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OJHAS Vol. 11, Issue 1:
(Jan-Mar 2012) |
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GIST- A Rare Tumor in Paediatric
Age Group |
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Kulwant Singh Ded, Professor
and Head, Dept. of Surgery,
Mandeep Singh Khurana, Assistant
Professor, Dept. of Paediatrics,
Gursharn Singh Narang, Professor, Dept. of Paediatrics
Arun Kumar Gupta, Assistant
Professor, Dept. of Surgery,
Loveleen Kaur, Junior Resident, Dept. of Paediatrics,
Sri Guru Ramdas Institute of
Medical Sciences and Research, Vallah, Amritsar. |
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Address for Correspondence |
Professor,
Dept. of Paediatrics
Sri Guru Ramdas Institute of
Medical Sciences and Research,
Vallah, Amritsar.
E-mail:
gsnarang321@gmail.com |
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Ded
KS, Khurana MS, Narang GS, Gupta AK, Kaur L. GIST- A Rare Tumor in Paediatric
Age Group. Online J Health Allied Scs.
2012;11(1):15 |
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Submitted: Feb 23,
2012;
Accepted: Mar 22, 2012; Published: Apr 15, 2012 |
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| Abstract: |
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A 13 years old male child
presented to us with complaints of pain abdomen, radiating towards back.
Child underwent CT-abdomen with contrast which showed volvulus and child
was operated immediately and resected part of gut was sent for histopathology
examination. Histopathology report showed features suggestive of gastrointestinal
stromal tumour and tumour marker CD-117 was sent for confirmation and
report was found to be positive.
Key Words:
GIST; Mesenchymal tumor.
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A 13 years old child presented to us with complaints
of pain abdomen since last 4 days which was insidious in onset progressive
in nature. Pain abdomen was severe, generalised and radiating towards
back. There was no history of any aggravating or relieving factors.
History of vomiting was present, around 3-4 episodes, which was bilious
in nature, non projectile. There was history of non passage of stools
since last 5 days. History of associated loss of appetite and loss of
weight was present. Child was previously admitted twice with similar
complaints, one and half years before and again 6 months before the present
admission
and child was managed conservatively with IV medications.
On examination, child was undernourished weighing
30kg. The child had tachycardia and the blood pressure was within normal
limits. On abdominal examination, guarding and rigidity was present and
severe tenderness was present throughout abdomen. There was a vague mass
palpable around umbilical area with irregular border. Child had dark
green aspirate through nasogastric tube. X ray abdomen erect was
done which showed features of obstruction. Ultrasonography abdomen
showed intraperitoneal mass with mesenteric lymphadenopathy and aperistaltic
gut loops with thickened walls. Contrast CT abdomen showed features suggestive of malrotation with midgut volvulus with
occlusion of distal superior mesenteric artery and superior mesenteric
vein with sub mucosal edema of gut loops with massive ascites. Child
was taken up for surgery immediately. Intra-operatively a
mass was found in the mesentery of jejunum, about 8x8 cm in size. The mass was 1.5ft from duodeno-jejunal
junction with malrotation of gut with gangrenous portion of jejunum
and enlarged lymph node in mesentery. Excision
of Ladd's band, with resection of about 15cm of jejunum and jejno-jejunal
anastomosis along with excision of mass was done. Appendicectomy was also done.
Post operatively, Ryle's tube aspirate was persistently green coloured and pelvic drain
had bloody drainage for around 5 days. On 6th post operative
day child had massive dark green aspirate and greenish drainage from
pelvic drain site. Observing the dark green drainage, child was taken
up for surgery again at 8th post operative day. Multiple perforations,
about 19 in number, were found. Jejunoileal anastomosis was done and massive gut
resection (about 60 cms) was done. Child was given multiple blood and FFP transfusions during post operative period. At around 12th
post operative day of 2nd surgery child developed faecal
fistula which was of low output type. Gradually fistula output was decreased
and patient was sent on oral feed. The histopathology report of the
mass in mesentery of jejunum showed appearances suggestive of gastrointestinal
stromal tumor and tumor marker CD-117 was advised which was found to
be positive.
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Photograph 1: Mass in mesentry of jejunum, about 8x8 cms |
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Photograph 2: Multiple perforations
and gangrenous gut |
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Photograph 3: Massive gut
resection, about 60 cms |
Gastrointestinal stromal tumors (GISTs) belong to a group of cancers called soft tissue sarcomas. Although
the exact incidence is still somewhat unclear, it is now estimated that,
in the United States, between 5,000 and 10,000 people each year develop
GISTs. GISTs occurring in young patients (under age 18) is much rarer,
occurring in perhaps 1% to 2% of all GIST patients.[1,2] GISTs having a first occurrence in patients less
than 18 years of age are now described as Pediatric GISTs. Pediatric GIST is much more common in girls than
in boys. It usually occurs between the ages of about 6 and 18. GISTs can also originate in the mesentery and omentum. These tumors
have been reported ranging in size from smaller than 1 cm to as large
as 40 cm in diameter. GISTs can occur anywhere along the gastrointestinal
tract, including stomach (70%), small intestine (20-30%), anorectum
(7%), colon, and esophagus.[3] Presenting signs and symptoms
are variable depending on the size and anatomic location of the tumour
and can include abdominal pain, hematemesis, melena, and anemia from
occult gastrointestinal bleeding.[4] Other symptoms are generally
associated with an enlarging abdominal mass and may include anorexia,
nausea, vomiting, weight loss, epigastric fullness, and early satiety.
No risk factors have been identified.[5] CD117 has become a
very important tool in the differentiation of GIST from other GI mesenchymal
tumors.[6,7] Despite the proven success of imatinib and other newer tyrosine-kinase
inhibitors, surgical resection remains the treatment of choice and offers
the only chance for cure from GIST.[8-10] The main operative
principle is resection of the tumor with negative microscopic margins.
Our case was a male child of 14 years with this
rare tumor who underwent its surgical removal.
- Prakash S, Sarran
L, Socci N et al. Gastrointestinal Stromal Tumors in Children and Young
Adults: A Clinicopathologic, Molecular, and Genomic Study
of 15 Cases and Review of the Literature. J Pediatr Hematol Oncol.
April 2005;27:4:179-187.
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Miettinen M, Lasota
J, Sobin LH. Gastrointestinal Stromal
Tumors of the Stomach in Children and Young Adults: A Clinicopathologic, Immunohistochemical, and Molecular
Genetic Study of 44 Cases With Long-Term Follow-Up and Review of the
Literature. Am J Surg Pathol October 2005;29(10):1373-1381
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Park J, Rubinas TC, Fordham LA, Phillips JD. Multifocal gastrointestinal stromal tumor (GIST) of the stomach in a
11-year-old girl. Pediatr Radiol. 2006;36:1212–1214.
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Hayashi Y, Okazaki T, Yamataka A, Yanai T, Yamashiro
Y, Tsurumaru M. Gastrointestinal stromal tumor in a child and review
of the literature. Pediatr Surg Int. 2005;21:914–917.
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Blanke C, Eisenberg BL, Heinrich M. Epidemiology
of GIST. Am J Gastroenterol. Oct 2005;100(10):2366.
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Franquemont DW. Differentiation and risk assessment
of gastrointestinal stromal tumors. Am J Clin Pathol. Jan 1995;103(1):41-47.
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Tzen CY, Mau BL. Analysis of CD117-negative gastrointestinal
stromal tumors. World J Gastroenterol. Feb 21 2005;11(7):1052-1055.
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Boni L, Benevento A, Dionigi G, Rovera F, Dionigi
R. Surgical resection for gastrointestinal stromal tumors (GIST): experience
on 25 patients. World J Surg Oncol. 2005;3:78.
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Bucher P, Egger JF, Gervaz P, Ris F, Weintraub D,
Villiger P. An audit of surgical management of gastrointestinal stromal
tumours (GIST). Eur J Surg Oncol. Apr 2006;32(3):310-314.
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Knoop M, St Friedrichs K, Dierschke J. Surgical management
of gastrointestinal stromal tumors of the stomach. Langenbecks Arch Surg. Apr 2000;385(3):194-198.
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