Humans ; Retroperitoneal Neoplasms ; pathology ; Rhabdomyosarcoma ; pathology

Humans ; Liposarcoma/surgery* ; Retroperitoneal Neoplasms/pathology* ; STAT6 Transcription Factor

Aged ; Humans ; Liver Neoplasms ; diagnosis ; pathology ; Male ; Neurilemmoma ; diagnosis ; pathology ; Retroperitoneal Neoplasms ; diagnosis ; pathology

We report a case of lipoleiomyoma which arose in retroperitoneum and presented with progressively distended abdomen in a 56-yr-old woman. The tumor was well encapsulated and consisted of two components, benign smooth muscle cells and mature adipose tissue without any atypia. It is likely to be mistaken as extrarenal angiomyolipoma, well-differentiated liposarcoma and leiomyoma with fatty change. We review the histologic characteristics of previously reported myolipoma and describe essential points of differential diagnosis.
Angiomyolipoma/*pathology ; Diagnosis, Differential ; Female ; Human ; Leiomyoma/*pathology ; Liposarcoma/pathology ; Middle Age ; Retroperitoneal Neoplasms/*pathology

We report the clinicopathologic findings of four cases of liposarcoma with meningothelial-like whorls. Two cases occurred in the retroperitoneum and the remaining cases in the anterior mediastinum and scrotum. The whorls varied in terms of amount and morphology and the type tissue surrounding the whorls also varied in every case. One of the retroperitoneal cases with large areas of whorl coalescence recurred in the abdominal wall as an inflammatory malignant fibrous histiocytoma one year after primary resection of the tumor, and a metastasis to the cervical spines was detected twenty months later. The other retroperitoneal tumor recurred locally two years after the resection of the tumor and the amount and cellularity of the whorls as well as p53 reactivity and Ki-67 labeling index were higher in the recurrent tumor. However, coalescence of the whorls was not present in the recurrent tumor in contrast to the primary tumor. The anterior mediastinal and scrotal cases have demonstrated neither local recurrence nor distant metastasis although the follow-up period has been less than one year. The cells comprising whorls showed positive reactions for CD10, CD56, CD99, factor XIII, and low-affinity nerve growth factor receptor in addition to vimentin and alpha-smooth muscle actin. Our results indicate that liposarcoma with meningothelial-like whorls is a heterogeneous group that shows wide variations in histologic findings and biologic behavior. The phenotypic transformation of the whorls to higher grade in two retroperitoneal tumors, which showed recurrence within two years of follow up, supports that a whorl is a sign of dedifferentiation. Although we demonstrate the expressions of several markers, such as CD10, CD56, CD99, factor XIII, and low-affinity nerve growth factor receptor, in the spindle cells of the whorls for the first time, the lineage of the whorls still cannot be addressed due to the fact that these markers are lineage nonspecific.
Adult ; Genital Neoplasms, Male/*pathology ; Human ; Liposarcoma/*pathology ; Male ; Mediastinal Neoplasms/*pathology ; Mesothelioma/*pathology ; Middle Aged ; Retroperitoneal Neoplasms/*pathology ; *Scrotum

Objective: To report a rare case of epididymal liposarcoma with multiple metastases to the retroperitoneal space and peritoneal cavity for the purpose of improving the diagnosis and treatment of the disease. METHODS: We retrospectively analyzed the clinical data about a case of epididymal liposarcoma with multiple metastases to the retroperitoneal space and peritoneal cavity, reviewed relevant literature at home and abroad, and investigated the presentation, diagnosis and treatment of the disease. RESULTS: A 47-year-old male patient with epididymal liposarcoma underwent radical left orchiectomy with high ligation of the spermmatic cord. Ten years later, recurrence and metastasis were observed in the retroperitoneal space with a mass of 15.6 × 9.4 × 25.5 cm occupying an area from the upper pole of the left kidney to the pelvic cavity and enclosing the left kidney and upper ureter, for which the patient received radical resection of the retroperitoneal tumor. However, multiple retroperitoneal and intraperitoneal metastases occurred again 4 years later, which was treated by another surgical resection. The patient died of lung failure a year later. CONCLUSIONS Epididymal liposarcoma is a rare entity, which can be diagnosed by careful clinical and radiological examinations and confirmed by pathology. For its treatment, radical inguinal orchiectomy should be performed as early as possible, but the roles of adjuvant radiotherapy and chemotherapy remain controversial. Given the high rate of recurrence and metastasis, long-term follow-up is necessitated for the patient postoperatively.
Epididymis ; pathology ; Genital Neoplasms, Male ; pathology ; Humans ; Kidney ; Liposarcoma ; pathology ; secondary ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Orchiectomy ; Peritoneal Cavity ; Peritoneal Neoplasms ; secondary ; Retroperitoneal Neoplasms ; secondary ; Retroperitoneal Space

OBJECTIVETo investigate the clinicopathological characteristics and prognostic factors in patients with intra-abdomen extragastrointestinal stromal tumors (EGISTs).

METHODSThe data of 47 patients of mesenchymal neoplasms that arose from the abdominal cavity and retroperitoneum, collected from July 1987 to June 2003 in our hospital with complete clinical and pathological data, were investigated retrospectively. EGISTs were diagnosed by reviewing the tumor slides stained with hematoxylin and eosin (H&E). Immunohistochemistry staining were performed on CD117, CD34, smooth muscle actin, Desmin and S-100 proteins. The relations of various clinicopathologic characteristics and outcomes were examined.

RESULTSAmong the 47 cases, 30 tumors were confirmed to be EGISTs. Twelve cases arose from the mesentery, six from small omentum, eight from retroperitoneum and four from the abdominal cavity. The size of tumors ranged from 4 to 30 cm (median 12.5 cm) in diameter and the tumor cell components mainly included spindle cells (23 cases), epithelioid cells (4 cases), and mixed cells (3 cases). The follow-up rate was 90% and the median follow up time was 44 months. The patient survival rates at 1, 5 and 10 years were 79.7%, 59.5% and 45.4% respectively. Univariate analysis showed that tumor size >10 cm, tumor necrosis, mitoses > or =5/50HPF, obvious nuclear atypia, moderate and poor differentiated tumor cells were predictors of poor prognosis.

CONCLUSIONSEGISTs have specific clinical behaviors. The parameters used for predicting GISTs prognosis are not completely applicable for EGISTs. Tumor necrosis, obvious nuclear atypia and mitoses > or =5/50HPF help to predict aggressive behaviors in EGISTs.

Adult ; Female ; Humans ; Immunohistochemistry ; Middle Aged ; Peritoneal Neoplasms ; pathology ; Retroperitoneal Neoplasms ; pathology ; Retrospective Studies

Primary retroperitoneal mucinous cystadenocarcinoma is a rare tumor. Only about 30 such cases have been reported in the worldwide literature, and a few Korean cases have been reported. The pathogenesis is not clear, and coelomic metaplasia of the retroperitoneal mesothelium has gained wide support. There is no consensus on the appropriate treatment, but surgical exploration is needed for the diagnosis and treatment, and adjuvant chemotherapy may be recommended following complete surgical excision. The long-term prognosis has not been established. We report here on a 32-year-old woman who was diagnosed as having a retroperitoneal mucinous cystadenocarcinoma with mural nodules of sarcomatoid change. Tumor excision and adjuvant chemotherapy were done and the patient is doing well without any evidence of recurrence at 42 months postoperatively.
Adult ; Cystadenocarcinoma, Mucinous/*diagnosis/pathology/surgery ; Female ; Humans ; Retroperitoneal Neoplasms/*diagnosis/pathology/surgery

We report here on a case of solitary fibrous tumor of the retroperitoneum, and the tumor displayed a predominantly myxoid histology. A 56-year-old man presented with an incidentally detected retroperitoneal mass. On the MR images, the mass was observed as having iso-signal intensity on the T1-weighted images and high signal intensity on the fat-saturated T2-weighted images. The mass showed intense enhancement on the Gd-DTPA enhanced T1-weighted images. At surgery, a well-defined solid mass was found in the left retroperitoneum. The histological diagnosis was made as solitary fibrous tumor with a predominantly myxoid histology.
Humans ; *Magnetic Resonance Imaging ; Male ; Middle Aged ; Retroperitoneal Neoplasms/*diagnosis/pathology ; Solitary Fibrous Tumors/*diagnosis/pathology

Female ; Humans ; Liposarcoma ; pathology ; therapy ; Middle Aged ; Retroperitoneal Neoplasms ; pathology ; therapy