1. Neurofibromas, neurilemmomas, and granular cell tumors are among the benign soft tissue tumors that can occur in the oral cavity. Neurofibromas arise from nerve sheaths and are rare in the mouth but can be a sign of neurofibromatosis or multiple endocrine neoplasia syndrome. 2. Rhabdomyosarcoma is the most common malignant soft tissue tumor in children, occurring most often in the head, neck, genitourinary tract, or extremities. It has several histologic subtypes with varying prognosis. 3. Kaposi's sarcoma, caused by human herpesvirus 8, has become more common in the oral cavity due

1. SOFT TISSUE(MESENCHYMAL) NEOPLASMS
KALIISA EDWARD
BDSIII
OCTOBER 2017
PRESENTED
BY

2. BENIGN NEOPLASMS
The common benign soft tissue swellings in the mouth are mostly hyperplastic as
mentioned in the previous presentation

3. NEUROFIBROMAS
 These uncommon tumours arise from nerve sheaths. They
form smooth, painless lumps but are particularly rare in the
mouth. When seen in the mouth, neurofibromatosis should be
suspected. Also, in multiple endocrine adenoma syndrome
type 2 (MEN2) neurofibromas along the lateral borders of the
tongue are atypical feature. Medullary carcinoma of the
thyroid in 95%, phaeochromocytoma in 50% and
hyperparathyroidism in 15% are associated. Since
phaeochromocytoma in particular is life threatening, screening
for MEN 2 should be carried out if these oral neurofibromas
are found.

4. Neurofibromas in oral cavity

5. Histologically
 Neurofibromas are cellular
with plump nuclei separated
by fine, sinuous collagen
fibres, among which mast
cells can usually be found.
Excision is curative

6. NEURILEMMOMAS
Neurilemmomas arise from axon
sheaths and, though
uncommon, are more frequently
found in the mouth than
neurofibromas. They also form
painless smooth swellings.
Histologically, the
appearance is distinctive
with multiple rounded
masses of elongated
spindle cells with
palisaded nuclei
 Excision is curative.

7. Neurilemmomas continue……
 Benign ,encapsulated tumor of the
nerve sheath. Their origin is thought
to be shwann cells derived from the
neural crest.
Histologically

8. Lipoma and fibrolipoma
 Lipomas can occasionally grow, particularly
from the buccal fat pad, as soft, sometimes
yellowish, swellings, which may be
pedunculated . They grow slowly and cause
no symptoms unless bitten, or become
conspicuous because of their size.
 Histologically, lipomas consist of globules of fat
supported by areolar tissue. Sometimes, fibrous
tissue forms a
 large part of the tumour (fibrolipoma). They
should be excised

9. Granular cell tumour
 Clinically, granular cell tumours typically form
painless smooth swellings. The tongue is the most
common site.
 Pathology. Large granular cells form the bulk of the
lesion but pseudoepitheliomatous hyperplasia of
the overlying epithelium may be conspicuous
Electron microscopy suggests that the granular cells
originate from Schwann cells. However, the
presence of all stages of apparent transition of
striped muscle cells into granular cells is a striking
histological feature Elsewhere, the granular cells
are large, with clearly defined cell membranes and
filled with eosinophilic granules
 The pseudoepitheliomatous
hyperplasia is such that
granular cell tumours have
been mistaken histologically
for carcinomas with resulting
overtreatment. Simple excision
should, however be curative.

10. Histologically
Granular cell tumour. The
irregular proliferation of the
epithelium
(pseudoepitheliomatous
hyperplasia) may mimic a
squamous
carcinoma in superficial
biopsy specimens.

11. Congenital (granular cell) epulis
 The rare congenital epulis is
typically present at birth as a
smooth but prominent soft
nodule, usually on the alveolar
ridge.
 Females are predominantly
affected and occasionally the
mass is so large as to obstruct
respiration.
 Excision is curative but
spontaneous regression is also
seen
 Histologically, large pale granular
cells with sharply-defined cell
membranes are covered by
epithelium which lacks
pseudoepitheliomatous
hyperplasia.
Immunohistochemistry suggests
that the origin is myogenous.

12. Congenital epulis
A firm pink non-ulcerated
nodule on the alveolar ridge
of a neonate is the typical
presentation

13. Haemangiomas
 Haemangiomas are mostly vascular malformations. Vascular
neoplasms (apart from Kaposi's sarcoma in patients with HIV
disease) are rare.
 Haemangiomas may be localized but are occasionally diffuse
and associated with similarly affected areas of the face. Rarely,
the meninges are also involved, causing epilepsy and mental
defect (Sturge-Weber syndrome). Isolated haemangiomas
form purple, flat or nodular lesions which blanch on pressure

14. Histologically
 Haemangiomas are either capillary, cavernous or mixed. The
capillary type consists of innumerable minute blood vessels and
vasoformative tissue — mere rosettes of endothelial cells The
cavernous type consists of
 large blood-filled sinusoids. Excision of mucosal haemangiomas
should be avoided unless trauma causes repeated episodes of
bleeding. If necessary, cryosurgery may allow removal of a
haemangioma without excessive bleeding

15. haemangioma
Cavernous haemangioma of the cheek. The
colour is deep purple and the structure, a
mass of thin-walled blood sinuses is visible
through the thin epithelium. A mass engorged
with blood and as prominent as this is liable
to be bitten and bleed profusely

16. Lymphangioma
 These uncommon tumours usually
form pale, translucent, smooth or
nodular elevations of the mucosa.
However, they may be noticed because
they suddenly swell and become dark
purple due to bleeding into the
lymphatic spaces. Rarely,
lymphangiomas are diffuse and
extensive, and cause generalized
enlargement of the tongue
(macroglossia) or lip.
 Histologically, lymphangiomas
consist of thin-walled vascular
spaces sometimes containing
pinkish amorphous material as a
result of fixation of lymph
Localized lymphangiomas can
be excised but this is more
difficult in the diffuse type where
the operation may have to be
done in stages

17. MALIGNANT CONNECTIVE-TISSUE TUMOURS
 Sarcomas of virtually any type can affect the oral soft tissue,
but most are rare. Kaposi's sarcoma has become the most
common type, but among HIV-negative persons,
rhabdomyosarcoma is the most common.
 Sarcomas tend to affect a considerably younger age group
than carcinomas, and rhabdomyosarcomas are the most
common oral sarcomas in children. Sarcomas grow rapidly,
are invasive, destroy surrounding tissues, and usually spread
by the bloodstream. Many sarcomas are clinically
indistinguishable from one another, but some, such as
Kaposi's sarcoma and malignant melanoma, are pigmented
and must be differentiated from benign pigmented lesions.

18. Rhabdomyosarcoma
 Rhabdomyosarcomas can affect children or
young adults and form rapidly growing soft
swellings.
 Histologically, several types are recognized.
The embryonal type, which more frequently
affects children, consists of cells of variable
shape and size. Some are strap or tadpole-
shaped, while muscle-like cells with cross
striations may be difficult to find. The alveolar
type consists of slit-like spaces into which hang
tear-shaped, darkly-staining cells attached to
the walls. These alveoli are separated by a
fibrous stroma.
Treatment is by excision and
combination chemotherapy
but the prognosis is poor.

19. Common sites of RMS include:
 Head and neck (such as near the eye, inside the nasal sinuses or throat, or near the
spine in the neck)
 Urinary and reproductive organs (bladder, prostate gland, or any of the female
organs)
 Arms and legs
 Trunk (chest and abdomen)

20. Embryonal rhabdomyosarcoma
 Embryonal rhabdomyosarcoma (ERMS) usually affects children in their first 5 years
of life, but it is the most common type of RMS at all ages.
 The cells of ERMS look like the developing muscle cells of a 6- to 8-week-old
embryo. ERMS tends to occur in the head and neck area, bladder, vagina, or in or
around the prostate and testicles.
 Two subtypes of ERMS, botryoid and spindle cell rhabdomyosarcomas, tend to
have a better prognosis (outlook) than the more common conventional form of
ERMS.

21. Alveolar rhabdomyosarcoma
 Alveolar rhabdomyosarcoma (ARMS) typically affects all age groups equally. It
makes up a larger portion of RMS in older children and teens than in younger
children (because ERMS is less common at older ages).
 ARMS most often occurs in large muscles of the trunk, arms, and legs. The cells
of ARMS look like the normal muscle cells seen in a 10-week-old fetus.
 ARMS tends to grow faster than ERMS and usually requires more intense
treatment.

22. Anaplastic rhabdomyosarcoma and
undifferentiated sarcoma
 Anaplastic rhabdomyosarcoma (formerly called pleomorphic
rhabdomyosarcoma) is an uncommon type that occurs in adults but is very rare
in children.
 Some doctors also group undifferentiated sarcomas with the
rhabdomyosarcomas. Using lab tests, doctors can tell that these cancers are
sarcomas, but the cells don’t have any features that help classify them further.
 Both of these uncommon cancers tend to grow quickly and usually require
intensive treatment.

23. Rhabdomyosarcoma in adults
 Most rhabdomyosarcomas develop in children, but they can also occur in adults.
Adults are more likely to have faster-growing types of RMS and to have them in
parts of the body that are harder to treat. Because of this, RMS in adults is often
harder to treat effectively.

24. Fibrosarcoma
Fibrosarcomas consist of broad
interlacing bands of fibroblasts
with a streaming or herring-
bone pattern. Some produce
abundant collagen, others are
highly cellular with close-packed
nuclei, among which there are
often mitoses.
 Treatment is by radical
excision. Local recurrence and
spread are common but
metastasis is rare.

25. Histology
 Fibrosarcoma of the
tongue. There are streams
of neoplastic fibroblasts,
but the striking feature is
the spindle-shaped, darkly
staining
 nuclei and their variation in
size and mitoses.

26. Kaposi's sarcoma
Ks is a malignant angiomatous tumor, first
described by Moritz Kaposi a Hungarian
dermatologist in 1872. Since the outbreak of
AIDS, Kaposi's sarcoma has become the most
common type of intraoral sarcoma. It mainly
affects men who have sex with men, who
have HIV infection.

27. Classification of Kaposi’s sarcoma
Classic (European KS)
More in men over 60 years of eastern European
decent.
Slow growing appear as multiple ,small, purple
dome shaped nodules or plaque on the sin,
especially on legs.
Viscera involvement bout 10% after many years.

28. Classic KS

29. African (endemic) KS
In equatorial Africa,
common in Uganda
that contributes 9%
of all malignant
tumors common in
boys and young
men.

30. Kaposi’s sarcoma in renal transplant
 The increased incidence of disease in transplant populations may, in part,
be attributed to the choice of immunosuppressive regimen, with
calcineurin inhibitor (CNI)-based immunosuppression being associated
with the development of the tumour. Studies have recently
demonstrated that conversion to proliferation signal inhibitors (PSIs)
along with the concomitant withdrawal of CNIs leads to a rapid
resolution of both cutaneous and visceral Kaposi's lesions.
 Histological examination of lesions from patients with KS supports data
from animal models which suggests that PSIs inhibit tumour
angiogenesis through impaired vascular endothelium growth factor
production, a key element in the development of the tumour.

31. AIDS associated( epidemic)KS
AIDS-associated KS can have a more aggressive
course with a more widespread distribution pattern,
including the oral cavity. With the advent of
antiretroviral therapy. AIDS-related KS has
diminished. KS is a “radio-responsive tumor,” which
means radiation therapy is effective in the treatment
of skin (cutaneous) lesions.

32. AIDS associated( epidemic)KS

33. pathogenesis
Pathogenesis of KS is complex ,
Its an opportunistic neoplasm in immunocompromised
patient which has excessive proliferation of spindle cells of
vascular origin having features of both endothelial and
smooth muscle cells.
Epidemiological studies have suggested viral association
implicating HIV and human herpes virus 8,HIV- 8 also called
Kaposi's sarcoma associated herpes virus (KSHV)

34. Cont…
Occurrence of KS includes interplay of HIV- 1 infection HHV- 8
infection, activation of immune system and cytokines IL-
6,TNF afa-1 ,GM-OMF, basic fibroblastic factor and oncostin
M)
Higher incidence of KS in homosexual is explained by
increased secretion of cytokines by their activated immune
system.
Defective immune regulation plays a role in the pathogenesis
is further substantiated by observation of secondary
malignancy( eg leukemias, lymphoma and myeloma is about
1/3 of patients.

35. Histology
Kaposi's sarcoma is a vascular tumour in which factor VIII
antigen (a marker for endothelial cells) can be identified
but is not the most sensitive marker. Immunoreactivity for
CD34 antigen is also positive in most spindle cells and in
cells lining vascular spaces or inconspicuous vascular slits
in small lesions, and in endothelium of surrounding CD34
reactivity appears to be the most reliable marker of
endothelial progenitor cells and is valuable in the
diagnosis of Kaposi's sarcoma.

36. Cont…
The early 'pre-sarcomatous' lesion consists of a mass of
capillary-size blood vessels, sometimes with mononuclear
cell cuffing. It resembles granulation tissue, particularly in
the mouth, where superficial lesions can be traumatized
and become secondarily inflamed. Later, there is
increasingly widespread angiomatous proliferation, and in
some areas the vessels may be slit-shaped when obliquely
sectioned. There is also proliferation of angular or spindle-
shaped interstitial cells

37. Conti…
Ultimately, the latter predominate and show increasing
numbers of mitoses. Central necrosis may develop and
extravasation of red cells can leave deposits of haemosiderin.
Associated HIV infection is usually suggested by other clinical
manifestations (such as opportunistic infections) and
lymphopenia in the blood picture.
Kaposi's sarcoma must be distinguished from AIDS associated
thrombocytopenic purpura and bacillary angiomatosis, which
may appear similar clinically. However, purpura may be seen
earlier and is distinguishable by haematological testing.

38. Cont….

39. Signs and symptoms
KS lesions are nodules or blotches that may be red,
purple, brown, or black, and are usually parpular (in
other words, palpable or raised).
They are typically found on the skin, but spread
elsewhere is common, especially the mouth,
gastrointestinal tract and respiratory tract. Growth
can range from very slow to explosively fast, and is
associated with significant mortality and morbidity.

40. Skin
Commonly affected areas include the lower limbs,
back, face, mouth, and genitalia. The lesions are
usually as described above, but may occasionally be
plaque-like (often on the soles of the feet) or even
involved in skin breakdown with resulting fungating
lesions. Associated swelling may be from either local
inflammation or lymphoedema (obstruction of local
lymphatic vessels by the lesion).

41. Mouth
The mouth is involved in about 30% of cases,
and is the initial site in 15% of AIDS-related
KS. In the mouth, the hard palate is most
frequently affected, followed by the gums.
Lesions in the mouth may be easily damaged
by chewing and bleed or suffer secondary
infection, and even interfere with eating or
speaking.

42. Gastrointestinal tract
Involvement can be common in those with
transplant-related or AIDS-related KS, and it may
occur in the absence of skin involvement. The
gastrointestinal lesions may be silent or cause weight
loss, pain, nausea/vomiting, diarrhea, bleeding (either
vomiting blood or passing it with bowel motions),
malabsorption, or intestinal obstruction.

43. Respiratory tract
Involvement of the airway can present with shortness
of breath, fever, cough, hemoptysis (coughing up
blood), or chest pain, or as an incidental finding on
chest x-ray. The diagnosis is usually confirmed by
bronchoscopy when the lesions are directly seen, and
often biopsied.

44. Transmission
 KSHV appears to be shed in saliva independent of the
subjects' immune status. Thus deep kissing has been
implicated in gay and bisexual men. viral DNA has been
detected in breast milk in African patients.
 HSHV8 infects dividing B cells CD45 phase.
 KSHV is also transmissible via organ transplantation and
blood transfusion. Testing for the virus before these
procedures is likely to effectively limit iatrogenic transmission.

45. virology
KSHV is a lymph tropic and closely related to
EBV and Herpes virus saimiri.
KSHV genome (165-kbp) contain numerous
genes related to cell regulatory genes involved
in cell apoptosis and host response
contributing to pathogenesis.

46. Clinical course
Quiet variable
Classic form is largely confined to the skin and the
course is generally slow and insidious with long
survival.
African (endemic and epidemic (AIDS associated) is
rapidly progressive ,often wide spread cutaneous as
well as visceral involvement and high mortality.

47. management
Various measures have been used to deal with Kaposi's sarcoma but in HIV
disease highly active antiretroviral therapy (HAART) will bring resolution.
In KS associated with immunodeficiency or immunosuppression, treating the
cause of the immune system dysfunction can slow or stop the progression of
KS. In 40% or more of peoples with AIDS-associated Kaposi sarcoma, the
Kaposi lesions will shrink upon first starting highly active antiretroviral
therapy (HAART). However, in a certain percentage of such people, Kaposi
sarcoma may again grow after a number of years on HAART, especially if HIV
is not completely suppressed.

48. Other sarcomas of oral soft tissues
Neurofibrosarcomas, liposarcomas,
leiomyosarcomas and even soft-tissue
osteosarcomas and chondrosarcomas may be
seen, but all are rare.

49. LYMPHOMAS
Lymphomas can arise from any type of lymphocyte,
but more frequently from B cells. They are all
malignant. They comprise Hodgkin's disease and the
more common non-Hodgkin lymphoma. Lymphomas
relatively frequently involve the cervical lymph nodes
but are rare in the mouth. However, in AIDS,
lymphomas may account for 2% of oral neoplasms