Primary orbital yolk sac tumor in an infant: A rare entity.

We report a case of pure orbital yolk sac tumor (YST) in an 11-month-old infant, which is a rare entity. The child presented with progressive painless swelling of the right eye and on examination had proptosis, chemosis, and lid edema. Systemic examination was within normal limits. Magnetic resonance imaging (MRI) orbit revealed a lobulated heterogeneously enhancing right retroocular mass extending up to the orbital apex, displacing the optic nerve and eroding the medial orbital wall. Biopsy of the lesion revealed pure YST histology. Serum alpha-fetoprotein (AFP) was markedly raised at 76900 ng/mL. She was started on infant bleomycin etoposide cisplatin (BEP) chemotherapy protocol. There was a good clinical and radiological response. A high index of malignancy is required in young children presenting with orbital proptosis. A multidisciplinary approach and early intervention are essential to save both vision and life.

Primary endodermal sinus tumor originating from the sacral ligament: a case report and review of the literature.

BACKGROUND: Endodermal sinus tumor (EST) is a malignant tumor originating from the ovary or testis. In most case, ultrasound examination shows ovarian mass. But there is a special kind of extra-gonadal endodermal sinus tumor, which occur in organs other than gonads with insidious onset. Here we reported a case of endodermal sinus tumor, which originated from the sacral ligament presenting as an acute lower abdominal pain. CASE PRESENTATION: A 14-year-old girl was admitted to the hospital because of acute lower abdominal pain. The ultrasound showed a mass with 72 mm × 64 mm × 50 mm in Douglas, and there was no abnormality in bilateral ovaries and fallopian tubes. Laparoscopic exploration showed a large amount of blood clots in the pelvic cavity. After removal of the blood, we found rotten fish-like tissue in the left sacral ligament, rapid pathology suggested endodermal sinus tumor. After the operation, we retrospectively examined the value of alpha-fetoprotein (AFP), which was found to be elevated, and post-operative paraffin pathology confirmed the diagnosis. After four cycles of BEP chemotherapy, exploratory laparotomy was performed to remove the visible lesion, but postoperative pathology showed no abnormality. At the one-year follow-up, the patient remained recurrence-free. CONCLUSION: Extra-gonadal germ cell tumors are rarely reported. When young teenagers complain of acute lower abdominal pain with elevated AFP, but there was no lesion in bilateral ovaries and fallopian tubes, we must think about the possibility of endodermal sinus tumors. Accurate diagnosis facilitates complete resection of lesions and improves patient's outcomes.

Primary Endodermal Sinus Tumor of the Intraspinal Cavity: A Case Report and Review of the Literature.

Endodermal sinus tumor (EST) occurs most frequently in the gonads and is relatively rare in other sites, particularly in the spinal cavity. We report a 19-year-old woman who presented with back pain and weakness of both lower extremities who was found to have an EST in the spinal canal cavity. She had severely elevated serum alpha-fetoprotein (AFP) level at presentation. Magnetic resonance imaging (MRI) revealed the mass in the spinal canal. The tumor was excised. Serum AFP returned to normal after three cycles of chemotherapy. We describe the imaging findings and the macroscopic and microscopic features of this rare tumor. EST is a relatively rare malignant germ cell tumor that usually originates in the gonads and has poor prognosis. This is a rare case of the primary EST in the spinal canal. Radiologists need to be aware of the MRI appearance of extragonadal EST.

Primary orbital yolk sac tumor presenting as fungating mass.

Primary yolk sac tumor of the orbit is a rare entity. Orbital involvement is usually seen in young children and proptosis is the commonest presentation. Aggressive orbital involvement and presentation as a fungating mass is rarely seen. We report a case of primary orbital yolk sac tumor with an aggressive presentation that responded well to systemic chemotherapy.

An unexpected intracardiac location of yolk sac tumor.

PURPOSE: In the pediatric population, intracardiac tumors are rare, usually benign, and mostly diagnosed as rhabdomyoma. Yolk sac tumors (YSTs) are a rare malignant type of germ celltumor that typically occurs in gonads. It can also be seen in midline locations but the intracardiac location is extremely rare. METHODS: The case herein comprises an asymptomatic 2.5-year-old girl with a murmur detected under general examination. RESULTS: Echocardiography showed a 3 × 3-cm mass in the right ventricle. Cardiac magnetic resonance imaging revealed a smooth contoured mass in the right ventricle lumen, which was compatible with rhabdomyoma. After surgical resection, the histopathological results showed a YST. This diagnosis was supported by high values of subsequent serum alpha feto-protein. There was no evidence for any other primary location. CONCLUSION: When an intracardiac mass is observed, a YST should be considered. The increase in the alpha feto-protein level can help in the differential diagnosis.

Testicular yolk sac tumour metastasis to the L2 vertebra.

The role of surgery for metastases to the vertebra from yolk sac tumours has not been established. The main treatment for disseminated disease is chemotherapy. We present a man in his 30s with a left orchiectomy for a testicular mixed germ cell tumour with a prominent yolk sac component who, 12 months later, developed an asymptomatic metastasis to the L2 vertebra unresponsive to chemotherapy and radiotherapy. The patient underwent resection of the L2 vertebral body, leaving a small residual tumour anterior to the vertebra attached to the great vessels. Pathology confirmed the diagnosis of a metastatic testicular yolk sac tumour in the vertebra. The postoperative MRI 6 months later demonstrated significant expansion of the tumour at the soft tissues anterior to the expandable titanium cage encasing the great vessels and extending to the paraspinal areas. Additional salvage surgery was not recommended because of the advanced stage of the tumour.

Pineal Yolk Sac Tumor Producing α-Fetoprotein Detected by 68Ga-FAPI PET/MRI.

ABSTRACT: Pineal yolk sac tumors (YSTs) are a rare type of extragonadal YST. They make up a small fraction of all intracranial germ cell tumors and an even small fraction of pineal masses overall. This study reported a case of pineal YST with α-fetoprotein production revealed by 18F-FDG and 68Ga-FAPI PET/MRI. In the PET images, 68Ga-FAPI showed a far better tumor-to-background ratio than 18F-FDG in the pineal YST because there is little 68Ga-FAPI uptake in the brain. This case indicates that 68Ga-FAPI PET/MRI may be a useful tool for evaluating intracranial YST and other types of tumors in central nervous system.

Management of a Primary Retroperitoneal Yolk Sac Tumor.

BACKGROUND Existing literature has detailed occurrences of retroperitoneal yolk sac tumors (YSTs) as the result of metastasis from a primary gonadal site. However, primary retroperitoneal YSTs are extremely rare, thus remaining a challenge to diagnose and treat. We present a complex case of a large primary retroperitoneal YST in a man treated with neoadjuvant chemotherapy followed by surgical resection. CASE REPORT A 31-year-old man presented with a chief symptom of severe lower abdominal pain. Diagnostic imaging revealed a large, rapidly progressing neoplasm in the retroperitoneal region, initially thought to be a sarcoma. However, the pathological results from further biopsies found the mass to be a retroperitoneal YST, which was tethered to a large portion of the small bowel. A testicular ultrasound was used to confirm that the mass was a primary tumor with no origins in the gonads. The tumor progressed to involve several fistulas connected to the small intestine and anterior abdominal wall. The patient was treated with 3 cycles of bleomycin, etoposide, and cisplatin, followed by surgical excision of the residual mass. The patient retained normal gastrointestinal functions, and subsequent imaging revealed no evidence of recurrence 2.5 years following resection. CONCLUSIONS Owing to the rarity of extragonadal primary YSTs, diagnostic and treatment standards have not yet been sufficiently explored. Our case demonstrates that a combination of chemotherapy and surgical resection should be considered for select patients with primary YST in the retroperitoneal region.

Multimodal imaging changed clinical decision-making: A rare mediastinal yolk sac tumor infiltrating the heart and follow-up.

BACKGROUND: Primary yolk sac tumor of the mediastinum is an extremely rare and highly malignant neoplasm, affecting mainly young men. METHOD AND RESULT: This case report describes a 60-year-old man with a mediastinal yolk sac tumor and infiltrating the heart. A large mobile mass was found in the right atrium by transthoracic echocardiography, and myxoma was suspected. However, contrast-enhanced ultrasonography imaging revealed a remarkable enhancement in the mass. Highly metabolized mass was not only found in the right atrium, also in superior vena cava and right superior mediastinum by whole-body positron emission tomography-computed tomography. Supraclavicular lymph node biopsy confirmed the tumor as a primary mediastinum yolk sac. The tumor started to shrink during chemotherapy and AFP levels decreased. This patient significantly benefited from the chemotherapy. CONCLUSION: Multimodal imaging plays a vital role in the diagnostic workup and management for cardiac mass.

Laparoscopic Trachelectomy for Cervix Yolk Sac Tumor in 11-Month-Old Girl: The Youngest Case.

BACKGROUND: Yolk sac tumor (YST) is a malignant entity that often occurs in girls less than 3 years of age and is the most frequent type of primary extragonadal germ cell tumor. CASE: We describe the case of an 11-month-old girl who was referred to our center for vaginal bleeding with evidence of a uterine mass on ultrasonography. Preoperative investigations confirmed YST of the uterine cervix without metastasis. After 4 cycles of systemic chemotherapy, the patient was treated with laparoscopic trachelectomy (fertility-sparing surgery) without perioperative complications. SUMMARY AND CONCLUSION: After 12 months of follow-up, no residual mass was seen. The laparoscopic technique for trachelectomy for uterine cervix YST seems to be feasible and safe in children under 1 year of age.

Yolk Sac Tumour of the Ovary in Pregnancy Treated With Surgery and Chemotherapy: A Case Report.

BACKGROUND: Ovarian carcinoma diagnosed in pregnancy is rare. Treatment should take both mother and fetus into consideration. CASE: We present the case of a patient diagnosed with a stage IC1 yolk sac tumour of the ovary at 15 weeks gestation, who underwent surgical staging and adjuvant chemotherapy during pregnancy. Intrauterine growth restriction was diagnosed and the patient delivered by cesarean at 36 weeks gestation for obstructed labour. CONCLUSION: Yolk sac tumour of the ovary in pregnancy with concomitant chemotherapy is uncommon. Adverse outcomes, including restricted fetal growth, are possible and their identification may help guide timing of delivery.

A Case of Primary Sinonasal Yolk Sac Tumor.

Yolk sac tumor (YST) is a rare and highly malignant germ cell tumor that is extremely rare in the nasal region. Herein, we present a case of biopsy-proven nasal YST admixed with transitional cell carcinoma in a 58-year-old woman. Complementary F-FDG PET/CT revealed the only FDG-avid mass in the right nasal cavity. This case indicates that YST could occur in the nasal region primarily, and F-FDG PET/CT is a useful tool to distinguish between primary and metastatic lesions.

Primary Pancreatic Yolk Sac Tumor Presenting as Diffusely Enlarged Pancreas in Initial 18F-FDG PET/CT.

Yolk sac tumor is a rare and highly malignant germ cell tumor. We report a case of yolk sac tumor primarily in the pancreas in a 32-year-old man. He presented with pancreatitis at presentation with significantly increased serum alpha-fetoprotein (AFP). F-FDG PET/CT revealed diffuse enlargement of the pancreas in the neck, body, and tail portion with homogeneously increased FDG uptake, similar to the change of pancreatitis. The lesion progressively developed to a huge pancreatic mass in the follow-up images, and endoscopic ultrasonography-guided aspiration biopsy of the pancreatic mass confirmed the diagnosis of yolk sac tumor.

Yolk sac tumor of the fourth ventricle in a 3-year-old boy: imaging features.

PURPOSE: Yolk sac tumor (YST) is a rare malignant germ cell tumor and typically occurs in gonads. It is extremely rare in the brain, especially in the fourth ventricle. METHODS: This study reported the case of a 3-year-old boy with intermittent vomiting for 11 days and then intermittent twitch for 5 h. RESULTS: Brain computed tomography showed a slightly higher density mass in the fourth ventricle. Brain magnetic resonance imaging showed an irregular lobulated expansive mass in the fourth ventricle, appearing with the low intensity on T1-weighted images, and uneven hyperintensity on T2-weighted images. The enhanced scan showed inhomogeneous and strong enhancement, with multiple small cyst-like areas of no enhancement. Tortuous tumor vessel also can be seen. The pathological results after surgical resection showed an YST in the fourth ventricle. CONCLUSION: When a mass is observed in the fourth ventricle in children, with uneven signal with hemorrhage and cystic change, inhomogeneous and strong enhancement, and facilitated diffusion on diffusion-weighted imaging, YST should be considered. The increase of α-fetoprotein value can help differential diagnosis.

Imaging in gynecological disease (17): ultrasound features of malignant ovarian yolk sac tumors (endodermal sinus tumors).

OBJECTIVE: To describe the clinical and sonographic characteristics of malignant ovarian yolk sac tumors (YSTs). METHODS: In this retrospective multicenter study, we included 21 patients with a histological diagnosis of ovarian YST and available transvaginal ultrasound images and/or videoclips and/or a detailed ultrasound report. Ten patients identified from the International Ovarian Tumor Analysis (IOTA) studies had undergone a standardized preoperative ultrasound examination, by an experienced ultrasound examiner, between 1999 and 2016. A further 11 patients were identified through medical files, for whom ultrasound images were retrieved from local image workstations and picture archiving and communication systems. All tumors were described using IOTA terminology. The collected ultrasound images and videoclips were used by two observers for additional characterization of the tumors. RESULTS: All cases were pure YSTs, except for one that was a mixed tumor (80% YST and 20% embryonal carcinoma). Median age at diagnosis was 25 (interquartile range (IQR), 19.5-30.5) years. Seventy-six percent (16/21) of women had an International Federation of Gynecology and Obstetrics (FIGO) Stage I-II tumor at diagnosis. Fifty-eight percent (11/19) of women felt pain during the ultrasound examination and one presented with ovarian torsion. Median serum α-fetoprotein (S-AFP) level was 4755 (IQR, 1071-25 303) µg/L and median serum CA 125 level was 126 (IQR, 35-227) kU/L. On ultrasound assessment, 95% (20/21) of tumors were unilateral. The median maximum tumor diameter was 157 (IQR, 107-181) mm and the largest solid component was 110 (IQR, 66-159) mm. Tumors were classified as either multilocular-solid (10/21; 48%) or solid (11/21; 52%). Papillary projections were found in 10% (2/21) of cases. Most (20/21; 95%) tumors were well vascularized (color score, 3-4) and none had acoustic shadowing. Malignancy was suspected in all cases, except in the patient with ovarian torsion, who presented a tumor with a color score of 1, which was classified as probably benign. Image and videoclip quality was considered as adequate in 18/21 cases. On review of the images and videoclips, we found that all tumors contained both solid components and cystic spaces, and that 89% (16/18) had irregular, still fine-textured and slightly hyperechoic solid tissue, giving them a characteristic appearance. CONCLUSION: Malignant ovarian YSTs are often detected at an early stage, in young women usually in the second or third decade of life, presenting with pain and markedly elevated S-AFP. On ultrasound, malignant ovarian YSTs are mostly unilateral, large and multilocular-solid or solid, with fine-textured slightly hyperechoic solid tissue and rich vascularization. © 2020 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology..