Chondrosarcoma/surgery* ; Humans ; Neoplasms, Connective and Soft Tissue ; Soft Tissue Neoplasms

Objective. Classic high-grade osteosarcoma is uncommon in preadolescents (less than or equal to 10 years of age). The possibilities of clinicopathologic differences from the typical adolescent osteosarcoma patient have been raised. We sought to compare the presentation, treatment and survival of this subgroup of patients with published rates in order to determine if there is a need to use a treatment regimen different from that for regular adolescent osteosarcoma patients. Methods. Records of the University of the Philippines-Musculoskeletal Tumor Unit (UP-MuST) over a 15-year period (1993-2008) were reviewed and data collected on patients 10 years and younger with biopsy-proven classic high-grade intramedullary osteosarcoma who underwent complete treatment by the Unit. Demographics and survival rates were then compared with published rates for preadolescent and regular adolescent osteosarcoma cases. Results. There were fourteen patients; (6M:8F; age: 4-10 years). The most common presentation was a painful mass in the distal femur (8); the tumors most commonly had osteoblastic histology (12). Treatment consisted of neoadjuvant chemotherapy, wide surgical excision through ablation (9) or limb-saving surgery (5), and postoperative chemotherapy. There was a good histologic response (over 90% tumor necrosis) in four patients. Seven patients are ANED (alive no evidence of disease) 25 to 186 months after diagnosis. Five-year survival estimate is 52%, compared to a dismal 5 to 10% 15 years ago. Conclusion. Clinicopathologic presentation, clinical course, and overall survival in this subgroup of patients are comparable with published results for both preadolescent and adolescent osteosarcoma patients. There is no need to alter the present treatment regimen for this group of young patients.
Human ; Male ; Female ; Child ; Child Preschool ; OSTEOSARCOMA ; THERAPEUTICS ; THERAPY ; NEOPLASMS ; NEOPLASMS BY HISTOLOGIC TYPE ; NEOPLASMS, CONNECTIVE AND SOFT TISSUE ; NEOPLASMS, CONNECTIVE TISSUE ; NEOPLASMS, BONE TISSUE ;

Gastrointestinal stromal tumors(GIST) are the most common mesenchymal tumors in gastrointestinal tract. At present, surgical and molecular targeted therapies are the main treatments. Operation is properly the only way of radical resection. The general principles of surgery are complete resection of the tumor, negative margins, as well as no intraoperative tumor rupture. The choice of surgical skills for GIST is obviously affected by different locations. This paper reviews current literatures combined with our experiences, and elaborates relevant contents in detail.
Gastrointestinal Neoplasms ; Gastrointestinal Stromal Tumors ; Humans ; Molecular Targeted Therapy ; Neoplasms, Connective and Soft Tissue

Female ; Humans ; Uterine Neoplasms/pathology* ; Uterus/pathology* ; Neoplasms, Connective and Soft Tissue

The purpose of this article is to provide a current review of the spectrum of multidetector CT (MDCT) and MRI findings for a variety of cardiac neoplasms. In the diagnosis of cardiac tumors, the use of MDCT and MRI can help differentiate benign from malignant masses. Especially, the use of MDCT is advantageous in providing anatomical information and MRI is useful for tissue characterization of cardiac masses. Knowledge of the characteristic MRI findings of benign cardiac tumors or thrombi can be helpful to avoid unnecessary surgical procedures. Presurgical assessment of malignant cardiac tumors with the use of MDCT and MRI may allow determination of the resectability of tumors and planning for the reconstruction of cardiac chambers.
Contrast Media/administration & dosage ; Electrocardiography ; Heart Neoplasms/*pathology ; Hemangioma/pathology ; Humans ; Lymphoma/pathology ; *Magnetic Resonance Imaging ; Neoplasms, Connective and Soft Tissue/pathology ; Papilloma/pathology ; Tomography, X-Ray Computed/*methods

OBJECTIVETo study the clinicopathologic features, diagnosis and differential diagnosis of extraskeletal myxoid chondrosarcoma (EMC).

METHODSThe clinical and pathologic features of 7 cases of EMC encountered in Fujian Provincal Hospital and Fuzhou General Hospital of Nanjing Military Command during the period of 2005 to 2015 were analyzed. Immunohistochemical study and PAS staining were carried out. Relevant literature was reviewed.

RESULTSThe male-to-female ratio was 6 to 1. The age of patients ranged from 21 to 50 years (median = 36 years). The maximum tumor dimension ranged from 2.5 to 15.0 cm (mean = 8.4 cm). The sites of involvement included left neck, right shoulder, left thigh, right thigh, right upper arm and abdomen. Most patients presented with painless lumps. Histologically, all cases showed similar features. Low-power examination showed a nodular or lobulated architecture, with intervening fibrous septa and myxoid matrix in the background. The tumor cells were arranged in cords or tufted clusters. They were spindly to epithelioid / rhabdoid (plasmacytoid) in shape, with eosinophilic to sometimes vacuolated cytoplasm. Intracytoplasmic eosinophilic inclusion bodies and coagulative necrosis were focally seen. Mitotic figures were rare (less than 2 per 10 high-power fields). Immunohistochemical study showed that the tumor cells were positive for vimentin (7/7) and INI1 (7/7). They were focally positive for CKpan (2/7), p63 (3/7), CD99 (3/7), S-100 protein (1/7) and synaptophysin (2/7). Ki-67 proliferation index ranged from 10% to 40%. The tumor cells were negative for α-smooth muscle actin, desmin, myoD1, CD34 and CD117. The cytoplasm of the tumor cells was positive for PAS. EWSR1 gene signal was detected in 5 cases.

CONCLUSIONSEMC is a rare malignant mesenchymal tumor. Arrival at correct diagnosis relies on morphologic examination and immunohistochemistry. Molecular pathology is helpful when necessary. The primary treatment modality for EMC is complete surgical excision and the prognosis is satisfactory.

Adult ; Chondrosarcoma ; diagnosis ; pathology ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Neoplasms, Connective and Soft Tissue ; diagnosis ; pathology ; S100 Proteins ; Synaptophysin ; Vimentin ; Young Adult

OBJECTIVETo investigate the diagnostic value of liquid-based cytology test (LCT) in pancreatic lesions sampled by ultrasound-guided fine needle aspiration (EUS-FNA).

METHODSA retrospective analysis of 556 cases of LCT smears sampled by EUS-FNA of pancreatic lesions was performed, and 164 cases had histologic diagnosis with subsequent surgical resection or biopsy and immunohistochemistry. The accuracy of the cytologic diagnosis was assessed using the histologic diagnosis as the gold standard. The discrepant cases were reviewed to identify sources of errors.

RESULTSThe satisfactory rate for EUS-FNA was 96.0%(534/556). The sensitivity, specificity, positive predictive value, negative predictive value and diagnostic accuracy were 87.7%(128/146), 13/16, 97.7%(128/131), 41.9%(13/31) and 87.0%(141/162) respectively. The diagnostic accuracy was lower in cystic lesions than that in solid lesions. The LCT sensitivities of adenocarcinoma, lymphoma and neuroendocrine tumors were higher than those of cystic tumors and mesenchymal tumors. False positive diagnosis was mainly due to epithelial abnormalities in inflammatory reaction. False negative diagnosis was mainly due to scanty or lack of tumor cells in the smears, or mild atypia that was insufficient for diagnosis.

CONCLUSIONSEUS-FNA is a valuable tool for the diagnosis of pancreatic lesions. Standardized terminology and nomenclature are helpful to improve the diagnostic accuracy.

Adenocarcinoma ; diagnosis ; Endoscopic Ultrasound-Guided Fine Needle Aspiration ; Humans ; Inflammation ; Neoplasms, Connective and Soft Tissue ; diagnosis ; Neuroendocrine Tumors ; diagnosis ; Pancreas ; cytology ; diagnostic imaging ; pathology ; Pancreatic Neoplasms ; diagnosis ; Retrospective Studies ; Sensitivity and Specificity ; Specimen Handling

OBJECTIVETo evaluate the correlations between extranodal metastasis(EM)and clinicopathologic features of gastric cancer and the relationship between EM and prognosis of gastric cancer.

METHODSData of patients with histologically proven adenocarcinoma were studied retrospectively to evaluate the prognostic factors in gastric cancer by univariate and multivariate analyses of Cox regression with SPSS 13.0 software. Two hundred and seventy-six patients with primary gastric cancer undergone operation in Tianjin Cancer Hospital from Jan. 2001 to Dec. 2001 were studied and followed up until Dec. 2006 or death.

RESULTSEMs were found in 58(21.0%) of the 276 patients. The overall 2-, 3-, and 5-year survival rates of the patients without EM were 71.2%, 55.4%, and 45.1% respectively. The overall 2-, 3-, and 5-year survival rates of the patients with EM were 24.1%, 15.5%, and 8.0% respectively. Postoperative overall survival rates were significantly lower for patients with EM than those without EM(P=0.000). EM was correlated with differentiation (r=0.163, P=0.008), invasive depth (r=0.215, P=0.003), lymph node metastasis (r=0.368, P=0.000), distant metastasis (r=0.375, P=0.000), advanced stage(r=0.441, P=0.000), and tumor size (r=0.167, P=0.007). Multivariate analysis identified EM as an independent prognostic factor.

CONCLUSIONSEM is correlated with many clinicopathological features of gastric cancer. EM is an independent prognostic factor of gastric cancer.

Adult ; Aged ; Aged, 80 and over ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Neoplasm Metastasis ; Neoplasm Staging ; Neoplasms, Connective and Soft Tissue ; pathology ; secondary ; Prognosis ; Retrospective Studies ; Stomach Neoplasms ; pathology ; Survival Rate

Classification ; methods ; Dermatofibrosarcoma ; classification ; pathology ; Gastrointestinal Stromal Tumors ; immunology ; pathology ; Hemangioendothelioma ; classification ; metabolism ; pathology ; Humans ; Neoplasms, Connective and Soft Tissue ; classification ; metabolism ; pathology ; Neurilemmoma ; pathology ; Rhabdomyosarcoma ; metabolism ; pathology ; Sclerosis ; World Health Organization

Objective: To investigate the clinicopathologic features,diagnosis and prognosis of pericytic tumor of the kidney. Methods: Three cases of pericytic tumor of the kidney (two cases were diagnosed as glomangiomyomas and one case as pericytic tumor,unclassified) were collected from the affiliated Hospital of Qingdao University between January 2014 to May 2021; the clinical and morphologic features, immunohistochemical and molecular characteristics were analyzed and the relevant literature was reviewed. Results: The three patients included one male and two females, with ages ranging from 21 to 70 years. In two patients the tumors were detected incidentally at physical examination and one patient presented with low back discomfort. Imaging showed a rounded nodular soft tissue density shadow in renal parenchyma, and enhancement scan showed uneven delayed enhancement. Grossly, two tumors were located in the renal hilum and one in the renal parenchyma; all were nodular. The tumors were measured in size from 1.6 cm to 5.1 cm (mean 4.1 cm) and showed gray or gray-red cut surface. Histologic examination showed the tumor cells were arranged in solid sheets or small nodules, closely related to vascular wall. Tumor cells were mostly epithelial-like with abundant cytoplasm, light eosinophilia, obscure boundary and round nuclei with visible nucleoli. Vague bundles and fascicular arrangements of smooth muscle component were noted in some areas, with transition of both components. There was no necrosis. By immunohistochemistry, the tumor cells strongly and diffusely expressed vimentin, SMA and collagen Ⅳ, two cases expressed CD34, all three cases expressed PDGFRB to varying extent, and the Ki-67 index was 2%-3%. PCR tests showed absent K-RAS, BRAF V600E gene mutation in all three cases. PDGFRB mutations in exons 3 and 18, respectively were found in two of the three cases by high-throughput sequencing, and no NOTCH 1/2/3 gene fusions were found in any of them. Follow-up information (range: 6-92 months) showed no evidence of local recurrence or distant metastasis in all three patients. Conclusions: Pericytic tumor of the kidney is a rare mesenchymal tumor originating in the kidney with differentiation to smooth muscle, most commonly glomus tumor. The mild pleomorphism, close relationship with vascular wall and spindled smooth muscle components suggest the diagnosis of the tumor. Expression of both epithelial and muscle-associated markers aids the diagnosis. PDGFRB gene mutations may have an important role in the development of this tumor. Most patients have a good prognosis, and a few cases have malignant biological behavior.
Adult ; Aged ; Biomarkers, Tumor/analysis* ; Collagen ; Diagnosis, Differential ; Female ; Glomus Tumor/pathology* ; Humans ; Ki-67 Antigen ; Kidney/pathology* ; Kidney Neoplasms/pathology* ; Male ; Middle Aged ; Neoplasms, Connective and Soft Tissue ; Proto-Oncogene Proteins B-raf ; Receptor, Platelet-Derived Growth Factor beta ; Vimentin ; Young Adult