Immature Teratoma Research Articles

A rare germline BMP15 missense mutation causes hereditary ovarian immature teratoma in human

Ovarian immature teratomas (OITs) are malignant tumors originating from the ovarian germ cells that mainly occur during the first 30 y of a female's life. Early age of onset strongly suggests the presence of susceptibility gene mutations for the disease yet to be discovered. Whole exon sequencing was used to screen pathogenic mutations from pedigrees with OITs. A rare missense germline mutation (C262T) in the first exon of the BMP15 gene was identified. In silico calculation suggested that the mutation could impair the formation of mature peptides. In vitro experiments on cell lines confirmed that the mutation caused an 84.7% reduction in the secretion of mature BMP15. Clinical samples from OIT patients also showed a similar pattern of decrease in the BMP15 expression. In the transgenic mouse model, the spontaneous parthenogenetic activation significantly increased in oocytes carrying the T allele. Remarkably, a mouse carrying the T allele developed the phenotype of OIT. Oocyte-specific RNA sequencing revealed that abnormal activation of the H-Ras/MAPK pathway might contribute to the development of OIT. BMP15 was identified as a pathogenic gene for OIT which improved our understanding of the etiology of OIT and provided a potential biomarker for genetic screening of this disorder.

Histopathological and Immunohistochemical Evaluation of Malignant Ovarian Tumours

Objective: To determine the frequency and histological types of malignant ovarian tumours using morphological features and immunohistochemistry. Study Design: Cross-sectional study. Place and Duration of Study: Department of Histopathology, Army Medical College, Rawalpindi Pakistan, Jan 2016 to Dec 2018. Methodology: Newly diagnosed cases of malignant ovarian tumour who had not received chemotherapy were included. Cases of benign ovarian tumours and those who were treated with pre-surgical chemotherapy were excluded. Results: In total, 118 cases of malignant ovarian tumours were evaluated. High-grade serous carcinomas were 61(51.7%), which outnumber others, followed by granulosa cell tumours 17(14.4%), germ cell tumours 13(11%), endometrioid carcinoma 9(7.6%), clear cell carcinoma 4(3.4%), mucinous carcinoma 4(3.4%), low-grade serous carcinoma 2(1.7%) and carcinosarcoma in one case (0.8%). Cancer in the ovary was metastatic in 7(5.9%) cases. No Sertoli Leydig cell tumour, malignant Brenner tumour, embryonal carcinoma or immature teratomas were diagnosed. Conclusion: Surface epithelial tumours were the most common malignancy, followed by granulosa cell tumours and dysgerminoma. An increase in the frequency of ovarian tumours in younger age groups was also noted. Immunohistochemistry was a useful adjuvant diagnostic tool in cases of ovarian malignancy. Metastases to the ovary were mostly gastrointestinal in origin.

Identification of Germline FOXE1 and Somatic MAPK Pathway Gene Alterations in Patients with Malignant Struma Ovarii, Cleft Palate and Thyroid Cancer.

Germline variants in the FOXE1 transcription factor have been associated with thyroid ectopy, cleft palate (CP) and thyroid cancer (TC). Here, we aimed to clarify the role of FOXE1 in Portuguese families (F1 and F2) with members diagnosed with malignant struma ovarii (MSO), an ovarian teratoma with ectopic malignant thyroid tissue, papillary TC (PTC) and CP. Two rare germline heterozygous variants in the FOXE1 promoter were identified: F1) c.-522G>C, in the proband (MSO) and her mother (asymptomatic); F2) c.9C>T, in the proband (PTC), her sister and her mother (CP). Functional studies using rat normal thyroid (PCCL3) and human PTC (TPC-1) cells revealed that c.9C>T decreased FOXE1 promoter transcriptional activity in both cell models, while c.-522G>C led to opposing activities in the two models, when compared to the wild type. Immunohistochemistry and RT-qPCR analyses of patients' thyroid tumours revealed lower FOXE1 expression compared to adjacent normal and hyperplastic thyroid tissues. The patient with MSO also harboured a novel germline AXIN1 variant, presenting a loss of heterozygosity in its benign and malignant teratoma tissues and observable β-catenin cytoplasmic accumulation. The sequencing of the F1 (MSO) and F2 (PTC) probands' tumours unveiled somatic BRAF and HRAS variants, respectively. Germline FOXE1 and AXIN1 variants might have a role in thyroid ectopy and cleft palate, which, together with MAPK pathway activation, may contribute to tumours' malignant transformation.

Outcome of patients with stage I immature teratoma after surveillance or adjuvant chemotherapy.

Immature teratomas are rare malignant ovarian germ cell tumours, typically diagnosed in young women, where fertility-sparing surgery is the treatment of choice. The role of adjuvant chemotherapy in stage I disease remains controversial. We evaluated the impact of surveillance versus chemotherapy on the recurrence rate in stage I immature teratomas. We collected a single centre retrospective series of patients with stage I immature teratomas treated with fertility-sparing surgery at San Gerardo Hospital, Monza, Italy, between 1980 and 2019. Potential risk factors for recurrence were investigated by multivariate logistic regression. Of the 74 patients included, 12% (9/74) received chemotherapy, while 88% (65/74) underwent surveillance. Median follow-up was 188 months. No difference in recurrence was found in stage IA/IB and IC immature teratomas [10% (6/60) vs. 28.6% (4/14) (P=0.087)], grade 1, grade 2, and grade 3 [7.1% (2/28) vs. 14.3% (4/28) vs. 22.2% (4/18) (p=0.39)], and surveillance versus chemotherapy groups [13.9% (9/65) vs. 11.1% (1/9)) (p = 1.00)]. In univariate analysis, the postoperative approach had no impact on recurrence. The 5-year disease-free survival was 87% and 90% in the surveillance and chemotherapy groups, respectively; the overall survival was 100% in both cohorts. Our results support the feasibility of surveillance in stage I immature teratomas. Adjuvant chemotherapy may be reserved for relapses. However, the potential benefit of chemotherapy should be discussed, especially for high-risk tumours. Prospective series are warranted to confirm our findings. To date, no consensus has been reached regarding the role of adjuvant chemotherapy in stage I immature teratomas of the ovary. Some studies suggest that only surveillance is an acceptable choice. However, guidelines are not conclusive on this topic. No difference in terms of recurrence was observed between the surveillance and the adjuvant chemotherapy group. All patients who relapsed were successfully cured with no disease-related deaths. Adjuvant chemotherapy should be appropriately discussed with patients. However, it may be reserved for relapse according to our data.

A neonate presenting with large immature sacrococcygeal teratoma – a challenging case with recurrence and malignant component

A neonate presenting with large immature sacrococcygeal teratoma – a challenging case with recurrence and malignant component

Brain cancer in the ovary? A paediatric immature teratoma showing a focus with morphology of nodular desmoplastic medulloblastoma

Brain cancer in the ovary? A paediatric immature teratoma showing a focus with morphology of nodular desmoplastic medulloblastoma

Immature Sacrococcygeal Teratoma: A Case Report and Extensive Review of the Literature

Immature sacrococcygeal teratoma represents a histological form with rapid tumor growth, a risk of premature birth, an enhanced rate of complications, an increased risk of recurrence, and a higher mortality rate than the mature type. Thus, prenatal diagnosis of immature forms would significantly improve the prognosis of these cases. To this end, we performed an extensive literature review on the diagnosis, therapeutic management, and follow-up of immature teratomas. Regarding this medical conduct, we also presented our case. In conclusion, the early identification of immature sacrococcygeal teratomas with or without other associated structural abnormalities and their correct therapeutic approach are basic principles for a favorable evolution of these cases.

Clinicopathological Evaluation of Extragonadal Germ Cell Tumors - A Retrospective Study from a Regional Cancer Center in India

Introduction: Extragonadal germ cell tumors (EGGCTs) are tumors arising at extragonadal ana-tomical locations. The biological behavior and prognosis of these tumors differ from their gonadal counterparts. Our study aims to analyze their histological subtypes, the outcome, and the prognosis of these lesions at various age groups.Material and Methods: We carried out a retrospective analysis of all patients with EGGCTs diag-nosed from January 2017 to December 2019. Results: Our study included 61 cases of EGGCTs. These cases were divided into neonatal (birth to six months), prepubertal (more than six months to 12 years), and post-pubertal group (>12 years). The most frequently affected group was prepubertal (44.2%). The age ranged from birth to 45 years with a male-to-female ratio of 1:1.03. The frequently involved site was sacrococcyx (36%) followed by mediastinum (31.1%). The common histologic subtype was mature teratoma (39.3%), followed by yolk sac tumor (YST) (18%), mixed germ cell tumor (18%), immature teratoma (14.7%), and germinoma (9.8%). Four cases of teratoma had somatic malignancies. There were 48 stage I, 8 stage II, and 2 stage III tumors. Statistical analysis revealed a significant association of age group concerning primary site (p=0.004), histological type (p=0.002), and clinical stage (p=0.034). In a median follow-up of 30 months, an Overall Survival (OS) of 93.2% and Event Free Survival (EFS) of 90.4% were noted across all age groups. OS of 100% (neon tal), 96% (prepubertal) and 88.0% (post-pubertal) revealing a declining trend (p=0.324) with increasing age was noted. The important prognostic factors include histological subtype (mixed germ cell tumors and teratomas with somatic malignancies had reduced overall survival) and stage (I vs II and III) for OS and stage alone for EFS (P<0.0001). Conclusion: Our study highlights the clinicopathological characteristics, prognostic parameters, and the outcome of GCTs occurring at extragonadal sites and emphasizes the role of pathologists in the detailed reporting of these lesions.

Beyond the Crib: Unraveling the Enigma of Neonatal Nasopharyngeal Mass

Germ cell tumours that develop at birth are rare, with teratomas most frequently occurring in the sacrococcygeal area. Nasopharyngeal teratomas are also rare, occurring in less than 10% of head and neck teratomas. Immature Nasopharyngeal Teratoma (INT) in neonates presents a rare yet challenging clinical scenario. It can manifest with significant respiratory distress and airway compromise, necessitating prompt recognition and intervention. Diagnostic modalities such as imaging studies and biopsy aid in accurate diagnosis and treatment planning. Surgical resection remains the cornerstone of management, with complete excision being essential to optimise outcomes. The present case report highlights the importance of multidisciplinary collaboration and continued research efforts to refine therapeutic approaches and improve patient outcomes. Here, the authors present a rare case of INT in a 51-day-old male child. A 51-day-old male child, normal vaginal delivery and cried immediately after birth with complaints of respiratory distress from 8th day of life, for which he was treated with antibiotics and started on mechanical ventilation. In view of failure of extubation, bronchoscopy was done which revealed a nasopharyngeal mass. Magnetic Resonance Imaging (MRI) showed a heterogeneous mass measuring 2.3×3.9×3.1 cm (anteroposterior x transverse x craniocaudal) involving the parapharyngeal space and carotid space, extending into the retropharyngeal space, with no evidence of intracranial extension. Histopathology showed features of an immature solid teratoma. Nasopharyngeal teratomas are extremely rare congenital tumours, with only a few cases reported in the medical literature. INT in neonates represents a rare yet clinically significant entity that requires prompt recognition and intervention. Multidisciplinary collaboration and comprehensive management strategies are essential to optimise outcomes for affected neonates.

Intriguing Cases of Rare Malignancies of Childhood and Adolescence- A Series of Six Cases

Intracranial teratomas constitute the majority of congenital Central Nervous System (CNS) germ cell tumours. Pure mature or immature teratomas present with giant intracranial masses that cause irreversible brain damage and impair the quality of life. Two such cases are reported here: the first involves a four-month-old female infant with a large right hemispheric lesion, and the second involves a three-month-old female infant with a similar large right hemispheric space-occupying lesion. Both underwent craniotomy but ultimately succumbed to death within a few days after the operation. Immature teratomas of the ovaries are the second most common malignant germ cell tumour following dysgerminoma, with good prognosis and excellent overall survival rates. One such case is presented here, involving a 16-year-old adolescent female with a history of irregular and painful menstruation presenting with a large abdomino-pelvic mass that was determined to be of ovarian origin through imaging studies. Alveolar Soft Part Sarcoma (ASPS) is a rare soft-tissue sarcoma with a poor prognosis, demonstrated in a 17-year-old girl who presented with a painless left thigh mass that was clinically misdiagnosed as a benign intramuscular lipoma. Another case involves a 14-year-old boy with Ewing sarcoma of the ring finger, an extremely rare hand tumour with a high potential for misdiagnosis and delayed treatment, accompanied by a focus of pulmonary metastasis. The final case involves a poorly differentiated carcinoma of the Ileum in a 17- year-old girl who was eventually lost to follow-up checkups.

Teratocarcinosarcoma: A rare malignant tumor of sinonasal tract

Sinonasal teratocarcinosarcoma is an extremely rare malignant tumor arising in the sinonasal tract, having combined histological features of teratoma and carcinosarcoma. Here, we are presenting a case of sinonasal teratocarcinosarcoma in a 44 year‑old male patient. 44 year old male presented to us with complains of nasal obstruction and intermittent epistaxis since 1 month. On examination polypoidal mass was noted in left nasal cavity which bleeds on touch. On MRI, well defined heterogeneously enhancing lesion completely filling the left nasal cavity was noted. Histopathological examination of resected specimen revealed, malignant epithelial components being adenocarcinoma and squamous cell carcinoma, mesenchymal component composed of spindle cells, along with primitive mesenchymal and blastemal components. Immunohistochemistry showed epithelial and stromal markers positive for Cytokeratin and Vimentin respectively. Sinonasal teratocarcinosarcoma (TCS) is a rare and aggressive malignant neoplasm characterized by the combination of malignant teratoma and carcinosarcoma. Most common presentation being nasal obstruction and epistaxis with male preponderance. The tumor shows heterogeneous morphology with varying proportions of benign and malignant epithelial, mesenchymal, and blastemal components. Less than 100 cases have been reported in literature. Teratocarcinosarcoma is a rare aggressive sinonasal tumor. Total excision of the tumor and aggressive sampling for histopathological examination is necessary to avoid misdiagnosis of olfactory neuroblastoma and carcinosarcoma.

Immature Teratoma of Ethmoid Sinus Masquerading as Antrochoanal Polyp: A Rare Case Report with Literature Review.

Teratomas are rare neoplasms that arise from totipotent stem cells. Teratomas of the head and neck are extremely rare, constituting about 10% of all cases and usually present in the neonatal period. Extensive literature search has shown that there are only two cases reportedof teratoma of the ethmoid sinus; one as a mature teratoma in a neonate and another was histologically immature teratoma in an adult male (Mwang'ombe et al. in East Afr Med J 79(2):106-107, 2002; Aggarwal et al. in J Postgrad Med 59(2):138-141, 2013). We hereby report the second case of immature teratoma of ethmoid sinus origin in an adult male.

10121-BT-8 CLINICOPATHOLOGICAL ANALYSIS OF CENTRAL NERVOUS SYSTEM GERM CELL TUMORS -387CASES IN THE JCCG COHORT AND THE IGCT CONSORTIUM COHORT

Abstract BACKGROUND Central nervous system germ cell tumors (CNS GCTs) are rare primary tumors that tend to occur in East Asian boys. In Japan, the Japan Children's Cancer Group (JCCG) and the CNS GCT Genome Analysis Consortium (iGCT Consortium) have been accumulating clinical and pathological data on CNS-GCT cases. METHODS Statistical analyses were performed on 178 cases in the JCCG's Solid Tumor Observational Study (2016-2022) and 209 cases in the iGCT Consortium (2011-2021) for age, gender, site of origin, and pathological diagnosis. RESULTS There were 306 primary cases and 44 recurrent cases, 296 (77%) males and 90 (23%) females, with a median age of 13 years, 11 years for JCCG and 16 years for iGCT Consortium, with differences between these two cohorts. The most common pathological diagnosis was germinoma in 200 cases (52%). Non-germinomatous germ cell tumors (NGGCTs) in 167 cases (43%), of which 69 cases (18%) were mixed GCT with germinoma, 34 cases were immature teratoma, mature teratoma in 32 cases (8%), yolk sac tumor in 20 cases (5%), mixed GCT without germinoma in 5 cases (1%), choriocarcinoma in 5 cases (1%), embryonal carcinoma in 2 cases (0.5%). The median age of patients with germinoma was 15 years, while with other histological types was 12 years. The pineal gland was the most common site (208 cases, 56%) and 114 cases (31%) occurred in the neurohypophysis. 38 cases of them were bifocal. Tumor development in the basal ganglia occurred in 40 cases (11%), and multiple lesions were seen in 37 cases (10%). In the neurohypophysis, the male-to-female ratio was 1:1, while in the pineal region was 11:1. In the neurohypophysis, 67% had germinoma and 9% had teratoma, whereas, in the pineal regions, 50% had germinoma and 19% had teratoma. CONCLUSIONS CNS GCT is closely correlated with age, sex, site of origin, and histology.

Variability in Surveillance Strategies Following Resection of Sacrococcygeal Teratoma

IntroductionSurveillance following sacrococcygeal teratoma (SCT) resection varies. The purpose of this study was to describe the clinical characteristics and outcomes of patients undergoing SCT resection and examine current institutional practices to detect recurrence. MethodsA single-institution retrospective review of children who underwent resection of an SCT from January 1, 2010 to December 31, 2020 was performed. Data were summarized and surveillance strategies compared between histopathologic subtypes using nonparametric methods. ResultsThirty six patients (75.0% female) underwent SCT removal at a median age of 8 d. Histopathology revealed 27 mature teratomas (75.0%), eight immature teratomas (22.2%), and one malignant germ cell tumor (2.8%). Median postoperative follow-up was 3.17 y (interquartile range [IQR]: 2.31-4.38 y). Patients had a median of 2.32 clinic visits per year (IQR: 2.00-2.70), alpha-fetoprotein levels were obtained at a median of 2.01 times per year (IQR: 0-1.66), and surveillance imaging was performed at a median of 2.31 times per year (IQR: 0-2.84). Patients with immature teratomas had alpha-fetoprotein laboratories obtained more frequently than patients with mature teratomas (3.10 times/year versus 0.93 times/year, P = 0.001). There was no significant difference in the number of imaging studies obtained between groups. Two patients (5.6%) developed recurrence, which were identified on magnetic resonance imaging at 191 and 104 d postresection, respectively. ConclusionsPostoperative surveillance practices varied widely. Recurrence was noted in a single malignant case in the first year following resection. Multi-institutional studies are needed to determine the optimal surveillance strategy to detect recurrence of SCT.

Long-Term Outcomes of Infantile Sacrococcygeal Teratoma: Results from a Multi-Institutional Retrospective Observational Study in Japan

BackgroundTumor recurrence, anorectal and urinary dysfunction, and lower limb dysfunction after surgery are observed in infantile sacrococcygeal teratoma (SCT). In this paper, a multi-institutional retrospective observational study was conducted to clarify the long-term functional prognosis in Japan. MethodsThis study was conducted using a paper-based questionnaire distributed to 192 facilities accredited by the Japanese Society of Pediatric Surgeons, covering patients who underwent radical surgery at less than 1 year old and who survived for at least 180 days after birth from 2000 to 2019. ResultsA total of 355 patients were included in this analysis. Altman type was I-II in 248 and type III-IV in 107, and the median maximum tumor diameter was 6.1 (range: 0.6–36.0) cm. There were 269 mature teratomas, 69 immature teratomas, and 10 malignant tumors. Total resection was performed in 325, subtotal or partial resection in 27, and surgical complications were noted in 54. The median postoperative follow-up was 6.6 (0.5–21.7) years. Eighty-three patients (23.4 %) had functional sequelae, including 62 (17.5 %) with anorectal dysfunction, 56 (13.0 %) with urinary dysfunction, and 15 (4.2 %) with lower limb motor dysfunction. Recurrence occurred in 42 (11.8 %) at a median age of 16.8 (1.7–145.1) months old. Risk factors for dysfunction included preterm delivery, a large tumor diameter, Altman type III-IV, incomplete resection, and surgical complications. Risk factors for recurrence included immature teratoma or malignancy, incomplete resection, and surgical complications. ConclusionsPostoperative dysfunction was not low at 23.4 %, and 11.8 % of the patients experienced recurrence occurring more than 10 years after surgery, suggesting the need for periodic imaging and tumor markers evaluations in patients with risk factors. It is necessary to establish treatment guidelines for best practice monitoring of the long-term quality of life. Level of evidenceLevel II Retrospective Study.

Development and validation of a nomogram for preoperative prediction of immature teratoma in children with teratoma: a retrospective, multicenter, diagnostic study.

Teratomas are the most common germ cell tumors in children, and histologically classified as mature teratomas (MTs) and immature teratomas (ITs). Preoperative IT identification can affect the surgical approach, the type of procedure, and future possible reproductive health. However, there is no complete diagnostic criterion for ITs nowadays. We aimed to establish and validate a nomogram based on clinical and computed tomography (CT) features for preoperative prediction of ITs in children. We retrospectively reviewed 519 teratoma patients from hospital I for training (n=364) and validation (n=155), and 113 patients from hospital II for external validation. Univariate and multivariate logistic regression analyses were performed on the training set to screen risk factors, including alpha-fetoprotein (AFP), age, gender, tumor site, size, tumor composition, calcification and fat. Then, a nomogram was established based on identified risk factors and validated on the validation set. The performance of the nomogram was evaluated in terms of discrimination, calibration and the clinical usefulness. Multivariate logistic regression showed that tumor composition, AFP, age, calcification and fat were independent risk factors for preoperative prediction of IT. The area under the receiver operating characteristic (ROC) curves (AUCs) for the nomogram on the training set, internal and external validation set were 0.92 (0.88-0.96), 0.91 (0.84-0.97) and 0.92 (0.86-0.97), respectively. The model demonstrated sensitivity of 80%, specificity of 90% at the cut-off value of 0.262. Whatever the set, the calibration curve indicated good calibration. Decision curve analysis (DCA) curves demonstrated that the nomogram had greater net benefits than either the treat-all tactics or the treat-none tactics within a large scope of threshold. The nomogram established based on clinical and CT findings had the favorable accuracy for the preoperative prediction of IT, and may help in clinical decision-making and risk stratification.

#356 : Concurrent Growing Teratoma Syndrome and Gliomatosis Peritonei in Childbearing Woman with Mature Ovarian Teratoma: A Case Report

Gliomatosis peritonei (GP) is a rare clinical condition associated with ovarian teratomas of any grade, in which benign glial implants develop on the peritoneal surface of the abdomen. Growing teratoma syndrome (GTS) is a rare entity defined as the appearance of benign enlarging peritoneal implants that occur during or after chemotherapy for malignant germ cell tumors but are histologically mature teratomas without any malignant component. Complete surgical resection is essential for the diagnosis and treatment of GTS. We report an association scarcely described in the literature to date, in which both GP and GTS were observed in a patient treated with chemotherapy for immature ovarian teratoma. We encountered a patient with suspected concurrent GTS and Gliomatosis peritonei 2 months after chemotherapy, and a second-look fertility-sparing surgery confirmed the diagnosis. A tumor suspected to be GTS was observed in a nulliparous woman in her 20’s immediately after the completion of three cycles of bleomycin, etoposide, and cisplatin therapy after fertility-sparing surgery for stage IIIb ovarian immature teratoma. As ovarian germ cell tumors are characterized by a young onset, fertility-sparing surgery is performed in most cases. Total tumor resection affects the prognosis of GTS; therefore, minimally invasive surgery before tumor growth is advisable.

Right ovarian Endodermal sinus tumor and left ovarian Gonadoblastoma in a young female of Turner’s syndrome: a case report.

Gonadal dysgenesis includes variety of clinical condition due to abnormalities of sex chromosomes. The most frequent of this condition is Turner’s syndrome, affecting 1 in every 2000 to 2500 live births. Gonadoblastoma is a benign lesion mostly detected in individuals with dysgenetic gonads with Y chromosome and has potential for malignant transformation; or may be associated with malignant germ cell tumors, most commonly dysgerminoma or occasionally immature teratoma, endodermal sinus tumor, embryonal carcinoma, or choriocarcinoma. We report a 22-year-old girl with primary amenorrhea and physical stigmas of Turner’s syndrome presented with huge abdominopelvic mass and abdominal pain. She underwent laparotomy with TAH with BSO with appendectomy with omentectomy with resection of portion of terminal ileum with ileoileal anastomosis with resection of sigmoid colon with colorectal anastomosis and right pelvic lymph node dissection. Patient had anastomotic site leak with sepsis with multi organ dysfunction syndrome and could not be revived. Final histopathology was consistent with endodermal sinus tumor in right ovary and gonadoblastoma in left ovary. Her karyotype analysis revealed 45, X0. Endodermal sinus tumor is a highly malignant gem cell tumor with poor prognosis. Gonadoblastoma associated with endodermal sinus tumor in Turner’s syndrome is very rare and challenging for the clinical management.

The Irish Experience with Sacrococcygeal Teratomas: Are Type IV Lesions More Common than We Think?

Sacrococcygeal teratomas (SCTs) are rare tumors occurring in approximately 1 in 35,000 to 40,000 live births. The Altman classification is used to describe SCTs. There are four types, with type 1 predominantly external through to type IV which is a presacral, completely internal mass. As far as the authors are aware, this is the first study to focus on type IV SCT lesions. Using ICD-10-AM (The International Statistical Classification of Diseases and Related Health Problems, Tenth Revision, Australian Modification) codes, we identified all patients in the Republic of Ireland with a diagnosis of SCT from 2004 to 2020. The following information was obtained for each patient: gender, time of diagnosis, clinical presentation, method of diagnosis, Altman classification, biomarkers, age at operation, surgical technique, pathology, recurrence, and age at most recent follow-up. There were 29 patients in total; 23 females (79%) and 6 males (21%). In total, 16 (55%) were diagnosed antenatally, 4 (14%) at less than 1 month, 4 (14%) less than 1 year, 3 (10%) age 2 to 4 years, and 2 (7%) were aged 5 to 12 years. In addition, 22 (76%) were mature teratomas, 2 (7%) immature teratomas, and 5 (17%) were malignant tumors. There were 6 (21%) type I lesions, 9 (30%) type II, 6 (21%) type III, and 8 (28%) type IV lesions. In Altman's original 1974 study, type IV lesions were present in 10% of cases. As a result, this is the most frequently quoted figure. Type IV lesions were present in 28% of cases in our study. We propose that type IV lesions may be more common than the current literature suggests and consequently a higher index of suspicion of their presence should be entertained.

Prognostic comparison of active surveillance and adjuvant chemotherapy in the treatment of patients with stage Ⅰ ovarian immature teratoma after fertility-sparing surgery

Objective: To compare the survival outcomes between surveillance and adjuvant chemotherapy in patients with stage Ⅰ ovarian immature teratoma (IMT) underwent fertility-sparing surgery. Methods: Clinical and pathological records of patients with stage Ⅰ ovarian IMT between Jan. 2011 to Feb. 2023 were collected from Peking Union Medical College Hospital, except stage Ⅰa grade 1. The consultation of risks and benefits regarding adjuvant chemotherapy was conducted by gynecologic oncologists. A shared decision about surveillance or chemotherapy was made by physician and patients or their guardians. Patients who finally decided to undergo surveillance were included in the surveillance group (n=40), the others were included in the adjuvant chemotherapy group (n=63). Clinical characteristics, treatment and survival outcomes were analyzed and compared between two groups. Results: A total of 103 patients were included. The median age of initial diagnosis was 20 years old (range: 3-39 years old), and the median follow-up time was 31 months (range: 1-254 months). The age, International Federation of Gynecology and Obstetrics (FIGO) stage, pathological grade, surgical method, and preoperative and postoperative alpha-fetoprotein levels in the surveillance group and the adjuvant chemotherapy group were similar (all P>0.05). The surgical approach and maximum tumor diameter between two groups were significantly different (all P<0.05). Forty patients of the surveillance group were identified, only one patient with stage Ⅰa grade 2 IMT who underwent cystectomy had malignant recurrence on the same ovary. Another 63 patients received adjuvant chemotherapy after surgery, five patients had malignant recurrence, and two of them died of disease progression after relapsed. There were no significant differences in disease-free survival (DFS;20 vs 36 months) and overall survival (OS; 23 vs 39 months) between the surveillance group and the adjuvant chemotherapy group (follow-up time censored at 72 months; DFS: P=0.325, OS: P=0.278). Conclusions: There are no differences in survival outcomes between patients with stage Ⅰ ovarian IMT underwent adjuvant chemotherapy or not. Active surveillance might be safe and preferable in stage Ⅰ IMT patients underwent complete resection of tumor.