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

Glomus Tumor ; diagnosis ; etiology ; Humans ; Neoplasms, Connective Tissue ; complications ; Osteomalacia

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

Tumour-induced osteomalacia (TIO) is a rare paraneoplastic syndrome, which is characterized by overproduction of FGF23 as a phosphaturic agent leading to chronic phosphaturia and hypophosphatemia, associated with inappropriately normal or low levels of vitamin D. We describe a rare case of a 57-year-old Indian female who presented with bone pains, muscle pains and lower limb weakness. On examination she was found to have hypophosphatemia. Our work up led to the identification of a FGF23 secreting parotid tumour. The tumour responsible for symptoms was a pleomorphic adenoma of the parotid gland. Its complete resection resulted in normalisation of patient's symptoms. Laboratory parameters and microsopic examination further revealed a mesenchymal tumour of mixed connective tissue type.

Human ; Female ; Middle Aged ; Adenoma, Pleomorphic ; Connective Tissue ; Hypophosphatemia ; Hypophosphatemia, Familial ; Lower Extremity ; Neoplasms, Connective Tissue ; Paraneoplastic Syndromes ; Parotid Gland ; Parotid Neoplasms ; Vitamin D ; Hypophosphatemia

Oncogenic osteomalacia is a rare cause that makes abnormalities of bone metabolism. Our case arose in a 47-year-old woman presenting a nasal mass associated with osteomalacia. We excised the mass carefully. After surgery, it was diagnosed as hemangiopericytoma and her symptoms related with osteomalacia were relieved and biochemical abnormalities were restored to normal range. We report and review a rare case of nasal hemangiopericytoma that caused osteomalacia.
Female ; Hemangiopericytoma ; Humans ; Middle Aged ; Neoplasms, Connective Tissue ; Osteomalacia ; Reference Values

Aggressive angiomyxoma (AAM) is a rare, benign tumor. It usually involves the connective tissue of the perineal regions in women of reproductive age. In this report, we present a case of AAM in a 66-year-old female, which presented itself as a retrovesical tumor on pelvic magnetic resonance imaging and caused lower urinary tract symptoms. The tumor was resected en bloc and the patient's voiding symptoms disappeared.
Aged ; Connective Tissue ; Female ; Humans ; Lower Urinary Tract Symptoms ; Magnetic Resonance Imaging ; Myxoma ; Pelvic Neoplasms

Rathke's cleft cysts are believed to be derived from remnants of Rathke's pouch, a dorsal invagination of the stomodeum. Although these cysts are characteristically small, asymptomatic and intrasellar in location, they occasionally provoke symtoms with enlargement to compress surrounding structures. It is characteristically lined by stratified squamous epithelium with keratinization on a layer of connective tissue. The cells of the anterior pituitary lobe, from which pituitary adenomas develop, are also formed by the proliferation of the anterior wall of Rathke's pouch. Thus, Rathke's cleft cyst and pituitary adenomas are considered to have a common ancestry. We report a rare case in which the preoperative diagnosis was pituitary adenoma, but the pathologic diagnosis was a combination of a Rathke's cleft cyst and a coincidental pituitary adenoma.
Adenoma* ; Central Nervous System Cysts ; Connective Tissue ; Diagnosis ; Epithelium ; Pituitary Neoplasms*

OBJECTIVETo investigate the distribution and change of the causes of fever of unknown origin(FUO).

METHODSThe clinical data of 500 inpatients with FUO in our center between December 2003 and June 2014 were retrospectively analyzed. The diagnostic methods,etiologies,and their possible relationship with age,sex,fever duration,and period.

RESULTSOf these 500 FUO patients,452(90.4%)were confirmed to be with fever caused by conditions including infectious diseases [(n=231,46.2%;e.g.tuberculosis(32.9%,76/231)],connective tissue diseases(CTD)(n=99,19.8%),neoplasms(n=58,11.6%),miscellaneous causes(n=64,12.8%). The causes were not identified in 48 cases(9.6%).The proportion of CTD in female patients was significantly higher than that in male patients(26.3% vs. 14.5%,P=0.025),whereas the proportion of neoplasms in male patients was significantly higher than that in female patients(14.5% vs. 8.0%,P=0.001). Infectious diseases was the most common cause in all age groups,CTD ranked the second in the 21-39-year group and 40-59-year group,and neoplasm was the second most coomon cause in the over 60 year group. Thus,the distribution of FUO etiologies significantly differed in different age groups(χ(2)=43.10,P=0.000). The duration of fever in patients with neoplasms [60(28,90)d] was longer than that in patients with infectious diseases [28(21,42)d,Z=-4.168,P=0.000] or CTD [30(21,60)d,Z=-2.406,P=0.016)]. Compared with the level in 2003-2008,the proportion of CTD significantly increased in 2009-2014(13.7% vs. 23.8%,χ(2)=8.598,P=0.003),along with the dicrease of the proportions of infectious diseases,neoplasms and miscellaneous diseases were decreased(all P>0.05).

CONCLUSIONSInfectious diseases(in particular,tuberculosis)remains the major cause of FUO. CTD and neoplasms also play important roles in the development of FUO. The distributions of the FUO etiologies have certain differences in terms of age,sex,duration of fever,and period.

Connective Tissue Diseases ; Female ; Fever of Unknown Origin ; Humans ; Male ; Neoplasms ; Retrospective Studies ; Tuberculosis