1) The document discusses several types of tumors that can occur in nerves and muscles in the oral cavity, including leiomyomas, leiomyosarcomas, rhabdomyomas, rhabdomyosarcomas, and paragangliomas.
2) Leiomyomas are benign smooth muscle tumors that commonly occur in the oral cavity on the tongue, hard palate, or buccal mucosa. Leiomyosarcomas are rare malignant counterparts.
3) Rhabdomyomas and rhabdomyosarcomas are rare as well, with rhabdomyosarcomas being malignant skeletal muscle tumors that commonly occur in children
1. TUMOR OF NERVES AND MUSCLES
DR. SUHASINI GP
SENIOR LECTURER
DEPT. ORAL &MAXILLOFACIAL PATHOLOGY AND MICROBIOLOGY
SUBHARTI DENTAL COLLEGE AND HOSPITAL
SWAMI VIVEKANAND SUBHARTI UNIVERSITY
MEERUT. UP
Subject: Oral Pathology
3. Leiomyoma
• Smooth muscle- benign neoplasms
• Relatively common and may arise anywhere in the body
• Leiomyomas - muscularis layer of the gut and in the body
of the uterus, skin (origin from vascular smooth muscle)
• Solid
• Vascular
• Epitheloid
4. • Rare in oral cavity- blood vessel wall & circumvallate
papillae (solid/ vascular), sal gland ducts
• Oral leiomyomas – slow growing, asymptomatic
submucosal masses
• Usually in the tongue, hard palate, or buccal
mucosa, gingiva, lips, salivary glands
• May be seen at any age and are usually discovered
when they are 1 to 2 mm in diameter
• Normal to red color
5.
6. Histopatology
• Composed of spindle shaped smooth muscle cells
with elongated nuclei that resemble fibroblasts
• Leiomyoma cells lack distinct cell margins
• Cytoplasm of one cell appears to be fused to
adjacent cells
• Elongated nuclei - cigar shaped
• Ischemic areas show fibrosis
23. Clinical features:
Adult Type
• The most common.
• Older men
• Asymptomatic polypoid or round mass
• Occurrence in tongue- often causes protrusion and
at times tingling sensation.
• Pharynx or larynx - obstructive symptoms.
• Also found in the FOM, soft palate, & the neck
muscles, but not in the muscles of the extremities.
24.
25. H/P
• well circumscribed large lobules of large poly
gonal cells, with abundant granular
eosinophilic cytoplasm
• Spider web appearance of cytoplasm bcoz of
peripheral vacuolization
• Focal areas with striations
• Positive for myoglobin, desmin, muscle
specific actin
26.
27.
28. Fetal rhabdomyomas
• Occur in young children
• Male predilection
• Most common locations are face and
periauricular region.
29. Histologic features
• Less mature appearance
• Haphazard arrangement of muscle cells with in a
myxoid stroma
• Some tumors show - cellularity and mild
pleomorphism
Treatment and prognosis
Local surgical excision
30.
31. Rhabdomyosarcoma
• Malignant tumor of striated muscle
• Derived from primitive mesenchyme that retained
capacity for skeletal muscle differentiation
• 60% soft tissue sarcoma- children
• Four forms of rhabdomyosarcoma are:
– pleomorphic
– alveolar
– embryonal
– botryoid
32. Embryonal rhabdomyosarcoma
• Marked predilection for head and neck area -60-70 %
• The genitourinary region -2nd common site
• Arises chiefly from the orbital, facial, nasal and cervical
musculature
• Intraoral- soft palate, tongue , PNS- break through into
oral cavity
• 16 months to 16 years, with no gender predilection.
33. • Chief presenting complaint - swelling, but
pain may be present if there is nerve
involvement
• Divergence of an eye, abnormal phonation,
dysphagia, cough, aural discharge or
deviation of the jaw
34.
35.
36.
37. Botryoid rhabdomyosarcoma
(sarcoma botryoides)
• Malignant tumor of the genitourinary tract in young
children
• Variant of embryonal rhabdomyosarcoma -------
maxillary sinus, nasopharynx, common bile duct and
middle ear
• 10 %of all rhabdomyosarcoma cases
• Exophytic polypoid growth
38. Alveolar rhabdomyosarcoma
• 20 % all rhabdomyosarcomas
• Generally between 10 and 20 years of age
• Occur in the extremities- Approx 18 % were
found in the head and neck region.
41. EMBYONAL
• Eosinophilic spindle cells, usually arranged in interlacing
fascicles
• Round eosinophilic cells, large & intermediate in size,
with a small nucleus and a granular eosinophilic
cytoplasm, interspersed among other cell types.
• Broad elongated eosinophilic cells, occasionally with
cross-striations.
• Small round and spindle cells with dark staining nuclei
and little cytoplasm
• Well-differentiated tumors - elongated, strap-shaped
or tadpole-shaped rhabdomyoblasts
42.
43. Botryoid Rhabdomyosarcoma
Polypoid, grape-like tumor masses
Scattered malignant cells in myxoid stroma
Cambium layer
Peripheral zone of
increased cellularity
beneath the mucosa
44. Pleomorphic
• Composed chiefly of spindle cells in a haphazard arrangement
• Cells - large and show considerable variation in appearance
• Nuclei are ovoid or elongated with packed chromatin
• Large bizarre cells, the nuclei situated often in an expanded
end of the cell
• 'racquet' cell , 'Strap' and 'ribbon' cells typically show
processes of long streaming cytoplasm.
• Positive immunostains for desmin and myoglobin
45.
46. Alveolar rhabdomyosarcoma
• Poorly differentiated round
and oval cells aggregated into
irregular clusters or nests
separated by fibrous septa
• Degenerated cells in the center
of the clusters show lack of
cohesiveness, while the
peripheral cells adhere in a
single layer to the septal walls
• Multinucleated giant cells
• 'Cambium layer'.
47. Trichrome stain
• Rhabdomyoblasts - bright red
• Myofilaments and cross-striations are stained by
PTAH - deep purple color
• Myxoid stroma may be positive for hyaluronidase
with acid mucopolysaccharide staining
• Desmin, myoglobin, MSA
• Rx- sugical excision+chemo, radiotherapy
48. Granular cell tumor
(GC myoblstoma, GC schwannoma)
• Not clear whether or not this tumor is a true
neoplasm, a developmental anomaly or a
trauma induced proliferation
• Origin- muscle, neural (schwann cells or
neuroendocrine), histiocytes, fibroblasts and
pericytes
• 30-60 yr
• Females
54. H/P
• Similar to GCT
• No psedoepitheliomatous hyperplasia
• Sheets
• More vascular
• S100 and other markers -ve
• Treatment- surgical excision
55. Alveolar soft part sarcoma
(Malignant granular cell myoblastoma)
• Uncertain origin-neural, striated muscle,
variant of malignant nonchromaffin
paraganglioma.
• Myogenic by immunophenotyping
• Young female
• Extremities
• One in four lesions occur in H &N
• Oral cavity, pharynx, orbit
56. • Pseudoalveolar pattern
• Large polygonal cells, with granular
eosinophilic cytoplasm
• Vesicular nuclei
• Vascular invasion
• PAS positive
• Treatment-
radical excision,
poor prognosis
57. Paraganglioma/ Carotid body tumor
• Paraganglia- specialized tissue of NC origin-
associated with AN & ganglia throughout the body
• Chemoreceptors- eg- carotid body
• CB paraganglioma, jugulotympanic paraganglioma
(glomus tumors)
58. Clinical features
• Rare- slow growing, painless mass
• Middle age , females
• Carotid body –most common
– Bifurcation of internal & external CA- upper lateral
neck below the angle of jaw
– High altitude- low O2- hyperplasia
• Head n neck- JT paraganglioma, vagal,
nasopharyngeal, laryngeal, orbital paraganglioma
• Multifocal- X linked
59. Histopathology
• Round, polygonal epitheloid cells- organized
into nests or zellballen (balls of cells)
• Nests consists of chief cells- centrally located
vesicular nuclei & granular eosinophilic
cytoplasm
• Vascular
• Thin fibrous capsule
• Rx- surgery, radiotherapy
