Sertoli cell tumor of the testis causing intracranial metastasis two years after orchiectomy. Report of a rare case and review of the literature

Sertoli cell tumor of the testis (SCTT) accounts for less than 1% of all testicular tumors with only 10% of cases exhibiting malignant behavior. In the present report, a case of malignant SCTT causing multiple metastases in a 32-year-old man is described. After being diagnosed and treated for bone and lymph nodes metastases, the patient presented with a brief history of worsening headaches and visual impairment. A head MRI demonstrated an extra-axial tumor located in the right fronto-parietal junction exhibiting avid contrast enhancement and leptomeningeal involvement. To the best of the authors’ knowledge, this represents the second case of intracranial metastasis from SCTT described to date.

Ten Reasons Why People With Down Syndrome are Protected From the Development of Most Solid Tumors -A Review

People with Down syndrome have unique characteristics as a result of the presence of an extra chromosome 21. Regarding cancer, they present a unique pattern of tumors, which has not been fully explained to date. Globally, people with Down syndrome have a similar lifetime risk of developing cancer compared to the general population. However, they have a very increased risk of developing certain tumors (e.g., acute leukemia, germ cell tumors, testicular tumors and retinoblastoma) and, on the contrary, there are some other tumors which appear only exceptionally in this syndrome (e.g., breast cancer, prostate cancer, medulloblastoma, neuroblastoma and Wilms tumor). Various hypotheses have been developed to explain this situation. The genetic imbalance secondary to the presence of an extra chromosome 21 has molecular consequences at several levels, not only in chromosome 21 but also throughout the genome. In this review, we discuss the different proposed mechanisms that protect individuals with trisomy 21 from developing solid tumors: genetic dosage effect, tumor suppressor genes overexpression, disturbed metabolism, impaired neurogenesis and angiogenesis, increased apoptosis, immune system dysregulation, epigenetic aberrations and the effect of different microRNAs, among others. More research into the molecular pathways involved in this unique pattern of malignancies is still needed.

Retroperitoneal lymph node dissection in patients with advanced non-seminomatous germ cell testicular tumors and incomplete radiological and serological response to chemotherapy: results

Objective: To evaluate the results of retroperitoneal lymph node dissection (RPLND) in patients with advanced non-seminomatous germ cell testicular tumors (NSGCT) and incomplete serological and radiological response to chemotherapy (CT).Materials and methods: The study included 96 patients with advanced NSGCT who underwent RPLND in N.N. Blokhin Russian Cancer Research Center in 1983-2020. The median age was 27 (15-57) years. All patients (n = 96, 100,0 %) received first-line cisplatin-based CT. Fifty-eight patients (60,4%) received second-line CT. After completion of CT, all patients presented with elevated levels of AFP and/or hCG and detectable tumor lesions (retroperitoneal metastases only in 77 cases (80,2 %), metastases in the retroperitoneal space and other sites in 19 cases (19,8%)). All patients underwent the follow-up surgery after CT completion: RPLND in 96 cases (100,0%) and resection of extra-retroperitoneal lesions in addition to RPLND in 8 cases (8,3%). In total, 29 (30,2%) of 96 patients received CT following surgery. The median follow-up was 39,4 (1-284) months.Results: Postoperative complications were reported in 10 (10,6%) patients, including grade 3-4 in 3 patients (3,1%). The mortality rate was 1,1%. The complete resection of retroperitoneal tumor lesions was performed in 80 cases (83,3 %), resection of all detectable tumor lesions in 69 cases (71,9%). None of the patients achieved complete response to postoperative CT. Pathological examination of retroperitoneal lesions revealed necrosis and fibrosis, teratomas, and malignant non-seminomatous tumors in 25 (26,0 %), 29 (30,2 %), and 42 (43,8 %) cases, respectively. The long-term overall survival (OS) and cancer-specific survival rates were 60,9 % and 61,7 %, respectively. The relapse-free survival rate in patients who underwent complete resection reached 65,2 %, the progression-free survival rate in patients who underwent incomplete resection was 35,9 %. A multivariate analysis revealed the following independent predictors of unfavorable OS: RPLND after second-line CT (odds ratio [OR] 4,667 (95% confidence interval [CI]: 1,987-10,961)), presence of a residual retroperitoneal mass of a malignant non-seminomatous tumor (OR 3,081 (95% CI: 1,178-8,055), and incomplete removal of residual lesions after CT (OR 4,445 (95% CI: 1,813-10,899)).Conclusion: Post-CT RPLND may be considered a viable option in the selected group of advanced NSGCT patients with an incomplete serological response to CT eligible for complete resection of all detectable tumor lesions.

Burned-Out Testicular Tumors in Adolescents: Clinical Aspects and Outcome

Purpose: Testicular germ cell tumors are the fourth most common neoplasm in adolescents, accounting for 8% of all tumors in the age group 15–19 years. On rare instances, the primary testicular lesion is not clinically or radiologically evident while nodal or visceral metastases represent the clinical manifestations of the disease. This phenomenon is described as “burned-out testicular tumor.” In this paper, the authors report a single-institution experience with burned-out testicular tumors in adolescents and discuss their clinical implications.Patients and Methods: All the patients diagnosed with metastatic testicular germ cell tumors at Bambino Gesù Children Hospital between January 1, 2010, and June 30, 2020, were included in the study. Patients were categorized into two groups: “primary testicular” and “burned out.” All the patients were staged and treated according to the AIEOP–TCGM 2004 protocol.Results: Eleven patients were classified as “primary testicular,” and five patients were classified as “burned out.” “Burned-out” tumors were associated with the presence of systemic symptoms compared to “primary testicular” tumors (80 vs. 0%; p = 0.0027) and higher aFP, hCG, and LDH levels (p < 0.00001). The “burned-out” population had a statistically significant higher incidence of relevant toxicity than the “primary testicular” population (80 vs. 18%; p = 0.0357) and a worse outcome in terms of both mean overall survival (15 vs. 43 months; p = 0.0299) and mean event-free survival (12 vs. 38 months; p = 0.0164).Conclusion: “Burned-out” testicular tumors seem to be a well-distinct clinical entity with a high treatment-related toxicity and poor prognosis. Further studies are needed to clarify the “burned-out phenomenon” and to identify more effective therapeutic strategies for these patients.

On the issue of mixed testicular tumors

Mixed testicular tumors are of great interest both clinically and pathologically. Being in most cases benign neoplasms, they sometimes give rise to metastases, and in some cases are subjected to malignant transformation, so that their timely recognition is of particular value. In the pathological and anatomical sense these neoplasms are of interest in relation to their microscopic structure, often extremely complex in particular in relation to their histogenesis.