Acute diffuse peritonitis due to spontaneous rupture of a primary gastrointestinal stromal tumor of the jejunum: A case report

IntroductionGastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Overt peritonitis caused by GIST rupture is very uncommon. Three types of GIST rupture have been described: closed perforation due to abscess (abscess type), hemoperitoneum leading to rupture of the hematoma capsule in the tumor (hemoperitoneum type), and perforation of the digestive tract via a fistula leading to central necrosis of the tumor (bowel perforation type). This report describes a patient with spontaneous tumor rupture and diffuse peritonitis, a variant of the bowel perforation type of GIST rupture.Presentation of caseA 74-year-old man presented with symptoms of vomiting and abdominal pain. Computed tomography (CT) scan revealed an approximately 10 × 7-cm mass in the pelvis with free air and fluid collection. Emergency laparotomy revealed a tumor in the jejunum, which was ruptured with a hole measuring 5 mm in diameter. The tumor and part of the jejunum were resected. Immunohistochemically, the mass was diagnosed as a GIST originating from the gastrointestinal tract. Despite chemotherapy with imatinib mesylate, the patient died 22 months after surgery.ConclusionsThis report describes a patient with acute diffuse peritonitis due to spontaneous rupture of a primary GIST of the jejunum.     

Letter to the Editor

Meckel’s diverticulum is a common congenital anomaly of the small intestine, affecting 2% of the population. Neoplastic transformation is infrequent and gastrointestinal stromal tumours are exceptional in this location. We report a case of gastrointestinal bleeding caused by a gastrointestinal stromal tumour of Meckel’s diverticulum.     

SUCCESSFUL ENDOSCOPIC ULTRASOUND‐GUIDED FINE‐NEEDLE ASPIRATION OF THE PELVIC LESION THROUGH THE SIGMOID COLON

Endoscopic ultrasound‐guided fine‐needle aspiration (EUS‐FNA) is a useful modality when the target is a lymph node located in the mediastinum, perigastric area or perirectum. Although it is difficult to carry out EUS‐FNA of the colon using an oblique view linear scope, we report two cases of successful EUS‐FNA of the lesions via the proximal sigmoid colon using a recently available new convex type EUS scope. Case 1 was a 77‐year‐old Japanese woman noted to have multiple lymph node swelling in the para‐aortic area and in the pelvis. Case 2 was a 60‐year‐old Japanese woman noted to have a large mass in the left lower abdomen. In case 1, oral EUS showed no lymph node swelling. In both cases, EUS with forward‐viewing radial echoendoscope was carried out via the anus, and multiple lymph‐node swelling or a large mass was observed near the proximal sigmoid colon. In the EUS‐FNA for these cases, we used a new convex‐type EUS scope that has an oblique view, but with a wide‐angled optical device giving a view similar to a forward one. EUS‐FNA was successfully carried out on the lesions. The pathological specimen revealed diffuse large B‐cell lymphoma in case 1 and gastrointestinal stromal tumor (GIST) in case 2.     

Molecular pathology in sarcomas

Abstract Bone and soft tissue sarcomas are an infrequent group of tumours. Their prevalence is 4 in 100 000 people/year, making the disease quite rare. Some of these tumours, such as synovial sarcoma, Ewing tumour and osteosarcoma, are more usual in adolescents or in young adults; there are, though, some neoplasias such as leiomyosarcoma or liposarcoma that are more frequent in patients over 55 years. There are more than a hundred different types of sarcomas from the histological point of view. This is the main limitation at the time of finding major clinic essays on patients with specific types of sarcomas. From the molecular point of view, these neoplasias are grouped into two main types: (a) sarcomas showing specific genetic alterations and relatively simple karyotypes, and translocations which originate gene fusions (e.g., EWS-FLI1 in Ewing tumour); or specific genetic mutations (e.g., c-kit in the gastrointestinal stromal tumour), and (b) sarcomas showing unspecific gene alterations and very complex karyotypes, and very numerous gains and losses. This review describes diverse types of molecular alterations as well, their utility in the clinical domain, as well as implications for the pathologist in translational research in sarcomas. *Supported by an unrestricted educational grant from Sanofi-Aventis.     

Results of plasma N-terminal pro B-type natriuretic peptide and cardiac troponin monitoring in GIST patients do not support the existence of imatinib-induced cardiotoxicity

Background: Recently, case reports of patients treated withimatinib (imatinib mesylate; Gleevec®; Glivec®) indicatedthat this tyrosine kinase inhibitor may induce cardiomyopathy.Consequently, careful cardiac monitoring was advocated for clinicalstudies. The purpose of this study was to prospectively evaluatewhether imatinib (Gleevec) induces early, subclinical, cardiactoxicity. Patients and methods: History and physical examination werecarried out with special attention for symptoms of heart failure.Additionally, assessments of serial plasma N-terminal pro B-typenatriuretic peptide (NT-proBNP) and serum cardiac troponin T(cTnT) measurement before and 1 and 3 months after the startof imatinib treatment (400–800 mg daily) were done inpatients with advanced and/or metastatic gastrointestinal stromaltumours (GIST). Results: A total of 55 GIST patients were enrolled. Only onepatient, with a normal pretreatment NT-proBNP, showed an increasein NT-proBNP to above age-specific normal values during imatinibtreatment and developed symptomatic heart failure due to pre-existentcardiac valvular disease. cTnT levels remained stable. Conclusions: In our study population, imatinib treatment forGIST was not associated with an increase in plasma NT-proBNPlevels, indicating that the risk of subclinical cardiac toxicityis limited with the use of this agent. These results do notsupport the current strategy to standard cardiac monitoringin all patients. This may be restricted to GIST patients witha history of cardiac disease. Key words: cardiotoxicity, GIST, imatinib mesylate, NT-proBNP, troponin T Received for publication August 15, 2007. Revision received August 24, 2007. Accepted for publication August 29, 2007.     

Simultaneously occurring chronic myelogenous leukemia and gastrointestinal stromal tumors treated with imatinib

Imatinib mesylate (imatinib, STI571, Gleevec; Novartis Pharma) is an orally administered competitive inhibitor of BCR-ABL tyrosine kinase that has demonstrated significant efficacy in chronic myelogenous leukemia (CML). Imatinib is also a potent inhibitor of the receptor-type KIT tyrosine kinase with demonstrated benefits in KIT-expressing gastrointestinal stromal tumors (GISTs). This article presents the case of a patient with simultaneous occurrence of CML and GIST who was treated with the single agent imatinib. To our knowledge, this is the first report of simultaneous occurrence of these two malignancies and of the efficacy of imatinib in concurrent treatment of both neoplasms in a single patient.     

A gastrointestinal stromal tumor in the stomach: usefulness of computed tomographic volumetry

We report herein the case of a 70-year-old man who was found to have a gastrointestinal stromal tumor (GIST) in the stomach following sigmoid colon resection. Preoperative gastroscopic and barium examinations revealed a submucosal tumor, measuring 10cm, on the upper part of the stomach. Using computed tomography (CT) images (i.e., computed tomographic volumetry) the doubling time of this tumor was calculated, accurately, as 3.3 months, which suggested a high growth rate and malignancy. A laparotomy and partial gastric resection were performed. Histologically, the tumor consisted of spindle-shaped cells with oval nuclei. In immunohistochemical studies, the tumor cells were positive with respect to c-kit, CD34, and vimentin, but negative with respect to smooth muscle actin and S-100 protein. There were 15–16 mitoses per 50 high-power fields (HPFs), and the Ki-67 antigen (MIB-1) index was 25.5% in the most active areas, which also indicated malignancy. The final pathological diagnosis of this tumor was malignant GIST. The patient was found to have hepatic metastasis 27 months after the surgery, and he subsequently received a hepatic subsegmentectomy. To our knowledge, there are very few reports concerning the growth rate of GISTs. Computed tomographic volumetry is useful for the follow-up of small or irregularly shaped gastric submucosal tumors, and for making decisions regarding surgical intervention.