Biomarkers, Tumor ; genetics ; metabolism ; Humans ; Molecular Targeted Therapy ; standards ; Mutation ; Neoplasms ; drug therapy ; genetics ; metabolism ; 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 study the clinical and pathologic characteristics of small cell neuroendocrine carcinoma of urinary tract.

METHODSAll cases of urinary tract carcinoma encountered in the General Hospital of People Liberation Army during the period from 1999 to 2010 were retrospectively reviewed. The clinicopathologic data of small cell neuroendocrine carcinomas were further analyzed, with literature review.

RESULTSA total of 16 cases of small cell neuroendocrine carcinoma were identified, including 10 from urinary bladder, 2 from ureter, 3 from renal pelvis, and 1 multifocal tumor involving renal pelvis and ureter. There were altogether 8 males and 8 females. The median age of the patients was 63 years (range = 24 to 79 years). Gross hematuria (11 cases) represented the main presenting symptom. Four patients had flank pain and 4 had urinary irritation symptoms. Seven patients underwent radical cystectomy. Six other patients underwent radical nephroureterectomy, 1 partial cystectomy, 1 TURBT and the remaining case biopsy only. The size of the tumor ranged from 0.8 to 8.0 cm (median = 4.5 cm). Histologically, 15 cases represented mixed small cell neuroendocrine carcinoma (with 13 mixed with transitional cell carcinoma and 2 with adenocarcinoma). Immunohistochemical study showed positive staining for neuroendocrine markers. On presentation, 1 patient was in stage pT1, 7 in stage pT2, 6 in stage pT3, 2 in stage pT4. Six patients died of the disease after operation. The overall survival was 25 months and the 5-year survival rate was 32.4%.

CONCLUSIONSSmall cell neuroendocrine carcinoma of urinary bladder is a highly malignant disease and associated with poor prognosis. The diagnosis relies on detailed histologic examination. Early diagnosis, when coupled with cystectomy or nephroureterectomy and adjuvant chemotherapy, represents the mainstay of management.

Adult ; Aged ; CD56 Antigen ; metabolism ; Carcinoma, Neuroendocrine ; drug therapy ; metabolism ; pathology ; surgery ; Carcinoma, Small Cell ; drug therapy ; metabolism ; pathology ; surgery ; Chemotherapy, Adjuvant ; Cystectomy ; Female ; Follow-Up Studies ; Humans ; Keratins ; metabolism ; Kidney Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Neoplasm Staging ; Nephrectomy ; Phosphopyruvate Hydratase ; metabolism ; Retrospective Studies ; Survival Rate ; Synaptophysin ; metabolism ; Ureteral Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Urinary Bladder Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Urologic Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Young Adult

Adult ; Carcinoma, Embryonal ; drug therapy ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Endodermal Sinus Tumor ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Keratin-19 ; metabolism ; Ki-1 Antigen ; metabolism ; Male ; Orchiectomy ; methods ; Testicular Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; alpha-Fetoproteins ; metabolism

Skeletal metastases result in significant morbidity and mortality. This is particularly true of cancers with a strong predilection for the bone, such as breast, prostate, and lung cancers. There is currently no reliable cure for skeletal metastasis, and palliative therapy options are limited. The Wnt signaling pathway has been found to play an integral role in the process of skeletal metastasis and may be an important clinical target. Several experimental models of skeletal metastasis have been used to find new biomarkers and test new treatments. In this review, we discuss pathologic process of bone metastasis, the roles of the Wnt signaling, and the available experimental models and treatments.
Animals ; Bone Neoplasms ; drug therapy ; metabolism ; radiotherapy ; secondary ; surgery ; Breast Neoplasms ; metabolism ; pathology ; Disease Models, Animal ; Drug Delivery Systems ; Female ; Humans ; Lung Neoplasms ; metabolism ; pathology ; Male ; Mice ; Prostatic Neoplasms ; metabolism ; pathology ; Wnt Proteins ; metabolism ; Wnt Signaling Pathway ; beta Catenin ; metabolism

We describe here a female patient who presented with a breast mass and giant abdominal mass. Fine needle aspiration cytology of the breast mass and histological examination after modified radical mastectomy confirmed metaplastic carcinoma of the breast. The epithelial components were formed by infiltrating ductal carcinoma with poor differentiation, and the sarcomatous components were formed by fibrosarcoma and osteosarcoma. Histological examination of the abdominal mass confirmed ovarian teratoma. The patient underwent modified radical mastectomy of the right breast and laparoscopic excision of the abdominal mass in the lower right quadrant. Having underwent six courses of chemotherapy, the patient is now in her tenth month after surgery and under follow-up, and she has no relapsed disease. These two diseases have never seen in one patient before. The case we report here provides some new data for research and clinical experience and it may also provide a new insight into the relationship between metaplastic breast carcinoma and ovarian teratoma.
Biopsy, Fine-Needle ; Breast Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Mastectomy, Modified Radical ; Middle Aged ; Neoplasms, Multiple Primary ; drug therapy ; metabolism ; pathology ; surgery ; Ovarian Neoplasms ; drug therapy ; pathology ; surgery ; Receptor, ErbB-2 ; metabolism ; Sarcoma, Myeloid ; drug therapy ; metabolism ; pathology ; surgery ; Teratoma ; drug therapy ; pathology ; surgery ; Vimentin ; metabolism

OBJECTIVETo study the clinicopathologic characteristics of perivascular epithelioid cell tumor (PEComa), not otherwise specified (NOS) and to evaluate the diagnostic criteria for malignancy.

METHODSThe clinical and pathologic features of 31 cases of PEComa-NOS were reviewed. The follow-up data available were analyzed.

RESULTSThere were a total of 24 females and 7 males. The age of the patients ranged from 13 to 66 years (mean = 40 years). The site of tumor occurrence included gynecologic organs (n = 12), intraabdominal/peritoneal soft tissue (n = 10), gastrointestinal tract (n = 4), thigh (n = 2), mediastinum (n = 1), left groin (n = 1) and urinary bladder (n = 1). None of the cases was associated with tuberous sclerosis complex. Histologic examination showed that 23 cases (74%) were clear cell sugar tumor-like, 4 cases (13%) were clear cell myomelanocytic tumor-like and 4 cases (13%) were of mixed epithelioid-spindled morphology. According to the classification system proposed by Folpe et al, 19 cases (61%) were classified as malignant, 7 cases (23%) as PEComa of uncertain malignant potential and 5 cases (16%) as benign. The expression rates of HMB45, smooth muscle actin and desmin in tested cases were 100% (31/31), 67% (14/21) and 6/18, respectively. Follow-up data (1 to 56 months) were available in 23 cases (74%). Amongst the 16 cases of malignant PEComa, 7 patients were still alive with no evidence of disease, 6 patients were alive with unresectable or recurrent/metastatic disease and 3 patients died of the disease. The local recurrence and metastasis in those 16 cases were 6 cases and 5 cases, respectively. One of the 4 patients with PEComa of uncertain malignant potential died, while the remaining 3 patients and all of the patients with benign PEComa had an uneventful clinical course.

CONCLUSIONSThe classification system of PEComas proposed by Folpe et al. is reliable in routine practice. Correlation with the clinical and radiologic findings however is prudent when dealing with core biopsy specimens or sampling from exploration laparotomy. Owing to the histologic heterogeneity of this entity, thorough understanding of the morphologic spectrum is essential in arriving at a correct diagnosis.

Abdominal Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Actins ; metabolism ; Adolescent ; Adult ; Aged ; Desmin ; metabolism ; Female ; Follow-Up Studies ; Gastrointestinal Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Genital Neoplasms, Female ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Lymphatic Metastasis ; Male ; Melanoma-Specific Antigens ; metabolism ; Middle Aged ; Neoplasm Recurrence, Local ; Perivascular Epithelioid Cell Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Prognosis ; Young Adult

Glioma-related edema (GRE) is a significant contributor to morbidity and mortality from glioma. GRE is a complicated process involving not only peritumoral edema but also the water content of the tumor body. In terms of etiology, this condition derives from both GRE in the untreated state and GRE secondary to clinical intervention, and different cell types contribute to distinct components of GRE. Peritumoral edema was previously believed to loosen glioma tissue, facilitating tumor-cell invasion; however, the nutrition hypothesis of the tumor microecosystem suggests that tumor cells invade for the sake of nutrition. Edema is the pathologic consequence of the reconstructed trophic linkage within the tumor microecosystem. Glioma cells induce peritumoral brain edema via an active process that supplies a suitable niche for peritumoral invasive cells, suggesting that glioma-related peritumoral brain edema is determined by the invasive property of tumor cells. There are differences between pivotal molecular events and reactive molecular events in the development of GRE. Molecular therapy should target the former, as targeting reactive molecular events will produce undesired or even adverse results. At present, brain glioma angiogenesis models have not been translated into a new understanding of the features of brain images. The effect of these models on peritumoral brain edema is unclear. Clinical approaches should be transformed on the basis of new knowledge of the molecular mechanism underlying GRE. Exploring clinical assessment methods, optimizing the existing control strategy of GRE, and simultaneously developing new treatments are essential.
Brain Edema ; diagnosis ; drug therapy ; metabolism ; pathology ; Brain Neoplasms ; diagnosis ; drug therapy ; metabolism ; pathology ; Glioma ; diagnosis ; drug therapy ; metabolism ; pathology ; Humans ; Magnetic Resonance Imaging ; Molecular Targeted Therapy ; Vascular Endothelial Growth Factor A ; metabolism

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

OBJECTIVETo study the effect and mechanisms of quercetin (Qu) on proliferation and apoptosis of human methotrexate resistant osteosarcoma cell U-2OS/MTX300.

METHODMTT assay was used to observe cell proliferation. The apoptosis was examined by using Annexin V/PI staining. Western blot of mitochondrial membrane potential and cytochrome c were used to detect mitochondria spoptosis pathway. The protein expressions related to Akt pathway was detected by continuous activated Akt transient transfection and western blot.

RESULTQu can obviously inhibit the growth of human MTX resistant osteosarcoma cell U-2OS/MTX300 cells in a dose- and time-dependent manner. Annexin V/PI staining showed obvious cell apoptosis. Reduction of mitochondrial membrane potential, release of mitochondrial cytochrome c to cytosol and dephoshorylation of Akt were observed after Qu treatment.

CONCLUSIONQu can inhibit proliferation and induce apoptosis of human MTX resistant osteosarcoma cell U-2OS/MTX300, which may be related with mitochondrial apoptosis pathway and Akt activity.

Apoptosis ; drug effects ; Bone Neoplasms ; drug therapy ; pathology ; Cell Line, Tumor ; Cell Proliferation ; drug effects ; Humans ; Osteosarcoma ; drug therapy ; pathology ; Proto-Oncogene Proteins c-akt ; metabolism ; Quercetin ; pharmacology