Tumors In Young Adults Research Articles

Vulvar dermatofibrosarcoma of Darier and Ferrand in a young woman: About a new observation

Darier and Ferrand Dermatofibrosarcoma is a rare mesenchymal tumor in young adults, often located in the trunk. We report an observation of a vulvar localization of dermatofibrosarcoma Darier and Ferrand. It was a young patient, aged 25, who presented a tumor of the vulvar region. This tumor has been evolving for about 10 years in two phases, the first of which is longer, with very slow development, and a second phase characterized by rapid growth of the tumor coinciding with the start of pregnancy. The examination noted a tumor developed at the expense of the vulvar region. The diagnosis of dermatofibrosarcoma was retained in view of a characteristic histopathological appearance. The treatment consisted of resection surgery with margins of about 5 cm followed by reconstruction by direct suture. The evolution, in the immediate aftermath, was good. Vulvar localization of dermatofibrosarcoma is possible. Key words: Darier and Ferrand dermatofibrosarcoma, Vulva, Young woman

S412 Association Between NAFLD/NASH and Colorectal Polyps and Neoplasms in Young Adults With Obesity: A Retrospective Study

S412 Association Between NAFLD/NASH and Colorectal Polyps and Neoplasms in Young Adults With Obesity: A Retrospective Study

Unusual location of a central neurocytoma: Particularities of a rare brain tumor

Introduction: Central neurocytoma is a typically rare intraventricular glioneuronal tumor in young adults. Clinically, it is revealed by symptomatology of intracranial hypertension. Intra-parenchymal, extraventricular, and intramedullary localizations are exceptional. Central neurocytoma is considered a benign tumor that can recur locally, but craniospinal dissemination remains as an exception. Case Report: We report a case in a 35-year-old male subject who consulted for quadriparesis and genito-sphincter dysfunctions. The clinical examination noted a syndrome of intracranial hypertension, a kinetic and static cerebellar syndrome, spastic tetraplegia with crural predominance, and anesthesia going up to the sixth thoracic vertebra (T6). A magnetic resonance imaging (MRI) showed a heterogeneous left cerebellar lesion. This lesion was excised, and the sample was freshly sent to the pathological anatomy laboratory. Extemporaneous cytology using the Smear technique was performed and was consistent with a hemangioblastoma. The standard histological examination also revealed the diagnosis of hemangioblastoma, and an additional immunohistochemical (IHC) study was performed for better diagnostic accuracy. The study was conducted in a North African laboratory and was conclusive of a neurocytoma. The evolution was marked by cerebellar tumor regrowth at five months, then the death of the patient at eight (08) months after surgery. Conclusion: Central neurocytoma is a rare tumor that can be confused with several other tumors of the central nervous system. Immunohistochemistry is essential for diagnostic confirmation.

Impact of testicular cancer on sperm small non-coding RNA signature: a pilot study

ABSTRACT Testicular germ cell tumours (TGCTs) are the most common tumours in young adults of European ancestry. The high heritability and the constantly increased incidence, which has doubled over the last 20 years, strongly suggest that both genetic and environmental factors are likely to shape the TGCT susceptibility. While genome-wide association studies have identified loci associated with TGCT susceptibility, the role played by environmental molecular vectors in TGCT susceptibility remains unclear. Evidence shows that sperm non-coding RNAs provide a good vision of the environmental stresses experienced by men. Here, to determine whether TGCT impacts the abundance of specific non-coding RNAs in sperm, small RNA deep sequencing analysis of sperm of 25 men aged between 19 and 42 years, diagnosed with (n = 16) or without (n = 9) TGCT was performed. The primary analysis showed no statistical significance in the sncRNA population between the TGCT and non-TGCT groups. However, when sperm physiological parameters were considered to look for differentially expressed sncRNA, we evidenced 11 differentially expressed sncRNA between patients and control which allow a clear discrimination between control and TGCT samples after Hierarchical Clustering analysis. Together, these findings indicate that sperm small non-coding RNAs abundance may have the potential for diagnosing men with TGCT. However, specific care should be taken regarding sperm physiological parameters of the TGCT patients. Hence, larger studies are needed to confirm our findings and to determine whether such a signature associates with the risks to develop TGCT.

Risk factors for advanced colorectal neoplasm in young adults: a meta-analysis.

Purpose: To evaluate the risk factors for young-onset advanced colorectal neoplasia. Methods: We performed a meta-analysis of 30 potential exposure risk factors from 28 original studies. Results: Several risk factors showed statistical significance, including male sex (odds ratio [OR]: 1.28; 95% CI: 1.12-1.47), metabolic syndrome (OR: 1.34; 95% CI: 1.25-1.44), hypertension (OR: 1.22; 95% CI: 1.17-1.28), diabetes (OR: 1.23; 95% CI: 1.15-1.32), inflammatory bowel disease (OR: 4.62; 95% CI: 1.12-17.54), obesity (OR: 1.23; 95% CI: 1.06-1.43), sedentary behavior (OR: 1.25; 95% CI: 1.06-1.48), smoking (OR: 1.19; 95% CI: 1.05-1.36), high alcohol consumption (OR: 1.37; 95% CI: 1.10-1.71), high intake of sugar (OR: 2.58; 95% CI: 1.61-4.13) and red meat(OR: 1.10; 95% CI: 1.04-1.16) andfamily history of colorectal cancer (OR: 3.14; 95% CI: 1.29-7.64). Conclusion: Our study identified potential risk factors for young-onset advanced colorectal neoplasms to help develop targeted primary prevention strategies.

Exploration of s new biomarker in osteosarcoma and association with clinical outcomes: TOP2A+ cancer associated fibroblasts.

Osteosarcoma (OS) is the leading malignant primary bone tumor in young adults and children and has a high mortality rate. Cancer-associated fibroblasts (CAFs) are major components of the tumor microenvironment, influencing cancer progression and metastasis. However, there is no systematic study on the role of CAF in OS. We collected six OS patients' single-cell RNA sequencing data from the TISCH database, which was processed using the Seurat package. We selected gene sets from the well-known MSigDB database and resorted to the clusterprofiler package for gene set enrichment analysis (GSEA). The least absolute shrinkage and selection operator (LASSO) regression model was used for identification of the variables. Receiver operating characteristic and decision curve analyses were utilized for determining the efficacy of the monogram model. TOP2A+ CAFs was recognized as the carcinogenic CAFs subset, given its intense interaction with OS malignant cells and association with the critical cancer driver pathway. We intersected the differentially expressed genes of TOP2A+ CAFs with the prognostic genes selected from 88 OS samples. The acquired gene set was selected using the LASSO regression model and integrated with clinical factors to obtain a monogram model of high prognosis predicting power (area under the curve of 5year survival at 0.883). Functional enrichment analysis revealed the detailed difference between two risk groups. We identified TOP2A+ CAFs as a subset of oncogenic CAFs in OS. Based on differentially expressed genes derived from TOP2A+ CAFs, combined with bulk transcriptome prognostic genes, we constructed a risk model that can efficiently predict OS prognosis. Collectively, our study may provide new insights for future studies to elucidate the role of CAF in OS.

Patient Reported Outcomes, Paternity, Relationship, and Fertility in Testicular Cancer Survivors: Results from a Prospective Observational Single Institution Trial.

Testicular cancer (TC) is the most common solid tumor in young adults. 95% of patients are cured, but they may experience late adverse effects (anxiety, fear of recurrence, and sexual dysfunction) with an impact on daily life. We attempted to assess Patient Reported Outcomes (PROMs), long-term sexual disorders, and difficulties in achieving fatherhood in a cohort of TC survivors, as well as their possible correlation with previous cancer treatments. Different questionnaires, such as the Impact of Cancer (IOC) and the Body Image Scale (BIS), were used to investigate the distinct areas of the PROMs. International Index of Erectile Function (IIEF15) and the Premature Ejaculation Diagnostic Tool (PEDT) focused on sexuality and fertility. Patients were prospectively recruited between February 2020 and February 2022. 144 participants completed all the questionnaires. Results showed a good QoL, a moderate fear of TC recurrence, a good satisfaction with their personal body image, low incidence of premature ejaculation and erectile dysfunction. 19.5% of patients who had a testicular implant reported general dissatisfaction. Only 18% of patients had unsuccessfully attempted fatherhood, while the majority had not yet tried, and 23.4% succeeded. A low percentage of patients used procedures assisted reproduction and adoption. This trial supports the use of various questionnaires as a multifactorial tool capable of investigating all the aspects of long-term cancer survivorship. The assessment of medical and psychosocial sequelae is an essential part of patient care and is important for the development of a comprehensive care plan for TC survivors.

Agreement between mothers and sons in early life exposures assessment in a multicentric case-control study investigating risk factors of testicular germ cell tumors in young adults (TESTIS study)

Agreement between mothers and sons in early life exposures assessment in a multicentric case-control study investigating risk factors of testicular germ cell tumors in young adults (TESTIS study)

Early-onset malignant solid tumours in young adult South Africans - an audit based on histopathological records of patients seen at the three academic hospitals in Johannesburg

BACKGROUND: Malignant tumours in adolescents and young adults (AYA) are referred to as early-onset cancers. This study analysed the histopathological profile of malignant solid tumours in AYA. METHODS: Records of patients who had confirmed malignant solid tumours were retrieved. Data collected included the treating hospital, year of presentation, age and histological diagnosis. The commonly diagnosed malignant tumours in AYA were compared with tumours in older adults. A p-value below 0.05 was considered significant. RESULTS: A total of 61 828 records were retrieved and 29 974 were excluded. Additionally, 10 55 post-excision results from AYA were excluded. Of the remaining 30 799 records, 13.1% (4 032/30 799) were diagnosed in AYA, of which 18.2% (734/4 032) were in-situ lesions. Overall, 11% (3 298/30 065) of invasive tumours were from the AYA. The majority, 81.1% (3 269/4 032), of invasive and non-invasive malignant tumours in AYA were from females. Breast and cervical cancer constituted 29.2% (962/3 298) and 23.2% (766/3 298) of diagnosed cancers in AYA, respectively. Ten (0.3%) cases of prostate and 0.4% (12/3 298) of lung cancers were reported in AYA. CONCLUSION: Eleven per cent of invasive malignancies were diagnosed in AYA and 81% involved females. Cancers of the breast, cervix, skin, and colon were the top four most common tumours in AYA. The burden of breast and colorectal cancer was higher in AYA than in older adults. Prostate cancer is rare in AYA and lung cancer was not among the top 10 malignant tumours in our setting. Over 11% of primary malignant tumours of the anus, breast, cervix, colon, conjunctiva, liver and rectum were diagnosed in AYA.

Global variation in young adult central nervous system tumor incidence by region, age, and sex from 1988 to 2012

BackgroundCentral nervous system (CNS) tumors result in tremendous morbidity and mortality. Incidence of CNS tumors in young adults is less studied. It is unknown how young adult CNS tumor incidence has changed globally in recent decades. MethodsWe used Cancer Incidence in Five Continents (CI5) data (1988–2012) to estimate incidence rates (IR), average annual percent change in incidence (AAPC; 95% confidence intervals [95% CI]), and male-to-female incidence rate ratios (IRR; 95% CI) by six histologies and age at diagnosis (20–29years, 30–39years). Tumors were classified as astrocytic, medulloblastoma, ependymal, oligodendroglial, meninges, and other embryonal. Geographic regions were defined using the United Nations Statistics Division geoscheme. ResultsThere were 78,240 CNS tumor cases included. 20–29-year-old (yo) rates were lower than 30–39 yo in most regions for astrocytic, oligodendroglial and ependymal tumors. Globally, astrocytic tumor incidence decreased (20–29 yo AAPC: − 0.70; 95% CI: − 1.32, − 0.08) while incidence increased for oligodendroglial (20–29 yo AAPC: 3.03; 95% CI: 1.57–4.51; 30–39 yo AAPC: 2.67; 95% CI: 0.79–4.58), ependymal (20–29 yo AAPC: 1.16; 95% CI: 0.31–2.03; 30–39 yo AAPC: 2.29; 95% CI: 1.14–3.46), medulloblastoma (30–39 yo AAPC: 0.6; 95% CI: 0.04–1.24) and tumors of the meninges (20–29 yo AAPC: 1.55; 95% CI: 0.04–3.07). There was a 20–40% male incidence excess in all histologies except for meninge tumors (30–39 yo IRR: 0.71; 95% CI: 0.61, 0.84). ConclusionsIncidence of oligodendroglial and ependymal tumors increased globally in 20–39 yo suggesting better diagnoses or changes in risk factors. Males had a higher incidence of CNS tumors for most tumors studied and in most regions.

Renal Neoplasms in Young Adults.

Renal cell carcinoma (RCC) is usually diagnosed in older adults (the median age of diagnosis is 64 years). Although less common in patients younger than 45 years, RCCs in young adults differ in clinical manifestation, pathologic diagnosis, and prognosis. RCCs in young adults are typically smaller, are more organ confined, and manifest at lower stages of disease. The proportion of clear cell RCC is lower in young adults, while the prevalence of familial renal neoplastic syndromes is much higher, and genetic testing is routinely recommended. In such syndromic manifestations, benign-appearing renal cysts can harbor malignancy. Radiologists need to be familiar with the differences of RCCs in young adults and apply an altered approach to diagnosis, treatment, and surveillance. For sporadic renal neoplasms, biopsy and active surveillance are less often used in young adults than in older adults. RCCs in young adults are overall associated with better disease-specific survival after surgical treatment, and minimally invasive nephron-sparing treatment options are preferred. However, surveillance schedules, need for biopsy, decision for an initial period of active surveillance, type of surgery (enucleation or wide-margin partial nephrectomy), and utilization of ablative therapy depend on the presence and type of underlying familial renal neoplastic syndrome. In this pictorial review, syndromic, nonsyndromic, and newer RCC entities that are common in young adults are presented. Their associated unique epidemiology, characteristic imaging and pathologic traits, and key aspects of surveillance and management of renal neoplasms in young adults are discussed. The vital role of the informed radiologist in the multidisciplinary management of RCCs in young adults is highlighted. Online supplemental material is available for this article. ©RSNA, 2022.

NanoString Digital Molecular Profiling of Protein and microRNA in Rhabdomyosarcoma.

Simple SummaryNanoString digital profiling methods are novel techniques to identify biologic markers from human formalin-fixed, paraffin-embedded cancer tissue. We have applied NanoString Digital spatial profiling and microRNA profiling methods in non-alveolar rhabdomyosarcoma, a common soft tissue tumor in young adults and children with variable prognosis. Our results have highlighted aberrant miRNA expression and over-expression of several members of PI3-AKT, MAPK and apoptosis signaling pathways in fusion-negative rhabdomyosarcoma, particularly in tumors with unfavorable prognosis. INPP4B, an entry molecule in the PI3/AKT pathway, was significantly over-expressed in tumors with poor prognosis, confirmed by traditional immunohistochemistry. Several microRNAs had increased expression in association with poor patient prognosis. These results highlight the utility of NanoString digital profiling as a screening method to identify prognostic biomarkers of interest in rhabdomyosarcoma from formalin-fixed paraffin-embedded tissue.Purpose: Rhabdomyosarcoma (RMS) exhibits a complex prognostic algorithm based on histologic, biologic and clinical parameters. The embryonal (ERMS) and spindle cell-sclerosing RMS (SRMS) histologic subtypes warrant further studies due to their heterogenous genetic background and variable clinical behavior. NanoString digital profiling methods have been previously highlighted as robust novel methods to detect protein and microRNA expression in several cancers but not in RMS. Methods/Patients: To identify prognostic biomarkers, we categorized 12 ERMS and SRMS tumor cases into adverse (n = 5) or favorable (n = 7) prognosis groups and analyzed their signaling pathways and microRNA profiles. The digital spatial profiling of protein and microRNA analysis was performed on formalin-fixed, paraffin-embedded (FFPE) tumor tissue using NanoString technology. Results: The detectable expression of several component members of the PI3K/AKT, MAPK and apoptosis signaling pathways was highlighted in RMS, including INPP4B, Pan-AKT, MET, Pan-RAS, EGFR, phospho-p90 RSK, p44/42 ERK1/2, BAD, BCL-XL, cleaved caspase-9, NF1, PARP and p53. Compared to cases with favorable prognosis, the adverse-prognosis tumor samples had significantly increased expression of INPP4B, which was confirmed with traditional immunohistochemistry. The analysis of microRNA profiles revealed that, out of 798 microRNAs assessed, 228 were overexpressed and 134 downregulated in the adverse prognosis group. Significant over-expression of oncogenic/tumor suppressor miR-3144-3p, miR-612, miR-302d-3p, miR-421, miR-548ar-5p and miR-548y (p < 0.05) was noted in the adverse prognosis group. Conclusion: This study highlights the utility of NanoString digital profiling methods in RMS, where it can detect distinct molecular signatures with the expression of signaling pathways and microRNAs from FFPE tumor tissue that may help identify prognostic biomarkers of interest. The overexpression of INPP4B and miR-3144-3p, miR-612, miR-302d-3p, miR-421, miR-548y and miR-548ar-5p may be associated with worse overall survival in ERMS and SRMS.

BRACHIAL PLEXOPATHY AND CARDIAC TAMPONADE: AN UNCOMMON PRESENTATION OF DIFFUSE LARGE B-CELL MEDIASTINAL LYMPHOMA

TOPIC: Disorders of the Mediastinum TYPE: Medical Student/Resident Case Reports INTRODUCTION: Primary mediastinal large B-cell lymphoma (PMLBCL) is a unique type of B-cell lymphoma probably arising from a putative thymic medulla B-cell. PMLBCL comprises seven percent of diffuse large B cell lymphomas (2.4 percent of all non-Hodgkin lymphomas). There is a female predominance and a median age at diagnosis in the third to fourth decade. In some cases, the presentation can be an oncological emergency with locally invasive anterior mediastinal mass arising from thymus causing external pressure on the heart and the surrounding structures. PMLBCL presenting as brachial plexopathy with cardiac tamponade is an uncommon presentation. CASE PRESENTATION: 37-year-old female, who presented to the ED with a 3-week history of left- sided neck pain and progressive swelling with associated 2 weeks history of numbness in the left wrist, tingling sensation in her 4th and 5th finger with weakness on the same side. She endorsed exertional dyspnea and palpitation that worsened over the last 4 days prior to presentation.On examination she was noted to have left sided neck mass about 6 x 8cm, firm, nontender, non-mobile with extension below the clavicle. Tachycardic with Muffled heart sounds and grade 3/6 Systolic Ejection Murmur at right upper sternal border. Flexion deformity with reduced sensation of the left 4th and 5th finger. Chest X-ray shows cardiomegaly with widened mediastinum. Computed Tomography scan was suggestive of anterior mediastinal mass. Echocardiogram done showed large pericardial effusion with Echocardiographic findings consistent with tamponade physiology.She underwent pericardiocentesis with 700 cc of serosanguinous fluid drained and subsequently had CT guided biopsy of the anterior mediastinal mass. Biopsy report showed high grade B-cell lymphoma with Immunostain showing lymphoma cells positive for CD20, BCL6 and negative for BCL2, CD5. She was diagnosed to be High grade stage IIB, commenced on chemotherapy and completed 6 cycles of DA-EPOCH-R.Left hand weakness and neuropathy resolved following chemotherapy. Repeat CT chest and Echocardiogram showed near resolution of the anterior mediastinal mass and pericardial effusion respectively. DISCUSSION: The majority of patients with primary diffuse large B-cell mediastinal lymphoma are symptomaticat the time of diagnosis. Patients present with a locally invasive anterior mediastinal massoriginating in the thymus, with frequent airway compromise and superior vena cava (SVC)syndrome. Symptoms related to the compression or invasion of local structures (i.e., cough, dyspnea, dysphagia, hoarseness) are common, systemic B-symptoms occurs in about 47% ofpatients, brachial plexopathy is an uncommon presentation. CONCLUSIONS: PMBL is a tumor of young adults who present with a rapidly proliferating tumor. At diagnosis, the tumor is usually limited to the mediastinum, therefore high index of suspicion is required in making the diagnosis. REFERENCE #1: A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. The Non-Hodgkin's Lymphoma Classification Project. Blood. 1997 Jun1;89(11):3909-18. PMID: 9166827 REFERENCE #2: Armitage JO, Weisenburger DD. New approach to classifying non-Hodgkin's lymphomas:clinical features of the major histologic subtypes. Non-Hodgkin's Lymphoma ClassificationProject. J Clin Oncol. 1998 Aug;16(8):2780-95. doi: 10.1200/JCO.1998.16.8.2780. PMID:9704731. REFERENCE #3: van Besien K, Kelta M, Bahaguna P. Primary mediastinal B-cell lymphoma: a review ofpathology and management. J Clin Oncol. 2001 Mar 15;19(6):1855-64. doi:10.1200/JCO.2001.19.6.1855. PMID: 11251018. DISCLOSURES: No relevant relationships by Regine Afable, source=Web Response No relevant relationships by Ibrahim Omore, source=Web Response No relevant relationships by Farbod Raiszadeh, source=Web Response

Presentation and survival of gastro-entero-pancreatic neuroendocrine tumors in young adults versus older patients

BackgroundA minority of patients with gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs) is diagnosed at younger age. This population-based study explores the broad clinical and pathologic features of the youngest 5% of adult patients with GEP-NETs. MethodsA retrospective study of the National Cancer Database (NCDB) of patients with a primary GEP-NET was performed. Patients were stratified by age. Kaplan-Meier and multivariate Cox proportional hazards analyses were performed. ResultsWe identified 31,983 patients with a diagnosis of a GEP-NET and only 5% of patients were under the age of 35. Young patients were found to have greater proportions of localized, well differentiated disease. On multivariate analysis, young age, well differentiated histology, early stage, and surgical intervention were associated with lower risk of mortality. ConclusionsYoung patients with GEP-NETs tend to have earlier stage of presentation and well differentiated tumors, which may be most amenable to surgical intervention.

Primary pancreatic Ewing’s sarcoma: Dreadful yet treatable alien

Ewing's sarcoma is a highly aggressive malignant tumour most commonly affecting long bones in children and adolescents. Primary presentation in Pancreas is considered to be an extremely rare and only a handful of cases have been published to date. Our case intensifies the importance to recognize this rare type of tumor in young adults with a primary pancreatic lesion as there is a broad spectrum of tumours with a similar morphological and radiological presentation. A 26-year-old man was evaluated for upper abdominal pain with cross sectional imaging studies which revealed 3 × 3.3 cm complex cystic lesion in head of pancreas. Serum tumor markers and other blood investigations were within normal limits. We conducted tumour board meeting and proceeded for laparoscopic pancreaticoduodenectomy. Post-operative whipples specimen was subjected for histopathological analysis and Immunohistochemistry which confirmed the diagnosis of Ewing's sarcoma of pancreas. Currently, the patient is receiving regular follow-up care and received 3 cycles of adjuvant chemotherapy till now and has no evidence of cancer at six months post-surgery. Primary pancreatic Ewing sarcoma is a very rare highly aggressive malignant tumour that should be considered in differential diagnosis of an unusual pancreatic tumour especially in young adults. Being rare, we only have 32 reported cases up-to-date. Establishing the correct diagnosis requires a multidisciplinary approach involving close cooperation between clinical, radiological and pathological teams with accurate application of immunohistochemistry or cytogenetic analysis. Complete surgical excision with adjuvant chemotherapy and with or without radiation therapy is the standard of care.

English

Primary malignant extra gonadal germ cell tumours (GCT) of mediastinum are very rare tumours, comprising 1 - 4 % of all mediastinal tumours.1 Even though they are rare tumours of mediastinum, they should always be considered in the differential diagnosis of mediastinal tumours in young adults. Primary malignant GCT can be divided into 2 groups: Seminomatous and non-seminomatous tumours. Mature teratomas (non-seminomatous) are the most common primary malignant germ cell tumours of mediastinum.2 Non-seminomatous tumours tend to be more aggressive than seminomatous tumours present as heterogeneous masses with areas of necrosis and show local invasion. Seminomas usually present as lobulated homogeneous masses and rarely show signs of local invasion. These tumours usually present with asymptomatic lesions or with symptoms due to compression of adjacent structures and usually have a better prognosis than non-seminomatous tumours. Here, we present a case of primary malignant extra gonadal seminoma with sternal involvement.

Clinical behavior of advanced giant cell tumor: 15 years observation study in N.N. Blokhin National Medical Research Center of Oncology.

e23501 Background: Giant cell tumor of bone (GCT) is a relatively rare, benign but locally aggressive osteolytic skeletal neoplasm of young adults, most frequently occurs at the epiphysis of long bones. Distant metastases occur approximately 2-6% of cases most often to the lungs. However, pulmonary metastases do not carry the same connotation as metastases associated with malignant tumors, such as lung cancer or sarcoma. Rarely, GCT undergoes true malignant transformation. The aim of this study is to analyze the clinical behavior of advanced GCT during long-term observer in N.N. Blokhin National Medical Research Center of Oncology. Methods: We observed 51 advanced cases among 298 GCT from 2005 till 2020 in N.N. Blokhin National Medical Research Center of Oncology. Disease was histologically confirmed by a sarcoma pathologist. Patients underwent CT/MRI every 2 or 3 months of treatment and every 3, 6 or 12 months of follow up period. Treatment options were included atypical pulmonary resection, palliative surgery, chemotherapy with CAP or AI combination, interferon alfa or observation until progression. After 2013 prefer treatment option for advanced GCT was denosumab with once in 3 month’s regimen after 2 years monthly therapy. All patients received daily calcium and vitamin D supplement. Logistic regression was using for statistic analyzes. Results: Median follow-up was 45 months. The average age of patients was 34,8 years, and the women and men ratio was about 1,8:1. The most commonly affected sites were tibia (23,5%, 12/21), sacrum (17,6%), radius (13,7%) and femur (9,8%). According Campanacci classification G3 was the most commonly grade (90%). 46 (56%) cases were anatomically compounded due to tumor localization and 19 (37%) cases were primary disease. 18/298 (6%) cases were with pulmonary metastases. Surgery in history (p &lt; 0,001) and tumor localization in extremity (p &lt; 0,001) were significant for metastasis development. The primary malignant GCT observed in 1% (3/298) cases and 1 % cases of malignant transformation GCT into sarcoma. Only 1/51 (1,9%) death was observed in advanced GCT group. Complications ≥3 grade were observed only in pre-denosumab era. Conclusions: In this study we showed long-term observation of advanced GCT and evaluated significant factors for metastasis development. Surgeries in history and tumor localization were associated with higher risk of metastasis. Denosumab for advanced GCT is a choice of treatment, we wasn’t observed any cases of GCT malignant transformation during denosumab treatment. Quality of live was better in compare with pre-denosumab era. Further investigation of long term denosumab complications is awaited.

Osteosarcoma of rib treated with regorafenib: a case report

Osteosarcoma is a common primary malignant bone tumor in young adults presenting with back and chest pain complaints. Here, we presented a rare case of osteosarcoma.

Diagnostic Dilemma: Synovial Sarcoma Masked As Common Gynecological Pathologies

Study Objective Synovial sarcoma most commonly presents as a soft tissue tumor in young adults. This case report illustrates a diagnostic dilemma, in which a rare tumor was misdiagnosed on imaging on two separate occasions as common gynecological pathologies. Design N/A Setting N/A Patients or Participants N/A Interventions N/A Measurements and Main Results 45-year-old woman presented to the GynOncology clinic as a referral for management of a pedunculated leiomyoma identified on MRI. The patient was counseled on options, and agreed to placement of an intrauterine device. She re-presented two years later, and pelvic ultrasound showed that the previously identified fibroid was now believed to be a dermoid cyst; with interval increase in size, measuring 10.6 × 12.1 × 15. The patient underwent diagnostic laparoscopy, where a mass of indeterminate origin was noted; involving the round ligament, broad ligament, abdominal peritoneum, and abutting the dome of the bladder. Intraoperative frozen section returned indeterminate. Given the patient's desire for future fertility and uncertainty of malignancy, the decision was made to await final pathology before proceeding with surgical management. Pathology showed low-grade spindle-cell sarcoma with molecular features of synovial sarcoma. In two weeks time, the patient underwent a robotic-assisted total laparoscopic hysterectomy, bilateral salpingo-oophorectomy, and tumor debulking. Per the operative report, the mass that was first thought to be a pedunculated fibroid and then a dermoid cyst was an approximately 20cm retroperitoneal mass originating from the left broad ligament and occupying the entire left pelvic sidewall, pararectal space, paravesical space, and the space of Retzius and encasing the left ureter. Final pathology showed synovial sarcoma, which was confirmed with FISH results. Conclusion This case demonstrates synovial sarcoma misdiagnosed as common gynecological pathologies. This rare pathology is one that gynecologists should be aware of, as its early recognition and treatment allows for improved survival benefit with adjuvant chemotherapy.

Midline Posterior Fossa Tumors in Young Adults: Evolution of Thought and Practice

Objective: The aim of our study was to evaluate the surgical outcome of posterior fossa lesions and to find out the relation between surgical skill and post-operative outcome and to determine how evolution in surgical skill dictates post-operative outcome.&#x0D; Material &amp; Methods: This was a retrospective analysis of 207 patients with midline posterior fossa lesions over a period of 10 years from Jan 2009 till December 2018. The degree of surgical outcome was assessed by degree of surgical resection, Post-operative neurological deficits and complications, Stay in ICU and duration in hospital.&#x0D; Results: 207 Midline posterior fossa tumors were operated. Headache was leading symptom in patients followed by vomiting and balance problem. Medulloblastoma was a most common pathology in 133, Ependymoma 32, Astrocytoma in 28 Choroid Plexus Papilloma in 3, dermoid cyst 3,arachnoid cyst 4 and Hemangioblastoma in 4 patients. Gross Total resection was possible in 110 patients. Post operatively gag reflex was impaired in 36 and facial palsy in 5 patients. Chest infection was the most common complication in 84 patients, followed by wound infection, CSF Leak and Meningitis. Mortality was 11.59% as 24 patients died within one month, while 28.5% patients stayed in ICU.&#x0D; Conclusion: Post-operative mortality and morbidity is relatively high among post fossa tumors. Improvement in surgical skills better instruments revealed better outcome over a period of 10 years due to better understanding of microsurgical anatomy and utilization of modern therapeutic strategies for resection.