| STUDY DESIGN: Single subject (male, 34 years-of-age) case report. OBJECTIVES: To describe a unique case of plasma cell granuloma (PCG) with simultaneous brain and spinal cord involvement. SETTING: Private hospital, capital city, turkey. methods: The patient presented with headache and paraplega. T2 weighted MR images showed multiple hyperintense cerebral, cerebellar, brainstem and spinal cord lesions that enhanced homogeneously with contrast medium. The patient was operated on, and the two intramedullary spinal cord lesions at T11 and T12 were totally excised. RESULTS: After 3 months of an intense physiotherapy programme the patient was able to walk with help. CONCLUSION: We report for the first time, the occurrence of multiple, simultaneous brain and intramedullary spinal cord plasma cell granulomas. ( info) |
122/541. Recurrent inflammatory pseudotumor of small bowel mesentery presenting as perforative peritonitis. A 62-year-old man with recurrent inflammatory pseudotumor of the small bowel mesentery presented with perforative peritonitis; such a presentation has not been reported. The mass was excised successfully. ( info) |
123/541. Mesenteric inflammatory pseudotumor: unusual presentation with leukemoid reaction and massive calcified mass. The authors describe a child with an unusual presentation of mesenteric inflammatory pseudotumor in association with leukemoid reaction. An 11-year-old-boy admitted with short stature was found to have an abdominal mass localized in the right lower quadrant. The leukocyte count was 92,000/mm3 with neutrophilic leukemoid reaction. Abdominal ultrasonography and computed tomography revealed a massive calcified mass in the pelvis. Total resection of the mass was performed and the pathologic diagnosis of inflammatory pseudotumor of the mesentery was made. leukemoid reaction dramatically resolved within a few days after surgical resection. physicians should be aware of the association of inflammatory pseudotumor, leukemoid reaction, and massive calcification. ( info) |
124/541. Inflammatory pseudotumor (myofibroblastic tumor) of the heart. Inflammatory pseudotumor (IPT) (also known as inflammatory myofibroblastic tumor) is an uncommon spindle cell lesion that was initially recognized in the lung and is now known to occur in virtually every major organ of the body. Its precise biological nature and clinical characteristics are still relatively uncertain. We report the case of a 7-month-old girl who was found to have an IPT of the right atrium. The rarity of IPT in the heart poses a diagnostic difficulty for both pediatricians and pathologists because of the clinical and morphologic overlap with other intracardiac masses in children. The differential diagnostic aspects of IPT of the heart are discussed and the literature on that lesion is reviewed. ( info) |
125/541. Intravascular inflammatory myofibroblastic tumors in infancy. Inflammatory myofibroblastic tumor (IMT), previously described as inflammatory pseudotumor, can occur at any age but is a recognized soft tissue tumor of childhood. Less than 10 previous cases have been described of IMT affecting the heart, in patients ranging from 5 months to 17 years of age. We present three unusual, but similar, cases of IMT in infants, which were all predominantly intravascular in location, one of which was associated with death due to angiodestructive lesions of the coronary and cerebral arteries. These cases demonstrate an apparently distinct phenotype, with a predominant intravascular location of the tumor. Furthermore, this series highlights the difficulty in categorizing such lesions as benign versus malignant on histological grounds alone. IMT should be considered in the differential diagnosis of unusual pediatric intravascular spindle cell lesions. ( info) |
126/541. Inflammatory myofibroblastic tumor (IMT) of the pancreas. Inflammatory myofibroblastic tumor (IMT) is an uncommon mass lesion composed of a variety of inflammatory and other mesenchymal cells. Although IMT was originally reported in the lung, it is now recognized that IMT can occur in a variety of organs. The occurrence of IMT in the pancreas, however, is rare. Here, we report a case of IMT arising from the head of the pancreas in a 55-year-old man. He underwent pancreaticoduodenectomy, with the diagnosis of carcinoma of the pancreas; the pathological diagnosis of the tumor was IMT. By referring to previously reported cases, we conclude that, in the management of IMT in the pancreas, surgical excision is the primary choice, in order to obtain a definitive diagnosis as well as to relieve symptoms, and strict follow-up after surgery is required. ( info) |
127/541. Identification of novel fusion partners of ALK, the anaplastic lymphoma kinase, in anaplastic large-cell lymphoma and inflammatory myofibroblastic tumor. ALK-positive anaplastic large-cell lymphoma (ALCL) has been recognized as a distinct type of lymphoma in the heterogeneous group of T/Null-ALCL. While most of the ALK-positive ALCL (ALKomas) are characterized by the presence of the NPM-ALK fusion protein, the product of the t(2;5)(p23;q35), 10-20% of ALKomas contain variant ALK fusions, including ATIC-ALK, TFG-ALK, CLTC-ALK (previously designated CLTCL-ALK), TMP3-ALK, and MSN-ALK. TMP3-ALK and TMP4-ALK fusions also have been detected in inflammatory myofibroblastic tumors (IMTs), making clear that aberrations of the ALK gene are not associated exclusively with the pathogenesis of ALK-positive ALCL. Here we report results of molecular studies on two lymphoma cases and one IMT case with variant rearrangements of ALK. Our study led to the detection of the CLTC-ALK fusion in an ALCL case and to the identification of two novel fusion partners of ALK: ALO17 (KIAA1618), a gene with unknown function, which was fused to ALK in an ALCL case with a t(2;17)(p23;q25), and CARS, encoding the cysteinyl-tRNA synthetase, which was fused to ALK in an IMT case with a t(2;11;2)(p23;p15;q31). These results confirm the recurrent involvement of ALK in IMT and further demonstrate the diversity of ALK fusion partners, with the ability to homodimerize as a common characteristic. ( info) |
128/541. Inflammatory pseudotumor of the mesentery causing portal venous thrombosis and cavernomatous transformation. A 32-year-old man was admitted to our hospital complaining of abdominal pain in the left upper quadrant. A mass was palpable on the left side of the umbilicus. Laboratory data revealed anemia, elevated erythrocyte sedimentation rate, hypergammaglobulinemia, and prolonged prothrombin time. Computed tomography demonstrated a soft tissue mass in the mesentery of the jejunum, portal venous thrombosis, and cavernomatous transformation in the porta hepatis. The patient was eventually diagnosed by laparoscopic partial resection as having inflammatory pseudotumor of the mesentery. Four months later, all of his symptoms and abnormal laboratory findings completely disappeared without any therapy. Inflammatory pseudotumor should be kept in mind as a cause of portal venous thrombosis, and/or cavernomatous transformation although it is rare. ( info) |
129/541. Hypertrophic pachymeningitis as a result of a retropharyngeal inflammatory pseudotumor: case report. OBJECTIVE AND IMPORTANCE: An extremely rare case of a patient with hypertrophic pachymeningitis that resulted from an inflammatory pseudotumor of retropharynx is described. CLINICAL PRESENTATION: A 59-year-old man with a 9-year history of retropharyngeal inflammatory pseudotumor sought care for severe headache and multiple cranial nerve palsies. magnetic resonance imaging and computed tomographic scans revealed pachymeningeal enhancement and obstructive hydrocephalus attributable to marked dural thickening around the foramen magnum. INTERVENTION: decompression of the foramen magnum, C1 laminectomy, and meningeal biopsy were performed. The histological examination of the biopsy specimen revealed chronic inflammatory infiltrate in the hypertrophic dura mater. Corticosteroid pulse therapy was subsequently completed. Clinical and neuroradiological findings improved remarkably. CONCLUSION: A new case of hypertrophic pachymeningitis as a result of a retropharyngeal inflammatory pseudotumor is presented. We review and discuss the clinical features and the pathogenic mechanisms of hypertrophic pachymeningitis. ( info) |
130/541. Inflammatory pseudotumor of the liver in a patient with rectal cancer: a case report. An inflammatory pseudotumor of the liver was discovered during a metastatic examination of a patient with rectal adenocarcinoma. magnetic resonance imaging showed a three-component lesion that infiltrated a large portal vein, and CT arteriography and CT during arterial portography showed a portal obstruction. A histopathological examination proved that necrosis was present in the central zone; infiltration by proliferating connective tissue with chronic inflammatory cells was prominent in the middle zone; and fibrous changes, including pseudo-bile duct proliferation, were present in the peripheral zone. magnetic resonance imaging revealed both portal infiltration and histopathological features including the above-noted three components. magnetic resonance imaging is perhaps the most useful diagnostic modality. ( info) |