Humans ; Mediastinal Neoplasms/pathology* ; Neoplasms, Germ Cell and Embryonal/surgery*

Adult ; Female ; Humans ; Liver Neoplasms ; diagnosis ; surgery ; Neoplasms, Germ Cell and Embryonal ; diagnosis ; surgery ; Sarcoma ; diagnosis ; surgery ; Tomography, X-Ray Computed

Objective: To evaluate the incidence, treatment, and survival outcomes of Swyer syndrome with gonadal non-dysgerminoma malignant germ cell tumor (MGCT-NDG). Methods: A retrospective study was performed on Swyer syndrome patients with MGCT-NDG between January 2011 and December 2022 in Peking Union Medical College Hospital to investigate their characteristics and outcomes. Results: A total of 15 patients (4.9%, 15/307) with Swyer syndrome were identified in 307 MGCT-NDG patients. The average age at diagnosis of MGCT-NDG and Swyer syndrome were (16.8±6.7) and (16.7±6.6) years, respectively. Six cases were preoperatively diagnosed as Swyer syndrome, of which 4 cases received bilateral gonadectomy with or without hysterectomy, while the other 2 cases underwent removal of gonadal tumor and unilateral gonadectomy with hysterectomy, respectively. Of the 9 patients postoperatively diagnosed as Swyer syndrome, unilateral gonadectomy, removal of gonadal tumor, and unilateral gonadectomy with hysterectomy were performed in 6 patients, 2 patients, and 1 patient, respectively. Mixed malignant germ cell tumor (MGCT;10 cases), yolk sac tumor (4 cases), and immature teratoma (1 case) were the pathological subtypes, in the descending order. There were International Federation of Gynecology and Obstetrics (FIGO) stage Ⅰ in 6 cases, stage Ⅱ in 3 cases, stage Ⅲ in 5 cases, and stage Ⅳ in 1 case, respectively. Eleven patients received reoperation for residual gonadectomy after a average delay of (7.9±6.2) months, including 8 MGCT-NDG patients and 1 gonadoblastoma patient, no tumor involved was seen in the remaining gonads in the other 2 cases. Ten patients experienced at least one recurrence, with a median event free survival of 9 months (5, 30 months), of which 2 patients received surgery only at the time of initial treatment. All patients with recurrence received surgery and combined with postoperative chemotherapy. After a median follow-up of 25 months (15, 42 months), 10 patients were disease-free, 3 patients died of the tumor, 1 died of side effects of leukemia chemotherapy, and 1 survived with disease. Conclusion: The incidence rate of Swyer syndrome in patients with MGCT-NDG is about 4.9%; timely diagnosis and bilateral gonadectomy should be emphasized to reduce the risk of reoperation and second carcinogenesis in this population.
Female ; Humans ; Retrospective Studies ; Gonadal Dysgenesis, 46,XY/surgery* ; Gonadoblastoma/surgery* ; Neoplasms, Germ Cell and Embryonal/surgery* ; Ovarian Neoplasms/pathology*

OBJECTIVETo report a rare case of ectopic mesonephric duct cyst with ectopic testicular malignancy and improve the diagnosis and treatment of the disease.

METHODSWe retrospectively analyzed the clinical data of a case of ectopic mesonephric duct cyst with ectopic testicular malignancy, reviewed relevant literature at home and abroad, and investigated the pathogenesis, diagnosis and treatment of the disease.

RESULTSA large cyst and the right ectopic malignant testis were removed via abdominal incision, and the left undescended testis was lowered into the scrotum. Pathological examination confirmed the lesion to be right ectopic mesonephric duct cyst with right ectopic testicular seminoma. No metastasis was found during a year of follow-up.

CONCLUSIONEctopic mesonephric duct cyst with ectopic testicular malignancy was a rare disease. Imaging examination contributes to its diagnosis, but it has to be confirmed by pathology. Surgical removal should be performed as early as possible and follow-up treatment depends on the pathologic type and stage of ectopic testicular malignancy.

Cryptorchidism ; therapy ; Cysts ; pathology ; Humans ; Male ; Neoplasms, Germ Cell and Embryonal ; surgery ; Seminoma ; surgery ; Testicular Neoplasms ; surgery ; Testis ; surgery ; Wolffian Ducts ; pathology

OBJECTIVETo summarize our initial experience with laparoendoscopic single-site (LESS) retroperitoneal lymph node dissection (RPLND) for treatment of nonseminomatous testicular cancer.

METHODSFrom September 2010 to June 2011, 3 patients (aged 19-27 years) with right testicle enlargement and elevated alpha-fetoprotein level underwent right radical orchidectomy. Histopathological analysis revealed nonseminomatous germ cell tumor. LESS-RPLND was performed 3 weeks after orchiectomy. The homemade port was inserted through a 3-cm right pararectal incision in the right lower quadrant for unilateral RPLND using nerve-sparing technique and modified right-sided template removal similar to those in open RPLND.

RESULTSThe operation was successfully performed with a mean operative time of 240 min and a mean estimated blood loss of 50 ml. No conversion to open or conventional laparoscopic surgery was required. No major perioperative complications were observed. For the first case, the number of lymph nodes obtained for final histopathological examination was 11, and two positive nodes were detected. For the other 2 cases, no positive nodes were detected. Chemotherapy was administered in the first case. Alpha-fetoprotein level decreased close to the baseline one week postoperatively and no relapse occurred in these cases 3 month after RPLND. Follow-up at 1 year after the surgery showed good tumor control and preservation of the sexual function.

CONCLUSIONLESS-RPLND is safe and feasible for treatment of nonseminomatous testicular cancer, and the pararectal incision provides an ideal surgical approach with good cosmetic result, but the long-term effect needs to be tested by further large population-based study.

Adult ; Follow-Up Studies ; Humans ; Laparoscopy ; methods ; Lymph Node Excision ; methods ; Male ; Neoplasms, Germ Cell and Embryonal ; surgery ; Orchiectomy ; Retroperitoneal Space ; surgery ; Testicular Neoplasms ; surgery ; Young Adult

Adolescent ; Diagnosis, Differential ; Disorders of Sex Development ; Female ; Humans ; Male ; Neoplasms, Germ Cell and Embryonal ; pathology ; radiotherapy ; surgery ; Seminoma ; pathology ; Testicular Neoplasms ; pathology ; radiotherapy ; surgery

OBJECTIVETo investigate different treatment methods for stage-Is testicular mixed germ cell tumors (TMGCTs).

METHODSWe retrospectively analyzed the clinical data about 3'cases of stage-Is TMGCTs (aged 26-39 years) treated in the 175th Hospital of PLA, reviewed relevant literature, and explored the clinical characteristics of TMGCTs.

RESULTSOf the 3 patients, 1 was treated by radical orchiectomy, 1 by radical orchiectomy + retroperitoneal lymph node dissection + BEP chemotherapy scheme, and the other by radical orchiectomy + radiotherapy. The pathological components of TMGCTs were immature teratoma, seminoma, spermatocytoma, chorioepithelioma, embryonal carcinoma, and yolk sac tumor. No recurrence or distant metastasis was found during the 24-month follow-up after surgery.

CONCLUSIONThe diagnosis of TMGCTs primarily depends on physical examination, ultrasonography, MRI, and measurement of serum tumor markers, while its confirmation necessitates pathological examination, and its treatment is basically radical orchiectomy.

Adult ; Carcinoma, Embryonal ; pathology ; Endodermal Sinus Tumor ; pathology ; Humans ; Lymph Node Excision ; Male ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Neoplasms, Germ Cell and Embryonal ; pathology ; surgery ; Orchiectomy ; Retrospective Studies ; Seminoma ; pathology ; Teratoma ; pathology ; Testicular Neoplasms ; pathology ; surgery

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.
Male ; Humans ; Adult ; Seminoma/secondary* ; Cicatrix/pathology* ; Hyperplasia ; Retrospective Studies ; China ; Testicular Neoplasms/pathology* ; Neoplasms, Germ Cell and Embryonal/surgery* ; Calcinosis ; Carcinoma ; Tumor Microenvironment

Here we report a case in a 41-year-old woman histologically proven cystic embryonal sarcoma of the kidney, with emphasis on the imaging findings and pathological features. A large lobulated solid mass in the cystically dilated pelvocalyceal region was accompanied with hydroureter as depicted on both ultrasound and contrast-enhanced CT images.
Adult ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Imaging, Three-Dimensional/methods ; Kidney/radiography/surgery/ultrasonography ; Kidney Neoplasms/*radiography/surgery/*ultrasonography ; Neoplasms, Germ Cell and Embryonal/*radiography/surgery/*ultrasonography ; Sarcoma/*radiography/surgery/*ultrasonography ; Tomography, X-Ray Computed/methods

Adenofibroma ; metabolism ; pathology ; Antigens, CD34 ; metabolism ; Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Neoplasms, Germ Cell and Embryonal ; metabolism ; pathology ; surgery ; Nephroma, Mesoblastic ; metabolism ; pathology ; Sarcoma, Clear Cell ; metabolism ; pathology ; Stromal Cells ; metabolism ; pathology ; Vimentin ; metabolism