CT detection of an ampullary somatostatinoma in a patient with von Recklinghausen's disease.

Hamissa, S; Rahmouni, A; Coffin, Carla S.; Wolkenstein, P.

doi:10.2214/ajr.173.2.10430168

Cited by 10 publications

(3 citation statements)

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“…Radiologic Features.-To our knowledge, only two case reports in the English language radiology literature have described the appearance of duodenal carcinoids in patients with NF1 (45,46). We have seen five patients with NF1 and gastrointestinal carcinoids.…”

Section: Neuroendocrine Tumors Carcinoidmentioning

confidence: 86%

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Abdominal Neoplasms in Patients with Neurofibromatosis Type 1: Radiologic-Pathologic Correlation

Levy¹,

Patel²,

Dow³

et al. 2005

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Neurofibromatosis type 1 (NF1) is one of the most common genetic disorders. NF1 is a complex disease resulting from a spectrum of mutations that may occur at many locations along the large, complex NF1 gene, which is located on chromosome 17. Mutations of the NF1 gene lead to abnormal tumor suppression. Consequently, patients with NF1 have an increased prevalence of benign and malignant neoplasms throughout the body. There are five categories of NF1 tumors that occur in the abdomen: neurogenic, neuroendocrine, nonneurogenic gastrointestinal mesenchymal, embryonal, and miscellaneous. Many of these tumors are age related, occur at specific anatomic locations, and have unique imaging features. Notably, many patients have a variety of organs affected because there is a high prevalence of multiple tumors occurring in the same patient. Neurofibromas are the most common benign neoplasms and may occur in the retroperitoneum or visceral organs. Malignant peripheral nerve sheath tumor is an aggressive malignancy that is the most common malignant tumor of the abdomen in patients with NF1. Interpreting abdominal imaging studies in patients with NF1 can be challenging because of the wide spectrum and diverse nature of tumors that occur in this disease.

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Section: Neuroendocrine Tumors Carcinoidmentioning

confidence: 86%

“…The CT attenuation in all cases was homogeneous. Both Hamissa et al (45) and Mendes Ribeiro and Woodham (46) reported marked en- hancement of the tumor on contrast-enhanced CT scans. Our cases showed little to no enhancement of the tumor on CT scans (Figs 25, 26).…”

Section: Neuroendocrine Tumors Carcinoidmentioning

confidence: 96%

Abdominal Neoplasms in Patients with Neurofibromatosis Type 1: Radiologic-Pathologic Correlation

Levy¹,

Patel²,

Dow³

et al. 2005

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112

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“…When the tumor is extrapancreatic, these symptoms are present in only 10%-15% of cases; they are often associated with von Recklinghausen disease [16] and produce symptoms due to their local effects or bleeding, even with very small lesions. The duodenal somatostatinoma produces jaundice, bowel obstruction, exocrine pancreatic insufficiency, acute pancreatitis, upper gastrointestinal hemorrhage, and abdominal pain; cases of complete somatostatinoma syndrome from ampullary somatostatinoma have been reported [12].…”

Section: Discussionmentioning

confidence: 99%

Incidental Small Ampullary Somatostatinoma Treated with Ampullectomy 2 Years After Diagnosis

et al. 2006

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Somatostatinomas are rare tumors; ampullary somatostatinomas are very rare. We report a case of a small pure somatostatin-producing neuroendocrine tumor of ampulla of Vater in a 54-year-old woman with neither neurofibromatosis nor somatostatinoma syndrome, "incidentally" discovered during an abdominal computed tomography. The patient initially refused other adjunctive exams but after 2 years she was admitted, presenting with itch, night sweats, severe fatigue, and unintentional weight loss. The size of the tumor (1.5 cm) and the other radiologic findings had not changed since the abdominal CT scan 2 years before. The somatostatin, gastrin, glucagons, serotonin, vasoactive intestinal peptide, dopamine, norepinephrine, epinephrine, and calcitonin plasma levels were normal. ERCP-obtained biopsies revealed a neuroendocrine tumor with psammoma bodies; immunohistochemical profile was positive for chromogranin and somatostatin. The patient underwent surgery; intraoperative histologic examination of lymph nodes sampling of perihepatic and periduodenal lymph nodes was negative for metastasis. We performed, therefore, a transduodenal ampullectomy. The patient continues to do well at 3 years' follow-up with no evidence of local or distance recurrence of disease.

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Advanced Imaging of the Abdomen

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CT detection of an ampullary somatostatinoma in a patient with von Recklinghausen's disease.

Cited by 10 publications

References 0 publications

Abdominal Neoplasms in Patients with Neurofibromatosis Type 1: Radiologic-Pathologic Correlation

Abdominal Neoplasms in Patients with Neurofibromatosis Type 1: Radiologic-Pathologic Correlation

Incidental Small Ampullary Somatostatinoma Treated with Ampullectomy 2 Years After Diagnosis

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