Extragonadal Yolk Sac Tumor Research Articles

Extragonadal yolk sac tumor of the head and neck region: A report of two cases

Extragonadal germ cell tumors (EGCTs) are rare and head and neck is a rarer primary site with most tumors being benign exhibiting teratoma as the leading histologic type. Yolk sac tumor (YST) is relatively uncommon in this location; most commonly described in association with a teratoma and rarely solo. We report two male children, aged 1 year 7 months and 3 years 5 months, with YST involving the head and neck region.

Rare case of conus medullaris syndrome from a metastatic yolk sac tumor originating from the mediastinum of an adult male: a case report and review of the literature.

Yolk sac tumors (YST) are malignant neoplasms. They are a subtype of germ cell tumors and typically originate in the gonads although extragonadal origins of such tumors have been described. Yolk sac tumors are commonly found in the gonads of infants and children and are extremely rare in adults. The goal of this case report is to describe the clinical presentation of a rare case of metastatic extragonadal yolk sac tumor originating from the mediastinum and causing an acute conus medullaris syndrome in an adult male. A 47-year old male presented to our emergency department with a one-day history of bilateral lower extremity weakness, urinary retention and bowel incontinence. Imaging revealed a destructive lesion and a burst fracture of the first lumbar vertebral body (L1) with severe spinal canal stenosis. An urgent spinal decompression and fusion was performed. Oncologic workup revealed a yolk sac tumor originating from the mediastinum. His neurologic function, including motor strength, bowel and bladder function improved in the postoperative period. Chemotherapy regimen of ifosfamide, etoposide and cisplatin was administered and radiation therapy was administered to the spine tumor bed. Yolk sac tumors, although rare, should be considered in the long list differential diagnosis of an otherwise healthy male presenting with conus medullaris or spinal cord compression from metastatic disease without evidence of a testicular mass. Prompt diagnosis with urgent decompression of neural structures and stabilization of the spine may result in improvement in neurological function.

Yolk sac tumor of the seminal vesicles: A rare malignant cause of hematospermia

Extra-gonadal yolk sac tumors (YSTs) are rare and generally associated with poor outcomes. Involvement of the seminal vesicles is extremely rare with only one previously described case. We report a case of a primary YST of the seminal vesicles and discuss the management strategy.

Primary extragonadal pure yolk sac tumor in a post-menopausal female

Primary extragonadal pure yolk sac tumor in a post-menopausal female

Primary yolk sac tumor of seminal vesicle: a case report and literature review

BackgroundYolk sac tumor (endodermal sinus tumor) is a rare malignant germ cell tumor arising in the testis or ovary. Extragonadal yolk sac tumor is even rarer and has only been described in case reports. Due to the rarity of the tumors, the appropriately optimal treatment remains unclear. We report a case of yolk sac tumor in the seminal vesicle.CaseA 38-year-old Asian male presented with gross hematuria and hemospermia. Transrectal ultrasound scan showed a solid mass in the left seminal vesicle and the scrotal sonography showed no abnormalities. Bilateral seminal vesicles were resected, and histopathological examination showed a typical pattern of yolk sac tumor (YST). The patient responded poorly to comprehensive treatment of radiotherapy, chemotherapy and surgeries, developed systemic multiple metastases, and died of cachexia one and half years after diagnosis.

Advanced extragonadal yolk sac tumor serially followed up with 18F-fluorodexyglucose-positoron emission tomography and computerized tomography and serum alpha-fetoprotein

Although yolk sac tumors (YSTs) are the second most common malignant germ cell tumor of the ovary, those arising from the omentum or pelvic peritoneum are extremely rare and have no established treatment guidelines. We report a case of a primary YST disseminated throughout the abdomen and associated with a high serum alpha-fetoprotein (AFP) elevation (441 611 ng/ml). Optimal cytoreduction was not achieved in order to preserve the patient's fertility and avoid adjacent organ injury. Residual tumor responded to adjuvant chemotherapy with a sharp decline in AFP levels, and confirmed remission was documented by serial (18)F-fluorodexyglucose-positoron emission tomography and computerized tomography (FDG-PET/CT). In cases of advanced YST with unresectable residual disease, AFP levels combined with FDG-PET/CT scans may be a helpful way to monitor treatment response and assist in treatment planning for a disease that primarily affects young women who may desire to preserve fertility.

Oral extragonadal yolk sac tumor in a patient with Aicardi syndrome: putative origin and differential diagnosis

We report, for the first time, a primary oral presentation of a germ cell yolk sac tumor in a 4-year-old girl with Aicardi syndrome. The diagnosis, differential diagnosis, and histogenesis are discussed.

Yolk Sac Tumor of the Thyroid Gland: A Case Report

Extragonadal yolk sac tumors are uncommon and usually seen in sacrococcygeal, mediastinal, intracranial, and retroperitoneal sites. Yolk sac tumors of the head and neck region are rare, and the few reported cases have arisen in neonates or infants in conjunction with a teratoma or other germ cell tumor subtypes. We report a unique case of a pure yolk sac tumor presenting as a primary lesion in the right thyroid lobe of a 10-year-old girl. The diagnosis was suspected after fine-needle aspiration, and extensive sampling of the thyroidectomy specimen revealed no teratoma or other germ cell tumor. Serum α-fetoprotein levels were markedly elevated 6 days after excision, and imaging disclosed numerous bilateral pulmonary nodules suggestive of metastatic disease but did not reveal a mediastinal mass. The tumor has shown a favorable response to bleomycin, etoposide, and cisplatin chemotherapy. To the best of our knowledge, this is the 1st description of a primary pure yolk sac tumor of the thyroid.

Primary yolk sac tumor of the common bile duct

Yolk sac tumor (YST) is a germ cell neoplasm usually arising from the gonads. Extragonadal YST has been reported in the mediastinum, retroperitoneum, liver, and pelvis. Primary YST of the common bile duct (CBD) has not been previously reported. We describe an 11-month-old girl with a YST arising from the CBD who presented with obstructive jaundice. Imaging studies revealed an intraluminal CBD mass with intrahepatic biliary dilatation and extraluminal infiltration of the head of the pancreas. A complete resection was carried out, and postoperative chemotherapy with cisplatin, etoposide and bleomycin was administered.

RNA-binding protein LIN28 is a marker for primary extragonadal germ cell tumors: an immunohistochemical study of 131 cases

LIN28 has been shown to have an important role in primordial germ cell development and malignant transformation of germ cells in mouse. In this study, we examined the immunohistochemical profile of LIN28 in 131 primary human extragonadal germ cell tumors (central nervous system (CNS) 76, mediastinum 17, sacrococcygeal region 30, pelvis 3, vagina 2, liver 1, omentum 1, and retroperitoneum 1), including the following tumors and/or components: 57 seminomas/germinomas, 10 embryonal carcinomas, 74 yolk sac tumors, 6 choriocarcinomas, 15 mature, and 13 immature teratomas. We compared LIN28 with SALL4 to assess its diagnostic value. To determine its specificity, we examined LIN28 in 406 extragonadal-non-germ cell tumors (103 carcinomas, 91 sarcomas, 9 melanomas, 12 mesotheliomas, 83 lymphomas, 9 plasmacytomas, 82 CNS tumors, and 17 thymic epithelial tumors). The staining was semi-quantitatively scored as 0 (no cell stained), 1+ (0–30%), 2+ (31–60%), 3+ (61–90%), and 4+ (>90%). LIN28 staining was seen in all seminomas/germinomas (3+ in 1 and 4+ in 56), embryonal carcinomas (4+ in all 10), and yolk sac tumors (3+ in 3 and 4+ in 71). Variable LIN28 staining was seen in 5 of 6 choriocarcinomas (1+ to 4+), 8 of 13 immature teratomas (1+ to 2+ in immature elements), and in 1 of 15 mature teratomas (1+). Only 11 of 406 non-germ cell tumors showed 1+ LIN28 staining. Therefore, LIN28 is a sensitive (100% sensitivity) marker for primary extragonadal seminomas/germinomas, embryonal carcinomas, and yolk sac tumors with high specificity. Compared with SALL4, LIN28 demonstrated a similar level of diagnostic sensitivity for seminomas/germinomas and embryonal carcinomas. For primary extragonadal yolk sac tumors, although SALL4 stained all tumors (1+ in 1, 2+ in 2, 3+ in 10, and 4+ in 61), LIN28 stained more tumor cells (mean 95 vs 90%, P=0.03) and was therefore more sensitive. For primary extragonadal yolk sac tumors, combining LIN28 and SALL4 can achieve a higher diagnostic sensitivity than either alone.

Primary Yolk Sac Tumor in Floor of Mouth in a Child

Approximately 1% of all cancers occur in individuals younger than 20 years old. Leukemias, lymphomas, brain and other nervous system tumors, soft tissue sarcomas, and bone, kidney, eye, and adrenal gland cancers are the most common in this age group. Germ cell tumors (GCTs) are a rare and heterogeneous group of tumors that include both benign and malignant subtypes. Approximately 20% of GCTs are malignant and account for 3% of pediatric cancers. GCTs are primarily yolk sac tumors (YSTs), also called endodermal sinus tumors. The most common YSTs have been found in the gonads (ovaries and testes) of young children. Pediatric extragonadal YSTs are very rare, especially those that occur in the head and neck region. Pediatric YSTs have been reported to occur in the head and neck region, including the parotid gland, gingiva, mandible, orbital region, nasopharyngeal region, maxilla, maxillary sinus, ear, and temporal bone. We report on a case of a very rare tumor with the typical pathologic features of primary pediatric YST that was located in the floor of the mouth. To the best of our knowledge, this is the second case found in the floor of mouth; the first case was reported in 1985. We hope to inform our colleagues about this tumor and its clinical and pathologic features.

Sacrococcygeal Yolk Sac Tumor Presenting as Subcutaneous Fluid Collection Initially Treated as Abscess

Malignant extragonadal germ cell tumors, though more common in infants and children, are rare. They occur in the body's midline and may have internal and external manifestations. We report a case of an 11-month-old female with sacrococcygeal extragonadal yolk sac tumor manifesting as a draining subcutaneous nodule after initial treatment as an abscess. Extragonadal germ cell tumors can present with external manifestations confusingly similar to other more benign soft tissue conditions.

Vnegonadnaya opukhol' zheltochnogo meshka v sredostenii v sochetanii s metastazami raka pochki v legkom

The paper describes a case of a rare combination of extragonadal yolk sac tumor with kidney cancer spreading to the lung. It shows a problem in the preoperative diagnosis of germinogenic mediastinal tumor that mimics a metastasis from renal cancer.

Primary Yolk Sac Tumor Arising in the Pancreas with Hepatic Metastasis: a Case Report

Extragonadal yolk sac tumor (YST) is a relatively rare entity. We describe here the case of an extragonadal YST that occurred in the pancreas with hepatic metastasis in an adult woman. The contrast enhanced CT images of the abdomen revealed a heterogeneous, solitary mass occupying the pancreatic neck and body with slightly inhomogeneous contrast enhancement. Two low-density lesions in the liver were also displayed on the CT images. The patient underwent surgery and the diagnosis of YST was pathologically verified.

Extragonadal yolk sac tumor of face in a female infant: A case report

Extragonadal yolk sac tumor of face in a female infant: A case report

Extragonadal Yolk sac Tumor outside of the Midline of the Body: A Case Report of a Child with a Yolk Sac Tumor of the Pontocerebellar Angle

Yolk sac tumor is a rare germ cell neoplasm occurring mainly in the gonads. Extragonadal yolk sac tumor is a very rare malignancy; its main distribution is along the midline of the body at three principal sites: mediastinum, central nervous system and retroperitoneum. Most yolk sac tumors are diagnosed between seven months and three years of age. We report a case of primary yolk sac tumor in a 13-month-old child. The tumor was located in the pontocerebellar angle, an atypical location that may not have suggested a yolk sac tumor as first diagnosis. We want to highlight the importance of performing tumor marker measurements during the first year of life, also for tumors located away from the midline.

Diagnostic Utility of SALL4 in Extragonadal Yolk Sac Tumors

Extragonadal yolk sac tumors (YSTs; primary and metastatic) are rare but are malignant germ cell tumors. Pathologic diagnosis of extragonadal YSTs can be challenging without immunohistochemical markers but markers used for diagnosing these tumors such as placental-like alkaline phosphatase (PLAP), alpha-fetoprotein (AFP), and glypican-3 lack adequate sensitivity and/or specificity. In earlier studies with gonadal germ cell tumors, SALL4 has been identified as a novel diagnostic marker for YSTs and other types of primitive germ cell tumors. Here, we investigated the diagnostic utility of SALL4 in 59 extragonadal YSTs (27 primary sacrococcygeal, 15 primary nonsacrococcygeal, and 17 metastatic) by immunohistochemical staining. We also compared SALL4 with PLAP, AFP, and glypican-3. In addition, we performed immunostains for pancytokeratin, epithelial membrane antigen, and OCT4 in these tumors. Our results showed that all 59 YSTs showed strong pancytokeratin staining (70% tumor cells in 1 case, >90% tumor cells in 58) and 10 (17%) of them also showed focal epithelial membrane antigen staining (<3% tumor cells). All 59 YSTs were negative for OCT4. Strong SALL4 staining was seen in all 59 YSTs (in more than 90% tumor cells in 54 and 70% to 85% tumor cells in 5 YSTs). Only 39 of 59 (66%) YSTs showed positive PLAP staining and the staining was often focal (in less than 30% tumor cells) (28 of 39 cases). Positive AFP staining was seen in the vast majority of YSTs (56 of 59 or 95%); however, 32 (54%) YSTs showed staining in less than 30% tumor cells. Although all 59 YSTs showed positive glypican-3 staining, 18 (30%) showed staining in less than 30% tumor cells, and additional 10 (17%) showed staining in between 30% and 60% tumor cells. In these 59 YSTs, the mean percentage of tumor cells stained with PLAP was 14% (range: 0% to 90%), with AFP 35% (range 0% to 95%), and with glypican-3 57% (range: 1% to 100%), whereas the mean percentage of tumor cells stained for SALL4 was 94% (range: 70% to 100%) (P<0.001). Our results indicate that SALL4 is a novel sensitive (100% sensitivity) diagnostic marker for extragonadal YSTs. SALL4 is a more sensitive marker than PLAP, AFP, or glypican-3 for extragonadal YSTs.

A case report: Primary extragonadal yolk sac tumor of penile shaft in a 2‐year‐old child

A 2-year-old boy, who had the chief complaints of penile swelling and pain, was brought to the hospital by his mother. Penile contusion/trauma was suspected and he was admitted the same day to undergo emergency surgery to eliminate hematoma. The surgery revealed that the origin of the bleeding was not trauma but a tumor lesion of the penile shaft. It was histopathologically identified as a yolk sac tumor and no tumorous lesions were found except that in the penis. Therefore the patient was diagnosed as definitely having a yolk sac tumor originating in the penis. The patient received four cycles of cisplatin, etoposide and bleomycin treatment as adjuvant chemotherapy. Although it was impossible to completely resect the tumor, cisplatin, etoposide and bleomycin chemotherapy was effective and a complete response was achieved. We plan to carefully monitor the patient in the future.

A yolk sac tumor in a female newborn with a subcutaneous metastasis of the abdominal wall

Extragonadal yolk sac tumors (YST) are extremely rare tumors. Only a few case reports can be found in the literature. We report about a female term newborn. After delivery a sacrococcygeal tumor was obvious. Alpha Fetoprotein (AFP) levels were in the upper normal range (91 006 ng/ml). The tumor was completely removed. Histological examinations revealed a typical YST. In the follow-up examinations a subcutaneous metastasis in the abdominal wall was found and excised at the age of 6 weeks. Curative polychemotherapy was induced. After 2 cycles AFP levels were decreased. However, the patient had a local relapse after the 4 cycle of chemotherapy and showed increased AFP levels. Therefore, hyperthermia combined with chemotherapy was applied and a second surgical resection was done. Despite of multiple therapy regime, the patient died due to tumor progress. This case demonstrates an uncommon dissemination of a primary YST. Therapeutic options in neonates are extremely difficult.

PRIMARY EXTRAGONADAL RETROPERITONEAL PURE YOLK SAC TUMOR

We describe a rare case of an extragonadal retroperitoneal pure yolk sac (endodermal sinus) tumor in the paraaortic region. Radiologic investigation, which included abdominal ultrasound ,computed tomography (CT), and magnetic resonance imaging (MRI) showed a large soft tissue mass occupying the inter paraaortic, renal, pancreatic and splenic region. Radionuclide bone scans demonstrated no bone metastases. The serum alpha fetoprotein (α-FP) level was high; however β-HCG was normal. Pathologic examination of the surgical specimen showed extragonadal yolk sac tumor. After postoperative chemotherapy, follow-up CT revealed decreased residual tumoral tissues and α-FP level returned to normal.