Swyer syndrome with gonadal non-dysgerminoma malignant germ cell tumors: a report of 15 cases in a national medical center / 中华妇产科杂志

Objective: To evaluate the incidence, treatment, and survival outcomes of Swyer syndrome with gonadal non-dysgerminoma malignant germ cell tumor (MGCT-NDG). Methods: A retrospective study was performed on Swyer syndrome patients with MGCT-NDG between January 2011 and December 2022 in Peking Union Medical College Hospital to investigate their characteristics and outcomes. Results: A total of 15 patients (4.9%, 15/307) with Swyer syndrome were identified in 307 MGCT-NDG patients. The average age at diagnosis of MGCT-NDG and Swyer syndrome were (16.8±6.7) and (16.7±6.6) years, respectively. Six cases were preoperatively diagnosed as Swyer syndrome, of which 4 cases received bilateral gonadectomy with or without hysterectomy, while the other 2 cases underwent removal of gonadal tumor and unilateral gonadectomy with hysterectomy, respectively. Of the 9 patients postoperatively diagnosed as Swyer syndrome, unilateral gonadectomy, removal of gonadal tumor, and unilateral gonadectomy with hysterectomy were performed in 6 patients, 2 patients, and 1 patient, respectively. Mixed malignant germ cell tumor (MGCT;10 cases), yolk sac tumor (4 cases), and immature teratoma (1 case) were the pathological subtypes, in the descending order. There were International Federation of Gynecology and Obstetrics (FIGO) stage Ⅰ in 6 cases, stage Ⅱ in 3 cases, stage Ⅲ in 5 cases, and stage Ⅳ in 1 case, respectively. Eleven patients received reoperation for residual gonadectomy after a average delay of (7.9±6.2) months, including 8 MGCT-NDG patients and 1 gonadoblastoma patient, no tumor involved was seen in the remaining gonads in the other 2 cases. Ten patients experienced at least one recurrence, with a median event free survival of 9 months (5, 30 months), of which 2 patients received surgery only at the time of initial treatment. All patients with recurrence received surgery and combined with postoperative chemotherapy. After a median follow-up of 25 months (15, 42 months), 10 patients were disease-free, 3 patients died of the tumor, 1 died of side effects of leukemia chemotherapy, and 1 survived with disease. Conclusion: The incidence rate of Swyer syndrome in patients with MGCT-NDG is about 4.9%; timely diagnosis and bilateral gonadectomy should be emphasized to reduce the risk of reoperation and second carcinogenesis in this population.

Tumor gigante de células germinales: un abordaje quirúrgico / Giant Mediastinal Germ Cell Tumor: a Surgical Approach

Los tumores de células germinales (TCGs) se forman a partir de células embrionarias y generalmente se presentan en pacientes de entre 11 y 30 años de edad. Los TCG pue-den presentarse como tumores extragonadales, siendo el mediastino anterior el sitio más común en el 50 a 70% de los casos. Presentamos a un paciente masculino de 21 años con un tumor sólido mediastinal de 17 x 15 cm que, de acuerdo a la tomografía de tórax (TC), ocupaba toda la cavidad torácica izquierda desplazando el corazón ha-cia la cavidad torácica derecha. El estudio patológico fue reportado por el patólogo co-mo un TCG.

Germ cell tumors (GCTs) are formed from embryonic cells and usually occur in patients between age 11 and 30 years. GCT can present as extra-gonadal tumors, with the an-terior mediastinum being the most common site in 50 to 70% of cases. We present a 21-year-old male patient with a solid mediastinal tumor of 17 x 15 cm that, according to the chest tomography (CT), it was occupying the entire left thoracic cavity moving the heart towards the right thoracic cavity. The pathological study was reported by the pathologist as a GCT tumor.

Burned-out testicular germ cell tumors: a clinicopathological analysis of three cases / 中华病理学杂志

Objective: To investigate the clinicopathological features and possible mechanisms of burned-out testicular germ cell tumors. Methods: The clinical and imaging data, histology and immunophenotypic characteristics of three cases of burned-out testicular germ cell tumors diagnosed at the Ruijin Hospital, Medical College of the Shanghai Jiaotong University, from 2016 to 2020 were retrospectively analyzed. The relevant literature was reviewed. Results: The mean age of the three patients was 32 years. Case 1 had an elevated preoperative alpha-fetoprotein level (810.18 μg/L) and underwent "radical pancreaticoduodenectomy and retroperitoneal lesion resection" for a retroperitoneal mass. Postoperative pathology showed embryonal carcinoma, which needed to exclude gonadal metastasis. Color Doppler ultrasound showed a solid mass of the right testis, with hypoechoic lesion and scattered calcification in some areas. Case 2 was a "right supraclavicular lymph node biopsy specimen." Chest X-ray showed multiple metastases in both lungs. The biopsy showed metastatic embryonic carcinoma and bilateral testicular color Doppler ultrasound revealed abnormal calcifications in the right testicle. Case 3 showed a cystic mass of the right testis with calcification and solid areas. All 3 patients underwent radical right orchiectomy. Grossly, borders of the testicular scar areas were well defined. Cross sectioning of the tumors showed a gray-brown cut surface and single focus or multiple foci of the tumor. The tumor maximum diameter was 0.6-1.5 cm. Microscopically, lymphocytes, plasma cells infiltration, tubular hyalinization, clustered vascular hyperplasia and hemosiderin laden macrophages were found in the scar. Atrophic and sclerotic seminiferous tubules, proliferation of clustered Leydig cells and small or coarse granular calcifications in seminiferous tubules were present around the scar. Seminoma and germ cell neoplasia in situ were seen in case 1, germ cell neoplasia in situ was seen in case 2 and germ cells with atypical hyperplasia were seen in case 3. Immunohistochemistry showed that embryonic carcinoma expressed SALL4, CKpan(AE1/AE3) and CD30, seminoma and germ cell tumor in situ expressed OCT3/4, SALL4 and CD117, and spermatogenic cells with atypical hyperplasia expressed CD99 and SALL4. The Ki-67 positive index was about 20%, while OCT3/4 and CD117 were both negative. Conclusions: Burned-out testicular germ cell tumors are rare. The possibility of gonad testicular metastasis should be considered first for extragonadal germ cell tumor. If fibrous scar is found in testis, it must be determined whether it is a burned-out testicular germ cell tumor. The burned-out mechanisms may be related to the microenvironment of tumor immune-mediated and local ischemic injury.

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.

The relationship of neutrophil to lymphocyte ratio with testicular cancer

ABSTRACT Purpose: To assess the relationship between testicular germ cell tumors (TGCT) and neutrophil to lymphocyte ratio (NLR) and to determine whether this ratio can be used as a serum tumor marker. Material and Methods: Sixty-one patients with testicular germ cell tumors were included into the study. Patients were grouped as localized and non-localized. Histologically patients were categorized as seminoma and nonseminomatous germ cell tumors. Complete blood cell count was measured the day before surgery and at the postoperative 1st month. Preoperative and postoperative mean NLR values were compared. Results: Thirty-six patients (59%) had seminomas and 25 patients (41%) had nonseminomatous testicular cancer. Forty-five patients (73.8%) had localized and 16 patients (26.2%) had non-localized testicular cancer. There was a statistically significant difference between preoperative and postoperative mean NLR of the localized patients (p=0.001) but no such difference was detected for non-localized patients (p=0.576). Nineteen patients with localized seminomas had normal preoperative serum tumor markers. There was a significant difference between preoperative and postoperative mean NLR in this group of patients (p=0.010). Twenty-six patients with localized tumors had preoperative increased serum tumor markers which normalized after orchiectomy. Mean NLR of these patients significantly decreased from 3.10±2.13 to 1.62±0.59 postoperatively (p=0.010). Conclusions: NLR appears to be a useful marker for TGCT. It is successful in predicting localized and non-localized disease in early postoperative period.

Spontaneous gas in a retroperitoneal mass: check the testis!

ABSTRACT Testicular germ cell tumor is the most common cancer in 20-to 35-years-old men. There are known risk factors such as undescended testicle(s) and history of testicular cancer. Most lesions are germ cell tumors with two main subtypes: seminomas and non-seminomatous germ cell tumors.

Tumores ováricos en niñas y adolescentes / Ovarían masses in infant-juvenile age

Introducción. El tratamiento quirúrgico adecuado para las pacientes pediátricas con lesiones ováricas es heterogéneo, y en las niñas es conveniente conservar los ovarios. El objetivo de este estudio es analizar los hallazgos relacionados con un grupo de pacientes a las que se operó por lesiones ováricas. Pacientes y métodos. Un estudio retrospectivo realizado durante 13 años con 56 pacientes menores de 17 años. Estas pacientes se dividieron en 3 grupos según el diagnóstico de patología ovárica: 25 tenían lesiones funcionales (quistes y torsión), 18 tenían lesiones ováricas epiteliales y 13 tenían tumores germinales. Se comparó a estos tres grupos en términos de menarquia, torsión, edad, duración, tamaño, dolor, tumor, vómitos, menstruación irregular, ubicación y tipo de operación. Resultados. Enestosgrupos,fueronmásfrecuentes los quistes foliculares, los cistoadenomas serosos y los teratomas. La media de edad de las pacientes fue de 12,18 ± 4,84 años. Los síntomas y signos más frecuentes fueron dolor (85,7%) e hinchazón (37,5%) en la región abdominopélvica. Se observó torsión en 21 pacientes (37,5%); la media del tamaño del tumor fue de 10,46 ± 6,55 cm. Se realizó una salpingoovariectomía (SO, por sus siglas en inglés) en 38 pacientes y una resección del quiste (CE, por sus siglas en inglés) en 18 pacientes. Se observaron más casos de torsión en las pacientes premenárquicas que en las menárquicas y en el grupo de lesiones funcionales. Se realizaron más resecciones quirúrgicas en el grupo de lesiones funcionales, y más SO en los grupos de lesiones epiteliales y tumores germinales. Conclusión. Se considera que la torsión y las patologías ováricas funcionales son frecuentes en la edad premenstrual, y las lesiones malignas son muy raras en todos los grupos etarios, por lo que se recomienda preservar la fertilidad en la cirugía.

Introduction. The appropraite surgical treatment to pediatric patients with ovarian lesions are heterogeneous and ovarian preservation is desirable in children. The aim of this study is to the discuss findings related to a set of patients who were operated on for ovarian lesions. Patients and methods. A retrospective study carried out in 13 years on 56 patients under the age of 17. These patients were divided into 3 groups according to ovarian pathologic diagnosis: 25 with functional (cyts and torsion), 18 with epithelial ovarian lesions and 13 with germ cell tumours. These three groups were compared in terms of menarche, torsion, age, duration, size, pain, mass, vomiting, irregular menstruation, location and operation type. Results. Follicle cysts, serous cyst adenomas and teratomas were the most common in these groups. The mean age of the patients was 12.18+4.84 years. The most common symptoms and signs were abdominal-pelvic pain (85.7%) and swelling(37.5%). Torsion was seen in 21 patients (37.5%), mean mass size was found to be 10.46+6.55 cm. A salpingo-oophorectomy (SO) was performed in 38 patients and cyst excision (CE) was performed in 18 patients. In premenarcheal cases, torsion was seen more in menarcheal cases and in the functional lesion group. CE was performed more often in the functional and t SO was performed often in the epithelial and germ cells groups. Conclusion. Torsion and functional ovarian pathologies are thought to be common in premenstrual ages and malign lesions are very rare in all age groups so we recommend ovarian protective surgery should be preferred.

Trombocitopenia severa como manifestación de un tumor testicular de células germinales con metástasis cardíaca intracavitaria / Testicular tumor with intracardiac metastasis presenting as severe thrombocytopenia

Se describe el caso de un hombre de 24 años que se presenta con Púrpura trombocitopénico, cuyo estudio ecocardiográfico y tomografía computada demostró masa tumoral ocupando las cavidades derechas. Se evidenció posteriormente un tumor testicular izquierdo con componentes de seminoma y teratoma. Se resecó el tumor testicular y posteriormente el tumor intracardíaco, con normalización del recuento plaquetario.

A 24 year old man presented with severe thrombocytopenia. An intracardiac mass was shown to be a metastasis from a malignant testicular tumor. Resection of the primary tumor and the intracardiac metastasis led to full recovery of thrombocytopenia.

Conservative treatment of ovarian cancer, Safety, ovarian function preservation, reproductive ability, and emotional attitude of the patients in Saudi Arabia

To evaluate the safety, ovarian function preservation, reproductive ability, and the emotional attitude after a conservative surgery for ovarian cancer. This is a retrospective study of women conservatively treated for primary ovarian cancer between January 2000 and December 2010 at King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia. Patient's charts were reviewed for pathology, stage, requirement of adjuvant chemotherapy, and recurrent, as well as menstrual history, and pregnancy after treatment. During follow up the patients were asked 3 questions about their emotional attitude toward their disease. There were 39 patients identified [mean age 22 years]. Thirty-one [80%] patients were presented with stage I and 20 [52%] were Germ cell tumor. Fifteen [39%] patients received initial chemotherapy after primary surgery. Three [8%] patients had recurrent. Thirty-eight [98%] patients retuned to a regular menstruation after treatment. Eight patients [20%] had a normal pregnancy. Of the respondents to the given questions, 10 [44%] patients claimed that their disease did not have any impact on their desire to have children and 12 patients [52%] feared that their ovarian disease could have damage in their reproductive potential. Only 9 patients [39%] had no concerned about the effect of the treatment they received on the offspring. Fertility sparing surgery in ovarian cancer appears to be safe, and a practical treatment option in selected cases with ovarian cancer diagnosis. Most patients can have ovarian preservation after treatment and should not be discouraged from getting pregnant

Sarcoma indiferenciado (embrionario) de hígado en el adulto / Undifferentiated (embryonal) sarcoma of the liver in adults

We report a 48 years old women presenting with a painful abdominal mass and hepatomegaly. An abdominal CAT scan showed a focal lesion of 14 cm diameter in liver segment 4. The patient was subjected to a left hepatectomy with a normal postoperative evolution. The pathological diagnosis of the surgical piece was an undifferentiated sarcoma. Nine months later, a local relapse was detected and the patient died 22 months after the operation.

Introducción: Los sarcomas indiferenciados (embrionario) del hígado (SIEH) son neoplasias infrecuentes. Se presentan principalmente en edad pediátrica y son considerados de mal pronóstico. En adultos, existen 71 pacientes publicados en el mundo, y en nuestro país no hay casos descritos en este grupo etario. Objetivos: Presentar el caso de una paciente adulta portadora de un SIEH, que fue sometida a cirugía resectiva y realizar una revisión del tema. Caso clínico: Mujer de 48 años de edad, con historia de dolor abdominal, masa palpable en epigastrio y hepatomegalia. Marcadores tumorales negativos, tomografía abdominal revela lesión focal de 14 centímetros en segmento 4. Se aborda quirúrgicamente, biopsia rápida revela tumor sólido maligno indiferenciado, y se realiza hepatectomía izquierda. Evolución postoperatoria favorable. Mediante el análisis histopatológico, histoquímico e inmunohistoquímico se diagnostica un SIEH y se corrobora una resección R0. Al noveno mes de evolución se pesquisa foco de recidiva hepática, inicia progresivo compromiso del estado general, falleciendo 22 meses después de la cirugía. Discusión: Actualmente se recomienda resección completa del tumor seguido de quimioterapia coadyuvante, con lo cual se han logrado sobrevidas libres de enfermedad mayores a 5 años. El dar a conocer las experiencias de casos aislados en esta patología tan infrecuente, permitiría aumentar la casuística mundial, mejorar las técnicas de enfrentamiento, y evaluar el impacto de la quimioterapia en el pronóstico.

Morbilidad de la linfadenectomía lumboaórtica laparoscópica en pacientes con tumor testicular no seminomatoso en estadío clínico A / Operative morbidity in laparoscopic retroperitoneal lymph node disection in clinical stage A non-seminomatous testis cancer

Background: Retroperitoneal Lymph node dissection is part of the treatment of patients with nonseminomatous germ cell tumors stage I (NSGCT). Aim: To report the immediate surgical outcomes of patients subjected to laparoscopic retroperitoneal lymph node dissection. Material and Methods: Prospective analysis of the surgical outcomes of 118 patients aged 15 to 44 years, who underwent Laparoscopic Retroperitoneal Lymph Node Dissection (L-RPLND) for NSGCT stage I. Results: Median blood loss was 50 cc (range 10-1.000 cc), median operative time was 120 min. (range 60-300 min). Mean hospital stay was 41.4 hours. Twelve patients (10.2 percent) had operative complications. Ten had vascular injuries (8.5 percent), one had a duodenal injury (0.85 percent) and one had an acute pulmonary edema (0.85 percent). Eight lesions were repaired laparoscopically and three required conversion to open surgery. There was no mortality in this series. Conclusions: L-RPLND had an acceptable complication rate in this series of patients. Most of the complications could be resolved laparoscopically.

Introducción: La linfadenectomía retroperitoneal lumboaórtica es el método de elección en el tratamiento de los pacientes con cáncer testicular no seminoma en estadio A. Este trabajo muestra los resultados inmediatos de una serie de 118 pacientes operados por vía laparoscópica. Material y Método: Se analizan los resultados quirúrgicos de 118 pacientes portadores de un cáncer testicular no seminoma estadio clínico A. Resultados: La media de edad fue de 29 años. La mediana de sangrado fue de 50 cc, el tiempo quirúrgico tuvo una mediana de 120 minutos y el tiempo de hospitalización tuvo una media de 41,4 horas. Complicaciones intraoperatorias ocurrieron en 12 pacientes (10,2 por ciento): 10 lesiones hemorrágicas (8,5 por ciento), 1 lesión duodenal (0,85 por ciento) y 1 edema pulmonar agudo (0,85 por ciento). De las 11 lesiones quirúrgicas, 8 fueron reparadas por vía laparoscópica, requiriendo conversión a cirugía abierta 3 casos. No hubo mortalidad en la serie. Conclusiones: La linfadenectomía lumboaórtica laparoscópica es un procedimiento estandarizado, con una tasa de complicaciones aceptable, la mayoría de las cuales puede ser resuelta en forma laparoscópica.

Linfadenectomía lumboaórtica laparoscópica para cáncer testicular no seminomatoso estadio clínico I / Laparoscopic lumbo-aortic lymph-node dissection in clinical stage i non-seminomatous testicular germ-cell tumors

Reportar la morbilidad y resultados oncológicos de la Linfadenectomía Lumboaórtica por vía laparoscópica (LLA lap) en pacientes portadores de tumor de células germinales no seminomatoso (TCGNS) estadio I, operados en el Hospital Ramón Barros Luco Trudeau. Pacientes y Métodos: Entre octubre de 2005 y agosto de 2008, 29 pacientes con TCGNS estadio I, fueron sometidos a LLA lap. No se realizó selección de pacientes en relación a los hallazgos anatomopatológicos o a la presencia de factores de riesgo. La Linfadenectomía retroperitoneal fue realizada por el mismo cirujano, respetando los límites descritos por Weissbach y Boedefeld. Resultados: De los 29 pacientes operados, el procedimiento fue completado en su totalidad por vía laparoscópica. La pérdida sanguínea promedio fue de 27,7 cc (5–250). No fue necesaria tranfusión sanguínea. El tiempo operatorio promedio fue de 170 min (150–240). El número de ganglios resecados promedio fue de 12,8 (4–25). El tiempo de hospitalización fue de 2 días. Se registró una complicación vascular intraoperatoria, que se manejo satisfactoriamente sin necesidad de conversión. Se preservó la eyaculación anterograda en la totalidad de los pacientes. Cuatro pacientes (13,8 por ciento) tuvieron estadio patológico IIa y recibieron quimioterapia adyuvante con Cisplatino, Etopósido y Bleomicina. Durante un periodo de seguimiento promedio de 19 meses (2-36) ninguno de los pacientes ha presentado recidiva. Conclusión: La Linfadenectomía Lumboaórtica por vía Laparoscópica ha demostrado ser una excelente herramienta de estadificación, la cual ofrece una alternativa mínimamente invasiva a la cirugía convencional abierta. Los resultados de la serie, durante el periodo de seguimiento, demuestran su equivalencia oncológica a la cirugía abierta, sumándose los beneficios de la técnica laparoscópica que incluyen, una menor morbilidad y una mejoría tanto en la visualización intraoperatoria, resultado estético como en la calidad de vida del paciente.

To report the morbidity and oncological results of laparoscopic lumbo-aortic lymph-node dissection (LLA lap) in clinical stage I non-seminomatous testicular germ cell tumors (TCGNS), operated at the Hospital Ramón Barros Luco Trudeau.Patients and Methods: Between October 2005 and August 2008, 29 patients with stage I TCGNS, underwent LLA lap. No patient selection was made in relation to the pathological findings or the presence of risk factors. Retroperitoneal lymphadenectomy was performed by the same surgeon within the limits described by Weissbach and Boedefeld. Results: The procedure was completed in its entirety with laparoscopic procedure. The average blood loss was 27.7 cc (5-250). There was no blood tranfusion. The average operative time was 170 min (150-240). The average number of lymph nodes resected was 12.8 (4-25). The hospitalization time was 2 days. There was an intraoperative vascular complication, which satisfactorily manage without conversion to open surgery. Antegrade ejaculation was preserved in all patients. 4 patients (13.8 percent) had pathological stage II received adjuvant chemotherapy with cisplatin, etoposide and bleomycin. During an average follow-up period of 19 months (2-36) none of the patients presented recurrence. Conclusion: The laparoscopic lumbo-aortic lymph-node dissection has proven to be an excellent staging tool, which offers a minimally invasive alternative to conventional open surgery. The results of the series during the follow-up period, demonstrate oncological equivalence to open surgery, adding the benefits of laparoscopic technique including a lower morbidity and an improvement in intraoperative visualization, aesthetic result and the quality of patient’s life.

Mixed germ cell tumor of the pituitary-hypothalamic region presenting as craniopharyngioma: case report and review of the literature / Tumor misto de células germinativas da região hipotálamo-hipofisária apresentando-se como craniofaringioma: relato de caso e revisão da literatura

Craniopharyngiomas and germ cell tumors (GCT) may affect the pituitary-hypothalamic region during childhood. Although different in origin, their clinical and radiological features may be similar. In this article we present a 5-year-old girl with clinical and radiological findings (computer tomography calcification) that were initially considered as craniopharyngioma. However clinical outcome, blood and cerebral spinal fluid tumoral markers, and results from anatomopathology and immunohistochemistry disclosed a mixed GCT. This case report highlights that some clinical features and radiological findings of pituitary-hypothalamic tumors may be misdiagnosed as craniopharyngioma mainly when there is a mature teratoma with cartilaginous tissue differentiation.

Craniofaringiomas e tumores mistos de células germinativas (TCG) podem acometer a região hipotálamo-hipofisária durante a infância. Embora tenham diferentes origens, as manifestações clínicas e achados radiológicos podem ser semelhantes. Nosso objetivo é relatar o caso de uma paciente de 5 anos de idade, cujas manifestações clínicas e achados radiológicos (presença de calcificações à tomografia computadorizada [TC]) foram inicialmente considerados como provável craniofaringioma. No entanto, a piora clínica progressiva, marcadores tumorais séricos e liquóricos elevados, assim como os resultados do estudo anatomopatológico e imunoistoquímico revelaram tratar-se de TCG. Este caso enfatiza que alguns achados clínicos e radiológicos de tumores da região hipotálamo-hipofisária podem ser erroneamente diagnosticados como craniofaringiomas, principalmente se houver presença de teratoma maduro com diferenciação em tecido cartilaginoso.

Long-term clinical outcome in patients with stage-i nonseminomatous germ cell cancer: a critical review of own treatment modalities in a retrospective study

PURPOSE: The optimal management of patients with clinical stage I non-seminomatous germ cell testicular cancer (NSGCT I) was considered controversial until the European Germ Cell Cancer Consensus Group determined unambiguous treatment strategies. In order to assess the long-term outcome we evaluated the data of patients with NSGCT I. MATERIALS AND METHODS: In a retrospective evaluation, we included 52 patients with a mean age of 26 years (range 15-58) who were treated with different modalities at our department between 1989 and 2003. Mean follow-up was 5.9 years (range 2-14 years). After orchiectomy, 39 patients were treated with chemotherapy, 7 patients underwent retroperitoneal lymph node dissection and 6 men were managed using a surveillance strategy. Survival, recurrence rate and time of recurrence were evaluated. The histological staging and treatment modality was related to the relapse. RESULTS: Tumor specific overall mortality was 3.8 percent. The mortality and relapse rate of the surveillance strategy, retroperitoneal lymph node dissection and chemotherapy was 16.7 percent / 50 percent, 14.3 percent / 14.3 percent and 0 percent / 2.5 percent respectively. All relapsed patients in the surveillance group as well as in the RPLND group had at least one risk factor for developing metastatic disease. CONCLUSIONS: Following the European consensus on diagnosis and treatment of germ cell cancer in patients with NSGCT Stage I any treatment decision must be individually related to the patient according to prognostic factors and care capacity of the treating centre. In case of doubt, adjuvant chemotherapy should be the treatment of choice, as it provides the lowest risk of relapse or tumor related death.

Refining the laparoscopic retroperitoneal lymph node dissection for testicular cancer

Since its initial description, the laparoscopic retroperitoneal lymph node dissection has evolved considerably, from a purely diagnostic tool performed to stage germ cell testicular cancer to a therapeutic operation that fully duplicates the open technique. Herein, we describe the current technique employed at our institution, along with illustrations of all surgical steps, and delineate the refinements of the technique over time.

Nd: YAG laser bronchoscopy; the first two successful cases in Puerto Rico

Two patients underwent bronchoscopic laser photoresection of tumors in the tracheobronchial tree with no mortality or significant morbidity. One patient had an adenoid cystic carcinoma of the trachea and the other a pleomorphic adenoma of left main stem bronchus. These were the first two cases performerd in Puerto Rico at San Pablo Medical Center. Both patients are symptomless at this time. We can conclude that laser is a safe and cost effective altenative in experienced hands to other procedures in properly selected patients with tumors of tracheobronchial tree in Puerto Rico

Blastoma pulmonar: relato de um caso / Pulmonary blastoma: case report

Os autores fazem o relato de um caso e revisäo da literatura acerca do blastoma pulmonar, neoplasia de ocorrência bastante rara e de histogênese discutida

Progonoma melanótico craniano: tumor neuro-ectodérmico melanótico da infância: relato de caso / Melanotic progonoma of the skull: melanotic neuroectodermal tumor of childhood: report of a case

Os autores relatam o caso de uma lactente de cor branca, de 3 meses de idade, que apresentou tumor craniano diagnosticado como progonoma melanótico, também denominado tumor neuro-ectodérmico melanótico da infância. Discutem-se a natureza, os aspectos clínicos e o prognóstico deste raro tumor de localizaçäo preferencialmente craniofacial

Linfadenectomía retroperitoneal: cirugía de estadificación y rescate en los tumores germinales "no seminoma" de testículo / Retroperitoneal lymphadenectomy: computerized staging surgery in non-seminomatous germ-cell testicular

Durante el período comprendido entre el 1§ de abril de 1980 y el 30 de marzo de 1985, fueron operados 27 pacientes con tumores germinales "no seminomas" de testículo a quienes se les realizó vaciamiento retroperitoneal. En 13 de ellos fue como procedimiento de "estadificación" y en los 14 restantes como "cirugía de rescate" luego de tratamientos quimioterápicos. En este último grupo se asoció en 1 metastasectomía hepática, en 3 metastasectomía pulmonar y en otro enfermo además, exéresis de una gran metástasis cervical izquierda. No hubo mortalidad operatoria y 6 pacientes en estadio I sobrevivieron libres de enfermedad entre 5 y 37 meses; 8 con estadio II a-b sobreviven también entre 5 y 37 meses y 3 fallecieron a los 2 meses por progresión de la enfermedad y como consecuencia de la quimioterapia respectivamente. De los 3 enfermos con estadio II c, 1 falleció a los 7 meses y 2 se hallan libress de enfermedad a los 10 y 20 meses. De los 8 con estadio III, 2 fallecieron a los 5 y 6 meses de operados mientras los 6 restantes viven libress de enfermedad entre 9 y 45 meses