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Melanotic Neuroectodermal
tumor of infancy.
Neuroectodermal tumor of infancy is a rare
benign, pigmented neoplasm of the jawbone,
which is derived from the primitive neural
crest cells.
Definition
Clinical features:-
Age: The lesion occurs mainly in
infants before the age of 6 months
Sex: Both sexes are equally affected.
(Slightly more common in males)
Site: anterior part of maxilla,
however mandible is also sometimes
affected
Neuroectodermal tumor of infancy clinically presents a fast enlarging
swelling of the jawbone with expansion and distortion of the cortical
plates.
The swelling often cases elevation of the lip and facial asymmetry.
Destruction of the underlying bone often causes displacement of
developing teeth in the jaw.
Pain and tenderness is usually not present.
The surface of the lesion may exhibit a brown or black pigmentation.
Rate of growth of the lesion often varies, few lesions grow quite rapidly
while others may be slow growing.
RADIOGRAPHIC FEATURES
Often exhibits a well-defined
radiolunceny in the jaw that often
resembles a cyst.
often causes displacement of the
developing tooth buds.
radiographically exhibit a typical
‘sunray’ appearance.
HISTOPATHOLOGY
composed of two types of cells— the pigmented
cells and the nonpigmented cells
The neoplastic cells often proliferate in the
patterns of nests or tubules or alveolar
structures, etc.
The pigmented cells are large with an open nucleus and a lightly staining
cytoplasm, which occasionally contains coarse melanin granules.
These cells are flattened or cubical in shape with large, pale nuclei and are
often arranged in large masses.
The nonpigmented cells are small with dark, dense
nuclei and a scanty cytoplasm and they often resemble
lymphocytes.
These unpigmented cells are arranged in clusters
within the connective tissue stroma and sometime
these cells are surrounded by the pigmented cells.
Mitotic activity is rare in the tumor cells.
DIFFERENTIAL DIAGNOSIS
Desmoplastic small round cell tumor
Rhabdomyosarcoma
Lymphoma
Ewing sarcoma
Olfactory neuroblastoma
DR LEO
TREATMENT
Surgical excision with thorough curettage.
THANK YOU

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Melanotic Neuroectodermal tumor of infancy.

  • 2. Neuroectodermal tumor of infancy is a rare benign, pigmented neoplasm of the jawbone, which is derived from the primitive neural crest cells. Definition
  • 3. Clinical features:- Age: The lesion occurs mainly in infants before the age of 6 months Sex: Both sexes are equally affected. (Slightly more common in males) Site: anterior part of maxilla, however mandible is also sometimes affected
  • 4. Neuroectodermal tumor of infancy clinically presents a fast enlarging swelling of the jawbone with expansion and distortion of the cortical plates. The swelling often cases elevation of the lip and facial asymmetry. Destruction of the underlying bone often causes displacement of developing teeth in the jaw. Pain and tenderness is usually not present. The surface of the lesion may exhibit a brown or black pigmentation. Rate of growth of the lesion often varies, few lesions grow quite rapidly while others may be slow growing.
  • 5. RADIOGRAPHIC FEATURES Often exhibits a well-defined radiolunceny in the jaw that often resembles a cyst. often causes displacement of the developing tooth buds. radiographically exhibit a typical ‘sunray’ appearance.
  • 6. HISTOPATHOLOGY composed of two types of cells— the pigmented cells and the nonpigmented cells The neoplastic cells often proliferate in the patterns of nests or tubules or alveolar structures, etc. The pigmented cells are large with an open nucleus and a lightly staining cytoplasm, which occasionally contains coarse melanin granules. These cells are flattened or cubical in shape with large, pale nuclei and are often arranged in large masses.
  • 7. The nonpigmented cells are small with dark, dense nuclei and a scanty cytoplasm and they often resemble lymphocytes. These unpigmented cells are arranged in clusters within the connective tissue stroma and sometime these cells are surrounded by the pigmented cells. Mitotic activity is rare in the tumor cells.
  • 8. DIFFERENTIAL DIAGNOSIS Desmoplastic small round cell tumor Rhabdomyosarcoma Lymphoma Ewing sarcoma Olfactory neuroblastoma DR LEO
  • 9. TREATMENT Surgical excision with thorough curettage.