2. Seminar on
Spindle cell tumors of the oral cavity
Part - II
By: Dr. Madhusudhanreddy
III year PG
VDC
3. • Part I seminar
I. Tumors of fibrous origin
II. Tumors of Fibro histiocytic origin
III.Tumors of adipose tissue origin
IV.Tumors of Smooth muscle origin
7. • Definition: Benign mesenchymal tumor with skeletal
muscle differentiation divided into the adult, fetal, and
genital types, according to degree of differentiation and
location
• Named by Zenker in 1864
• Rare benign tumor that exhibits mature skeletal muscle
differentiation.
8. • Demographics:
• Age: 4 years (age range - 3 days to 58 years)
• Sex: M:F ratio is 2.4:1
• Site: Histologic types
– Myxoid type – preauricular and post auricular
– Intermediate type – head and neck
9. • Clinical features:
• Kapadia et al
– 42% - younger than 1 year old
– 25% - congenital
– 50% - older than 15 years of age
• Symptoms: Solitary mass involving soft tissue or mucosa
• Some times fetal rhabdomyoma may be associated with
nevoid basal cell carcinoma syndrome.
• Adult Rhabdomyoma
• Fetal Rhabdomyoma
Douglas R.Gnepp; diagnostic surgical pathology of head and neck; second edition
11. Histopathology:
Varying size and shape of muscle cells
with bipolar nucleus and eosinophillic
cytoplasm in myxoid background
Mixture of undifferentiated and
Differentiated skeletal muscle cells
15. • Initially described by Weber in 1854.
• First published was probably a tongue lesion
• Accounts for 6% of all malignancies in children under 15
years of age
• RMS of the oral cavity accounts for 10 –12% of all the head
and neck RMS cases
• Definition: A primitive soft tissue sarcoma, showing a
variable degree of embryonic skeletal muscle differentiation
Ankita Tandon et al; Oral Rhabdomyosarcoma: A review; J Clin Exp Dent. 2012;4(5):e302-8.
16. • Demographics:
• Age: First and second decades
• Sex: Males have slight high predelection
• Site: Head and neck > Orbit (35%) > trunk and extremities
> intra-abdominal organs and genitourinary tract (23%)
• Oral cavity – Tongue > soft palate > hard palate > baccal
mucosa
• Signs and symptoms: pain, parasthesia, loss of teeth and
trismus
Ankita Tandon et al; Oral Rhabdomyosarcoma: A review; J Clin Exp Dent. 2012;4(5):e302-8.
17. Clinical features:
Smooth or lobulated surface, sometimes botryoid or grape cluster–like in
appearance and become fixed to surrounding tissues
25. • Definition:
• Vascular tumor of intermediate malignancy
• Weiss and Enzinger described in 1986
• It was considered to be an intermediate or low-grade
malignancy, with a biologic behavior between a hemangioma
and an angiosarcoma
• Relatively uncommon lesion
26. • Fletcher et al, Imayama et al - nonneoplastic reactive
vascular proliferation
• Later Perkins and Weiss
– “spindle cell hemangioma” (SCH) - solitary lesions
– “spindle cell hemangiomatosis” - multifocal lesions
K.I. Tosios et al; Spindle cell hemangioma (hemangioendothelioma) of the head and neck: case report
of an unusual (or underdiagnosed) tumor; Oral Surg Oral Med Oral Pathol Oral Radiol Endod
2008;105:216-21
27. • Demographics:
• Age: Young Adults
• Sex: no sex predelection
• Site: dermis and subcutis of distal extremities, axilla,
trunk, vulva, penis, spleen, pancreas, bones, head and
neck
28. • Clinical features:
• Solitary tumor or multiple asymptomatic nodules
clustered
• Color - Normal or bluish
• Consistency - firm
• Size - few millimeters to a few centimeters - less than
2.0 cm.
35. • Definition: Fully malignant sarcoma showing endothelial
differentiation.
• Rarely affecting the oral cavity
• Demographics:
• Age: 1 year to 20 years with an average of 7.3 years
• Sex: Equal sex predilection
• Site: Tongue > Parotid gland > Lip > Submandibular gland
> Palate
36. • Clinical features:
• Poorly demarcated
• Round or ovoid nodules
• Red-blue to purplish
• Painful
• Spontaneously bleeding
• Firm on palpation
41. • Histological differential diagnosis
• Haemangioma and its variants
• Pyogenic granuloma
• Spindle cell carcinoma
• Mucosal malignant melanoma
• Rhabdomyosarcoma
• Leiomyosarcoma
• Liposarcoma
• Synovial sarcoma
• Fibro-histiocytic sarcomas, MPNST and epithelioid
sarcoma
• Malignant myoepithelioma of salivary gland
• Anaplastic non-Hodgkin’s lymphoma with spindle cell
features
G. Favia et al; Angiosarcoma of the head and neck with intra-oral presentation. A clinico-pathological
study of four cases; Oral Oncology 38 (2002) 757–762
42. • IHC markers:
• Vimentin
• CD31
• CD34
• FLI-1 protein
• D2-40 and VEGFR-3
• Low-molecular-weight cytokeratin expression in 25%
of cases, particularly epithelioid tumors
G. Favia et al; Angiosarcoma of the head and neck with intra-oral presentation. A clinico-pathological
study of four cases; Oral Oncology 38 (2002) 757–762
45. • Treatment
• Radiotherapy of 4000–5000 rads,
• Followed by radical surgery - most effective procedure
• Prognosis
• Survival - longer in low grade angiosarcoma than high grade
angiosarcoma
• 50% - die within 15 months from the diagnosis
• 12% - survive 5 years or longer
• Death is usually due to uncontrolled local progression and/or
distant metastases to the lungs, liver and bone
G. Favia et al; Angiosarcoma of the head and neck with intra-oral presentation. A clinico-pathological
study of four cases; Oral Oncology 38 (2002) 757–762
47. • Definition: Malignant vascular tumor caused by infection
with HHV-8, most often in immunocompromised patients
• Kaposi sarcoma (KS) - multifocal angioproliferative
disorder of vascular endothelium, primarily affecting
mucocutaneous tissues with the potential to involve viscera
48. Mahnaz Fatahzadeh; Kaposi sarcoma: review and medical management update; Oral Surg Oral Med
Oral Pathol Oral Radiol 2012;113:2-16
• Demographics:
Clinical
variants of KS
Etiology Age sex site
Classic KS - 5th decade older men
M:F, 17:1
Skin and extrimities
Endemic KS - children and
young
male
individuals
lymph node and soft
tissue
Iatrogenic KS - - - kidney transplant
patients
Epidemic KS Homosexual male
intravenous drug
Abusers
Adults Males skin,
mucosal
and visceral locations
63. • Definition: Disorganized proliferation of nerve in response
to injury or disruption.
• Occurs in the area of any nerve that has been surgically or
auto-amputated
• Demographics:
• Age: Middle-aged adults
• Sex: Slightly more common in females than in males
• Site: Mental foramen area
lateral tongue
lower lip
64. • Clinical features
• Patient has firm, painful nodule
• Constant or intermittent aching, a burning sensation,
or severe radiating pain
• Clinical differential diagnosis
• Traumatic fibroma
• Mucocele
• Solitary neurofibroma
70. • Definition:
• Neurofibromas are the most frequent benign neoplasms
originating from the peripheral nerve sheath and occur as
solitary or multiple tumours when associated to the von
Recklinghausen disease, better known as neurofibromatosis
71. • Demographics:
• Age: Most patients are younger than 45 years of age
• Sex: Females are more frequently affected,
• F:M ratio being 2:1.
• Site:
• Buccal mucosa, palate, alveolar ridge, vestibule and
tongue, lips and gingival
• Intraosseous lesions are quite rare.
• Most common site being the posterior mandible
72. • Clinical features
• ORAL NEUROFIBROMA - uncommon benign tumor
of the oral cavity
• Neurofibroma occurs as a
– Solitary lesion – unknown cause
– As part of the generalized syndrome of
neurofibromatosis (von Recklinghausen disease of
the skin) - inherited as an autosomal dominant trait
• 50% of cases are reported to be the result of
spontaneous mutation in
– Neurofibromatosis type 1 (NF1) gene
– Neurofibromatosis type 2 (NF2) gene
73. • Solitary oral lesions - non-ulcerated nodules, which
tend to be of same color of normal mucosa.
• Lesions associated with neurofibromatosis – solitary
lesions, iris Lisch nodules, cafe-au-lait spots and
freckling in the axillary region.
75. • Central neurofibromas
– Early stages – Asymptomatic
– Grow in size - Cause expansion of the cortical plates
of the mandible, with or without destruction,
– Pain and anesthesia or paresthesia of the lower lip
• Radiographically - Well-circumscribed or poorly
demarcated radiolucent lesion
81. • Once termed “neurilemoma,” - Stout, 1935
• Benign tumor, apparently derived from the Schwann cells
• Definition: A peripheral nerve sheath tumor arising
eccentric to nerve
• Demographics:
• Age: 20 to 50 years common in 2nd and 3rd decades
• Sex: Female Sex Predilection
• Site: Tongue > Palate > Buccal mucosa
• Some times intraosseous – mandible
• Bone expansion, pain, parasthesia
94. • Definition: A malignant neoplasm with a poor
prognosis of perineural fibroblasts or schwann cells,
with propensity to rapidly extend along the associated
nerve trunk.
• WHO has grouped this lesion under the heading of
MPNSTs
– Mlignant schwannoma
– Malignant neurilemmoma
– Neurogenic sarcoma
– Neurofibrosarcoma
95. • Demographics
• Age: 20 – 50 yrs of age
• Sex: no sex predelection
• Site: any where in the body but most commonly in skin
• Clinical features
• Exact mechanism of occurance of the lesion is not yet
identified
• Occurs as a complication of neurofibromatosis I (15%)
• Or occurs denovo
96. • Mostly occurs in soft tissue
• Intraosseous neurofibrosarcoma
– Due to the nerve present in the neutrient canal
– Due invasion into the bone from soft tissue
– Symptoms include pain, parasthesia
Neurofibromatosis-I patient with
neurofibrosarcoma of the left cheek
100. • Treatment
• Highly aggressive tumor and difficult to treat
• Prognosis - Grade of tumor, tumor size
• Overall survival rate is 40-75%
• Early detection and appropriate treatment is crucial
for survival
101. 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
• Net source and articles