title: Combined presence of multiple gastrointestinal stromal tumors along with duodenal submucosal somatostatinoma in a patient with neurofibromatosis type 1.
abstract: Neurofibromatosis type-1 (NF-1) is an autosomal dominant disorder, with increased risk of developing benign and malignant tumors of the gastrointestinal tract (GIT). However, the synchronous presence of multiple GIT stromal tumors and duodenal submucosal somatostatinoma, like in this 50-year-old female NF-1 patient, is very rare. She presented with hematemesis, malena, along with multiple neurofibromas all over the body. Thorough radiological and peroperative work-up revealed multiple ulcerated submucosal and serosal nodules in the proximal small intestine. Histological work-up revealed diagnosis of a duodenal submucosal somatostatinoma with multifocal serosal gastrointestinal stromal tumors. This case is being reported to highlight the rare coincidence of multiple GIT tumors in an NF-1 patient.
authors: Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
authors: Tarun
authors: T
authors: Kumar
authors: Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
authors: Brijnandan
authors: B
authors: Gupta
authors: Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
authors: Prasenjit
authors: P
authors: Das
authors: Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
authors: Deepali
authors: D
authors: Jain
authors: Department of Gastrointestinal Surgery, All India Institute of Medical Sciences, New Delhi, India.
authors: Hemant Ashok
authors: HA
authors: Jain
authors: Department of Radiology, All India Institute of Medical Sciences, New Delhi, India.
authors: Kumble S
authors: KS
authors: Madhusudhan
authors: Department of Gastrointestinal Surgery, All India Institute of Medical Sciences, New Delhi, India.
authors: Nihar Ranjan
authors: NR
authors: Dash
authors: Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
authors: Siddhartha Datta
authors: SD
authors: Gupta
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis; D005260:Female; D046152:Gastrointestinal Stromal Tumors / Q000150:complications* / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D006396:Hematemesis / Q000209:etiology; D006651:Histocytochemistry; D006801:Humans; D007150:Immunohistochemistry; D007421:Intestine, Small / Q000473:pathology; D008551:Melena / Q000209:etiology; D008853:Microscopy; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D011860:Radiography, Abdominal; D013004:Somatostatin / Q000032:analysis; D013005:Somatostatinoma / Q000150:complications* / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D016708:Synaptophysin / Q000032:analysis; D014057:Tomography, X-Ray Computed
chemical_list: D014408:Biomarkers, Tumor; D016708:Synaptophysin; D013004:Somatostatin