Soft Tissue Tumors (Mesenchymal Pathology) Flashcards
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Abbreviated Classification of Soft Tissue Tumors by Histologic Differentiation
(6)
- Adipose tissue
- Peripheral nerve
- Smooth muscle
- Skeletal muscle
- Fibrous tissue
- Vascular tissue
Lipoma
(4)
- 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
Solitary Neurofibroma
(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
- The neurofibroma may be
seen in two clinical
settings
(2)
– A solitary lesion
– As part of a syndrome -
neurofibromatosis type I
Neurofibromatosis Type I
(5)
- Von Recklinghausen’s disease of skin
- Autosomal dominant
- Cutaneous neurofibromas
- Café-au-lait pigmentation
- Malignant transformation
Neurofibromatosis Type I – Lisch Nodules
Benign, pigmented lesions on the iris that do not interfere with vision
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Neurofibromatosis Type I
Ages 21, 36, 56 and 69
Diagnostic Criteria for Neurofibromatosis Type I - at least 2 of these:
* Café au lait macules
(2)
– Prepubertal
* 6 or more over 0.5 cm
– Postpubertal
* 6 or more over 1.5 cm
Diagnostic Criteria for Neurofibromatosis Type I - at least 2 of these:
Neurofibromas
– Plexiform
* Any
– Conventional
* 2 or more
Diagnostic Criteria for Neurofibromatosis Type I - at least 2 of these:
* Freckling
– Axillary, or
– Inguinal
Diagnostic Criteria for Neurofibromatosis Type I - at least 2 of these:
* Eye
– Optic glioma
– Lisch nodules (iris hamartoma)
* 2 or more
Diagnostic Criteria for Neurofibromatosis Type I - at least 2 of these:
* Bone
(2)
– Sphenoid dysplasia, or
– Thinning of long bone cortex
Diagnostic Criteria for Neurofibromatosis Type I - at least 2 of these:
* — degree relative with NF I
First
Six or more café au lait macules
— cm or larger is strong
evidence of neurofibromatosis
1.5
Schwannoma - Neurilemmoma
(2)
- An encapsulated benign
neoplasm of Schwann cells
that pushes the nerve trunk
aside - Histopathology exhibits
Verocay bodies
skipped
Verocay Body
A central eosinophillic zone surrounded by palisaded nuclei
Multiple Endocrine Neoplasia Type IIb
(3)
- Mucosal neuromas - markers for internal malignancy
- Medullary Carcinoma of Thyroid (75%)
- Adrenal Pheochromocytoma
Granular Cell Tumor
(5)
- 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
Congenital Epulis of the Newborn
(4)
- 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
Traumatic Neuroma
(3)
- Damage to peripheral
nerve causes reactive
proliferation of neural
tissue - A reactive lesion and not
a true neoplasm - Pain is variable
Malignant Peripheral Nerve Sheath Tumor
(3)
- Malignant schwannoma,
neurofibrosarcoma - Malignant transformation of a
neurofibroma in NF I - Poor prognosis
Hemangioma
(5)
- 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
Kasabach-Merritt Syndrome
(3)
- Infants
- Large, extensive hemangiomas
trap platelets, producing
thrombocytopenia, leading to
hemorrhage - High mortality
Angiosarcoma
(3)
- Malignancy of vascular
endothelium - May resemble bruise on
the scalp or forehead of
the elderly - Rarely seen in the oral
mucosa
Kaposi Sarcoma
(3)
- 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
Lymphangioma
(3)
- Benign tumor of lymphatic vessels
- Sequestration of lymphatic tissue
- Most arise during childhood
LYMPHANGIOMA -
benign tumor of lymphatic vessels
LYMPHOMA -
malignant tumor of lymphocytes
Lymphangioma
(2)
- Oral mucosal lymphangioma
- Cervical lymphangioma
(Cystic Hygroma)
Oral Mucosal Lymphangioma
(2)
A focal superficial lesion of oral
mucosa frequently with a pebbly
surface
* The tongue is the most common
site and may produce
macroglossia
Cystic Hygroma - Cervical Lymphangioma
(2)`
- A lymphangioma involving the
soft tissues of the neck - May be associated with
dysphagia and airway
obstruction
Treatment and Prognosis for Lymphangiomas
(3)
- 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
(2)
- Malignant neoplasm of lymphatic
endothelium - 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
(3)
- 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
Leiomyoma of the Oral Mucosa
Oral mucosal leiomyomas usually
arises from vascular smooth muscle
and are referred to as —
angiomyomas
Skeletal Muscle
- Malignant neoplasms are more common than benign neoplasms
- Rhabdomyoma –
a benign neoplasm of skeletal muscle
- Rhabdomyosarcoma –
a malignant neoplasm of skeletal muscle
Rhabdomyosarcoma
(2)
- Rhabdomyosarcoma is the most
common soft tissue sarcoma of
children - Most frequent site is head and neck
followed by genitourinary
Multimodal Treatment of Rhabdomyosarcoma
(4)
- Local surgical excision
- Multi-agent
chemotherapy - Postoperative radiation
therapy - Five-year survival
improved with multimodal
treatment
Keloid
(4)
- Complication of wound healing
- Excessive scar formation
- Scar tissue grows beyond the
boundaries of the original
wound - African-Americans
