Endocr Pathol DOI 10.1007/s12022-014-9350-7

Gastrointestinal Stromal Tumor of the Adrenal Gland: A Case Report and Review of the Literature Hussam Abou Al-Shaar & Shrouq Solimanie & Ayman Azzam & Tarek Amin & Ahmed Abu-Zaid

# Springer Science+Business Media New York 2014

Abstract Gastrointestinal stromal tumors (GISTs) are rare neoplasms of the gastrointestinal (GI) tract. The occurrence of these neoplasms ectopically outside the GI tract is extremely uncommon. Only one case of primary adrenal GIST has been reported in the literature. In this account, we report a second case of primary adrenal GIST in a 34-year-old male who presented with a 5-week history of gradually progressive left flank pain and early satiety. Whole-body positron emission tomography (PET)/computed tomography (CT) scan showed a 14×11 cm hypermetabolic 18fluorodeoxyglucose (FDG)-avid mass lesion involving the left adrenal gland and dorsal part of the left hemi-diaphragm. Biopsy of the lesion revealed tumor cells that are immunoreactive to CD-117 and CD-34 and negative to CD-31, S-100, cytokeratin, desmin, and vimentin, features characteristic of GIST. The patient was given imatinib, which drastically decreased his complaints with almost complete resolution of the tumor on his last follow-up radiographic images. Primary left adrenal GIST is an extremely rare neoplasm and can be confused with GISTs arising from the greater curvature of the stomach. Imatinib therapy is optimal in the management of these tumors.

Keywords Gastrointestinal stromal tumor . Extra-gastrointestinal stromal tumor . Adrenal gland . ?Greater curvature . Stomach . Imatinib H. Abou Al-Shaar : S. Solimanie : A. Abu-Zaid (*) College of Medicine, Alfaisal University, P.O. Box 50927, Riyadh 11533, Saudi Arabia e-mail: [email protected] A. Azzam : T. Amin Oncology Center, King Faisal Specialist Hospital and Research, Riyadh, Saudi Arabia A. Azzam Faculty of Medicine, Alexandria University, Alexandria, Egypt

Introduction Gastrointestinal stromal tumors (GISTs) arise from the interstitial cells of Cajal, specialized cells in the autonomic nervous system of the intestine [1, 2]. These tumors account for the majority of gastrointestinal (GI) mesenchymal neoplasms [1, 3]. GISTs commonly affect adults with an equal male-tofemale distribution [4, 5]. Extra-gastrointestinal primary GISTs (EGISTs) rarely occur [6, 7]. To the best of our knowledge, the English medical literature describes only two patients with gastric GISTs presenting with left adrenal mass and a single patient with primary adrenal GIST [6, 7]. Herein, we report the second ever primary EGIST arising from the adrenal gland. In addition, a literature review on GISTs is presented.

Case Report A 39-year-old otherwise healthy male patient was referred from a primary care hospital for further evaluation and management of a left adrenal mass. Patient had a 5-week history of gradually progressive left flank pain and early satiety. Patient denied any history of trauma and systemic review was unremarkable. Physical examination was notable for a moderately enlarged and tender mass involving the left flank region. All laboratory tests including complete blood count, renal, bone, hepatic, coagulation, and tumor profiles were normal. Whole-body positron emission tomography (PET)/computed tomography (CT) scan showed a 14×11 cm hypermetabolic 18fluorodeoxyglucose (FDG)-avid mass lesion involving the left adrenal gland and dorsal part of the left hemidiaphragm (Fig. 1). There was no evidence of distant metastasis. In consideration of a neoplastic lesion, an ultrasound-guided core-needle biopsy was recommended. Histopathological

Endocr Pathol Fig. 1 Whole-body positron emission tomography (PET)/ computed tomography (CT) scan. The scan showed a 14×11 cm hypermetabolic 18 fluorodeoxyglucose (FDG)avid mass lesion involving the left adrenal gland and dorsal part of the left hemi-diaphragm

analysis showed a spindle-cell neoplasm (Fig. 2a). Immunohistochemical analysis showed positive immunoreactivity to CD-117 (Fig. 2b) and CD-34 (Fig. 2c) and negative immunoreactivity to CD-31, S-100, cytokeratin, desmin, and vimentin. The final pathological diagnosis was consistent with primary GIST of the left adrenal gland. Surgical resection was advised; however, patient refused. Subsequently, patient was offered imatinib (a tyrosine-kinase inhibitor). Following therapy, a 3-month follow-up PET/CT scan showed almost complete resolution of the metabolic activity of the known left adrenal mass lesion (Fig. 3). Moreover, patient reported markedly reduced left flank symptoms.

Discussion GISTs are considered the most common mesenchymal neoplasms of the GI tract constituting 5 and ≤10 cm and no more than 5 mitoses per 50 HPFs [24, 36]. However, some authors deny the efficiency of these factors for EGISTs [38, 39]. Metastasis of GISTs to lymph nodes and skin is extremely rare. The most common sites of metastasis include, in a descending order of frequency, liver, peritoneum, lung, pleura, bones, and soft tissues [12]. Radiation and chemotherapy are generally ineffective with these tumors [3, 4, 9]. Surgical excision for localized resectable primary tumors, according to surgical oncology protocols, is considered the mainstay treatment for GISTs [8]. The resection should be carried out meticulously in order to avoid the risk of tumor rupture [4]. The resection should be complete with safe margins, due to high risk of recurrence [3, 13]. Owing to the extreme unlikelihood of lymph node involvement, lymph node dissection is not routinely required [4]. The role of imatinib mesylate has revolutionized the management of GISTs, especially with unresectable tumors. It has been proven that imatinib can tremendously decrease tumor size and recurrence rate [4, 9, 12, 13, 40]. The drug is generally safe and well tolerated, yet some side effects have been reported including, diarrhea, edema, skin rashes, and myalgia [8]. Moreover, resistance to the drug has also been recorded [7, 8, 40, 41]. It is estimated that 10 % of patients will develop resistance to imatinib mesylate within 3–6 months of therapy and 40–50 % will develop resistance within 24 months of therapy [40]. Those patients may benefit and respond well to sunitinib (a new tyrosine-kinase inhibitor) [42]. Our patient responded to imatinib therapy with a drastic decrease of his symptoms and almost complete resolution of the tumor on radiographic imaging.

Conclusion EGIST arising primarily from the left adrenal gland is extremely rare. The proximity of the left adrenal gland to the greater curvature can mislead the physician to reach accurate diagnosis. Radiological investigations and immunohistochemical staining of the biopsied lesion are essential to delineate the tumor and aid in reaching the diagnosis. Imatinib therapy for GISTs and EGISTs shows promising results in the management of these neoplasms with complete resolution of both symptoms and tumor as seen in our patient.

Conflict of Interest The authors declare no conflict of interest regarding the production of this article. The authors have no personal financial or institutional interest in any of the drugs, materials, or devices described in this article.

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Gastrointestinal stromal tumor of the adrenal gland:a case report and review of the literature.

Gastrointestinal stromal tumors (GISTs) are rare neoplasms of the gastrointestinal (GI) tract. The occurrence of these neoplasms ectopically outside t...
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