Teratocarcinoma con componente de coriocarcinoma: presentación de un caso / Teratocarcinoma with a component of choriocarcinoma: a case report

Los teratocarcinomas representan aproximadamente entre el 3-5 por ciento de los tumores intracraneales en la infancia. Su manifestación clínica puede variar desde un estado asintomático hasta un amplio espectro de síntomas neurológicos, visuales y endocrinos. Se presenta el caso de una paciente femenina de 9 años de edad atendida en el Hospital Pediátrico Pepe Portilla de Pinar del Río, por un cuadro de cefalea, vómitos y pérdida brusca de la visión, a la cual se le realizan varias investigaciones y se le diagnostica un teratocarcinoma con componente de coriocarcinoma. Se opera y logra una evolución satisfactoria(AU)

Teratocarcinomas represent 3-5 per cent of the intracranial tumors in childhood approximately. Their clinical manifestations can vary from an asymptomatic status to a wide spectrum of neurological, visual and endocrine symptoms. A 9 year-old female patient presenting headache, vomits and sudden loss of vision attended to Pepe Portilla Children Hospital, Pinar del Rio. Several medical examinations were performed, diagnosing a Teratocarcinoma with a component of choriocarcinoma; the patient underwent a surgery having a satisfactory evolution(AU)

Unusual autopsy finding in a case of metastatic teratocarcinoma.

An unusual autopsy finding was seen in a young case of metastatic teratocarcinoma of the right testis. The patient presented with features of myocardial ischemia and died of sudden cardiac arrest. At autopsy, the lungs were studded with variably sized metastatic nodules composed of glistening hyaline cartilage. Examination of the coronary arteries revealed complete occlusion of the left anterior descending and left circumflex artery lumina by tumor emboli composed of glistening white chondromyxoid material. Histopathology confirmed the presence of mature hyaline cartilage and chondromyxoid material in tumor emboli. The individual died due to severe coronary insufficiency and sudden cardiac arrest secondary to complete luminal occlusion of the left anterior descending and left circumflex artery lumina by tumor emboli composed of hyaline cartilage.

Seminoma y teratocarcinoma: ¿presentación sincrónica monotesticular como nódulos independientes con distintas histologías? Caracteres ecográficos / Seminoma and teratocarcinoma: synchronic unitesticular presentation as independent nodules with different histologies? Ultrasound characteristics

Objetivo: Describir las características ecográficas, el patrón de vascularización (eco-doppler color) y la posible histogénesis de un caso de presentación sincrónica monotesticular de tumor seminomatoso y teratocarcinoma como nódulos tumorales independientes e histológicamente distintos, en un paciente de 19 años, con una masa testicular de 8 meses de evolución. Métodos: Se realizó estudio convencional ecográfico, eco doppler color y angio-doppler con ecógrafo de alta resolución, analizando los flujos vasculares. Tras la resección del tumor se correlacionaron las secciones macroscópicas e histológicas con los planos ecográficos realizados. Resultados: El paciente mostraba, a nivel testicular derecho, tres nódulos tumorales independientes y bien delimitados: dos de ellos heterogéneos, de 20 y 33 mm de diámetros, con áreas quísticas y calcificaciones. El tercer nódulo era sólido hipoecoico y homogéneo, de 26 mm de diámetro. Todos los nódulos presentaban un aumento de la vascularización con flujos arteriales de baja resistencia. Histológicamente los dos primeros correspondían a teratocarcinomas (teratoma maduro y carcinoma embrionario predominante) y el tercero a un seminoma clásico. Conclusiones: Si bien el seminoma y el tumor mixto de células germinales son habituales, la presentación en un mismo testículo como nódulos independientes, con diferentes histologías es un hecho escasamente referido en la literatura, que nos permite aplicar un modelo histogenético y de correlación ecográfico-patológica en tumores seminomatosos y no seminomatosos. La presencia de cavidades quísticas y calcificaciones groseras se correlaciona altamente con teratoma. En nuestro caso no existen diferencias significativas en el patrón de vascularización con doppler (AU)

Objective: To describe the ultrasound characteristics, vascularization pattern (colour Doppler ultrasound) and possible histogenesis of one case of synchronic uniesticular seminoma and teratocarcinoma as independent tumor nodules, histologically different, in a 19-year-old patient with testicular mass for eight months. Methods: Conventional ultrasound, colour Doppler ultrasound, and high resolution Doppler angiogram were performed, analyzing vascular flows. After resection of the tumor, macroscopic and histological sections were related with ultrasound images. Results: The patient showed three independent, well limited, tumoral nodules in the right testicle: two of them heterogeneous, 20 and 33 mm in diameter, with cystic areas and calcifications. The third nodule was solid, hypoechoic and homogeneous, 26 mm in diameter. All nodules presented an increase in vascularization with low resistance arterial flows. Histologically the first two nodules were teratocarcinomas (predominantly mature teratoma and embryonal carcinoma) and the third classic seminoma. Conclusions: Although seminoma and mixed germ cell tumors are common, their presentation in the same testicle as independent nodules with different histologies is a rarely referred case in the literature, which allows us to apply a histogenetic and ultrasound-pathologic correlation model in seminomatous and nonseminomatous tumors. The presence of cystic cavities and gross calcifications is highly correlated with teratoma. In our case there are not significant differences in the vascularization pattern with Doppler ultrasound (AU)

Disgenesia gonadal pura o Síndrome de Swyer. A propósito de un caso con degeneración tumoral: melanoma / Pure gonad dysgenesia or Swyer sindrome. A case report having tumoral development: melanoma

Se presenta una niña de 14 años de edad, con 10 días de evolución de dolor lumbar, polaquiuria y discreto dolor generalizado a la palpación abdominal. Tras diferentes estudios de laboratorio y de imagen se le halló una masa tumoral que abarcaba toda la gónada derecha con siembra metastática en hígado. Los marcadores tumorales fueron normales. En la exploración quirúrgica, en donde se resecó completamente el tumor primario, se observó además, infiltración peritoneal masiva, ovario izquierdo en estría y un útero de tipo infantil. El diagnóstico presuntivo de disgenesia gonadal pura se confirmó con el estudio cromosómico que reveló ser 46 XY. Los análisis de inmunomarcaje y microscópicos informaron de melanoma primario de la gó- nada resecada. Con dicho diagnóstico se inició una serie de quimioterapia para melanoma avanzado, no obteniéndose respuesta. Se le indicaron cuidados paliativos hasta su fallecimiento ocurrido dos meses después (AU)

A 14 year old girl having 10-days lumbar pain, polaquiuria and moderate pain to palpation is reported. Blood and urine analysis were normal. Abdominal ultrasound scan showed cavity free and solid, rounded, heterogeneous, intrapelvic mass compressing bladder and uterus. Magnetic resonance image was performed showing right gonad compromise with extensive liver and sacro-lumbar spine invasion. Tumoral markers were ruled out. During surgery, primary tumor mass localizad in the right gonad was completely excised. Melanotic peritoneal and hepatic disemination were observed. The patient had left (..) (AU)

Nasopharyngeal teratocarcinosarcoma.

Congenital germ cell tumors are uncommon. The most common site of origin is in the saccrococygeal region. Teratomas arising from the head and neck comprise a small proportion of this entity, and of these, nasopharyngeal lesions are rare. Also known by various synonyms such as hamartoma and hairy polyp, the teratoma is a well-recognized, and generally benign, clinical and histopathological entity. We present a case of a nasopharyngeal teratocarcinosarcoma associated with a cleft palate and the congenital replacement or absence of the ipsilateral Eustachian tube.

[Surgical treatment of intrathoracic compression of respiratory ways caused by mediastinal teratocarcinoma in children]. / Khirurgicheskoe lechenie vnutrigrudnoi kompressii dykhatel'nykh putei, obuslovlennoi teratodermoidnoi opukhol'iu sredosteniia, u detei.

Twelve children with mediastinal teratodermoid tumor, causing intrathoracic compression of respiratory ways (ITCRW) in 8 of them, were treated in clinic. In 75% of children with ITCRW the conduction of special preoperative preparation was necessary. All the patients were operated on. Tumor was removed en bloc or using the fragmentation method. In secondary tracheomalacia the anterior aortopexy for the tracheal lumen preservation was done simultaneously.

Endovascular repair of an aortoduodenal fistula.

PURPOSE: To demonstrate the utility of endovascular stent-graft repair for the management of an unusual aortoduodenal fistula. METHODS AND RESULTS: A 23-year-old man with an aortoduodenal fistula secondary to tumor necrosis was treated with a Corvita endoluminal stent-graft after several failed surgical attempts to repair the defect. At 2-year follow-up, the patient was clinically and radiographically devoid of any evidence of occult stent-graft infection. CONCLUSIONS: This case illustrates the usefulness of endovascular repair for the treatment of a primary aortoduodenal fistula. Endovascular repair should be included in the armamentarium for the management of difficult aortoduodenal fistulas.

Hematologic disorders associated with primary mediastinal nonseminomatous germ cell tumors.

BACKGROUND: The association between primary germ cell tumors of the mediastinum (the space between the lung pleura that contains the heart and other chest viscera) and hematologic malignancies has been described by retrospective analysis of patients treated at individual clinical centers. To better characterize the risk of hematologic disorders in patients with extragonadal germ cell tumors and to describe the clinical and biologic features of the disorders, we studied an unselected population in a large, international, multicenter database. METHODS: Six hundred thirty-five patients treated at 11 centers in the United States and Europe from 1975 through 1996 were evaluated retrospectively. RESULTS: A hematologic disorder was observed in 17 patients with germ cell tumors. All cases developed among the 287 patients with primary mediastinal nonseminomatous germ cell tumors, giving an incidence rate in this group of 2.0% (95% confidence interval [CI] = 1.1%-3.1%) per year over a median follow-up time of 3 years. The risk of developing hematologic disorders was statistically significantly increased in patients with primary mediastinal nonseminomatous germ cell tumors in comparison with the age-matched general population (standardized incidence ratio = 250; 95% CI = 140-405). The median time to onset of hematologic neoplasia was 6 months (range, 0-47 months), and the median survival after diagnosis of the hematologic disorder was 5 months (range, 0-16 months) (two-sided P<.0001, comparing survival from the time of diagnosis of the germ cell tumor of patients with and without hematologic disorders). CONCLUSION: In our study, approximately one in 17 patients with primary mediastinal nonseminomatous germ cell tumors was affected by a hematologic disorder, whereas no cases were seen among 334 patients with other extragonadal germ cell tumors. The hematologic disorder had a statistically significant impact on prognosis, with none of the 17 reported patients surviving for more than 2 years.

The management of inferior vena cava obstruction complicating metastatic germ cell tumors.

BACKGROUND: Inferior vena cava obstruction (IVCO) is a poorly characterized complication of metastatic germ cell tumor (GCT). The authors identified 31 cases to describe the clinical features, radiologic findings, complications, and treatment of this clinical entity. METHODS: Patients with GCT and IVCO were identified from case records of a GCT database. The records of 333 male patients with metastatic GCT (27% with seminoma and 73% with nonseminomatous GCTs) were screened for either clinical or computed tomography (CT) scan evidence of inferior vena cava compression or involvement. RESULTS: Thirty-one patients were identified (9.3% of patients with metastatic GCT). Common presenting symptoms were leg swelling and dilated abdominal wall veins. Approximately 29% of patients had thromboembolic complications and there was a single case of fatal pulmonary embolism. Nine patients had no clinical features at presentation but either had CT scan evidence of IVCO or developed symptoms during treatment. Right-sided testicular primary tumors were associated more frequently with IVCO compared with left-sided primary tumors (14% vs. 4% of cases of metastatic GCT, respectively). All patients had an abdominal mass measuring > 5 cm in maximum transverse dimension. CONCLUSIONS: The authors recommend careful clinical and radiologic evaluation for the presence of IVCO in cases of bulky metastatic GCT. A high index of suspicion must accompany the evaluation of a patient with a right-sided primary testicular tumor and a paracaval abdominal mass measuring > 5 cm in maximum transverse dimension. When IVCO is identified, prophylactic anticoagulation is recommended.

[Giant testicular tumor associated with scrotal gangrene: a case report].

A case of giant testicular tumor associated with scrotal gangrene is reported. A 33-year-old man had a swollen scrotum (15 cm) accompanied with necrosis and a foulodor. The tumor had invaded the scrotum, perineum and the left spermatic cord. Serum alpha-fetoprotein level was 15,487 ng/ml. The chest radiograph revealed multiple metastases. Bilateral high orchiectomies and local resection with a wide margin were performed. The tumor weighed 2.5 kg and the histopathological diagnosis was teratocarcinoma. He was treated with 3 courses of chemotherapy with cisplatin, etoposide and peplomycin. A second-look surgery revealed no viable cancer cells in the residual masses. The patient has been free of recurrence 4 months postoperatively.

Acute myeloblastic leukemia associated with mediastinal nonseminomatous germ cell tumors. Report on two cases.

The demonstrated association with hematologic neoplasms may partially account for the poor survival of patients with mediastinal nonseminomatous germ cell tumors (MNSGCT) compared to patients with testicular and retroperitoneal counterparts. It has been shown that the median interval from the diagnosis of MNSGCT to the diagnosis of the hematologic disorders is 6 months, which contrasts sharply with the average time of 2 to 3 years for the development of therapy-related leukemias. The 2 cases herein described, 1 male and 1 female, developed acute M2 leukemia 4 and 2 years after the diagnosis of MNSGCT. In the second patient (the first female ever described), we cannot exclude a pathogenetic role of the PEB regimen (platinum, etoposide, bleomicin), even though the total dose of etoposide administered has been demonstrated to have a mild leukemogenic potential. This is not the case of the first patient, who did not receive adjuvant chemotherapy after the radical resection of primary MNGSCT and developed the hematologic disorder a few months after local recurrence. In conclusion, the time elapsed from chemotherapy administration does not discriminate the hematologic neoplasms associated to MNGSCT from those related to therapy.

Extragonadal nonseminomatous germ cell tumour with hypercalcemia, masquerading as renal cell carcinoma: a case report.

Humoral hypercalcemia secondary to the production of parathyroid hormone-related peptide has been reported to occur in up to 17% of patients with renal cell carcinoma but has been reported only rarely in patients with other genitourinary cancers and never in patients with testicular or extragonadal nonseminomatous cancers. A 54-year-old man is reported who had an extragonadal nonseminomatous germ cell tumour with hypercalcemia that masqueraded as a renal cell carcinoma with metastases. The hypercalcemia was suspected to be humorally mediated.