• Extremely rare tumors
• Predilection for girls
• multifocal location
• Usually familial disease but sporadic cases have been
• lack oncogenic mutations in KIT/PDGFRA genes
(present in adulthood).
all pediatric GISTs show
consistent biochemical activation
immunehistochemistry of the
KIT receptor tyrosine kinase.
Morphologically the tumors have
epithelioid or mixed epithelioidspindle cell appearance.
In contrast with adult GIST, pediatric
tumors tend to have a multinodular
growth, with partial excisions being
often followed by local recurrence at
Another important distinction
from the adult counterpart is their
indolent clinical behavior, even
in the presence of loco-regional
or distant metastatic disease.
•Imitinib is effective in GIST but
surgery is preffered.
•broad-based, kinase inhibitors,
such as sunitinib, has been
questioned as the first-line
therapy in metastatic pediatric
imatinib-alternative drugs, such as
nilotinib, sorafenib and dasatinib
are being tested for their efficacy
in an in vitro murine Ba/F3 cell
system expressing the wild-type
There is some role
of radiotherapy in
treatment of GIST.
Infantile myofibromatosis is a
mesenchymal tumor that can arise in the
skin, muscle, bone, subcutaneous tissue,
or viscera. It is the most common
fibrous tumor of infancy.
Myofibromatosis presents with either
solitary or generalized lesions, with or
without visceral involvement. Most
lesions spontaneously regress; however,
extensive intestinal myofibromatosis is
associated with significant morbidity and
Various chemotherapeutic interventions have
demonstrated limited efficacy, significant
treatment toxicity, and long-term morbidity.
However, the combination of low-dose
chemotherapy and long-term total parental
myofibromatosis can provide symptomatic relief
and inhibit disease progression.
Carcinoid tumors originate from neuroendocrine
cells within the GI tract. These neoplasms derive
from GI epithelial and subepithelial endocrine
progenitor cells that function as part of the amine
precursor uptake and decarboxylation (APUD)
Pediatric carcinoid tumors typically
occur in the GI tract—stomach, small
intestine, appendix (most common),
and rectum. Carcinoid tumors of the
appendix occur with an estimated
incidence of 1 case per million children
per year, with a slight female
Adenocarcinoma of the colon and rectum is the most
common cancer of the GI tract. The lifetime risk of
developing colorectal cancer in the general population
is 1 in 19. However, colorectal cancer in children is rare,
with an estimated incidence of 0.3 to 1.5 cases per
million. Although reported as early as 9 months of age,
the median age at diagnosis for pediatric cases is 15 to
19 months. Pediatric colorectal cancer accounts for 2%
of malignancies in adolescents.
• Inflammatory myofibroblastic tumour, plasma cell
granuloma, and inflammatory fibrosarcoma.
• Inflammatory myofibroblastic tumours are
characterized by a mix of inflammatory cells, e.g.
plasma cells, lymphocytes and eosinophils, and
spindle cells without nuclear atypia. These tumours
may have necrosis, hemorrhage, focal calcification and
Leiomyoma (plural leiomyomas or
leiomyomata) (leio- + myo- + -oma,
"smooth-muscle tumor") is a benign smooth
muscle neoplasm that is very rarely (0.1%)
premalignant. They can occur in any organ,
but the most common forms occur in the
small bowel and the esophagus.
Gastrointestinal tract is the most common extranodal
site involved by lymphoma with the majority being
non-Hodgkin type. Although lymphoma can involve
any part of the gastrointestinal tract, the most frequent
sites in order of its occurrence are the stomach
followed by small intestine and ileocecal region.
Gastrointestinal tract lymphoma is usually
secondary to the widespread nodal diseases
and primary gastrointestinal tract lymphoma
is relatively rare. Gastrointestinal lymphomas
are usually not clinically specific and
indistinguishable from other benign and
Diffuse large B-cell lymphoma is the
most common pathological type of
gastrointestinal lymphoma in essentially
all sites of the gastrointestinal tract,
although recently the frequency of other
forms has also increased in certain
regions of the world.