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Zhonghua Bing Li Xue Za Zhi = Chinese... Sep 2022
Topics: Endodermal Sinus Tumor; Female; Humans; Mixed Tumor, Malignant; Neoplasms, Germ Cell and Embryonal; Teratoma; Uterus
PubMed: 36097906
DOI: 10.3760/cma.j.cn112151-20220713-00599 -
Pathologie (Heidelberg, Germany) Nov 2022The great variety of pathological patterns in germ cell tumours, especially in yolk sac tumours but also the possibility of somatic-type malignancies, can complicate... (Review)
Review
The great variety of pathological patterns in germ cell tumours, especially in yolk sac tumours but also the possibility of somatic-type malignancies, can complicate daily diagnosis. For the correct diagnosis, knowledge of morphological aspects and additional immunohistochemical staining can be helpful. Also, rare entities like sex cord stromal tumours, tumours of testicular adnexa or mesenchymal tumours of the spermatic cord can be diagnostically challenging.
Topics: Humans; Male; Consultants; Testicular Neoplasms; Neoplasms, Germ Cell and Embryonal; Sex Cord-Gonadal Stromal Tumors; Endodermal Sinus Tumor
PubMed: 36069909
DOI: 10.1007/s00292-022-01103-2 -
Internal Medicine (Tokyo, Japan) Mar 2023Yolk sac tumor (YST) is a rare primary brain tumor that occurs almost exclusively in patients under 30 years old. Intracranial germ cell tumors are most frequently... (Review)
Review
Yolk sac tumor (YST) is a rare primary brain tumor that occurs almost exclusively in patients under 30 years old. Intracranial germ cell tumors are most frequently located in the pineal and suprasellar region. Medulla oblongata YSTs are particularly rare. Extragonadal YSTs may be difficult to diagnose because of their characteristics, such as the rarity and variety of growth patterns. Furthermore, they are known to have a very poor prognosis. We herein report a case of YST of the medulla oblongata in a 50-year-old woman. She was followed up for 18 months without any tumor recurrence.
Topics: Female; Humans; Middle Aged; Adult; Endodermal Sinus Tumor; Neoplasm Recurrence, Local; Neoplasms, Germ Cell and Embryonal; Medulla Oblongata; Skull
PubMed: 35989277
DOI: 10.2169/internalmedicine.9447-22 -
Orbit (Amsterdam, Netherlands) Dec 2022The aim of this study is to report two cases of paediatric Yolk sac tumours (YST) of the orbit and sinonasal tract, with a major review on the subject. (Review)
Review
PURPOSE
The aim of this study is to report two cases of paediatric Yolk sac tumours (YST) of the orbit and sinonasal tract, with a major review on the subject.
METHODS
Two case reports along with a comprehensive retrospective literature review of all English language publications between 1974 and 2021 is presented. Literature review examined the demographics, clinical presentation and diagnostic and prognostic factors of extragonadal YSTs of the orbit and sinonasal tract.
RESULTS
Orbit and sinuses are rare sites for YST, with only 25 paediatric cases reported in the literature. Extragonadal yolk sac tumours carry a significantly worse outcome than those localised to the gonads, with the 5-year survival of 66% and 81-89%, respectively. Our review found the median age of presentation to be 18 months (18 months for males and 24 months for females), and females are more commonly affected. The most common presentations were proptosis, facial swelling and ophthalmoplegia. Treatments and therefore outcomes varied in the cases due to the large time period. Of the cases reported in the last 10 years, all patients with data provided were alive and disease-free at follow-up.
CONCLUSION
Sino-orbital yolk sac tumours are rare and have variable presentations, dependent on the extent of local invasion. Early diagnosis and treatment with multimodal therapy are paramount in having improved overall survival.
Topics: Male; Female; Humans; Child; Infant; Endodermal Sinus Tumor; Retrospective Studies; Combined Modality Therapy; Exophthalmos; Paranasal Sinuses
PubMed: 35938374
DOI: 10.1080/01676830.2022.2106375 -
Journal of the College of Physicians... Aug 2022The testicular tumour is the most common solid malignancy in males between the ages of 15 and 35 years. Testicular tumours most commonly present with a painless...
The testicular tumour is the most common solid malignancy in males between the ages of 15 and 35 years. Testicular tumours most commonly present with a painless testicular mass. Fournier's gangrene is necrotising fasciitis of the genital, perineal, and perianal region characterized by the microvascular thrombosis and skin necrosis, and is most commonly seen in elderly males with the comorbid conditions. To the best of our knowledge, there is no published case of testicular tumour presenting as Fournier's gangrene. Herein, we report a case of a young adult male, otherwise healthy, who presented to the emergency room with Fournier's gangrene and was found to have a metastatic post-pubertal pure yolk-sac tumour of the testis which is extremely rare in the adults. Key Words: Fournier's gangrene, Yolk sac tumour, Testicular neoplasm.
Topics: Adolescent; Adult; Aged; Endodermal Sinus Tumor; Fournier Gangrene; Humans; Male; Testicular Neoplasms; Young Adult
PubMed: 35932138
DOI: 10.29271/jcpsp.2022.08.1073 -
Pathologie (Heidelberg, Germany) Nov 2022Germ cell tumors (GCTs) are the most common type of cancer in Germany in young men between 15 and 44 years of age. The routinely performed diagnostic procedures are... (Review)
Review
BACKGROUND
Germ cell tumors (GCTs) are the most common type of cancer in Germany in young men between 15 and 44 years of age. The routinely performed diagnostic procedures are essential for the patient's treatment, but can be difficult due to heterogenous histologies. Additionally, the molecular mechanisms of the development of the special forms growing teratoma syndrome (GTS) and testicular tumors with malignant somatic transformation (MST) as well as of therapy resistance are not fully understood.
OBJECTIVES
Updated understanding of the molecular processes underlying GCT development and their special forms as well as recommendations for new and useful biomarkers.
RESULTS
The development of GCTs is a dynamic process largely influenced by the microenvironment. Seminomas (SEs) in particular seem to posses a higher cellular plasticity than previously assumed, allowing SEs to be reprogrammed into an embryonal carcinoma (EC) or differentiate into extra-embryonal tissues (yolk sac tumors [YSTs], trophoblastic differentiation). Novel serological (mi371a-3p) and pathological (FOXA2) biomarkers are well suited to early detect GCTs and YSTs, respectively. For more aggressive tumors and special cases (GTS, MST), there are still no reliable diagnostics or specific/tailored therapies available.
CONCLUSION
The ability of SEs to transit into EC or YSTs should be considered during therapy. Future research should focus on deciphering the special forms GTS and MST as well as the early recognition of YSTs, since their development seems to be an escape mechanism to chemotherapy.
Topics: Humans; Male; Immunohistochemistry; Testicular Neoplasms; Neoplasms, Germ Cell and Embryonal; Seminoma; Carcinoma, Embryonal; Endodermal Sinus Tumor; Biomarkers; Tumor Microenvironment
PubMed: 35925316
DOI: 10.1007/s00292-022-01094-0 -
Medicine Jul 2022Testicular neoplasms are not commonly found in children and are a formidable threat if treated inappropriately. However, there is no consensus regarding its management....
Testicular neoplasms are not commonly found in children and are a formidable threat if treated inappropriately. However, there is no consensus regarding its management. This study aimed to create a holistic picture of the interprofessional team in the management of malignant testicular tumors. Seventeen patients had mixed germ cell tumors, 15 had pure yolk sac tumors, 2 had immature teratomas, 2 had teratocarcinomas, and 1 had a sex cord stromal tumor. Five lesions were diagnosed as nongerm cell tumors: 2 embryonal rhabdomyosarcomas, 2 lymphomas, and 1 acute myeloid leukemia. At initial presentation, retroperitoneal (n = 2), bone marrow (n =1), and mediastinal (n = 1) metastases were identified in 4 (10%) patients. The operative interventions performed included radical inguinal orchiectomy (n = 5), scrotal orchiectomy (n = 31), and testicular biopsy or testis-sparing enucleation of the tumor (n = 6). Postoperatively, 18 patients received either adjuvant chemotherapy (n = 14) or chemoradiation (n = 5). Five patients with mixed germ cell tumors (n = 2), group IV paratesticular rhabdomyosarcoma (n = 2), and acute myeloid leukemia with myeloid sarcoma (n =1) died of disease progression. Thirty-six patients remained alive and disease-free at the last visit. Malignant testicular tumors in children deserve proper diagnostic support from a therapeutic perspective. Any concern or suspicion of a testicular tumor warrants an inguinal approach to avoid scrotal violation.
Topics: Child; Endodermal Sinus Tumor; Humans; Male; Neoplasms, Germ Cell and Embryonal; Orchiectomy; Teratoma; Testicular Neoplasms
PubMed: 35866814
DOI: 10.1097/MD.0000000000029735 -
Medicine Jul 2022Ovarian yolk sac tumor (YST) is a very rare malignant tumor in young women. This study aimed to explore the clinicopathological prognostic characteristics and... (Review)
Review
Clinicopathological features, prognosis, and fertility outcomes in Chinese Han women treated for ovarian yolk sac tumor: A retrospective case series study from two tertiary-care academic medical centers.
OBJECTIVE
Ovarian yolk sac tumor (YST) is a very rare malignant tumor in young women. This study aimed to explore the clinicopathological prognostic characteristics and reproductive outcomes of Chinese Han patients.
METHODS
To describe a case series study, we reviewed the clinicopathological data of 50 YST patients treated from 2 tertiary medical academic medical centers from January 2009 to December 2019. The Akaike information criterion was used to select variables. The influence of relevant characteristics on prognosis factors was analyzed by the Cox proportional hazard model.
RESULTS
The median follow-up time was 64.5 months (range from 3 to 124 months). The median age was 22.7 years (3 to 34 years). Abdominal pain (54.0%) or mass (42.0%) were the most common clinical symptoms in the early stage of diagnosis. The tumors were located bilaterally in 4 cases. 27 patients, 7 patients, 13 patients, and 3 patients were in stage I, II, III, and IV, respectively. Twenty-one stage I patients and 12 stage II to IV patients underwent fertility-preserving surgery. Of the 50 patients who received postoperative chemotherapy, 49 received the BEP regimen. At the last follow-up, 92% of the patients were still alive. The overall survival rate and disease-free survival rate were 91.6% and 90.6%, respectively. Recurrence occurred in 7 (14%) patients with a median survival time of 16.7 months (range from 3 to 50 months). Six patients had recurrence in the abdominal space. The percentage of Ki67 (P = .01) and tumor size (P = .03) were 2 important prognostic factors in multivariate analysis. In terms of survival outcomes, fertility-preserving surgery can be equivalent to radical surgery. Sixteen patients tried to conceive, and 6 patients with advanced-stage succeeded in 10 pregnancies. Of these, 6 patients successfully gave birth to 6 healthy babies.
CONCLUSIONS
The diagnosis of YST of childbearing age is very rare. Because the failure of primary treatment is related to the residual disease after salvage surgery, the fertility and survival results of patients undergoing fertility-preserving surgery are promising.
Topics: Academic Medical Centers; Adult; China; Endodermal Sinus Tumor; Female; Fertility; Humans; Neoplasm Staging; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Pregnancy; Prognosis; Retrospective Studies; Young Adult
PubMed: 35866762
DOI: 10.1097/MD.0000000000029868 -
European Journal of Cancer (Oxford,... Sep 2022Ovarian immature teratoma is a rare subtype of germ cell tumour that can be pure or associated with non-teratomatous germ cell tumour elements and is graded based on... (Review)
Review
Ovarian immature teratoma is a rare subtype of germ cell tumour that can be pure or associated with non-teratomatous germ cell tumour elements and is graded based on extent of the immature neuroectodermal component. Immature teratoma (IT) can also be associated with somatic differentiation in the form of sarcoma, carcinoma, or extensive immature neuroectodermal elements and may produce low levels of serum alpha-fetoprotein. Variable interpretation of these issues underlies diagnostic and management dilemmas, resulting in substantial practice differences between paediatric and adult women with IT. The Malignant Germ Cell International Consortium (MaGIC) convened oncologists, surgeons, and pathologists to address the following crucial clinicopathologic issues related to IT: (1) grading of IT, (2) definition and significance of 'microscopic' yolk sac tumour, (3) transformation to a somatic malignancy, and (4) interpretation of serum tumour biomarkers. This review highlights the discussion, conclusions, and suggested next steps from this clinicopathologic conference.
Topics: Adult; Child; Consensus Development Conferences as Topic; Endodermal Sinus Tumor; Female; Humans; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Teratoma
PubMed: 35863107
DOI: 10.1016/j.ejca.2022.06.006 -
Seminars in Diagnostic Pathology Jan 2023Germ cell neoplasia in situ (GCNIS) is the precursor of both seminomatous and non-seminomatous germ cell tumors. It consists of distended tubules that may have either... (Review)
Review
Germ cell neoplasia in situ (GCNIS) is the precursor of both seminomatous and non-seminomatous germ cell tumors. It consists of distended tubules that may have either intratubular seminoma or intratubular embryonal carcinoma cells. Many invasive non-seminomatous tumors contain a mixture of tumor types, which are reviewed here. Morphology, aided by a panel of immunostains, can determine the presence and percent of embryonal carcinoma, yolk sac tumor, choriocarcinoma, or teratoma in such tumors. Use of immunostains, required for diagnosis in perhaps 25% of testicular neoplasms, is reviewed. Changes of classification in the AJCC (8 edition) in 2016 are discussed, including the partitioning of two tumor types: the central role of chromosome 12p amplification allows both teratoma and yolk sac tumor to be divided into prepubertal types (lacking amplification) and post-pubertal types. Occasionally, sex cord-stromal tumors, hematolymphoid tumors, or epididymal adenomatoid tumors enter the differential diagnosis of germ cell neoplasms.
Topics: Male; Humans; Endodermal Sinus Tumor; Neoplasms, Germ Cell and Embryonal; Testicular Neoplasms; Carcinoma, Embryonal; Teratoma; Seminoma
PubMed: 35840444
DOI: 10.1053/j.semdp.2022.07.001