mesenchymal neoplasias Flashcards
what tissues can have soft tissue tumors
- Adipose tissue
- Peripheral nerve
- Smooth muscle
- Skeletal muscle
- Fibrous tissue
- Vascular tissue
lipoma
A benign tumor of fat that is usually seen in adults
most common benign
mesenchymal neoplasm?
lipoma
metabolic state of lipoma tissues
metabolically inactive
what can be mistaken for a lipoma
Herniated buccal fat pads may be
mistaken for lipomas
lipoma histo
would see many fat globules
Solitary Neurofibroma
A benign neoplasm of peripheral nerve that expands nerve the nerve trunk
what cells are seen in solitary neurofibromas
It includes all cell types of peripheral nerve: Schwann cells, perineural fibroblasts, axons
what clinical settings can a neurofibroma be seen in?
– A solitary lesion
– As part of a syndrome
-neurofibromatosis type I
neurofibroma histo app/possible stain?
like swimming mennows, can have s100 stain
neurofibroma xray
multilocular luceny
plexiform neurofibromas occurs with:
Neurofibromatosis Type I
neurofibromatosis type 1
additonal name?
inheritence?
skin?
malignant?
- Von Recklinghausen’s disease of skin
- Autosomal dominant
- Cutaneous neurofibromas
- Café-au-lait pigmentation
- Malignant transformation
lisch nodules of NF-1
Benign, pigmented lesions on the iris that do not interfere with vision
Solitary Neurofibroma vs. Neurofibromatosis Type I
one= solitary
many=NF-1
progression?
where can plexiform neurofibromas occur with NF-1
everywhere, can be progressively disfguring
skin? nnfibromas? freckling? eye? bone? relative?
Diagnostic Criteria for Neurofibromatosis Type I
AT LEAST TWO OF THESE:
* Café au lait macules
– Prepubertal
* 6 or more over 0.5 cm
– Postpubertal
* 6 or more over 1.5 cm
- Neurofibromas
– Plexiform - Any
– Conventional - 2 or more
- Freckling
– Axillary, or
– Inguinal - Eye
– Optic glioma
– Lisch nodules (iris hamartoma) - 2 or more
- Bone
– Sphenoid dysplasia, or
– Thinning of long bone cortex - First degree relative with NF I
big indication of NF-1
Six or more café au lait macules
1.5 cm or larger is strong
evidence of neurofibromatosis
schwannoma/ neurilemmoma
An encapsulated benign neoplasm of Schwann cells that pushes the nerve trunk aside
schwannoma histopath exhibits:
Histopathology exhibits
Verocay bodies
verocay bodies associated with? histo?
seen in schwannomas
A central eosinophillic zone surrounded by palisaded nuclei
Intra-Osseous Neurilemmoma xray
well capsulated lucency
pathgnomic sign of MEN 2b
comissural neuromas
MEN 2b oral signs
multiple neuromas
MEN 2b common signs
- Mucosal neuromas - markers for internal malignancy
- Medullary Carcinoma of Thyroid (75%)
- Adrenal Pheochromocytoma
Granular Cell Tumor defined:
B/M?
origin cell type?
A benign peripheral nerve sheath neoplasm believed to be of Schwann cell origin
most common site of granular cell tumors
The dorsal tongue most is the most commonly involved site in the body
app of granular cell tumor
Non-encapsulated, fixed
can be one or many
granular cell tumor cells contain:
lysosomes
granular cell tumors may be associated with? mistaken for?
May be associated with pseudo-epitheliomatous hyperplasia (PEH) and mistaken for squamous cell carcinoma
Congenital Epulis of the Newborn
Congenital granular cell tumor of the newborn, A hamartomatous lesion that does not recur
congenital epulis of newborn demo/location
Anterior jaws of females (8:1)
neonates
Congenital Epulis of the Newborn cells contain?
Like the granular cell tumor, the
granular cells of the granular cell epulis contain lysosomes
pain? true neoplasm?
traumatic neuromas
- Damage to peripheral nerve causes reactive proliferation of neural tissue
- A reactive lesion and not a true neoplasm
- Pain is variable
prognosis?
Malignant schwannoma/neurofibrosarcoma
- Malignant transformation of a neurofibroma in NF I
- Poor prognosis
diascopy
can be used to test if a mass is of vessel origin- press on it t see if blood moves
varix
dialated vv
sublingual varices
can be seen on ventral tongue as a result of hypertension, “caviar tongue”
where else could a varix be orally/facially?
upper lip
Hemangioma
A benign proliferation of blood vessels
hermangioma classification
May be classified on the basis of the size of the vascular channels as cavernous (large vessels) or capillary (small vessels)
hermangioma most common in what demo?
Most common in children, where most are located in the skin (birthmarks) and most involute by end of puberty
hermangioma development/cycle
arise when?
phases?
- Not ususally congenital, but arise during first few weeks of post-natal life
- Undergo a rapid growth phase and then gradually involute
seen in? effects? mortality?
Kasabach-Merritt Syndrome
- Infants
- Large, extensive hemangiomas trap platelets, producing thrombocytopenia, leading to hemorrhage
- High mortality
Angiosarcoma
Malignancy of vascular
endothelium
angiosarcoma may resemble what in the elderly?
bruise on the scalp or forehead of the elderly
where are angiosarcomas rarely seen?
Rarely seen in the oral
mucosa
association?
Kaposi Sarcoma
A type of multi-centric angiosarcoma associated with Human Herpesvirus Type 8 (HHV-8, KS-associated Herpes virus)
* Rare before AIDS
* There are HIV-associated and non-HIV-associated forms
where might we see karposi sarcoma orally?
mucosal surfaces
def? due to? most arise at what age?
Lymphangioma
- Benign tumor of lymphatic vessels
- Sequestration of lymphatic tissue
- Most arise during childhood
lymphangioma vs lymphoma
LYMPHANGIOMA - benign tumor of lymphatic vessels
LYMPHOMA - malignant tumor of lymphocytes
head/neck forms lymphangiomas
- Oral mucosal lymphangioma
- Cervical lymphangioma (Cystic Hygroma)
app? most common site/effect?
Oral Mucosal Lymphangioma
- A focal superficial lesion of oral mucosa frequently with a pebbly surface
- The tongue is the most common site and may produce
macroglossia
may be associated with what effects?
Cervical
Lymphangioma/
Cystic Hygroma
- A lymphangioma involving the soft tissues of the neck
- May be associated with dysphagia and airway obstruction
Treatment and Prognosis for Lymphangiomas:
compared to hermangiomas?
do not respond to what agents?
surgical?
complication if in head/neck region?
- Unlike hemangiomas, spontaneous regression is rare and they do not respond to sclerosing agents
- Complete surgical excision may not be possible and recurrence is common
- Airway obstruction
Lymphangiosarcoma
Malignant neoplasm of lymphatic endothelium
when do lymphangiosarcomas occurs
Occurs in long-standing cases of lymphedema secondary to
lymphatic dysfunction
Hamartoma -
developmental
overgrowth of tissue native to
the site
Choristoma -
developmental overgrowth of tissue not native to the site
Leiomyoma
- A benign neoplasm of smooth
muscle - The smooth muscle of the uterus most common site, where it is commonly referred to as a “fibroid”
leiomyomas may also arise from what other tissues
May also arise from vascular or hair follicle (arrector pili) smooth muscle
Leiomyoma of the Oral Mucosa, referred to as?
Oral mucosal leiomyomas usually arises from vascular smooth muscle and are referred to as angiomyomas
M or B?
most common neoplasias of skeletal mm
Malignant neoplasms are more common than benign neoplasms
Rhabdomyoma
benign neoplasm of skeletal muscle
Rhabdomyosarcoma
a malignant neoplasm of skeletal muscle
the most common soft tissue sarcoma of
children?
Rhabdomyosarcoma is the most common soft tissue sarcoma of
children
most frequent sites of Rhabdomyosarcoma
Most frequent site is head and neck
followed by genitourinary
Rhabdomyosarcoma app resembles?
grapes on vine
Multimodal Treatment of Rhabdomyosarcoma
- Local surgical excision
- Multi-agent chemotherapy
- Postoperative radiation
therapy - Five-year survival
improved with multimodal
treatment
demo?
Keloid
- Complication of wound healing
- Excessive scar formation
- Scar tissue grows beyond the
boundaries of the original
wound - African-Americans
tumor/cancer def
- Tumor –casual for a neoplasm
- Cancer –casual for a malignant neoplasm
adenoma/ adenocarcinoma/ sarcoma def
- Adenoma –a benign neoplasm of glandular epithelial origin
- Adenocarcinoma –a malignant neoplasm of glandular epithelial origin
- Sarcoma –a malignant neoplasm of mesodermal tissue origin (connective tissue) –bone, cartilage, smooth muscle, skeletal muscle, nerve, adipose tissue
suffix –benign or malignant
-oma: B
-sarcoma: M
B and M
- Fibrous connective tissue (fibroblast) tumors names
– Benign –fibroma, myxoma *
– Malignant –fibrosarcoma
adipose B/M tumors
– Benign - lipoma
– Malignant- liposarcoma
bone B/M tumors
– Benign –osteoma
– Malignant –osteosarcoma
smooth mm B/M tumors
– Benign –leiomyoma
– Malignant –leiomyosarcoma
skel mm B/M tumors
– Benign - rhabdomyoma
– Malignant - rhabdomyosarcoma
nerve B/M tumors names
Benign –neurofibroma, schwannoma, neuroma
Malignant –neurofibrosarcoma, malignant peripheral nerve sheath tumor, malignant schwannoma
cartilage b/m tumors
Benign - chondroma
– Malignant –chondrosarcoma
blood vessels b/m tumors
– Benign - hemangioma
– Malignant –hemangiosarcoma or
angiosarcoma
lymphatic vessels b/m tumors
– Benign - lymphangioma
– Malignant –lymphangiosarcoma
Eponyms - Examples of Non-Standard Tumor
Nomenclature
- Hodgkin lymphoma / Hodgkin disease –tumor of lymphocytes
- Wilm’stumor –primitive tumor of kidney
- Ewing sarcoma –primitive tumor of bone
Tumors that Sound Benign but are Malignant
* melanocytes
* lymphocytes
* plasma cells
* testicular tumor
*hepatocytes
* childhood ones
* hematopoietic elements in bone marrow that circulate in the blood
- Melanoma - melanocytes
- Lymphoma - lymphocytes
- Multiple myeloma –plasma cells
- Seminoma –testicular tumor
- Hepatoma –hepatocytes –hepatocellular carcinoma
- Retinoblastoma - childhood
- Neuroblastoma –childhood
- Leukemia - hematopoietic elements in bone marrow that circulate in the blood
