[Hypercalcemia in a pediatric patient with ovarian dysgerminoma: A case report]. / Hipercalcemia en paciente pediátrica con disgerminoma ovárico: a propósito de un caso.

Hypercalcemia associated with malignant tumors is a rare entity in pediatrics (0.5-1.3 % of pediatric cancers). It responds to different pathophysiological mechanisms and the typical symptoms shown are usually nonspecific, but potentially serious. We present a clinical case of a 12-year-old patient with a bilateral ovarian dysgerminoma who was diagnosed with severe hypercalcemia at the onset, which required hyperhydration associated with loop diuretics, bisphosphonates and, eventually, the tumor resection surgery that allowed the final resolution of the clinical picture. Despite being a rare hydroelectric disorder in pediatrics, given the potential severity of hypercalcemia, early detection and treatment are important in order to avoid potential short- and long-term complications.

La hipercalcemia asociada a tumores malignos es una entidad poco frecuente en pediatría (0,5-1,3 % de los cánceres pediátricos). Es causada por diferentes mecanismos fisiopatológicos y los síntomas de presentación suelen ser inespecíficos, pero potencialmente graves. Presentamos un caso clínico de una paciente de 12 años con diagnóstico de disgerminoma ovárico bilateral. La enfermedad se presentó con hipercalcemia grave, tratada con hiperhidratación asociada a diuréticos de asa, bifosfonatos y, por último, la resección quirúrgica del tumor, que permitió la resolución definitiva del cuadro. A pesar de tratarse de un trastorno hidroeléctrico poco habitual en pediatría, dada la potencial gravedad de la hipercalcemia, es importante la detección y el tratamiento tempranos, con el fin de evitar complicaciones en el corto y el largo plazo.

Comparison of immunohistochemical profiles of ovarian germ cells in dysgerminomas of a captive maned wolf and domestic dogs.

We characterized the immunohistochemical expression profiles of dysgerminomas from a 16-y-old maned wolf and 13 domestic dogs using the following biomarkers: Sal-like protein 4 (SALL4), octamer-binding transcription factor 3/4 (OCT3/4), placental alkaline phosphatase (PLAP), c-kit, and vimentin. The maned wolf had nonspecific and long-standing clinical signs of lethargy, anorexia, and weight loss, and was euthanized because of poor prognosis. At autopsy, the left ovary was effaced by a 12 × 8 × 6 cm mass, comprised of anaplastic cells with a mitotic count of 20 mitoses in 10 high power fields. Dysgerminomas from 7 of 13 domestic dogs had nuclear expression of SALL4. Dysgerminomas from the maned wolf and 2 domestic dogs had both nuclear and cytoplasmic expression of SALL4. Cytoplasmic expression of PLAP and OCT3/4 was present in dysgerminomas from the maned wolf and 3 (PLAP) or 4 (OCT3/4) domestic dogs. All dysgerminomas expressed vimentin. Membranous c-kit expression was rare in the dysgerminoma from the maned wolf, and variable in dysgerminomas from 4 domestic dogs. A dysgerminoma from a domestic dog had cytoplasmic expression of c-kit. SALL4 is a useful marker to confirm germ cell origin of dysgerminoma in canids.

Hipercalcemia en paciente pediátrica con disgerminoma ovárico: a propósito de un caso / Hypercalcemia in a pediatric patient with ovarian dysgerminoma: A case report

La hipercalcemia asociada a tumores malignos es una entidad poco frecuente en pediatría (0,5-1,3 % de los cánceres pediátricos). Es causada por diferentes mecanismos fisiopatológicos y los síntomas de presentación suelen ser inespecíficos, pero potencialmente graves. Presentamos un caso clínico de una paciente de 12 años con diagnóstico de disgerminoma ovárico bilateral. La enfermedad se presentó con hipercalcemia grave, tratada con hiperhidratación asociada a diuréticos de asa, bifosfonatos y, por último, la resección quirúrgica del tumor, que permitió la resolución definitiva del cuadro. A pesar de tratarse de un trastorno hidroeléctrico poco habitual en pediatría, dada la potencial gravedad de la hipercalcemia, es importante la detección y el tratamiento tempranos, con el fin de evitar complicaciones en el corto y el largo plazo

Hypercalcemia associated with malignant tumors is a rare entity in pediatrics (0.5-1.3 % of pediatric cancers). It responds to different pathophysiological mechanisms and the typical symptoms shown are usually nonspecific, but potentially serious. We present a clinical case of a 12-year-old patient with a bilateral ovarian dysgerminoma who was diagnosed with severe hypercalcemia at the onset, which required hyperhydration associated with loop diuretics, bisphosphonates and, eventually, the tumor resection surgery that allowed the final resolution of the clinical picture.Despite being a rare hydroelectric disorder in pediatrics, given the potential severity of hypercalcemia, early detection and treatment are important in order to avoid potential short- and long-term complications.

[A woman with a pure seminoma and a contralateral intratubular germinal neoplasm. A case report]. / Mujer con seminoma puro y neoplasia intratubular germinal contralateral. Informe de un caso.

BACKGROUND: Androgen insensitivity syndrome is an X-linked disorder, and is characterised by a female phenotype in a chromosomally male individual. It usually occurs in puberty with primary amenorrhoea or as an inguinal tumour in a female infant. In recent years, it is often also diagnosed in fertility clinics in adulthood. OBJECTIVE: The case is presented of a pure seminoma in a woman with the reference diagnosis of inguinal hernia. CLINICAL CASE: A 53 year old woman, who was operated on in 2014 due to a nodule in left groin. Androgen insensitivity syndrome was corroborated, and histopathology reported it as a right testicular seminoma. DISCUSSION: The importance of early diagnosis is discussed, highlighting the consequences of misdiagnosis, and question whether these patients have been adequately treated in the past. The risk of malignant transformation of an undescended testicle increases with age, thus gonadectomy should be performed after puberty, and in some cases hormone replacement therapy.

[Malignant ovarian germ cell tumors. Current status of diagnosis and treatment]. / Tumores malignos de células germinales del ovario. Estado actual de su diagnóstico y tratamiento.

Malignant ovarian germ cell tumors (MOGCT) are extremely aggressive and rapidly growing neoplasms, with a peak incidence occurring in adolescent girls and young women. Its incidence in Mexico is three times higher than the observed in Western countries and the United States. Dysgerminoma, immature teratoma, yolk salc tumor, and mixed germ cell tumors make up more than 90% of all MOGCT.

Cáncer de ovario en el embarazo: reporte de un caso / Ovarian cancer in pregnancy: case report

Antecedentes: La asociación entre cáncer y embarazo es infrecuente, con una incidencia entre 0,02-0,1 por ciento ocupando el cáncer de ovario (CAO) el tercer lugar entre las neoplasias ginecológicas más frecuentemente asociada a la gestación, con tasas entre 1/10.000 a 1/100.000 embarazos. Objetivo: Divulgar un caso clínico de interés para la comunidad médica. Caso clínico: Gestante de 22 años que consulta por presentar dolor abdominal, vómitos, disnea y aumento de circunferencia abdominal. Presenta tumoración parauterina que alcanzaba a hipocondrio derecho, e ingresa con diagnostico de embarazo interrumpido de 12 semanas, tumoración de ovario; síndrome de pseudo Meigs y anemia. Mediante ecografía transabdominal se confirma lesión en fosa ilíaca y flanco derecho multilobulada de 17,3 x 9,9 x 13,7 cm, concordante con imágenes de RMN donde se aprecia como una tumoración ovárica sólido-quística. Se práctica laparotomía y la biopsia por congelación diagnosticó tumor ovario de células germinales (disgerminoma). Se procede a practicar histerectomía total con feto obitado in útero, más salpingo-ooforectomía bilateral. Egresando en buenas condiciones y actualmente en quimioterapia. Conclusión: La coincidencia de CAO durante el embarazo es rara, siendo los digerminomas los tumores malignos más frecuentemente diagnosticados.

Background: The association between cancer and pregnancy is infrequent, with an incidence of 0.02 to 0.1 percent; occupying ovarian cancer (OCA) in third place among the most common gynaecological malignancies associated with pregnancy, with rates between 1/10,000 to 1/100,000 pregnancies. Aim: To disseminate clinical case of interest to the medical community. Case report: A 22 years old pregnant who consulted for abdominal pain, vomiting, dyspnoea and increased abdominal girth. Who has an anexial tumour reaching right upper quadrant, which was admitted with diagnosis of interrupted pregnancy of 12 weeks, ovary's tumour, pseudo Meigs' syndrome and anaemia. Transabdominal ultrasound confirmed space occupying lesion in the right lower quadrant and flank multilobed of 17.3 x 9.9 x 13.7 cm, with concordance in MRI which is seen as a mixed ovarian tumour. Laparotomy was practice and the frozen biopsy was diagnosed as ovarian germ cell tumour (dysgerminoma). It proceeds to total hysterectomy with death fetus in uterus, with bilateral salpingo oophorectomy. Withdrawal in good condition and currently under chemotherapy regimen. Conclusion: The coincidence of OCA during pregnancy is rare, the dysgerminoma are the most frequently diagnosed malignancy.

Utility of OCT3/4, TSPY and ß-catenin as biological markers for gonadoblastoma formation and malignant germ cell tumor development in dysgenetic gonads.

BACKGROUND: Gonadoblastoma (GB) is regarded as an in situ form of germ cell tumor in dysgenetic gonads, and 30% of patients with GB develop a dysgerminoma/seminoma tumor. OBJECTIVE: Determine whether OCT3/4 and ß-catenin are expressed in dysgenetic gonads before GB development and whether TSPY participates in the OCT3/4-ß-catenin pathways in the malignant invasive behavior. METHODS: dysgenetic gonads of Disorders of sex differentiation (DSD) patients with mixed gonadal dysgenesis were analyzed by immunohistochemistry and immunofluorescence for comparison with GB and dysgerminoma/seminoma. RESULTS: Our results suggest that the development of GB is secondary to the interaction of OCT3/4 and TSPY, that ß-catenin does not participate in this process. CONCLUSIONS: The use of this biological markers detects the potential high risk gonads.

Disgerminoma bilateral e hiperplasia endometrial cística com piometra em cadela / Bilateral Dysgerminoma and Cystic Endometrial Hyperplasia with Pyometra in a Bitch

Background: Ovarian tumors are considered rare both in dogs and cats. Germ cell neoplasms correspond to approximately 15% of the cases and can be classified into dysgerminomas, teratomas and teratocarcinomas. Reports of bilateral dysgerminoma in dogs and cats are rare. The aim of this work was to describe a case of bilateral dysgerminoma and cystic endometrial hyperplasia with pyometra in a bitch by presenting the clinical, surgical, pathological and immunohistochemical findings. Case: A 10-year-old female crossbred dog was presented with a history of vomiting, apathy and purulent vulvar discharge. Physical examination revealed hyperthermia and pain was observed on abdominal palpation. Ultrasound examination revealed cystic formation and increased echogenicity inside the uterus. The animal underwent ovariohysterectomy (OHE). Macroscopically, the ovaries were multinodular, spherical to ovoid in shape, measuring 6 cm in diameter, and with a dark red color; they exhibited a firm consistency and a hemorrhagic cut surface with cystic areas and foci of necrotic tissue. At microscopic evaluation of both ovaries, the neoplasm consisted of large and polyhedral cells with amphophilic cytoplasm which were arranged in cords separated by connective tissue. The cells exhibited an anaplastic appearance, with central nuclei, large amounts of granular chromatin and one or more evident nucleoli. Several mitotic figures, often incomplete and with consequent formation of multinucleated cells similar to giant cells, were observed. Immunohistochemical detection of cytokeratin and vimentin was performed for further examination. The ovarian tumor exhibited positive staining for cytokeratin and negative staining for vimentin. Discussion: Most ovarian tumors are asymptomatic and many times are considered incidental findings during spaying surgeries(...)

Disgerminoma bilateral e hiperplasia endometrial cística com piometra em cadela / Bilateral Dysgerminoma and Cystic Endometrial Hyperplasia with Pyometra in a Bitch

Background: Ovarian tumors are considered rare both in dogs and cats. Germ cell neoplasms correspond to approximately 15% of the cases and can be classified into dysgerminomas, teratomas and teratocarcinomas. Reports of bilateral dysgerminoma in dogs and cats are rare. The aim of this work was to describe a case of bilateral dysgerminoma and cystic endometrial hyperplasia with pyometra in a bitch by presenting the clinical, surgical, pathological and immunohistochemical findings. Case: A 10-year-old female crossbred dog was presented with a history of vomiting, apathy and purulent vulvar discharge. Physical examination revealed hyperthermia and pain was observed on abdominal palpation. Ultrasound examination revealed cystic formation and increased echogenicity inside the uterus. The animal underwent ovariohysterectomy (OHE). Macroscopically, the ovaries were multinodular, spherical to ovoid in shape, measuring 6 cm in diameter, and with a dark red color; they exhibited a firm consistency and a hemorrhagic cut surface with cystic areas and foci of necrotic tissue. At microscopic evaluation of both ovaries, the neoplasm consisted of large and polyhedral cells with amphophilic cytoplasm which were arranged in cords separated by connective tissue. The cells exhibited an anaplastic appearance, with central nuclei, large amounts of granular chromatin and one or more evident nucleoli. Several mitotic figures, often incomplete and with consequent formation of multinucleated cells similar to giant cells, were observed. Immunohistochemical detection of cytokeratin and vimentin was performed for further examination. The ovarian tumor exhibited positive staining for cytokeratin and negative staining for vimentin. Discussion: Most ovarian tumors are asymptomatic and many times are considered incidental findings during spaying surgeries(...)(AU)

Germ cell tumours of the ovary.

Germinal cell tumours represent only 2-5% of all cancers of the ovary. However, the characteristics of the tumour and the patients have some special qualities as high rates of healing goes together with a strong desire to keep fertility intact because this condition occurs in female children and adolescent girls. Neither the prognosis nor the treatment of these tumours is homogeneous; the low incidence is the reason it is hard to develop prospective studies for establishing prognostic factors and specific treatments. The introduction of adjuvant chemotherapy into initial surgery has improved the prognosis of these patients. The BEP scheme has proved to be equally efficient and less toxic than PVB, and for this reason it has become the standard scheme despite the fact that no randomised studies have been carried out. The surgical treatment demands the application of the same principles seen in cytoreduction surgery of epithelial cancers of the ovary (maximum possible cytoreduction), though in many cases hysterectomy and double adnexectomy may be obviated. In view of the rarity of these tumours, it is advisable to work within cooperative groups that may have subgroups for the treatment of rare tumours. This will probably be the only way to move forward in the prospective knowledge of prognostic factors for these tumours.

Y chromosome sequences in Turner's syndrome: association with virilization and gonadoblastoma.

UNLABELLED: The presence of Y chromosome fragments in patients with Turner's syndrome is known to increase the risk of gonadoblastoma and virilization. Y chromosome material is detected in up to 6% of patients with Turner's syndrome by karyotype. By DNA analysis, Y chromosome sequences have been reported in 0-60% of patients. The putative gonadoblastoma gene has been mapped to the pericentromeric region of the Y chromosome increasing the interest in studying these sequences. AIMS: 1. To determine the frequency of occult Y chromosome sequences in patients with Turner's syndrome. 2. To analyze the clinical implications of Y sequences detected by karyotype and occult Y sequences. STUDY DESIGN: Cross-sectional study of 58 patients with Turner's syndrome (30 45,X; two with structural anomalies; 26 mosaic [two of whom were 45,X/46,XY]). SRY, TSPY and DYZ3 sequences were amplified by PCR using genomic DNA from peripheral blood. RESULTS: All three Y chromosome sequences were found in one out of 56 patients whose karyotype was not suggestive of having Y chromosome material and in one patient with 45,X/46,Xr(X) karyotype. The patients with the ring chromosome and 45,X/46,XY karyotype underwent surgery and were found to have a gonadoblastoma and dysgerminoma. The four patients with Y chromosome material had non-virilized female genitalia. CONCLUSIONS: Analysis by PCR was more sensitive in detecting Y chromosome sequences than conventional karyotype. The presence of Y material was not associated with virilization. We confirmed the association of Y fragments and gonadoblastoma at an early age.

[Germinal cancer of the ovary and pregnancy]. / Cáncer germinal de ovario y embarazo.

The association of ovarian cancer with pregnancy, in a young patient with acute abdominal syndrome was reviewed. The prognosis is related to early diagnosis and the stage, therefore ultrasonography is the corner stone to detect these tumors allowing a conservative management, mainly when the fertility must be maintained in younger patients. Chemotherapy is an excellent adjuvant in the midtrimester and can be administered without an important deleterious effect on the fetus.

Ovarian germ cell cancer: clinicopathologic analysis and outcome of 31 cases.

AIMS: The aim of the study was to evaluate some clinicopathologic characteristics and the outcome of patients with ovarian germ cell cancer (OGCC) treated with cisplatin-based chemotherapy. METHODS: It was a clinical retrospective study. The clinical charts of 31 patients with OGCC assisted at the Department of Obstetrics and Gynecology of the State University of Campinas, Brazil, from January 1986 to June 1997 were reviewed. RESULTS: Ten patients had dysgerminoma and 21 patients nondysgerminomatous tumors. Women with dysgerminoma and nondysgerminomatous tumors did not present differences regarding surgical staging, age, ascites or residual tumor after the initial surgery. Frozen section, performed in 16 patients, showed some discrepancy with paraffin histology diagnosis in 8 patients. Platinum-based chemotherapy was used in 5/10 patients with dysgerminoma and in 17/21 patients with nondysgerminomatous tumors, with a 5-year survival of 100% for the dysgerminoma and 53% for the nondysgerminomatous group. CONCLUSIONS: Women with dysgerminoma and nondysgerminomatous tumors did not present differences regarding clinicopathologic characteristics. The prognosis for patients with dysgerminoma was better than for those with nondysgerminomatous tumors. Frozen section had a high error rate in diagnosing OGCC intraoperatively.

Uso de BEP y BEPIM en cáncer de células germinales de testiculo: experiencia de diez años en el Instituto Oncológico Luis Razetti / Use of BEP and BEPIM in cancer of germ cell of testis: experience of ten years in the Oncologic Institute Luis Razetti

El cáncer de testículo es la neoplasia más común en los hombres entre 15 y 35 años de edad. Con el advenimiento de la quimioterapia basada en el cisplatino, este tumor se ha convertido en el prototipo de cáncer curable, aún en estadios avanzados. Se revisaron las historias clínicas de 28 pacientes del IOLR con diagnóstico de cáncer de células germinales de testículos que recibieron quimioterapia con los esquemas BEP o BEPIM desde 1986 a 1996. Seis pacientes tenían seminomas clásicos, y 22 tumores no seminomatosos. Nueve pacientes tenían enfermedad catalogada como avanzada, 13 tenían enfermedad moderada y 6 presentaron enfermedad mínima. Diecinueve pacientes recibieron el esquema BEP y 9 el esquema BEPIM. Quince pacientes tuvieron respuesta completa con BEP y 2 con BEPIM

Neoplasia maligna germinal de ovario / Malignant ovarian germ cell tumor

Se realizó un estudio retrospectivo de 157 pacientes portadores de neoplasia maligna germinal de ovario atendidas en el Departamento de Ginecología del INEN (Lima-Perú) durante los períodos enero 1980-diciembre 1993. Estos 157 casos representan el 14.05 por ciento de las NM de ovario de éste periodo. El tipo histológico más frecuente fue el disgerminoma (50.4 por ciento), el tumor del seno endodermal (16.5 por ciento), T.G. mixto (15.9 por ciento), teratoma inmaduro (14.6 por ciento), coriocarcinoma (1.3 por ciento) y cacinoma embrionario (1.3 por ciento). La edad de presentación promedio fue 17.26 semanas y el sítoma principal fue el dolor en 42 por ciento. De los 201 pacientes, 157 (64.4 por ciento) fueron tratados quirurgicamente en nuestra institución (64.4 por ciento), ya sea en forma primaria o re-exploración: 56 pacientes (35.6 por ciento) tuvieron solo cirugía en otra institución. En la re-exploración (50 casos) se encontró sub-estadiaje del orden 30-40 por ciento. El estadio cínico quirúrgico-patológico (101 pacientes) mostró: I (38 pac.), II (4 pac.), III (51 pac.) y IV (8 pac.), 121 pacientes tuvieron un seguimiento global de 31 meses. Analizándose los dos grandes grupos (disgerminoma y no disgerminoma) la sobrevida global de 5 años fue de 81 por ciento con el régimen quimioterápico a base de platino, 42 pacientes tuvieron un tratamiento quimioterápico complementario a base de platino, se realizaron 9 second look y en ninguno se encontró enfermedad con un promedio de seguimiento de 14.5 meses (SEE). Hubieron 8 pacientes (19.1 por ciento) que presentaron resistencia a la Qt (PVB), utilizándose Qt de rescate sin efectividad alguna. Factores pronósticos evaluados: La enfermedad residual (> o < de 2 cm) y el manejo quirúrgico (conservador vs no conservador) no mostraron diferencias significativas. En conclusión estamos ante una neoplasia maligna con mayor incidencia en la segunda década de la vida, quimio-sensible; que gracias a los agentes quimioterápicos actuales nos permiten realizar un manejo conservador con el objeto de mantener la fertilidad de las pacientes. Quedando aún por resolver al 20 por ciento de pacientes quimio-resistentes a los esquemas tradicionales.