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Good morning
Seminar on
Spindle cell tumors of the oral cavity
Part - I
By: Dr. Madhusudhanreddy
III year PG
VDC
Contents
• Introduction
• Causes for development of spindle cell tumors
• Various classifications proposed for spindle cel...
Introduction
• These lesions are highly variable both biologically and
clinically
• Clinical, histological, and immunohist...
Causes
• Spindle cells arise among actively dividing connective
tissue/epithelial cells
• Causes
– Genetic predisposition
...
Classification
• Classification – based on the tissue of origin into –
I. Tumors of fibrous origin
II. Tumors of Fibro his...
Benign Malignant
Fibroma Fibrosarcoma
Fibromatosis
Myofibroma
Benign Malignant
Fibrous
histiocytoma
Malignant fibrous
hist...
• Histological patterns:
• Monomorphic - Uniform spindle cells
– Fascicles
– Storiform pattern or a ‘herringbone’ pattern
...
• Biphasic - 2 distinct components
– Spindle cell areas
– Epithelioid areas
• Epithelioid areas may be sparse
• Myxoid - S...
Monomorphic spindle cell
lesion
Pleomorphic
spindle cell
lesion
Biphasic spindle
cell lesion
Myxoid pattern
MPNST MPNST MP...
• Neural tumor
– Neurofibroma
– Schwannoma
– Traumatic neuroma
– Ancient Schwannoma
– Palisaded encapsulated neuroma
– Mal...
• Neural tumors
– Neurofibroma
– Neurilemmoma
– Palisaded encapsulated neuroma
– Traumatic neuroma
– Malignant peripheral ...
• Odontogenic tumors
– Ameloblastic fibroma
– Ameloblastic fibrosarcoma
– Central odontogenic fibroma
– Desmoplastic amelo...
Fibroblastic tumors
• Benign
• Fibroma
• Fibromatosis
• Myofibroma
• Malignant
• Fibrosarcoma
Fibroma
• Synonyms: Irritation fibroma; Traumatic fibroma; Focal
fibrous hyperplasia; Fibrous nodule
• Reactive fibrosis and scarr...
• Demographics:
• Age: Most common in the 4th to 6th decades of life.
• Sex: No sex predilection
• Site: Any oral site
• C...
Elevated smooth-
surfaced pink nodule
HyperkeratosisMillimeters to a few
centimeters
Asymptomatic
Clinical features
• Histopathology:
Pedunculated fibroma
Atrophy of the epithelium, PSSE
CT gradually blends
Collagen bundles – streaming pa...
Dense, hyalinized collagen in
sclerotic fibroma
Hyperkeratosis due to irritation
Collagen bundles in whirling pattern with numerous
blood capillaries – less inflammatory cell
• Differential diagnosis:
– Giant cell fibroma
– Solitary Neurofibroma (encapsulation)
– Palisaded encapsulated neuroma (e...
Giant cell fibroma
• Not associated with any chronic irritation.
• It represents approximately 2% to 5% of all oral fibrou...
• Clinical features:
• It appears as an asymptomatic, sessile, or a pedunculated
nodule, usually less than 1 cm in size, w...
• Histopathology:
Lobulated growth, hyperparakeratinized
SSE
Thin elongated rete ridges
Avascular CT
Stellate fibroblasts within the
superficial connective tissue
Mono or multinucleated Fibroblasts
• Differential diagnosis:
• Papilloma
• Retrocuspid papilla (Site specific)
• Irritational fibroma
• Treatment:
• Conserva...
fibromatosis
• Definition: Benign, recurring but nonmetastasizing, infiltrative,
fibroblastic proliferations arising in any part of the...
Fibromatosis gingivae
• Synonyms: Elephantiasis gingivae; Hereditary gingival
fibromatosis; Congenital macrogingivae
• A d...
• Demographics:
• Age: Occurs before 20 yr of age
• Sex: No sex predilection
• Site: Maxillary gingiva is frequently effec...
• Clinical features:
• May be familial or idiopathic.
• Hereditary condition – dominant autosomal gene.
• Sometimes Sporad...
• Other findings seen in conjunction with gingival
fibromatosis are
- Hyper trichosis
- Epilepsy
- Mental retardation
- Se...
• Can be associated with one of the several hereditary
syndromes like –
- Zimmermann-laband syndrome
- Murray-Puretic-Dres...
• Dense hypocellular, hypovascular collagenous tissue
• Irregularly arranged interlacing bundles
• Epithelium – thing long...
• Differential diagnosis:
• Plasma cell gingivitis
• Systemic granulomatosis causing gingival hyperplasias
• Amyloidosis
•...
Juvenile fibromatosis
• Fibromatoses – a broad group of fibrous proliferations
• Biologic behavior & histologic pattern – ...
Clinical Features:
A firm, painless mass - rapid or
insidious growth.
occurs in children or in young adults
Most common in...
Poorly circumscribed &
infiltrates the adjacent tissues
Streaming fascicles of spindled cells
highly cellular with
variabl...
Reactive to smooth muscle actin (SMA), Vimentin and
nonreactive for Desmin, S-100, cytokeratin
• Differential diagnosis:
• Dysplastic mesenchymal cells
– Fibrosarcoma
– Malignant fibrous histiocytoma (MFH)
– Fibroblas...
• Treatment:
• A locally aggressive tumor
• Wide excision, including a generous margin of the adjacent
normal tissues.
• R...
Aggressive fibromatosis
• A non-metastasizing tumor-like fibroblastic growth
• Unknown pathogenesis
• It involves the volu...
Clinical features:
• Commonly occurs in the shoulder girdle, thigh & buttock
of young adults.
• In oral cavity anterior ma...
Cellular interlacing bundles
Elongated fibroblasts
• Histopathology:
Little or no Pleomorphism
Little or no mitotic activity
Slit-like vascular spaces
No inflammation.
Markers:
• Actin, desmin, & S-100 are negative – some cases may show
patchy expression.
Beta catanin
• Differential diagnosis:
• Reactive fibroblastic proliferation/ myofibroblastic
proliferation
• Desmoplastic fibroma
• Fi...
• Treatment:
• Complete surgical excision, with generous margins of
normal tissue.
• Frequent recurrence
• Prolonged follo...
Good morning
Myofibroblastictumors
• Myofibroma
• Nodular fascitis
• Main cell which are involved in tumor formation is – myofibroblasts
• Discovered in 1971
• Distinct biochemically, pharm...
• Occurs both physiologically and pathologically
• Physiologically
– Developing human palatal mucosa
– Intestinal mucosa
–...
Origin
pathogenesis
Myofibroma
• Also called as myofibromatosis
• A rare spindle cell neoplasm that consists of myofibroblasts
• It was originally descri...
Clinical features
• Demographics:
• Age: Newborns to old age
• Sex: Common in males M-F ratio - 1.5:1
• Site: Most common ...
Clinical features:
• Rare – in head & neck region.
• Usually a painless mass – some times exhibits a rapid
enlargement.
• ...
• Clinical differential diagnosis
• Tongue
– Irritation fibroma,
– Granular cell tumor
– Neurofibroma
– Schwannoma
• Bucca...
Histopathology:
• Nodular fascicles may alternate with more cellular zones,
imparting a biphasic appearance to the tumor.
...
Interlacing bundles of spindle cells
blunt-ended nuclei & eosinophilic
cytoplasm
Hemangiopericytoma like
areas
Scattered mitoses
Cells are positive for smooth muscle
actin
• Histological Differential diagnosis:
• Tongue
– Neurofibroma (S-100)
– Leiomyoma (desmin and h-caldesmon)
• Buccal mucos...
• Markers:
• Positive for vimentin and actin
• Negative for Desmin, CD34, h-caldesmon, cytokeratin and
S-100.
• Differenti...
Treatment:
• Solitary tumors – surgical excision
• Recurrent tumors – re-excision.
• In some cases – spontaneous regressio...
Nodular fascitis
• Definition: A pseudosarcomatous, self-limiting, reactive process
composed of fibroblasts and myofibrobl...
• Clinical features:
• Rapidly growing (1 to 2 months), sometimes painful nodule
• Exophytic lesion some times may be ulce...
• Clinical differential diagnosis
• Exophytic lesion, occasionally secondarily ulcerated,
especially when located in the b...
• Clinical differential diagnosis
• Solitary, deep nodule
– Salivary gland tumors
– Solitary fibrous tumor
– Neurofibroma
...
Well delineated, unencapsulated with focal
areas of infiltration into the adjacent
tissues.
Histopathology
Spindle cells i...
• Histological differential diagnosis
– Neurofibroma
– Fibrous histiocytoma
– Fibromatosis
• Spindle cell shows both myofi...
Smooth muscle actin
vimentin that shows strong and
diffuse cytoplasmic positivity
• Treatment
• Local excision is the treatment of choice
FIBROSARCOMA
• Defination: a malignant tumor composed of Fibroblasts
arranged in a herringbone pattern, with varying amounts of
collage...
• Demographics:
• Age: 30 – 50 yrs (wide age range)
• Sex: no sex predilection
• Site: Buccal mucosa, tongue – 1/4th of th...
• Clinical features:
• Large, deep-seated, slow-growing, solitary Mass
• May occur at site of prior injury, burn
• Radioth...
• Clinical differential diagnosis
• Squamous cell carcinoma
• Osteosarcoma
• Radiological features:
• If in contact with bone, may be visible periosteal reaction
• Purely osteolytic lesion without ...
• Histological grading of fibrosarcoma
– Based on
• Cellularity
• Differentiation
• Mitotic activity
• Necrosis
Herring bone pattern
Histopathology:
Bipolar spindle cells scanty
cytoplasm
Variation in shape and size
Mitotic activity moderate Mild
pleomorphism
Low grade fibrosarcoma resembling
fibromatosis
High grade fibrosarcoma
Closely packed and less well oriented cells
Round tumor cells with high grade nuclear
features
Marked vimentin positivity
• Differential diagnosis
• Benign:
– Fibromatosis
– Nodular fascitis
– Cellular BFH (VIIIa, CD68)
Lesion Cytokeratin S-100 CD 34 SMA
Fibrosarcoma Negative Negative Negative Positive Positive for Vimentin
MPNST Negative P...
Fibrous histiocytic tumors
• Histiocytes – connective tissue macrophages.
• Also called sessile macrophages or resting migratory cells.
• Part of the...
• Nuclei - Eccentrically located are smaller & more densely
structured than fibroblast nuclei.
• Apart from the usual cell...
• Fibrohistiocytic tumors – cellular elements resemble both
fibroblasts & histiocytes.
• Originally, they were believed to...
BENIGNFIBROUS HISTIOCYTOMA
• Diverse group of neoplasms – both fibroblastic & histiocytic
differentiation.
• The cell of origin – histiocyte
• Varied...
Clinical features:
• Skin of the extremities – as a small,
firm nodule.
• Predominantly on the buccal mucosa
& vestibule.
...
Submucosal aggrigation of Spindle-
shaped, fibroblast-like cells
Scattered rounded histiocytic cells and
Foamy histiocytes...
Spindle cells in BFH
Storiform arrangement of
histiocytes
Touton giant cells engulf lipid and
hemosiderin
Mixture of spindle cells and
xanthomatous cells
BFH with myxoid changes
Hemangiopericytoma like areas in
BFH
Plump spindle cells with
ocassionally mitotic figures
Factor XIIIa immunostaining of BFH
• Differential diagnosis:
• Xanthogranuloma or juvenile xanthogranuloma
– Proliferating sheets of histiocytes with few Tou...
• Above lesions are usually diagnosed during the first two
decades of life.
• The histiocytic cells of a xanthoma
– Typica...
• Benign fibrous histiocytoma is often confused with other
benign fibrous lesions like
- Nodular fasciitis
- Myofibroma
- ...
• BFH should also be differentiated from aggressive forms of
fibrous and fibrohistiocytic neoplasms such as
- Dermatofibro...
• Treatment & Prognosis:
• Wide surgical excision.
• Recurrence rate – 5-10%
• Deeper & larger lesions – higher rate of re...
Malignent fibrous histiocytoma
• Definition: A high-grade, pleomorphic spindle cell sarcoma with
a storiform pattern; matrix calcification or ossificatio...
• Demographics:
• More common in white patients than in patients of African
or Asian descent.
• Site: Mandible, maxilla, s...
• Clinical features:
• United States – MFH accounts 20-24% of soft-tissue sarcomas
• Enlarging painless intramuscular soft...
• Clinical differential diagnosis
• Rabdomyoma
• Leiomyoma
• Lipoma
• Liposarcoma
• Five histological subtypes of MFH
– Storiform/pleomorphic (most common),
– Myxoid,
– Giant cell,
– Inflammatory (usually...
lesions show pleomorphic, spindle cells in
a storiform pattern
Predominately histiocytic-like pattern
with bizarre round c...
Malignant spindle cell having abundant
eosinophilic cytoplasm with
hyperchromatic, pleomorphic nuclei
Positive expression ...
• Differential diagnosis:
– Cellular benign fibrous histiocytoma
– Aggressive fibromatosis
– Fibrosarcoma
Markers:
• Histochemistry of the tumor reveals
- Vimentin positivity
- CD 68 positive
- Melanoma markers (S100, HMB45) neg...
• Treatment:
• Complete surgical ablation with disease-free margins
• Chemotherapy is often a helpful adjuvant
• 5-year su...
Lipomatous tumors
Lipomatous tumors
• Benign tumors
– Spindle cell lipoma/
Pleomorphic
– Vascular (angiolipoma)
– Lipoblastoma
– Hibernoma
–...
Spindle cell/pleomorphic lipoma
• Enzinger and Harvey in 1975
• Both the lesions were grouped under “atypical lipomas”
• Defination: A spectrum of benign ...
• Clinical features:
– Slow growing
– Solitary
– Circumscribed
– Painless
– Firm nodule
Clinical differential diagnosis
• Buccal mucosa
– Salivary gland tumors
– Solitary fibrous tumor
– Neurofibroma
– Schwanno...
Well circumscription
Mature adipocytes
Cellular area
Ropy collagen
Histopathology:
Bland spindle cells with elongated
nucleus
Myxoid change
Hyperchromatic multinucleated giant
cells
CD 34 positivity
• Differential diagnosis
• Nodular fascitis
• Schwannoma
• Neurofibroma
• Solitary fibrous tmor
• Hemangiopericytoma
• Myx...
Spindle cell liposarcoma
• Definition: Proliferation of mature adipocytes exhibiting
marked variation in cell size with at least focal nuclear atyp...
• Demographics:
• Age: 4 - 6th decade
• Sex: Male
• Site: Majorly effects cheeks
• Other sites: floor of the mouth, palate...
Clinical differential diagnosis
• Buccal mucosa
– Salivary gland tumors
– Solitary fibrous tumor
– Neurofibroma
– Schwanno...
Prominent spindle cells
Resembling SCL
Scattered lipoblasts
In various shapes and sizes
Histopathology:
Variable spindle cells devoid of
lipoblasts
Nuclear Atypia
• IHC Markers: S-100 positivity in lipogenic areas; nuclear
expression of MDM2 and CDK4
• Differential diagnosis
– Neural
...
Smoothmuscle tumors
• Benign tumors
• Pilar
• Genital
• Vascular/Angiomyoma
• Leiomyoma of deep soft
tissue
• Retroperitonial
• Malignant tumo...
Leiomyoma
• Definition: Benign mesenchymal tumor with smooth muscle
differentiation that is classified according to location as pila...
Clinical features:
• Clinical differential diagnosis
– Fibroma
– Neurofibroma
– Lipoma
– Leiomyosarcoma
– Mucocele
– Pleomorphic adenoma
– Ly...
Monomorphic spindle cells with
cigar shaped nuclei
Stori form pattern
Masson trichrome
Histopathology:
VASCULAR LEIOMYOMA
Actin immunostain
Fascicles of spindle cells
Spindle cells are SMA positive
CD31 reveals many vessels
Other markers
Vimentin, Desmin, h-caldesmon
• Histological Differential diagnosis
– Fibromatosis
– Myofibroma
– Neurofibroma
– Schwannoma
– Special stains – collagen
...
Leiomyosarcoma
• Definition: Malignant mesenchymal tumor with smooth
muscle differentiation that can be categorized as cutaneous,
vascula...
• Clinical features:
• Typically presents as a painless, lobulated, fixed mass of
the submucosal
• Secondary ulceration of...
• Clinical differential diagnosis
– Fibroma
– Neurofibroma
– Lipoma
– Leiomyosarcoma
– Mucocele
– Pleomorphic adenoma
– Ly...
Fascicles of interlacing monomorphic
spindle-shaped cells with abundant
eosinophilic cytoplasm
Moderately large, blunt-end...
• One study on leiomyosarcoma of head and neck found that
– LMS are well circumscribed
– Focal areas of infiltration
– Mit...
Differential diagnosis:
• Treatment
• Early surgical excision with radical neck dissection for
lymph node metastasis
• Chemotherapy and radiothera...
Referrences
• Brad . W . Neville , Douglas D . Damm , Carl M . Allen Oral and
maxillofacial pathology 2nd edition 2004
• S...
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Spindle cell lesions of oral cavity part I

Introduction, various types of Classification of spindle cell tumors

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Spindle cell lesions of oral cavity part I

  1. 1. Good morning
  2. 2. Seminar on Spindle cell tumors of the oral cavity Part - I By: Dr. Madhusudhanreddy III year PG VDC
  3. 3. Contents • Introduction • Causes for development of spindle cell tumors • Various classifications proposed for spindle cell lesions • Clinical and histological features of individual tumor • Approach for diagnosis of spindle cell tumors
  4. 4. Introduction • These lesions are highly variable both biologically and clinically • Clinical, histological, and immunohistochemical some times electron microscopy • Predominant cells – spindle cells / altered spindle cells • Ranging from simple reactive to malignant lesions • Uncommon - oral cavity, • Common in other sites of the body. – Head and neck – Soft tissues of scalp – Orbit – Upper aerodigestive tract (UADT) • Account for - 1% of all tumors in the oral regions.
  5. 5. Causes • Spindle cells arise among actively dividing connective tissue/epithelial cells • Causes – Genetic predisposition – Chromosomal mutations – Cellular genetic defects in Oncogene, tumor suppressor genes • Example NF1 in neurofibromatosis – Exposure to radiation or certain chemicals, – Trauma, and inflammation, • All of which may stimulate the tissues to divide more rapidly than normal.
  6. 6. Classification • Classification – based on the tissue of origin into – I. Tumors of fibrous origin II. Tumors of Fibro histiocytic origin III.Tumors of adipose tissue origin IV.Tumors of Smooth muscle origin V. Tumors of Skeletal muscle origin VI.Tumors of Nerve tissue origin VII.Tumors of Vascular origin VIII.Tumors of Bone IX. Tumors of epithelial origin
  7. 7. Benign Malignant Fibroma Fibrosarcoma Fibromatosis Myofibroma Benign Malignant Fibrous histiocytoma Malignant fibrous histiocytoma Benign Malignant Lipoma (spindle cell and pleomorphic lipoma) Dedifferentiated liposarcoma 1. Fibroblastic tumors 2. Fibrohistiocytic tumors 3. Lipomatous tumors Benign Malignant Cellular rhabdomyoma Spindle cell rhabdomyosarcoma Benign Malignant Leiomyoma (angiomyoma) Leiomyosarcoma Benign Malignant Spindle cell hemangioendothelioma Angiosarcoma Kaposi sarcoma 4. Smooth muscle tumors 5. Skeletal muscle tumors 6. Vascular tumors Benign Malignant Traumatic neuroma Neurofibrosarcoma Neurofibroma Schwannoma Malignant Fibroblastic osteosarcoma Malignant Synovial sarcoma 7. Neural tumors 8. Bone tumors 9. Synovial tumor 10. Epithelial tumors Benign Malignant Nevus (intramucosal, spitz nevus) Melanoma Spindle cell carcinoma
  8. 8. • Histological patterns: • Monomorphic - Uniform spindle cells – Fascicles – Storiform pattern or a ‘herringbone’ pattern – Sometimes associated with a dense collagenous stroma. • Pleomorphic - Spindle cells - variation in size and shape, often with marked nuclear pleomorphism and tumour giant cells.
  9. 9. • Biphasic - 2 distinct components – Spindle cell areas – Epithelioid areas • Epithelioid areas may be sparse • Myxoid - Spindle cell component may be conspicuous or less easily identified, but it is set in a loose, myxoid stroma. • Prominent inflammatory infiltrate. Catriona E Anderson, Awatif Al-Nafussi; Spindle cell lesions of the head and neck: an overview and diagnostic approach. Diagnostic histopathology, 2009; 15:5 265 -272
  10. 10. Monomorphic spindle cell lesion Pleomorphic spindle cell lesion Biphasic spindle cell lesion Myxoid pattern MPNST MPNST MPNST Leiomyosarcoma Leiomyosarcoma Leiomyosarcom Synovial sarcoma Synovial sarcoma Synovial sarcoma Solitary fibrous tumour Carcinosarcoma Schwannoma Cellular schwannoma Myxoma Vascular leiomyoma Inflammatory myoblastic tumour Nasopharyngeal angiofibroma Nodular fasciitis Desmoid –type fibromatosis Myofibrosarcoma Myofibroma/ Myofibromatosis Myofibrosarcoma Myoepithelioma Odontogenic fibroma • Sarcomatoid squamous carcinoma, Melanoma occurs in all the 4 patterns.
  11. 11. • Neural tumor – Neurofibroma – Schwannoma – Traumatic neuroma – Ancient Schwannoma – Palisaded encapsulated neuroma – Malignant peripheral nerve sheath tumor • Myofibroblastic tumors – Myofibroma – Inflammatory myofibroblastic tumor – Low-grade myofibroblastic sarcoma • Smooth muscle tumors – Angiomyoma/vascular leiomyoma • Fibroblastic tumors – Solitary fibrous tumor – Nodular fasciitis – Fibromatosis – Desmoplastic fibroma – Fibrosarcoma • Vascular tumors – Vascular malformation – Kaposi’s sarcoma • Carcinomas – Spindle cell SCC • Other – Sarcoma NOS/Atypical spindle cell neoplasm – Benign fibrous histiocytoma Jordan RC, Regezi JA. Oral spindle cell neoplasms: a review of 307 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2003;95:717-24.
  12. 12. • Neural tumors – Neurofibroma – Neurilemmoma – Palisaded encapsulated neuroma – Traumatic neuroma – Malignant peripheral nerve sheath tumors • Myofibroblastic tumors – Myofibroma – Inflammatory myofibroblastic tumor – Low grade myofibrosarcoma • Muscle tumors – Leiomyoma – Vascular leiomyoma – Leiomyosarcoma – Rabdomyoma – Rabdomyosarcoma • Fibroblastic tumors – Solitary fibrous tumor – Fibromatosis – Nodular fascitis – Desmoplastic fibroma – Fibrosarcoma • Vascular tumors – Spindle cell hemangioma – Hemangiopericytoma – Kaposi sarcoma • Epithelial tumors – Pleomorphic adenoma – Spindle cell carcinoma – Malignant melanoma Thorakkal Shamim: A simple working type classification proposed for spindle cell neoplasma of oral cavity; journal of cytology, 2013; vol- 30; issue- 1; 85
  13. 13. • Odontogenic tumors – Ameloblastic fibroma – Ameloblastic fibrosarcoma – Central odontogenic fibroma – Desmoplastic ameloblastoma • Miscellaneous tumors – Benign fibrous histiocytoma – Malignant fibrous histiocytoma – Synovial sarcoma – Ossifying fibromyxoid tumor – Giant cell angiofibroma – Blue nevus Thorakkal Shamim: A simple working type classification proposed for spindle cell neoplasma of oral cavity; journal of cytology, 2013; vol- 30; issue- 1; 85
  14. 14. Fibroblastic tumors • Benign • Fibroma • Fibromatosis • Myofibroma • Malignant • Fibrosarcoma
  15. 15. Fibroma
  16. 16. • Synonyms: Irritation fibroma; Traumatic fibroma; Focal fibrous hyperplasia; Fibrous nodule • Reactive fibrosis and scarring - bite trauma • True neoplasm ? • Common benign soft tissue neoplasm of the oral cavity. • Reactive hyperplasia of the fibrous connective tissue, in response to local irritation or trauma.
  17. 17. • Demographics: • Age: Most common in the 4th to 6th decades of life. • Sex: No sex predilection • Site: Any oral site • Common along the plane of occlusion, on the buccal mucosa. • Other sites – Labial mucosa, tongue & the palate.
  18. 18. Elevated smooth- surfaced pink nodule HyperkeratosisMillimeters to a few centimeters Asymptomatic Clinical features
  19. 19. • Histopathology: Pedunculated fibroma Atrophy of the epithelium, PSSE CT gradually blends Collagen bundles – streaming pattern
  20. 20. Dense, hyalinized collagen in sclerotic fibroma Hyperkeratosis due to irritation
  21. 21. Collagen bundles in whirling pattern with numerous blood capillaries – less inflammatory cell
  22. 22. • Differential diagnosis: – Giant cell fibroma – Solitary Neurofibroma (encapsulation) – Palisaded encapsulated neuroma (encapsulation) – Fibroepithelial polyp – Epulis fissuratum – Peripheral reactive lesions – Peripheral odontogenic lesions – Mucocele • Treatment: • Conservative surgical excision • Recurrence is rare
  23. 23. Giant cell fibroma • Not associated with any chronic irritation. • It represents approximately 2% to 5% of all oral fibrous • In 1974 Weathers and Callihan identified • Demographics: • Age: 10 – 30 years • Sex: No gender predilection • Site: Most commonly it occurs on the mandibular gingiva, followed by tongue, and palate.
  24. 24. • Clinical features: • It appears as an asymptomatic, sessile, or a pedunculated nodule, usually less than 1 cm in size, with a bossilated or a papillary surface.
  25. 25. • Histopathology: Lobulated growth, hyperparakeratinized SSE Thin elongated rete ridges Avascular CT
  26. 26. Stellate fibroblasts within the superficial connective tissue Mono or multinucleated Fibroblasts
  27. 27. • Differential diagnosis: • Papilloma • Retrocuspid papilla (Site specific) • Irritational fibroma • Treatment: • Conservative surgical excision • Recurrence is rare
  28. 28. fibromatosis
  29. 29. • Definition: Benign, recurring but nonmetastasizing, infiltrative, fibroblastic proliferations arising in any part of the oral cavity. • Fibromatosis – Fibromatosis gingivae – Juvinile fibromatosis – Aggressive fibromatosis
  30. 30. Fibromatosis gingivae • Synonyms: Elephantiasis gingivae; Hereditary gingival fibromatosis; Congenital macrogingivae • A diffuse fibrous over growth of gingival tissues. • A slowly progressive - over growth of the gingival fibrous connective tissue. • Occur during shedding of deciduous teeth and early eruption of permanent teeth
  31. 31. • Demographics: • Age: Occurs before 20 yr of age • Sex: No sex predilection • Site: Maxillary gingiva is frequently effected • Generalized or localized • Delayed eruption
  32. 32. • Clinical features: • May be familial or idiopathic. • Hereditary condition – dominant autosomal gene. • Sometimes Sporadic - with no familial background. • Diffuse, smooth or nodular overgrowth of the gingival tissues • Facial and lingual aspects of the attached and marginal gingiva • Single quadrant or all quardents • Painless, slowly progressive
  33. 33. • Other findings seen in conjunction with gingival fibromatosis are - Hyper trichosis - Epilepsy - Mental retardation - Sensori neural deafness - Hypo thyroidism - Chondro dystrophia - Growth hormone deficiency
  34. 34. • Can be associated with one of the several hereditary syndromes like – - Zimmermann-laband syndrome - Murray-Puretic-Drescher syndrome - Ruthefurd syndrome - Multiple hamartoma syndrome (Cowden) - Cross syndrome
  35. 35. • Dense hypocellular, hypovascular collagenous tissue • Irregularly arranged interlacing bundles • Epithelium – thing long rete ridges extending deep into CT • Dystrophic calcification – some times seen. • Electron microscopy demonstrates a mixture of both fibroblasts and myofibroblast-like cells. • Histopathology:
  36. 36. • Differential diagnosis: • Plasma cell gingivitis • Systemic granulomatosis causing gingival hyperplasias • Amyloidosis • Treatment: • Conservative treatment – gingivectomy • Recurrence is very often
  37. 37. Juvenile fibromatosis • Fibromatoses – a broad group of fibrous proliferations • Biologic behavior & histologic pattern – Intermediate between those of benign fibrous lesions and fibrosarcoma. • Head and neck - these lesions are called as – Juvenile aggressive fibromatoses or extra abdominal desmoids. • Lesions within the bone are called as – Desmoplastic fibromas.
  38. 38. Clinical Features: A firm, painless mass - rapid or insidious growth. occurs in children or in young adults Most common in paramandibular soft tissue can occur at other sites also. Facial disfigurement Destruction of the adjacent bone
  39. 39. Poorly circumscribed & infiltrates the adjacent tissues Streaming fascicles of spindled cells highly cellular with variable amount of collagen Hyperchromatic and pleomorphic nuclei are seldom seen Histopathology:
  40. 40. Reactive to smooth muscle actin (SMA), Vimentin and nonreactive for Desmin, S-100, cytokeratin
  41. 41. • Differential diagnosis: • Dysplastic mesenchymal cells – Fibrosarcoma – Malignant fibrous histiocytoma (MFH) – Fibroblastic osteosarcoma (if attached to bone) • Treatment: • A locally aggressive tumor. • Wide excision, including a generous margin of the adjacent normal tissues. • Recurrence rate of for oral and paraoral fibromatses – 23%. • No metastases.
  42. 42. • Treatment: • A locally aggressive tumor • Wide excision, including a generous margin of the adjacent normal tissues. • Recurrence rate for oral and paraoral fibromatses – 23%. • No metastases
  43. 43. Aggressive fibromatosis • A non-metastasizing tumor-like fibroblastic growth • Unknown pathogenesis • It involves the voluntary muscles as well as aponeurotic & fascial structures. • The lesion has a strong tendency to local recurrence & shows an aggressive infiltrating growth.
  44. 44. Clinical features: • Commonly occurs in the shoulder girdle, thigh & buttock of young adults. • In oral cavity anterior maxilla - most common site • Rapidly enlarging or Slow growth. • Pain may or may not be present. • When it is present near the bone, bone destruction occurs.
  45. 45. Cellular interlacing bundles Elongated fibroblasts • Histopathology:
  46. 46. Little or no Pleomorphism Little or no mitotic activity Slit-like vascular spaces No inflammation.
  47. 47. Markers: • Actin, desmin, & S-100 are negative – some cases may show patchy expression. Beta catanin
  48. 48. • Differential diagnosis: • Reactive fibroblastic proliferation/ myofibroblastic proliferation • Desmoplastic fibroma • Fibrosarcoma transformation of fibromatosis
  49. 49. • Treatment: • Complete surgical excision, with generous margins of normal tissue. • Frequent recurrence • Prolonged follow-up
  50. 50. Good morning
  51. 51. Myofibroblastictumors • Myofibroma • Nodular fascitis
  52. 52. • Main cell which are involved in tumor formation is – myofibroblasts • Discovered in 1971 • Distinct biochemically, pharmacologicaly and immunohistochemically • Features are typically of smooth muscle • Myofibroblast is seen ultrastructurally • Under light microscopy – Large spindle cell – Stellate with several cytoplasmic extensions – Acidophillic or amphopillic and fibrillar cytoplasm – Nucleus finely granular with conspicuous nucleoli
  53. 53. • Occurs both physiologically and pathologically • Physiologically – Developing human palatal mucosa – Intestinal mucosa – PDL of mouse and rat – tooth eruption – Lymphnodes, spleen, bone marrow – Pericytes – Wound healing • Pathologically – Helps in tumor progression
  54. 54. Origin
  55. 55. pathogenesis
  56. 56. Myofibroma
  57. 57. • Also called as myofibromatosis • A rare spindle cell neoplasm that consists of myofibroblasts • It was originally described as a multicentric tumor process affecting infants & young children • The tumor occurs as a solitary mass & at any age.
  58. 58. Clinical features • Demographics: • Age: Newborns to old age • Sex: Common in males M-F ratio - 1.5:1 • Site: Most common Tongue > buccal mucosa > palate > gingiva > mandibular vestibule > retromolar area • least common in the lip • Occurs as an exophytic mass Marilena Vered et al, Clinico-pathologic correlations of myofibroblastic tumors of the oral cavity. II. Myofibroma and myofibromatosis of the oral soft tissues; J Oral Pathol Med (2007) 36: 304–14
  59. 59. Clinical features: • Rare – in head & neck region. • Usually a painless mass – some times exhibits a rapid enlargement. • Intra bony tumors create poorly defined radiolucent defects. • Some may be well defined or multilocular.
  60. 60. • Clinical differential diagnosis • Tongue – Irritation fibroma, – Granular cell tumor – Neurofibroma – Schwannoma • Buccal mucosa – Lymphoid hyperplasia – Lipoma – Salivary gland tumors Marilena Vered et al, Clinico-pathologic correlations of myofibroblastic tumors of the oral cavity. II. Myofibroma and myofibromatosis of the oral soft tissues; J Oral Pathol Med (2007) 36: 304–14
  61. 61. Histopathology: • Nodular fascicles may alternate with more cellular zones, imparting a biphasic appearance to the tumor. • Unencapsulated but circumscribed • Has monomorphic and biphasic spindle cells
  62. 62. Interlacing bundles of spindle cells blunt-ended nuclei & eosinophilic cytoplasm Hemangiopericytoma like areas
  63. 63. Scattered mitoses Cells are positive for smooth muscle actin
  64. 64. • Histological Differential diagnosis: • Tongue – Neurofibroma (S-100) – Leiomyoma (desmin and h-caldesmon) • Buccal mucosa – Nodular fasciitis – Fibrous histiocytoma (CD68) – Solitary fibrous tumor (CD34 and CD99) – Infantile/congenital fibrosarcoma Marilena Vered et al, Clinico-pathologic correlations of myofibroblastic tumors of the oral cavity. II. Myofibroma and myofibromatosis of the oral soft tissues; J Oral Pathol Med (2007) 36: 304–14
  65. 65. • Markers: • Positive for vimentin and actin • Negative for Desmin, CD34, h-caldesmon, cytokeratin and S-100. • Differential diagnosis: Fibromatosis Leiomyoma Nodular fascitis Neural tumors Solitary fibrous tumor S-100 Patchy positive Negative Negative Positive Positive SMA Negative Positive Positive Negative Negative CD-34 Negative Negative Negative Negative positive Desmin Negative Positive Negative Negative Negative β- Catanin Positive Negative Negative Negative Negative
  66. 66. Treatment: • Solitary tumors – surgical excision • Recurrent tumors – re-excision. • In some cases – spontaneous regression. • Those involving the viscera or vital organs in infants – more aggressive & may prove to be fatal.
  67. 67. Nodular fascitis • Definition: A pseudosarcomatous, self-limiting, reactive process composed of fibroblasts and myofibroblasts • Cause of NF is unknown • Trauma is believed to be important • Demographics: • Age: Fourth and fifth decades of life, • Sex: Equal gender distribution • Site: Buccal mucosa, labial mucosa, tongue, Angle of the mandible, inferior border of the mandible, zygomatic arch, anterior mandible
  68. 68. • Clinical features: • Rapidly growing (1 to 2 months), sometimes painful nodule • Exophytic lesion some times may be ulcerated Dan Dayan et al, Clinico-pathologic correlations of myofibroblastic tumors of the oral cavity: 1. nodular fasciitis; J Oral Pathol Med (2005) 34: 426–35
  69. 69. • Clinical differential diagnosis • Exophytic lesion, occasionally secondarily ulcerated, especially when located in the buccal mucosa, – Traumatic ulcer (chronic), – Traumatic granuloma – Salivary gland tumors, – Various infectious processes (e.g. of bacterial or deep fungal origin) – Squamous cell carcinoma Dan Dayan et al, Clinico-pathologic correlations of myofibroblastic tumors of the oral cavity: 1. nodular fasciitis; J Oral Pathol Med (2005) 34: 426–35
  70. 70. • Clinical differential diagnosis • Solitary, deep nodule – Salivary gland tumors – Solitary fibrous tumor – Neurofibroma – Schwannoma – Intramuscular lipoma, – Myofibroma – Fibromatosis Dan Dayan et al, Clinico-pathologic correlations of myofibroblastic tumors of the oral cavity: 1. nodular fasciitis; J Oral Pathol Med (2005) 34: 426–35
  71. 71. Well delineated, unencapsulated with focal areas of infiltration into the adjacent tissues. Histopathology Spindle cells in myxoid background
  72. 72. • Histological differential diagnosis – Neurofibroma – Fibrous histiocytoma – Fibromatosis • Spindle cell shows both myofibroblastic and histiocytic immunoprofile – SMA – MSA – Vimentin – CD68 Dan Dayan et al, Clinico-pathologic correlations of myofibroblastic tumors of the oral cavity: 1. nodular fasciitis; J Oral Pathol Med (2005) 34: 426–35
  73. 73. Smooth muscle actin vimentin that shows strong and diffuse cytoplasmic positivity
  74. 74. • Treatment • Local excision is the treatment of choice
  75. 75. FIBROSARCOMA
  76. 76. • Defination: a malignant tumor composed of Fibroblasts arranged in a herringbone pattern, with varying amounts of collagen in the background
  77. 77. • Demographics: • Age: 30 – 50 yrs (wide age range) • Sex: no sex predilection • Site: Buccal mucosa, tongue – 1/4th of the oral lesions
  78. 78. • Clinical features: • Large, deep-seated, slow-growing, solitary Mass • May occur at site of prior injury, burn • Radiotherapy (postirradiation fibrosarcoma) • Associated with mobility of adjacent teeth and ulceration of overlying mucosa. • Pain and paresthesia - late symptoms indicating nerve involvement
  79. 79. • Clinical differential diagnosis • Squamous cell carcinoma • Osteosarcoma
  80. 80. • Radiological features: • If in contact with bone, may be visible periosteal reaction • Purely osteolytic lesion without calcification • Poorly defined, irregular margins • Destruction of the cortical plates without expansion • Misdiagnosed as an odontogenic abscess or cyst.
  81. 81. • Histological grading of fibrosarcoma – Based on • Cellularity • Differentiation • Mitotic activity • Necrosis
  82. 82. Herring bone pattern Histopathology: Bipolar spindle cells scanty cytoplasm
  83. 83. Variation in shape and size Mitotic activity moderate Mild pleomorphism Low grade fibrosarcoma resembling fibromatosis
  84. 84. High grade fibrosarcoma Closely packed and less well oriented cells Round tumor cells with high grade nuclear features
  85. 85. Marked vimentin positivity
  86. 86. • Differential diagnosis • Benign: – Fibromatosis – Nodular fascitis – Cellular BFH (VIIIa, CD68)
  87. 87. Lesion Cytokeratin S-100 CD 34 SMA Fibrosarcoma Negative Negative Negative Positive Positive for Vimentin MPNST Negative Positive Variable Negative MFH Variable Negative Negative Negative Positive for Leu-3 Synovial sarcoma Positive Variable Negative Negative Desmoplastic leiomyosarcoma Negative Negative Negative Positive Malignant melanoma Negative Positive Negative Negative Spindle cell carcinoma Positive Negative Negative Negative Treatment Wide local excision Radical neck dissection
  88. 88. Fibrous histiocytic tumors
  89. 89. • Histiocytes – connective tissue macrophages. • Also called sessile macrophages or resting migratory cells. • Part of the mononuclear phagocyte system (MPS). • Precursor cells – monocytes – derived from bone marrow. • Function – phagocytose & store substances (scavenger cells). • Phagocytes have irregular shapes. • They are flattened & often show pseudopodia-like processes.
  90. 90. • Nuclei - Eccentrically located are smaller & more densely structured than fibroblast nuclei. • Apart from the usual cell organelles, there are small vesicles, vacuoles, filaments & osmiophilic inclusions. • These may be primary lysosomes, secondary lysosomes or often also inclusions, which have become part of phagolysosomes or residual bodies.
  91. 91. • Fibrohistiocytic tumors – cellular elements resemble both fibroblasts & histiocytes. • Originally, they were believed to be neoplasms of histiocytes. • But studies suggest that the phenotype of the neoplastic cells most closely resembles that of fibroblasts. • Thus, the term fibrohistiocytic is used, which describes its histogenetic origin.
  92. 92. BENIGNFIBROUS HISTIOCYTOMA
  93. 93. • Diverse group of neoplasms – both fibroblastic & histiocytic differentiation. • The cell of origin – histiocyte • Varied microscopic appearances – numerous alternative diagnostic terms such as - Dermatofibroma - Sclerosing hemangioma - Xanthogranuloma - Fibroxanthoma - Nodular subepidermal fibrosis - Epithelioid histiocytoma - Reticulohistiocytoma
  94. 94. Clinical features: • Skin of the extremities – as a small, firm nodule. • Predominantly on the buccal mucosa & vestibule. • Middle-aged & older adults. • Painless sub-mucosal nodule • Variable size – a few millimeters to several centimeters. • Deeper tumors – larger & most lesions cannot be easily moved
  95. 95. Submucosal aggrigation of Spindle- shaped, fibroblast-like cells Scattered rounded histiocytic cells and Foamy histiocytes Histopathology:
  96. 96. Spindle cells in BFH Storiform arrangement of histiocytes
  97. 97. Touton giant cells engulf lipid and hemosiderin Mixture of spindle cells and xanthomatous cells
  98. 98. BFH with myxoid changes Hemangiopericytoma like areas in BFH
  99. 99. Plump spindle cells with ocassionally mitotic figures Factor XIIIa immunostaining of BFH
  100. 100. • Differential diagnosis: • Xanthogranuloma or juvenile xanthogranuloma – Proliferating sheets of histiocytes with few Touton giant cells and foamy cytoplasm. – Better circumscribed and less fibrosed • Langerhan’s cell disease (Histiocytosis X) – Multinucleated giant cells nuclei not pushed to the cell periphery. – Reactive histiocyte – after trauma – Hyperlipidemia or hypercholesterolemia
  101. 101. • Above lesions are usually diagnosed during the first two decades of life. • The histiocytic cells of a xanthoma – Typically express S-100 protein, while the lesional cells of the fibrous histiocytoma do not – Cholesterol clefts are commonly seen in the xanthoma
  102. 102. • Benign fibrous histiocytoma is often confused with other benign fibrous lesions like - Nodular fasciitis - Myofibroma - Palisading encapsulated neuroma - Neurofibroma - Leiomyoma - Spindle cell type of myoepithelioma.
  103. 103. • BFH should also be differentiated from aggressive forms of fibrous and fibrohistiocytic neoplasms such as - Dermatofibrosarcoma protuberans - Malignant fibrous histiocytoma and - Fibrosarcoma
  104. 104. • Treatment & Prognosis: • Wide surgical excision. • Recurrence rate – 5-10% • Deeper & larger lesions – higher rate of recurrence
  105. 105. Malignent fibrous histiocytoma
  106. 106. • Definition: A high-grade, pleomorphic spindle cell sarcoma with a storiform pattern; matrix calcification or ossification is not present • First described by O'Brien & Stout – 1964. • Most common soft-tissue sarcoma of late adult life
  107. 107. • Demographics: • More common in white patients than in patients of African or Asian descent. • Site: Mandible, maxilla, soft tissue of oral cavity • Age: 5th & 6th decades, but an age range of 10-90 years • Sex: Male-to-female ratio – 2:1
  108. 108. • Clinical features: • United States – MFH accounts 20-24% of soft-tissue sarcomas • Enlarging painless intramuscular soft-tissue mass, typically 5–10 cm in diameter • Usually occurs -soft tissues of upper followed by lower extremities. MFH in oral cavity is very rare
  109. 109. • Clinical differential diagnosis • Rabdomyoma • Leiomyoma • Lipoma • Liposarcoma
  110. 110. • Five histological subtypes of MFH – Storiform/pleomorphic (most common), – Myxoid, – Giant cell, – Inflammatory (usually retroperitoneal – Angiomatoid (often located more superficially than other varieties)
  111. 111. lesions show pleomorphic, spindle cells in a storiform pattern Predominately histiocytic-like pattern with bizarre round cells and highly pleomorphic nuclei Histopathology:
  112. 112. Malignant spindle cell having abundant eosinophilic cytoplasm with hyperchromatic, pleomorphic nuclei Positive expression for S-100 protein
  113. 113. • Differential diagnosis: – Cellular benign fibrous histiocytoma – Aggressive fibromatosis – Fibrosarcoma
  114. 114. Markers: • Histochemistry of the tumor reveals - Vimentin positivity - CD 68 positive - Melanoma markers (S100, HMB45) negativity • Carcinoma markers (cytokeratin AE1/3, EMA, CK7, CK20), lymphoma markers (CD45, CD20, CD3, CD4, CD43, CD5, CD30) and markers for specific sarcomas (SMA, CD34, CD117, HHF35, & desmin) are all negative. • Therefore, MFH has become a diagnosis of exclusion
  115. 115. • Treatment: • Complete surgical ablation with disease-free margins • Chemotherapy is often a helpful adjuvant • 5-year survival rate is 34% to 50%
  116. 116. Lipomatous tumors
  117. 117. Lipomatous tumors • Benign tumors – Spindle cell lipoma/ Pleomorphic – Vascular (angiolipoma) – Lipoblastoma – Hibernoma – Chondroid type – Myolipoma – Myelolipoma – Angiomyolipoma • Malignant tumors • Liposarcoma • Classified by WHO as – Well differentiated (WDL)/Atypical lipomatous neoplasm(ANL) – Didifferentiated (DL) – Myxoid/round cell (MRCL) – Pleomorphic – Spindle cell liposarcoma
  118. 118. Spindle cell/pleomorphic lipoma
  119. 119. • Enzinger and Harvey in 1975 • Both the lesions were grouped under “atypical lipomas” • Defination: A spectrum of benign lesion composed of an admixture of mature adipocytes, spindle cells, and hyperchromatic multinucleated giant cells. • Demographics: • Site: Tongue, cheek/buccal mucosa, floor of mouth, lip, hard palate, alveolar ridge, maxilla • Age: mean age of 55 yrs • Sex: males are more frequently effected
  120. 120. • Clinical features: – Slow growing – Solitary – Circumscribed – Painless – Firm nodule
  121. 121. Clinical differential diagnosis • Buccal mucosa – Salivary gland tumors – Solitary fibrous tumor – Neurofibroma – Schwannoma – Intramuscular lipoma, – Myofibroma – Fibromatosis • Tongue – Irritation fibroma, – Granular cell tumor – Neurofibroma – Schwannoma
  122. 122. Well circumscription Mature adipocytes Cellular area Ropy collagen Histopathology:
  123. 123. Bland spindle cells with elongated nucleus Myxoid change Hyperchromatic multinucleated giant cells
  124. 124. CD 34 positivity
  125. 125. • Differential diagnosis • Nodular fascitis • Schwannoma • Neurofibroma • Solitary fibrous tmor • Hemangiopericytoma • Myxoid liposarcoma • Spindle cell liposarcoma • Treatment • Completely benign lesion • Exceptionally recur • Dedifferentiation and metastasis - no
  126. 126. Spindle cell liposarcoma
  127. 127. • Definition: Proliferation of mature adipocytes exhibiting marked variation in cell size with at least focal nuclear atypia • Variants - Enzinger and Weiss - developmental stage of the lipoblasts, cellularity, pleomorphism – Well-differentiated – Myxoid – Round-cell – Pleomorphic – Spindle cell – Dedifferentiated
  128. 128. • Demographics: • Age: 4 - 6th decade • Sex: Male • Site: Majorly effects cheeks • Other sites: floor of the mouth, palate, gingiva, mandible, tongue • Clinical features: • Painless • Slowly enlarging mass
  129. 129. Clinical differential diagnosis • Buccal mucosa – Salivary gland tumors – Solitary fibrous tumor – Neurofibroma – Schwannoma – Intramuscular lipoma, – Myofibroma – Fibromatosis • Tongue – Irritation fibroma, – Granular cell tumor – Neurofibroma – Schwannoma
  130. 130. Prominent spindle cells Resembling SCL Scattered lipoblasts In various shapes and sizes Histopathology:
  131. 131. Variable spindle cells devoid of lipoblasts Nuclear Atypia
  132. 132. • IHC Markers: S-100 positivity in lipogenic areas; nuclear expression of MDM2 and CDK4 • Differential diagnosis – Neural – Fibroblastic – Myofibroblastic • Treatment • Wide surgical excision is the treatment of choice • Radiation therapy remains controversial
  133. 133. Smoothmuscle tumors
  134. 134. • Benign tumors • Pilar • Genital • Vascular/Angiomyoma • Leiomyoma of deep soft tissue • Retroperitonial • Malignant tumors • Cutaneous leiomyosarcoma • Leiomyosarcoma of vascular origin • leiomyosarcoma of deep soft tissue
  135. 135. Leiomyoma
  136. 136. • Definition: Benign mesenchymal tumor with smooth muscle differentiation that is classified according to location as pilar, genital, vascular, deep soft tissue and retroperitonial. • In H&N regions smooth muscle tumors believed to arise from the tunica media of the blood vessels • Demographics: • Age: middle age to older age • Sex: females • Site: Very rare, if present - hard palate, tongue, buccal mucosa
  137. 137. Clinical features:
  138. 138. • Clinical differential diagnosis – Fibroma – Neurofibroma – Lipoma – Leiomyosarcoma – Mucocele – Pleomorphic adenoma – Lymphangioma – Hemangioma – Pyogenic granuloma
  139. 139. Monomorphic spindle cells with cigar shaped nuclei Stori form pattern Masson trichrome Histopathology:
  140. 140. VASCULAR LEIOMYOMA Actin immunostain Fascicles of spindle cells
  141. 141. Spindle cells are SMA positive CD31 reveals many vessels Other markers Vimentin, Desmin, h-caldesmon
  142. 142. • Histological Differential diagnosis – Fibromatosis – Myofibroma – Neurofibroma – Schwannoma – Special stains – collagen – IHC – Vimentin, desmin, α-smooth muscle actin (SMA) and muscle specific actin (MSA) • Treatment • Surgical excision • No recurrence
  143. 143. Leiomyosarcoma
  144. 144. • Definition: Malignant mesenchymal tumor with smooth muscle differentiation that can be categorized as cutaneous, vascular, or arising in deep soft tissue or the retroperitoneum • Demographics: • Age: Middle-aged or older individual • Sex: Females • Site: any of the above mentioned sites
  145. 145. • Clinical features: • Typically presents as a painless, lobulated, fixed mass of the submucosal • Secondary ulceration of the mucosal surface
  146. 146. • Clinical differential diagnosis – Fibroma – Neurofibroma – Lipoma – Leiomyosarcoma – Mucocele – Pleomorphic adenoma – Lymphangioma – Hemangioma – Pyogenic granuloma
  147. 147. Fascicles of interlacing monomorphic spindle-shaped cells with abundant eosinophilic cytoplasm Moderately large, blunt-ended nuclei, often with mild atypia Histopathology: Leiomyosarcoma arising from a blood vessel.
  148. 148. • One study on leiomyosarcoma of head and neck found that – LMS are well circumscribed – Focal areas of infiltration – Mitotic activity – 1-50 MF per 10 high power fields Montgomery E, Goldblum JR, Fisher C. Leiomyosarcoma of the head and neck: a clinicopathological study. Histopathology 2002; 40: 518–25. • IHC Markers: • Positive for MSA, SMA, Desmin, h-caldesmon • Negative for cytokeratin, CD34 • Few cases may also expresses cytokeratin, CD34
  149. 149. Differential diagnosis:
  150. 150. • Treatment • Early surgical excision with radical neck dissection for lymph node metastasis • Chemotherapy and radiotherapy • Recurrence and metastasis is about 50%
  151. 151. Referrences • Brad . W . Neville , Douglas D . Damm , Carl M . Allen Oral and maxillofacial pathology 2nd edition 2004 • SHAFER; Text book of oral pathology 5th edition 2006 • Enzinger and weiss; Soft tissue tumors fifth edition • Douglas .R. Gnepp; Diagnostic surgical pathology of head and neck; second edition • Andrew L.Folpe, Carrie Y. Inwards; Bone and soft tissue pathology foundation in surgical pathology. • Sook-Bin Woo; Oral pathology A comprehensive atlas and text

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