To improve the diagnosis and treatment of pulmonary lymphangiomyomatosis, clinical data for the first successfully treated case of pulmonary and retroperitoneal lymphangiomyomatosis in our hospital has been comprehensively analyzed, and the relevant literature has been reviewed. A 45-year-old Han Chinese woman initially presented six months ago with increasing shortness of breath on exertion and was admitted to our hospital after four days of chest pain. Admission examination revealed chylothorax, interstitial lung disease, and enlarged retroperitoneal lymph nodes. The patient was finally diagnosed with pulmonary and retroperitoneal lymphangiomyomatosis based on laparotomy examination and biopsy of the retroperitoneal lymph nodes. After six months of rapamycin treatment, the symptoms - lung function, arterial blood gas, and imaging of the patient- were improved significantly. Pulmonary lymphangiomyomatosis clinically manifests as progressive dyspnea, recurrent pneumothorax, and chylothorax, and can be diagnosed by its characteristic features in high-resolution computed tomographic images or pathological examination. The successful treatment of pulmonary lymphangiomyomatosis with rapamycin brings new hope to those afflicted with this disease.
Female ; Humans ; Lung Neoplasms ; complications ; drug therapy ; Lymphangioleiomyomatosis ; drug therapy ; Middle Aged ; Retroperitoneal Neoplasms ; complications ; drug therapy ; Sirolimus ; therapeutic use

To develop a more appropriate therapeutic strategy for treatment of nonpulmonary visceral metastatic testicular seminoma based on the International Germ Cell Consensus Classification, we reviewed the medical records of patients with nonpulmonary visceral metastatic testicular seminoma who were treated over a 20-year period. Only 15 (2.2%) of the 686 cases of testicular seminoma were nonpulmonary visceral metastatic seminoma. The median age of patients was 38 years (range, 22-53 years). Ten (67%) of the patients had an initial diagnosis of supradiaphragmatic or visceral metastatic disease. In addition to nonpulmonary visceral metastasis, all patients had lymph node metastasis as well, the majority of which involved the retroperitoneal lymph nodes. The median and mean progression-free survival durations after chemotherapy for advanced disease were 19 months and 63.7 months, respectively. Six patients (40%) survived, five relapsed after radiation therapy and four died of chemorefractory disease not dependent on the specific regimen. Although the number of cases reviewed in this study was small, we conclude that the choice of chemotherapeutic regimen among the current treatments for nonpulmonary visceral metastatic seminoma of testis primary does not present a different outcome. Therefore, multimodality therapies using new strategies or new agents are well indicated.
Adult ; Antineoplastic Agents, Combined/administration & dosage* ; Bone Neoplasms/secondary ; Bone Neoplasms/radiotherapy ; Bone Neoplasms/drug therapy ; Combined Modality Therapy ; Human ; Lung Neoplasms/secondary ; Lung Neoplasms/radiotherapy ; Lung Neoplasms/drug therapy ; Lymphatic Metastasis ; Male ; Middle Age ; Retroperitoneal Neoplasms/secondary* ; Retroperitoneal Neoplasms/radiotherapy ; Retroperitoneal Neoplasms/drug therapy* ; Retrospective Studies ; Seminoma/secondary* ; Seminoma/radiotherapy ; Seminoma/drug therapy* ; Testicular Neoplasms/pathology*

Wilms' tumor is the most common malignant abdominal tumor in the adolescent and pediatric period, whereas adult Wilms' tumor is uncommon. Wilms' tumor often originates in the kidney. Extrarenal Wilms' tumor is rare and has been considered "unstageable". Therefore, treatment and long-term survival have not been uniformly reported. We report a 24 year-old female who developed extrarenal Wilms' tumor, in the retroperitoneal space. She was misdiagnosed for ovarian neoplasm and underwent debulking operation, by which Wilms' tumor was confirmed. Palliative chemotherapy and radiotherapy were performed.
Adolescent ; Adult* ; Drug Therapy ; Female ; Humans ; Kidney ; Ovarian Neoplasms ; Radiotherapy ; Retroperitoneal Space ; Wilms Tumor* ; Young Adult

The morphological features of a retroperitoneal teratoma in a 10-month-old girl are reported. Unlike the usual pattern of the teratoma, this tumor was composed predominantly of nephroblastomatous tissue. Histologically, glomeruloid and tubular structures were identified in nests of undifferentiated blastemal elements. Hyaline cartilage, adipose tissue, glial tissue and glands lined by mucin-secreting columnar epithelium were minor elements. A focal cystic structure lined by thin flattened epithelium was also noted. Retroperitoneal teratoma with predominance of nephroblastic elements is of interest not only because of its rarity but also because it needs to be differentiated from extrarenal Wilms' tumor, since both of these tumors have different origins.
Diagnosis, Differential ; Female ; Humans ; Infant, Newborn ; Kidney Neoplasms/*pathology ; Magnetic Resonance Imaging ; Retroperitoneal Neoplasms/drug therapy/*pathology/surgery ; Teratoma/drug therapy/*pathology/surgery ; Wilms Tumor/*pathology

The incidence of retroperitoneal tumor is low, and treatment is difficult.According to the recent updates of evidence-based medical evidence at home and abroad, the consensus on the standardized treatment of retroperitoneal tumors were discussed including examination and diagnosis , surgical treatment comprehensive treatment, nutrition, rehabilitation, and review and follow-up, etc.
Antineoplastic Agents ; administration & dosage ; adverse effects ; therapeutic use ; China ; Consensus ; Delivery of Health Care ; standards ; Humans ; Practice Guidelines as Topic ; Retroperitoneal Neoplasms ; diagnosis ; drug therapy ; pathology

If a testicular cancer patient has a mass in the retroperitoneum, a metastasis is often the first suspicion, probably leading to improper diagnosis and overtreatment. Here we report a case of retroperitoneal schwannoma mimicking metastatic seminoma. A 29-year-old man, who had a history of seminoma, presented with a single retroperitoneal mass suspected to be a metastasis. Because the patient refused radiotherapy, 3 cycles of cisplatin, etoposide, and bleomycin were offered. Post-chemotherapy computed tomography scan revealed persistence of the retroperitoneal mass, with no change in tumor size or characteristics. Subsequently, retroperitoneal lymph node dissection was performed. The dissected tissue contained negative lymph nodes but a single mass in the attached fat. Pathology revealed retroperitoneal schwannoma, which was confirmed by immunohistochemistry. Thus, clinicians should be aware of retroperitoneal schwannoma and its distinction from metastatic seminoma to avoid misdiagnosis and ensure proper treatment.
Adult ; Antibiotics, Antineoplastic ; therapeutic use ; Antineoplastic Agents ; therapeutic use ; Antineoplastic Agents, Phytogenic ; therapeutic use ; Bleomycin ; therapeutic use ; Cisplatin ; therapeutic use ; Diagnostic Errors ; Etoposide ; therapeutic use ; Humans ; Lymph Node Excision ; Lymph Nodes ; pathology ; Lymphatic Metastasis ; Male ; Neoplasms, Multiple Primary ; Neurilemmoma ; diagnostic imaging ; drug therapy ; pathology ; Radiography ; Retroperitoneal Neoplasms ; diagnostic imaging ; drug therapy ; pathology ; secondary ; Retroperitoneal Space ; Seminoma ; secondary ; surgery ; Testicular Neoplasms ; surgery

INTRODUCTIONBladder cancer is a common malignancy but presentation with metastatic disease is rare. This is the fi rst reported case of duodenal obstruction as a presentation of metastatic bladder cancer.

CLINICAL PICTUREA middle-aged woman presented with nausea, vomiting, weight loss and intermittent haematuria. Radiology and histology confirmed metastatic bladder cancer to the retroperitoneum encasing the duodenum and causing obstruction.

TREATMENTInsertion of a duodenal stent relieved the obstruction and palliative chemoradiotherapy was initiated.

OUTCOMEThe patient died 15 months after diagnosis.

CONCLUSIONSClinicians and radiologists should be aware of atypical presentations of common malignancies.

Adult ; Carcinoma, Transitional Cell ; drug therapy ; secondary ; Diagnosis, Differential ; Duodenal Obstruction ; diagnosis ; etiology ; surgery ; Fatal Outcome ; Female ; Humans ; Palliative Care ; Retroperitoneal Neoplasms ; complications ; diagnosis ; secondary ; Stents ; Urinary Bladder Neoplasms ; drug therapy ; pathology

OBJECTIVETo study the clinicopathologic features and biologic behavior of pediatric immature teratoma.

METHODSThe clinical data, pathologic features, immunohistochemical findings (for cyclin D1, P27 and Ki-67) and follow-up information of 39 cases of pediatric immature teratoma were analyzed.

RESULTSAmongst the 39 cases studied, 12 arose in the sacrococcygeal region, 12 in testis, 5 in retroperitoneum, 4 in ovary, 4 in abdomen and 2 in mediastinum. Histologically, 16 cases were of grade 1, 8 cases of grade 2 and 15 cases of grade 3. Seven of the cases contained foci of yolk sac tumor. Immature neuroepithelial features used in histologic grading included the presence of primitive neural tubules, immature rosettes, undifferentiated neuroblastoma cells and primitive neuroectodermal structures. Immunohistochemical study showed that cyclin D1 was positive in 3 cases of grade 1 tumors, 4 cases of grade 2 tumors and 9 cases of grade 3 tumors. The positivity rates for p27 were 8, 3 and 6 cases respectively, while those for Ki-67 were 3, 4 and 13 cases respectively. Follow-up data were available in 30 cases. Three of them, including 2 cases with histologic grade 3 (with or without yolk sac tumor component), recurred after operation.

CONCLUSIONSThe expression of cyclin D1 and Ki-67 is a useful adjunct in histologic grading. On the other hand, p27 overexpression shows little correlation with tumor grade. The prognosis of immature teratoma in children is different from that in adults. Sacrococcygeal immature teratoma occurring in patients younger than 1 year old and with low histologic grade do not require postoperative chemotherapy if the tumor is completely excised. Similarly, for testicular immature teratoma occurring in patients below 1 year of age, regardless of tumor grading, need no adjunctive therapy. On the other hand, ovarian immature teratoma with high histologic grade requires postoperative chemotherapy, regardless of age of the patients. The presence of microscopic foci of yolk sac tumor is a useful predictor of recurrence in pediatric immature teratoma.

Adolescent ; Cyclin D1 ; metabolism ; Endodermal Sinus Tumor ; drug therapy ; metabolism ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Ki-67 Antigen ; metabolism ; Male ; Mediastinal Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Ovarian Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Proliferating Cell Nuclear Antigen ; metabolism ; Retroperitoneal Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Sacrococcygeal Region ; Survival Rate ; Teratoma ; drug therapy ; metabolism ; pathology ; surgery ; Testicular Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; alpha-Fetoproteins ; metabolism

OBJECTIVETo analyze the retroperitoneal lymph node metastasis in epithelial cancer of the ovary and offer scientific indications for lymph node radical dissection.

METHODSFifty-eight patients with ovarian cancer treated from January 1990 to December 2000 were retrospectively reviewed. Single-factor and multifactor analysis with Logistic regression model were performed by SPSS 10.0 statistic software.

RESULTSThe metastasis rates of overall lymph nodes, pelvic nodes and para-aortic nodes were 48.3%, 37.9% and 25.9% respectively, among which no significant difference was noted (P > 0.05). Single-factor analysis showed that tumor location, ascitic condition, clinical stage and the size of residual tumor were associated with retroperitoneal lymph node metastasis. Multifactor analysis revealed that clinical stage and size of residual tumor were independent risk factors for metastasis of retroperitoneal lymph nodes.

CONCLUSIONFor early ovarian cancer patients, it is extremely important to perform radical dissection of the retroperitoneal lymph nodes. For advanced or residual lesions, radical dissection of pelvic nodes and para-aortic nodes could be considered in the second exploration.

Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; administration & dosage ; therapeutic use ; Cisplatin ; administration & dosage ; Cyclophosphamide ; administration & dosage ; Cystadenocarcinoma, Mucinous ; drug therapy ; secondary ; surgery ; Cystadenocarcinoma, Serous ; drug therapy ; secondary ; surgery ; Doxorubicin ; administration & dosage ; analogs & derivatives ; Female ; Humans ; Lymph Node Excision ; Lymph Nodes ; pathology ; Lymphatic Metastasis ; Middle Aged ; Ovarian Neoplasms ; drug therapy ; pathology ; surgery ; Retroperitoneal Space ; Retrospective Studies ; Second-Look Surgery

OBJECTIVETo study the clinicopathologic features of leiomyosarcoma with prominent osteoclast-like giant cells.

METHODSThe clinical and pathologic features of 7 cases of leiomyosarcoma with prominent osteoclast-like giant cells were analyzed. Immunohistochemical and ultrastructural studies were performed. The literature was reviewed.

RESULTSAll cases occurred in adults, with a mean age of 63 years. There was no significant sex predilection (male-to-female ratio = 4:3). The tumor involved subcutaneous soft tissue of thigh (number = 2), left back (number = 1), retroperitoneum (number = 1), small intestine (number = 1), breast (number = 1) and uterus (number = 1). Histologic examination showed that the tumor was composed of relatively uniform spindly cells arranged in interlacing fascicles. The hallmark was the presence of prominent osteoclast-like giant cells, either intimately admixed with the spindly cells (number = 6) or forming giant cell tumor-like nodules (number = 1). Immunohistochemically, the spindly cells expressed smooth muscle actin, muscle-specific actin, desmin and h-caldesmon in various degrees, whereas the osteoclast-like giant cells expressed CD68. Ultrastructural study showed smooth muscle differentiation in the spindly cells and histiocytic differentiation in the osteoclast-like giant cells. Follow-up data were available in 6 cases. There were local recurrences and/or metastases in all the 6 patients. Three patients were alive with unresectable or recurrent/metastatic disease and two patients died of the disease.

CONCLUSIONSLeiomyosarcoma with prominent osteoclast-like giant cells is a rare variant of leiomyosarcoma which should be distinguished from the so-called giant cell variant of malignant fibrous histiocytoma. The osteoclast-like giant cells are of histiocytic differentiation. Surgical resection remains the mainstay of management of this high-grade sarcoma.

Actins ; metabolism ; Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Back ; Breast Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Calmodulin-Binding Proteins ; metabolism ; Desmin ; metabolism ; Female ; Follow-Up Studies ; Giant Cells ; metabolism ; pathology ; Humans ; Immunohistochemistry ; Intestinal Neoplasms ; metabolism ; pathology ; surgery ; Leiomyosarcoma ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Osteoclasts ; metabolism ; pathology ; Retroperitoneal Neoplasms ; metabolism ; pathology ; surgery ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Thigh ; Uterine Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Vimentin ; metabolism