OBJECTIVE: To summarize and analyze the clinical characteristics of children with basal ganglia germinoma and to improve the level of early clinical diagnosis. METHODS: The clinical data of children diagnosed with basal ganglia germinoma admitted to the Pediatric Surgery Ward of Peking University First Hospital from January 2013 to December 2020 were retrospectively analyzed, and descriptive statistics were used to analyze the clinical characteristics of children with basal ganglia germinoma. RESULTS: A total of 30 patients were included in the study, 28 were male, 2 were female, the mean age at onset was (9.7±2.2) years, the median disease duration was 7 months, 27 had unilateral disease, and 3 had bilateral disease. The clinical manifestations were decreased limb muscle strength, cognitive function disorders, polydipsia, precocious puberty, intracranial hypertension, dysphonia and swallowing dysfunction. The serum and cerebrospinal fluid tumor marker alpha-fetoprotein (AFP) were normal in the 30 patients, and the serum and cerebrospinal fluid tumor marker β-human chorionic gonadotropin (β-HCG) were normal in 8 patients.The serum β-HCG was normal in 11 patients but the cerebrospinal fluid β-HCG was slightly elevated, and the serum and cerebrospinal fluid β-HCG were slightly elevated in 11 patients. A total of 33 lesions with irregular shapes were found by imaging examination, including 15 (45.5%) patchy lesions, 10 (30.3%) patchy lesions, and 8 (24.2%) round-like high-density lesions. Tumors showed obvious high-density shadows on computed tomography (CT) scan. Magnetic resonance imaging (MRI) scan of the tumors showed low or isointensity on T1WI and isointensity on T2WI, accompanied by mild peritumoral edema, hemispheric atrophy, cerebral peduncle atrophy, calcification, cystic degeneration, ventricular dilatation and wallerian degeneration. On contrast-enhanced scans, the tumor showed no enhancement or heterogeneous enhancement. CONCLUSION The main age of onset of germ cell tumors in the basal ganglia in children is about 10 years old, and males are absolutely dominant. The clinical features and imaging manifestations have certain characteristics. With both combined, the early diagnosis of germ cell tumors in the basal ganglia can be improved.
Atrophy/pathology* ; Basal Ganglia/pathology* ; Biomarkers, Tumor ; Brain Neoplasms/diagnostic imaging* ; Child ; Chorionic Gonadotropin, beta Subunit, Human ; Female ; Germinoma/pathology* ; Humans ; Magnetic Resonance Imaging ; Male ; Neoplasms, Germ Cell and Embryonal ; Retrospective Studies

Objective: To investigate immunohistochemical patterns of CXorf67 and H3K27me3 proteins in central nervous system germ cell tumors (GCTs) and to assess their values in both diagnosis and differential diagnosis. Methods: A total of 370 cases of central nervous system GCTs were collected from 2013 to 2020 at Huashan Hospital of Fudan University, Shanghai, China. The expression of CXorf67, H3K27me3 and commonly-used GCT markers including OCT4, PLAP, CD117, D2-40, and CD30 by immunohistochemistry (EnVision method) was examined in different subtypes of central nervous system GCTs. The sensitivity and specificity of each marker were compared by contingency table and area under receiver operating characteristic (ROC) curve. Results: Of the 370 cases there were 282 males and 88 females with a mean age of 19 years and a median age of 17 years (range, 2-57 years). Among the GCTs with germinoma, the proportions of male patients and the patients with GCT located in sellar region were both higher than those of GCTs without germinoma (P<0.05), respectively. CXorf67 was present in the nuclei of germinoma and normal germ cells, but not in other subtypes of GCT. H3K27me3 was negative in germinoma, but positive in the nuclei of surrounding normal cells and GCTs other than germinoma. In the 283 GCTs with germinoma components, the expression rate of CXorf67 was 90.5% (256/283), but no cases were positive for H3K27me3. There was also an inverse correlation between them (r²=-0.831, P<0.01). The expression rates of PLAP, OCT4, CD117 and D2-40 were 81.2% (231/283), 89.4% (253/283), 73.9% (209/283) and 88.3% (250/283), respectively. In 63 mixed GCTs with germinoma components, the expression rate of CXorf67 was 84.1% (53/63), while all cases were negative for H3K27me3. The expression rates of PLAP, OCT4, CD117 and D2-40 were 79.4% (50/63), 79.4% (50/63), 66.7% (42/63) and 87.3% (55/63), respectively. The 6 markers with largest area under ROC curve in ranking order were H3K27me3, CXorf67, D2-40, OCT4, PLAP and CD117 (P<0.05). Conclusions: CXorf67 and H3K27me3 have high sensitivity and high specificity in diagnosing germinoma. There is a significant inverse correlation between them. Therefore, they can both be used as new specific immunohistochemical markers for the diagnosis of GCTs.
Adolescent ; Adult ; Brain Neoplasms/pathology* ; Central Nervous System/pathology* ; Central Nervous System Neoplasms/metabolism* ; Child ; Child, Preschool ; China ; Female ; Germinoma/pathology* ; Histones ; Humans ; Male ; Middle Aged ; Neoplasms, Germ Cell and Embryonal/diagnosis* ; Oncogene Proteins ; Transcription Factors/metabolism* ; Young Adult

OBJECTIVETo examine the recent incidences and trends of childhood malignant solid tumors in Shanghai.

METHODData from the population-based Shanghai Cancer Registry and related retrospective survey were used to analyze the patterns of incidence and trends of malignant solid tumors diagnosed between 2002 and 2010 in children aged 0-14 years. The distributions of incidences were described according to gender, age and cancer types which were classified according to International Classification of Childhood Cancer (ICCC). Annual age-standardized rates (ASRs) were adjusted by the world standard population. Approximate confidence intervals for standardized rate ratios (SRR) based Poisson distribution test-based methods were used to assess changes in incidence over the period 2002 - 2006 and 2007 - 2010.

RESULT(1)A total of 868 cases of childhood malignant solid tumors were diagnosed in Shanghai during 2002 - 2010, accounting for 65.8% of all childhood cancers. The ASR of 2002 - 2010 was 80.2 per million for all solid tumors. (2) The ASR was higher in boys (86.3 per million) than in girls (73.8 per million) with SRR 1.2 (95%CI 1.0 - 1.3). Incidence rate was the highest in the first five years of life with 93.4 per million. The age-specific incidence rates in 5 - 9 and 10 - 14 age groups were 65.2 and 79.3 per million, respectively. (3) CNS tumors, lymphomas, germ cell tumors, neuroblastoma, and soft tissue sarcomas were the top 5 most common solid tumors in children, with the incidence rate of 23.8, 11.0, 7.8, 7.7 and 6.8 per million, respectively. The patterns of subgroups varied in different age groups. Blastomas, such as neuroblastoma, retinoblastoma, were more common in the children aged 0 - 4 years, whereas epithelial carcinomas and bone tumors developed more frequently in elder children aged 10 - 14 years. (4) Compared with the ASR in 2002 - 2006, the ASR for both genders in 2007 - 2010 had no substantial changes (78.7 per million in 2002 - 2006 and 82.9 per million in 2007 - 2010). However, among boys, the incidence rate in 2007 - 2010 was significantly higher than that in 2002 - 2006 with SRR 1.2 (95%CI: 1.0 - 1.4). For specific subgroups of cancer, there were no substantial changes. Some cautions should be taken when interpreting results involving a small number of cases per year and those with wide 95% confidence intervals.

CONCLUSIONThe incidence rate of pediatric malignant solid tumors among males was higher than females during 2002 - 2010, and it differed among different age groups with the highest in the first five years of life. CNS tumor was the most common type of solid tumors in children. This was a unique characteristics comparing with adult reflected in disease spectrum and age of onset. The patterns of incidence and its trends for childhood malignant solid tumors in Shanghai could provide a basis for etiologic research and preventive interventions. The findings also suggest an urgent need for longer population-based surveillance to verify the pattern and changing trends.

Adolescent ; Age Distribution ; Central Nervous System Neoplasms ; epidemiology ; pathology ; Child ; Child, Preschool ; China ; epidemiology ; Female ; Germinoma ; epidemiology ; pathology ; Humans ; Incidence ; Infant ; Lymphoma ; epidemiology ; pathology ; Male ; Neoplasm Staging ; Neoplasms ; classification ; epidemiology ; pathology ; Registries ; Risk Factors ; Sex Distribution ; Time Factors ; Urban Population

Testicular microlithiasis (TM) refers to the calcium deposits within the seminiferous tubules. Owing to the wide use of scrotal ultrasonography, more cases of TM have been diagnosed clinically. TM can be associated with many conditions, including cryptorchidism, infertility, varicocele, testicular torsion, Klinefelter's syndrome, intratubular germ cell neoplasia (IGCN) and testicular germ cell tumor (TGCT), and its frequent association with IGCN, TGCT and infertility is the particular concern of urologists and andrologists. The etiology of TM is not yet certain. Its biological meaning and clinical significance have not been well defined. Currently, there are controversies on the correlation of TM with IGCN, TGCT and infertility. This article reviews the definition, prevalence, etiology, and histopathology of TM, and summarized its association with IGCN, TGCT, and male infertility as well as the management of the disease.
Calculi ; diagnostic imaging ; Germinoma ; diagnostic imaging ; Humans ; Incidence ; Male ; Scrotum ; diagnostic imaging ; Testicular Diseases ; diagnosis ; epidemiology ; pathology ; Testicular Neoplasms ; diagnostic imaging ; Ultrasonography

OBJECTIVETo study the clinicopathologic features, immunohistochemical findings and prognosis of primary diffuse large B-cell lymphoma (DLBCL) of testis.

METHODSFourteen cases of primary DLBCL of testis, diagnosed according to the 2001 World Health Organization staging standards for hematopoietic and lymphoid tumors, were retrospectively studied. Immunohistochemical study was performed and follow-up information analyzed.

RESULTSThe median age of the patients was 62 years. There were 10 patients in stage I, 3 in stage II and 1 in stage IV. Follow-up information was available in 11 patients (78.6%). Three of which were still alive and eight died (with duration of survival ranging from 5 to 19 months). The patients usually presented with unilateral painless enlargement of testis. Histologically, the lymphoma cells of all cases showed a centroblastic appearance. One case belonged to the germinal center B cell-like subtype on immunohistochemical study, while the remaining 13 cases were classified as non-germinal center B cell-like subtype. Ten of the 14 cases (71.4%) showed overexpression of p53 protein. Most cases demonstrated high proliferation index. Six of the 14 cases (42.9%) were positive for bcl-2 protein. The overall 1-year, 2-year and 5-year survival rates were 45.5%, 17.0% and 17.0%, respectively.

CONCLUSIONSMost cases with primary DLBCL of testis were of peripheral activated B-cell origin. The prognosis is usually not favorable, with propensity of local relapse and systemic dissemination. Accurate pathologic diagnosis relies on detailed histologic examination and immunohistochemical study.

Adult ; Aged ; Aged, 80 and over ; Antigens, CD20 ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Follow-Up Studies ; Gene Expression Regulation, Neoplastic ; Germinoma ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Lymphoma, Large B-Cell, Diffuse ; drug therapy ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Neprilysin ; metabolism ; Orchiectomy ; Prednisone ; therapeutic use ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Retrospective Studies ; Seminoma ; pathology ; Survival Rate ; Testicular Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Tumor Suppressor Protein p53 ; metabolism ; Vincristine ; therapeutic use

OBJECTIVETo study the immunohistochemical expression of OCT4, CD117 and CD30 in germ cell tumors and to assess their diagnostic value.

METHODSImmunohistochemical study for OCT4 was performed on formalin-fixed, paraffin-embedded tissues of 63 cases of germ cell tumors, including seminoma (21), dysgerminoma (7), germinoma (8), embryonal carcinoma (8), yolk sac tumor (6), mature teratoma (10) and immature teratoma (3), as well as 25 cases of non-germ cell tumors, including granulosa cell tumor (8), clear cell adenocarcinoma (4), Leydig's cell tumor (5), diffuse large B-cell lymphoma (4) and malignant melanoma (4). Besides, the expression of CD117 and CD30 in all germ cell tumors was studied.

RESULTSAll cases of seminoma and germinoma, 6/7 cases of dysgerminoma and 7/8 cases of embryonal carcinoma were positive for OCT4, with strong nuclear staining. All other germ cell tumors and non-germ cell tumors were negative for OCT4, except for 1 case of yolk sac tumor and 1 case of clear cell adenocarcinoma which showed weak staining. Positive membranous expression of CD117 was demonstrated in 19/21(90.5%) seminoma, 5/7 dysgerminoma and 7/8 germinoma. Focal weak membranous staining was also noted in 1 case of yolk sac tumor. The melanocytes in teratoma were also positive for CD117. All cases of embryonal carcinoma were negative. On the other hand, positive membranous expression of CD30 were demonstrated in 6/8 embryonal carcinoma. One case of germinoma and 1 case of yolk sac tumor showed weak cytoplasmic positivity. All cases of seminoma and dysgerminoma, 7/8 germinoma and all cases of teratoma were negative for CD30.

CONCLUSIONSOCT4 is a sensitive and relatively specific marker for diagnosing seminoma, dysgerminoma, germinoma and embryonal carcinoma. CD117 and CD30 immunostains, when used in combination, represent valuable tools for distinguishing embryonal carcinoma and seminoma, dysgerminoma, germinoma.

Carcinoma, Embryonal ; metabolism ; pathology ; Diagnosis, Differential ; Dysgerminoma ; metabolism ; pathology ; Endodermal Sinus Tumor ; metabolism ; pathology ; Female ; Germinoma ; metabolism ; pathology ; Humans ; Ki-1 Antigen ; metabolism ; Male ; Neoplasms, Germ Cell and Embryonal ; metabolism ; pathology ; Octamer Transcription Factor-3 ; metabolism ; Ovarian Neoplasms ; metabolism ; pathology ; Proto-Oncogene Proteins c-kit ; metabolism ; Seminoma ; metabolism ; pathology ; Teratoma ; metabolism ; pathology ; Testicular Neoplasms ; metabolism ; pathology

OBJECTIVETo evaluate the impact of lymphadenectomy on the relapse and survival of malignant ovarian germ cell tumor (OGCT).

METHODSThe clinical data of 102 OGCT cases treated in Peking Union Medical College Hospital from June 1980 to June 2003 were analyzed retrospectively. All the data about lymphadenectomy during primary and secondary surgery were collected, and other factors related to prognosis were also collected at the same time. Chi-squared test was applied in the univariate analysis related to relapse of disease. Cox model was applied in multivariate analysis related to relapse and survival of disease.

RESULTSPelvic and paraaortic lymph node metastasis was not significantly related to prognosis in primary and secondary treated patients. Lymphadenectomy showed no significant impact on disease relapse and survival. In the primary treatment, International Federation of Gynecology and Obstetrics (FIGO) staging, chemotherapy regimen, residual tumor and lymphadenectomy were the significant factors related to the relapse. After being stratified for the chemotherapy regimen, lymphadenectomy was not significantly related to the relapse in bleomycin +etoposide +cisplatin or cisplatin +vincristine +bleomycin regimen group, and lymphadenectomy could prevent relapse in no chemotherapy or other chemotherapy regimen group. In relapsed patients, only residual tumor was significantly related to survival time after relapse.

CONCLUSIONSPelvic lymph node metastasis is not the significant risk factor related to prognosis. Lymphadenectomy may have a beneficial effect on survival, although such effect is not significant. Although lymphadenectomy provides important information for prognosis, they provide little benefit to those patients already requiring chemotherapy based on the original operative findings. Lymphadenectomy should be performed to primary or relapsed patients by an expert surgical team.

Adolescent ; Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Child ; Combined Modality Therapy ; Female ; Germinoma ; mortality ; pathology ; surgery ; therapy ; Humans ; Lymph Node Excision ; methods ; Lymphatic Metastasis ; Neoplasm Recurrence, Local ; mortality ; pathology ; surgery ; therapy ; Neoplasm Staging ; Ovarian Neoplasms ; mortality ; pathology ; surgery ; therapy ; Prognosis ; Retroperitoneal Space ; Retrospective Studies

OBJECTIVETo assess the effects of laparoscopic retroperitoneal lymph node dissection in the treatment of stage I nonseminomatous testicular cancer.

METHODSFrom January 2001 to May 2002, laparoscopic retroperitoneal lymph node dissection was performed on 9 patients with stage I nonseminomatous testicular cancer.

RESULTSThe procedure was successful in all patients. The mean operation time was 260 minutes. None of the patients required blood transfusion and had major complications intraoperatively or postoperatively. The average period of hospitalization after the operation was 5.5 days. With a mean following-up of 9 months, retroperitoneal recurrence was not seen.

CONCLUSIONSLaparoscopic retroperitoneal lymph node dissection is feasible for stage I nonseminomatous testicular cancer and its procedure is safe, effective and minimally invasive.

Adult ; Follow-Up Studies ; Germinoma ; pathology ; surgery ; Humans ; Laparoscopy ; Lymph Node Excision ; methods ; Male ; Retroperitoneal Space ; Testicular Neoplasms ; pathology ; surgery ; Treatment Outcome ; Young Adult

OBJECTIVETo study the clinical characteristics, outcome, prognostic factors and survival of patients with testicular germ cell tumors (TGCTs).

METHODS107 TGCT patients received chemotherapy after orchiectomy. The median age of the patients was 32 years. 30.8% (33/107) patients had seminomas with 14 (42.4%) stage I lesions. Seventy-four patients had non-seminomatous germ cell tumors (NSGCTT) with 21 (28.4%) stage I lesions. The response rate was analyzed with chi(2) test. The survival rate was calculated with Kaplan-Meier method and log-rank test. Therapy including chemotherapy, radiation and necessary salvage operation were performed after orchiectomy.

RESULTSClinical stage and pathological type were the main prognostic factors. The 3-, 5- and 10-year survival rates were 75.8%, 73.5%, 73.5% for all patients, 100%, 96.8%, 96.8% for seminoma and 63.5%, 61.7%, 61.7% for NSGCTTs, respectively. Sixty-four patients were evaluable for response. Seventeen (26.6%) patients achieved CR by chemotherapy alone and an additional 8 patients (12.5%) achieved CR by chemotherapy plus salvage operation or radiation. The 5-year survival rates were 91.7% and 26.2% for patients who achieved CR or not.

CONCLUSIONThe long-term outcome for stage I germ cell tumors is excellent. The treatment outcome and survival in patients with metastatic TGCTs can be greatly improved by adopting multi-modality therapy with combined chemotherapy as the chief means.

Adolescent ; Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bleomycin ; therapeutic use ; Child ; Child, Preschool ; Cisplatin ; therapeutic use ; Combined Modality Therapy ; Etoposide ; therapeutic use ; Follow-Up Studies ; Germinoma ; drug therapy ; secondary ; surgery ; Humans ; Lung Neoplasms ; drug therapy ; secondary ; Male ; Middle Aged ; Neoplasm Staging ; Orchiectomy ; Seminoma ; drug therapy ; secondary ; surgery ; Survival Rate ; Testicular Neoplasms ; drug therapy ; pathology ; surgery ; Treatment Outcome

Child ; Germinoma ; chemistry ; pathology ; surgery ; Humans ; Immunohistochemistry ; Male ; Spinal Cord Neoplasms ; chemistry ; pathology ; surgery