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Sisli Etfal Hastanesi Tip Bulteni 2023Holt-Oram syndrome (HOS) is characterized by upper-limb defects and congenital heart malformation, and its prevalence is very rarely. Mature cystic teratoma is the most...
Holt-Oram syndrome (HOS) is characterized by upper-limb defects and congenital heart malformation, and its prevalence is very rarely. Mature cystic teratoma is the most common tumor seen in neonates and its most common location is sacrococcygeal region. Diagnosis of a sacrococcygealteratoma should be confirmed by pathology. Surgical resection is the mainstay therapeutic approach of this tumor. Some malformations such as genitourinary system, musculoskeletal anomalies, neural defects, cardiovascular anomalies, and pulmonary disorders associated with this tumor have been reported. Herein, we reported a male neonate diagnosed with HOS associated with sacrococcygealteratoma. To our knowledge, it has been not reported a case with HOS associated with sacrococcygealteratoma. Patients with sacrococcygealteratomas (SCTs) may have multiple and extreme congenital abnormalities; therefore, patients with SCTs should be carefully evaluated clinically, laboratory, and radiologically and it should be also considered that HOS may accompany them.
PubMed: 38268646
DOI: 10.14744/SEMB.2022.02359 -
European Journal of Pediatric Surgery... Jan 2024A fetiform sacrococcygeal teratoma (homunculus) is a highly differentiated subgroup of mature cystic teratoma that resembles a malformed fetus. These tumors originate at...
A fetiform sacrococcygeal teratoma (homunculus) is a highly differentiated subgroup of mature cystic teratoma that resembles a malformed fetus. These tumors originate at the base of the coccyx and may vary in their intrapelvic and extrapelvic extent and location. It is important to differentiate this anomaly from fetus-in-fetu which has a higher degree of structural organization. A 5-day-old neonate presented with a type II sacrococcygeal fetiform teratoma. The mass contained both cystic and solid components. Upon surgical excision and coccygectomy, fully formed bowel was found inside the mass, as well as bones and other well-defined structures. The tumor was confirmed to be fully excised and no malignant or immature features were found on histopathological examination. The patient was last seen growing well with an alpha-fetoprotein of 3.5 μg/L, 14 months after resection.
PubMed: 38259260
DOI: 10.1055/a-2206-4825 -
Journal of Surgical Case Reports Jan 2024Sacrococcygeal teratoma (SCT) in adults is very rare with only a few cases documented in the literature, adult prevalence varies between 1 in 40 000 and 1 in 63 000....
Sacrococcygeal teratoma (SCT) in adults is very rare with only a few cases documented in the literature, adult prevalence varies between 1 in 40 000 and 1 in 63 000. Most SCTs are located either mainly extra-pelvic (types I and II), which are more commonly seen in neonates; however, mainly intra-pelvic tumors (types III and IV) are more typical in adulthood. Extra-pelvic teratomas are extremely rare in adults. When SCT manifests in an adult, it appears as a slow-growing tumor without symptoms and usually manifests after becoming large enough to cause compression symptoms. SCT has a 1-2% probability of malignant transformation. Herein, we report a 20-year-old female, who was diagnosed with lower back swelling since childhood that increased in size over the last 2 months; imaging revealed an extra-pelvic mass. This case describes an atypical scenario for SCT, which was successfully managed with surgery. The histopathology report confirmed the diagnosis.
PubMed: 38250128
DOI: 10.1093/jscr/rjad731 -
Cureus Dec 2023The present study aimed to review the treatment experience and outcomes of Currarino syndrome (CS) complicated with anorectal stenosis to evaluate the current treatment...
PURPOSE
The present study aimed to review the treatment experience and outcomes of Currarino syndrome (CS) complicated with anorectal stenosis to evaluate the current treatment strategies.
METHODS
Seven cases of CS complicated with anorectal stenosis, treated at our hospital between 1998 and 2021, were retrospectively investigated. This is a case series article from a single institution.
RESULTS
In six and three cases and one case, the presacral mass was a mature teratoma, meningocele, and lipoma, respectively. Resection of the lesion was performed in all six cases of mature teratoma, and duraplasty was performed before resection in all three cases of meningocele. Moreover, surgery for anorectal stenosis was performed simultaneously in four patients. Surgery was performed for six cases of anorectal stenosis, with the remaining case relieved by dilation using a metal bougie. The surgical methods used were a partial resection with end-to-end anastomosis, anorectal strictureplasty, pull-through, posterior sagittal anorectoplasty, and cutback after mass resection. Pathological analysis of the anorectal stenoses revealed disorganized and rough smooth muscle fibers and the replacement of the stroma by an increased quantity of collagen fibers.
CONCLUSIONS
The clinical outcomes of CS can be improved by establishing a treatment flow chart and understanding the complicated pathophysiology of the disease.
PubMed: 38226073
DOI: 10.7759/cureus.50512 -
Cancers Dec 2023Congenital tumors are rare and, owing to this rarity, there is limited information on many of them. A total of 839 fetal and postnatal MRI studies performed in the first...
Congenital tumors are rare and, owing to this rarity, there is limited information on many of them. A total of 839 fetal and postnatal MRI studies performed in the first 3 months of life were retrospectively reviewed. They were performed with the use of 1.5 T scanners. Seventy-six tumors were diagnosed based on fetal MRI between 20 and 37 gestational weeks, and 27 were found after birth, from 1 day of age to 3 months of life. Teratomas were the most common tumors in our dataset, mainly in the sacrococcygeal region (SCT), followed by cardiac rhabdomyomas and subependymal giant cell astrocytomas (SEGA) associated with TSC, and neuroblastomas. The group of less common tumors consisted of infantile fibrosarcomas, malignant rhabdoid tumors, mesoblastic nephromas and Wilms tumor, craniopharyngiomas, brain stem gliomas, desmoplastic infantile astrocytoma, choroid plexus carcinoma, glioblastoma, hemangiopericytoma, rhabdomyosarcoma, melanoma, mesenchymal hamartomas of the chest wall and the liver, and juvenile xanthogranuloma, with special consideration of blue rubber bleb nevus syndrome. MRI plays a significant role in further and better characterization of congenital tumors, leading to a correct diagnosis in many cases, which is crucial for pregnancy and neonatal management and psychological preparation of the parents. No diagnosis is impossible and can be absolutely excluded.
PubMed: 38201471
DOI: 10.3390/cancers16010043 -
Journal of Indian Association of... 2023A rare extragonadal mixed germ cell tumor of the sacrococcygeal area presenting with mature thymic tissue in the teratomatous component, a rare finding and the first...
A rare extragonadal mixed germ cell tumor of the sacrococcygeal area presenting with mature thymic tissue in the teratomatous component, a rare finding and the first reported case of such an association.
PubMed: 38173627
DOI: 10.4103/jiaps.jiaps_150_23 -
Cancer Innovation Dec 2023The aim of this study was to review clinical features of adolescent malignant germ cell tumors (MGCTs) in Beijing and analyze the peculiar characteristics of this age...
BACKGROUND
The aim of this study was to review clinical features of adolescent malignant germ cell tumors (MGCTs) in Beijing and analyze the peculiar characteristics of this age group.
METHODS
Clinical characteristics, pathological presentations, and survival outcomes of 34 patients were analyzed retrospectively.
RESULTS
Of 34 patients, 12 girls and 22 boys, 18 (52.9%) had an extra-cranial tumor, including one testicular tumor, five ovarian tumors, one sacrococcygeal tumor, and 11 mediastinal tumors. Histologically, we found immature teratomas ( = 6), yolk sac tumors ( = 5), mixed malignant tumors ( = 5), an embryonic carcinoma ( = 1), and seminoma ( = 1). Three-year event-free survival (EFS) and overall survival (OS) were 48.8% and 62.9%, respectively. Another 16 (47.1%) patients had an intracranial tumor, including nine in the pineal region, five in the suprasellar region, one in basal ganglia, and one in cerebellopontine. All patients had localized disease and an excellent outcome with 3-year EFS and OS of 93.7% and 100%, respectively.
CONCLUSIONS
Adolescent MGCTs are rare with a strong dependence on gender, and the mediastina and pineal region are the most common tumor locations. The prognosis is promising compared with that of other adolescent tumors and MGCTs in other age groups. MGCTs in mediastina have a tendency to companion with other hematological malignancies, and the prognosis is extremely poor in these patients.
PubMed: 38125762
DOI: 10.1002/cai2.87 -
Global Pediatric Health 2023Fetus in fetu is an uncommon medical anomaly characterized by the presence of a malformed fetus within the body of a living twin. Although the retroperitoneum is the...
Fetus in fetu is an uncommon medical anomaly characterized by the presence of a malformed fetus within the body of a living twin. Although the retroperitoneum is the most typical location, occurrences in the sacrococcygeal and dorsolumbar regions are rare. Ongoing debates center around its embryopathogenesis, debating whether it is a highly specialized teratoma or a result of parasitic twinning in a monozygotic monochorionic diamniotic pregnancy. Notably, distinguishing factors such as vertebral bodies and limbs are pivotal in discerning FIF from teratomas. Diagnostic imaging techniques are integral for preoperative diagnosis, with histopathological examination confirming the condition's presence.
PubMed: 38024466
DOI: 10.1177/2333794X231210621 -
Annals of Medicine and Surgery (2012) Nov 2023A sacrococcygeal teratoma (SCT) is a rare embryonal tumor that emerges in the sacrococcygeal area. It affects one in every 35 000-40 000 live births. Herein, we report...
INTRODUCTION AND IMPORTANCE
A sacrococcygeal teratoma (SCT) is a rare embryonal tumor that emerges in the sacrococcygeal area. It affects one in every 35 000-40 000 live births. Herein, we report a case of a substantial SCT in a neonate.
CASE PRESENTATION
A neonate girl from consanguineous parents was delivered by cesarean section with a large mass (18×17 cm) in the sacrococcygeal area. The baby's birth weight was 5 kg, of which 2.5 belonged to the mass. The vital signs were within normal ranges and she had weak movement with bluish peripheral limbs. Oxygen saturation was around 85% for a short period after birth. According to the American Academy of Pediatric Surgical Section, the tumor was type I. After the fifth day of delivery, a complete resection was done through a chevron incision. The patient was put on 'nil by mouth' for about 24 h and given intravenous fluid.
CLINICAL DISCUSSION
The histopathological examination of the surgical specimen confirmed extragonadal immature teratoma. The histological classification of SCT is divided into three types: malignant teratomas (consisting of malignant germ cells); immature teratomas (incompletely differentiated structures with a high risk of malignancy or embryonal components); and mature teratomas (fully differentiated tissues).
CONCLUSION
SCT has rarely been reported as a giant mass. Radiologic examinations in the early stages of pregnancy may be essential to the early diagnosis of the condition.
PubMed: 37915688
DOI: 10.1097/MS9.0000000000001274 -
Surgical Case Reports Oct 2023Sacrococcygeal teratomas (SCTs) are known to cause urological complications, but urethrovaginal (UV) fistula as a complication of SCT is rare. We herein report a case of...
BACKGROUND
Sacrococcygeal teratomas (SCTs) are known to cause urological complications, but urethrovaginal (UV) fistula as a complication of SCT is rare. We herein report a case of SCT with UV fistula and hydrocolpos.
CASE PRESENTATION
A 1-day-old female neonate presented to our department with prominent swelling in the sacrococcygeal region. She was born at 37 gestational weeks via spontaneous vaginal delivery from a 39-year-old woman. The weight of the baby was 2965 g, and her Apgar scores were 4/10 (at 1 and 5 min). An MRI examination confirmed an 11 × 11 cm Altman classification typeII SCT associated with hydrocolpos, a dilated urinary bladder, and bilateral hydronephrosis. When she was 5 days, the SCT was excised totally and a coccygectomy was performed. After the operation, as her urinary output appeared unstable, a cystoscopic examination was performed on the third postoperative day. This revealed that the UV fistula was located approximately 1 cm from the urethral opening. In addition, the proximal urethra was unobstructed and connected to the bladder. The cystoscope allowed for the passage of a urinary catheter through the urethra. After 1 month of catheter placement, she was discharged from the hospital at 57 days of age. Follow-up was uneventful, with neither urinary infection nor retention.
CONCLUSIONS
SCTs are associated with not only trouble with rectal function and lower extremity movement but also urinary complications. The pathogenesis of this UV fistula is thought to be the rapid growth of the SCT that developed in the fetal period, resulting in obstruction of the urethra by the tumor and the pubic bone, which in turn caused urinary retention and the formation of a fistula as an escape route for the pressure. Because SCTs can cause a variety of complications depending on the course of the disease, careful examination and follow-up are necessary.
PubMed: 37903968
DOI: 10.1186/s40792-023-01772-y