Outcomes of Extended Curettage with and without Bone Allograft for Grade II Giant Cell Tumors around the Knee. A Retrospective Comparative Study / Resultados da curetagem estendida com e sem aloenxerto ósseo para tumores de células gigantes de grau II no joelho. Um estudo comparativo retrospectivo

Abstract Objective This is the first study to establish the utility of extended curettage with or without bone allograft for Grade II giant cell tumors GCTs around the knee joint with the aim of exploring postoperative functional outcomes. Methods We retrospectively reviewed 25 cases of Campanacci grade II GCTs undergoing extended curettage between January 2014 and December 2019. The participants were divided into two groups: one group of 12 patients underwent extended curettage with bone allograft and bone cement, while the other group of 13 patients underwent extended curettage with bone cement only. Quality of life was assessed by the Revised Musculoskeletal Tumor Society Score and by the Knee score of the Knee Society; recurrence and complications were assessed for each cohort at the last follow-up. The Fisher test and two-sample t-tests were used to compare the categorical and continuous outcomes, respectively. Results The mean age was 28.09 (7.44) years old, with 10 (40%) males and 15 females (60%). The distal femur and the proximal tibia were involved in 13 (52%) and in 12 (48%) patients, respectively. There was no significant difference in the musculoskeletal tumor society score (25.75 versus 27.41; p= 0.178), in the knee society score (78.67 versus 81.46; p= 0.33), recurrence (0 versus 0%; p= 1), and complications (25 versus 7.69%; p= 0.21). Conclusions Extended curettage with or without bone allograft have similar functional outcomes for the knee without any major difference in the incidence of recurrence and of complications for Grade II GCTs. However, surgical convenience and cost-effectiveness might favor the bone cement only, while long-term osteoarthritis prevention needs to be investigated to favor bone allograft.

Resumo Objetivo Este é o primeiro estudo a estabelecer a utilidade da curetagem estendida com ou sem enxerto ósseo em tumores de células gigantes (TCGs) de grau II na articulação do joelho com o objetivo de explorar os resultados funcionais pós-operatórios. Métodos Revisamos retrospectivamente 25 casos de TCGs de grau II de Campanacci submetidos a curetagem estendida entre janeiro de 2014 e dezembro de 2019. Os participantes foram divididos em 2 grupos: um grupo de 12 pacientes foi submetido a curetagem estendida com aloenxerto ósseo e cimento ósseo, enquanto o outro grupo, com 13 pacientes, foi submetido a curetagem estendida apenas com cimento ósseo. A qualidade de vida foi avaliada pela Pontuação Revista da Musculoskeletal Tumor Society (MTS, na sigla em inglês) e pela Pontuação da Knee Society (KS, na sigla em inglês), enquanto as taxas de recidiva e complicações foram avaliadas em cada coorte na última consulta de acompanhamento. O teste de Fisher e os testes t de duas amostras foram usados para comparação de resultados categóricos e contínuos, respectivamente Resultados A média de idade dos pacientes foi de 28,09 (7,44) anos; 10 (40%) pacientes eram do sexo masculino e 15 (60%) pacientes eram do sexo feminino. O fêmur distal e a tíbia proximal foram acometidos em 13 (52%) e 12 (48%) dos pacientes, respectivamente. Não houve diferença significativa na pontuação revista da MTS (25,75 versus 27,41; p= 0,178), na pontuação da KS (78,67 versus 81,46; p= 0,33) e nas taxas de recidiva (0 versus 0%; p= 1) e complicações (25 versus 7,69%; p= 0,21). Conclusões A curetagem estendida com ou sem aloenxerto ósseo tem resultados funcionais semelhantes em pacientes com TCGs de grau II no joelho, sem qualquer diferença importante na incidência de recidivas e complicações. No entanto, a conveniência cirúrgica e o custo-benefício podem favorecer a utilização apenas de cimento ósseo, enquanto a prevenção da osteoartrite em longo prazo precisa ser investigada para favorecer o enxerto ósseo.

Ankle arthrodesis with intramedullary retrograde nail for bone tumors. preliminary results and surgical technique / Artrodese do tornozelo com cavilha intramedular retrógrada para tumores ósseos. resultados preliminares e técnica cirúrgica

ABSTRACT Objective Present the preliminary results of a case series using the surgical ankle arthrodesis technique with an intramedullary retrograde nail for bone tumors. Methods We present the preliminary data of 4 patients, 3 males and 1 female, with a mean age of 46,2 (range 32 to 58) years, with histology proven Giant Cell Tumour of bone in 3 and osteosarcoma in 1. The mean resection length of distal tibia was 11,75 (range 9 to 16) cm, and all the patients underwent reconstruction with a tibiotalocalcaneal arthrodesis with an intercalary allograft fixed by a retrograde intramedullary nail. Results Oncological follow-up evolved without evidence of local recurrence or disease progression in all patients. After a mean time of 69.5 (range 32 to 98 months), patients had a mean MSTS12 functional score of 82.5% (range 75 to 90). All tibial arthrodesis and diaphyseal osteotomy sites were fused within 6 months with a return to activities without complications related to coverage skin or infection. Conclusion No complications were recorded; all arthrodesis and diaphysial tibial osteotomy sites fused by 6 months, and the mean follow-up of those patients was 69,5 (range 32 to 988) months, with a mean functional MSTS score of 82,5% (range 75-90). Level of Evidence: IV; Retrospective Case Series.

RESUMO Objetivo Apresentar os resultados preliminares de uma série de casos utilizando a técnica cirúrgica de artrodese do tornozelo com haste intramedular retrógada para tumores ósseos. Métodos Apresentamos os dados preliminares de quatro pacientes, três homens e uma mulher, com idade média de 46,2 (variação de 32 a 58) anos, com histologia comprovada de tumor de células gigantes em três e osteossarcoma em um. O comprimento médio de ressecção da tíbia distal foi de 11,75 (variação de 9 a 16) cm, e todos os pacientes foram submetidos à reconstrução com uma artrodese tibiotalocalcaneana com um aloenxerto intercalar fixado por uma haste intramedular retrógrada. Resultados O acompanhamento oncológico evoluiu sem evidências de recidiva local ou progressão da doença, em todos os pacientes. Após um tempo médio de 69,5 (variação de 32 a 98 meses), os pacientes tiveram uma pontuação média funcional MSTS12 de 82,5% (variação de 75 a 90). Todos os locais de artrodese e osteotomia diafisária tibiais foram fundidos em 6 meses com retorno às atividades de vida diária sem complicações relacionadas à cobertura ou infecção. Conclusão Não foram registradas complicações; todos os locais de artrodese e osteotomia diafisária da tíbia fundiram-se em 6 meses, e o acompanhamento médio desses pacientes foi de 69,5 (variação de 32 a 988) meses, com uma pontuação média funcional MSTS de 82,5% (variação de 75-90). Nível de Evidência IV; Série de Casos Retrospectivos.

Sobrecrecimiento lateral aislado - caracterización auxológica y clínico-radiológica de una cohorte de 76 casos de un único centro / Isolated lateralized overgrowth: clinical, radiological, and auxological characteristics of a single-site cohort of 76 cases

Introducción. El sobrecrecimiento lateral aislado (SLA), antes denominado hemihiperplasia/ hemihipertrofia, se refiere al sobrecrecimiento corporal lateral en ausencia de un patrón reconocible de malformaciones o síndromes genéticos. El objetivo fue analizar el crecimiento y las características clínico-radiológicas de pacientes con SLA en seguimiento en un hospital de tercer nivel en Argentina entre 1993 y 2020. Población y métodos. Estudio retrospectivo, observacional, de una cohorte de pacientes con SLA. Resultados. Se incluyeron 76 casos, 41 varones. Mediana de años de seguimiento: 5,85 (rango intercuartílico [RIC] 2,60-10,96), máximo 15,76 años. Cuarenta y ocho de 76 pacientes presentaron sobrecrecimiento en más de un segmento corporal (SLA complejo). El puntaje Z promedio de peso al nacer de niñas de término con SLA complejo fue +0,51 (desviación estándar [DE] 0,91) (p 0,022). El crecimiento en estatura de la mayoría de los niños se ubicó entre los centilos 50 y 97 de la población de referencia. La mediana de asimetría de longitud de miembros inferiores fue 1,5 cm (RIC 1,01-2,2) en pacientes con tratamiento médico y 3,70 cm (RIC 2,953,98 cm) en aquellos que requiriero epifisiodesis. El 75 % mostró una progresión de la asimetría menor o igual a 2 cm. Ocho casos presentaron asimetría renal mayor o igual a 1 cm; 2 casos presentaron nefroblastoma: edad promedio al diagnóstico 0,75 años. Conclusiones. El crecimiento prenatal de niños con SLA es normal, excepto en niñas con SLA complejo en quienes tiende a estar aumentado. La estatura promedio se ubica en centilos altos con crecimiento normal. Se recomienda realizar cribado de tumores embrionarios en este grupo de niños.

Introduction. Isolated lateralized overgrowth (ILO), formerly referred to as hemihyperplasia/hemihypertrophy, is the overgrowth of one-half of the body to its contralateral in the absence of a recognizable pattern of malformations or genetic syndromes. Our objective was to analyze the growth clinical and radiological characteristics of patients with ILO under follow-up in a tertiary care hospital in Argentina between 1993 and 2020. Population and methods. Retrospective, observational, single cohort study of patients with ILO. Results. A total of 76 cases were included; 41 were males. Median years of follow-up: 5.85 (interquartile range [IQR]: 2.60­10.96), maximum: 15.76 years. Forty-eight of 76 patients had overgrowth compromising more than 1 body segment (complex ILO). The mean birth weight Z-score of term girls with complex ILO was +0.51 (standard deviation [SD]: 0.91) (p 0.022). Most children grew between the 50th and 97th centile of the Argentinian population height reference. The median leg length discrepancy was 1.5 cm (IQR: 1.01­2.2) in patients receiving medical treatment and 3.70 cm (IQR: 2.95­3.98 cm) in those who required epiphysiodesis. Progression of discrepancy ≤ 2 cm was observed in 75% of cases. Renal asymmetry ≥ 1 cm was observed in 8 cases; Wilms tumor was noted in 2 cases: mean age at diagnosis: 0.75 years. Conclusions. Prenatal growth of children with ILO is normal, except in girls with complex ILO, in whom it tends to be increased. The average height of boys and girls tends to be located in high centiles with normal growth over time. Embryonal tumor screening is recommended in this group of children.

Treatment of Patients Diagnosed with Giant Cell Tumor of Bone: Experience of a Philanthropic Hospital in the State of Piauí, Brazil / Tratamento de pacientes com diagnóstico de tumor ósseo de células gigantes: Experiência de um hospital filantrópico no Piauí, Brasil

Abstract Objective To evaluate the treatment of patients with giant cell tumors of bone treated from 2009 to 2019 in a philanthropic hospital, as well as to try and determine the regional clinical and epidemiological profile, aiming to enrich the Brazilian data set and compare our findings with those of the literature. Methods An analytical, observational, and cross-sectional study with retrospective data collection and a quantitative approach, analyzing medical records of patients with giant cell tumors treated at a philanthropic hospital from 2009 to 2019. Results We evaluated 49 medical records; 55.1% of the patients were women, 53.1% were aged between 20 and 40 years, 69.4% of the cases were Campanacci grade III, and 30.6% affected the proximal end of the tibia. The rate of pathological fractures secondary to the tumor and pulmonary metastasis was low. More than 69% of the patients underwent intralesional surgery. Recurrence occurred in 16.3% of the cases. Conclusion The criteria used for the diagnosis, classification, and treatment at our service followed the standards established by the literature, and they can guide further research and improve local prognosis in the future.

Resumo Objetivo Avaliar o tratamento fornecido a pacientes com diagnóstico de tumor de células gigantes ósseo atendidos no período de 2009 a 2019 em um hospital filantrópico, bem como determinar o perfil clínico e epidemiológico regional, visando enriquecer os dados nacionais e comparar os achados com a literatura existente. Métodos Estudo analítico, observacional e transversal, com coleta retrospectiva e abordagem quantitativa, com análise de prontuários de pacientes diagnosticados com tumor de células gigantes atendidos em um hospital filantrópico no período de 2009 a 2019. Resultados Foram avaliados 49 prontuários, sendo que 55,1% eram de mulheres, com 53,1% dos casos na faixa etária de 20 a 40 anos, 69,4% de casos de grau III de Campanacci, e 30,6% acometendo a extremidade proximal da tíbia. Observou-se baixo índice de fratura patológica secundária ao tumor e de metástase pulmonar. A cirurgia intralesional foi realizada em 69,5% dos pacientes. Houve recidiva em 16,3% dos casos. Conclusão Os critérios usados para diagnóstico, classificação e tratamento em nosso serviço seguiram os padrões estabelecidos pela literatura, e podem orientar novas pesquisas e melhorar o prognóstico local futuramente.

Sacrococcygeal teratoma: A case report

Sacrococcygeal teratomas (SCTs) are the most common extragonadal germ cell tumors, comprised different types of tissues that come from at least two of three germ cell layers. Depending on the tissues that are included, they are divided into mature, immature, and malignant. The incidence of SCT in infants and children is 1 in 35,000–40,000 live births. We are reporting a case of type I SCT. The patient was gravida 3, para 1, and abortion 1, which was diagnosed during antenatal ultrasound examination at 22 weeks of gestation and the termination was done after counseling the parents. A female fetus with a tumor in the sacrococcygeal region, weighing 800 g was delivered. The baby was sent to the department of anatomy. SCTs develop at the base of the coccyx and are thought to be derived from Henson’s node a rounded and elevated area at the cranial end of the primitive streak. This primitive streak consists of totipotent cells, which are able to transform into any type of cells.

Quiste odontogénico calcificante asociado a odontoma complejo en la región posterior del maxilar. Reporte de caso clínico y revisión de la literatura / Calcifying odontogenic cyst associated with complex odontoma in the posterior litera of the maxilla. A case report and review of the literature

El quiste odontogénico calcificante (QOC) es raro y representa <1% de todos los quistes odontogénicos, pertenece a las lesiones conocidas como "tumores de células fantasma", estos son una familia de lesiones que varían en presentación clínica de quiste o neoplasia sólida, en su comportamiento biológico de benigno a localmente agresivo o metastásico, esta diversidad ha generado extenso debate sobre la clasificación, terminología, manejo y pronóstico de estas patologías. Presentamos el caso clínico de un paciente masculino de 17 años de edad con diagnóstico de quiste odontogénico calcificante asociado a odontoma complejo situado en la región posterior del maxilar, en la literatura existen reportados solo 27 casos de QOC en esa zona. Fue tratado mediante enucleación quirúrgica, curetaje, rotación de almohadilla grasa bucal y colocación de malla de titanio para reconstruir la pared anterior del seno maxilar. El control postoperatorio clínico y radiográfico a 12 meses no muestra recidiva de la lesión además exhibe un excelente resultado funcional y estético, esto nos permite concluir que el tratamiento fue adecuado. Realizamos una revisión de la literatura en Science Direct, PubMed y Biblioteca Cochrane, con objeto de informar las características clínicas, histopatológicas, radiográficas y el tratamiento de estas lesiones.

The calcifying odontogenic cyst (COC) is rare and represents <1% of all odontogenic cysts, it belongs to the lesions known as "ghost cell tumors", these are a family of lesions that vary in clinical presentation of cyst or solid neoplasia, in their behavior biological from benign to locally aggressive or metastatic, this diversity has generated extensive debate on the classification, terminology, management and prognosis of these pathologies. We present the clinical case of a 17-year-old male patient with a diagnosis of calcifying odontogenic cyst associated with a complex odontoma located in the posterior region of the maxilla, in the literature there are only 27 reported cases of (COC) in that area, he was treated by surgical enucleation, curettage, rotation of the buccal fat pad and placement of titanium mesh to reconstruct the anterior wall of the maxillary sinus. The clinical and radiographic postoperative control at 12 months does not show recurrence of the lesion, in addition it exhibits an excellent functional and aesthetic result, this allows us to conclude that the treatment was adequate. We conducted a review of the literature in Science Direct, PubMed and Cochrane, in order to report the clinical, histopathological, radiographic characteristics and the treatment of these lesions.

Analysis of therapeutic effect of pediatric patients with intracranial primary non-germinomatous germ cell tumors / 中华放射肿瘤学杂志

Objective:To investigate the clinical features of pediatric patients with intracranial primary non-germinomatous germ cell tumors (NGGCT) and evaluate the treatment outcomes and prognostic factors of NGGCT.Methods:Clinical data of 40 children with NGGCT who were treated with radiotherapy (RT) at our department between November 2008 and June 2019 were retrospectively analyzed. Ninety percent of them received craniospinal irradiation (CSI). All children received platinum-based chemotherapy. Survival analysis was conducted using the Kaplan-Meier estimate. The prognostic factors were analyzed by log-rank test.Results:The primary sites were pineal gland, sellar / suprasellar region and basal ganglia. The median age of onset was 108 months (20-204 months). The median follow-up time was 33 months (8-131 months), and the 3-year and 5-year overall survival (OS) rates were 82.0%. The 3-year and 5-year progression-free survival (PFS) rates were 78.6% and 73.0%. Univariate analysis showed that increased alpha-fetoprotein (AFP) ( P=0.02), age at first diagnosis>10 years ( P=0.006), metastasis at first diagnosis ( P<0.001), and the pathological type (choriocarcinoma, yolk sac tumor and / or embryonal carcinoma) ( P=0.036) were independent adverse prognostic factors. Conclusions:Increased AFP, age>10 years at first diagnosis, tumor metastasis and pathological type were independent adverse prognostic factors of NGGCT. The overall prognosis of NGGCT children is worse than that of their counterparts with germinoma, and multidisciplinary intensive therapy is needed to improve survival.

Three cases of mediastinal germ cell tumors and concurrent hematologic malignancy and literature review / 中华实用儿科临床杂志

To analyze the clinical characteristics, treatment and prognosis of mediastinal germ cell tumors (GCTs) with concurrent hematologic malignancy (HM). The clinical features, treatment and prognosis of 3 cases of HM associated with mediastinal GCTs treated in the Department of Medical Oncology, Beijing Children′s Hospital from November 2014 to September 2018 were retrospectively analyzed.Meanwhile, relevant cases were searched in the PubMed and Wanfang database from their establishment to December 2019.Three male cases of HM associated with mediastinal GCTs aged from 12 to 16 years.The pathogenesis of mediastinal masses suggested teratoma or yolk sac tumor.All of them were treated with surgery and chemotherapy.Acute myeloid leukemia (AML) or myelodysplastic syndromes (MDS) was diagnosed respectively at 5 months, 9 months and 31 months after initial GCTs in the 3 cases.Two patients died and 1 child survived at the last follow-up.A total of 135 cases of concurrent GCTs and HM (or leukemia) were reviewed in online databases, involving 127 cases (94.1%) with the mediastinal GCTs associated with HM and 8 cases(5.9%) with GCTs related HM from another original sites.One hundred and twenty-six cases (99.2%) were male and the median age of GCTs diagnosis was 22 (10-48) years.Fifty-three cases (41.7%) were teratoma and 94 cases (74.0%) were GCTs containing teratoma with or without yolk sac tumor.Among the types of HM, 72 cases (56.7%) were AML and 31 cases (24.4%) were AML-M7.The median interval between GCTs and HM was 3 (0-122) months.Forty-six cases (36.2%) presented 2 malignancies simultaneously.HM were diagnosed within 12 months of GCTs in 85 cases (66.9%). The survival data were known in 107 cases, involving 94 (87.9%) deaths and 13 (12.1%) survivors.The median survival time after diagnosis of HM was 2 (0-48) months.The tendency of HM must be highly concerned in adolescent male patients with primary mediastinal GCTs, especially those with yolk sac tumor or teratoma.Their prognoses are very poor.Allogeneic hematopoietic stem cell transplantation is an alternative treatment.

Reconstruction with a Custom made Prosthetic Wrist Arthrodesis after Bone Tumor Resections of the Distal Radius. Single Centre Experience / Reconstrução com implante metálico personalizado de artrodese após ressecções de tumor ósseo do rádio distal. Experiência em um único centro

Abstract Objective The present study aimed at analyzing the clinical, radiological and functional results of the reconstruction of the distal radius after tumor resection with a custom-made metal arthrodesis implant and compare them with other types of distal radius reconstruction, as presented in the literature. To our best knowledge, this is the first article describing this particular type of implant and patient functionality. Methods Functional outcomes of reconstruction of the distal radius were assessed in a series of 4 patients. Three of the patients having had resection of giant cell tumors (GCTs), one patient having had resection of osteosarcoma. Results There were no major implant-related complications like infection, nonunion or loosening. Two patients had to undergo further surgery for protruding metalwork. Overall function was good according to the Musculoskeletal Tumor Society MSTS and Disabilities of the Arm, Shoulder, and Hand (DASH) scores. Conclusion The present study shows that custom-made metal arthrodesis implant benefits from the fact that it can be used as a salvage option when other treatments have failed, or it can be used as a primary option in cases in which there is limited bone stock after distal radius tumor resection.

Resumo Objetivo O presente estudo teve como objetivo analisar os resultados clínicos, radiológicos e funcionais da reconstrução do rádio distal após a ressecção do tumor com implante metálico personalizado de artrodese e compará-los com outros tipos de reconstrução do rádio distal, conforme apresentado na literatura. Pelo que conhecemos, este é o primeiro artigo descrevendo esse tipo particular de implante e funcionalidade no paciente. Métodos Os desfechos funcionais de reconstrução do rádio distal foram avaliados em uma série de 4 pacientes. Três dos pacientes tiveram ressecção de tumores de células gigantes (TCGs), sendo um paciente com ressecção de osteossarcoma. Resultados Não houve complicações relacionadas ao implante, como infecção, não sindicalidade ou afrouxamento. Dois pacientes tiveram que passar por uma nova cirurgia para a protusão da prótese metálica. A função geral foi boa de acordo com as pontuações da Musculoskeletal Tumor Society (MSTS) e Disabilities of the Arm, Shoulder, and Hand (DASH). Conclusão O estudo mostra que o implante metálico personalizado de artrodese se beneficia do fato de que pode ser usado como opção de salvamento quando outros tratamentos falharam, ou pode ser usado como opção primária nos casos em que há estoque ósseo limitado após a ressecção do tumor do rádio distal.

Testicular germ cell tumors type 2 have high RNA expression of / 亚洲男科学杂志(英文版)

This study analyzed RNA expression of genes for three serum tumor markers, alpha fetoprotein (AFP), human chorionic gonadotropin (hCG), and lactate dehydrogenase (LDH), in patients with testicular germ cell tumors (TGCT) type 2. The gene AFP encodes AFP, the gene for chorionic gonadotropin beta polypeptide 5 (CGB5) encodes a major part of the specific beta subunit of hCG, and the genes for LDH subunit A (LDHA), LDH subunit B (LDHB), and LDH subunit C (LDHC) encode three different subunits of LDH. LDHB encodes the LDHB subunit present as a tetramer in LDH isoenzyme 1 (LDH-1). We examined three datasets with 203 samples of normal testis tissue (NT) and TGCT type 2. Yolk sac tumor (YST) expressed RNA of AFP fourteen thousand times higher than seminoma (SE), embryonal carcinoma (EC), and teratoma (TER) combined (P = 0.00015). In the second microarray, choriocarcinoma (CC) expressed RNA of CGB5 ten times higher than other histologic types of TGCT combined. EC expressed RNA of LDHB twice higher than SE, YST and TER combined (P = 0.000041). EC expressed RNA of LDHB higher than that YST expressed RNA of AFP and that CC expressed RNA of CGB5. In conclusion, TGCT type 2 expressed RNA of LDHB markedly higher than the RNA of 23 other candidate genes for TGCT type 2.

Primary malignant giant cell tumor of the sternum

Primary malignant giant cell tumor (PMGCT) is a diagnosis based on the presence of a high-grade sarcomatous component along with a typical benign giant cell tumor (GCT). We report the first case of PMGCT of the sternum in a 28-year-old male with painless swelling over the manubrium sterni. The differential diagnoses of PMGCT and giant cell-rich osteosarcoma were considered. Surgical resection was performed, and the reconstruction was done with a neosternum using polymethyl methacrylate and prolene mesh. At 30 months follow-up, the patient is disease-free.

Preoperative differentiation of benign and malignant non-epithelial ovarian tumors: clinical features and tumor markers / Diferenciação pré-operatória de tumores ovarianos não epiteliais benignos e malignos: características clínicas e marcadores tumorais

Abstract Objective To evaluate the role of clinical features and preoperativemeasurement of cancer antigen 125 (CA125), human epididymis protein(HE4), and carcinoembryonic antigen (CEA) serum levels in women with benign and malignant non-epithelial ovarian tumors. Methods One hundred and nineteen consecutive women with germ cell, sex cordstromal, and ovarian leiomyomas were included in this study. The preoperative levels of biomarkers were measured, and then surgery and histopathological analysis were performed. Information about the treatment and disease recurrence were obtained from the medical files of patients. Results Our sample included 71 women with germ cell tumors (64 benign and 7 malignant), 46 with sex cord-stromal tumors (32 benign and 14 malignant), and 2 with ovarian leiomyomas. Among benign germ cell tumors, 63 were mature teratomas, and, amongmalignant, fourwere immatureteratomas. Themost common tumors in the sex cordstromal group were fibromas (benign) and granulosa cell tumor (malignant). The biomarker serum levels were not different among benign andmalignant non-epithelial ovarian tumors. Fertility-sparing surgeries were performed in 5 (71.4%) women with malignant germ cell tumor. Eleven (78.6%) patients with malignant sex cord-stromal tumors were treated with fertility-sparing surgeries. Five women (71.4%) with germ cell tumors and only 1 (7.1%) with sex cord-stromal tumor were treated with chemotherapy. One woman with germ cell tumor recurred and died of the disease and one woman with sex cord-stromal tumor recurred. Conclusion Non-epithelial ovarian tumors were benign in the majority of cases, and the malignant caseswere diagnosed at initial stages with good prognosis. Themeasurements of CA125, HE4, and CEA serum levels were not useful in the preoperative diagnosis of these tumors.

Resumo Objetivo Avaliar o papel das características clínicas e a medida pré-operatória dos níveis séricos de CA125, HE4, e CEA em mulheres com tumores de ovário não epiteliais benignos e malignos. Métodos Cento e dezenovemulheres consecutivas comtumores ovarianos de células germinativas, do cordão sexual-estroma, e miomas ovarianos foram incluídas neste estudo. Os níveis pré-operatórios dos biomarcadores foram medidos, a cirurgia e a análise histopatológica foram realizadas. Informações sobre tratamento e recorrência da doença foram obtidas dos prontuários médicos das pacientes. Resultados Nossa amostra incluiu 71 mulheres com tumores de células germinativas (64 benignos e 7 malignos), 46 com tumores do cordão sexual-estroma (32 benignos e 14 malignos), e 2 com leiomiomas ovarianos. Entre os tumores benignos de células germinativas, 63 eram teratomas maduros, e, entre os malignos, quatro eram teratomas imaturos. Os tumores mais comuns do grupo do cordão sexual-estroma foram fibromas (benignos) e tumores de células da granulosa (malignos). Os níveis séricos dos biomarcadores não diferiram entre os tumores de ovário não epiteliais benignos e malignos. A cirurgia preservadora de fertilidade foi realizada em 5 (71,4%) mulheres com tumores malignos de células germinativas. Onze (78,6%) mulheres com tumores do cordão sexual-estromamalignos foram tratadas comcirurgia preservadora de fertilidade. Cinco (71,4%)mulheres com células germinativas e apenas 1 (7,1%) com tumor do cordão sexual-estroma foram tratadas com quimioterapia. Uma mulher com tumor de células germinativas recidivou e morreu da doença. Uma mulher com tumor do cordão sexual-estroma recidivou. Conclusão Os tumores de ovário não epiteliais foram benignos namaioria dos casos e os malignos foram diagnosticados em estágios iniciais, com bom prognóstico. A medida dos níveis séricos de CA125, HE4, e CEA não foram úteis no diagnóstico préoperatório desses tumores.

Non seminomatous mixed germ cell tumor of the testis with predominant component of somatic type malignancy (Rhabdomyosarcoma) – A rare occurrence

Testicular germ cell tumors (GCT) are a diverse group of neoplasms, broadly divided into seminomatous and non seminomatous types, with varying histomorphology. Mixed germ cell tumors express more than one germ cell component. Somatic type malignancy occurring in testicular GCT is rare. Most often these components present as metastasis, particularly following chemotherapy, rather than primarily involving the testis. We describe a rare case of a young adult with no significant past history, who presented with testicular mass which on histology revealed a non-seminomatous mixed germ cell tumor with additional somatic type malignancy component of a rhabdomyosarcoma.

Huge Sertoli-Leydig cell tumor: a rare case report

Sertoli-Leydig cell tumors (SLCTs), constitute less than 0.5% of all ovarian tumors. SLCT are rare after menopause (less than10%), 75 years, unmarried, postmenopausal since 30 years, presented with complaints of abdominal swelling. On examination, abdominal mass of 36 weeks, hard, immobile felt. Ultrasonography of abdomen showed lobulated solid cystic lesion of size 22 x 15 x 27 cms with bilateral ovaries not separately visualized. Moderate free fluid in the abdomen, suggestive of malignant neoplastic etiology. CECT Abdomen + pelvis suggestive of primary ovarian malignancy with omental deposits and pelvic adenopathy. CA 125- 415.1 Patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy with huge tumour mass excision (weight 5.25 kg) with umbilical hernia repair. Frozen section suggestive of a huge mass of 30 x 20 x 15 cm, multilobulated, yellowish white tan in colour, solid-cystic in consistency, seen arising from right ovary. Omentum appears normal. Histopathology report s/o- Moderately differentiated Sertoli Leydig cell tumor. Reporting the first, this huge, in this age group with atypical presentation.

Progress in the treatment of intracranial germ cell tumors / 中国肿瘤临床

Intracranial germ cell tumors (IGGCTs) are rare intracranial embryonal tumors with various histological types. Most types of germ cell tumors are sensitive to radiotherapy and chemotherapy and can be cured with early treatment. The 5-year overall survival rate (OSR) of people with IGGCT has been reported as high as 90%, while the 5-year OSR of other malignant non-germinomatous germ cell tumors (NGGCTs) has been reported as less than 70%. Due to developments in surgery, imaging, nuclear medicine, pharmaceuticals, and other disciplines, the therapeutic effect of IGGCTs has improved in the last decade. However, due to the slow progress in researching its pathological genes, a treatment plan has not been standardized, and there is no standardized and clear clinical pathway or diagnosis and treatment guide. Here, the treatment experience and progress of IGGCTs is reviewed.

FLI1 and MIC2 expression in precursor B-lymphoblastic leukemia with Burkitt-like morphology and extensive extramedullary involvement: A diagnostic challenge in pediatric small round cell tumor

Pediatric small round cell tumors (PSRCTs) constitute a large proportion of childhood malignancies with overlapping diagnostic and clinical features but radically different therapies. Here, we report a case of 16-year-old male child presenting with diffuse abdominal and mediastinal mass, axillary lymphadenopathy, and pleural effusion. Bone marrow aspirate showed near total replacement by small round malignant cells. The bone marrow biopsy showed interstitial infiltration by malignant cells, which were CD45? CD3? CD20? MIC2+ FLI1+ and diagnosis of Ewing's sarcoma was established. In contrast, flowcytometric immunophenotyping of the bone marrow aspirate showed CD45? cells, which were CD19+ cytCD79a+ CD10+ CD81+ CD38+ HLA-DR+ CD22+ CD20? consistent with B-cell acute lymphoblastic leukemia (B-ALL). The extended immunostaining panel on bone marrow biopsy also showed positivity for cytCD79a, CD10, CD19, and BCL-2, whereas fluorescent in-situ hybridization for EWSR1 gene rearrangement was negative. Thus, a final diagnosis of CD45? FLI1+ MIC2+ B-ALL was established. Rare cases of CD45? B-ALL with immunoreactivity for MIC2 and Friend leukemia virus integration 1 (FLI1) have posed a diagnostic challenge for PSRCTs in the recent past. This case report highlights the role of multimodality approach in establishing a correct diagnosis in CD45? PSRCTs to ensure definitive therapy and better clinical outcome.

Hipertiroidismo como clave diagnóstica de un coriocarcinoma avanzado: Una asociación inusual / Hyperthyroidism as a diagnostic key to advanced choriocarcinoma. An unusual association

El hipertiroidismo es una condición relativamente frecuente con múltiples etiologías. La más común es la enfermedad de Graves, seguida del bocio multinodular y el adenoma tóxico. La asociación entre hipertiroidismo y cáncer es infrecuente en la práctica clínica. Presentamos el caso de un varón de 42 años con síntomas de hipertiroidismo de dos meses de evolución. Al examen físico se constató una marcada hepatomegalia de consistencia duro pétrea. El examen de testículos se reveló normal. Se llevó a cabo el diagnóstico de hipertiroidismo a través del dosaje hormonal. Los estudios por imágenes mostraron la presencia de múltiples lesiones sólidas compatibles con metástasis hepáticas. Luego de descartar las causas habituales de hipertiroidismo y las neoplasias primarias de la glándula tiroides, se consideró la posibilidad de mimetismo molecular a través de la producción ectópica de gonadotrofina coriónica humana. Se obtuvieron valores críticamente elevados de esta hormona y en un segundo tiempo se confirmó el diagnóstico histológico de coriocarcinoma a través de una biopsia hepática. Consideramos que el reconocimiento de este mecanismo poco frecuente de hipertiroidismo, puede ser una clave diagnóstica para arribar rápidamente al diagnóstico correcto, particularmente en los tumores extragonadales.

Hyperthyroidism is a relatively frequent condition with multiple causes. The most common cause is Graves' disease; followed by hyperthyroid multinodular goiter and toxic adenoma. Association between hyperthyroidism and cancer is infrequent in daily practice. We present the case of a 42-year-old man who developed severe symptoms of hyperthyroidism within a period of two months. Physical examination revealed significant hepatomegaly. Testicular examination proved normal. Imaging studies showed the presence of multiple hepatic solid lesions consistent with metastases. After discarding the most common causes of hyperthyroidism and primary thyroid gland neoplasm, the possibility of molecular mimicry was considered through human chorionic gonadotrophin production. Critical high values of this hormone were found and choriocarcinoma histological diagnosis was confirmed through a liver biopsy. We consider that the recognition of this rare mechanism of hyperthyroidism may be a clue permitting a faster diagnosis, particularly when extragonadal tumors are present.

Inoperable Sacral Giant Cell Tumor: Therapeutic Options and Pain Control / Tumor de células gigantes do sacro inoperável: opções terapêuticas e controle da dor

Abstract Sacral giant cell tumor (GCT) is a rare condition. Its treatment is complex, since surgical removal is difficult and the response to other therapeutic options is low. The control of its growth and pain is an additional challenge. The present paper reports a case of inoperable sacral GCT, with embolization and radiotherapy for pain control as therapeutic options. The patient, a 39-year-old male, presented pain in the sacral region with lower limb irradiation due to an inoperable sacral giant cell tumor. The patient was submitted to embolization, radiotherapy, pain management with opioids and other drugs, and a rehabilitation program. Despite the difficulty in tumor growth and pain control during the follow-up, the outcome is stable after 9 years.

Resumo O tumor de células gigantes (TCG) do sacro é raro e seu tratamento é complexo, devido à dificuldade para a exerese cirúrgica e a baixa resposta às outras opções terapêuticas. Entre os desafios relacionados a este tumor está o controle do seu crescimento e da dor. No presente trabalho, relatamos um caso de tumor de células gigantes do sacro inoperável, apresentando as opções terapêuticas de embolização e de radioterapia para o controle da dor. Relato do caso: paciente do sexo masculino, admitido aos 39 anos de idade, apresentando dor na região sacral com irradiação para os membros inferiores (MMII), com diagnóstico de TCG do sacro inoperável. Realizou-se embolização, uso de interferon, radioterapia, tratamento da dor com opioides e medicamentos adjuvantes, associados a programa de reabilitação. Descreveu-se o difícil controle do crescimento tumoral e da dor ao longo do seguimento, com desfecho estável após 9 anos.

Retrospective Study of the Results of Surgical Treatment of 31 Giant Cell Tumors of the Tendon Sheath in the Hand / Estudo retrospectivo dos resultados do tratamento cirúrgico de 31 tumores de células gigantes da bainha do tendão da mão

Abstract Objective To evaluate the results of the treatment of 31 giant-cell tumors of the tendon sheath of the hand cared for between 2006 and 2015. Methods A group of patients for the present retrospective evaluation was defined, covering the period between February 2006 and November 2015, in which 31 records of patients who underwent surgery due to cell tumor of the tendon sheath of the fingers were studied and evaluated. The recommended treatment was complete excision of the tumor with preservation of the adjacent structures. The diagnosis was confirmed by pathological examination. The progress of the treatment after surgery was evaluated, especially regarding the rate of tumor recurrence. Results Taking all 31 patients into consideration, there was a predominance of the female gender and white ethnicity. Most of these patients were aged between 30 and 50 years. The most affected side was the left one, and most tumors were in the flexor face. There was a predominance of the radial fingers, along with their distal end. A total of 27 patients are being followed up at regular intervals at an outpatient clinic, and three cases of tumor recurrence have been identified. Conclusion An appropriate surgical technique is essential in order to prevent GCTTS recurrences. The results obtained in this research are in agreement with the literature.

Resumo Objetivo Avaliar os resultados do tratamento de 31 tumores de células gigantes da bainha do tendão da mão encontrados entre 2006 e 2015. Métodos Entre fevereiro de 2006 e novembro de 2015, um grupo de pacientes foi selecionado para avaliação retrospectiva, na qual foram estudados e avaliados 31 prontuários de pacientes submetidos a procedimento cirúrgico devido a tumor de células gigantes da bainha do tendão dos dedos da mão. O tratamento preconizado foi a excisão completa do tumor com a preservação das estruturas adjacentes. A confirmação diagnóstica foi feita pelo exame anatomopatológico. Foi avaliada a evolução do tratamento após a cirurgia, principalmente no tocante ao índice de recidivas dos tumores. Resultados Dos 31 pacientes da amostra, houve predomínio do sexo feminino e da etnia branca. O tumor acometeu principalmente indivíduos entre 30 e 50 anos. O lado mais acometido foi o esquerdo, e amaioria dos tumores estava na face flexora. Houve predominância dos dedos radiais, juntamente com sua extremidade distal. Esses pacientes têm sido acompanhados em intervalos regulares em ambulatório. Dos 27 pacientes reavaliados, foram identificados 3 casos de recidiva tumoral. Conclusão Uma técnica cirúrgica adequada é essencial para a prevenção de recidivas do TCGBT. Os resultados obtidos na pesquisa estão em concordância com a literatura atual.

Use of different fixation times and application of two immunohistochemical methods for detection of KIT and Ki67 proteins in canine cutaneous mast cell tumors / Utilização de diferentes tempos de fixação e métodos imuno-histoquímicos na detecção das proteínas KIT e Ki67 em mastocitomas cutâneos caninos

Due to the high prevalence of mast cell tumors (MCTs) in the diagnostic routine, several factors, especially prognostic, have been sought to determine the biological behavior of these neoplasms. Immunohistochemistry (IHC) is one of the main tools utilized to biologically differentiate more aggressive tumors from less aggressive ones. However, some immunostainings are influenced by formalin fixation, interfering with the results. This is both a retrospective and prospective study of MCTs diagnosed in laboratory routine. A total of 25 samples, without knowledge about fixation time, were analyzed in the retrospective study, whereas 12 samples, with known fixation times, were assessed in the prospective study. Two histologic grading systems (Patnaik and Kiupel), special staining of toluidine blue, and IHC for KIT and Ki67 proteins were applied in both studies. Additionally, two amplification systems (biotinylated and non-biotinylated) for Ki67 protein and counting of the argyrophilic nucleolar organizing regions (AgNOR method) were tested in the prospective study. In the retrospective study, greater agreement between the evaluating pathologists was observed when the Kiupel system was used. IHC staining for KIT protein was effective in both studies, regardless of fixation time. IHC staining for Ki67 protein was highly sensitive to formaldehyde, and staining failure was observed in 56% of the cases in the retrospective study. In the prospective study, samples fixed for longer than 24 hours showed a reduction in the number of stained cells (altering the determination of the cell growth fraction) or showed absence of IHC staining in both amplification systems. The use of the AgNOR method to evaluate the rate of cell proliferation may be an alternative when the fixation time of the neoplasm is unknown or longer than 24 hours.(AU)

Devido a alta prevalência dos mastocitomas cutâneos caninos (MCCs) na rotina diagnóstica, vários fatores, especialmente fatores prognósticos, têm sido buscados para auxiliar na determinação do comportamento biológico desse neoplasma. A imuno-histoquímica é uma das principais ferramentas empregadas para diferenciar tumores biologicamente mais agressivos de tumores menos agressivos. Entretanto, algumas imunomarcações sofrem influência pela fixação em formol, interferindo nos resultados. Este estudo compreendeu avaliar através de uma etapa retrospectiva e uma etapa prospectiva casos de MCCs diagnosticados na rotina laboratorial. Um total de 25 amostras, sem conhecimento do tempo de fixação, foi analisado no estudo retrospectivo e 12 amostras, com tempos de fixação conhecidos, no estudo prospectivo. Foram aplicados nos dois estudos, dois sistemas de graduação histológica (Patnaik e Kiupel), a coloração especial de azul de toluidina e a imuno-histoquímica para as proteínas KIT e Ki67. Adicionalmente, no estudo prospectivo, foram testados dois sistemas de amplificação (biotinilado e não biotinilado) para a proteína Ki67 e a técnica de AgNOR (contagem das regiões organizadoras nucleolares argirofílicas). Na etapa retrospectiva, observou-se uma maior concordância entre os patologistas avaliadores quando o sistema Kiupel foi utilizado. A imunomarcação para KIT se manteve eficaz em ambos os estudos, independentemente do tempo de fixação. A imunomarcação para o Ki67 mostrou-se altamente sensível ao tempo de fixação em formol, sendo observada falha na imunomarcação em 56% dos casos do estudo retrospectivo. No estudo prospectivo, constatou-se que amostras fixadas por mais de 24 horas em formol apresentaram redução na quantidade de células imunomarcadas (alterando a determinação da fração de crescimento celular) ou apresentaram ausência de imunomarcação em ambos os sistemas de amplificação. A utilização do método AgNOR, para avaliar a taxa de proliferação celular, pode ser uma alternativa quando o tempo de fixação do neoplasma for desconhecido ou superior a 24 horas.(AU)