Synchronous bilateral testicular cancer with discordant histopathology occurring in a 20-year-old patient: A case report and review of the literature.

INTRODUCTION: Bilateral testicular tumors are very rare, accounting for 1%-5% of all testicular germ-cell tumors (TGCTs). The vast majority of primary bilateral TGCTs are metachronous, with synchronous tumors comprising approximately 0.5%-1% of all cases. Those occurring synchronously share mostly the same histological pattern, predominantly seminoma, with synchronous bilateral TGCTs (SBTGCTs) with discordant subtypes being extremely rare. CASE PRESENTATION: We present the case of a 20-year-old male complaining of a palpable painless right testicular mass incidentally noticed during sexual intercourse. Ultrasonography (US) and magnetic resonance imaging (MRI) of the scrotum demonstrated bilateral testicular lesions, while staging with contrast-enhanced computed tomography (CT) exhibited normal findings. Right radical orchiectomy and left testis-sparing surgery (TSS) with concomitant onco-testicular sperm extraction (onco-TESE) were initially performed. Histology of the right testis revealed a mixed germ-cell tumor, consisting of seminoma and embryonal carcinoma, while that from the left testis disclosed embryonal carcinoma and intratubular germ-cell neoplasia unclassified (IGCNU) infiltrating the surgical margins. Hence, left orchiectomy was subsequently scheduled with histology unveiling IGCNU in the greatest part of the remaining testicular parenchyma. Following adjuvant chemotherapy, with bleomycin, etoposide, and cisplatin (BEP), the patient received testosterone replacement therapy and remained free of recurrence at an 18-month follow-up. CONCLUSION: This case highlights both the rarity of a bilateral testicular tumor's synchronous appearance and its extremely infrequent discordant histopathology. A comprehensive review of the major series of SBTGCTs with discordant histology cited in the literature is additionally presented.

A Case of Concurrent Disseminated Coccidioidomycosis and Embryonal Carcinoma When Lice and Fleas Coexist.

Coccidioidomycosis (CM) is a fungal infection endemic to the southwestern United States with a wide range of clinical presentations depending on the infected organ systems. Most infections are asymptomatic. Coccidioidomycosis causes a primary pulmonary infection and when symptoms occur, they most often resemble community-acquired pneumonia. One percent of cases disseminate, typically via hematogenous or lymphatic spread. It is in these cases that more severe symptoms may present and potentially overlap with those characteristics of other systemic illnesses. This is a case of CM disseminated to lymph nodes in a 24-year-old man with concomitant metastatic embryonal carcinoma. It is difficult to identify the primary etiology for many components of this patient's presentation, including diffuse lymphadenopathy and multiple pulmonary nodules. Furthermore, the relationship between these 2 concurrent disease processes is not entirely clear. Factors that may contribute include the well-known phenomenon of locus minoris resistentiae (LMR) or potentially a shared immune failure between infectious organisms and malignant cells.

Paraneoplastic Limbic Encephalitis in a Patient with Primary Well-differentiated Teratoma and Metastatic Poorly Differentiated Embryonal Carcinoma.

Testicular tumors account for 1-2% of all tumors in men, with 95% of these being germ cell tumors. Paraneoplastic limbic encephalitis is a rare sequela of testicular tumors associated with anti-Ma2 and KLH11 antibodies. The most effective treatment for paraneoplastic limbic encephalitis is treatment of the primary malignancy. We report a 41-year-old male that presented to the emergency department with episodic alteration of consciousness and memory disturbances. Negative neurologic evaluation and imaging led to concern for a paraneoplastic process from a distant malignancy. CT imaging revealed an enlarged, necrotic para-aortic lymph node and subsequent ultrasound demonstrated a right-sided testicular mass. Right radical orchiectomy was performed. Microscopically, the mass consisted of mixed respiratory epithelium, gastrointestinal glands, and squamous epithelium with keratinization consistent with a post-pubertal testicular teratoma with associated in situ germ cell neoplasia. Resection of the para-aortic mass revealed large anaplastic cells with epithelioid features, nuclear pleomorphism and frequent mitoses. Immunostaining was positive for Pan-Keratin and OCT4, consistent with poorly differentiated embryonal carcinoma. Resection of the primary and metastatic disease, as well as treatment with corticosteroids, resulted in resolution of the encephalitis. This presentation of severe neurological disturbances in the setting of a metastatic mixed non-seminomatous germ cell tumor represents a rare presentation of paraneoplastic limbic encephalitis.

Brentuximab-vedotin maintenance following chemotherapy without irradiation for primary intracranial embryonal carcinoma in down syndrome.

BACKGROUND: Germ cell tumors (GCT) are the most common central nervous system (CNS) tumors in individuals with Down syndrome. Patients with Down syndrome treated with CNS irradiation are at increased risk of developing cerebrovascular complications such as moyamoya disease. Embryonal carcinoma components are recognized to be more resistant to conventional chemotherapy and radiotherapy and confer a very poor prognosis. CD30 is a member of the tumor necrosis factor-receptor superfamily. CD30+ has a limited expression in normal cells but is the defining marker for embryonal carcinoma. Brentuximab-vedotin is a novel antibody-drug conjugate consisting of the chimeric anti-CD30 antibody conjugated to an anti-tubulin synthetic analog monomethyl auristatin E. METHODS: A retrospective review of the patient's records was conducted in September 2017. RESULTS: We report upon our management of a teenage girl with Down syndrome and a suprasellar pure embryonal carcinoma utilizing an intensive chemotherapy regimen followed by brentuximab-vedotin without irradiation. The patient received two cycles of carboplatin and etoposide interspersed with one cycle of cyclophosphamide and etoposide for induction followed by three cycles of marrow-ablative thiotepa and carboplatin rescued by autologous hematopoietic stem cell. Finally, She received six cycles of intravenous brentuximab-vedotin. The patient continues without evidence of recurrent tumor by MRI and tumor marker surveillance 24 months since diagnosis, with no adverse sequelae of her treatment. CONCLUSIONS: Brentuximab-vedotin may provide a selective and safe alternative (or adjunct) to radiotherapy in the management of patients with CD30-positive CNS embryonal carcinoma, especially for those patients at high risk of developing irradiation-related complications.

Hematoma retroperitoneal como presentación de cáncer testicular / Retroperitoneal hematoma debut as testicular cancer

Resumen Introducción: El hematoma retroperitoneal (HR) es una enfermedad infrecuente con una elevada morbimortalidad, siendo complicado cuando se presenta con dolor y shock hipovolémico. Presentación del caso: Paciente del sexo masculino, de 20 años de edad, sin antecedentes mórbidos. Ingresa en Urgencias por dolor abdominal en el flanco izquierdo, irradiado a dorso y testículo ipsilateral, de 6 h de evolución, de inicio súbito e intensidad severa; el paciente está pálido, hemodinámicamente estable, sin signos de irritación peritoneal. Se solicita pielo-TC por sospecha de litiasis ureteral, que muestra un extenso HR, probable aneurisma aórtico roto. Una angio-TC informa HR adyacente y anterior a psoasilíaco izquierdo, de20 × 11 × 8,5 cm, volumen 972 cc, adenopatías retroperitoneales paraaórticas bilaterales sangrantes y múltiples nódulos pulmonares bilaterales indicativos de diseminación secundaria. Se constata testículo derecho duro, de tamaño normal, eco testicular con masa sólida quística, que indica de lesión orgánica. Discusión: Trauma y enfermedad tumoral son las principales causas de HR. El cáncer testicular suele presentarse en pacientes jóvenes, requiriendo una pronta derivación y estudio debido a su rápida progresión. En nuestro caso, el HR fue un hallazgo imagenológico, destacando que el sangrado de un conglomerado de adenopatías es anecdótico.

Abstract Introduction: Retroperitoneal hematoma (RH) is a rare disease with high morbidity, being complicated when presented with pain and hypovolemic shock. Case report: Male, 20 years old, no morbid history. Arrive to Emergency Service for abdominal pain in the left flank radiating to the back and ipsilateral testis, 6 h of evolution, sudden onset, high intensity; pacient pale, hemodynamically stable without signs of peritoneal irritation. Pielo-TC is requested on suspicion of ureteral stones showing extensive RH, likely ruptured aortic aneurysm. CT angiography reports RH and adjacent preceding left iliopsoas, 20 × 11 × 8.5 cm, volume 972 cc, retroperitoneal bleeding bilateral para-aortic lymphadenopathy and multiple bilateral pulmonary nodules suggestive of secondary spread. Hard right testicle with normal size, testicular ultrasound pointing solid cystic mass, suggestive of organic lesion. Discussion: Trauma and tumor pathology are the main causes of RH. Testicular cancer usually occurs in young patients, requiring early referal and study because of its rapid progression. In our case, the HR was an imaging finding, highlighting that the bleeding of a cluster of lymph nodes is anecdotal.

Clinical and Radiologic Features of Pediatric Basal Ganglia Germ Cell Tumors.

BACKGROUND AND OBJECTIVE: Pediatric basal ganglia germ cell tumors (GCTs) represent a rare subset of tumors about which little is known. We aimed to summarize the clinical features and radiological findings of this special subgroup of GCTs. METHODS: From January 2010 to January 2015, 12 pediatric patients with basal ganglia GCTs were treated in our hospital. The clinical features, radiologic findings, diagnosis, treatment, and outcome of these patients were analyzed retrospectively. Our institutional diagnostic principle and treatment strategy of this disease were discussed. RESULTS: GCTs accounted for 25.5% of all the pediatric basal ganglia tumors treated in our hospital. There were 9 male and 3 female patients with a mean age of 11.5 ± 2.1 years. The most common symptom was progressive hemiparesis (n = 9, 75%). The radiologic findings showed that the lesions predominately located in caput of caudate nucleus (n = 9, 75.0%), followed by lenticular nucleus (n = 3, 25.0%). Hemiatrophy was commonly observed (n = 8, 66.7%). Eight patients were diagnosed as having germinomas, and 4 patients as having nongerminomatous germ cell tumors. During the follow-up period, preoperative neurologic dysfunctions improved in 7 patients and remained stable in 3. Two patients developed new onset of neurologic dysfunction after the treatment. Two patients suffered from tumor recurrence. CONCLUSIONS: GCTs are not as rare as considered in pediatric basal ganglia tumors. They bear some distinctive clinical and radiologic features, which can help with the accurate diagnosis and successful management of such tumors.

A CASE OF ADVANCED NON-SEMINOMATOUS TESTICULAR GERM CELL TUMOR ACCOMPANIED PANIC DISORDER.

We report a 33-year-old male with a left advanced non-seminomatous testicular germ cell tumor (NSGCT) accompanied panic disorder. He had experienced palpitation and hyperpnea in crowds in his twenties. He was admitted to the Department of Otorhinolaryngology with the chief complaint of left neck swelling. 18F-fluorodeoxy glucose positron emission tomography/computed tomography (FDG-PET/CT) demonstrated left neck, left supraclavicular, left axillary, and paraaortic lymph node (LN) swelling and left testicular swelling. He was referred to our department. The left testis had enlarged to the size of a fist. He rejected admission at that time, but next day, he was taken to our hospital by an ambulance because he lost consciousness at home. No abnormalities were found in the brain CT and electrocardiogram. He was admitted and left high orchiectomy was performed. The human chorionic gonadotropin (HCG) level had elevated to 9,717 IU/L and alpha fetoprotein level (AFP) had elevated to 427 ng/ml. The histopathological diagnosis was tumors of more than one histological type, mixed forms: seminoma and embryonal carcinoma.He had palpitation and hyperpnea after admission and was diagnosed with panic disorder by a psychiatrist. Psychotropic drugs (fluvoxamine maleate 50 mg/day, alprazolam 0.8 mg/day) were prescribed and the panic attacks disappeared afterwards. The psychiatric social worker supported his mind side. Bleomycin, etoposide, and cisplatin (BEP) therapy was performed for 4 courses. He put on a blanket to his face and came to avoid a conversation with other people during the chemotherapy. He was diagnosed with depression and psychotropic drugs were increased (fluvoxamine maleate 50→75 mg/day, alprazolam 0.8→1.2 mg/day) in quantity.Lymphadenectomies for LN metastases were performed and their histopathological examination revealed the existence of viable embryonal carcinoma in the supraclavicular LN. Etoposide, ifosfamide, and cisplatin (VIP) therapy was performed for 2 courses.The pateint has remained alive without tumor recurrence. Psychotropic drugs were reduced and the recent drug is fluvoxamine maleate 25 mg/day.

[Intracranial remote epidural haematoma as a complication after resection of an occipital lobe metastatic tumour from a testicular embryonal carcinoma ­ a case report].

We present the case of a patient who suffered from intracranial epidural haematoma in the left fronto -temporo -parietal region as a complication after left parieto -occipital craniotomy and a resection of a metastatic lesion from a testicular embryonal carcinoma to the left occipital lobe. We also discuss possible causes of this complication.

Pulmonary artery stenosis due to embryonal carcinoma with primary mediastinal location.

A 29-year old man was admitted to the intensive care unit after losing consciousness. On physical examination, a loud systolic murmur over the heart was found. Echocardiography revealed narrowing of pulmonary artery with high pressure gradient. Computed tomography of the chest revealed the presence of large tumour localised in the upper anterior mediastinum. Due to the risk of total closure of the pulmonary artery, interventional mediastinotomy was performed and diagnosis of carcinoma embryonale was established. Subsequent chemotherapy (BEP regimen) has brought regression of tumour and significant improvement in haemodynamic parameters (relief of pressure gradient in pulmonary artery). During the second surgery, the resection of all accessible tumour mass together with marginal resection of the right upper lobe was performed. No signs of cardiac or great vessels infiltration was found. Histopathologic examination revealed the necrotic masses and neoplastic foci diagnosed as teratoma immaturum. In a four-month follow-up the patient's condition remained good. The patient is still under the care of both oncological and cardiological specialists. Thus far he has not required further chemotherapy. Holter ECG monitoring revealed no arrhythmia, but the patient is still treated with mexiletine. The patient is planning to return to work.

A case of mediastinal embryonal carcinoma successfully treated by integrative therapy.

Mediastinal embryonal carcinoma is rare, and the life prognosis of this disease is assumed to be relatively short. We encountered a case of mediastinal embryonal carcinoma for which we could perform radical surgical resection. The patient was male, aged 16 years, and acutely aware of back pain. Because the pain increased during the same year, he visited a local doctor, and an expanding neoplastic lesion was detected in the right thoracic wall by computed tomography (CT). Then he was referred to our institution. Magnetic resonance imaging (MRI) showed a dumbbell type tumor (Eden type 3) at the Th7/8 level. Malignant disease was suspected, so the authors planned and performed CT-guided biopsy. The result showed that this tumor pathologically corresponded to malignant peripheral nerve sheath tumor (MPNST). Therefore, chemotherapy was considered the main treatment. After 2 courses of chemotherapy, the tumor size decreased dramatically. The authors thought that radical resection is possible if there is no intrathoracic tumor dissemination as a result of a favorable response to chemotherapy. We thus perfomed surgical resection after we confirmed by a thoracoscopic exploratory thoracotomy that there was no intrathoracic tumor dissemination. Pathological findings were consistent with an embryonal carcinoma. Both the cutting ends of the thoracic wall and the epidural lateral sides of the excised lesion were negative for tumor cells. There is no image finding from the MRI and PET-CT suggesting metastasis or recurrence in the MRI and PET-CT 18 months after surgical resection. Therefore, the long-term vital prognosis can be expected in this patient.

Gonadotrophin-independent precocious puberty associated with later diagnosis of testicular embryonal carcinoma.

BACKGROUND: Testicular tumours are very rare in children. Germ cell tumours (GCTs) account for the majority of testicular tumours in young people, and embryonal carcinomas are a common component of GCTs in adolescents. CASE PRESENTATION: A 9.8-year-old boy presented with the development of pubic and facial hair over a period of 2 years. He had a growth spurt and examination revealed pubertal staging of G4 P4 A2 with a 6-mls testis on the right and a 4-mls testis on the left. Investigations revealed suppressed gonadotrophins, a testosterone concentration of 10.3 nmol/l and normal 17-hydroxyprogesterone and adrenal androgen levels. Tumour markers were negative. Following treatment with anastrazole, his height velocity slowed down. At the age of 13.7 years, his treatment was stopped. At the age of 14.8 years, he presented with a grossly enlarged right testis and elevated beta human chorionic gonadotrophin (>1,400 IU/l). He underwent right orchidectomy and histology revealed an embryonal carcinoma with no vascular invasion. Analysis of luteinizing hormone/choriogonadotrophin receptor revealed no mutation. CONCLUSION: We present a case of testicular embryonal carcinoma in a boy who had presented 5 years before with features suggestive of gonadotrophin-independent precocious puberty.

Echocardiography in imaging an extremely rare cause of extrinsic pulmonary stenosis: rapidly progressive primary mediastinal embryonal carcinoma.

We report a case of a young man with a very rare cause of atypical chest pain in whom primary mediastinal embryonal carcinoma causing extrinsic pulmonary stenosis was diagnosed with echocardiography and computed tomography. This patient illustrates an unusual presentation of the very rapid progression of the tumor in as little as 6 months. The patient underwent surgical resection and was successfully treated with adjuvant chemotherapy.

Evolución natural del carcinoma embrionario / Natural progression of embryonal carcinoma

OBJETIVO: Presentamos el caso de un varón de 37 años con un carcinoma embrionario en una forma poco frecuente en su debut, y que representa la historia natural de la enfermedad sin recibir atención sanitaria así como la efectividad del tratamiento con quimioterapia.MÉTODOS: Paciente de 37 años con antecedentes de retraso mental desde el nacimiento, acude a urgencias por presentar una masa escrotal ulcerada maloliente de meses de evolución. Tras biopsia es diagnosticado de carcinoma embrionario pT4; en el estudio de extensión se evidencian múltiples nódulos pulmonares compatibles con metástasis.Recibió un total de cinco ciclos de Bleomicina/Etoposido/Cisplatino con una respuesta total tras el tratamiento.RESULTADOS: Los tumores testiculares representan el tumor maligno sólido más frecuente en varones entre 20 y 39 años. Comprenden el 1% de todas las neoplasias masculina, y son responsables del 0,1% de todas las muertes por cáncer. Algunos autores apuntan que la incidencia real del tumor testicular ha aumentado y se sitúa alrededor del 3 % y se estima en 450 nuevos casos al año en España.CONCLUSIONES: Con respecto al pronóstico de los tumores no seminomatosos, se acepta que la tasa de curación de los pacientes de riesgo intermedio se sitúa alrededor del 70% con tratamiento convencional con cuatro ciclos de BEP. El caso que presentamos resulta muy llamativo puesto que en nuestro medio los tumores testiculares son diagnosticados en estadios iniciales, sin afectación cutánea extensa, ni simulando otros tipos de tumores epiteliales y muestra la historia natural de la enfermedad (AU)

OBJECTIVE: We report a rare case of advanced testicular cancer that describes the natural progression of testicular cancer without medical treatment. This study also describes the effectiveness of chemotherapy, which was the approach used for treatment.METHODS: 37 year old male with history of mental retardation, presented to the emergency room with an ulcer on his right scrotum that had been present for a few months. He was diagnosed of pT4 embryonal carcinoma by biopsy. CT scan showed multiple lung nodes. He was treated with five cycles of Bleomycin/Etoposide/Cisplatin with complete response after treatment.RESULTS: Testicular tumors are the most frequent solid tumors in males between the ages of 20 and 39 years old. Testicular tumors represent 1% of all neoplasias diagnosed in males and 0.1% of all male deaths due to cancer. Several studies have reported the current real incidence rate of testicular tumors has increased to 3%, which accounts for the diagnosis of 450 new cases of testicular cancer a year in Spain.CONCLUSIONS: The cure rate for patients with intermediate risk non-seminoma is around 70% following a conventional treatment approach of four cycles of BEP. The present case is noteworthy because, in our experience, testicular tumors are diagnosed at an early stage without extensively affecting the skin or simulating another type of epithelial tumor. As a result, the present study describes the natural progression of testicular cancer (AU)

Embryonal carcinoma in a cryptorchid testis of a 3-year old.

The undescended testis is at an increased risk of malignant transformation. Almost all such tumours occur in the second to fourth decades of life and are usually seminomas. A case of a mixed germ-cell tumour with yolk sac and embryonal carcinoma components arising in one of the hitherto uncorrected bilateral cryptorchid testes of a3-year old child is presented.