issues involved with chronic disseminated histiocytosis?
Bones, skin, viscera
What characterizes eosinophilic granuloma type of LCH?
Seen in teenagers and adults
No visceral involvement, only bone
What are the most frequent bones affected with LCH?
Skull, mandible, ribs
What is the radiographic appearance of LCH in the jaws?
Scooped out appearance can mimic periodontitis and make teeth look like they are floating
How can LCH be identified histologically?
Birbeck bodies seen in cytoplasm of langerhans cells
Which type of LCH can be treated with curettage?
Eosinophilic granuloma
Which type of LCH has the worst prognosis?
Acute
__________ describes a group of malignancies characterized by tumor cells circulating in the blood.
Leukemia
Where does leukemia start?
In the bone marrow
Hematopoietic stem cells turns malignant
What is the order of prevalence for the types of leukemia in the U.S.?
- Acute myeloid leukemia
- Chronic lymphocytic leukemia
- Chronic myeloid leukemia
- acute lymphocytic leukemia
What are the symptoms of myelophthisic anemia?
Fatigue, SOB
Easy bruising
Infection
All associated with decreased normal blood cells
Which types of leukemia are most likely to produce oral involvement?
AML and CML
What is a granulocytic sarcoma?
Focal proliferation of leukemic cells at one soft tissue site
T/F: Gingival enlargement can be a feature of CML and AML.
True
What are the treatment options for leukemia?
Chemotherapy, bone marrow transplant, targeted gene therapy
What is the prognosis for ALL?
Children 90% cured
Adults 80% remission
What is the prognosis for AML?
<60y.o. 40% 5 year survival
> 60 y.o. Less than 10%
Which type of leukemia does not have a cure?
CLL
2-10 year survival
What is the prognosis for CML?
5 year survival 80%
If blast transformation death in 3-6 months
Which type of lymphoma can be seen in soft tissue or bone?
Non-Hodgkin
T/F: Hodgkin lymphoma has a male predilection.
True
What is a bimodal age distribution?
Hodgkin lymphoma will affect teenagers and young adults and will also affect those over 50 y.o.
Where is Hodgkin lymphoma often seen?
Cervical and supraclavicular nodes
T/F: Category A Hodgkin lymphoma has systemic signs.
False
Category B Hodgkin
Fever, weight loss, night sweats, pruritus
What cells are seen in a histo section of Hodgkin lymphoma?
Reed-Sternberg cells
T/F: Hodgkin lymphoma has a good prognosis.
True
T/F: Non-Hodgkin lymphoma is more common than Hodgkin.
True
Seen in older population
What are some features of oral non-Hodgkin lymphoma?
Often primary site, soft palate or buccal mucosa
May be mistaken for periapical/perio disease
Roughly how many patients diagnosed with non-Hodgkin lymphoma die each year?
1/3
What is the origin for multiple myeloma?
Plasma cells
Start proliferating an antibody that is not normal or functional
What results in Bence Jones proteins found in urine of 30-50% of multiple myeloma cases?
Manny unattached light chain antibodies filtered through the kidney
T/F: Smoldering is an asymptomatic finding in blood that can be very early stages of multiple myeloma.
True
Who is at the most risk for multiple myeloma?
Black males around 65 years old
What is the most common hematologist malignancy in black persons?
Multiple myeloma
What is the most characteristic symptom of multiple myeloma?
Bone pain, esp lumbar spine
T/F: Patients with multiple myeloma will have pathological fractures and renal failure.
True
T/F: Calcifications in soft tissue are common with multiple myeloma.
True
Accumulation of light chains can lead to deposition of __________ in various soft tissues in patients with multiple myeloma.
Amyloid
What are the sites most often effected with deposition of amyloid?
Periorbital skin - waxy, firm lesions
Oral mucosa (tongue) - enlargement, firmness
What is the radiographic presentation of multiple myeloma?
Widespread “punched out” lesions in the bone
What is the survival rate for a patient with multiple myeloma?
6-7 years
