Primary round-cell liposarcoma of the bladder

OBJECTIVE: To report a rare case of primary round cell liposarcoma of the urinary bladder. METHODS: A case of primary round cell liposarcoma of the urinary bladder in an 80-year-old female with hematuria is presented. The patient had a large, solid, non-circumscribed bladder tumor of 6.5 cms. The radiological, histological and immunohistochemical findings are discussed. RESULTS: The neoplasm was composed of isolated lipoblasts among numerous small round cells that were positive for S-100 and vimentin, and a high proliferation rate was demonstrated by Ki-67. The patient died 10 months after the histologic diagnosis. CONCLUSION: Primary liposarcoma of the urinary bladder is a very rare tumor with a poor prognosis that usually presents as a large tumor mass.     

Management of renal angiomyolipoma: analysis of 15 cases

OBJECTIVE: We present our experience with 15 patients with renal angiomyolipoma warranting intervention. METHODS: The medical records and radiological studies were reviewed for patient age and sex, tumor location and size, association with tuberous sclerosis, and treatment approach. All patients were regularly followed by ultrasound and computed tomography scan. RESULTS: Presenting symptoms were retroperitoneal bleeding in 9 patients and flank pain in 6. Excluding cases of tuberous sclerosis (mean tumor diameter 11 cm), the mean diameter of the two tumors that bled was 5.4 cm, similar to those in the patients presenting with flank pain. Two patients with retroperitoneal bleeding had tumors < 3 cm. Angioinfarction was performed in 7 patients, partial nephrectomy in 3, and total nephrectomy in 4. One patient with tuberous sclerosis, who was observed only, died of bleeding and sepsis. The mean follow-up period of 4.3 years revealed stable creatinine levels and no recurrent hemorrhage. CONCLUSIONS: The management approach of angiomyolipoma should be aimed at parenchymal preservation which can be effectively accomplished by limited surgery or preferably by selective embolization. Preventive embolization may be feasible even for small tumors. However, any doubt about the diagnosis of angiomyolipoma should be clarified by surgery.     

A case report of angiomyolipoma arising from the renal capsule

A case of angiomyolipoma arising from the renal capsule is reported. A 49-year-old female was admitted to our hospital with a complaint of abdominal mass pointed out by ultrasonography during her yearly health check. The tumor was 10 cm in size. On abdominal enhanced CT, the tumor was existed behind the left kidney. The capsule of the tumor was enhanced and the septums were seen inside of the tumor. MRI revealed a hemorrhage in the tumor and selective renal angiography showed the tumor was fed by the renal capsular artery. A tumor of the renal capsule was suspected and a laparotomy was done. Pathological results by the frozen section suggested malignancy and radical nephrectomy was performed. Angiomyolipoma was diagnosed histologically. Tumors of the renal capsule are uncommon and angiomyolipoma of the renal capsule is extremely rare. We have found only two cases in Japanese medical literature.     

Renal angiomyolipoma as cause of massive retroperitoneal haemorrhage

A previously healthy woman was admitted to the hospital because of sudden pain in the right flank. Massive retroperitoneal haemorrhage was found to be the cause, produced by spontaneous rupture of an angiomyolipoma in the right kidney. Both the tumour and the kidney were removed. Owing to the rare occurrence of angiomyolipoma, preoperative diagnosis is seldom arrived at. If the presence of the tumour could be suspected and its benign character confirmed, the kidney itself or its intact part could often be saved.     

Small angiomyolipoma of the liver diagnosed by fine-needle aspiration biopsy under ultrasound guidance

The first reported case of small hepatic angiomyolipoma to be diagnosed by fine-needle aspiration biopsy (FNAB) is described. A 53 year old man presented with a tumour in segment VI of the liver measuring 0.9 x 0.8 cm. The tumour was hyperechoic on ultrasound examination, showed relatively low density (+ 33 Hounsfield units) on computed tomography (CT), and was hypervascular on angiography. Computed tomography during arterial portography demonstrated a perfusion defect. Magnetic resonance imaging (MRI) revealed high intensity by both T1- and T2-weighted imaging. Diagnosis could not be obtained by these imaging modalities, but it was established successfully by FNAB under ultrasound guidance. Histologically, the tumour was an angiomyolipoma made up of three components: blood vessels, smooth muscle and fatty tissue. Surgery is unnecessary for this benign condition, and the patient has been followed up. Ten months later, the patient is currently doing well without growth of the hepatic angiomyolipoma.     

Bilateral retroperitoneal liposarcoma in immunosuppressed patient with systemic lupus erythematosus

A rare case of a patient with a diffuse, infiltrating retroperitoneal myxoid liposarcoma is presented. The patient had been treated for thirteen years with corticosteroids for systemic lupus erythematosus (SLE). The increased risk of cancer in SLE and in the immunosuppressed patient is documented.     

Cardiac angiomyolipoma: radiologic and pathologic correlation

Primary tumors of the heart are rare. We report a case of large cardiac angiomyolipoma (hamartoma) that presented as a fat-containing tumor mass on imaging studies, diagnosed radiographically as teratoma. The patient was admitted through the emergency room at Tainan Municipal Hospital because of severe dyspnea. A chest radiograph revealed marked widening of the mediastinum. Echocardiography and computed tomographic scanning of the thorax showed a mass of mixed density with calcification. A teratoma with intrapericardial invasion was suspected. Sternotomy disclosed a large intrapericardial lobulated mass (34 x 30 x 12 cm, 3,150 g) arising from the right atrium, with severe adhesion to the origin of the inferior vena cava. Histopathologic examination demonstrated an angiomyolipoma of the heart. To our knowledge, this is the largest cardiac angiomyolipoma reported. We report this case to emphasize that a differential diagnosis of angiomyolipoma must be included in a patient with a fat-containing cardiac tumor.     

"Healthy Dubec" -design of a joint Czech-American community project for the reduction of cardiovascular and cerebrovascular disease (adapted from American experience)

A case of renal angiomyolipoma composed primarily of smooth muscle element is presented. It was a solitary small tumor without evidence of tuberous sclerosis. The imaging findings including ultrasonography, computerized tomography, magnetic resonance imaging, and angiography were not characteristic of angiomyolipoma, but suggestive of atypical renal cell carcinoma, and a simple nephrectomy was performed. The diagnostic considerations, particularly imaging features of angiomyolipoma, are discussed.     

Residual masses after chemotherapy for metastatic testicular cancer: the clinical implications of the association between retroperitoneal and pulmonary histology. Re-analysis of Histology in Testicular Cancer (ReHiT) Study Group

PURPOSE: We determined the need and sequence of retroperitoneal lymph node dissection and thoracotomy in patients with nonseminomatous testicular cancer, and with residual retroperitoneal and pulmonary masses after chemotherapy. MATERIALS AND METHODS: We studied 159 patients undergoing retroperitoneal lymph node dissection and a thoracotomy following cisplatin based induction chemotherapy for metastatic testicular nonseminomatous germ cell tumor. Several well-known predictors for residual histology (necrosis, mature teratoma and cancer) were evaluated. RESULTS: As expected, necrosis was found more often at retroperitoneal lymph node dissection if the primary tumor was negative for teratoma, the residual mass was small or the decrease in size was great. Contrary, neither residual mass size nor the decrease in size was predictive of the histological status of the residual lung lesion. Histological findings in the retroperitoneum and lung were strongly correlated, such that necrosis at retroperitoneal lymph node dissection was associated with an 89% probability of necrosis in the lung. CONCLUSIONS: Retroperitoneal lymph node dissection should be performed before thoracotomy is considered, since the histological status at dissection is a strong predictor of that at thoracotomy.     

Purification and partial characterization of (6-4) photoproduct DNA photolyase from Xenopus laevis

Liposarcomas of the head and neck are exceedingly rare, and fewer than 90 cases have been reported in the literature. Liposarcoma of the oral cavity is an even less common entity, and to our knowledge only nine cases have been reported to date. We report the clinical and pathologic findings of a case of well-differentiated liposarcoma of the base of tongue and tonsillar fossa. The patient is a 76-year-old white man with a long-standing history of a mass in the oral cavity and hypopharynx. The mass had been resected several times over the span of 23 years, and diagnoses of lipoma, neurofibroma, mesenchymoma, and angiofibrolipoma have been rendered on different occasions. At the last admission, a polypoid mass of the left tonsillar fossa and base of tongue was resected. The tumor was multinodular and measured 2.5 cm in greatest diameter. Histologically the tumor was ill-defined with infiltrating borders and was composed predominantly of mature adipose tissue with occasional lipoblasts. A small proportion of the tumor consisted of clusters of spindle cells and pleomorphic lipoblasts. Mitotic activity was not seen. The pleomorphic cells were positive for S100 protein and negative for muscle-specific markers. Ultrastructural analysis confirmed the nature of the lipoblasts. Our case depicts the typical natural history and histologic features of liposarcoma of the oral cavity. This tumor is usually well differentiated and has a high recurrence rate and almost no tendency for metastasis. Based on our case and review of the literature, it appears that well-differentiated liposarcoma of the oral cavity can occasionally be underdiagnosed because of the low mitotic activity and long latent period between the original diagnosis and first recurrence.     

Parasitic lumbar arterial blood supply in renal angiomyolipoma

A young woman with azotemia was found to have an angiomyolipoma which had parasitic blood supply from a lumbar artery. Parasitic lumbar arterial blood supply from a lumbar artery. Parasitic lumbar arterial blood supply has previously been considered a strong indication of malignancy. Since this benign lesion had not invaded the adjacent retroperitoneal structures, the phenomenon tends to confirm the postulate that parasitic blood supply to a lesion may occur through hypertrophy of small anastomotic channels normally present between adjacent vessels but not normally seen in angiography.     

Refractory angina despite patent coronary artery bypass grafts: treatment with transmyocardial laser revascularization and scintigraphic evidence of improved myocardial perfusion

Erdheim-Chester disease is a rare sporadic systemic histiocytic disease of unknown aetiology that affects multiple organ systems. The case records of all patients with Erdheim-Chester disease who had been seen at the Mayo Clinic between 1975 and 1996 were reviewed to assess the neurological manifestations of the disease. Two of 10 patients had neurological involvement. A 42 year old woman developed central diabetes insipidus and a progressive cerebellar syndrome. Brain MRI showed a lesion in the left pons with patchy gadolinium enhancement and T2 weighted signal abnormalities extending into both cerebellar peduncles and the medulla. Biopsy of the brainstem mass showed a xanthogranulomatous lesion. The second patient was a 53 year old man with retroperitoneal fibrosis secondary to xanthogranulomatous infiltration. Although he had no neurological symptoms and a normal neurological examination, MRI of the head showed multiple uniformly enhancing extra-axial masses along the dura of both convexities and the falx, and a mass in the left orbital apex. Both patients had the characteristic radiographic and bone scan findings of Erdheim-Chester disease. Review of the literature disclosed a wide variety of neurological manifestations in Erdheim-Chester disease. The most frequent CNS manifestations are diabetes insipidus, cerebellar syndromes, orbital lesions, and extra-axial masses involving the dura.     

Pararenal angiomyolipoma

OBJECTIVES: To review the more relevant clinical aspects of extrarenal angiomyolipoma, an uncommon disease entity. METHODS: The patient was a middle-aged female in whom an irregular right extrarenal mass had been detected. The pathological analysis disclosed a hamartoma. RESULTS: Complete resection of this lesion achieved cure. METHODS: Angiomyolipoma is a benign tumor that generally involves one or both kidneys. Extrarenal angiomyolipoma is extremely uncommon and may warrant the use of diagnostic tools distinct from the widespread algorithm and may require aggressive maneuvers to make the correct diagnosis.     

Changes in mRNA abundance of microtubule-associated proteins in the rat brain following electroconvulsive shock

STUDY DESIGN: Case report and review of the literature. OBJECTIVE: To describe a 72-year-old man with thoracic spinal angiomyolipoma in the ventral aspect of the epidural space and extracanal extension to the posterior mediastinum, to discuss the clinical and radiologic features and unique biologic behavior of this entity, and to review of the literature on angiolipoma and angiomyolipoma. SUMMARY OF BACKGROUND DATA: Spinal angiolipoma and angiomyolipoma are rare tumors, which are localized almost exclusively in the dorsal epidural space of the thoracic spine. Most reported cases have no tendency to involve the surrounding tissue. METHODS: The authors describe the radiologic, surgical, and pathologic findings of this patient and review the findings from other reported cases. RESULTS: Anterior decompression was performed using a right transthoracic incision, and the neurologic symptoms improved immediately. There were no signs of recurrence of the tumor or neurologic deficit within a 2-year follow-up period. CONCLUSION: Results of a literature survey of these tumors support management by prompt and radical surgical intervention for long-term cure, even in cases in which the infiltrating nature is recognized.     

Primary retroperitoneal liposarcoma: a case report and review of the literature

We report a case of a 43 years old woman with a retroperitoneal liposarcoma located in the left iliac fossa. The condition was discovered because of the association of left flank pain, abdominal distention and persistent urinary infection with symptoms of vesical irritation. The diagnosis was suggested by intravenous pyelography (IVP), abdominal and pelvic ultrasonography (USG) and computed tomography (CT). Treatment was exclusively surgical. During a six-month follow-up, no recurrence was observed.     

Increased gene transfer into human CD34+ progenitor cells using retroviral vectors produced by a canine packaging cell line

Magnetic resonance imaging (MRI) was used in 13 patients with peripheral lymphedema and 2 patients with extensive cavernous lymphangioma of the limb for the purpose of evaluating its role in diagnosis of lymphatic disorders. In chronic lymphedema, MRI showed deformity of lymphatics at different tissue levels. In the subcutis, MRI characteristically displayed diffuse edema or a honeycombed pattern consistent with reticular lymphangiectasis and "lakes" with a marked increase in signal intensity with T2-weighted imaging. In lymphedema hyperplasia and chylous reflux, MRI depicted dilated retroperitoneal lymphatic collectors and lumbar trunks. In cavernous lymphangiomatosis, MRI demonstrated a prominent lattice-like pattern which had lower signal intensity on T1-weighted imaging and higher intensity on T2-weighted imaging. The findings of MRI are valuable not only for accurate assessment of lymphatic dysplasia syndromes but also provide a blueprint for treatment options.     

Fluoxetine as a treatment for post-stroke emotionalism

OBJECTIVES: To review the experience at a children's hospital of lipoblastoma and liposarcoma and to identify any factors that would differentiate one type of tumour from the other. DESIGN: A retrospective case series. SETTING: British Columbia's Children's Hospital a tertiary-care pediatric centre. PATIENTS: All patients with a pathological diagnosis of lipoblastoma and liposarcoma recorded over 12 years. MAIN OUTCOME MEASURES: The frequency of lipoblastoma and liposarcoma, identified from biopsy specimens of pediatric adipose tumours. The clinical, pathological and cytogenetic variables between lipoblastoma and liposarcoma. RESULTS: One hundred and forty-nine adipose tumours were recorded. Seven (4.7%) were lipoblastomas and 2 (1.3%) were liposarcomas. All tumours presented as asymptomatic, slow-growing, soft-tissue masses. The children with lipoblastoma tended to be younger, but 29% were over 3 years of age. The liposarcoma patients were aged 9 and 14 years. One liposarcoma was of myxoid type and the other was a round cell variant. Karyotypes were reported for 1 lipoblastoma and 1 liposarcoma. The myxoid liposarcoma karyotype was 46,XY,t(12;16)(q13;p11), and the lipoblastoma was reported as 46,XY,der(8)?t(8q;?),+mar. CONCLUSIONS: Lipoblastoma is an unusual childhood neoplasm and liposarcoma is very rare in children. Both tumours may present in a similar fashion, and differentiating them histologically can be difficult. Age cannot be relied upon to accurately predict their behaviour. The tumour karyotype is very helpful in differentiating these neoplasms.     

Calcified retroperitoneal fibroma

A case of 31-year-old male with a retroperitoneal tumor is described. Abdominal ultrasound revealed a left para-aortic calcific mass, adjacent to the left lobe of the liver and to the upper pole of the left kidney. A CT-scan of the abdomen showed the mass to originate from the left adrenal gland. At operation, a large, retroperitoneal mass, adherent to the left kidney and the spleen, but not infiltrating, was excised. Histologically the tumor was diagnosed as a calcified osteo-producing fibroma. Benign retroperitoneal tumors represent about 25% of all retroperitoneal neoplasm. This reported case represents a retroperitoneal tumor of slow growth and benign clinical course whose characteristic consists of the heavy calcifications which are normally absent in a fibroma type mass.     

Residual retroperitoneal mass following chemotherapy for infantile yolk sac tumor

A child treated with chemotherapy for disseminated yolk sac tumor had a residual retroperitoneal mass after normalization of serum alpha-fetoprotein. The mass was excised and histologically showed only fibrous tissue, necrosis and calcification. To our knowledge this is the first case reported of a prepubertal yolk sac tumor associated with a residual retroperitoneal mass.     

Retroperitoneal ganglioneuroma. Report of a case

OBJECTIVE: To report an additional case of retroperitoneal ganglioneuroma. METHODS/RESULTS: A case of retroperitoneal ganglioneuroma that had been incidentally discovered in a 27-year-old male during abdominal US evaluation is described. The diagnosis was based on the histopathological findings after US-guided biopsy. The clinical features and the findings of the complementary tests, which included radiological assessment, intravenous urography, CT and cavography are presented, as well as the pathology findings. Treatment was by complete surgical excision of the tumor. CONCLUSION: Treatment of ganglioneuroma is by surgery, since the diagnosis is generally based on the histopathological analysis of the surgical specimen. For those cases with a preoperative diagnosis, some authors advocate surgery for patients with clinical evidence of neuroblastoma or another pathology arising from this disease. Although infrequent, ganglioneuroma coexisting with neuroblastoma can occur. For this reason, complete excision of the tumor is preferred.