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Nonimmune Hydrops Fetalis
Published in Vincenzo Berghella, Maternal-Fetal Evidence Based Guidelines, 2022
Chelsea DeBolt, Katherine Connolly, Mary E. Norton, Joanne Stone
Fetal tumors, such as lymphangiomas; hemangiomas; sacrococcygeal, mediastinal, and pharyngeal teratomas; and neuroblastomas have been associated with NIH [15, 32, 34]. The likely mechanism in these cases is the development of high output cardiac failure due to their vascular nature. In utero treatment has been offered in cases of sacrococcygeal teratoma and open surgery resulted in survival in 6/11 cases and minimally invasive approaches resulted in survival in 6/20 cases [35]. Rhabdomyomas, cardiac tumors often associated with tuberous sclerosis, can obstruct outflow or filling and result in NIH. The associated liver fibrosis can lead to hepatic failure and NIH. Intrapericardial teratomas are usually benign but rapidly growing fetal tumors and are almost always associated with pericardial effusions. Subsequent development of NIH is attributed to the mass effect of the tumor itself, tamponade physiology secondary to the pericardial effusion or a result of a combination of these factors [36].
Currarino Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Currarino syndrome has a favorable prognosis. Early diagnosis and appropriate treatment can reduce life-threatening complications (e.g., meningitis, rectal fistulas, and malignant transformation of sacrococcygeal teratoma) and further improve quality of life for affected patients.
Spinal Abnormalities
Published in Amar Bhide, Asma Khalil, Aris T Papageorghiou, Susana Pereira, Shanthi Sairam, Basky Thilaganathan, Problem-Based Obstetric Ultrasound, 2019
Amar Bhide, Asma Khalil, Aris T Papageorghiou, Susana Pereira, Shanthi Sairam, Basky Thilaganathan
Sacrococcygeal teratoma usually presents as a vascular, semi-solid/semi-cystic tumor at the terminal end of the spine. Sacrococcygeal teratomas are associated with fetal hydrops and polyhydramnios from high-output cardiac failure related to the arterio-venous shunting within the tumor. These tumors are only rarely malignant, and the prognosis tends to be good after resection.
Congenital Sacrococcygeal Neuroblastoma: A Report of Two Cases with Summary of Prior Published Cases
Published in Fetal and Pediatric Pathology, 2022
Soumya Dey, Arindam Ghosh, Kaushik Sil, Kalyani Saha Basu, Uttara Chatterjee
Case-1: A 6 weeks old boy was referred to our hospital with a large sacral mass since birth. Serum alpha-fetoprotein (AFP) and human chorionic gonadotropin (hCG) levels were within normal limits. CT scan of abdomen suggested the possibility of sacrococcygeal teratoma. Other organs including both adrenals were normal (Fig. 1a–c). The mass was excised along with coccyx and on gross examination it was a tan colored, solid mass measuring 6 cm across. Microscopic examination revealed a tumor composed of small round cells with hyperchromatic nuclei, scanty cytoplasm and stippled chromatin arranged in nests. There were rosettes with neuropil in the center of the rosettes. The tumor was stroma poor variety of neuroblastoma with low-intermediate MKI. There was evidence of ganglionic differentiation of more than 5%. On extensive sampling no heterologous elements were identified. The tumor cells were diffusely immunoreactive for synaptophysin and chromogranin and negative for CD99, FLI 1, desmin, myogenin, LCA, TLE 1, CD 117 and OCT 4 (Fig. 2a–d). Based on the morphologic features and IHC findings, the diagnosis of differentiating neuroblastoma was made. On FISH analysis MYCN was not amplified. The tumor was resected completely without any post-operative neurological deficit. No additional treatment including chemotherapy was given to the patient. The baby was discharged on sixth post-operative day without any complaints. On eighteen months follow up, the baby is doing well without any residual disease. Follow up MRI, urinary vanillylmandelic acid (VMA), serum AFP, hCG levels are normal. PET scan did not show any evidence of metastasis at 8 months of follow up.
Late effects in patients with sacrococcygeal teratoma: A single center series
Published in Pediatric Hematology and Oncology, 2018
Salih Güler, Metin Demirkaya, Emin Balkan, İrfan Kırıştıoğlu, Nizamettin Kılıç, Betül Sevinir
Sacrococcygeal teratoma (SCT) is one of the most frequently seen tumors in fetal and neonatal period. Its incidence is one per 30,000–40,000 live births and the female–male ratio is 3:1[1]. It may be caused by any location at midline and sacrum is the most frequent location[2,3]. Sacrococcygeal tumors are generally benign and the follow-up after early surgical excision is sufficient[2]. Delays in diagnosis increase the risk of malignant transformation and adjuvant chemotherapy and rarely radiotherapy may be required[2,4]
Congenital Spinal Lipomatous Malformations. Part 1. Spinal Lipomas, Lipomyeloceles, and Lipomyelomeningoceles
Published in Fetal and Pediatric Pathology, 2020
The ultimate terminology for a closed spinal lipomatous malformation may be of little consequence, yet postoperative communications can address other important findings. Specifically, aside from the fibrolipomatous malformation, evidence of only partial removal of an unexpected dermal sinus tract or of an epidermoid or dermoid cyst brings concern of regrowth with re-tethering symptoms [69]. An infrequent concomitant tumor, which is most likely a benign sacrococcygeal teratoma [64, 70–73], needs to have malignancy ruled out.