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PATH II > mesenchymal neoplasias > Flashcards

mesenchymal neoplasias Flashcards

1
Q

what tissues can have soft tissue tumors

A
  • Adipose tissue
  • Peripheral nerve
  • Smooth muscle
  • Skeletal muscle
  • Fibrous tissue
  • Vascular tissue
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2
Q

lipoma

A

A benign tumor of fat that is usually seen in adults

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3
Q

most common benign
mesenchymal neoplasm?

A

lipoma

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4
Q

metabolic state of lipoma tissues

A

metabolically inactive

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5
Q

what can be mistaken for a lipoma

A

Herniated buccal fat pads may be
mistaken for lipomas

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6
Q

lipoma histo

A

would see many fat globules

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7
Q

Solitary Neurofibroma

A

A benign neoplasm of peripheral nerve that expands nerve the nerve trunk

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8
Q

what cells are seen in solitary neurofibromas

A

It includes all cell types of peripheral nerve: Schwann cells, perineural fibroblasts, axons

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9
Q

what clinical settings can a neurofibroma be seen in?

A

– A solitary lesion
– As part of a syndrome
-neurofibromatosis type I

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10
Q

neurofibroma histo app/possible stain?

A

like swimming mennows, can have s100 stain

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11
Q

neurofibroma xray

A

multilocular luceny

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12
Q

plexiform neurofibromas occurs with:

A

Neurofibromatosis Type I

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13
Q

neurofibromatosis type 1
additonal name?
inheritence?
skin?
malignant?

A
  • Von Recklinghausen’s disease of skin
  • Autosomal dominant
  • Cutaneous neurofibromas
  • Café-au-lait pigmentation
  • Malignant transformation
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14
Q

lisch nodules of NF-1

A

Benign, pigmented lesions on the iris that do not interfere with vision

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15
Q

Solitary Neurofibroma vs. Neurofibromatosis Type I

A

one= solitary
many=NF-1

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16
Q

progression?

where can plexiform neurofibromas occur with NF-1

A

everywhere, can be progressively disfguring

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17
Q

skin? nnfibromas? freckling? eye? bone? relative?

Diagnostic Criteria for Neurofibromatosis Type I

A

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
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18
Q

big indication of NF-1

A

Six or more café au lait macules
1.5 cm or larger is strong evidence of neurofibromatosis

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19
Q

schwannoma/ neurilemmoma

A

An encapsulated benign neoplasm of Schwann cells that pushes the nerve trunk aside

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20
Q

schwannoma histopath exhibits:

A

Histopathology exhibits
Verocay bodies

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21
Q

verocay bodies associated with? histo?

A

seen in schwannomas
A central eosinophillic zone surrounded by palisaded nuclei

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22
Q

Intra-Osseous Neurilemmoma xray

A

well capsulated lucency

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23
Q

pathgnomic sign of MEN 2b

A

comissural neuromas

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24
Q

MEN 2b oral signs

A

multiple neuromas

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25
Q

MEN 2b common signs

A
  • Mucosal neuromas - markers for internal malignancy
  • Medullary Carcinoma of Thyroid (75%)
  • Adrenal Pheochromocytoma
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26
Q

Granular Cell Tumor defined:
B/M?
origin cell type?

A

A benign peripheral nerve sheath neoplasm believed to be of Schwann cell origin

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27
Q

most common site of granular cell tumors

A

The dorsal tongue most is the most commonly involved site in the body

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28
Q

app of granular cell tumor

A

Non-encapsulated, fixed
can be one or many

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29
Q

granular cell tumor cells contain:

A

lysosomes

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30
Q

granular cell tumors may be associated with? mistaken for?

A

May be associated with pseudo-epitheliomatous hyperplasia (PEH) and mistaken for squamous cell carcinoma

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31
Q

Congenital Epulis of the Newborn

A

Congenital granular cell tumor of the newborn, A hamartomatous lesion that does not recur

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32
Q

congenital epulis of newborn demo/location

A

Anterior jaws of females (8:1)
neonates

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33
Q

Congenital Epulis of the Newborn cells contain?

A

Like the granular cell tumor, the
granular cells of the granular cell epulis contain lysosomes

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34
Q

pain? true neoplasm?

traumatic neuromas

A
  • Damage to peripheral nerve causes reactive proliferation of neural tissue
  • A reactive lesion and not a true neoplasm
  • Pain is variable
35
Q

prognosis?

Malignant schwannoma/neurofibrosarcoma

A
  • Malignant transformation of a neurofibroma in NF I
  • Poor prognosis
36
Q

diascopy

A

can be used to test if a mass is of vessel origin- press on it t see if blood moves

37
Q

varix

A

dialated vv

38
Q

sublingual varices

A

can be seen on ventral tongue as a result of hypertension, “caviar tongue”

39
Q

where else could a varix be orally/facially?

A

upper lip

40
Q

Hemangioma

A

A benign proliferation of blood vessels

41
Q

hermangioma classification

A

May be classified on the basis of the size of the vascular channels as cavernous (large vessels) or capillary (small vessels)

42
Q

hermangioma most common in what demo?

A

Most common in children, where most are located in the skin (birthmarks) and most involute by end of puberty

43
Q

hermangioma development/cycle
arise when?
phases?

A
  • Not ususally congenital, but arise during first few weeks of post-natal life
  • Undergo a rapid growth phase and then gradually involute
44
Q

seen in? effects? mortality?

Kasabach-Merritt Syndrome

A
  • Infants
  • Large, extensive hemangiomas trap platelets, producing thrombocytopenia, leading to hemorrhage
  • High mortality
45
Q

Angiosarcoma

A

Malignancy of vascular
endothelium

46
Q

angiosarcoma may resemble what in the elderly?

A

bruise on the scalp or forehead of the elderly

47
Q

where are angiosarcomas rarely seen?

A

Rarely seen in the oral
mucosa

48
Q

association?

Kaposi Sarcoma

A

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

49
Q

where might we see karposi sarcoma orally?

A

mucosal surfaces

50
Q

def? due to? most arise at what age?

Lymphangioma

A
  • Benign tumor of lymphatic vessels
  • Sequestration of lymphatic tissue
  • Most arise during childhood
51
Q

lymphangioma vs lymphoma

A

LYMPHANGIOMA - benign tumor of lymphatic vessels
LYMPHOMA - malignant tumor of lymphocytes

52
Q

head/neck forms lymphangiomas

A
  • Oral mucosal lymphangioma
  • Cervical lymphangioma (Cystic Hygroma)
53
Q

app? most common site/effect?

Oral Mucosal Lymphangioma

A
  • A focal superficial lesion of oral mucosa frequently with a pebbly surface
  • The tongue is the most common site and may produce
    macroglossia
54
Q

may be associated with what effects?

Cervical Lymphangioma

A
  • A lymphangioma involving the soft tissues of the neck
  • May be associated with dysphagia and airway obstruction
55
Q

Treatment and Prognosis for Lymphangiomas:
compared to hermangiomas?
do not respond to what agents?
surgical?
complication if in head/neck region?

A
  • 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
56
Q

Lymphangiosarcoma

A

Malignant neoplasm of lymphatic endothelium

57
Q

when do lymphangiosarcomas occurs

A

Occurs in long-standing cases of lymphedema secondary to
lymphatic dysfunction

58
Q

Hamartoma -

A

developmental
overgrowth of tissue native to
the site

59
Q

Choristoma -

A

developmental overgrowth of tissue not native to the site

60
Q

Leiomyoma

A
  • A benign neoplasm of smooth
    muscle
  • The smooth muscle of the uterus most common site, where it is commonly referred to as a “fibroid”
61
Q

leiomyomas may also arise from what other tissues

A

May also arise from vascular or hair follicle (arrector pili) smooth muscle

62
Q

Leiomyoma of the Oral Mucosa, referred to as?

A

Oral mucosal leiomyomas usually arises from vascular smooth muscle and are referred to as angiomyomas

63
Q

M or B?

most common neoplasias of skeletal mm

A

Malignant neoplasms are more common than benign neoplasms

64
Q

Rhabdomyoma

A

benign neoplasm of skeletal muscle

65
Q

Rhabdomyosarcoma

A

a malignant neoplasm of skeletal muscle

66
Q

the most common soft tissue sarcoma of
children?

A

Rhabdomyosarcoma is the most common soft tissue sarcoma of
children

67
Q

most frequent sites of Rhabdomyosarcoma

A

Most frequent site is head and neck
followed by genitourinary

68
Q

Rhabdomyosarcoma app resembles?

A

grapes on vine

69
Q

Multimodal Treatment of Rhabdomyosarcoma

A
  • Local surgical excision
  • Multi-agent chemotherapy
  • Postoperative radiation
    therapy
  • Five-year survival
    improved with multimodal
    treatment
70
Q

demo?

Keloid

A
  • Complication of wound healing
  • Excessive scar formation
  • Scar tissue grows beyond the
    boundaries of the original
    wound
  • African-Americans
71
Q

tumor/cancer def

A
  • Tumor –casual for a neoplasm
  • Cancer –casual for a malignant neoplasm
72
Q

adenoma/ adenocarcinoma/ sarcoma def

A
  • 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
73
Q

suffix –benign or malignant

A

-oma: B
-sarcoma: M

74
Q

B and M

  • Fibrous connective tissue (fibroblast) tumors names
A

– Benign –fibroma, myxoma *
– Malignant –fibrosarcoma

75
Q

adipose B/M tumors

A

– Benign - lipoma

– Malignant- liposarcoma

76
Q

bone B/M tumors

A

– Benign –osteoma

– Malignant –osteosarcoma

77
Q

smooth mm B/M tumors

A

– Benign –leiomyoma

– Malignant –leiomyosarcoma

78
Q

skel mm B/M tumors

A

– Benign - rhabdomyoma
– Malignant - rhabdomyosarcoma

79
Q

nerve B/M tumors names

A

Benign –neurofibroma, schwannoma, neuroma

Malignant –neurofibrosarcoma, malignant peripheral nerve sheath tumor, malignant schwannoma

80
Q

cartilage b/m tumors

A

Benign - chondroma
– Malignant –chondrosarcoma

81
Q

blood vessels b/m tumors

A

– Benign - hemangioma
– Malignant –hemangiosarcoma or
angiosarcoma

82
Q

lymphatic vessels b/m tumors

A

– Benign - lymphangioma
– Malignant –lymphangiosarcoma

83
Q

Eponyms - Examples of Non-Standard Tumor
Nomenclature

A
  • Hodgkin lymphoma / Hodgkin disease –tumor of lymphocytes
  • Wilm’stumor –primitive tumor of kidney
  • Ewing sarcoma –primitive tumor of bone
84
Q

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

A
  • 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

PATH II (25 decks)

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