Leiomyosarcoma of urinary bladder (LMS-UB) is a highly malignant mesenchymal tumor, accounting for less than 0.5% of all bladder malignancies, with a predominant clinical presentation of hematuria. Here we report a case of low-grade LMS-UB. A 44-year-old male patient was admitted to the hospital with urodynia for 2 weeks. The patient's pelvis CT showed a mass on the right part of the bladder. For this reason, he was initially diagnosed with bladder cancer. We performed a robot-assisted laparoscopic enucleation of the bladder tumor and low-grade LMS-UB was diagnosed with the histopathological examination. He underwent 5 cycles of adjuvant chemotherapy after surgery. At 19months postoperative follow-up, the patient had no symptoms, recurrence, or distant metastasis. There is no report on the treatment of LMS-UB with minimally invasive enucleation worldwide. This case provides a new comprehensive treatment method of enucleation combined with adjuvant chemotherapy for early low-grade LMS-UB to reduce complications and improve patients' quality of life after surgery.
Male ; Humans ; Adult ; Urinary Bladder/surgery* ; Leiomyosarcoma/secondary* ; Robotics ; Quality of Life ; Pelvis/pathology* ; Urinary Bladder Neoplasms/pathology* ; Laparoscopy/methods*

Leiomyosarcoma(LMS)of the great saphenous vein(GSV)is a rare condition that is often misdiagnosed due to the lack of typical clinical manifestations.This article reported a GSV-LMS case diagnosed and treated in Peking Union Medical College Hospital.Meta-analysis of the current case and 41 cases derived from CNKI,WANFANG DATA,and MEDLINE database was also conducted,which revealed that the male to female ratio of GSV-LMS was 11:10;the median age of disease onset was 59.5 years;the most commonly affected region was thigh.Due to lack of specific clinical features,42.9% of GSV-LMS patients were misdiagnosed at presentation.Radical resection is the most effective therapy,and the indications,effectiveness and protocols of adjuvant radiochemotherapy remain unclear.The 3-and 5-year overall survival rate was 86.1% and 77.5%,respectively.Recurrence and metastasis occurred in 31.0% of patients after surgery.
Female ; Humans ; Leiomyosarcoma ; pathology ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Saphenous Vein ; pathology ; Survival Rate

BACKGROUND: To investigate the clinicopathological characteristics of urinary bladder tumors, a rare malignancy, in patients 20 years or younger. METHODS: Using a retrospective chart review among patients who received bladder surgery at 2 institutions between July 1996 and January 2013, we analyzed the clinicopathological characteristics of urinary bladder tumors in 21 pediatric patients (male:female = 4.25:1.00; mean age, 12.1 years). RESULTS: Pathology revealed 9 urothelial tumors, 6 rhabdomyosarcomas, 1 low-grade leiomyosarcoma, 1 large cell neuroendocrine carcinoma, 1 inflammatory myofibroblastic tumor, and 3 cases of chronic inflammation without tumors (including 1 xanthogranulomatous inflammation). Urothelial tumors (mean patient age, 16.0 years) were benign or low-grade; and only transurethral resection of the bladder tumor was necessary for treatment. Patients with rhabdomyosarcomas (mean age, 5 years) underwent radiotherapy (if unresectable) or transurethral resection of the bladder tumor (if resectable), after chemotherapy. Of these patients, 2 underwent radical cystectomy, with the remaining patients not receiving a cystectomy. With the exception of one patient, all patients are currently alive and recurrence-free. CONCLUSION: Urothelial tumors were the most commonly found pediatric bladder tumor, with embryonal rhabdomyosarcoma being the second most common. Urothelial tumors are common in relatively older age. Since urothelial tumors in children typically have a good prognosis and rarely recur, transurethral resection of the bladder tumor is the treatment of choice. Rhabdomyosarcomas are common in younger patients. Since rhabdomyosarcoma is generally chemosensitive, chemotherapy and radiotherapy are the treatment of choice for bladder preservation in these patients.
Carcinoma, Neuroendocrine ; Child ; Cystectomy ; Drug Therapy ; Humans ; Inflammation ; Leiomyosarcoma ; Myofibroblasts ; Pathology ; Prognosis ; Radiotherapy ; Retrospective Studies ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Embryonal ; Urinary Bladder Neoplasms ; Urinary Bladder*

Malignant kidney neoplasms are the most frequently encountered solid kidney masses. Although renal cell carcinoma is the major renal malignancy, other solid malignant renal masses should be considered in the differential diagnosis of solid renal masses that do not contain a macroscopic fatty component. In this pictorial essay, we present the imaging findings of a primitive neuroectodermal tumor, primary liposarcoma of the kidney, primary neuroendocrine tumor, leiomyosarcoma, synovial sarcoma, malignant fibrous histiocytoma, sclerosing fibrosarcoma and renal metastasis of osteosarcoma.
Bone Neoplasms/secondary ; Carcinoma, Renal Cell/pathology/radiography ; Diagnosis, Differential ; Fibrosarcoma/radiography ; Histiocytoma/radiography ; Humans ; Kidney Neoplasms/*pathology/radiography ; Leiomyosarcoma/pathology/radiography ; Magnetic Resonance Imaging ; Middle Aged ; Neuroectodermal Tumors, Primitive/pathology/radiography ; Osteosarcoma/pathology ; Sarcoma ; Sarcoma, Synovial/radiography ; Tomography, X-Ray Computed

Leiomyosarcoma is an uncommon tumor that originates from various organs, including uterus, kidney, retroperitoneum, and soft tissues. In particular, leiomyosarcoma of the stomach is extremely rare. Only 9 cases have been reported worldwide since the discovery of KIT-activating mutation. A 48-year-old woman was admitted to our hospital with abdominal discomfort and generalized weakness. Upon detection of multiple nodules in both lung on chest posterior-anterior radiograph taken at the time of admission, chest CT was performed and it revealed multiple mass lesions in the lung, liver, and pancreas along with multiple lymph node metastases. On endoscopic examination, a 2.0 cm sized ulcerofungating mass lesion was found on the stomach body. Biopsy was performed and the mass lesion proved to be leiomyosarcoma confirmed by immunohistochemical staining. Chemotherapy was thus initiated, but the patient died after one year due to tumor progression. Our experience suggests that leiomyosarcoma can manifest aggressive behavior in its early stage. Herein, we report a case of gastric leiomyosarcoma with multiple metastases along with review of relevant literature.
Female ; Gastroscopy ; Humans ; Leiomyosarcoma/*diagnosis/pathology ; Liver Neoplasms/pathology/secondary ; Lung Neoplasms/pathology/secondary ; Lymphatic Metastasis ; Middle Aged ; Pancreatic Neoplasms/pathology/secondary ; Stomach Neoplasms/*diagnosis/pathology ; Tomography, X-Ray Computed

OBJECTIVETo study the treatment strategy and survival of patients with primary leiomyosarcoma of inferior vena cava (PIVCLMS).

METHODSClinical data of 12 cases with PIVCLMS admitted in Peking University People's Hospital from January 2006 to September 2014 were reviewed retrospectively. All cases were confirmed by pathology examination. Among them, there were 4 male and 8 female patients with a mean age of (54 ± 9) years old. Tumors arose from the inferior vena cava (IVC) upper segment in 5 patients, from the middle in other 7 patients. Cardiac extension was observed in 4 cases. Tumor resection was undertaken in 8 patients, the other 4 patients were inoperable. The series was analyzed to identify clinical outcome of surgical strategy and protective factors for patient survival.

RESULTSIn tumor resection group, 6 patients had radical resection and 2 underwent palliative resection. As for IVC reconstruction, caval wall resection with a direct suture was carried out in 6 patients or with prosthetic patch in 1 patient. The other 1 patient underwent a segment caval resection and prosthetic graft replacement in situ. In 4 cases of suprahepatic PIVCLMS cardiopulmonary bypass or perfusion by right atrial intubation was performed to assist bleeding control and maintain circulation stabilization, among them 1 patient survived for more than 101 months with no tumor recurrence or metastasis. Among the patients submitted to tumor resection 2 early postoperative deaths occurred, and another 2 patients had complications. All 4 patients submitted to non-resective operation (only neoplasm biopsy) died of PIVCLMS within 8 months. Except for 2 cases of early death, mean survival after tumor resection was (54 ± 40) months. Two patients presented local recurrence and hepatic metastasis at follow-up of 16 months and 68 months.

CONCLUSIONSTumor resection is the only therapy for PIVCLMS with an expectation for long-term survival. The applicant of cardiopulmonary bypass makes some inoperable indicated to tumor resection.

Adult ; Blood Vessel Prosthesis Implantation ; Cardiopulmonary Bypass ; Female ; Humans ; Leiomyosarcoma ; diagnosis ; surgery ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Retrospective Studies ; Vascular Neoplasms ; diagnosis ; surgery ; Vena Cava, Inferior ; pathology

PURPOSE: The aim of this study was to compare survival of patients with uterine sarcomas using the 1988 and 2008 International Federation of Gynecologists and Obstetricians (FIGO) staging systems to determine if revised 2008 staging accurately predicts patient survival. MATERIALS AND METHODS: A total of 83 patients with leiomyosarcoma and endometrial stromal sarcoma treated at Yonsei University Health System between March of 1989 and November of 2009 were reviewed. The prognostic validity of both FIGO staging systems, as well as other factors was analyzed. RESULTS: Leiomyosarcoma and endometrial stromal sarcoma comprised 47.0% and 53.0% of this study population, respectively. Using the new staging system, 43 (67.2%) of 64 eligible patients were reclassified. Among those 64 patients, 45 (70.3%) patients with limited uterine corpus involvement were divided into stage IA (n=14) and IB (n=31). Univariate analysis demonstrated a significant difference between stages I and II and the other stages in both staging systems (p<0.001) with respect to progression-free survival and overall survival (OS). Age, menopausal status, tumor size, and cell type were significantly associated with OS (p=0.011, p=0.031, p=0.044, p=0.009, respectively). In multivariate analysis, revised FIGO stage greater than III was an independent poor prognostic factor with a hazard ratio of 9.06 [95% confidence interval (CI) 2.49-33.0, p=0.001]. CONCLUSION: The 2008 FIGO staging system is more valid than the previous FIGO staging system for uterine sarcomas with respect to its ability to distinguish early-stage patients from advanced-stage patients.
Adult ; Disease-Free Survival ; Female ; Humans ; Leiomyosarcoma/mortality/pathology ; Male ; Middle Aged ; Neoplasm Staging ; Prognosis ; Uterine Neoplasms/*mortality/*pathology

Actins ; metabolism ; Calcium-Binding Proteins ; metabolism ; Calmodulin-Binding Proteins ; metabolism ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Hysterectomy ; Leiomyoma, Epithelioid ; metabolism ; pathology ; Leiomyomatosis ; metabolism ; pathology ; surgery ; Leiomyosarcoma ; pathology ; Microfilament Proteins ; metabolism ; Middle Aged ; Receptors, Estrogen ; metabolism ; Receptors, Progesterone ; metabolism ; Rhabdoid Tumor ; metabolism ; pathology ; surgery ; Sarcoma, Endometrial Stromal ; metabolism ; pathology ; Uterine Neoplasms ; metabolism ; pathology ; surgery ; Vascular Neoplasms ; metabolism ; pathology ; surgery ; Veins ; pathology ; Vimentin ; metabolism

Actins ; metabolism ; Adrenal Gland Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Leiomyosarcoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Middle Aged ; Neoplasm Recurrence, Local ; surgery ; Reoperation ; Tomography, X-Ray Computed ; Vimentin ; metabolism

OBJECTIVETo investigate the predictors of local recurrence and survival in primary retroperitoneal leiomyosarcoma (PRLS).

METHODSA retrospective analysis was conducted on 46 cases of PRLS between August 2006 and April 2012. There were 9 male and 37 female patients, and they were 30-79 year old (median 49 year old). Kaplan-Meier estimations and Cox regression analyses were performed.

RESULTSThe tumor size were 5-36 cm, and 29 cases (63.1%) of this group was more than 10 cm. Local 3- and 5-year control rates after complete resection were 47% and 30%. FNCLCC (Fédération Nationale des Centres de Lutte Contre le Cancer) grading (RR = 3.10, 95%CI: 1.59-6.04), gender (RR = 5.30, 95%CI: 1.64-17.13) and blood loss (RR = 3.12, 95%CI: 1.28-7.60) were independent prognostic factor of local recurrence in multivariable analysis (P < 0.05). The 5-year disease-free survival rates after complete resection of PRLS was 26%; and the 5-year disease-specific survival (DSS) rates after complete resection was 48%. The median DSS was 60 months in negative margin of resection and 50 months in positive margin. However, by multivariate analysis, only FNCLCC grading was significant independent predictors of DSS (RR = 2.01, 95%CI: 1.16-3.49, P = 0.013).

CONCLUSIONSGender, FNCLCC grading and blood loss were independent prognostic factor of local recurrence. The disease-specific survival in PRLS varied significantly according to FNCLCC grading.

Adult ; Aged ; Female ; Follow-Up Studies ; Humans ; Kaplan-Meier Estimate ; Leiomyosarcoma ; pathology ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; pathology ; Prognosis ; Retroperitoneal Neoplasms ; pathology ; Retrospective Studies ; Survival Rate