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

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

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

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

Adult ; Calmodulin-Binding Proteins ; metabolism ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Leiomyoma ; metabolism ; pathology ; surgery ; Leiomyosarcoma ; pathology ; Spinal Canal ; Spinal Neoplasms ; metabolism ; pathology ; surgery ; Young Adult

Actins ; metabolism ; Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Breast Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Calcium-Binding Proteins ; metabolism ; Carcinoma ; metabolism ; pathology ; Cyclophosphamide ; therapeutic use ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Fibrosarcoma ; metabolism ; pathology ; Fluorouracil ; therapeutic use ; Follow-Up Studies ; Humans ; Leiomyosarcoma ; drug therapy ; metabolism ; pathology ; surgery ; Mastectomy, Segmental ; Methotrexate ; therapeutic use ; Microfilament Proteins ; metabolism ; Neurilemmoma ; metabolism ; pathology ; Phyllodes Tumor ; metabolism ; pathology ; Postoperative Period ; Vimentin ; metabolism

Antiporters ; metabolism ; Carcinosarcoma ; epidemiology ; metabolism ; pathology ; surgery ; ultrastructure ; Diagnosis, Differential ; Hemangiosarcoma ; pathology ; Humans ; Leiomyosarcoma ; pathology ; Mucin-1 ; metabolism ; Neoplasm Grading ; Neoplasm Staging ; Prognosis ; Rhabdomyosarcoma ; pathology ; Urinary Bladder Neoplasms ; epidemiology ; metabolism ; pathology ; surgery ; ultrastructure ; Vimentin ; metabolism

Actins ; metabolism ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Hemangiosarcoma ; metabolism ; pathology ; Humans ; Leiomyosarcoma ; metabolism ; pathology ; Middle Aged ; Multimodal Imaging ; Pulmonary Artery ; pathology ; Radiography ; Sarcoma ; diagnostic imaging ; metabolism ; pathology ; Soft Tissue Neoplasms ; secondary ; Vascular Neoplasms ; diagnostic imaging ; metabolism ; pathology ; Vimentin ; metabolism

OBJECTIVETo study the clinicopathologic features and differential diagnosis of perivascular epithelioid cell tumors (PEComas) occurring in the urinary system.

METHODSThe clinicopathologic features of 21 cases of PEComa from September 2002 to September 2010 occurring in the urinary system were retrospectively reviewed. Immunohistochemical study for HMB 45, S-100 protein, smooth muscle actin, desmin, Melan A and Ki-67 was carried out.

RESULTSAmongst the 21 cases studied, there were 5 males and 16 females. The age of patients ranged from 16 to 76 years (median = 51.3 years). Twenty cases occurred in the kidney and 1 in the bladder. The predominant histopathologic subtype of renal PEComas was classic type (10/20), followed by epithelioid type (5/20), smooth muscle type (3/20), inflammatory type (1/20) and sclerosing type (1/20). Immunohistochemical study showed that HMB 45 and smooth muscle actin were positive in 95.2% (20/21) and 80.9% (17/21) cases, respectively. Melan A, desmin and S-100 protein were expressed in 71.4% (15/21), 61.9% (13/21) and 33.3% (7/21) cases, respectively. The mean proliferative index was 1.29% (range = 0 to 5%). HMB 45 and Melan A were expressed in all of the 5 cases of epithelioid PEComas, whereas smooth muscle actin and desmin were only expressed in one of them. There was no significant difference between epithelioid PEComas and non-epithelioid PEComas in the expression of HMB 45, Melan A, smooth muscle actin and desmin. Positive staining for HMB 45 and smooth muscle actin was demonstrated in the case of bladder PEComa.

CONCLUSIONSPEComas of the urinary system predominantly affect the kidney. Epithelioid renal PEComas and bladder PEComa are relatively rare and have unique pathologic features. It is necessary to distinguish PEComas from other malignant tumors. Immunohistochemical study for HMB 45, Melan A and smooth muscle actin is helpful for confirmation of diagnosis.

Actins ; metabolism ; Adolescent ; Adult ; Aged ; Carcinoma, Renal Cell ; metabolism ; pathology ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Gastrointestinal Stromal Tumors ; metabolism ; pathology ; Humans ; Immunohistochemistry ; Kidney Neoplasms ; metabolism ; pathology ; Leiomyosarcoma ; metabolism ; pathology ; MART-1 Antigen ; metabolism ; Male ; Melanoma ; metabolism ; pathology ; Melanoma-Specific Antigens ; metabolism ; Middle Aged ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; S100 Proteins ; metabolism ; Urinary Bladder Neoplasms ; metabolism ; pathology ; Young Adult