Glypican 3 (GPC3), a membrane-bound heparin sulfate proteoglycan, may play a role in promoting embryonic cell growth and differentiation. GPC3 is mutated in Simpson-Golabi-Behmel syndrome, characterized by tissue overgrowth and an increased risk of embryonal malignancies Glypican 3 is a sensitive, but not a specific, marker for the diagnosis of yolk sac tumours. Preda O, Nicolae A, Aneiros-Fernández J, Borda A, Nogales FF. Comment on Histopathology. 2010 May;56(6):750-7 Ovarian (> 90%) and extragonadal (> 90%) yolk sac tumors (may be focal, Am J Surg Pathol 2009;33:1529), ovarian choriocarcinoma, teratomas (variable, immature > mature, Am J Clin Pathol 2008;130:224), ovarian clear cell carcinoma (Mod Pathol 2009;22:824), placental site trophoblastic tumor (Diagn Pathol 2010;5:64 Immunohistochemical profile of Yolk Sac Tumor (YST): Both glypican 3 and AFP are useful diagnostic markers of yolk sac tumor; however, Glypican 3 is more sensitive than AFP (100% +ve vs 80% +ve). Other positive markers include KIT, SALL4, cytokeratin AE1/AE3, and CAM5.2. GATA-4, a transcription factor, is expressed in pediatric cases
Glypican-3 is a heparan sulfate proteoglycan that is overexpressed in various neoplasms such as hepatocellular carcinoma, malignant melanoma, and testicular yolk sac tumor. Glypican-3 is currently. Urothelial carcinoma was focally positive for GATA3 (GATA-binding protein 3) and p63; SALL4 and glypican-3 were negative. Overall findings supported a yolk sac tumor with a smaller component of squamous cell carcinoma (<1%). Subsequent cystectomy showed similar morphologic features and immunoprofile in addition to foci of urothelial carcinoma. Recent studies suggest that glypican-3 (GPC3), an oncofetal protein expressed in fetal liver and malignant tumors of hepatocytic lineage, is also expressed in germ cell tumors, particularly YST
Glypican-3 shows strong positive cytoplasmic staining in more than 95% of yolk sac tumors, and it has emerged as an additional positive confirmatory stain for the diagnosis. 394 Glypican-3 rarely stains clear cell carcinoma, but, like AFP, it is positive in tumors with hepatoid features, including hepatoid yolk sac tumor, hepatoid carcinoma, and metastatic hepatocellular carcinoma. 394 Yolk sac tumor is positive for broad-spectrum cytokeratins On immunostaining, AE1/AE3 cytokeratin and glypican 3 provided the most intense and diffuse reactivity for solid yolk sac tumor, whereas α-fetoprotein was negative in 38%. CD117 stained 59%, whereas only rare cells in 1 case (3%) were weakly reactive for podoplanin; OCT3/4 was uniformly negative More recently this variant has been found to occasionally produce bile in canalicular-like structures and to stain strongly for both SALL4 and glypican 3, 2 recently described markers of yolk sac tumor. Recognition of hepatoid yolk sac tumor was followed by the description of a potential mimic, primary ovarian hepatoid carcinoma, which, however. Yolk sac tumor is a type of germ cell tumor. Germ cell tumors commonly arise in the gonads but sometimes can occur outside the gonads and are called extragonadal germ cell tumors. The most common testicular tumor in young children under the age of 3 is yolk sac tumor, and it is also known as infantile embryonal carcinoma
al. reported a non-seminoma, teratoma RV tumor in a 32year- Glypican-3 and pancytokeratin are more modern markers that old male; the tumor was not surgically resected but treated are useful in aiding in the diagnosis of a yolk sac tumor albe- with chemotherapy  Immunohistochemistry was conducted on whole tumor sections using HNF1β. SALL4, OCT4, CD30, CDX2, Cytokeratin 19, Glypican 3, and GATA3 were used for classification of the GCT components. Patients were mostly male (39/45), aged 14 months to 49 years, with primary testicular tumors (37/39), or primary mediastinal pure YSTs (2/39) 1 Esheba Oncofetal Protein Glypican-3 Distinguishes Yolk Sac Tumor From Clear Cell Carcinoma of the Ovary. Am J Surg Pathol 2008;32:600 607 Am J Surg Pathol 2008;32:600 607 2 Capurro M, Wanless IR, Sherman M, et al. Glypican-3: a novel serum and histochemical marker for hepatocellular carcinoma All yolk sac tumors showed positive glypican-3 staining (1+ in 6, 2+ in 6, 3+ in 7, and 4+ in 8). The mean percentage of yolk sac tumor cells stained was 84% with SALL4, 45% with alpha-fetoprotein, and 63% with glypican-3 (P<0.01). No non-germ cell tumors showed SALL4 staining
Oncofetal protein glypican-3 distinguishes yolk sac tumor from clear cell carcinoma of the ovary. Esheba GE, Pate LL, Longacre TA Am J Surg Pathol 2008 Apr;32(4):600-7. doi: 10.1097/PAS.0b013e31815a565a
Glypican-3 (GPC3) belongs to a six-member family of membrane-bound heparan sulfate proteoglycans with a gene that is localized to Xq26.2. As a highly expressed protein in fetal tissues, GPC3 is thought to play a role in the regulation of cell growth and differentiation and functions as an oncofetal protein in tumors with high levels of expression Yolk Sac Tumor in the Anterior Mediastinum: A Rare Case. Initially treated empirically for acute bronchitis, this patient came to the ED with a 6-week history of worsening cough, dyspnea, fevers, weakness, and a 20-lb weight loss. Figure 1. Transverse thoracic CT (click to enlarge) Figure 2. Coronal thoracic CT . (90.9%) of the 11 subjects with yolk sac tumors and 6 (60.0%) of the 10 subjects with hepatoblastoma. In addition, 9 (30.0%) of the 30 subjects with Wilms.
Few cases in many other tumor categories are also found positive (positivity found in 10,3% among different tumors (347/3353 cases) and in 76 out of 139 different tumor categories in a large TMA study). Glypican 3. Staining of yolk sac elements in mixed germ cell tumor of testis 10x Glypican 3. Staining of microcystic hepatocellular. It is more sensitive than glypican 3 for diagnosing yolk sac tumor Nuclear staining is expected Seminomas are typically positive Teratomas are invariably negative Board review style answer #1. A. It can be positive in other liver diseases besides hepatocellular carcinoma and hepatoblastoma. It is a specific marker but less sensitive than.
DDX: Yolk sac tumor→Positive Glypican-3, AFP, SALL4; Negative CK7 Dysgerminoma→Positive Oct3/4, SALL4 ; Negative CK7 Serous carcinoma→Positive WT-1, often ER; Diffuse or Null p5 Oncofetal protein glypican-3 distinguishes yolk sac tumor from clear cell carcinoma of the ovary. (CCC) of the ovary is the surface epithelial neoplasm most often confused with primitive germ cell tumors, particularly yolk sac tumor (YST) and dysgerminoma. OCT3/4 has proven to be a sensitive and relatively specific marker for the latter. Gypican‐3 (GPC3) has been recognized as an oncofetal protein in hepatic neoplasms and yolk sac tumors. To characterize a distinct subgroup of gastric carcinoma (GC) expressing GPC3 (GPC3‐GC), primary and metastatic GC tissues were evaluated by immunohistochemistry with special focus on their related entities: hepatoid, clear‐cell, and α‐fetoprotein‐producing GC Glypican-3, an oncofetal protein which can regulate cell growth and differentiation during embryogenesis is also found positive in yolk sac tumors. It might have provided a better sensitivity compared with AFP according to the previous studies [ 9 , 19 ]
In challenging cases, a concise panel of immunomarkers including OCT3/4, AE1/AE3, and glypican-3 can separate these 2 entities.8,22,32,34-36 The first marker labels seminoma but not solid yolk sac tumor, and the latter 2 are positive in solid yolk sac tumors but negative (glypican-3) or mostly negative (AE1/AE3) in seminomas Yolk sac tumor Mixed Germ cell tumor Post-pubertal Usually 20s-30s Yes Aneuploid Frequent gains and losses. Overexpression of isochrome 12p Malignant, but responsive to therapy 3 Spermatocytic Tumor Usually > 50 yrs No Aneuploid No i12p gains Excellent Intratubular non-seminoma→Same concept, but almost exclusively embryonal carcinoma
YST = yolk sac tumor; table based on McKenney JK, Heerema-McKenney A, Rouse RV. Extragonadal germ cell tumors: a review with emphasis on pathologic features, clinical prognostic variables and differential diagnostic features. Adv Anat Pathol 2007, 14:69-92. Non-Thymic Neoplasms that May Arise Primarily in the Thymus. Hematopoieti Histologically, it was a 2.3 cm mixed germ cell tumor with extensive tumor necrosis, comprised 60% embryonal carcinoma, 30% teratoma, and 10% yolk sac tumor. Immunostains were positive for CD30 in the embryonal carcinoma component and positive for glypican-3 in the yolk sac tumor component, confirming the diagnosis ( Figure 3 )
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. a-fetoprotein (AFP) much less sensitive than glypican-3 in solid yolk sac tumors, with both markers being 100% specific in the differential diagnosis versus seminoma. 32 Even giventhe improvedsensitivityof glypican-3 over AFP for solid yolk sac tumor, the intensity of staining in solid patterns of yolk sac tumor is less than it is in the other yolk Key Words: yolk sac tumor, hepatoid yolk sac tumor, teratoma, hepatoid teratoma, hepatocellular carcinoma, germ cell tumors (Am J Surg Pathol 2021;45:127-136) T. he yolk sac tumor (YST) of the testis is known for its divergent differentiation, including the formation of hepatocyte-like tumor cells, so-called hepatoid yolk sac tumor (HYST).
superior to AFP and Glypican-3 in metastatic and extragonadal settings. Key words: ZBTB16, yolk sac tumor, metastatic, extragonadal INTRODUCTION Accurate histologic diagnosis and classification of germ cell tumors (GCTs) is key to informing successful treatment and surveillance strategy. Modern therapeutic approach fo Multidisciplinary team for the diagnosis and treatment of 2 cases of primary intestinal yolk sac tumor. Qiujuan Huang, Changxu Liu, Ruijun Tang, Jie Li, Wei Li, Lei Zhu, Yuhong Guo, Lin Zhang, Tongyuan Qu, Lingyi Yang, Lingmei Li, Yalei Wang, Baocun Sun, Bin Meng, Jie Yan, Wengui Xu, Huilai Zhang, Dengfeng Cao, Wenfeng Cao. Also known as endodermal sinus tumor, testicular yolk sac tumor more frequently occur in children. In adults, they usually present as a part of mix germ cell tumor. AFP is usually elevated in these cases the degree of elevation is correlated with the severity of disease. While the IHC stains and clinical history are all suggestive of yolk sac.
Diagnostic utility of SALL4 in primary germ cell tumors of the central nervous system: A study of 77 cases Kaiyong Mei, Aijun Liu, Robert W. Allan, Peng Wang, Zhaoli Lane, Ty W. Abel, Lixin Wei, Hong Cheng, Shuangping Guo, Yan Peng, Dinesh Rakheja, Min Wang, Joe Ma, Maria M. Rodriguez, Jianping Li, Dengfeng Ca Interleukin-15 Armored Glypican 3-specific Chimeric Antigen Receptor Expressed in T Cells for Pediatric Solid Tumors. Liposarcoma Wilms' Tumor Rhabdoid Tumor Testicular Yolk Sac Tumor Soft Tissue Sarcoma. U.S. FDA Resources. Arms and Interventions. Go t
Yolk sac tumor (endodermal sinus tumor) is the second most common malignant germ cell neoplasm after dysgerminoma. It is a highly malignant neoplasm, with early metastasis and invasion of surrounding structures and hematogenous metastases (frequently to lungs and liver). They occur most frequently in the second and third decades, followed by. Glypican-3 is an oncofetal protein that is expressed in fetal liver and trophoblast. Increased expression in a hepatic tumor may favor a hepatocellular carcinoma, and positive expression may be seen in germ cell tumors, particularly yolk sac tumor and choriocarcinoma
Ghada E. Esheba, Lisa L. Pate, Teri A. Longacre, Oncofetal Protein Glypican-3 Distinguishes Yolk Sac Tumor From Clear Cell Carcinoma of the Ovary, The American Journal of Surgical Pathology, 10.1097/PAS.0b013e31815a565a, 32, 4, (600-607), (2008) Yolk sac tumor is characterized by significantly elevated serum AFP level. Schiller-Duval body is the defining morphologic feature of yolk sac tumor. Yolk sac tumor shows positive immunostaining for AFP, pankeratin, WT1, SALL4, and glypican-3 and demonstrates negative immunoreactivity for OCT 3-4 Positivity for immunohistochemical markers Glypican-3 and SALL4 confirmed pure YST nature of the tumor. The child received six cycles of cisplatin-based chemotherapy with significant reduction in size of the tumor, followed by exenteration of the orbit. Pure yolk sac tumor (YST) of orbit is a rare neoplasm and only a few cases are reported. The tumors formed trabeculae and cords, occasional gland-like structures, and had frequent basement membrane deposits. SALL4 (100%), glypican-3 (100%), CK19 (85%), CDX2 (85%) and villin (75%) were prominently positive; HepPar-1 stained rare single tumor cells (70%) and arginase was infrequently reactive (26%) (patchy in 1 and rare cells in 4) EC, embryonal carcinoma; EGGCTs, extragonadal germ cell tumors; YST, yolk sac tumor. Figure 3. Open in figure viewer PowerPoint. Histological features of mixed EGGCTs. Teratoma is characterizedby the coexistence in the same neoplasm of different mature or immature tissues (A). which also expresses Glypican-3 and SALL4. 51 The diagnosis of.