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45,X/46,XY mosaicism is a rare condition with clinical and genetic heterogeneity and have a greatly increased risk of developing germ cell tumors. We describe a rare 45,X/46,XY Chinese girl with malignant tumors, especially focusing on the molecular genetics of gonadal tumor. We report a phenotypically Turner-like Chinese adolescent girl who presented primary amenorrhea and a pelvic mass as the chief complaint, which finally demonstrated dysgerminoma replacing the left gonad and gonadoblastoma arising from right gonad respectively. Her chromosome karyotype was 45,X(4)/46,XY(46); Y-chromosome microdeletions in AZFb regions were found on gonadal DNA rather than peripheral blood lymphocyte (PBL) DNA, while no variants were found in the promoter and coding region of SRY gene in both PBL and gonadal tissues. She underwent bilateral gonadectomy; no recurrence or serious complications were identified after 3 years of follow-up. This case emphasizes the probable correlation between Y chromosome microdeletions in gonadal tissue and the severity of the phenotype in patients with 45,X/46,XY mosaicism and highlights the importance of clinical genetic testing at the chromosomal and molecular level. [ABSTRACT FROM AUTHOR]

Fish maintained in managed care may have longer lifespans as a result of advances in veterinary medicine and husbandry and reduced risk of predation. Neoplasia is of increasing interest in managed aquarium populations. However, few studies have systematically evaluated neoplasia in managed fish populations. Our objective in this retrospective study was to review and describe neoplasia diagnosed in fish at a large public display aquarium between 2005 and 2021. Any fish diagnosed with neoplasia on either antemortem or postmortem evaluation during the study period was included, and all medical records, biopsy, and autopsy reports were reviewed. Sixty-two fish met the inclusion criteria; 37 species were included in the study population, most of which were tropical freshwater fish (n = 34 fish). Thirty-two types of neoplasia were identified. Ten fish had benign neoplasms, and 53 fish had malignant neoplasms. The most common neoplasms were of epithelial and neuroectodermal origin. The most common site of tumor origin was the skin. Our data suggest that mesenchymal neoplasms may be more common in cold saltwater fish than in tropical freshwater and saltwater fish. Malignant neoplasms were most commonly diagnosed in the study population and should be a top differential when neoplasms are identified in fish managed under human care. Our study contributes to the overall knowledge of the health of aquarium fish and may aid clinicians in characterizing neoplasia that may be present in fish under human care. [ABSTRACT FROM AUTHOR]

The evolution of treatment for malignant ovarian germ cell tumors has been one of the most successful in the history of gynecologic oncology, with dysgerminoma as the most common type of malignant ovarian germ cell tumors. Since the introduction of platinumbased chemotherapy in the 1980s, 5-year survival rates for early-stage dysgerminomas have been close to 100%, and as high as 98% for advanced stages. Despite this remarkable achievement, many questions remain in routine treatment. By performing a literature review, we aim to highlight both the current treatment of malignant dysgerminoma and unanswered questions in the modern management of this disease. These issues relate firstly to surgical therapy, such as the role of routine omentectomy and lymphadenectomy, the value of complete surgical resection, and the possibility of fertility-sparing surgery. Second, chemotherapy and the question of the possibility of de-escalation in early stages and the potential of neoadjuvant chemotherapy in advanced stages will be addressed. Finally, a brief overview of the current developments of new drug treatment regimens will be given. [ABSTRACT FROM AUTHOR]

We characterized the immunohistochemical expression profiles of dysgerminomas from a 16-y-old maned wolf and 13 domestic dogs using the following biomarkers: Sal-like protein 4 (SALL4), octamer-binding transcription factor 3/4 (OCT3/4), placental alkaline phosphatase (PLAP), c-kit, and vimentin. The maned wolf had nonspecific and long-standing clinical signs of lethargy, anorexia, and weight loss, and was euthanized because of poor prognosis. At autopsy, the left ovary was effaced by a 12 × 8 × 6 cm mass, comprised of anaplastic cells with a mitotic count of 20 mitoses in 10 high power fields. Dysgerminomas from 7 of 13 domestic dogs had nuclear expression of SALL4. Dysgerminomas from the maned wolf and 2 domestic dogs had both nuclear and cytoplasmic expression of SALL4. Cytoplasmic expression of PLAP and OCT3/4 was present in dysgerminomas from the maned wolf and 3 (PLAP) or 4 (OCT3/4) domestic dogs. All dysgerminomas expressed vimentin. Membranous c-kit expression was rare in the dysgerminoma from the maned wolf, and variable in dysgerminomas from 4 domestic dogs. A dysgerminoma from a domestic dog had cytoplasmic expression of c-kit. SALL4 is a useful marker to confirm germ cell origin of dysgerminoma in canids. [ABSTRACT FROM AUTHOR]

To describe the rates of GCNIS-free and GCT-free pathology based on age at gonadal surgery and to describe long-term oncologic outcomes in patients with DSD who have GCNIS or GCT at the time of gonadal surgery. A systematic review was conducted using MEDLINE to identify patients with DSD who underwent gonadal surgery. DSD diagnoses were stratified based on malignancy risk. GCNIS/GCT and GCT-free survival by age of gonadal surgery, RFS and OS were calculated using the Kaplan–Meier method, with groups compared using log-rank testing. 386 articles from 1951 to 2017 were included (2037 patients). Median age at gonadal surgery was 17 years (y) (IQR 11–20), median follow-up was 60 months (m) (IQR 30–68.1). GCNIS/GCT- and GCT-free survival at the time of gonadal surgery was lowest for those in the high/intermediate risk group (p < 0.001) but decreased sharply around age 15y, regardless of risk category. 5y RFS and OS was similar for those with no GCNIS/GCT and GCNIS and was worse for those with GCT (p < 0.001). When patients undergo gonadal surgery, regardless of indication (i.e. prophylactic vs. tumor), it appears that GCTs are more commonly found when surgery is done around age 15 y or older, despite risk category. This is similar to ovarian and testicular GCTs. Patients with GCNIS can be reassured that long-term oncologic outcomes are excellent. While RFS and OS for GCTs are not as good as for ovarian and testicular GCTs (95%), they are still >80%. This similar trend was found in a COG review of 9 patients with DSD and ovarian GCT. There were several limitations to this study. This is a retrospective analysis that included aa wide time frame of publications. The indication for surgical intervention was not addressed in the majority of publications. Thus these data provide pathologic outcomes based on age at gonadal surgery rather than the age at which GCNIS/GCT develops over a lifetime, if at all. The risk of GCNIS or GCT at the time of gonadal surgery appears to increase with age, accelerating between 15 and 20y regardless of risk category. 5y RFS and OS for those with GCNIS is equivalent to those without GCNIS/GCT but is worse for those with GCT. These data may be used when counseling families on timing of gonadal surgery and quantification of outcomes should GCNIS or malignancy be identified. Summary Fig. Image 1 [ABSTRACT FROM AUTHOR]

Germ cell tumours of ovary (GCT) are rapidly growing neoplasms derived from pleuripotent primordial germ cells. They comprise 15–20% of all ovarian neoplasms of which 95% are mature cystic teratomas and 5% are malignant ovarian germ cell tumours (MOGCT). MOGCT continues to be the cause of diverse, diagnostically challenging issues for the pathologist, and they are academically interesting because of the biological diversity exhibited by these tumours in the two gonads (testis and ovaries). Their correct diagnosis often has major important therapeutic and prognostic implications. This review intends to provide a refresher and update on the diagnostic aspects MOGCT including clinical, morphological, immunocytochemical and where relevant the molecular features. Somatic malignancies with a germ cell components and the use of new pluripotency markers that have improved diagnostic accuracy have also been included. [ABSTRACT FROM AUTHOR]

Objective: To investigate the prognostic significance of complete gross resection following cytoreductive surgery for patients with advanced stage malignant ovarian germ cell tumors. Methods: The National Cancer Data Base was accessed and patients diagnosed with an advanced stage (II-IV) malignant ovarian germ cell tumor who underwent primary cytoreductive surgery between 2011 and 2014 were selected for further analysis. For analysis purposes two groups were formed: patients with complete gross resection and those with macroscopic residual disease. Demographic and clinico-pathological characteristics were compared with the chi-square and Mann–Whitney U test. Univariate survival analysis was performed with the log-rank test after generation of Kaplan–Meier curves, while a Cox proportional hazard model was constructed to evaluate mortality after controlling for confounders. Results: A total of 343 patients who met the inclusion criteria were identified. Residual disease status was available for 276 patients: the rate of complete gross resection was 69.2 %. By univariate analysis there was no difference in overall survival between patients in the complete gross resection and macroscopic residual disease groups, P= 0.26; 3-year overall survival rates: 86.4 % and 82.8 %, respectively. No difference in overall survival was noted following stratification by histology; P = 0.64 and P = 0.24 for dysgerminoma and non-dysgerminoma tumor groups. After controlling for stage IV disease, histology and the administration of chemotherapy, macroscopic residual disease was not associated with a worse mortality (HR: 1.22, 95% CI: 0.61 to 2.46). Conclusions: Macroscopic residual disease following primary cancer-directed surgery was not associated with a worse prognosis in a cohort of patients with advanced stage malignant ovarian germ cell tumors. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the role of omentectomy and lymphadenectomy in the treatment of clinically apparent early-stage malignant ovarian germ cell tumors. Methods: We retrospectively reviewed 245 patients with malignant ovarian germ cell tumors (yolk sac tumor, dysgerminoma, and immature teratoma) and with clinically early-stage disease, who were treated at Sun Yat-sen University Cancer Center between January 1, 1970 and December 31, 2017. The survival of patients who underwent either omentectomy or lymphadenectomy, or both (omentectomy/lymphadenectomy group) was compared with that of patients who did not undergo omentectomy or lymphadenectomy (non-omentectomy/lymphadenectomy group). Results: Sixty patients were diagnosed with yolk sac tumor, 74 with dysgerminoma, and 111 with immature teratoma. Of these 245 patients, 216 patients had stage I disease, 28 patients had stage II, and 1 patient had stage IIIA. There were 190 patients who underwent omentectomy and/or lymphadenectomy and 55 patients in the non-omentectomy/lymphadenectomy group, respectively. In the omentectomy/lymphadenectomy group, 112 patients underwent both omentectomy and lymphadenectomy, 71 underwent omentectomy only, and 7 underwent lymphadenectomy only. Two hundred and fourteen of 245 patients (87.3%) received post-operative chemotherapy. Median follow-up was 73 months (range 1–388). The 10-year overall survival rates in the omentectomy/lymphadenectomy group and non-omentectomy/lymphadenectomy groups were 96.8% and 100%, respectively (p=0.340). Multivariate analysis evaluating all potential prognostic factors showed that omentectomy and lymphadenectomy are not prognostic factors for survival. Conclusions: Omentectomy and lymphadenectomy do not appear to improve survival and may be omitted in patients with clinically apparent early-stage malignant ovarian germ cell tumors. [ABSTRACT FROM AUTHOR]

Copyright of Revista Medica Herediana is the property of Universidad Peruana Cayetano Heredia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The article presents a case study of a 13-year-old female captive-born orange-spot freshwater stingray with an acute caudodorsal swelling. The captive-born ocellate river stingray underwent an ultrasonography using manual restraint and revealed an irregular organized intracoelomic mass of mixed echogenicity. The orange-spot freshwater stingray has also underwent a surgical excision as well as radiation and chemotherapy for treating dysgerminomas.

We discuss germ cell tumours of the ovary, beginning with dysgerminoma as it is the most common malignant germ cell tumour in this location. Issues in differential diagnosis are highlighted as this tumour is associated with an excellent outcome nowadays and can be confused with small cell carcinoma, clear cell carcinoma, and rarely other neoplasms. The many patterns of yolk sac tumour are noted including the recently emphasized solid growth that may mimic dysgerminoma. Immunohistochemical stains are helpful in the diagnosis of both these primitive tumours, both being SALL4 positive and dysgerminoma expressing OCT4, D2-40 and c-KIT and yolk sac tumour having been recently reported to be GATA3 positive. Glypican-3 is also positive in yolk sac tumour but should be used with caution as it is often expressed in surface epithelial carcinomas, particularly clear cell carcinoma which can be in its differential. Where the fascinating polyembryoma should be placed in the classification of ovarian tumours is briefly considered. In the teratoma family emphasis is placed on the gross differences between the mature cystic and immature forms. The most important categories of monodermal teratoma are then considered emphasizing their varied problems in differential diagnosis. [ABSTRACT FROM AUTHOR]

Copyright of Revista Chilena de Obstetricia y Ginecología is the property of Revista Chilena de Obstetricia y Ginecologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Residual mature teratoma,a frequent finding in clinical pathology since the introduction of cisplatin-based chemotherapy, put Wolter Oosterhuis on the track of germ cell tumors (GCTs). These neoplasms in the borderland between developmental biology and oncology have fascinated him ever since. He tells the story on how GCTs brought him in contact with leading investigators in the field like Ivan Damjanov, Peter Andrews, and Niels Skakkebaek. His fruitful line of research was made possible through a longstanding collaboration with Bauke de Jong and, to this day, Leendert Looijenga who joined his group as a student in 1988. Probably their most important contribution to the field of GCTs is an integrated approach to GCTs, combining epidemiology, pathology, (cyto) genetics and molecular biology, that has resulted in a pathobiology-based classification of GCTs in five types. It has clinical relevance and stimulates further research on these intriguing neoplasms and their corresponding animal models. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: Diagnosis of dysgerminoma in the paediatric age group is uncommon, and most cases arise from dysgenetic gonads of 46, XY pure gonadal dysgenesis (PGD) patients. Bilateral gonadectomy is mandatory in these patients. So, the preoperative diagnosis of PGD is important in order to avoid multiple surgical procedures and delayed patient information in the case of a suspected ‘ovarian’ tumour. Our aim was to discuss preoperative clues that can lead to suspicion of dysgerminoma in the context of PGD. Materials and methods: We reviewed the charts of six patients treated for dysgerminoma associated with 46, XY PGD. We focused on particularities of clinical and biological evaluations. Results: Median age at diagnosis was 11 years. Pubertal development was absent or incomplete even at late ages. Dysgerminoma was associated with gonadoblastoma foci in all cases. Tumoral marker profile was a normal alfafetoprotein level, a high lactate dehydrogenase level and normal or moderate human chorionic gonadotropin (βHCG) secretion, except for one patient who had a mixed tumour with notably a choriocarcinoma share (high βHCG). Follicle-stimulating hormone (FSH) level was very high in all patients tested and, interestingly, also in one prepubertal patient. Conclusions: In the case of a suspected ovarian tumour, delayed pubertal development, moderate βHCG level and elevated FSH level are clinical and biological clues to a diagnosis of dysgerminoma in the context of PGD and should prompt karyotype analysis before surgery. Because FSH is an efficient indirect marker of this condition, we suggest including this analysis in the management of gonadal tumours. [Copyright &y& Elsevier]

The article presents a retrospective study of the prognosis and development of ovarian germ cell tumors in children. The study, which involved 137 patients with a median age of 9.5 years, revealed that most of the patients were found to have teratomas, followed by yolk sac tumors, and dysgerminoma. The authors also conclude that patients with ovarian germ cell tumors are expected to have excellent survival rates with accurate staging, complete resection, and adjuvant therapy.

Abstract: Patients: Eleven patients with 46,XY PGD were divided into two groups. Six symptomatic girls (group 1) were referred for amenorrhea (n = 3), gonadal tumor (n = 2) or campomelic dysplasia (n = 1). Five asymptomatic screened patients (group 2) were diagnosed as 46,XY PGD after familial investigation of the two probands with gonadal tumor. Bilateral gonadectomy was performed in all patients. Results: In group 1, pathologic examination revealed an association of dysgerminoma with gonadoblastoma (n = 2), bilateral gonadoblastoma (n = 2) and streak gonads (n = 2). Prophylactic gonadectomy in asymptomatic patients (group 2) also showed asymptomatic dysgerminoma with gonadoblastoma (n = 1), bilateral gonadoblastoma (n = 2) and streak gonads (n = 2). Conclusions: A gonadal tumor arising in a girl with pubertal delay may be related to dysgenesis of the gonad. Primary amenorrhea or diagnosis of dysgerminoma should warrant karyotype, and familial study if 46,XY PGD is found. Considering the high incidence of gonadoblastoma and the early occurrence of dysgerminoma, early bilateral gonadectomy is recommended. [ABSTRACT FROM AUTHOR]

Abstract: An 8-year-old phenotypic female with campomelic dysplasia (CD) and 46,XY sex-reversal presented with renal colic. Medullary nephrocalcinosis, urolithiasis, and renal malrotation were diagnosed by computed tomographic scanning. Pelvic sonogram identified an enlarged left gonad. Genetic testing revealed a novel SOX9 heterozygous deletion of a cytosine at nucleotide 972 (972delC), causing a frameshift at codon 200, introducing a stop codon 18 codons further downstream (P200fsX218). At laparoscopic gonadectomy, a left dysgerminoma was removed. This first reported case of dysgerminoma in a sex-reversed patient with CD who also had urolithiasis stresses the importance of prophylactic gonadectomy and urologic evaluations in this susceptible population. [Copyright &y& Elsevier]

Summary: Dysgerminoma is a malignant germ cell tumor of the ovary that shares morphological, immunophenotypic, and genetic features with its testicular counterpart, seminoma. Recent evidence supports the hypothesis that seminoma can differentiate into non-seminomatous germ cell tumor types. The progression of these tumors can be measured by their acquisition of the potential to express cytokeratin intermediate filaments, a characteristic specific to epithelial differentiation. Although testicular seminomas have been widely investigated, little is known about cytokeratin or E-cadherin expression in dysgerminomas. We investigated 26 formalin-fixed, paraffin-embedded ovarian dysgerminomas with immunohistochemical stains for CAM5.2, AE1/AE3, epithelial membrane antigen, cytokeratin 7, cytokeratin 20, high-molecular-weight keratin, and E-cadherin. In addition, we investigated the CD30 and vimentin immunoreactivity of these tumors. Immunoreactivity for CAM5.2 and for AE1/AE3 was present in more than 10% of neoplastic cells in 5 (19.2%) of 26 cases and in 2 (7.7%) of 26 cases, respectively. Cytokeratin 7 showed only focal positivity and never showed positive staining in greater than 10% of dysgerminoma cells. E-cadherin staining was positive in 2 cases showing weak membranous immunostaining in more than 10% of cells. Vimentin immunoreactivity was observed in only 2 dysgerminomas, both of which had less than 10% of the neoplastic cells staining. Cytokeratin 20, epithelial membrane antigen, high-molecular-weight keratin, and CD30 were consistently negative in all cases. Our study demonstrates that cytokeratin expression in dysgerminomas is not unusual and is consistent with the hypothesis that dysgerminomas have the capacity to differentiate along epithelial lines. Furthermore, the immunohistochemical staining patterns for cytokeratins, E-cadherin, and CD30 in dysgerminomas need to be considered when assessing differential diagnoses in difficult cases of primary ovarian tumors. [Copyright &y& Elsevier]

Satgé D, Honoré L, Sasco AJ, Vekemans M, Chompret A, Réthoré M-O. An ovarian dysgerminoma in Down syndrome. Hypothesis about the association. Int J Gynecol Cancer 2006; 16(Suppl. 1):375–379. An 11-year-old girl with Down syndrome (DS) was operated for a stage I right ovary dysgerminoma. She is in good health 33 years later. Some data in the literature suggest that ovarian cancers could be slightly overrepresented in DS. Despite the rarity of ovarian dysgerminoma, our case is the fifth reported in DS. This case is the second one associated with a family history suggesting the possibility of a familial predisposition to cancer. A hypothesis explaining the development of dysgerminoma in DS is proposed. [ABSTRACT FROM AUTHOR]

Summary: Carcinoma in situ (CIS) is the precursor of malignant testicular germ cell tumors (GCTs) of adolescents and young adults, being the neoplastic counterpart of primordial germ cells/gonocytes. Carcinoma in situ cells will develop into invasive seminoma/nonseminoma. Gonadoblastoma (GB) is the precursor of invasive GCTs in dysgenetic gonads, predominantly dysgerminoma (DG). In this process, part of the Y chromosome (GBY region) is involved, for which TSPY is a candidate gene. A detailed immunohistochemical survey was performed for the known diagnostic markers, germ cell/placental alkaline phosphatase (PLAP), c-KIT, and OCT3/4, as well as testis-specific protein on the Y chromosome (TSPY) on a series of GBs, and adjacent invasive DGs. All 5 patients were XY individuals (4 females and 1 male). In contrast to c-KIT, PLAP was positive in all cases. The immature germ cells of GBs were positive for OCT3/4, whereas the mature germ cells were negative for this marker, but positive for TSPY. In every GB, a minor population of germ cells positive for both markers could be identified, similar to most CIS cells and early invasive DG. On progression to an invasive tumor, TSPY can be lost, a process that is also detectable in invasive testicular GCTs compared to CIS. These results indicate that GB is a heterogeneous mix of germ cells, in which the OCT3/4-positive cells have the potential to undergo progression to an invasive tumor. These early invasive stages are initially also positive for TSPY (like CIS), supporting a positive selection mechanism. Therefore, OCT3/4 in combination with TSPY is valuable to identify malignant germ cells in dysgenetic gonads. This could allow better prediction of the risk of progression to a GCT. In addition, the data support the model that GB represents the earliest accessible developmental stage of malignant GCTs. [Copyright &y& Elsevier]

Thirty-six patients with malignant germ cell tumors (GCT) of the ovary were treated during the period from 1971 to 1990. Twenty-five of these patients underwent initial surgery at this institution; 11 were referred after initial surgery. Endodermal sinus tumor (EST) was the most common subtype (N = 13), followed by immature teratoma (IMT) N = 11), dysgerminoma (N = 9), and mixed germ cell tumor (N = 3). Eight of nine patients with dysgerminoma and 14 of 27 patients with non-dysgerminomatous germ cell tumors had stage I disease. Surgical staging resulted in a change of stage in 10 of 31 patients; seven were upstaged, and three were downstaged. Thirty-five of 36 patients are alive with no evidence of disease at a median follow-up of 47 months (range 12-210 months). Two of nine patients with dysgerminoma received postoperative radiotherapy; two additional patients required radiotherapy for recurrence. Twenty-five of 27 patients with non-dysgerminomatous tumors were treated with combination chemotherapy. Second-look laparotomy was done in 18 patients, four of whom had positive results. Three of the four patients with positive second-look laparotomy results remain free of disease after salvage chemotherapy; the fourth patient died of progressive grade 3 IMT. Nineteen of 27 patients undergoing fertility-preserving surgery have resumed normal menses, and four of these have delivered normal infants. The other eight patients remain on oral contraceptives. We conclude that surgical staging provided important information in this study of 36 patients with ovarian germ cell malignancies. As noted by many previous reports, combination chemotherapy (developed during the past 20 years) has dramatically improved prognosis for this group of patients. Second-look laparotomy (SLL), especially for patients with advanced disease, was able to identify patients requiring additional therapy. Most patients with this disease can retain their normal, uninvolved ovary with preservation of normal menstrual and reproductive function. [ABSTRACT FROM AUTHOR]

Extraovarian germ cell tumors are very rare and their occurrence during pregnancy is exceptional. In this case report an abdominal mass was shown by ultrasonography, during a routine monitoring of a 26-year-old pregnant woman. The patient was left under radiological control in the following months in order to bring the pregnancy to term. A few months after the delivery, the patient underwent surgery and a diagnosis of extraovarian (abdominal) dysgerminoma was made. To the best of our knowledge, there are only 3 other case reports describing an extra-gonadal dysgerminoma occurring during pregnancy. The aim of this study was to report an extremely rare tumor, whose management can be challenging first because this neoplasm has some differences from its ovarian and testicular counterparts. Furthermore, the occurrence during pregnancy makes the multidisciplinary approach mandatory since 3 distinct but not independent entities are involved (tumor, mother and fetus). [ABSTRACT FROM AUTHOR]

Abstract: This report concerns a 40-year-old woman case of ovarian dysgerminoma presenting the MRI findings of predominantly hemorrhagic multilocules with papillary projections and irregular septa. [Copyright &y& Elsevier]

Prado S, Yazigi R, Garrido J, Gonzalez M, Torres R, Oddo D. Recurrent ovarian dysgerminoma after laparoscopy. Int J Gynecol Cancer 2006; 16(Suppl. 1):397–399. To our knowledge, recurrent dysgerminoma at the site of tumor removal by laparoscopy in a patient with stage IA disease has not been previously reported. A woman with ovarian dysgerminoma treated by laparoscopy and tumor removed through the cul-de-sac recurred the 17 months later at the site of tumor removal. She was successfully treated with etoposide, bleomycin, and cisplatin chemotherapy with complete response. This case illustrates the potential for surgical site implant of an ovarian dysgerminoma; surgeons should follow strict guidelines when performing laparoscopic procedures for ovarian malignancies in order to prevent this type of incident. [ABSTRACT FROM AUTHOR]

Gonadal dysgerminomas developed in two girls, aged 12 and 15 years. Both were initially treated with conservative unilateral gonadectomy. Forty-six, XY gonadal dysgenesis was not suspected in either patient due to the normal appearance of the contralateral gonads and internal female genital organs. One died of a second germ cell malignancy which developed in the contralateral ovary 9½ years later. The diagnosis of 46, XY gonadal dysgenesis was established by karyotype in both patients. Although conservative surgical management is desirable for nulliparous women with unilateral dysgerminomas, the presence of 46, XY gonadal dysgenesis should be suspected in all premenarchal girls with ovarian germ cell malignancies. If karyotyping reveals the presence of an Y chromosome, bilateral gonadectomy is indicated because of the risk that another neoplasm may develop in the contralateral ovary. [ABSTRACT FROM AUTHOR]

Summary Introduction Patients with gonadal dysgenesis (GD) with a Y chromosome have an increased risk of gonadal neoplasm. Few data exist on the ability of imaging to detect malignancy in intra-abdominal gonads in these patients. Objective We aimed to determine the correlation between preoperative imaging findings and gonadal pathology in GD patients with Y chromosome material. Methods A retrospective review was performed of patients with XY or XO/XY GD who underwent gonadectomy at our institution from 2003 to 2017. Patients were assessed preoperatively with ultrasonography; some additionally underwent MRI. Results The series consisted of 10 patients, all with female gender and non-palpable gonads. Median age was 13.1 years (range 2.4–18.3 years). Overall, four of the ten patients (40%) had a tumor (gonadoblastoma or dysgerminoma) on final pathology. Four patients had a gonad or gonads that were definitively seen on ultrasonography. All visualized gonads were described as “normal” or “small” with the exception of one patient, who had a normal MRI. Three of the four patients in this group had a tumor on final pathology. The remaining six patients had a gonad or gonads that were not definitively visualized on ultrasound; one patient in this group had a tumor on final pathology. Overall, five of seven gonads (71%) definitively visualized on ultrasound had tumor on final pathology, and two of thirteen gonads (15%) not visualized on ultrasound had tumor on final pathology; this difference was statistically significant ( p = 0.012). Three patients were imaged with MRI. Of the gonads that could be visualized on MRI, no definitive abnormalities were seen. All patients imaged with MRI had tumors on final pathology. Discussion Both ultrasound and MRI are relatively poor at identifying and characterizing intra-abdominal gonads in GD patients. The majority of patients who had a neoplasm had normal imaging findings. Gonads that were definitively visualized on ultrasound were more likely to contain neoplasms that could not be visualized, which perhaps because of tumor growth. No other consistent imaging findings of malignancy were found. Our study included ultrasound evaluations that were completed over 10 years ago and not performed by pediatric ultrasonographers, which may have biased the results. However, results suggest that when discussing gonadectomy with GD patients, one should not be reassured by “normal” imaging findings. Neither ultrasound nor MRI should be relied on for surveillance in GD patients who decide against gonadectomy. Conclusion A normal ultrasound or MRI does not rule out neoplasm in GD patients with intra-abdominal gonads. [ABSTRACT FROM AUTHOR]

Abstract: Hypercalcaemia is a rare life-threatening complication of paediatric cancer that is commoner in haematological than solid malignancies and associated rarely with acute renal failure. Often refractory to medical therapy, control of hypercalcaemia in children with solid tumours may necessitate excision of localised tumours or urgent chemotherapy for metastatic ones. We present a child with refractory hypercalcaemia, bulky chemosensitive metastatic tumours and acute renal failure in whom chemotherapy posed high-risk of tumour lysis syndrome (TLS). Resection of the metastatic tumours successfully normalised the hypercalcaemia and represents a practical alternative control strategy in cases at high risk of TLS. [ABSTRACT FROM AUTHOR]