Hydrocephalus secondary to chemotherapy in a case of prenatally diagnosed giant immature grade 3 sacrococcygeal teratoma: A case report and literature review.

INTRODUCTION: Sacrococcygeal teratoma (SCT) is a rare tumor in the general population, arising from multipotent stem cells. Whereas most of the cases diagnosed postnatally have good prognosis, the rate of mortality and morbidities associated with prenatally diagnosed SCT remain high, with a reported mortality rate of 30% to 50%. The outcome of fetal SCT can be unpredictable, with some cases with slow growth during fetal life, whereas others grow rapidly, causing multiple complications; also, some of these tumor will develop triggering fetal (preterm delivery, high-output cardiac failure, hydrops fetalis, intrauterine death) or maternal complications (distocia, placentomegaly, maternal mirror syndrome-preeclampsia). Even if prenatal criteria seem to define tumors at risk, it can not totally predict postnatal outcome as treatment-related complications can occur.We present a case of giant prenatally detected SCT. The case was diagnosed at 24th week of gestation, and was closely monitored by serial ultrasound. The morphology of the lesion was defined by fetal MRI performed at 25th week of gestation. A baby girl with a huge sacrococcygeal tumor was born and surgery was performed 48 hours later. Pathological examination revealed a grade 3 immature teratoma. Because of the tumor size and pathological aspect, adjuvant chemotherapy was considered. The outcome was complicated by wound infection, sepsis, and subsequent hydrocephalus, induced by chemotherapy-induced immunosuppression. CONCLUSION: Our case emphasizes not only the importance of prenatal monitoring of these cases but also the importance of individualized postnatal management, as unusual and unpredictable complications can occur and affect outcome.

Bifid scrotum and anocutaneous fistula associated with a perineal lipomatous tumor complicated by temporary bilateral cryptorchidism in utero mimicking ambiguous genitalia: 2-D/3-D fetal ultrasonography.

Ambiguous genitalia (AG) is a morphological diagnosis defined as genitalia not typical of a male or female. Findings mimicking AG, such as penoscrotal anomalies, anorectal malformations, and perineal lipomatous tumors, may prevent accurate identification of the fetal sex. We report a case of bifid scrotum and anocutaneous fistula associated with a perineal lipomatous tumor complicated by temporary bilateral cryptorchidism in utero, which were findings mimicking AG. Several perineal anomalies are associated developmental occurrences. In the present case, the combination of bifid scrotum and temporary bilateral cryptorchidism in the male fetus mimicked the combination of clitoromegaly and prominent labia, which are commonly observed in female fetuses. However, serial systemic assessments using prenatal 2-D/3-D ultrasonography and magnetic resonance imaging were unable to detect the anocutaneous fistula and differentiate the perineal lipomatous tumor. This case report suggests that the prenatal detection of perineal abnormalities may warn obstetricians of potentially undetected congenital perineal anomalies.

Going out on a limb for a difficult diagnosis: a case report.

Sacral tumors with fetiform features are rare and pose a diagnostic challenge to the ultrasonologist. Sacrococcygeal teratomas (SCT) and parasitic twins can have very similar sonographic features but have different implications to an affected pregnancy. While postnatal histopathology is ultimately necessary to distinguish between a SCT and a heteropagus twin, certain characteristics, such as the presence of a vertebral column and the pattern and rate of tumor growth, may be useful to guide counseling and management decisions.

Early delivery as an alternative management strategy for selected high-risk fetal sacrococcygeal teratomas.

BACKGROUND: Large, prenatally diagnosed sacrococcygeal teratomas (SCTs) present a formidable challenge because of their unpredictable growth and propensity for complications. In our experience, even with aggressive serial imaging, many fetuses have died under a policy of "watchful waiting." We propose "early delivery" as the best option for selected cases of high-risk fetal SCT. METHODS: The medical charts of all fetuses with SCT followed up at our institution and delivered before 32 weeks of gestation were reviewed for radiologic findings, fetal interventions, delivery information, perinatal inpatient course, and autopsy or discharge report. RESULTS: Between 1996 and 2009, excluding those that underwent fetal surgery, 9 patients with fetal SCT were delivered before 32 weeks of gestation. Four had type I tumors, and 5 had type II tumors. Of the 9 fetuses, 4 survived the neonatal period. The only surviving patient delivered before 28 weeks underwent an ex utero intrapartum therapy procedure. CONCLUSIONS: A significant number of pregnancies complicated by high-risk SCT will manifest signs of fetal or maternal decompensation, or both, between 27 and 32 weeks of gestation. In the absence of fulminant hydrops, preemptive early delivery can be associated with surprisingly good outcomes in appropriately selected fetuses with high-risk SCT.

Supernumerary ovary: embryology.

This paper documents the fourteenth-ever recorded case of supernumerary ovary, and the second-largest reported thus far, a spherical 6 x 5 x 4 cm convoluted mass. Histologic examination revealed a hemorrhagic corpus luteum cyst within normal limits. Proposed mechanisms of formation are discussed, but definitive evidence of origin remains elusive. Although the presence of a supernumerary ovary may be clinically significant, many are overlooked, emphasising the importance of vigilance at laparotomy.

Study on histogenesis of pelvic endometriosis.

The histogenesis of pelvic endometriosis can not be sufficiently accounted for by a single theory. Thus it may be necessary to pressure plural theories for the explanation of their histogenesis. On collected 253 cases of endometriosis, some clinical and pathological studies were carried out and classified to adenomyosis (A) 37%, ovarian and pelvic endometriosis (O.P) 17%, and others (A+O.P) 45%. Age of the group O.P adenomyosis (A) 37%, ovarian and pelvic endometriosis (O.P) 17%, and others (A+O.P) 45%. Age of the group O.P tended to be younger, as compared with other groups. Pathological study revealed that in group (A+O.P), the lesion tended to be younger, as compared with other groups. Pathological study revealed that in group (A+O.P), the lesion in uterine wall was extensive and deep proliferation in many cases, and it was frequently more at least than group A. Especially, it is necessary to distinguish from each other two different cases which showed endometriosis in the ovary and pelvic peritoneum, that is, to distinguish them by the presence or absence of adenomyosis. In the case of group O.P complicated by A, it is thought highly probable that discrete dissemination may take place from the foci of adenomyosis. In the case of group O.P there may be sufficient room for the application of the Sampson's theory or metaplastic theories. In view of conspicuous trait in the age, however, the possibility of lymphogenous dissemination of endometriosis may be valuable.