The rare association between intestinal leiomyosarcoma, von Recklinghausen’s disease (type-1 neurofibromatosis) and gastroparesis is described. A 20-year-old male, diagnosed 12 years earlier as having pelvic von Recklinghausen’s disease, presented with nausea and vomiting. A gastric scintigraphy demonstrated an extremely slow gastric emptying time in the absence of obvious causes for gastroparesis. A small ileal leiomyosarcoma was later found and removed by surgery. The latter was followed by a marked improvement in the clinical condition of the patient.

Journal Section:
Case Report
Keywords:
Leiomyosarcoma, von Recklinghausen’s disease, Gastroparesis
1.
Riccardi VR: Neurofibromatosis: Phenotype, History and Pathogenesis, ed 2. Baltimore, Johns Hopkins University Press, 1992.
2.
Ishizaki T, Tada Y, Bandai Y: Leiomyosarcoma of the small intestine associated with von Recklinghausen’s disease: Report of a case. Surgery 1992;111:706–710.
[PubMed]
3.
Miyoshi LM, Tamiya S, Iida M, et al: Primary jejeunal malignant mixed tumor in a patient with von Recklinghausen’s neurofibromatosis. Am J Gastroenterol 1996;91:795–797.
[PubMed]
4.
Gutmann DH, Collins FS: Recent progress towards understanding the molecular biology of von Recklinghausen’s neurofibromatosis. Ann Neurol 1992;31:555–561.
[PubMed]
5.
Stumpf DA: Neurofibromatosis: NIH Consensus Development Conference Statement. Arch Neurol 1988;45:575.
[PubMed]
6.
Jeremie B, Djuric LJ, Mijatovic LJ: Severe late intestinal complications after abdominal and/or pelvic external irradiation with high energy photon beams. Clin Oncol R Coll Radiol 1991;3:100–104.
[PubMed]
7.
Rokkas T, Palmer TJ, Sladen GE: Tumors of the pancreas as a sequel to abdominal irradiation. Postgrad Med J 1989;65:493–496.
[PubMed]
8.
Ogilvie H: Large intestine colic due to sympathetic deprivation: A new clinical syndrome. Br Med J 1948;ii:6671–6673.
9.
Schuffler MD, Baird W, Fleming R: Intestinal pseudo-obstruction as the presenting manifestation of small cell carcinoma of the lung. Ann Intern Med 1983;98:129–134.
[PubMed]
10.
Gerl A, Stork M, Schalhorn A: Paraneoplastic chronic intestinal pseudo-obstruction as a rare complication of bronchial carcinoid. Gut 1992;33:1000–1003.
[PubMed]
11.
Choe AL, Zeissmann HA, Fleischer DE: Tumor associated gastroparesis with esophageal carcinoma. Dig Dis Sci 1989;34:1132–1134.
[PubMed]
12.
Lhermitte F, Grey F, Lyon Caen O: Paralysis of the digestive tract with lesion of myenteric plexus: a new paraneoplastic syndrome. Rev Neurol 1980;136:825–836.
13.
Lautenbach E, Lichtenstein GR: Retroperitoneal leiomyosarcoma and gastroparesis: A new association and review of tumor associated intestinal pseudo-obstruction. Am J Gastroenterol 1995;90:1338–1341.
[PubMed]
14.
Lennon VA, Sas DF, Busk MF: Enteric neuronal antibodies in pseudo-obstruction with small cell lung carcinoma. Gastroenterology 1991;101:1143–1144.
15.
Graus F, Elkon KB, Cordon-Cardo C: Sensory neuropathy and small cell lung cancer-antineural antibody that also reacts with the tumor. Am J Med 1986;80:45–52.
16.
Liang BC, Albers JW, Sima AAF: Paraneoplastic pseudo-obstruction, mononeuropathy multiplex and sensory neuronopathy. Muscle Nerve 1994;17:91–96.
[PubMed]
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1999
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