Soft Tissue Tumors (Mesenchymal Pathology) Flashcards

1
Q

skipped
Abbreviated Classification of Soft Tissue Tumors by Histologic Differentiation
(6)

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

Lipoma
(4)

A
  • A benign tumor of fat that is usually
    seen in adults
  • The lipoma is the most common benign
    mesenchymal neoplasm
  • The adipose tissue of lipoma is
    metabolically unavailable
  • Herniated buccal fat pads may be
    mistaken for lipomas
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3
Q

Solitary Neurofibroma
(3)

A
  • A benign neoplasm of
    peripheral nerve that
    expands nerve the nerve
    trunk
  • It includes all cell types of
    peripheral nerve: Schwann
    cells, perineural
    fibroblasts, axons
  • The neurofibroma may be
    seen in two clinical
    settings
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4
Q
  • The neurofibroma may be
    seen in two clinical
    settings
    (2)
A

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

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5
Q

Neurofibromatosis Type I
(5)

A
  • Von Recklinghausen’s disease of skin
  • Autosomal dominant
  • Cutaneous neurofibromas
  • Café-au-lait pigmentation
  • Malignant transformation
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6
Q

Neurofibromatosis Type I – Lisch Nodules

A

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

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7
Q

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Neurofibromatosis Type I

A

Ages 21, 36, 56 and 69

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8
Q

Diagnostic Criteria for Neurofibromatosis Type I - at least 2 of these:
* Café au lait macules
(2)

A

– Prepubertal
* 6 or more over 0.5 cm
– Postpubertal
* 6 or more over 1.5 cm

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9
Q

Diagnostic Criteria for Neurofibromatosis Type I - at least 2 of these:
Neurofibromas

A

– Plexiform
* Any
– Conventional
* 2 or more

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10
Q

Diagnostic Criteria for Neurofibromatosis Type I - at least 2 of these:
* Freckling

A

– Axillary, or
– Inguinal

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11
Q

Diagnostic Criteria for Neurofibromatosis Type I - at least 2 of these:
* Eye

A

– Optic glioma
– Lisch nodules (iris hamartoma)
* 2 or more

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12
Q

Diagnostic Criteria for Neurofibromatosis Type I - at least 2 of these:
* Bone
(2)

A

– Sphenoid dysplasia, or
– Thinning of long bone cortex

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13
Q

Diagnostic Criteria for Neurofibromatosis Type I - at least 2 of these:
* — degree relative with NF I

A

First

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14
Q

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

A

1.5

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15
Q

Schwannoma - Neurilemmoma
(2)

A
  • An encapsulated benign
    neoplasm of Schwann cells
    that pushes the nerve trunk
    aside
  • Histopathology exhibits
    Verocay bodies
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16
Q

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Verocay Body

A

A central eosinophillic zone surrounded by palisaded nuclei

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17
Q

Multiple Endocrine Neoplasia Type IIb
(3)

A
  • Mucosal neuromas - markers for internal malignancy
  • Medullary Carcinoma of Thyroid (75%)
  • Adrenal Pheochromocytoma
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18
Q

Granular Cell Tumor
(5)

A
  • A benign peripheral nerve sheath
    neoplasm believed to be of Schwann
    cell origin
  • The tongue most is the most
    commonly involved site in the body
  • Non-encapsulated, fixed
  • Granular cells contain lysosomes
  • May be associated with pseudo-
    epitheliomatous hyperplasia (PEH) and
    mistaken for squamous cell carcinoma
19
Q

Congenital Epulis of the Newborn
(4)

A
  • Congenital granular cell tumor of
    the newborn
  • Anterior jaws of females (8:1)
    neonates
  • A hamartomatous lesion that does
    not recur
  • Like the granular cell tumor, the
    granular cells of the granular cell
    epulis contain lysosomes
20
Q

Traumatic Neuroma
(3)

A
  • Damage to peripheral
    nerve causes reactive
    proliferation of neural
    tissue
  • A reactive lesion and not
    a true neoplasm
  • Pain is variable
21
Q

Malignant Peripheral Nerve Sheath Tumor
(3)

A
  • Malignant schwannoma,
    neurofibrosarcoma
  • Malignant transformation of a
    neurofibroma in NF I
  • Poor prognosis
22
Q

Hemangioma
(5)

A
  • A benign proliferation of blood
    vessels
  • May be classified on the basis of
    the size of the vascular channels
    as cavernous (large vessels) or
    capillary (small vessels)
  • Most common in children, where
    most are located in the skin
    (birthmarks) and most involute
    by end of puberty
  • Not ususally congenital, but
    arise during first few weeks
    of post-natal life
  • Undergo a rapid growth
    phase and then gradually
    involute
23
Q

Kasabach-Merritt Syndrome
(3)

A
  • Infants
  • Large, extensive hemangiomas
    trap platelets, producing
    thrombocytopenia, leading to
    hemorrhage
  • High mortality
24
Q

Angiosarcoma
(3)

A
  • Malignancy of vascular
    endothelium
  • May resemble bruise on
    the scalp or forehead of
    the elderly
  • Rarely seen in the oral
    mucosa
25
Q

Kaposi Sarcoma
(3)

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
26
Q

Lymphangioma
(3)

A
  • Benign tumor of lymphatic vessels
  • Sequestration of lymphatic tissue
  • Most arise during childhood
27
Q

LYMPHANGIOMA -

A

benign tumor of lymphatic vessels

28
Q

LYMPHOMA -

A

malignant tumor of lymphocytes

29
Q

Lymphangioma
(2)

A
  • Oral mucosal lymphangioma
  • Cervical lymphangioma
    (Cystic Hygroma)
30
Q

Oral Mucosal Lymphangioma
(2)

A

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

31
Q

Cystic Hygroma - Cervical Lymphangioma
(2)`

A
  • A lymphangioma involving the
    soft tissues of the neck
  • May be associated with
    dysphagia and airway
    obstruction
32
Q

Treatment and Prognosis for Lymphangiomas
(3)

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
33
Q

Lymphangiosarcoma
(2)

A
  • Malignant neoplasm of lymphatic
    endothelium
  • Occurs in long-standing cases of
    lymphedema secondary to
    lymphatic dysfunction
34
Q
  • Hamartoma -
A

developmental
overgrowth of tissue native to
the site

35
Q
  • Choristoma -
A

developmental
overgrowth of tissue not native
to the site

36
Q

Leiomyoma
(3)

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”
  • May also arise from vascular or hair
    follicle (arrector pili) smooth
    muscle
37
Q

Leiomyoma of the Oral Mucosa
Oral mucosal leiomyomas usually
arises from vascular smooth muscle
and are referred to as —

A

angiomyomas

38
Q

Skeletal Muscle

A
  • Malignant neoplasms are more common than benign neoplasms
39
Q
  • Rhabdomyoma –
A

a benign neoplasm of skeletal muscle

40
Q
  • Rhabdomyosarcoma –
A

a malignant neoplasm of skeletal muscle

41
Q

Rhabdomyosarcoma
(2)

A
  • Rhabdomyosarcoma is the most
    common soft tissue sarcoma of
    children
  • Most frequent site is head and neck
    followed by genitourinary
42
Q

Multimodal Treatment of Rhabdomyosarcoma
(4)

A
  • Local surgical excision
  • Multi-agent
    chemotherapy
  • Postoperative radiation
    therapy
  • Five-year survival
    improved with multimodal
    treatment
43
Q

Keloid
(4)

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