An elderly case of idiopathic retroperitoneal fibrosis]

An elderly case of idiopathic retroperitoneal fibrosis (IRPF) with bilateral ureteral obstruction was reported. A 74-year-old man was admitted with complaints of general fatigue and loss of appetite. An elastic hard mass with a smooth surface was palpated in the left upper quadrant of the abdomen. Abnormal ESR and CRP were noticed. Abdominal ultrasonography showed both a homoechoic mass which surrounded the aorta in the retroperitoneal space and bilateral hydronephrosis. Computed tomography revealed a retroperitoneal mass involving bilateral ureters. Magnetic resonance imaging demonstrated a mass with a sharp image and signal intensity. Furthermore the morphological relationship between the mass and the ureters and major vessels clearly indicated the characteristics of benign retroperitoneal fibrosis. Based upon the above findings, a diagnosis of IRPF was made. After confirming the histological diagnosis by biopsy, treatment consisting of bilateral ureterolysis, intra-abdominal transposition of ureters and oral administration of prednisolone was performed, resulting in a normalization of laboratory findings. Analysis of 86 previously cases of IRPF reported up to 1990 in Japan, revealed the peak age to be in the 7th decade with predominance in males (males: females = 57:29).     

A case of seronegative spondylarthropathy with iritis and retroperitoneal fibrosis]

In 1985 a 41-year old male visited a local hospital because of congestion in the bulbar conjunctiva, which was diagnosed as iritis. In August 1990, right coxalgia and arthralgia of metatarsophalangeal joints appeared, with recurrence of iritis. In October, stiffness in the hands and arthralgia of proximal interphalangeal joints also started. In July 1991, the right coxalgia worsened, resulting in walking difficulty. He was admitted to the Kitasato University Hospital. He presented with bilateral iritis, polyarthritis with limited ranges of motion and sacroilitis. The Schober's test was positive at 3 cm. Serological tests for rheumatoid factor and HLA-B 27 were negative. Abdominal computer tomographic scan revealed low density lesion around the aorta. PSL 10 mg was initiated, and iritis and arthritis remitted. Progression of the periaortic lesion was not observed during the subsequent 5 years. In this case, iritis preceded limited ranges of motion in the vertebrae and sacroilitis. From these findings, seronegative spondylarthropathy with peripheral arthritis was diagnosed. The periaortic lesion seen in this case probably corresponds to chronic periaortitis recently reported as a subset of idiopathic retroperitoneal fibrosis. The two lesions observed in the present case may be interpreted as caused by inflammation of the connective tissue initially either at the vertebrae or around the aorta, which had advanced to involve the other lesion.     

A case of idiopathic sclerosing mesenteritis with retroperitoneal fibrosis

Sclerosing mesenteritis is a rare inflammatory disease of the bowel mesentery. It produces tumor-like masses of the mesentery composed of varying degrees of fibrosis, chronic inflammation, and fat necrosis. It has been described variously as fibrosing mesenteritis, retractile mesenteritis, mesenteric Weber Christian disease, and systemic nodular panniculitis. The etiology and pathogenesis of the disease are as yet unknown, but autoimmune disorder, previous abdominal surgery, trauma, and ischemia could play a role. The clinical features include abdominal pain, vomiting, diarrhea, and constipation. Occasionally, patients with this condition may present with bowel obstruction. Rarely, It can be associated with other idiopathic inflammatory disorders such as retroperitoneal fibrosis, sclerosing cholangitis, and orbital pseudotumors. We report a case of idiopathic sclerosing mesenteritis with retroperitoneal fibrosis in a 58-year-old man.     

A case of cap polyposis complicated with idiopathic retroperitoneal fibrosis

An optimal treatment for cap polyposis has not been established. Several treatment approaches, including anti-inflammatory agents, antibiotics, immunomodulators, and endoscopic therapy have been described. Surgical resection of the affected colon and rectum may be indicated for patients with persistent disease. Repeat surgery is indicated in cases of recurrence after surgery. However, symptomatic polyposis may still recur, and spontaneous resolution of cap polyposis is possible. We report a case of recurrent cap polyposis complicated with retroperitoneal fibrosis after inadequate low anterior resection with a positive resection margin. Surgical approaches for the treatment of cap polyposis should be carefully considered before treatment.     

A case of idiopathic retroperitoneal fibrosis with chronic pericarditis.

A 66-year-old male with chronic pericarditis accompanied by idiopathic retroperitoneal fibrosis was originally admitted for cardiac tamponade. A firm diagnosis was obtained only after the patient's sudden death when autopsy was performed. Chest X-rays showed cardiac enlargement and pleural effusion. Echocardiogram revealed bright echo density on the myocardium.     

A case of plasmacytoma of the ribs with intrathoracic tumors]

A 75-year-old male was admitted because of two tumors, one in the left middle lung field and one in the right upper lung field. Chest CT revealed intrathoracic tumors extending from destroyed ribs. Biopsy specimens of both tumors showed well-differentiated plasmacytoma. Retrospective investigation suggested that solitary plasmacytoma of bone (SPB) originating in the left fourth rib had developed into multiple myeloma (MM). Both tumors were treated with doses of 50 Gy irradiation and responded very well. Intrathoracic plasmacytomas have rarely been observed, so we have no established classification or therapy. According to reported cases, we classified intrathoracic plasmacytomas into 5 groups, and consider that treatment with doses of over 40 Gy irradiation was adequate for local control.     

A case of retroperitoneal schwannoma of the vagus nerve]

Schwannomas are benign nerve sheath tumors that originate from any anatomical site. Most schwannomas occur in the head, neck or limbs, but rarely occur in the retroperitoneal space. Furthermore, the schwannoma originating from the vagus nerve of retroperitoneal space is much rare. We experienced a case of retroperitoneal schwannoma of the vagus nerve. A 34-year-old male was referred to our hospital for the evaluation of abdominal mass on ultrasonography. Endoscopic examination revealed submucosal tumor-like lesion on high body of the stomach. Computed tomography (CT) revealed that the stomach was compressed by a solid tumor in the retroperitoneum. On exploratory laparotomy, this mass turned out to be a baseball sized mass in the retroperitoneal space. The mass was excised in an encapsulated state. Histological examination with immunohistochemical stains revealed a schwannoma of the vagus nerve.     

A case of intrathoracic schwannoma derived from the left vagus nerve]

We report a case of schwannoma asymptomatic for 9 years, derived from the left vagus nerve in the middle mediastinum. This spindle-shaped tumor caused paralysis of the left recurrent nerve as an initial clinical manifestation with cough. T2-weighted magnetic resonance imaging (MRI) showed a neurogenic tumor with a characteristic target appearance and with constituents of different intensities: mucinous material in the peripheral zone and solitary tissue in the central zone. But, this different intensity is not directly reflected by the histopathologic features of Antoni types A and B. These findings suggest that MRI is useful for determining the parent nerve of a neurogenic tumor and is helpful for the diagnosis.     

18F-fluorodeoxyglucose positron emission tomography in a case of retroperitoneal fibrosis

A patient with recurrent abdominal pain was admitted to our hospital. Computed tomography showed a soft dense mass surrounding the abdominal aorta at the infrarenal level, which was compatible with retroperitoneal fibrosis. (18)F-fluorodeoxyglucose ((18)F-FDG) positron emission tomography showed abnormal uptake of (18)F-FDG into these lesions. Two months after the initiation of corticosteroid therapy, the abnormal uptake of (18)F-FDG had ceased along with a reduction in the fibrous mass surrounding the abdominal aorta.