Spontaneously ruptured multilocular cystic nephroma

Multilocular cystic nephroma is a relatively rare benign tumor of the kidney. An adult case of spontaneously ruptured multilocular cystic nephroma was reported. A 33-year-old woman who presented with a right flank colic attack. The preoperative diagnosis was spontaneously ruptured renal tumor. A right nephrectomy was performed. A multilocular cystic lesion, 10 cm in diameter, was found in the removed specimen, which had a hematoma inside and direct tumor extension into the renal pelvis. On microscopic examination, the surface of loculi was covered by a layer of hobnail epithelium, and septa were composed of fibrous tissue with dilated vessels, lacking in normal renal components. There were no signs of malignancy. The lesion extending into the renal pelvis showed the same microscopic findings. The final diagnosis was multilocular cystic nephroma. Two respects, spontaneous rupture and tumor extension into the renal pelvis, were unique to the present case. There has been no report of spontaneously ruptured mutilocular cystic nephroma. The tumor extension into the renal pelvis, however, has been found in several reports. This growth pattern might be one of the characteristics of this benign renal tumor.     

Dedifferentiated Liposarcoma of the Pleura: Report of a Case

We report a case of dedifferentiated liposarcoma of the pleura in a 59-year-old man who presented with pain in the right chest wall, 3 years after a routine chest X-ray showed a large tumorous mass. The resected tumor was composed of three distinct histological features: well-differentiated liposarcoma with low-grade leiom-yosarcomatous and low-grade osteosarcomatous components. To our knowledge, this is the first report of dedifferentiated liposarcoma of the pleura.     

A case of retroperitoneal neurinoma

Retroperitoneal neurinoma is a rare disease, although retroperitoneal tumor is rather common. A 40-year-old man without any clinical symptoms was admitted to our hospital for a medical examination. Ultrasonogram demonstrated a cystic mass over the right kidney. This cystic mass was suggested to be a right adrenal tumor by CT scan and angiogram. All of the laboratory data including endocrinological data were within the normal limits. Under the diagnosis of right non-functioning adrenal tumor, an operation was performed through a right lumbal incision. A round and tennis ball sized tumor with a fibrous capsule was identified and freed from the right kidney and the right adrenal gland, and the tumor was extirpated uneventfully. The surgical specimen was 9.5 X 8.0 X 6.5 cm in size, 260 g in weight, and the histological diagnosis was neurinoma (Antoni-B type).     

A case of retroperitoneal dedifferentiated liposarcoma initially diagnosed as malignant fibrous histiocytoma: a case report

We report a case of retroperitoneal tumor which turned out to be liposarcoma by the histological evaluation of its recurrent tumor, although the initial tumor was diagnosed as malignant fibrous histiocytoma (MFH). A retroperitoneal tumor in a 62-year-old man was removed and pathologically diagnosed as MFH. Five years after the initial surgery, computed tomography (CT) demonstrated a recurrent tumor near the spleen. The tumor was resected together with the spleen, tail of pancreas, and connective tissue due to adhesion and diagnosed as well-differentiated liposarcoma with sclerosing component. Generally dedifferentiated liposarcoma is difficult to distinguish from MFH and the presence of a well-differentiated liposarcoma component in the adjacent adipose tissue leads to the diagnosis of dedifferentiated liposarcoma. The clinical course of the present case indicated that the initial tumor was dedifferentiated liposarcoma and the recurrent tumor developed from the surrounding well-differentiated liposarcoma.     

Malignant fibrous histiocytoma originating in a renal capsule: a case report]

We report a case of malignant fibrous histiocytoma originating in a renal capsule. A 43-year-old woman was admitted with a chief complaint of right lower abdominal pain. Physical examination was unremarkable. Serum C-reactive protein and erythrocyte sedimentation rate increased to 3.8 mg/dl and 60 mm/hr., respectively. Computed tomography (CT) and magnetic resonance image (MRI) showed a heterogeneous enhanced mass, 4 x 9 x 13 cm in size, in contact with the lateral part of the right kidney. Selective right renal arteriography revealed a hypovascular tumor, the main feeding artery of which was the right adrenal artery. Preoperative clinical diagnosis was a retroperitoneal sarcoma and transabdominal tumor resection was performed. The adhesion between the tumor and the right kidney was so severe that right nephrectomy was also necessary for a radical surgery. The tumor, measuring 13 x 9 x 6 cm, was located laterally adhering to the right renal capsule. Microscopic examination of the tumor demonstrated spindle-shaped fibroblast-like cells arranged in a storiform pattern with fibrous stroma and clusters of rounded histiocyte-like cells and pleomorphic giant cells with bizarre nuclei. Histopathological diagnosis was malignant fibrous histiocytoma arising from the renal capsule and there was no tumor invasion to renal parenchyma. No adjuvant therapy was performed but she has remained well for 31 months since the operation without evidence of disease.     

The retroperitoneal liposarcoma: a case report

A 62-year-old woman was admitted to our hospital because of a mass in the right abdomen. Ultrasonography, aortography and computed tomographic (CT) scanning revealed the right kidney displaced by a huge and avascular mass containing fatty tissue. Also magnetic resonance imaging (MRI) indicated that it was contained of lipomatous tissue and demonstrated the mass occupying the entire right abdominal cavity. The tumor including the right kidney was resected, following diagnosis of the retroperitoneal tumor. The exercised tumor measured 28 X 22 X 18 cm and weighed 2,010g. The histological diagnosis was well-differentiated liposarcoma. She has been healthy for three months following postoperative chemotherapy. Many reports have been made on the ultrasonographic and CT appearance of retroperitoneal liposarcoma, but few have been made on the MRI appearance. According to our case, imaging diagnosis by MRI also seems to be useful in making a histological diagnosis of liposarcoma.     

多房性囊性肾癌一例报告及文献复习

目的 提高多房性囊性肾癌的诊治水平.方法多房性囊性肾癌患者1例,男,49岁.体检B超偶然发现右肾下极多囊性肿物1个月.囊内无回声区,其间混杂低回声区;CT检查右肾下极见多囊性低密度病变,边界清楚、光整,内见薄壁分隔,增强扫描分隔可见强化;MRI检查示右肾下极多囊性病变,增强扫描囊壁可见强化.行右肾部分切除术,完整切除肿瘤.结果 病理报告:肾被膜下见多房状肿物,大小约3.0 cm×2.0 cm×2.0 cm,囊壁光滑,腔内含清亮的浆液性及血性液体,囊壁厚约0.1～0.2 cm,与肾周脂肪粘连.镜下多数囊腔内衬单层或复层立方状透明细胞,细胞核小而圆,位于细胞中央,无明显核仁;有的囊腔内衬扁平上皮细胞或无内衬上皮,偶见由透明细胞覆盖的小乳头;囊腔间隔由胶原纤维组成,部分间隔内可见灶状透明细胞,但未形成肉眼可见的结节.病理诊断:多房性囊性肾癌.术后随访20个月未见复发和转移.结论 多房性囊性肾癌是肾癌的一种罕见亚型,发病率低,是一种完全由囊腔构成的肿瘤;影像学检查可提供直接依据,确诊需依靠病理学检查;外科手术治疗预后良好.     

A case of dedifferentiated liposarcoma of the spermatic cord

We report a case of dedifferentiated liposarcoma of the spermatic cord. A 79-year-old man was referred to our hospital with the chief complaint of a painless right scrotal swelling. A hard mass was palpable beside the soft mass which was suspected of lipoma or liposarcoma, and under this mass there was right testis. Therefore, we performed a right radical orciectomy. Histopathological diagnosis was dedifferentiated liposarcoma of the spermatic cord. Dedifferentiated liposarcoma of the spermatic cord was rare, and preoperative diagnosis was difficult.     

Malignant fibrous histiocytoma arising from the renal capsule: report of a case

Malignant fibrous histiocytoma (MFH) is now considered the most common soft tissue sarcoma in adults, but MFH arising from the renal capsule is very rare. A 77-year-old woman was admitted with a painful mass in the left flank region on September 10, 1986. Preoperative diagnosis was hypovascular retroperitoneal tumor in contact with the upper pole of the right kidney. The tumor was removed together with the right kidney on December 18, 1986, and the specimen weighted 640 gm. Histological examination revealed storiform-pleomorphic malignant fibrous histiocytoma. No postoperative adjuvant therapy was carried out. Local recurrence of the disease was found about 9 months after the operation, and the patient died on February 23, 1988. Fifteen cases with MFH arising from the kidney reported in the Japanese literatures are reviewed, and the diagnosis, treatment, prognosis and tumor marker are discussed.     

Primary intracerebral malignant fibrous histiocytoma: case report

We report a rare case of a primary intracerebral malignant fibrous histiocytoma (MFH) with malignant progression from a premalignant stage. A 73-year-old male presented with mild hemiparesis on the right side of his body 4 months prior to admission. MR images showed a homogenously enhanced mass in the left parietal lobe. The patient underwent an uneventful extirpation of the solid mass. Microscopically, the solid tumor contained histiocytic cells and spindle-shaped fibroblastic cells arranged in a storiform pattern. The tumor cells were immunoreactive for vimentin, MAC 387 (macrophage marker) and alpha-1-antitrypsin. The MIB-1-positive rate was 1%. The histological diagnosis was fibrous histiocytoma. Postoperative MR images demonstrated no abnormally enhanced lesions. The patient's neurological symptoms and signs gradually improved. One year after surgery, he developed right hemiparesis. MR images showed a heterogeneously enhanced mass in the left sensorimotor area. This recurrent tumor was totally resected. Microscopically, the tumor was composed of atypical spindle-shaped cells, forming a storiform pattern. Pleomorphism was prominent, and necrotic foci were also seen. The MIB-1 labeling index averaged 11%. The histological findings corresponded to those of MFH. Unfortunately, the patient died of interstitial pneumonia contracted during postoperative radiotherapy. Early detection and resection at a stage of low malignant potential, followed by radiochemotherapy, are crucial for the management of this mesenchymal tumor.     

Liposarcoma arising from the renal capsule

Liposarcoma is one of the most common soft tissue sarcomas, but liposarcoma arising from the renal capsule is rare. We report a case of liposarcoma arising from the renal capsule. Preoperative diagnosis was achieved by computerized tomography and and selective renal angiography. The tumor resected with he left kidney which weighed 4,370 g. Postoperative adjuvant chemotherapy with cyclophosphamide, bleomycin, actinomycin D, adriamycin and vinblastine was carried out. Although the patient's general conditions was fine, local recurrence occurred after 24 months postoperatively. Five cases of liposarcoma arising from the renal capsule including our case have been reported in Japan.     

Asymptomatic dedifferentiated liposarcoma mimicking renal cell carcinoma—A rare case report and review of literature

Introduction

Soft tissue sarcomas are rare and account for one percent of all cancers globally. Approximately 15% of these tumours are found in the retroperitoneum and have a peak incidence at around 40 to 60 years of age.

Malignant fibrous histiocytoma of the perirenal tissue: report of a case--a statistical study of 58 cases of urological malignant fibrous histiocytoma in Japanese literature

A 78-year-old man was admitted in June 18, 1982 with a two-year history of general fatigue and loss of appetite. Physical examination revealed a child's head sized, firm, not tender, right upper quadrant mass which had an almost smooth surface and had respiratory displacement. Preoperative diagnosis was a hypovascular renal tumor presenting at the lower part of the right kidney. Right nephrectomy was performed on July 6, which displayed a specimen 1,300 g and 17 X 12 X 10 cm. The light yellow tumor appeared between the renal parenchyma and large fatty masses. The tumor was histologically diagnosed as storiform-pleomorphic malignant fibrous histiocytoma (MFH) and disclosed infiltration of both the fibrous renal capsule and adjacent perirenal fatty tissue. There was no invasion of the tumor into the renal parenchyma and the case was considered to arise from the fibrous renal capsule or the perirenal tissue. Although he had been treated with ifosfamide and adriamycin three times after operation and with immunotherapy of 3 g of PSK per day for about five months, he died three years and one month after operation. We reviewed 58 cases of MFH arising from the retroperitoneum and genitourinary tract (urological MFH) in the Japanese literature.     

Nephron-sparing radical excision of a giant perirenal liposarcoma involving a solitary kidney

We describe our experience in a patient with a congenital solitary kidney encased by a perirenal liposarcoma managed by nephron-sparing excision. The best predictor of survival with liposarcoma is complete resection of the tumor. Generally a diffuse peri-renal liposarcoma arising within Gerota's fascia would necessitate a radical nephrectomy. Having a congenitally solitary kidney, this patient refused nephrectomy, therefore a kidney-sparing excision of his liposarcoma was attempted. To obtain negative margins and to provide a nephron-sparing excision, the capsule of the kidney was resected with the mass. Direct extension into the diaphragm necessitated an en bloc resection of 4 x 6 cm of the left hemidiaphragm. The defect was reconstructed with a Gortex patch graft. Pathology revealed a 32 x 22 x 8-cm liposarcoma with areas of sclerosing liposarcoma and poorly differentiated spindle cell sarcoma, focally Grade 3 of 3, with the remaining tumor being Grade 1. There was diaphragmatic invasion, but all surgical margins were negative. At 22 months CT follow-up, the patient has no radiographic evidence of disease. Excision of this mass with the renal capsule allowed our patient to be margin negative and maintain normal renal function. This is the only report in the literature describing nephron-sparing resection of a giant perirenal liposarcoma involving a solitary kidney.     

Infantile multilocular renal cyst.

A case of a Japanese 14-month-old male infant with multilocular renal cyst is presented. He was admitted with a chief complaint of an abdominal mass. Although clinically and radiographically multilocular renal cyst in the right kidney was suspected, total nephrectomy was performed because of extreme difficulty of partial nephrectomy and impossibility of complete neglect of malignant cystic tumor. Microscopically, immature glomeruloid tissue, primitive tubules, rhabdomyocytes and nerve fibers were noted. The histological diagnosis of multilocular renal cyst with partially differentiated dysplasia was made. Since the first report by Edmunds in 1892, the etiology of multilocular renal cyst has been discussed for years. In our present case, the pathologic findings suggest that multilocular cystic formation in the right kidney might be the result of developmental disturbances.     

A case of malignant fibrous histiocytoma arising from the renal capsule

A 62-year-old man was admitted with a chief complaint of general malaise. Computed tomography showed a large mass adjacent to the parenchyma of the left kidney. The mass was 17 x 13 x 12 cm in size. Preoperative diagnosis was left renal cell carcinoma and left radical nephrectomy was performed. Histopathologically, the tumor was diagnosed as malignant fibrous histiocytoma (MFH), and the tumor was considered to have arisen from the renal capsule. There has been no recurrence for 7 months postoperatively. We review 40 cases of MFH arising from the kidney or the renal capsule in the literature.     

A case of metanephric adenoma treated with partial nephrectomy

A 32-year-old female presented with a right renal mass detected by abdominal ultrasound examination at a routine medical check up. Computed tomography scans and magnetic resonance images revealed a solid tumor in the upper pole of the right kidney measuring 1.5 cm in diameter. Partial nephrectomy was performed on suspicion of the renal cell carcinoma. Histologically, the tumor is composed of small, uniform, epithelial cells with scant cytoplasm and hyperchromatic round nuclei that formed a tubular or glomerular-like structure. The histological diagnosis was metanephric adenoma.     

Retroperitoneal tumors: a case of liposarcoma

The Authors report a case of retroperitoneal sarcoma, in the histological subtype "liposarcoma", occasionally diagnosed in a 57 year old woman, who presented aspecific symptoms, characterized by dry cough, vomiting and chest pain. Chest TC showed a neoplasm of the retroperitoneum, close to liver, right colon, duodenum and right kidney. The arteriography of the right renal artery showed a neovascolarization inside the mass. A surgical resection was performed. The histological examination confirmed the diagnosis of liposarcoma. Liposarcomas account for 30% of the retroperitoneal soft tissue sarcomas; symptoms and diagnosis are late and the surgical resection of primary neoplasm, metastases and local recurrences representing the only therapeutic choice.     

A case of renal malacoplakia that was difficult to distinguish from cystic renal cell carcinoma

A 66 years old man visited our hospital with a chief complaint of right flank pain and abdominal palpable mass. Abdominal enhanced computed tomography scan showed a large cystic mass with infiltrating surrounding tissue in the right kidney. We diagnosed it infected cystic renal cell carcinoma because of high grade fever and acute inflammatory response. It was surgically unresectable tumor. Therefore, percutaneous puncture and drainage was performed under ultrasound guidance. In the culture of drainage fluid, Escherichia coli (E. coli) was isolated. Then, we confirmed reduction in the size of the tumor, right radical nephrectomy was performed. Because of tumor invasion to ascending colon, we performed the excision of right kidney together with ascending colon. Histological study confirmed target-shaped Michaelis-Gutmann body, and the definitive diagnosis was malacoplakia of the kidney. We reported a case of renal malacoplakia that was difficult to distinguish from cystic renal cell carcinoma. Our case is 12th case of renal malacoplakia in the Japanese literature.     

A case of retroperitoneal venous aneurysm]

A case of retroperitoneal venous aneurysm is reported. A 73-year-old woman was referred to us with the chief complaint of left abdominal mass. A giant abdominal mass was palpable and diagnostic imaging examination including ultrasound tomography, excretory pyelography, computed tomography, magnetic resonance imaging and angiography revealed a giant cystic mass encircled by calcification in the left retroperitoneal space. Operation for this cystic mass was performed under the preoperative diagnosis of a giant left renal cyst. During operation the mass was located between the left kidney and the left adrenal gland. Because it was difficult to separate the mass from the left kidney the mass was removed with the left kidney. The extirpated tumor measured 15.5 x 15.0 x 9.5 cm and contained old blood clots and red-yellow colored fluid. A histological examination revealed that the tumor wall was composed of smooth muscle and elastic fibers. Therefore, pathological diagnosis was retroperitoneal venous aneurysm. Retroperitoneal venous aneurysm is very rare. To our knowledge, this is the 8th case of retroperitoneal venous aneurysm reported in Japan.