Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Plexiform neurofibromatosis of the liver was recognized by needle biopsy of the liver in an 11-yr-old boy who had a 2-yr history of diarrhea, intermittent abdominal pain, failure to gain weight and progressive abdominal distention. Imaging studies demonstrated a large retroperitoneal mass; a laparotomy was performed. At surgery, the mesentery was greatly thickened by neurofibromas, and plexiform neurofibroma extended through the hilum of the liver. Light and electron microscopy demonstrated that in addition to the direct involvement by tumor, neural hyperplasia existed throughout the liver. The most distal ramifications of the portal spaces were filled with Schwann cells, bundles of unmyelinated nerves and perineurium-surrounded nerves containing myelinated and unmyelinated fibers. The ultrastructural findings were consistent with stimulation of proliferation of all the portal neural elements and tumoral tissue. The nontumoral response was more than simple hyperplasia because it appeared to result in fibrotic changes in the most involved areas and active breaching of the limiting plate with destruction of hepatocytes and collagen deposition throughout the liver.
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PMID:Plexiform neurofibromatosis of the liver and mesentery in a child. 211 33

A 23-year-old man with Von Recklinghausen's disease was admitted because of abdominal pain. The preoperative diagnosis was malignant mesenteric tumor. Laparotomy was done and the tumor was located in the mesentery of the ileum which showed numerous tortuous cords or bulbous projections from the surface. Partial resection of the involved ileum and the mesentery was performed. Histological examination revealed plexiform neurofibroma and malignant Schwannoma of the mesentery. It is very difficult to preoperatively diagnose early associated lesions of Von Recklinghausen's disease. Therefore, careful examination of the GI tract is important so that earlier therapy can be carried out.
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PMID:[A case of malignant schwannoma of the mesentery in a patient with von Recklinghausen's disease]. 643 67

We report computerized tomographic and magnetic resonance imaging findings of neurofibromatosis type 1 with mesenteric involvement in two patients. The first patient was a 13-year-old female with a panmesenteric plexiform neurofibroma with segmental involvement of the bowel wall; she had a 3-year history of abdominal pain. The second patient was a 60-year-old female who presented with malignant transformation of multiple mesenteric neurofibromas 4 months after primary operation.
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PMID:Mesenteric involvement in neurofibromatosis type 1: CT and MRI findings in two cases. 1475 98

A 32-year-old female patient visited the emergency room because of sudden onset of abdominal pain, which was found to be induced by hemorrhagic infarction of a plexiform neurofibroma arising from the lesser omentum. No other classic clinical characteristics of systemic von Recklinghausen's disease had been seen up until now. The tumor, classified as sporadic plexiform neurofibroma, rarely occurs in the digestive tract. This diagnosis was confirmed by exploratory laparotomy with complete resection of the tumor. Here, we describe the rare case of a sporadic intra-abdominal plexiform neurofibroma and discuss the clinical characteristics and management of plexiform neurofibroma.
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PMID:Acute abdominal pain caused by spontaneous hemorrhagic infarction of a solitary plexiform neurofibroma of lesser omentum. 1839 52

We present an extremely rare case of plexiform neurofibroma involving the hepatic hilum. A 24-year old woman who had been diagnosed with neurofibromatosis type 1 was referred to our hospital for evaluation of an abdominal mass found on computed tomography and progressive aggravation of intermittent abdominal pain. Abdominal computed tomography revealed a multilobulated non-enhancing mass involving the celiac trunk and hepatic artery, that extended to the hepatic hilum through the hepatoduodenal ligament. Magnetic resonance imaging showed the lesion extending along the intrahepatic Glisson's sheath. Based on the imaging findings, the patient was diagnosed to have a neu-rofibroma, although sarcomatous differentiation could not be excluded. The tumor was resected, leaving behind the intrahepatic extension, with the aim of alleviating the abdominal pain and preventing obstructive jaundice. Histopathological examination revealed the diagnosis of plexiform neurofibroma. At present, three years after the surgery, the patient remains symptom-free, without any evidence of recurrence.
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PMID:Plexiform neurofibroma in the hepatic hilum associated with neurofibromatosis type 1: a case report. 2113 95

Plexiform neurofibroma (PN) of the digestive tract is very rare and usually part of the generalized syndrome of neurofibromatosis type 1 (von Recklinghausen disease). Solitary PN of the stomach is extremely rare and has not been reported in the literatures. Here we present a case of solitary PN of the stomach, which was not associated with von Recklinghausen disease. A 38-year-old male presented abdominal pain and distention for 7 d. The patient underwent endoscopy of the upper gastrointestinal tract, which revealed a 3.5 cm protruding and cauliflower-shaped mass with a shallow 1 cm central ulcer in the greater curvature of the stomach. The lesion was removed by laparoscopic surgery. Histological examination demonstrated characteristic histological findings of spindle-shaped cells. Immunohistochemical analysis showed that the tumor cells were positive for S-100 protein, but negative for CD34, KI-67, CD117, and actin. Based on histological findings, gastrointestinal stromal tumor could be excluded, and thus the case was confirmed as PN. We described the clinical features, physical examination, endoscopic findings, and histopathological examination of this case.
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PMID:Solitary plexiform neurofibroma of the stomach: a case report. 2480 34

Malignant peripheral nerve sheath tumors (MPNSTs) are extremely uncommon neoplasms, predominantly seen within the deep soft tissues of the extremities, in close proximity to the nerve trunks. Retroperitoneal MPNSTs are exceedingly rare, usually seen in association with neurofibromatosis type 1 (NF-1), and often result from malignant degeneration of a plexiform neurofibroma. These tumors are highly malignant and the prognosis is worsened if they occur in association with NF-1. Metastasis is not uncommon, but local invasion by these tumors is rarely reported. Renal artery pseudoaneurysms are mostly iatrogenic and rarely result from invasion by retroperitoneal neoplasms. Ultrasonography is a valuable tool in early diagnosis of these neoplasms and vascular complications, particularly in emergency cases requiring immediate operative intervention. To the best of our knowledge, no case of renal artery pseudoaneurysm caused by retroperitoneal MPNST has been reported to date. We present a case of a middle-aged female with Von Recklinghausen disease, complaining of sudden onset of severe abdominal pain, where ultrasound accurately diagnosed renal artery pseudoaneurysm caused by a large retroperitoneal MPNST, and who was later treated by radical excision of the tumor and nephrectomy.
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PMID:Role of ultrasonography in detection of renal artery pseudoaneurysm caused by retroperitoneal malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1. 2727 39

We present a rare case of histologically proven neurofibromatosis of the liver, hepatic hilum, retroperitoneum, and mesentery. An adult male who had been diagnosed with neurofibromatosis (NF) type 1 underwent a computed tomography (CT) scan for abdominal pain and vomiting. The CT scan showed a large low-attenuating lesion in the region of porta hepatis which was infiltrating along portal tracts into the liver, encasing the major vessels, and extending into the retroperitoneum and mesentery. Based on the radiological findings, a differential diagnosis of plexiform neurofibroma was given, although sarcomatous transformation could not have been entirely excluded from imaging alone. The tumor was subsequently biopsied, and the histopathological analysis confirmed the diagnosis of neurofibroma. This case highlights the importance and diagnostic dilemmas in the presence of this tumor at atypical locations in this disease spectrum.
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PMID:Hepatic, Periportal, Retroperitoneal, and Mesenteric Neurofibromatosis in von Recklinghausen's Disease. 2979 73