endodermal sinus tumor
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Pure endodermal sinus tumor (EST) of the ovary is an uncommon germ cell tumor characterized by rapid growth. Its occurrence during pregnancy is extremely rare. The case of a 34-year-old woman with EST stage IC diagnosed in the 22nd week of gestation is presented. She received four cycles of cisplatin monotherapy during pregnancy. Further, three cycles of combination chemotherapy (cisplatin, bleomycin, and etoposide) were administered. Twenty-eight months posttreatment the patient was in complete remission with good health. A healthy female infant was delivered by cesarean section in the 35th week of pregnancy. The child showed normal laboratory, pediatric, and neurologic examination at the time of discharge from hospital and during the first and second years of her life. We conclude that the prognosis of EST is very good if patient receives adjuvant therapy. Cisplatin monotherapy seems to be effective and safe during pregnancy.
- MeSH
- bleomycin aplikace a dávkování MeSH
- cisplatina aplikace a dávkování MeSH
- dospělí MeSH
- etoposid aplikace a dávkování MeSH
- kombinovaná terapie MeSH
- lidé MeSH
- nádor z entodermálního sinusu diagnóza terapie MeSH
- nádorové komplikace v těhotenství diagnóza terapie MeSH
- nádory vaječníků diagnóza terapie MeSH
- novorozenec MeSH
- protokoly protinádorové kombinované chemoterapie terapeutické užití MeSH
- těhotenství MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- novorozenec MeSH
- těhotenství MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- Názvy látek
- bleomycin MeSH
- cisplatina MeSH
- etoposid MeSH
SMARCB1 (INI1) deficient sinonasal carcinoma is a recently recognized entity with wide histomorphologic spectrum. We present a case of this carcinoma that contained, in addition to a "common" morphology, scattered foci of yolk sac tumor differentiation. The tumor occurred in paranasal sinuses in a 44-year-old woman. Immunohistochemically, it was diffusely negative for INI1, whereas an expression of yolk sac tumor markers (α-fetoprotein, glypican-3, CDX2) was limited to the yolk sac tumor component. For comparison with the present case, we performed INI1 immunostaining on a series of 11 gonadal germ cell tumors with yolk sac tumor differentiation. All of these cases showed strong and diffuse expression of INI1, in contrast with the present sinonasal tumor. Our findings expand the morphologic spectrum of SMARCB1 (INI1) deficient sinonasal carcinoma. In addition, we show preliminarily that gonadal germ cell tumors with yolk sac tumor differentiation are not SMARCB1/INI1-deficient.
- Klíčová slova
- SMARCB1 (INI1) deficient carcinoma, gonadal mixed germ cell tumor, sinonasal carcinoma, yolk sac tumor,
- MeSH
- buněčná diferenciace MeSH
- dospělí MeSH
- gen SMARCB1 analýza biosyntéza MeSH
- germinální a embryonální nádory metabolismus MeSH
- karcinom metabolismus patologie MeSH
- lidé MeSH
- nádor z entodermálního sinusu patologie MeSH
- nádorové biomarkery analýza MeSH
- nádory sinu maxillaris metabolismus patologie MeSH
- nádory vaječníků metabolismus MeSH
- testikulární nádory metabolismus MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- Názvy látek
- gen SMARCB1 MeSH
- nádorové biomarkery MeSH
- SMARCB1 protein, human MeSH Prohlížeč
BACKGROUND: Yolk sac tumor (YST) is a germ cell tumor. It is primarily located in the gonads but can also occur extragonadally (extragonadal yolk sac tumor - EGYST), most commonly in the pelvis, retroperitoneum or mediastinum. Only a few YSTs of the urachus have been described. CASE REPORT: We present a rare case report of a 37-year-old male with episodes of macroscopic hematuria. The histological specimen obtained by transurethral resection showed a solid, and in some parts papillary infiltrative, high-grade tumor with numerous areas of marked nuclear atypia and clear invasion between the detrusor bundles. Glandular pattern has been observed in only minority of the tumor. Immunohistochemistry showed significant positivity for GPC3, SALL4 and cytokeratins AE1/AE3, while KRT7 and GATA3 were negative. We concluded that the biopsy findings were consistent with urothelial carcinoma with infrequent YST differentiation. In definitive surgical specimens we found a malignant epithelial, glandular and cystically arranged tumor of germinal appearance arising from urachus. The surrounding urothelium was free of invasive or in situ tumor changes. We reclassified the tumor as a urachal YST. CONCLUSION: EGYST was suspected because glandular and hepatoid structures were found, but the presence of these structures should be verified by immunohistochemistry.
- Klíčová slova
- Extragonadal yolk sac tumor, Urachal yolk sac tumor, Yolk sac tumor,
- MeSH
- dospělí MeSH
- lidé MeSH
- nádor z entodermálního sinusu diagnóza metabolismus patologie MeSH
- nádorové biomarkery metabolismus MeSH
- nádory močového měchýře diagnóza metabolismus patologie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- přehledy MeSH
- Názvy látek
- nádorové biomarkery MeSH
This is a case report of a rare finding of an extragonadal yolk sac tumor in a 37-year-old patient who presented with shortness of breath and abdominal bloating. During imaging and staging surgery, the findings were strongly suggestive of an extragonadal advanced tumor presenting with peritoneal dissemination, predominantly affecting omentum, with no clear primary origin. Histology revealed an extragonadal yolk sac tumor in a pure form outside the ovaries. Lacking an obvious origin elsewhere, the tumor was highly suspected to have truly originated from the omentum. The patient underwent surgery and four cycles of chemotherapy consisting of cisplatin, etoposide, and bleomycin. One-year outpatient follow-up thereafter showed no relapse. We herein discuss a possible site of the tumor origin and its development, as well as diagnostic challenges and disease prognosis.
- Klíčová slova
- biopsy, diagnostic imaging, endodermal sinus tumor, female, omentum, ovary, ultrasonography, yolk sac,
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
Primary hepatic yolk sac tumour (YST) is a very rare neoplasm. The authors present a case of a 20-year-old woman with fever and pain in the epigastrium, clinically resembling acute appendicitis along with high levels of serum alpha-fetoprotein. From laparoscopic appendectomy there was bioptically verified a diagnosis of YST. Consequently, there was discovered a tumor in the liver and the patient underwent right hemihepatectomy, hemicolectomy and ovariectomy, from which the YST was disclosed in the liver and in the coecum with spread into regional lymph nodes. While the gonadal, mediastinal and central nervous system origin of the YST was excluded, the diagnosis of the primary hepatic YST could have been established. The exact etiology of primary hepatic YST is still unclear. It has been suggested that these YSTs might originate from the germ cells that escaped during migration course from the yolk sac to the genital ridge during embryogenesis with subsequent malignant transformation or from persistent pluripotent embryonic cells in the liver, which escaped from the influence of differentiation during embryogenesis. This tumour is aggressive with ability to spread into distant sites, where it can lead to the first clinical presentation.
We report 5 patients with cellular ovarian fibromas, which contained heavy depositions of hyaline globules, a remarkable and very conspicuous histologic feature of all tumors. The hyaline globules ranged in size from 1 to 25 microm. Some cells were stuffed with myriads of small hyaline globules 1 to 3 microm in size, whereas in the other cells the globules were up to 25 microm in size. The hyaline globules were stained with periodic acid-Schiff, and were mucicarmine and Alcian blue negative. These hyaline globules were present, at least in small numbers, in all 100 hematoxylin and eosin slides reviewed from all 5 cases in this series. Often the cells containing the hyaline globules showed a signet-ring appearance in the more compact cellular areas. All the tumors in our series appear to have a benign course. The patients were without signs of recurrence and without metastasis 1, 2, 3, and 6 years after the excision of the neoplasms. Follow-up in one of the patients was not available. The distinction of the ovarian fibromas with hyaline globules from the Krukenberg tumors and yolk sac tumor is discussed in the paper.
- MeSH
- diferenciální diagnóza MeSH
- fibrom metabolismus patologie MeSH
- hyalin metabolismus MeSH
- imunohistochemie MeSH
- Krukenbergův nádor patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- nádor z entodermálního sinusu patologie MeSH
- nádory vaječníků metabolismus patologie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
