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.

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.

A rare case of extragonadal immature teratoma mimicking as subserosal pedunculated myoma

Immature teratomas are usually derived from a malignant transformation of mature teratoma. The pure immature teratoma accounts for less than 1% of all ovarian cancers. It is the second most common germ cell malignancy and accounts for 10-20% of all ovarian malignancies seen in women younger than 20 years of age. Extragonadal origin are extremely rare and the most common extragonadal site of these teratomas is the omentum. We hereby describe a case report of a 29-year-old lady who presented with abdominal pain and her imaging with an ultrasound revealed a mass with features suggestive of a subserosal fibroid. She underwent a laproscopic myomectomy. A histopathologic diagnosis of Immature teratoma was made following her primary surgery. She subsequently underwent a staging laparotomy which was followed by chemotherapy. Immature teratomas predominantly occur in young patients, and preservation of fertility is an important factor in its management. Treatment should be initiated as soon as possible after surgery, preferably within 7-10 days, in those patients who require chemotherapy.

Extragonadal malignant teratoma in a dog - case report / Teratoma extragonodal maligno em cão - relato de caso

Teratomas are neoplasms thought to arise from germ cells which usually occur within gonads and are rarely described on extragonadal sites. The present study reports a case of a 15 year old female mongrel dog with a lumbosacral tumor. The tumor was microscopically composed of embryonic tissues with areas containing atypical undifferentiated cells, leading to the diagnosis of a malignant teratoma. Malignant teratomas are uncommon tumors in domestic animals.(AU)

Os teratomas são neoplasias originárias das células germinativas que têm como sítio principal as gônadas; raramente ocorrem em sítios extragonodais. O presente estudo relata um caso de uma cadela de 15 anos de idade, sem raça definida, apresentando aumento de volume na região lombossacral que ao exame microscópico revelou neoformação composta por tecidos de origem embrionária, com áreas indiferenciadas exibindo atipia celular, características compatíveis com teratoma maligno. Teratomas malignos são incomuns em animais domésticos.(AU)

Primary extragonadal vaginal yolk sac tumour: A case report

@#Yolk sac tumour (YST) or endodermal sinus tumour is rare and typically seen in gonads. Case Report: We described a case of extragonadal vaginal YST in a one year and seven months old girl who presented with vaginal discharge and bleeding, and discuss its differential diagnosis and potential pitfalls in immunohistochemistry. She was found to have a suprapubic mass on examination. The serum alpha fetoprotein was 11919.4 ng/mL. Computed tomography of the pelvis revealed a large 6.4 cm heterogenous pelvic mass. Colposcopic examination of the pelvis showed a fungating vaginal mass that was subsequently confirmed as a yolk sac tumour. Immunohistochemically, the malignant cells were positive toward CKAE1/AE3, AFP and glypican-3, as well as CD117. Discussion: Solid pattern extragonadal vaginal YST may morphologically resemble dysgerminoma that is also CD117 positive, while the glandular pattern YST may have clear cytoplasm and is positive for cytokeratin; hence, may resemble clear cell carcinoma. Being mindful of these potential diagnostic caveats is necessary to prevent misdiagnosis.

Unusual abscess masquerading as poorly differentiated adenocarcinoma of the colon showing characteristics of choriocarcinoma

Extragonadal non-gestational choriocarcinoma (ENC) is an uncommon malignant tumor occasionally found in the gastrointestinal tract. ENC is characterized by a biphasic tumor growth with distinct areas of adenocarcinoma and choriocarcinoma differentiation. Primary choriocarcinoma of the colon is extremely rare, with only 21 cases reported in the literature. Most of the perforation of colorectal cancers occurs in the abdominal cavity, while abdominal wall abscess is rare; the psoas abscess associated with colon carcinoma is even less observed. Herein, we report the case of a 61-year-old female with poorly differentiated adenocarcinoma of the ascending colon and sigmoid, with choriocarcinomatous differentiation, masquerading a psoas abscess formation. Unfortunately, despite the aggressive therapy, the patient's disease rapidly progressed, and she died within 2 months after the diagnosis. The typical morphological pattern, immunohistochemistry, and its correlation with serum β-human chorionic gonadotropin enabled a correct diagnosis.

Teratocarcinoma gigante de mediastino anterosuperior / Giant teratocarcinoma of anterosuperior mediastinum

RESUMEN Los tumores de células germinales no son tan frecuentes, dentro de ellos se encuentra el teratoma como el más usual en ubicación mediastinal, es más habitual en hombres jóvenes cuando es maligno (teratocarcinoma), con una relación por género de 13,5:1, y una edad promedio de 26 años. Se describe la evolución de un hombre de 19 años de edad con un tumor mediastinal, con metástasis pulmonares e infiltración intracardíaca, resecado satisfactoriamente, cuyos síntomas más frecuentes fueron: disnea, dolor retroesternal, fiebre, tos, pérdida de peso y síndrome de vena cava superior. El diagnóstico se realizó por radiografía de tórax y tomografía axial computarizada. Se logró realizar la excéresis total del tumor y las metástasis, así como la tumoración intracardíaca, con reparación de la válvula tricúspide. El paciente ha tenido una evolución satisfactoria durante el primer año de seguimiento.

ABSTRACT Germ cell tumors are not so frequent; among them, teratomas are most common in the mediastinal location. Teratomas in young men are usually malignant (teratocarcinoma), with a gender ratio of 13.5:1, and an average age of 26 years. We describe the evolution of a 19-year-old man with a mediastinal tumor, with pulmonary metastases and intracardiac infiltration, successfully removed; whose most frequent symptoms were: dyspnea, retrosternal pain, fever, cough, weight loss and superior vena cava syndrome. The diagnosis was made by chest x-ray and computed tomography. Total removal of the tumor and metastases was achieved, as well as the intracardiac tumor, with tricuspid valve repair. The patient had a satisfactory outcome during the first follow-up year.

CT findings and clinical diagnosis of extragonadal seminoma / 实用放射学杂志

Objective To investigate the CT imaging findings and diagnostic methods of extragonadal seminoma.Methods The CT imaging findings and the causes of misdiagnosis in 6 cases of extragonadal seminoma before and after CT-guided biopsy were analyzed retrospectively in this study.Results One case of seminoma was found in the right supraclavicular region,mediastinuml,retroperitoneal cavity and retroperitoneal-retroperitoneal cavity,respectively.Two cases located in the pelvic-abdominal cavity.Lesions were substantially isodensity soft tissue masseswith creeping growth,clear boundary,mild to moderate enhancement and peripheral blood vessels embedded in the tumor.All the cases performed CT-guided biopsy.The diagnoses before and after puncture were inconsistent,so the misdiagnosis rate was 100%. Conclusion Extragonadal seminoma occurs randomly in the different location and imaging features are lack of specificity,therefore, it is difficult to diagnose qualitatively.CT-guided biopsy is the most effective clinical method of the diagnosis of extragonadal seminoma.

Primary thymic mediastinal seminoma with florid granulomatous reaction.

Primary mediastinal seminomas are rare tumors. Morphologically, they can be associated with secondary changes in the form of thymic remnants, cystic change, epithelioid granulomas, abundant fibrosis, and syncytiotrophoblast like cells. Ours was an interesting case where a 17‑year‑old adolescent male presented with anterior mediastinal mass. Fine needle aspiration and trucut biopsy of the mass favored tuberculosis; however, he did not respond to antituberculosis treatment regime. He was subjected to video‑assisted thoracoscopic surgery and the histopathological examination along with supportive immunohistochemistry of the mass revealed scattered seminoma cells which were being masked by extensive granulomatous reaction. The aim of this article is to report a case of primary mediastinal thymic seminoma in a young male associated with extensive granulomatous reaction masking the neoplastic population of cells leading to erroneous diagnosis on small biopsy.

CT and MRI features of extragonadal primary seminoma / 实用放射学杂志

Objective To investigate CT and MRI features of extragonadal primary seminoma and improve its diagnostic accuracy.Methods The clinical and imaging features of 8 patients with extragonadal primary seminoma proved by histopathology were analyzed retrospectively.Results In 8 cases,4 cases were located in intracranial regions,4 cases in the abdominal and pelvic cavity.Detailedly,roundlike solid and cystic tumors were seen in the basal ganglia and thalamus in 2 cases.The mass density and signal were inhomogeneous in the necrotic area with slight peritumoral edema and heterogeneous enhancement,and hemorrhage and metastasis of the third ventricle were occurred inone case.Shallow lobulated tumor was occurred in the pineal region forone case,which MRI features included slightly low signal on T1 WI,equal or higher signal on T2 WI,with obvious enhancement and peripheral cystic change.There was one case in the sella area with slight high density on CT routine scan,and on MRI,slight equal signal on T1 WI,equal or higher signal on T2 WI.The pituitary stalk was involved with thickening.Tumor in suprasellar region was obviously enhanced,with a small uniformity enhancement nodule in the mouth of corpus callosum.4 cases were occurred in the abdominal and pelvic cavity,shallow lobulated tumors with varying degrees of necrosis were found on CT with infiltrating growth and slight to moderate enhancement,and swollen lymph nodes were found in retroperitoneal and mesenteric root areas.Conclusion Extragonadal primary seminoma is rare,there are some characteristic imaging findings,which contributes to improve diagnosis accuracy when combining with clinical data.

CT and MRI diagnose of primary extragonadal seminoma / 实用放射学杂志

Objective To explore the CT and MRI performances of primary extragonadal seminoma .Methods The imaging find‐ings of primary extragonadal seminoma in 16 patients proved by histopathology were analyzed retrospectively .The tumor location , size ,contour ,periphery ,density or signal and contrast enhancement patterns were evaluated ,and these were compared with the pathological results .Results The lesions in 16 patients were solitary including the ovoid lesions in 6 and lobulated ones in 10 .They were located in intracranial area in 10 patients ,anterior middle mediastinum in 4 and abdomen in 2 .Mean size of the tumors was(7 .0 ± 5 .6) cm (ranging from 1 .3 cm to 18 .6 cm) .Unenhanced CT in 13 patients showed the soft tissue masses with necrosis in 8 ,stip‐pled calcification in 2 and hemorrhage in 2 .Enhanced CT in 6 patients showed homogeneity in 2 and heterogeneity in 4 ,and the de‐grees of enhancement increased gradually .The plain and enhanced MRI in 10 patients showed iso‐or slight hypointensity on T1WI and slight hyperintensity on T2 WI in 8 lesions ,heterogeneous enhancement in 7 and slight enhancement in one ,peripheral enhance‐ment in 2 due to central hemorrhage .Pathology showed most of the lesions were well‐vascularized with inner cystic change .Tumor stroma was divided by the fibrovascular tissue .Conclusion The imaging findings of primary extragonadal seminoma are not specific , CT ,MRI can demonstrate the invasive extent accurately .

Pelvic mature cystic teratoma with neuroendocrine carcinoma: Report of a rare association and review of literature.

Extra-gonadal malignant transformation of teratoma is rare and there are only a few reports available citing malignancy arising in the non-germ cell components. We hereby report a case of a 35-year-old female, who presented with lower backache with a radiologically identifi able mass lesion in the pre-sacral region. Clinical and radiological fi ndings suggested the possibility of a cystic teratoma. Histopathological examination and relevant immunohistochemical tests detected a mature cystic teratoma with features of a grade 2 neuroendocrine tumor in it. Like the index case, most of the previously reported cases of teratoma with malignant transformation of the somatic components were found in extra-gonadal site. This case is being reported to emphasize that any extra-gonadal mass in reproductive age group, even if it appears radiologically and per-operatively benign, must be subjected to histopathological examination to rule out possibility of malignant transformation of the germ cell or non-germ cell components.

Primary extragonadal yolk sac tumor:a clinicopathologic analysis of 40 cases / 临床与实验病理学杂志

Purpose To investigate the clinicopathological features, histogenesis, morphological characteristics, immunophenotypes and differential diagnosis of primary extragonadal yolk sac tumor ( eYST) . Methods Clinicopathological data, morphological charac-teristics and immunophenotypes results of 40 cases of eYST were retrospectively studied and the relevant literatures were reviewed. Re-sults All 40 patients, which included 24 male and 16 female, aged from 6 months to 42 years with a 12 years average age and 17 (42.5%) cases were over 12 years old. Mediastinum, sacrococcygeal, retroperitoneal, pineal gland and vagina were involved in 16 (40.0%), 12(30.0%), 5(12.5%), 4(10.0%) and 3(7.5%), respectively. All 40 cases included 32(80.0%) cases pure YSTs, while other 8(20.0%) cases contained other one or two types of germ cell tumor (GCT) components. Conclusions Primary eYST is rare, mediastinum and sacrococcygeal are the most common anatomic sites for eYST, and these mediastinal tumor patients are overwhelmingly confined to adult males, whose average age are significantly older than that in sacrococcygeal, retroperitoneal, pineal gland and vaginal tumor patients (P<0.05), while other sites eYST are restricted to prepubertal children. Adult eYST contain other types of GCT components in some cases, and children's counterparts are always pure YST. Extragonadal eYST manifestate pleomorphic histological features, which combine with immunohistochemical markers is definite value for diagnosis, differential diagnosis.

Tumores de células germinales del mediastino: experiencia con 29 pacientes / Mediastinal germ cell tumors: surgical experience with twenty nine patients

Objetivo: revisar e informar la experiencia adquirida con el manejo de tumores de células germinales del mediastino, en el Hospital Calderón Guardia. Metodología: previa autorización acumulada en la base de datos del Servicio de Cirugía de Tórax y los expedientes clínicos de 29 pacientes con tumores de células germinales del mediastino, operados entre 1986 y 2012. Resultados: se encontró mayor frecuencia en hombres jóvenes con una relación por género de 13.5:1, y edad promedio de 26 años. La localización correspondió en 28 casos al mediastino antero superior y 1 al medio. La presentación en 27 casos fue una radiografía de tórax con ensanchamiento del mediastino, uno con derrame pleural y uno con derrame pleural y mediastino ensanchado. Los síntomas más frecuentes fueron: disnea, dolor retrosternal, fiebre, tos, pérdida de peso, síndrome de vena cava superior, hemoptisis y ginecomastía en un varón con coriocarcinoma. La muestra para diagnóstico se obtuvo por mediastinotomía en 14 casos, toracotomía en 8, videotoracoscopía en 4 esternotomía en 1, mediastinoscopía en 1 y broncoscopía en 1. Conclusión: los tumores germinales del mediastino son infrecuentes; la extirpación quirúrgica es el tratamiento de elección para los técnicamente resecables y se acompaña de curación de los benignos. En los malignos, la aparente resección completa no es garantía de curación, por lo que debe considerarse la quimioterapia adyuvante, que demostró ser de gran utilidad, por cuanto varios tumores parcialmente resecados se curaron con quimioterapia, y algunos con aparente resección completa recidivaron...

Tumor extragonadal de células de la granulosa / Extragonadal granulosa cell tumor

Los tumores extragonadales de la granulosa son extremadamente infrecuentes. Originados a lo largo del recorrido de las gónadas en formación durante su evolución embriológica, se presentan fundamentalmente en la mujer perimenopáusica. Son por lo general tumores de bajo grado de malignidad con recurrencia frecuente. Se estudió a una paciente de 51 años que evolucionó durante más de 10 años con muy pocos síntomas y un crecimiento lento hasta alcanzar 20 cm en el retroperitoneo. La intervención realizada fue satisfactoria(AU)

Extragonadal granulosa cell tumors are extremely infrequent. Originated along the path of the gonads in their formation during embryologic evolution, they are mainly present in the perimenopausal woman. Generally, they are tumors with a low degree of malignancy and frequent recurrence. A 51 year - old patient was studied who, for more than 10 years, presented few symptoms and a slow growth, reaching 20 cm in the retroperitoneum. The surgical procedure performed was satisfactory(AU)

Extragonadal Intraabdominal Mixed Germ Cell Tumor with Liver Metastasis: Successful Treatment and Long-term Follow-up

No abstract available.

Extra-gonadal Germ Cell Tumour – What About the Testis!

Extra-gonadal germ cell tumours (EGGCT) are rare. Therefore further investigations of the testis is aimed at sourcing a possible primary origin of gonadal tumour. Over the years, various case series on EGGCT have been reported questioning its true nature as in a majority of them, a primary source is found in the testis, thus representing a metastatic gonadal tumour. The testis pathology could be either a true germ cell foci, an intra-tubular epithelial neoplasia or an area of fibrosis, indicating a ‘burnt out tumour’. We report a 39-year-old male who underwent laparotomy and excision of a retroperitoneal tumour. Histopathological examination revealed retroperitoneal lymph node of mixed germ cell tumour origin. Clinical and ultrasound examination of bilateral testis was normal. The patient refused orchidectomy or a testicular biopsy. He underwent four cycles of bleomycin, cisplatin, and etoposide with no evidence of tumour recurrence on follow up and remains disease free after 12 months of diagnosis. A literature review of EGGCT, its relation and factors relating with future testicular tumour is presented.

Seminomatous Extragonadal Germ Cell Tumor with Complete Obstruction of the Superior Vena Cava Responding to Intensive Chemotherapy / Journal of Rural Medicine

A 20-year-old man was admitted to our hospital with persistent cough and dyspnea. He had bilateral distention of the jugular veins, and swollen lymph nodes were palpable in the right subclavicular region. Plain X-ray and computed tomography (CT) of the chest showed a solid soft tissue mass in the upper mediastinum, with leftward displacement of the trachea and complete obstruction of the superior vena cava. Mediastinal radiotherapy (1.8 Gy/day) and methylprednisolone (100 mg/day) were started immediately. Biopsy of the right subclavicular lymph nodes revealed metastatic seminoma. The patient was referred for chemotherapy, which was performed with a combination of cisplatin, bleomycin and etoposide (BEP). A partial response was observed after completion of 3 cycles of chemotherapy, but there was no further tumor shrinkage after additional salvage chemotherapy. The patient is being followed up on an outpatient basis and has been free of recurrence for 32 months after intensive treatment.

Suprasellar germ cell tumor in a dog

A case of suprasellar germ cell tumor is described in a 6-year -old Airedale Terrier bitch that presented behavioral changes and abrupt onset of blindness. The neoplasm compressed the ventral surface of the cerebrum from the level of basal ganglia to the mesencephalon. Histologically the neoplasm consisted of nests and trabeculae of round to polygonal cells that occasionally surround tubules and cysts formed by columnar cells. Neoplastic cells are immunopositive for cytokeratin and alpha-fetoprotein. The diagnosis was based on location, histological appearance and on the immunohistochemical staining.

É descrito um caso de tumor de células germinativas supra-selar em numa cadela Airedale Terrier de seis anos de idade, que apresentou transtornos do comportamento e aparecimento abrupto de cegueira. O neoplasma comprimia a superfície ventral do cérebro desde a altura dos núcleos basais até o mesencéfalo. Histologicamente, o neoplasma consistia de ninhos e trabéculas de células redondas ou poligonais que ocasionalmente arranjavam-se ao redor de túbulos e cistos formados por células colunares. As células neoplásicas foram positivas na imunoistoquímica para citoqueratina e a-fetoproteína. O diagnóstico foi feito com base na localização do tumor, no seu aspecto histológico e nos resultados da marcação imunoistoquímica.

Tumor germinal no seminomatoso del mediastino con invasión pulmonar / Mediastinal non seminomatous germ cell tumor with pulmonary compromise

Los tumores germinales extragonadales representan entre el 1 y 2.5% de los tumores de células germinales (TCG), siendo el mediastino la segunda localización en frecuencia luego de las gónadas. Se presenta el caso de un paciente masculino de 29 años de edad que consulta por tos irritativa de cinco meses de evolución. Se realizaron radiografía, tomografía computada (TC) y resonancia magnética (RM) de tórax y ecografía testicular. Los hallazgos por imágenes, sumados a la presencia de marcadores tumorales elevados (alfa-fetoproteína y gonadotrofina coriónica humana), confirmaron el diagnóstico de TCG extragonadal, avalado posteriormente por la cirugía y la anatomía patológica.

The prevalence of extragonadal germ cell tumors is only 1- 2.5% of all germ cell tumors. The mediastinum is the second most common site affected. We present the case of a 29 years old male pacient, with a persistent cough dating back to five months. We performed chest X-R, thorax CT and MRI and testicular US. The findings of this images besides the presence of elevated levels of alpha-fetoprotein and beta-human gonadotropin confirm the diagnosis of extragonadal germ cell tumor.