Berrag et al. Int J GastroenterolHepatol Transpl Nutr 2017;2(iii):11-13 ISSN 2455–9393

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Case Report

Multifocal Gastrointestinal Stromal Tumor in the Adult: a rare entity Berrag Sanaa, Rouibaa Fedoua, Chakkor Amal, Tamzaourte Mouna, Miyabe Fidele, Touibi Youssef and Aourarh Aziz

ABSTRACT Gastrointestinal stromal tumours (GISTs) are the most common primary

mesenchymaltumour of gastro-intestinal tract. Typically, they occur as solitary lesions.

Their occurrence as disseminated intra-abdominal tumoursis considered an exceptional

event.

We report a case of 62 years old male who presented with large abdominal lump.

Computed tomography scan showed many hypoattenuating lesions and multiple

extrahepatic lesions with same behavior patterns in the coeliomesenteric and

retroperitoneal spaces.

The diagnosis of GIST was confirmed by immune-histochemical study of the biopsy

material. Patient was started on Imatinib therapy. Computed tomography scan done 6

months later showed an decrease in size and lesions number.

INTRODUCTION

Gastrointestinal stromal tumors (GIST) are the most common

non-epithelial tumors of the gastrointestinal tract.1

It represents a wide clinical spectrum of tumors with different

clinical presentations, locations, histology and prognosis.

Typically, they occur as solitary lesions, whereas their

occurrence as disseminated intra-abdominal tumours is

considered as an exceptional event. It usually connected with

metastasis from the site primarily affecting the GI tract wall.2

In this paper, we report a case of multifocal Gastrointestinal

stromal tumors presenting as a large abdominal mass admitted at

the military hospital Mohamed V in rabat, Morocco.

CASE REPORT

A 62-year-old male presented with a five-month history of an

lump in abdomen, gradually increasing in size associated with

intermittent, non-radiating pain. He denied nausea, vomiting,

weight loss, or change in bowel habits. Physical abdominal

examination revealed a 12cm × 10 cm well-defined mobile firm

to hard mass in the epigastrium extending to the right

hypochondrium. Systemic examination showed no distant or

lymph node metastasis.

CT scan of abdomen showed an enlarged liver seat of many

hypoattenuating lesions. Those lesions had necrosis at the center

and peripheral enhancement on post-contrast studies. The large

mass measuring 90*75cm in segment IV (Fig. 1).

Figure 1: Computed tomography abdomen showing many

hypoattenuating lesions corresponding to GIST (GIST

metastases).

International Journal of Gastroenterology, Hepatology,

Transplant & Nutrition

Department of Gastroenterology I, Military Hospital, Mohamed V University of Rabat,

Rabat Morocco

Address for Correspondence:

Sanaa Berrag

E-mail: sanaa.berrag@gmail.com

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Website:

www.journal.pghtn.com

Key words: Gastrointestinal stromal tumours; multifocal; Imatinib

Berrag et al. Int J GastroenterolHepatol Transpl Nutr 2017;2(iii):11-13 ISSN 2455–9393

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CT scan showed multiple extrahepatic lesion of different sizes

and with same behavior patterns in the coeliomesenteric and

retroperitoneal spaces.

Exploratory laparotomy does not occur considering the

anesthetic contraindications.

The patient underwent a transcutaneous CT-guided biopsy of

the tumor. Histopathology analysis of tissues sent identified a

malignant GIST tumor. On Immunohistochemistry the tumor

was positive for Ckit (CD 117), CD34 and SMA whereas it was

negative for Desmin and h-caldesmone.

DISCUSSION

Gastrointestinal stromal tumors (GISTs) are uncommon

mesenchymal tumors that arise predominantly in the

gastrointestinal tract (GIT), first described by Clarke and

Mazur3,4 in 1983.GISTs are derived from the interstitial cells of

Cajal which serves as pace maker of gastrointestinal tract

triggering smooth muscle contraction.3,4

GISTs represent the most common mesenchymal neoplasms of

the GIT with an annual incidence of 11-14 per 10,6 they form

0.1%-3.0% of gastrointestinal malignant tumors.5,6 GISTs can

arise at any age, but more than 80% are reported in individuals

older than 50 years (median 63 years).7,8 There is usually no

predilection for either gender but some series suggest a slight

male predominance.

GISTs can develop anywhere along the GI tract from the

esophagus to the rectum; however, stomach (60%) and small

intestine (30%) are the most common locations. Up to 30%of

GISTs exhibit high-risk (malignant) behavior such as metastasis

and infiltration.9-12 The metastatic pattern is predominantly

intra-abdominal, with spread throughout the peritoneal cavity

and to the liver. Lymph nodal invasion is uncommon.

GISTs are characterized by genetic expression of c-kit (a trans-

membrane tyrosine kinase receptor) and immune-histo-chemical

staining of CD 117, CD34 (70%), SMA (40%) and a novel gene

DOG1.4,13

GISTs are generally considered as solitary tumors. Most GIST

patients present with localized disease. The occurrence of

multiple primary neoplasms is considered an exceptional finding

limited to specific conditions: multifocal primary GISTs may be

observed in pediatric patients or in individuals affected by

hereditary GIST, NF1, or paraganglioma/ sarcoma and Carney’s

triad syndromes.14,15-24

Beyond these well-defined situations, the detection of multifocal

disease, irrespective of the number, size, and location of the

lesions, is commonly viewed as the result of the metastatic

dissemination of a single primary GIST. Based on this axiom,

patients with multifocal GISTs are by default classified as

advanced stage and treated as such.

First-line systemic treatment of metastatic GIST is imatinib.

Imatinib is a selective inhibitor of tyrosine kinase who has

revolutionized the management of this disease in recent years

and has also become the first line of treatment for metastatic

GISTs,27 as described for the patient in this case report.

With imatinib treatment, 83–89% of patients either respond or

achieve durable stable disease whereas only 11–17%

progress.28,29

CONCLUSION

GISTs are generally considered as solitary tumors and the

occurrence of multiple primary neoplasms is considered an

exceptional situation.

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