Malignant Mesenchymal Tumors and Lymphoreticular Malignancies - part I Flashcards
malignant mesenchymal tumors
- fibrous - fibrosarcoma
- nerve - malignant peropheral nerve sheath tumor (MPNST)
- endothelial cell - Kaposi sarcoma
- muscle - rhabdomyosarcoma
lymphoreticular malignancies
- Langerhans cell histiocytosis
- leukemia
- lymphoma
- multiple myeloma/plasmacytoma
fibrosarcoma
malignancy of fibroblastic differentiation
who is affected by fibrosarcoma?
adults but any age
clinical features of fibrosarcoma
- soft tissue mass or centrally as an intrabony lesion
2. slow growing
T/F: pain is an early clinical feature of fibrosarcoma
false, late feature
histopathologic features of fibrosarcoma
- fascicles of spindle-shaped cells often forming a “herringbone” pattern
- variable number of mitoses
treatment of fibrosarcoma
wide to radical surgical excision
T/F: fibrosarcoma responds well to radiation or chemotherapy
false, little to no response to radiation or chemotherapy
how does fibrosarcoma metastasize?
blood
which organs does fibrosarcoma metastasize to?
- lung
- liver
- bone
what is malignant peripheral nerve sheath tumor (MPNST) also known as?
- neurogenic sarcoma
2. neurofibrosarcoma
what percent of MPNST is associated with NF1?
~50%
T/F: MPNST arise spontaneously
true
clinical features of MPNST
- may have pain
- may have nerve deficit
- may be in soft tissue or centrally as an intrabony lesion
histopathologic features of MPNST
- spindle-shaped cells with wavy nuclei
2. mitoses
treatment for MPNST
- surgical resection
- radical excision
- amputation
- ± radiation
how was Kaposi sarcoma (KS) first described by Moritz Kaposi in the late 19th century?
described as a disease affecting elderly white males of Mediterranean descent
Kaposi sarcoma was identified in what type of people?
- sub-Saharan Africans
- transplant recipients
- HIV+ males
what is KS caused by?
infection with HHV-8
what are the 4 clinical presentations of KS?
- classic
- endemic (African)
- iatrogenic (transplant-associated)
- epidemic (AIDS-related)
who is affected by classic KS?
elderly patients, most often male
where does classic KS typically appears?
on lower extremities
clinical features of classic KS
- slow growing
- painless
- purple-red
- macules –> plaques –> tumors
T/F: classic KS blanches on pressure
false, does NOT
who is affected by endemic (African) KS?
wide age range especially young adults and children
T/F: course of endemic (African) KS can vary widely from indolent skin lesions to aggressive tumors
true
who is most often affected by lymphadenopathic endemic (African) KS?
young children –> causes visceral tumors
cause of iatrogenic (transplant-associated) KS
solid organ transplants
T/F: iatrogenic KS can occur several months to years after transplant
true
where does iatrogenic KS affect?
skin and oral mucosa
what can cause regression of iatrogenic KS?
reducing degree of immune-suppression
who is affected by AIDS-related KS?
primarily HIV-infected adult male homosexuals (other types of KS seen in both genders; children in endemic)
AIDS-related KS skin lesions have a predilection for which locations?
face and lower extremities
where is the initial site of AIDS-related KS presentation?
oral cavity in ~22% of patients
where does AIDS-related KS affect intraorally?
- hard palate
- gingiva
- tongue
T/F: AIDS-related KS can invade bone
true
what happens when AIDS-related KS invades bone?
tooth mobility
histopathologic features of KS
- cellular spindle cell tumor within the CT, extravasated blood
- many poorly defined vascular slits
- HHV-8 positivity in endothelial cells and some spindle cells
treatment for KS
- excision for small cosmetically problematic lesions
- radiation
- injections of chemotherapeutic agents
prognosis of classic KS
fair
T/F: patients with classic KS usually die of something else and not the disease
true, MI, CVA, etc.
prognosis of endemic lymphadenopathic KS
poor
prognosis of iatrogenic KS
fair to poor
prognosis of AIDS-related KS
fair
what are the different types of rhabdomyosarcoma?
- embryonal
- alveolar
- pleomorphic
T/F: rhabdomyosarcoma is common
false, rare
who does rhabdomyosarcoma affect?
children or adolescents
what is the most common soft tissue sarcoma in children under 15 y.o.?
rhabdomyosarcoma
clinical features of rhabdomyosarcoma
- usually painless
- rapidly growing
- sarcoma botryoides
what is the most common location for rhabdomyosarcoma?
head and neck
what is the most common intraoral site for rhabdomyosarcoma?
palate
sarcoma botryoides
term used when rhabdomyosarcoma grows in a body cavity e.g. oral cavity, vagina
how does sarcoma botryoides appear clinically?
“bunch of grapes”
histopathologic features of rhabdomyosarcoma
- small round cells, hyperchromatic nuclei
2. may see strap-shaped rhabdomyoblasts with cross striations
treatment of rhabdomyosarcoma
- wide excision
- multiagent chemotherapy
- post-op radiation
prognosis for rhabdomyosarcoma depends on what?
- type
- location
- stage
- age
Langerhans cell histiocytosis (LCH)
SPECTRUM of disorders characterized by proliferation of histiocyte-like cells accompanied by varying number of eosinophils, lymphocytes, plasma cells and multinucleated giant cells
what was Langerhans cell histiocytosis previously known as?
histiocytosis X
what are distinctive histiocytic cells?
Langerhans cells
Langerhans cells
tissue-resident macrophages (ralted to monocytes) serve as antigen-presenting cells
there was previous controversy on whether LCH was neoplastic or non-neoplastic but what confirms the neoplastic process?
clonal proliferation
T/F: LCH is rare
true
who is affected by LCH?
wide age range
T/F: LCH has a slight female predilection
false, NO gender predilection
T/F: LCH is painless
false, dull pain or tenderness
what are the 3 clinical presentations of LCH?
- acute disseminated histiocytosis
- chronic disseminated histiocytosis
- eosiniohilic granuloma (monostotic or polyostotic)
polyostotic
localized to multiple skeletal sites
who is affected by acute disseminated histiocytosis (Letterer-Siwe disease)?
infants
what does acute disseminated histiocytosis involve?
- skin rash
- splenic
- hepatic
- marrow involvement
T/F: acute disseminated histiocytosis are on an aggressive, malignant course
true
who is affected by chronic disseminated histiocytosis (Hand-Schuller-Christian disease)?
older children
T/F: chronic disseminated histiocytosis is more ggressive than acute disseminated histiocytosis
false, not as aggressive as acute disseminated histiocytosis
classic triad (bone, skin, viscera) of chronic disseminated histiocytosis
- exophthalmos
- diabetes insipidus (kidneys unable to prevent excretion of water)
- bone lesions
T/F: eosinophilic granuloma has both intrabony and visceral involvement
false, intrabony ONLY, no visceral involvement
who is affected by eosinophilic granuloma?
teenagers, young adults
who is most affected by polyostotic eosinophilic granuloma?
teenagers
who is most affected by monostotic eosinophilic granuloma?
adults
where can LCH be seen radiographically?
any bone can be affected but skull, mandibule, ribs, vertebrae most frequent
radiographic features of LCH
- solitary or multiple
- may break out of bone
- radiolucent
- well-defined (usually) but not corticated
- occasionally ill-defined
- “scooped-out” appearance of superficial bone esp. posterior mandible
what can severe bone loss due to LCH resemble?
perio disease
extensive alveolar involvement can cause what to LCH on radiographs?
causes teeth to appear as if they’re “floating in air”
histopathologic features of LCH
- sheets of large, pale histiocytic-appearing cells (neoplastic Langerhans cells)
- variable numbers of eosinophils
what could be seen in the cytoplasm of Langerhans cells under an electron microscope?
Birbeck bodies
treatment of LCH depends on what?
on form of LCH
treatment for acute disseminated histiocytosis
chemotherapy
treatment for chronic disseminated histiocytosis
radiation and/or chemotherapy
treatment for eosinophilic granuloma
curettage or radiation
prognosis of LCH are better for what type of patients?
patients who are older at the time of onset
prognosis of acute disseminated histiocytosis
poor
prognosis of chronic disseminated histiocytosis
guarded
prognosis of eosinophilic granuloma
good
leukemia
groups of hematologic malignancies characterized by tumor cells circulating in the blood
where does leukemia begin?
in the bone marrow
how does leukemia start?
with malignant transformation of one of the hematopoietic stem cells, which proliferates and eventually overflows into the peripheral blood
types of leukemia
- lymphocytic
- myelomonocytic
- acute
- chronic
lymphocytic leukemia
acute lymphocytic leukemia (ALL) < chronic lymphocytic leukemia (CLL)
myelomonocytic leukemia
acute myeloid leukemia (AML) > chronic myeloid leukemia (CML)
T/F: patients with leukemia often present with myelophthisic anemia
true
myelophthisic anemia
normal bone marrow cells replaced by leukemic cells
decreased RBC’s in leukemic patients with myelophthisic anemia suffer from what?
- fatigue, shortness of breath (SOB)
2. pallor
decreased platelets in leukemic patients with myelophthisic anemia are more prone to what?
easy bruising
decreased WBC’s in leukemic patients with myelopthisic anemia are more prone to what?
infection
oral involvement is most often seen in what form of leukemia?
myelomonocytic forms
clinical features of leukemia
- diffuse gingival enlargement
2. “granulocytic sarcoma”
granulocytic sarcoma
focal proliferation of leukemic cells at one soft tissue site
diagnosis of leukemia is based on what?
finding of increased numbers of atypical white blood cells in the circulation and marrow
how is the type of leukemia determined?
immunohistochemical/cytogenetic studies
histopathologic features of leukemia
diffuse infiltration and destruction of normal host tissue by sheets of poorly differentiated cells with either myelomonocytic characeristics or lymphoid features
treatment for leukemia
- chemotherapy
- bone marrow or stem cell transplantation
- targeted gene therapy
prognosis of leukemia depends on what?
- type of leukemia
- age of patient
- cytogenetic alterations
T/F: CLL is curable
false, incurable but highly variable course of disease, 2-10 years
T/F: CML has an indolent period
true
T/F: CML has a blast transformation that may lead to death in 3-6 months
true, less differentiated, proliferate wildly
T/F: lymphoma tends to grow as solid masses
true
what are the two forms of lymphoma?
- Hodgkin
2. non-Hodgkin
where does Hodgkin lymphoma develop?
in the lymph nodes
where does non-Hodgkin lymphoma arise in?
mostly arises in lymph nodes, but can also arise in soft tissue or bone
T/F: lymphoma has a female predilection
false, MALE
what is the most common site of initial involvement for Hodgkin lymphoma?
head and neck
T/F: oral involvement for Hodgkin lymphoma is rare
true
what is the age distribution for Hodgkin lymphoma?
BIMODAL
- teenagers and young adults, 2nd-3rd decades
- > 50 years old
T/F: any lymph node can be involved in Hodgkin lymphoma
true
clinical features of Hodgkin lymphoma
- one or more non-tender, palpable, rubbery-firm, enlarging lymph nodes
70-75% of patients with Hodgkin lymphoma will experience what?
painless cervical, supraclavicular lymphadenopathy
category A of Hodgkin lymphoma
no systemic signs
category B of Hodgkin lymphoma
“B signs”
B signs of Hodgkin lymphoma
- fever
- weight loss
- drenching night sweats
- generalized pruritus (itching)
histopathologic features of Hodgkin lymphoma
- effacement of normal nodal architecture by infiltrate of inflammatory cells
- Reed-Sternberg cell
Reed-Sternberg cells
atypical neoplastic lymphoid cell
what are the two forms of Reed-Sternberg cells?
- “owl-eye”
2. pennies on a plate”
treatment of Hodgkin lymphoma depends on what?
on stage
treatment for Hodgkin lymphoma
- multiagent chemotherapy
2. radiation for later stages
T/F: treatment of Hodgkin lymphoma can result in post-treatment complications
true
prognosis of Hodgkin lymphoma
good
prognosis for Hodgkin lymphoma is worse for what types of patients?
worse for patients who show “B” signs
after 15 years post-tx mortality of Hodgkin lymphoma is due to what?
more to secondary malignancy or cardiovascular disease
