Extragonadal Germ Cell Tumors Research Articles

Burned-out testicular germ cell tumors: a clinicopathological analysis of three cases

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.

Diagnosis and treatment of primary seminoma of the prostate: A case report and review of literature

Primary seminoma of the prostate (PSP) is a rare type of extragonadal germ cell tumour that is easily misdiagnosed, owing to the lack of specific clinical features. It is therefore necessary for clinicians to work toward improving the accuracy of PSP diagnosis. A 59-year-old male patient presenting with acute urinary retention was admitted to a local hospital. A misdiagnosis of benign prostatic hyperplasia led to an improper prostatectomy. Histopathology revealed PSP invading the bladder neck and bilateral seminal vesicles. Further radiotherapy treatment for the local lesion was performed, and the patient had a disease-free survival period of 96 mo. This case was analysed along with 13 other cases of PSP identified from the literature. Only four of the cases (28.6%) were initially confirmed by prostate biopsy. In these cases, imaging examinations showed an enlarged prostate (range 6-11 cm) involving the bladder neck (13/14). Of the 14 total cases, 11 (78.6%) presented typical pure seminoma cell features, staining strongly positive for placental alkaline phosphatase, CD117, and OCT4. The median age at diagnosis was 51 (range 27-59) years, and patients had a median progression-free survival time of 48 (range 6-156) mo after treatment by cisplatin-based chemotherapy combined with surgery or radiotherapy. The remaining three were cases of mixed embryonal tumours with focal seminoma, which had clinical features similar to those of pure PSP, in addition that they also had elevated serum alpha-fetoprotein, beta-human chorionic gonadotropin, and lactose dehydrogenase. PSP should be considered in patients younger than 60 years with an enlarged prostate invading the bladder neck. Further prostate biopsies may aid in proper PSP diagnosis. Cisplatin-based chemotherapy is still the main primary therapy for PSP.

MP33-06 EXPERIENCE OF THE VIRTUAL CANADIAN TESTICULAR CANCER SECOND OPINION GROUP

You have accessJournal of UrologyCME1 Apr 2023MP33-06 EXPERIENCE OF THE VIRTUAL CANADIAN TESTICULAR CANCER SECOND OPINION GROUP J Jesus Cendejas-Gomez, Robert James Hamilton, Michel Jewett, Victor Sandoval, and Nicholas Edgar Power J Jesus Cendejas-GomezJ Jesus Cendejas-Gomez More articles by this author , Robert James HamiltonRobert James Hamilton More articles by this author , Michel JewettMichel Jewett More articles by this author , Victor SandovalVictor Sandoval More articles by this author , and Nicholas Edgar PowerNicholas Edgar Power More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003266.06AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Testicular cancer (TC) is the most frequent solid neoplasia in male patients between 15-35 years. The cases complexity increases in the metastatic stages and the implementation of evidence into the practice is more difficult. Second-opinion groups have shown their utility in improving the implementation of evidence-based treatments. Our main objective is to analyze the utility of a second opinion group in changing decision-making in patients with TC in Canada. As secondary objectives, we will analyze the number of answers per question, the average waiting time for second opinions, the different types of questions, and the over and undertreatment. METHODS: This is a retrospective analysis of 132 cases from the virtual Canadian testicular cancer second opinion group. The discussion of cases was carried out anonymously on the Google groups platform, in which doctors from 9 different provinces and different centers throughout Canada participate, including oncologists, urologist oncologists, and radiotherapists. Collected data include patients' demographic, histological, and treatment data. From the medical standpoint the number of answers, type of question, measured concordance between first and second opinions, and finally change in treatments after the discussion. The information was analyzed from June 2014 to July 2022. RESULTS: We included 132 cases of patients with testicular tumors and extragonadal primary germ cell tumors (GCT) in Canada.The most common histology was GCT in 94% (124/132), non-seminomas represented 72.7% (96/ 124). The most common clinical stage was metastatic in 94.7%.The mean of second opinions was 4.7, the 81% of seekers got responses from at least 3 different centers, and 56.8% of questions received a response from at least two different specialties. The average waiting time for the total of second opinions was less than 1 day. The questions come from academic centers in 81%, and the most common seeker doctors were the oncologists with 86.4% of the questions. The most frequent kind of question was related to chemotherapy in 49%.Comparing the first and second opinions, we found 24% of overtreatment and 26% of undertreatment, with a surprising 52% of potentially changing decision-making. In the multivariable analysis we did not find statistically significant predictors for changing decision-making. CONCLUSIONS: A virtual second opinion group could be a very valuable and easily accessible tool to improve the treatment of patients with complex testicular cancers, with an important percentage of potential changes in treatment even in academic cancer centers. Source of Funding: None © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e452 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information J Jesus Cendejas-Gomez More articles by this author Robert James Hamilton More articles by this author Michel Jewett More articles by this author Victor Sandoval More articles by this author Nicholas Edgar Power More articles by this author Expand All Advertisement PDF downloadLoading ...

Upper gastrointestinal bleed as the initial presentation of seminoma involving the duodenum in an elderly patient with 9year follow up: A case report and literature review.

Germ cell tumors are the most common malignancies in males between 20 and 40 years. However, primary extragonadal germ cell tumors are rare and account for 2%-5% of all germ cell neoplasms in adults. Extragonadal germ cell tumors are characterized by their midline locations including pineal and suprasellar regions, mediastinum, retroperitoneum and sacrococcyx. These tumors have also been reported in rare locations such as prostate, bladder, vagina, liver and scalp. Extragonadal germ cell tumors can be primary but may also represent metastases from primary gonadal germ cell tumors. In this report we describe a case of a duodenal seminoma in a 66-year-old male with no history of testicular tumors, who presented with upper gastrointestinal bleed as the initial manifestation. He was effectively treated with chemotherapy and continues to progress well clinically, with no episodes of recurrence.

A Long-Term Outcome of the Patients with Sacrococcygeal Teratoma: A Bosnian Cohort.

Sacrococcygeal teratoma is an extragonadal germ cell tumor that develops during fetal and neonatal periods and is associated with significant perinatal morbidity and even mortality. This study aimed to determine the clinicopathologic characteristics, the clinical outcomes, and postoperative complications of sacrococcygeal teratoma cases in the low-volume pediatric surgery service in a developing country. The study included data from all sacrococcygeal teratoma cases diagnosed from 2011 to 2020. All the relevant clinicopathologic data were recorded and analyzed. A total of 7 pediatric (5 females and 2 males) patients with sacrococcygeal teratoma were identified, ranging in age from 3 to 222 days. A prenatal diagnosis was made in 57.1% of cases. The mean gestational age for all cases was 37.1 weeks (34-38 weeks), and the mean birth weight was 3285 g (range, 2300-4700 g). Preoperative alpha-fetoprotein levels had a mean value of 24.327 ng/mL (range, 649.7-110.600 ng/mL). The surgery involved resection of the primary tumor and coccygectomy in all cases. Three (42.9%) tumors were classified as Altman type II lesions, 2 (28.6%) tumors were type IV, and 2 remaining cases were types I and III, respectively. Histology was benign in 4 (57.1%) and immature in 3 patients (42.9%). The mean follow-up time was 101.4 months (30-146 months), with 2 recurrences of high-grade immature teratomas at 11 and 30 months following the surgery. Three patients had postoperative bladder and rectal dysfunctions. Sacrococcygeal teratomas are rare tumors associated with frequent postoperative dysfunctions. Recurrences may also be seen, particularly in immature, high-grade forms of sacrococcygeal teratomas.

Postoperative recurrence of mixed extragonadal germ cell tumor in the right shoulder: a case report

BackgroundExtragonadal germ cell tumours (EGGCTs) originated in Shoulder are extremely rare, with 1 case described in the literature. We report a case of a patient with a primary Right Shoulder mixed EGGCT.Case presentationA 36-year-old male patient was hospitalized for 6 months due to progressive right shoulder swelling accompanied by pain. Subsequently, the right shoulder tumor was removed entirely. Gross pathological examination showed that the size of the tumor mass was about 14 × 10 × 6 cm.Mutations were observed in ENPEP (4q25), ZCCHC11, RREB1 (6p24.3), CKAP4 (12q23.3), and other genes were detected by whole exome sequencing. Histology revealed a mixed EGGCT of the Right Shoulder with immature teratoma and yolk sac tumour. The patient went through 6 cycles of chemotherapy. After 7 months of follow-up, the patient is recurrence.ConclusionThe primary MEGCT of the shoulder is an extremely rare condition. However, the recurrence and metastasis rates are high. Therefore, further research is necessary to determine this rare disease’s genetic and clinical characteristics to develop an effective treatment plan.

Histopathological Study and Immunohistochemistry of Mediastinal Masses of Anterior Compartment

Background: The mediastinum is the mid portion of the thoracic cavity, further divided into the anterior, middle, and posterior mediastinum. The numerous organs within the mediastinum make it prone to the development of various lesions. About half of all mediastinal masses occur in the anterior compartment. The most common are thymoma, lymphoma and mature teratoma. An accurate histopathological diagnosis is required for the management of mediastinal lesions.
 Methods: This is a 2-year prospective study conducted in the Department of Pathology on Biopsies and Resected Specimens of Anterior Mediastinal Masses (AMM) in a tertiary care hospital from September2020-September2022. Sections were studied after staining with hematoxylin and eosin(H&E). Immunohistochemical (IHC) stains were done wherever required.
 Result: The present study included 58 cases of AMM. The age of the patients ranged from 2-77 years. Males constituted 68.90 % of cases. The male-to-female ratio was 5:2.2. Cough was the most common presenting symptom. Thymic pathology was detected in 56.8% of cases, of which thymoma is the most common. Thymoma presented with Myasthenia Gravis (M.G.) in 2 cases. Lymphoma constituted 12% of cases. Metastatic carcinoma in 12%, Extragonadal Germ Cell Tumor (EGCT) in 8.6% and lymphangioma in 3.4% cases. Lipoma, sarcoidosis and myofibroblastic tumor each constitute 1.72% cases.
 Conclusion: The present study describes the various lesions of the anterior compartment.

Outcomes of relapsed/refractory extracranial germ cell tumors treated on conventional salvage chemotherapy without stem cell rescue: Experience from a tertiary cancer center.

Data on the outcome and prognostic indicators in extracranial relapsed/refractory germ cell tumors (rel/ref-GCTs) in children are limited to a few studies. This study looks at remission rates and outcomes of rel/ref-GCTs treated with conventional salvage chemotherapy (SC) regimens without stem cell rescue at a single center in the developing world. Patients treated at our center from January 2009 to December 2018 were included. Risk at primary presentation was stratified as all completely excised teratomas and stage I gonadal tumors being low risk (LR); stage IV ovarian, stage III-IV extragonadal GCTs as high risk (HR), and the remaining as intermediate risk (IR). SC regimens were: vinblastine-ifosfamide-cisplatin/carboplatin or paclitaxel-ifosfamide-cisplatin/carboplatin, or cisplatin/carboplatin-etoposide-bleomycin. Local therapy was either surgery and/or radiotherapy. The analyzable cohort comprised 50 patients (44 = rel-GCTs; 6 = ref-GCTs) with a median age of 3.8years and male:female ratio of 1.27:1. Primary location was ovary in 16 (32%), testicular in 10 (20%), and extragonadal in the rest (48%). Local, metastatic, and combined progression was noted in 28 (56%), 14 (28%), and eight (16%) patients, respectively, at a median time of 8.5months. At a median follow-up of 60months, the 5-year event-free survival (EFS) and overall survival (OS) of the entire cohort (n=50) were 42.4% and 50.0%, respectively. In patients previously exposed to platinum analogs (n=38), 5-year-EFS and OS were 27.7% and 31.7%, respectively. Local relapses did better when compared to metastatic and combined relapses (5-year EFS: 64% vs. 23% vs. 0%; p=.009). LR and IR tumors did better compared to HR (5-year EFS: 81.5% vs. 49.3% vs. 6.5%; p=.002). Patients with normalization of tumor markers after two cycles had a superior EFS (57.6% vs. 0%; p<.001). Relapsed tumors fared better than primary refractory GCTs (5-year EFS: 48.6% vs. 0%; p<.001). Primary refractory GCTs, extragonadal rel-GCTs, and rel/ref-GCTs with a poor biochemical response did poorly with conventional SC and need alternative treatment strategies. The rel/ref-testicular GCTs had the best chance of salvage despite a second recurrence (5-year EFS and OS: 28.60% and 42.90%, respectively).

Extragonadal germ cell tumor of the mediastinum: local spread estimated by direct injection of a contrast agent into the pericardial cavity

Purpose of the study: to study the possibilities of computed tomographic pericardiography in assessing the invasion of a mediastinal tumor into the myocardium.Materials and methods. Traditional computed tomography with intravenous contrasting and direct contrasting of the pericardial cavity (computed tomographic pericardiography) were compared in a 19-year-old patient with mediastinal germ cell tumor.Results. Due to the introduction of a contrast agent under manual pressure into the pericardial cavity, it became possible to exclude tumor invasion into the myocardium at the level of the atria. This made it possible to assess the technical feasibility of tumor removal at the preoperative stage.Conclusion. Extragonadal germ cell tumors of the mediastinum often have an asymptomatic course, which leads to the detection of tumors that are already large in size, with signs of spread to neighboring organs and structures. Traditional computed tomography with intravenous contrast does not always allow to exclude invasion of the myocardium, pericardium and large vessels. This article demonstrates a clinical case of performing direct contrasting of the pericardial cavity (computed tomographic pericardography) in a 19-year-old patient with a germ cell tumor of the mediastinum, which made it possible to exclude myocardial invasion and subsequently successfully operate the patient.

A 16-year-old Adolescent With Mediastinal Seminoma: A Case Report and Literature Review

Germ cell tumors (GCTs) are a heterogeneous group of neoplasms that arise from the primordial germ cells of the human embryo, which are normally destined to produce reproductive cells sperm, or ova. GCTs can be present in both gonadal GCTs and extragonadal GCT sites. Pediatric GCTs are relatively rare tumors with an incidence of 2%-3%. Primary mediastinal germ cell tumors GCTs are very rare extragonadal GCTs that arise in the anterior mediastinum. In this report, we present the case of a 16-year-old boy with primary seminoma arising in the anterior mediastinum. The patient presented with the symptoms of cough, fever, and chest tightness. CT finding was in favor of a large expansive process measuring 12.4x6.7x14.2 cm in the anterior mediastinum, accompanied by a conglomeration of hilar lymph nodes in the level of brachiocephalic veins juncture. Fine needle biopsy and core biopsy were performed transthoracically, under the control of MSCT. Based on histology and immunohistochemistry, the diagnosis of mediastinal germ cell tumor with immunophenotype of seminoma was made. The patient was treated with 4 cycles of chemotherapy by BEP protocol without significant side effects and toxicities. The patient remained disease-free for 16 months. The purpose of reporting this case is to confirm that chemotherapy with cisplatin-based regimens has markedly improved the outcome of adults and children with GCTs as well.

Extragonadal Mixed Germ Cell Tumour of the Right Scapular Region: A Case Report.

germ cell tumor; immunohistochemistry; tumor markers.

A rare case: 47-years-old male with mature Mediastinal Teratom

Introduction: Mediastinal teratoma is a tumor originating from germ cells. The global incidence of teratoma is 1 per 4000 births. In the mediastinum, germ tumors account for 1% to 3% of all cases. The anterior mediastinum is where extragonadal germ cell tumors are most prevalent and are often benign. The symptoms are manifestations of pushing the tumor into the surrounding environment. Case Presentation: Our hospital received a referral for a 47-year-old male bus driver for diagnostic purposes with complaints of shortness of breath 3 months before his admission to the hospital, which has been getting worse in the last 1 month. A chest X-ray was performed in previous hospital showed a mediastinal mass. Bronchoscopy showed no source of bleeding. A contrast chest CT scan showed a mass in the superior anterior mediastinum with a well-defined, lobulated that deviated the trachea to the right and compressed the heart. Open thoracotomy was performed followed by pathology examination which shows mature teratoma. The patient was given antibiotics and discharged three days later with pretty good outcome. No mass or infiltration was found. Conclusion: Mature mediastinal teratoma is a rare case. These tumors might cause compression to the adjacent organs and structures and cause symtoms. Chest CT scan is considered good modality choice to evaluate compression of the surrounding organs and post-treatment comparation.

Extragonadal Germ Cell Tumors of the Mediastinum and Retroperitoneum: A Surveillance, Epidemiology, and End Results-based Study.

Extragonadal germ cell tumors (EGCT) are a rare entity, most of them being located in the mediastinum and retroperitoneum. Information on these tumors is scarce, requiring carrying out large population-based studies to better understand these diseases. We aimed to determine the clinical features and prognosis of patients with EGCT of the mediastinum and retroperitoneum. Demographic and clinicopathological features of patients diagnosed with EGCT of the mediastinum and retroperitoneum from 1975 to 2016 were retrieved from the Surveillance, Epidemiology, and End Results (SEER) database. A total of 1674 patients were included, 1297 (77.5%) of mediastinal origin and 377 (22.5%) of retroperitoneum. Nonseminomatous tumors (56.3%) were slightly more frequent than seminomas (43.7%) with similar distribution between mediastinum and retroperitoneum. After a median follow-up of 137 months, the median overall survival was 263 months (95% CI, 220-296) whereas the median cause-specific survival (CSS) has still not been reached. The 10-year overall survival and CSS were 57.4% (95% CI, 55-59.7) and 63% (95% CI, 60.6-65.2) respectively. Multivariate analysis showed that older age, mediastinal location, nonseminomatous histology, and distant disease at diagnosis were independent prognostic factors correlated with a worse prognosis. Patients with mediastinal choriocarcinoma and embryonal carcinoma have the worst prognosis, both with a median CSS of only 12 months. Despite a decreasing incidence observed in recent decades, EGCT continues to represent a challenge for oncologists. The prognosis of choriocarcinoma and embryonal carcinoma of the mediastinum remains poor and treatment strategies need to be improved urgently.

A Rare Case of Metastatic Extragonadal Yolk Sac Tumor – A Challenging Diagnosis on Cytomorphology

Abstract Introduction/Objective Yolk sac tumors are characterized by variable histologic patterns and broad cytologic spectrum; thereby making the distinction from a poorly differentiated adenocarcinoma in a metastatic site extremely challenging. We present cytology and histology of a metastatic extragonadal germ cell tumor with predominant solid yolk sac component. Methods/Case Report 36-year-old male presented with bilateral supraclavicular masses (right: 6 x 7 cm, left: 3 x 4 cm). CT chest and abdomen revealed large mediastinal and hilar nodal mass (11.6 cm), right hilar mass (8.5 cm), bilateral heterogeneous adrenal masses (right: 11.4 cm, left: 3.1 cm), and an enhancing retroperitoneal implant. Differential diagnoses of lymphoma, adrenocortical carcinoma, small cell lung carcinoma, and metastatic germ cell tumor were considered. Testicular ultrasound revealed no masses. Fine needle aspiration of supraclavicular lymph nodes showed highly cellular smears comprised of tumor cells, dispersed singly and in clusters, with irregular nuclear contours, increased nuclear to cytoplasmic ratio, coarse chromatin, occasional prominent nucleoli, and vacuolated cytoplasm. Tumor cells were positive for KRT7 and TTF-1, and negative for p40 and PAX8. A diagnosis of metastatic poorly differentiated carcinoma was rendered. Subsequent excisional biopsy of the lymph node showed a metastatic tumor with tumor cells similar to those seen in cytology, appearing in solid sheets with focal papillary architecture, replacing the entire lymph node. Additional immunohistochemistry showed expression of SALL4, Glypican 3, with lack of expression for OCT3/4, AFP, hCG, and CD30, supporting the diagnosis of metastatic yolk sac tumor, solid pattern. Results (if a Case Study enter NA) NA. Conclusion Aggressive clinical presentation, broad clinical differential diagnoses, rarity of a primary mediastinal yolk sac tumor, non-diagnostic cytomorphology along with expression of divergent differentiation markers (TTF-1 in our case) makes a definitive diagnosis of metastatic extragonadal yolk sac tumor extremely challenging. High index of clinical suspicion is required for a definitive diagnosis on cytomorphology.

Cytopathologic Diagnosis of Extragonadal Germ Cell Tumors: A 10-year Institutional Review

Cytopathologic Diagnosis of Extragonadal Germ Cell Tumors: A 10-year Institutional Review

Late mortality reduction among survivors of germ cell tumors in childhood and adolescence in Europe: A report from the PanCareSurFup cohort.

Data on late mortality from pediatric germ cell tumors (GCTs) are limited to small case series. Our population-based study aimed to investigate excess risk of death in survivors of GCT in childhood and adolescence, whether long-term mortality changed over time and by period of diagnosis. The PanCare Childhood and Adolescent Cancer Survivor Care and Follow-Up Studies (PanCareSurFup) cohort includes 2773 five-year survivors diagnosed under 21years of age with gonadal and extragonadal GCT (from 1940 to 2008). We calculated standardized mortality ratios (SMRs) and absolute excess risks (AERs). We fitted a Cox's model to assess the impact of treatment period. We estimated 10-year survival and calculated average percentage changes between periods of diagnosis (1970-1979, 1980-1989, 1990-1999) to assess whether late mortality decreased. GCT survivors had an almost four-fold excess risk of dying compared to general population. The risk of death for patients treated after 1980 was nearly halved compared to patients treated before 1980. Survivors diagnosed in 1990-1999 had a 10-year survival rate of 99%, which was 2.4% and 1.1% higher than for patients treated in 1970-1979 and 1980-1989, respectively. This is the largest population-based study in Europe and showed a decrease in long-term mortality for survivors of GCTs in childhood and adolescence over the last decades. After the introduction of platinum compound in 1980, which is a paradigm of success compared to the previous treatments, no major changes in drug therapies have been made to treat GCTs in the last 40years. However, GCT survivors maintain an excessive risk of death that requires long-term care.

S2896 A Rare Case of Necrotic Testicular Seminoma Presenting as a Large Retroperitoneal Mass Diagnosed With EUS and Laparotomy

Introduction: Retroperitoneal seminomas are a rare form of cancer that accounts for approximately 2% of all seminomas. Seminomas commonly present with fever, weight loss, abdominal or back pain, and a painless testicular nodule. The differential diagnosis for a retroperitoneal mass is broad, including abscess, lymphoma, sarcoma, metastatic lymph nodes, and extragonadal tumors. The pathogenesis of germ cell tumors is unclear, but the failure of primordial germ cell migration during embryonal development has been suggested. We report an atypical case of a large necrotic retroperitoneal mass diagnosed as a primary retroperitoneal seminoma after EUS and laparotomy. Case Description/Methods: A 33-year-old male with no medical history presented to the ED for new-onset flank pain and night sweats. On admission, the patient was febrile and tachycardic. The testicular exam and US of the testes were normal. CT abdomen and pelvis revealed a well-circumscribed, round mass posterior to the pancreatic head. EUS with FNA was performed, noting a round heterogenous 7.1cm x 6.2cm mass with well-defined borders and a necrotic center located posterior to the pancreatic head. An intact interface was seen between the mass and the duodenum, IVC, pancreas, and right kidney, suggesting a lack of invasion. The FNA yielded 0.5ml of blood-tinged, thick, and purulent fluid with cytology significant for malignant tumors with extensive necrosis. An exploratory laparotomy with excisional biopsy established the diagnosis of extragonadal germ cell tumor with extensive necrosis, consistent with seminoma. The patient completed four cycles of Etoposide followed by a right-sided orchiectomy for residual lesions found on the right testicle. A post-surgical PET scan did not demonstrate recurrence. Discussion: Primary retroperitoneal seminoma is a rare entity that must be considered when evaluating a retroperitoneal mass. GCTs are the most common neoplasm arising from the testes, with < 2% originating in other locations, termed primary extragonadal GCTs. Studies suggest the remission rate with chemotherapy and resection of residual masses was 92%, and the five-year overall survival rate was 88% for primary retroperitoneal tumors. Early identification of primary retroperitoneal seminomas may expand therapeutic possibilities and help improve outcomes for patients with a favorable prognosis. EUS-FNA was attempted in this case to obtain the diagnosis; however, an excisional biopsy was required to confirm the diagnosis.

Ultrasound and Magnetic Resonance Imaging of Burned-Out Testicular Tumours: The Diagnostic Keys Based on 48 Cases.

Simple SummaryIn rare cases, testicular cancers regress spontaneously. This is known as a burned-out testicular tumour. Few data have been published regarding the radiological appearance of these lesions. In this study, we describe the lesions observed in 48 patients using several imaging techniques (conventional ultrasound, advanced ultrasound techniques and MRI). In both ultrasound and MRI, there are features that allow the recognition of such lesions and may help in the correct management of these patients by proposing orchiectomy.The spontaneous regression of testicular germ-cell tumours is a rare event whose mechanisms have yet to be elucidated. In the majority of published cases, tumour regression is concomitant with the metastatic development of the disease. Residual lesions, often referred to as burned-out testicular tumours (BOTTs), are difficult to diagnose due to the paucity of published data, especially in the field of imaging. The aim of this article is to describe the radiological signs of BOTTs on multimodal ultrasound and multiparametric MRI from a series of 48 patients whose diagnosis was confirmed histologically. The demographic, clinical and laboratory characteristics of the patients are studied, as well as the data of the imaging examinations, including conventional scrotal ultrasound, shear-wave elastography, contrast-enhanced ultrasound (CEUS) and multiparametric MRI. A total of 27 out of 48 patients were referred for investigation of primary testicular lesion following the discovery of retroperitoneal metastases, 18/48 patients were referred because of lesions suspected on an ultrasound that was performed for an infertility work-up, and 3/48 were referred because of scrotal clinical signs. Of these last 21 patients (infertility work-up/scrotal clinical sign), 6 were found to be metastatic on the extension work-up. Of the 48 orchiectomy specimens, tumour involution was complete in 41 cases, and a small active contingent remained in 7 cases, with 6 suspected upon advanced US and MRI. Typically, BOTTs appear on a conventional ultrasound as ill-delineated, hypoechoic and hypovascular nodular areas. Clustered microliths (60.4%) and macrocalcifications (35.4%) were frequent. Shear-wave elastography showed areas of focal induration (13.5 ± 8.4 vs. 2.7 ± 1.2 kPa for normal parenchyma, p < 0.01) in 92.5% of the patients for whom it was performed, and contrast ultrasonography demonstrated hypoperfusion of these lesions. Of the 42 MRIs performed, BOTTs corresponded to nodules on T2-weighted sequences (hyposignal) with significantly increased ADC values compared with healthy parenchyma (2 ± 0.3 versus 1.3 ± 0.3 × 10−3 mm2/s, p < 0.01) and an enhancement defect after injection. This enhancement defect overlapped the lesions visible on T2-weighted sequences in most cases. In the case of predominant partial regression, an enhanced portion after contrast injection was visible on MRI in all seven patients of our series, and in six of them a focal diffusion restriction zone was also present. Spontaneously involuted testicular germ-cell tumours have specific radiological signs, and all of the mentioned examinations contribute to this difficult diagnosis, even histologically, because there is no tumour cell left. These signs are similar whether the patient is initially symptomatic metastatic or whether the discovery is fortuitous on the occasion of an infertility work-up, and whatever the seminomatous or non-seminomatous nature of the germ-cell tumour, when this can be determined. The appearance of regressed germ-cell tumours is often trivialized, which can lead to the wrong diagnosis of an extra gonadal germ-cell tumour (in metastatic patients) or of scarring from an acute event such as trauma or infection, which is not recognized or forgotten. In our series, two patients had an unrecognized diagnosis in their history, with local and/or distant recurrence. An improvement in diagnosing burned-out tumours, combining advanced US and MRI, is necessary in order to optimize patient management, with special attention paid to asymptomatic patients, to prompt extension screening and orchiectomy with analysis of the whole testis. This may reveal a persistent viable tumour or lesions of germinal neoplasia in situ, which are precursors of testicular germ-cell tumours.

Type IV sacrococcygeal teratoma presenting with complete urinary obstruction

Sacrococcygeal teratomas (SCT) are a rare entity, occurring in approximately 1 of every 35,000 to 40,000 births [1]. Although it remains the most common extragonadal germ cell tumor, type IV SCT represents less than 10% of those [1]. The diagnosis is challenging, as the initial presentation has been described as chronic pelvic pain, rectal mass and mild to severe urinary symptoms [2-4]. To our knowledge urinary obstruction is a rare initial presentation, described in the literature only three times [5], [6], [7]. Here in, we present a rare case of type IV SCT with a complete urinary obstruction as an initial symptom.

A Rare Case of a Germ-Cell Tumour Associated with Acute Megakaryoblastic Leukaemia- An Autopsy Report with Review of Literature

His association of mediastinal germ-cell tumours (MGCTs) with haematologic neoplasms is a rare though well known circumstance, and few cases are found in the literature. Most of these are non-seminomatous tumours in young males. The diagnosis of the haematological condition is usually either synchronic or metachronic with that of the germ-cell tumour. The prognosis is poor and basically determined by the haematologic neoplasia. Less than 20 cases of PMGCT with evolution into acute megakaryocytic leukemia have been reported in worldwide literature. Hematologic neoplasias associated with extragonadal germ cell tumors represent one of the most intriguing and biologically distinctive aspects of male germ cell cancers. The case report we present is that of a young 33 year old male with an initial diagnosis of both conditions which were detected synchronously. Despite timely intervention he succumbed to multiorgan failure following chemotherapy and an autopsy was performed.