Soft Tissue Tumors (Mesenchymal Pathology)

Question 1

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

Answer 1

• Adipose tissue
• Peripheral nerve
• Smooth muscle
• Skeletal muscle
• Fibrous tissue
• Vascular tissue

Question 2

Lipoma
(4)

Answer 2

• 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

Question 3

Solitary Neurofibroma
(3)

Answer 3

• 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

Question 4

• The neurofibroma may be
  seen in two clinical
  settings
  (2)

Answer 4

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

Question 5

Neurofibromatosis Type I
(5)

Answer 5

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

Question 6

Neurofibromatosis Type I – Lisch Nodules

Answer 6

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

Question 7

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Neurofibromatosis Type I

Answer 7

Ages 21, 36, 56 and 69

Question 8

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

Answer 8

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

Question 9

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

Answer 9

– Plexiform
* Any
– Conventional
* 2 or more

Question 10

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

Answer 10

– Axillary, or
– Inguinal

Question 11

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

Answer 11

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

Question 12

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

Answer 12

– Sphenoid dysplasia, or
– Thinning of long bone cortex

Question 13

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

Answer 13

First

Question 14

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

Answer 14

1.5

Question 15

Schwannoma - Neurilemmoma
(2)

Answer 15

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

Question 16

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Verocay Body

Answer 16

A central eosinophillic zone surrounded by palisaded nuclei

Question 17

Multiple Endocrine Neoplasia Type IIb
(3)

Answer 17

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

Question 18

Granular Cell Tumor
(5)

Answer 18

• 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

Question 19

Congenital Epulis of the Newborn
(4)

Answer 19

• 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

Question 20

Traumatic Neuroma
(3)

Answer 20

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

Question 21

Malignant Peripheral Nerve Sheath Tumor
(3)

Answer 21

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

Question 22

Hemangioma
(5)

Answer 22

• 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

Question 23

Kasabach-Merritt Syndrome
(3)

Answer 23

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

Question 24

Angiosarcoma
(3)

Answer 24

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

Question 25

Kaposi Sarcoma (3)

Answer 25

* 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

Question 26

Lymphangioma (3)

Answer 26

* Benign tumor of lymphatic vessels * Sequestration of lymphatic tissue * Most arise during childhood

Question 27

LYMPHANGIOMA -

Answer 27

benign tumor of lymphatic vessels

Question 28

LYMPHOMA -

Answer 28

malignant tumor of lymphocytes

Question 29

Lymphangioma (2)

Answer 29

* Oral mucosal lymphangioma * Cervical lymphangioma (Cystic Hygroma)

Question 30

Oral Mucosal Lymphangioma (2)

Answer 30

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

Question 31

Cystic Hygroma - Cervical Lymphangioma (2)`

Answer 31

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

Question 32

Treatment and Prognosis for Lymphangiomas (3)

Answer 32

* 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

Question 33

Lymphangiosarcoma (2)

Answer 33

* Malignant neoplasm of lymphatic endothelium * Occurs in long-standing cases of lymphedema secondary to lymphatic dysfunction

Question 34

* Hamartoma -

Answer 34

developmental overgrowth of tissue native to the site

Question 35

* Choristoma -

Answer 35

developmental overgrowth of tissue not native to the site

Question 36

Leiomyoma (3)

Answer 36

* 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

Question 37

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

Answer 37

angiomyomas

Question 38

Skeletal Muscle

Answer 38

* Malignant neoplasms are more common than benign neoplasms

Question 39

* Rhabdomyoma –

Answer 39

a benign neoplasm of skeletal muscle

Question 40

* Rhabdomyosarcoma –

Answer 40

a malignant neoplasm of skeletal muscle

Question 41

Rhabdomyosarcoma (2)

Answer 41

* Rhabdomyosarcoma is the most common soft tissue sarcoma of children * Most frequent site is head and neck followed by genitourinary

Question 42

Multimodal Treatment of Rhabdomyosarcoma (4)

Answer 42

* Local surgical excision * Multi-agent chemotherapy * Postoperative radiation therapy * Five-year survival improved with multimodal treatment

Question 43

Keloid (4)

Answer 43

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